This document summarizes the neurologic presentations of several systemic vasculitides:
1) Temporal arteritis (TA) commonly causes headaches, jaw pain, vision loss, and neuropathy. It primarily affects cranial arteries in those over 50.
2) Takayasu arteritis most often presents in young women and causes strokes, aneurysms, and ischemia from occlusion of the aorta and its branches.
3) The document reviews the pathophysiology, diagnostic criteria, and treatments of these two vasculitides as examples of neurologic involvement in systemic inflammatory vessel diseases.
1. Childhood strokes can be ischemic, caused by blocked blood vessels, or hemorrhagic, caused by bleeding. Common risk factors include arteriopathies, hematological disorders like sickle cell disease, and cardiac conditions.
2. Clinical presentation depends on the location of the stroke, with anterior circulation strokes commonly causing weakness or speech problems and posterior circulation strokes causing vision or balance issues.
3. Diagnosis involves neuroimaging like MRI and MRA to identify infarcts and vascular abnormalities, along with blood tests to investigate underlying causes.
4. Acute management focuses on stabilization, while long-term management emphasizes rehabilitation and prevention of recurrence through treatments like anticoagulants or antiplate
Stroke can occur in young adults, accounting for 10-12% of total stroke patients. Risk factors for young adult stroke include diabetes, smoking, chronic renal disease, and hypertension. An analysis of 2004-2015 New York state hospital data found higher rates of ischemic stroke and subarachnoid hemorrhage among young African Americans and women compared to other groups. Both conditions increased in prevalence over time. Ischemic stroke was strongly associated with diabetes, hypertension, and smoking, while subarachnoid hemorrhage was only associated with hypertension. Prevention and treatment of risk factors is important to reduce morbidity and mortality from stroke in young adults.
Cerebral amyloid angiopathy (CAA) refers to the deposition of β-amyloid in the arteries of the cerebral cortex. It is commonly seen in Alzheimer's disease but can also occur in healthy elderly individuals. CAA can cause intracerebral hemorrhage, dementia, or transient neurological symptoms. The deposition damages blood vessels and increases the risk of hemorrhage. Imaging such as CT scans can detect hemorrhages characteristic of CAA, which are often lobar and cortical. Genetic factors like the ApoE genotype can influence the severity and presentation of CAA.
The document discusses several single-gene disorders that can cause ischemic stroke, including:
1. CADASIL, caused by mutations in the NOTCH3 gene, which is characterized by recurrent strokes, dementia, and MRI findings of white matter lesions.
2. Fabry's disease, an X-linked disorder caused by alpha-galactosidase A deficiency, which can cause both large and small vessel strokes in young patients.
3. Sickle cell disease, where strokes typically occur in childhood and are caused by intimal thickening leading to thrombus formation in large arteries or recurrent small subcortical infarcts.
It also briefly mentions other disorders like homocystinuria,
The document provides information on pediatric stroke. It defines stroke and describes the different types that can occur in children, including arterial ischemic stroke, cerebral sinovenous thrombosis, and hemorrhagic stroke. Risk factors and potential causes are discussed for each type. Clinical features may include seizures, weakness on one side of the body, difficulty speaking or swallowing. Diagnosis involves neuroimaging like CT or MRI along with other lab tests. Treatment focuses on neuroprotection, recanalization of blocked vessels, and anticoagulation or antiplatelet therapies to prevent further clotting.
This document discusses posterior reversible encephalopathy syndrome (PRES). It begins by providing the historical background of the condition. It then describes PRES as a clinicoradiological entity characterized by neurological symptoms like headaches and seizures as well as bilateral white matter abnormalities visible on imaging. It discusses various conditions and triggers that can cause PRES and explores the potential pathophysiological mechanisms involved. The document outlines the typical clinical features and radiological patterns seen in PRES. It compares the differential diagnosis of PRES with other conditions and discusses the general management principles of treating underlying causes and controlling blood pressure.
This document provides an overview of pediatric stroke, including:
- Historical accounts of pediatric stroke dating back to the 17th century.
- The main types of pediatric stroke are arterial ischemic stroke (AIS), intracerebral hemorrhage (ICH), and cerebral venous thrombosis (CVT), with a focus on AIS.
- Risk factors for childhood AIS include cardiac disorders, hematological disorders like sickle cell disease, thrombophilias, arteriopathies, neurofibromatosis, transient cerebral arteriopathy, and primary angiitis of the central nervous system.
- Treatment recommendations are based on small trials and expert consensus, with no guidelines recommending acute thrombolysis for pediatric stroke currently
1. Childhood strokes can be ischemic, caused by blocked blood vessels, or hemorrhagic, caused by bleeding. Common risk factors include arteriopathies, hematological disorders like sickle cell disease, and cardiac conditions.
2. Clinical presentation depends on the location of the stroke, with anterior circulation strokes commonly causing weakness or speech problems and posterior circulation strokes causing vision or balance issues.
3. Diagnosis involves neuroimaging like MRI and MRA to identify infarcts and vascular abnormalities, along with blood tests to investigate underlying causes.
4. Acute management focuses on stabilization, while long-term management emphasizes rehabilitation and prevention of recurrence through treatments like anticoagulants or antiplate
Stroke can occur in young adults, accounting for 10-12% of total stroke patients. Risk factors for young adult stroke include diabetes, smoking, chronic renal disease, and hypertension. An analysis of 2004-2015 New York state hospital data found higher rates of ischemic stroke and subarachnoid hemorrhage among young African Americans and women compared to other groups. Both conditions increased in prevalence over time. Ischemic stroke was strongly associated with diabetes, hypertension, and smoking, while subarachnoid hemorrhage was only associated with hypertension. Prevention and treatment of risk factors is important to reduce morbidity and mortality from stroke in young adults.
Cerebral amyloid angiopathy (CAA) refers to the deposition of β-amyloid in the arteries of the cerebral cortex. It is commonly seen in Alzheimer's disease but can also occur in healthy elderly individuals. CAA can cause intracerebral hemorrhage, dementia, or transient neurological symptoms. The deposition damages blood vessels and increases the risk of hemorrhage. Imaging such as CT scans can detect hemorrhages characteristic of CAA, which are often lobar and cortical. Genetic factors like the ApoE genotype can influence the severity and presentation of CAA.
The document discusses several single-gene disorders that can cause ischemic stroke, including:
1. CADASIL, caused by mutations in the NOTCH3 gene, which is characterized by recurrent strokes, dementia, and MRI findings of white matter lesions.
2. Fabry's disease, an X-linked disorder caused by alpha-galactosidase A deficiency, which can cause both large and small vessel strokes in young patients.
3. Sickle cell disease, where strokes typically occur in childhood and are caused by intimal thickening leading to thrombus formation in large arteries or recurrent small subcortical infarcts.
It also briefly mentions other disorders like homocystinuria,
The document provides information on pediatric stroke. It defines stroke and describes the different types that can occur in children, including arterial ischemic stroke, cerebral sinovenous thrombosis, and hemorrhagic stroke. Risk factors and potential causes are discussed for each type. Clinical features may include seizures, weakness on one side of the body, difficulty speaking or swallowing. Diagnosis involves neuroimaging like CT or MRI along with other lab tests. Treatment focuses on neuroprotection, recanalization of blocked vessels, and anticoagulation or antiplatelet therapies to prevent further clotting.
This document discusses posterior reversible encephalopathy syndrome (PRES). It begins by providing the historical background of the condition. It then describes PRES as a clinicoradiological entity characterized by neurological symptoms like headaches and seizures as well as bilateral white matter abnormalities visible on imaging. It discusses various conditions and triggers that can cause PRES and explores the potential pathophysiological mechanisms involved. The document outlines the typical clinical features and radiological patterns seen in PRES. It compares the differential diagnosis of PRES with other conditions and discusses the general management principles of treating underlying causes and controlling blood pressure.
This document provides an overview of pediatric stroke, including:
- Historical accounts of pediatric stroke dating back to the 17th century.
- The main types of pediatric stroke are arterial ischemic stroke (AIS), intracerebral hemorrhage (ICH), and cerebral venous thrombosis (CVT), with a focus on AIS.
- Risk factors for childhood AIS include cardiac disorders, hematological disorders like sickle cell disease, thrombophilias, arteriopathies, neurofibromatosis, transient cerebral arteriopathy, and primary angiitis of the central nervous system.
- Treatment recommendations are based on small trials and expert consensus, with no guidelines recommending acute thrombolysis for pediatric stroke currently
Pediatric stroke can be caused by a variety of factors such as cardiac diseases, infections like varicella, sickle cell disease, moyamoy disease, cerebral sinus thrombosis, and genetic conditions like MELAS. The presentation of pediatric stroke depends on the location and size of the lesion in the brain. Diagnosis involves imaging techniques like CT, MRI, MRA and angiography. Early diagnosis and treatment is important to prevent long term neurological deficits in children.
This document discusses pediatric stroke. It begins with definitions, types, epidemiology, etiology, and pathophysiology of pediatric stroke. The main types are ischemic and hemorrhagic stroke. Risk factors in children include structural heart disease, vasculopathies, hematological disorders, and prothrombotic states. Clinical features can include focal neurological deficits like hemiparesis. Diagnosis involves neuroimaging such as MRI and distinguishing stroke from other conditions. Management aims to prevent recurrence and support rehabilitation.
1. Stroke in children differs from adults, with congenital and developmental risk factors being more common than chronic risk factors. Presentation can also be more subtle.
2. Guidelines recommend brain imaging, preferably MRI, for any child presenting with clinical stroke symptoms. Further vascular imaging and cardiac echocardiography within 48 hours is also advised.
3. A thorough evaluation should include blood tests to check for coagulation disorders, inflammation, infection and other metabolic causes. Prothrombotic factor screening is important to identify inherited risks and guide family screening.
This document discusses various causes and presentations of pediatric stroke. Some key points:
- Arterial ischemic stroke and cerebral venous thrombosis have incidences of 5/100,000/yr and 1 in 2000 newborns respectively. Neonates have a higher risk than older children.
- Common causes of pediatric stroke include cardioembolism from congenital heart defects, arteriopathies like moyamoya disease, hematologic disorders like sickle cell anemia, and various genetic/metabolic conditions.
- Presentations depend on age but can include seizures, motor deficits, headaches, and decreased consciousness. Diagnosis involves imaging like MRI/MRA while treatment depends on the underlying cause but may include
This document discusses pediatric stroke, including definitions, incidence, causes, investigations, management, and prognosis. Some key points include:
- Pediatric stroke can be ischemic or hemorrhagic and has a variety of potential causes including congenital heart disease, sickle cell anemia, infections, and hypercoagulable states.
- Brain MRI is the preferred imaging modality to diagnose stroke in children. Additional tests may include MRA, CTA, echocardiogram, and lab work to investigate underlying conditions.
- Initial management involves supportive care while determining the cause. Long-term treatment depends on the etiology but may include anticoagulation/antiplatelet therapy and management of underlying conditions to
Strokes can occur in children and are usually caused by arterial blockages or venous clots. The incidence of arterial ischemic stroke and cerebral venous thrombosis is approximately 5 per 100,000 children per year. While relatively rare in children, strokes are an important cause of acquired brain injury in newborns and children. The main causes of pediatric strokes include congenital heart defects, infections, vascular abnormalities, genetic conditions, and trauma.
This document discusses stroke in children. Key points include:
- Stroke in children differs from adults and can have developmental, genetic, or environmental causes rather than lifestyle factors.
- Presentation is often subtle with a wide differential diagnosis. Risk factors are multiple and poorly understood.
- Neonates are at highest risk. Incidence of ischemic stroke is around 1 in 4000-5600 term births. Cerebral venous thrombosis accounts for 0.67 cases per 100,000 children per year.
- Evaluation involves detailed history and physical exam looking for signs of bleeding disorders, infections, cardiac abnormalities, or genetic syndromes. Imaging and lab tests are needed to make an accurate diagnosis.
This document discusses paediatric stroke, including its causes, presentation, diagnosis, and management. The main types of paediatric stroke are arterial ischemic stroke, cerebral sinovenous thrombosis, and hemorrhagic stroke. Common causes include arteriopathy, cardiac issues, hematologic disorders, and perinatal factors. Clinical presentation depends on the age of onset. Diagnosis involves neuroimaging such as MRI. Management is often supportive but may include anticoagulation, revascularization procedures, or transfusions depending on the underlying condition. Prognosis depends on factors like age of onset and severity of neurological deficits.
1) Pediatric strokes account for less than 5% of all strokes and affect 2-3 in 100,000 newborns and 12 in 100,000 children under 18 years of age. The annual incidence of pediatric strokes is reported to be between 2.5-2.7 per 100,000 children.
2) Risk factors for pediatric strokes include congenital heart defects, sickle cell anemia, coagulation disorders, and other conditions.
3) Prognosis after a pediatric stroke varies depending on the underlying cause, with 80% of children surviving 10 years after an ischemic stroke though most have residual hemiparesis. Hemorrhagic strokes carry a higher mortality risk than ischemic strokes.
Mini Overview of a Prime Interoceptor: From Basics to Role in Pathologiesasclepiuspdfs
Frequently overlooked is arguably the most important sensor of the organism’s internal environment, the carotid body (CB). In human subjects this structure alone warns the organism when the partial pressure of oxygen in the arterial blood is becoming insufficient to meet the organism’s needs. But the structure is also stimulated by hypercarbia, glucopenia, acidosis, increases in temperature and osmolarity of the arterial blood. Reflex responses generated by a stimulated CB are found in the respiratory, cardiovascular, renal, and endocrine systems. Recent widespread pathologies also involve the CB. A simple, brief overview of this important structure could prove helpful for biomedical investigators focused on other important biomedical issues.
This study reviewed data on 215 young adults aged 18-45 admitted with ischemic stroke or transient ischemic attack (TIA) to a university hospital stroke center between 2005-2010.
The results showed:
- High rates of traditional vascular risk factors like hypertension (20%), diabetes (11%), dyslipidemia (38%), and smoking (34%).
- Extensive diagnostic testing including blood tests, echocardiograms, vessel imaging found relevant abnormalities in 136 of 203 patients on angiography and detected the likely stroke cause in nearly 90% of patients.
- Common causes were cardioembolism (47%), including 17% with patent foramen ovale, and arterial lesions in the middle cerebral artery (23%),
This document is a master's thesis written by Mohamed Ragab Ali Mohamed on microvascular diseases of the brain. It was supervised by Professor Mohamed Yasser Metwally and others from Ain Shams University. The thesis provides an overview of microvascular diseases, including their classification, risk factors, effects on cognition, neuroimaging findings, treatment and prevention. It aims to provide clinicians an updated understanding of microvascular diseases to help with diagnosis and management.
1. Childhood stroke is more common than brain tumors and is among the top 10 causes of death in childhood. The incidence is about 8 per 100,000 children and risk factors include congenital heart disease and prematurity.
2. The most common causes of acute ischemic stroke are arteriopathy, cardioembolism from structural heart disease, and hematological conditions like sickle cell anemia. Diagnosis involves CT, MRI, and angiography. Treatment focuses on antithrombotics and rehab.
3. Hemorrhagic stroke risk factors include vascular malformations, blood disorders, and trauma. Subarachnoid hemorrhage is the most common type. Cerebral sinovenous
Cerebral venous thrombosis (CVT) is an uncommon type of stroke caused by a blood clot in the brain's venous sinuses or veins. It has a significant morbidity. Common presentations include headache, seizures, and long-lasting neurological deficits. Diagnosis is made through imaging studies like MRI and MRV. Treatment involves management of increased intracranial pressure, seizures, and anticoagulation with heparin or thrombolytics to prevent extension of clots. Prognosis depends on factors like impaired consciousness, underlying cause and location of clots. Most patients recover without sequelae, but mortality can be high if left untreated.
This document discusses various rheumatologic emergencies that require prompt treatment to prevent serious complications or death. It defines emergencies as situations that immediately endanger life or organ function. True emergencies have mortality rates around 50% even with timely intervention. Examples of rheumatologic emergencies discussed include septic arthritis, Atlantoaxial dislocation, acute upper airway obstruction in RA, acute transverse myelitis and cerebrovascular accident in SLE, alveolar hemorrhage and pulmonary renal syndrome in vasculitis, scleroderma renal crisis, respiratory muscle weakness in inflammatory myopathies, and catastrophic antiphospholipid syndrome. The document provides details on symptoms, diagnostic evaluation, and treatment approaches for each of
Stroke can occur in children and presents with rapidly developing neurological deficits lasting more than 24 hours. Mortality from pediatric stroke is 6-20% and over 50% of children have residual disabilities. There are several types of stroke in children including hemorrhagic, caused by bleeding, and ischemic, caused by blocked blood vessels. Etiologies of pediatric stroke include various vascular, cardiac, hematologic and other conditions. Aggressive management in the PICU focuses on treating the underlying cause, preventing secondary brain injury, and managing complications.
Stroke is a major cause of death and disability globally. Diagnosis depends on clinical features and brain imaging to differentiate between ischemic stroke and intracerebral hemorrhage. Management of ischemic stroke has advanced with therapies like intravenous thrombolysis and endovascular thrombectomy shown to reduce disability if applied rapidly. Both hemorrhagic and ischemic strokes require identifying risk factors and mechanisms to target prevention interventions, while lifestyle changes are common to reducing risk for all stroke subtypes.
MRI SPECTRUM OF POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROMENirav Kadvani
This document discusses the MRI spectrum of Posterior Reversible Encephalopathy Syndrome (PRES). It presents 9 case studies demonstrating various brain lesions seen in PRES patients, including watershed area lesions, frontal lobe lesions, occipital lobe lesions, splenial lesions, basal ganglia lesions, and cerebellar lesions. The document also reviews the definition, etiology, MRI features, differentials, and typical patterns of PRES lesions. In summary, PRES can involve multiple areas of the brain beyond just the posterior regions, and MRI is useful for evaluating and diagnosing this potentially reversible condition associated with high blood pressure or other predisposing factors.
- Temporal arteritis (TA), also known as giant cell arteritis, is an inflammatory disease that affects medium and large arteries, especially the cranial arteries. It is more common in adults over 50 years of age.
- Left untreated, TA can cause permanent vision loss. Treatment involves high-dose corticosteroids to suppress inflammation. Prednisone is gradually tapered over months or years to prevent relapses.
- Takayasu arteritis is a large vessel vasculitis that predominantly affects women aged 10-30. It causes stenosis and occlusion of the aorta and its main branches. Treatment consists of corticosteroids along with monitoring for vascular complications like strokes.
The idea for the Hope medical camp began in 2003 when Dr. Abdulrahman Al-Shammari spoke to Dr. Hisham Buzurg and the Kuwaiti Hope medical team during a visit to Montreal, Canada. He discussed the great impact that could be achieved by providing direct medical assistance to poor patients in remote parts of Africa. This inspired the team to establish the first Hope surgical camp in Kenya the following year. The camps have since provided critical medical services and performed hundreds of surgeries for those in need in Gambia and other nations.
Rocío is a 14-year-old student and the second daughter in her family of six. She has one brother, Fernando José, and two sisters, María Guadalupe and María Fernanda. Her mother Rocío works as a secretary and her father Fernando is a teacher. Rocío enjoys listening to rap and rock music and her favorite colors are black and taz.
Pediatric stroke can be caused by a variety of factors such as cardiac diseases, infections like varicella, sickle cell disease, moyamoy disease, cerebral sinus thrombosis, and genetic conditions like MELAS. The presentation of pediatric stroke depends on the location and size of the lesion in the brain. Diagnosis involves imaging techniques like CT, MRI, MRA and angiography. Early diagnosis and treatment is important to prevent long term neurological deficits in children.
This document discusses pediatric stroke. It begins with definitions, types, epidemiology, etiology, and pathophysiology of pediatric stroke. The main types are ischemic and hemorrhagic stroke. Risk factors in children include structural heart disease, vasculopathies, hematological disorders, and prothrombotic states. Clinical features can include focal neurological deficits like hemiparesis. Diagnosis involves neuroimaging such as MRI and distinguishing stroke from other conditions. Management aims to prevent recurrence and support rehabilitation.
1. Stroke in children differs from adults, with congenital and developmental risk factors being more common than chronic risk factors. Presentation can also be more subtle.
2. Guidelines recommend brain imaging, preferably MRI, for any child presenting with clinical stroke symptoms. Further vascular imaging and cardiac echocardiography within 48 hours is also advised.
3. A thorough evaluation should include blood tests to check for coagulation disorders, inflammation, infection and other metabolic causes. Prothrombotic factor screening is important to identify inherited risks and guide family screening.
This document discusses various causes and presentations of pediatric stroke. Some key points:
- Arterial ischemic stroke and cerebral venous thrombosis have incidences of 5/100,000/yr and 1 in 2000 newborns respectively. Neonates have a higher risk than older children.
- Common causes of pediatric stroke include cardioembolism from congenital heart defects, arteriopathies like moyamoya disease, hematologic disorders like sickle cell anemia, and various genetic/metabolic conditions.
- Presentations depend on age but can include seizures, motor deficits, headaches, and decreased consciousness. Diagnosis involves imaging like MRI/MRA while treatment depends on the underlying cause but may include
This document discusses pediatric stroke, including definitions, incidence, causes, investigations, management, and prognosis. Some key points include:
- Pediatric stroke can be ischemic or hemorrhagic and has a variety of potential causes including congenital heart disease, sickle cell anemia, infections, and hypercoagulable states.
- Brain MRI is the preferred imaging modality to diagnose stroke in children. Additional tests may include MRA, CTA, echocardiogram, and lab work to investigate underlying conditions.
- Initial management involves supportive care while determining the cause. Long-term treatment depends on the etiology but may include anticoagulation/antiplatelet therapy and management of underlying conditions to
Strokes can occur in children and are usually caused by arterial blockages or venous clots. The incidence of arterial ischemic stroke and cerebral venous thrombosis is approximately 5 per 100,000 children per year. While relatively rare in children, strokes are an important cause of acquired brain injury in newborns and children. The main causes of pediatric strokes include congenital heart defects, infections, vascular abnormalities, genetic conditions, and trauma.
This document discusses stroke in children. Key points include:
- Stroke in children differs from adults and can have developmental, genetic, or environmental causes rather than lifestyle factors.
- Presentation is often subtle with a wide differential diagnosis. Risk factors are multiple and poorly understood.
- Neonates are at highest risk. Incidence of ischemic stroke is around 1 in 4000-5600 term births. Cerebral venous thrombosis accounts for 0.67 cases per 100,000 children per year.
- Evaluation involves detailed history and physical exam looking for signs of bleeding disorders, infections, cardiac abnormalities, or genetic syndromes. Imaging and lab tests are needed to make an accurate diagnosis.
This document discusses paediatric stroke, including its causes, presentation, diagnosis, and management. The main types of paediatric stroke are arterial ischemic stroke, cerebral sinovenous thrombosis, and hemorrhagic stroke. Common causes include arteriopathy, cardiac issues, hematologic disorders, and perinatal factors. Clinical presentation depends on the age of onset. Diagnosis involves neuroimaging such as MRI. Management is often supportive but may include anticoagulation, revascularization procedures, or transfusions depending on the underlying condition. Prognosis depends on factors like age of onset and severity of neurological deficits.
1) Pediatric strokes account for less than 5% of all strokes and affect 2-3 in 100,000 newborns and 12 in 100,000 children under 18 years of age. The annual incidence of pediatric strokes is reported to be between 2.5-2.7 per 100,000 children.
2) Risk factors for pediatric strokes include congenital heart defects, sickle cell anemia, coagulation disorders, and other conditions.
3) Prognosis after a pediatric stroke varies depending on the underlying cause, with 80% of children surviving 10 years after an ischemic stroke though most have residual hemiparesis. Hemorrhagic strokes carry a higher mortality risk than ischemic strokes.
Mini Overview of a Prime Interoceptor: From Basics to Role in Pathologiesasclepiuspdfs
Frequently overlooked is arguably the most important sensor of the organism’s internal environment, the carotid body (CB). In human subjects this structure alone warns the organism when the partial pressure of oxygen in the arterial blood is becoming insufficient to meet the organism’s needs. But the structure is also stimulated by hypercarbia, glucopenia, acidosis, increases in temperature and osmolarity of the arterial blood. Reflex responses generated by a stimulated CB are found in the respiratory, cardiovascular, renal, and endocrine systems. Recent widespread pathologies also involve the CB. A simple, brief overview of this important structure could prove helpful for biomedical investigators focused on other important biomedical issues.
This study reviewed data on 215 young adults aged 18-45 admitted with ischemic stroke or transient ischemic attack (TIA) to a university hospital stroke center between 2005-2010.
The results showed:
- High rates of traditional vascular risk factors like hypertension (20%), diabetes (11%), dyslipidemia (38%), and smoking (34%).
- Extensive diagnostic testing including blood tests, echocardiograms, vessel imaging found relevant abnormalities in 136 of 203 patients on angiography and detected the likely stroke cause in nearly 90% of patients.
- Common causes were cardioembolism (47%), including 17% with patent foramen ovale, and arterial lesions in the middle cerebral artery (23%),
This document is a master's thesis written by Mohamed Ragab Ali Mohamed on microvascular diseases of the brain. It was supervised by Professor Mohamed Yasser Metwally and others from Ain Shams University. The thesis provides an overview of microvascular diseases, including their classification, risk factors, effects on cognition, neuroimaging findings, treatment and prevention. It aims to provide clinicians an updated understanding of microvascular diseases to help with diagnosis and management.
1. Childhood stroke is more common than brain tumors and is among the top 10 causes of death in childhood. The incidence is about 8 per 100,000 children and risk factors include congenital heart disease and prematurity.
2. The most common causes of acute ischemic stroke are arteriopathy, cardioembolism from structural heart disease, and hematological conditions like sickle cell anemia. Diagnosis involves CT, MRI, and angiography. Treatment focuses on antithrombotics and rehab.
3. Hemorrhagic stroke risk factors include vascular malformations, blood disorders, and trauma. Subarachnoid hemorrhage is the most common type. Cerebral sinovenous
Cerebral venous thrombosis (CVT) is an uncommon type of stroke caused by a blood clot in the brain's venous sinuses or veins. It has a significant morbidity. Common presentations include headache, seizures, and long-lasting neurological deficits. Diagnosis is made through imaging studies like MRI and MRV. Treatment involves management of increased intracranial pressure, seizures, and anticoagulation with heparin or thrombolytics to prevent extension of clots. Prognosis depends on factors like impaired consciousness, underlying cause and location of clots. Most patients recover without sequelae, but mortality can be high if left untreated.
This document discusses various rheumatologic emergencies that require prompt treatment to prevent serious complications or death. It defines emergencies as situations that immediately endanger life or organ function. True emergencies have mortality rates around 50% even with timely intervention. Examples of rheumatologic emergencies discussed include septic arthritis, Atlantoaxial dislocation, acute upper airway obstruction in RA, acute transverse myelitis and cerebrovascular accident in SLE, alveolar hemorrhage and pulmonary renal syndrome in vasculitis, scleroderma renal crisis, respiratory muscle weakness in inflammatory myopathies, and catastrophic antiphospholipid syndrome. The document provides details on symptoms, diagnostic evaluation, and treatment approaches for each of
Stroke can occur in children and presents with rapidly developing neurological deficits lasting more than 24 hours. Mortality from pediatric stroke is 6-20% and over 50% of children have residual disabilities. There are several types of stroke in children including hemorrhagic, caused by bleeding, and ischemic, caused by blocked blood vessels. Etiologies of pediatric stroke include various vascular, cardiac, hematologic and other conditions. Aggressive management in the PICU focuses on treating the underlying cause, preventing secondary brain injury, and managing complications.
Stroke is a major cause of death and disability globally. Diagnosis depends on clinical features and brain imaging to differentiate between ischemic stroke and intracerebral hemorrhage. Management of ischemic stroke has advanced with therapies like intravenous thrombolysis and endovascular thrombectomy shown to reduce disability if applied rapidly. Both hemorrhagic and ischemic strokes require identifying risk factors and mechanisms to target prevention interventions, while lifestyle changes are common to reducing risk for all stroke subtypes.
MRI SPECTRUM OF POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROMENirav Kadvani
This document discusses the MRI spectrum of Posterior Reversible Encephalopathy Syndrome (PRES). It presents 9 case studies demonstrating various brain lesions seen in PRES patients, including watershed area lesions, frontal lobe lesions, occipital lobe lesions, splenial lesions, basal ganglia lesions, and cerebellar lesions. The document also reviews the definition, etiology, MRI features, differentials, and typical patterns of PRES lesions. In summary, PRES can involve multiple areas of the brain beyond just the posterior regions, and MRI is useful for evaluating and diagnosing this potentially reversible condition associated with high blood pressure or other predisposing factors.
- Temporal arteritis (TA), also known as giant cell arteritis, is an inflammatory disease that affects medium and large arteries, especially the cranial arteries. It is more common in adults over 50 years of age.
- Left untreated, TA can cause permanent vision loss. Treatment involves high-dose corticosteroids to suppress inflammation. Prednisone is gradually tapered over months or years to prevent relapses.
- Takayasu arteritis is a large vessel vasculitis that predominantly affects women aged 10-30. It causes stenosis and occlusion of the aorta and its main branches. Treatment consists of corticosteroids along with monitoring for vascular complications like strokes.
The idea for the Hope medical camp began in 2003 when Dr. Abdulrahman Al-Shammari spoke to Dr. Hisham Buzurg and the Kuwaiti Hope medical team during a visit to Montreal, Canada. He discussed the great impact that could be achieved by providing direct medical assistance to poor patients in remote parts of Africa. This inspired the team to establish the first Hope surgical camp in Kenya the following year. The camps have since provided critical medical services and performed hundreds of surgeries for those in need in Gambia and other nations.
Rocío is a 14-year-old student and the second daughter in her family of six. She has one brother, Fernando José, and two sisters, María Guadalupe and María Fernanda. Her mother Rocío works as a secretary and her father Fernando is a teacher. Rocío enjoys listening to rap and rock music and her favorite colors are black and taz.
Online engagement to drive an innovative culturemikebradford
Landgate is the statutory authority responsible for land and property information in Western Australia. To drive an innovation culture, Landgate established an online engagement strategy including a website, social media accounts, and an internal online forum. This approach centralized $2 million annual funding for innovation projects, identified champions for new ideas, and encouraged staff participation. As a result, over 3,200 ideas were discussed, 50+ projects were funded, and new partnerships and $800k in new revenue were generated, while achieving significant efficiencies. Landgate was recognized as a Premier's Award finalist in 2010 for these innovation efforts.
Dokumen ini merupakan iklan produk kapsul herbal DBS Habbazzaitun yang diproduksi oleh PT. Duta Future International. Kapsul herbal ini diformulasikan dari tanaman obat tradisional dan diproses secara higienis untuk meningkatkan stamina, menyegarkan tubuh, dan meningkatkan vitalitas. Produk ini tidak mengandung zat kimia tambahan dan memiliki manfaat seperti menstimulasi tulang dan sel-sel tubuh, berperan dalam penyembuhan kank
Benefits of location Information a State\'s perspectivemikebradford
The document discusses how location information is used by the Western Australian government to drive efficiencies and support decision making. It provides details on several key programs and systems that utilize a shared land information platform (SLIP) to integrate and provide access to location data from over 25 government agencies. Examples highlighted include improving housing asset management, emergency management, and powering applications like an interest inquiry system and vehicle engine management systems.
Landgate is Western Australia's land and property information authority that launched an Innovation Program in 2008 to identify new efficiencies and ways of improving service delivery. The program is funded at $2 million annually and involves staff champions, timely execution of ideas, and recognition of both successes and failures. So far the program has discussed over 3,200 ideas, generated 100,000 forum hits, funded over 40 projects, identified $800k in new revenue and significant efficiencies, and engaged 85% of staff in creative thinking. Lessons learned include the importance of direction, capitalizing on intellectual property, risk-taking, and celebrating innovation.
The document discusses environmental aspects and their importance in an environmental management system. It notes that identifying and prioritizing significant environmental aspects and impacts is a critical step. The document also outlines ISO14001 requirements for establishing procedures to identify environmental aspects of activities, products, and services that can impact the environment.
This document provides an overview of reverse mortgages for professionals. It begins with an introduction to the reverse mortgage industry, including its history and growth. It then discusses the various reverse mortgage product types, including adjustable rate and fixed rate loans. Key details are provided around eligibility requirements, proceeds options, property eligibility, and consumer protections. The document concludes with an overview of the latest product developments, focusing on the HECM Saver option.
Systematization and diagnosis of vasculitides. Mikhail ValivachMikhail Valivach
This document provides an overview of the systematization and diagnosis of vasculitides. It discusses that vasculitides can be primary or secondary, and focuses on primary vasculitides. The key factors in diagnosing and classifying primary vasculitides are the size of affected vessels, the primary pathogenic mechanisms (such as immune complexes, ANCA, or anti-GBM antibodies), the type of secondary inflammatory reaction (granulomatous or eosinophilic), the distribution of affected organs, and the severity of vascular damage. Histology through biopsy is important for diagnosis, showing features like angiocentric inflammatory infiltration and vascular wall damage. Different classification systems are used, including the Chapel Hill definitions.
A great tutorial from Dr Alistair Jones NHS medical educator (http://www.yorkshiremedicaleducation.co.uk/about-us) on ECG syndromes. Beyond the basics (but essential knowledge for training emergency physicians)
There are bilateral upper lobe predominant alveolar opacities.
2. Bilateral lobar pneumonia. The distribution of the opacities in the upper lobes is characteristic of pneumonia due to Streptococcus pneumoniae (pneumococcus).
3. The lungs are divided into zones to help localize abnormalities:
- Upper zone: above the level of the carina
- Middle zone: between the carina and inferior pulmonary veins
- Lower zone: below the inferior pulmonary veins
Knowing the zone helps determine the likely causative organism - S. pneumoniae causes upper lobe pneumonia typically.
This document provides an overview of how to interpret a chest x-ray. It discusses the normal anatomy seen on a CXR and various patterns of abnormality. It describes how to systematically analyze a CXR by examining the airways, bones, cardiovascular system, diaphragm, lungs, and soft tissues. Common abnormalities are outlined, including consolidation, interstitial lung disease, atelectasis, nodules/masses, cavities/cysts, and calcification. Specific examples of different pathological processes are also reviewed.
extra articular manifestation of rheumatoid arthritis.pptxGyaltsenTenzin1
Rheumatoid arthritis is a chronic inflammatory disease that causes swelling and stiffness in the joints. It can also cause extra-articular manifestations affecting various organs. The document discusses the epidemiology, etiology, clinical features, extra-articular manifestations and treatment of rheumatoid arthritis. It highlights that rheumatoid arthritis predominantly affects women aged 25-55 years and can involve the lungs, heart, blood vessels and skin. Treatment involves NSAIDs, corticosteroids, conventional DMARDs like methotrexate and biological DMARDs that target cytokines like TNF-alpha if first line treatments are inadequate.
Temporal arteritis (TA), also known as giant cell arteritis (GCA), is a systemic vasculitis that commonly affects medium and large arteries in adults over 50 years old. It is characterized by inflammation of the arteries, especially the temporal arteries, which can cause headaches. Left untreated, TA can lead to permanent vision loss by damaging the ophthalmic and retinal arteries. The cause is unknown but may involve a maladaptive immune response triggered by endothelial injury. Treatment involves high-dose corticosteroids to suppress inflammation.
This document provides information on anterior ischemic optic neuropathy (AION), which is the most common cause of acute optic neuropathy in older age groups. It can be divided into two types: arteritic AION, which is due to giant cell arteritis; and non-arteritic AION, which makes up most cases. Both types present with sudden painless vision loss and optic disc swelling. Arteritic AION carries a worse prognosis and requires high-dose steroid treatment to prevent loss of vision in the fellow eye. Non-arteritic AION has a variable course but generally a poor rate of recovery without any proven effective treatments.
Rheumatoid arthritis is a systemic inflammatory disease that commonly affects the small joints of the hands and feet. It is characterized by symmetric polyarthritis, constitutional symptoms, rheumatoid nodules, and can involve various organ systems. Early diagnosis is important to prevent long-term joint damage and complications. The pathogenesis involves autoimmune processes leading to inflammation of the synovial membranes of joints. Diagnosis is based on clinical features and confirmation with serological markers such as rheumatoid factor and anti-CCP antibodies.
This document summarizes several potential rheumatological emergencies that may present in patients with common rheumatic disorders. It describes conditions like septic arthritis in patients with rheumatoid arthritis, spinal fractures in ankylosing spondylitis patients, renal crisis in systemic sclerosis, alveolar hemorrhage in systemic lupus erythematosus, and hypokalemic paralysis in Sjogren's syndrome patients. It provides details on clinical presentations, diagnostic considerations, and importance of timely recognition and treatment for each emergency. The goal is to raise awareness among emergency physicians to facilitate early diagnosis and management of these potentially life-threatening rheumatic complications.
Vasculitis refers to inflammation of blood vessels. It is classified based on vessel size and pathology. The most common pediatric vasculitides are Henoch-Schonlein purpura and Kawasaki disease. Diagnosis involves evaluating symptoms, radiology like angiograms, histopathology of biopsied tissues, and serology tests like ANCA. Treatment depends on type and severity of vasculitis. Prognosis varies, with most children recovering fully from HSP or KD, while other types like AAV carry higher risks of organ damage and mortality if not properly treated.
This document summarizes cerebrovascular disease and stroke. Stroke is the third leading cause of death and a common cause of disability. Imaging such as CT and MRI are used to distinguish between ischemic and hemorrhagic stroke and identify underlying vascular abnormalities. Risk factors include age, hypertension, cardiac sources of embolism, and other conditions. Treatment aims to reverse pathology, prevent complications, and reduce risk of further strokes.
This document summarizes cerebrovascular disease and stroke. Stroke is the third leading cause of death and a common cause of disability. Imaging such as CT and MRI are used to distinguish between ischemic and hemorrhagic stroke and identify underlying vascular abnormalities. Risk factors include age, hypertension, cardiac sources of embolism, and vascular diseases. Acute stroke is characterized by rapid onset neurological deficits localized to the brain region supplied by an occluded artery. Differential diagnosis includes tumors, infections, and seizures.
The document discusses several medical conditions and their ocular manifestations. It covers anaemia, sickle cell disease, demyelinating diseases, tumours of the nervous system, cerebrovascular disease, and seronegative arthropathies. For sickle cell disease, it describes ocular features such as vascular segments on the conjunctiva and retinal findings including venous tortuosity and peripheral scars. For demyelinating diseases like multiple sclerosis, it lists ocular features such as optic neuritis, eye movement disorders, and nerve palsies.
This document discusses infective endocarditis (IE), including its changing epidemiology, pathogenesis, clinical manifestations, diagnosis, complications, and management. Some key points:
- The median age of IE patients has increased to over 60 years old. Rheumatic heart disease is less common while intracardiac devices and nosocomial sources have risen.
- Vegetations form from platelet-fibrin deposition on damaged heart valves, allowing bacterial colonization and abscess formation.
- Echocardiography is important for diagnosis. Findings include vegetations, abscesses, and valve dysfunction. Blood cultures help identify causative organisms.
- Complications include heart failure, embolization, and periannular
an overview of Lupus for journalist
Lupus has a wide spectrum of manifestation. Some mild but in most cases it has a high impact of life and quality of life
by Cono Grasso, MD
Jamaica Hospital Medical Center
Presented at the S.L.E. Lupus Foundation's "Get into the Loop!" New York City Lupus Education Series on October 6, 2010.
This document discusses peripheral arterial disease (PAD), which is usually caused by atherosclerosis and shares risk factors with coronary artery disease such as smoking, diabetes, hyperlipidemia, and hypertension. PAD can present as intermittent claudication (pain in the leg muscles brought on by walking) or critical limb ischemia (rest pain and tissue loss). Treatment depends on the severity and location of the disease. Other vascular conditions mentioned include aneurysms, arterial dissections, and various forms of arteritis.
This document discusses stroke mimics and chameleons. It begins by introducing stroke mimics, which account for 20-25% of suspected stroke cases. Common mimics include seizures, hypoglycemia, sepsis, migraines, and tumors. Functional disorders and delirium can also mimic strokes. The document then discusses stroke chameleons, which imitate other diseases due to their gradual onset or non-specific symptoms. Examples given include vertigo, monoparesis, and delirium. Several case studies are presented to illustrate specific mimics and chameleons. The document emphasizes the importance of thorough clinical assessment to distinguish strokes from mimicking conditions.
This document provides an overview of Posterior Reversible Encephalopathy Syndrome (PRES). It defines PRES and discusses its etiology, pathophysiology, clinical presentation, diagnostic approach, management, complications, and prognosis. PRES is characterized by reversible brain edema typically seen in the posterior regions. It is often associated with conditions like hypertension, preeclampsia, immunosuppressants, and renal failure. Clinical features include headaches, seizures, altered mental status, and visual disturbances. Diagnosis relies on clinical features and MRI findings of vasogenic edema. Management involves treating the underlying cause, controlling blood pressure, and supportive care. Outcomes are generally good if treated promptly, but recurrence or malignant forms can occur with persistent risk factors.
Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system characterized by breakdown of the myelin sheath covering nerve axons. It affects over 400,000 people in the US and more than 2.1 million worldwide. Genetic factors, autoimmunity, infection, vitamin D levels, and loss of protective childhood infections may play a role in MS etiology. Clinically, MS presents with a variety of neurological symptoms depending on the location of lesions in the brain and spinal cord, including visual, motor, sensory and cognitive impairments. Disease courses include relapsing-remitting MS, secondary progressive MS, primary progressive MS and progressive-relapsing MS.
Stroke is also known as Cerebrovascular Accident. This results in the sudden death of the brain cells due to the O2 deficiency when the blood flow to the brain is lost by obstruction/rupture of cerebral arteries which supplies to the brain. Stroke prevention requires the management of the many risk factors important to stroke development. It is important to diagnose stroke as early as possible to reduce the risk of more damage and functional loss. Stroke recovery is an inhomogeneous process, therefore, it is challenging to predict the actual post-stroke outcomes which assure a holistic approach. Rehabilitation can be provided in inpatient or outpatient departments to stroke survivors.
Angioid streaks are irregular, spoke-like lesions that radiate from the optic disc. They represent cracks in the calcified Bruch's membrane and were first described in 1889. While often asymptomatic, they can be associated with vision loss due to complications like choroidal neovascularization or macular degeneration. The most common systemic associations are pseudoxanthoma elasticum, Paget's disease, and hemoglobinopathies. Treatment may include observation, laser photocoagulation, photodynamic therapy, or surgery depending on complications.
A 12-year-old female child from Tanga, Tanzania presented with left hip and knee pain. On examination, she had some paleness but no jaundice, fever, or swelling of joints. Moving the left hip and knee caused unbearable pain. She was diagnosed with sickle cell anemia and severe avascular necrosis of the left hip and knee secondary to a sickle cell crisis. Sickle cell anemia is a hereditary blood disorder common in Africa where red blood cells can sickle and block blood vessels. This blocking of vessels can cause joint damage like avascular necrosis over time.
Similar to 171 neurologic presentations of systemic vasculitides (20)
8 Surprising Reasons To Meditate 40 Minutes A Day That Can Change Your Life.pptxHolistified Wellness
We’re talking about Vedic Meditation, a form of meditation that has been around for at least 5,000 years. Back then, the people who lived in the Indus Valley, now known as India and Pakistan, practised meditation as a fundamental part of daily life. This knowledge that has given us yoga and Ayurveda, was known as Veda, hence the name Vedic. And though there are some written records, the practice has been passed down verbally from generation to generation.
The skin is the largest organ and its health plays a vital role among the other sense organs. The skin concerns like acne breakout, psoriasis, or anything similar along the lines, finding a qualified and experienced dermatologist becomes paramount.
5-hydroxytryptamine or 5-HT or Serotonin is a neurotransmitter that serves a range of roles in the human body. It is sometimes referred to as the happy chemical since it promotes overall well-being and happiness.
It is mostly found in the brain, intestines, and blood platelets.
5-HT is utilised to transport messages between nerve cells, is known to be involved in smooth muscle contraction, and adds to overall well-being and pleasure, among other benefits. 5-HT regulates the body's sleep-wake cycles and internal clock by acting as a precursor to melatonin.
It is hypothesised to regulate hunger, emotions, motor, cognitive, and autonomic processes.
Adhd Medication Shortage Uk - trinexpharmacy.comreignlana06
The UK is currently facing a Adhd Medication Shortage Uk, which has left many patients and their families grappling with uncertainty and frustration. ADHD, or Attention Deficit Hyperactivity Disorder, is a chronic condition that requires consistent medication to manage effectively. This shortage has highlighted the critical role these medications play in the daily lives of those affected by ADHD. Contact : +1 (747) 209 – 3649 E-mail : sales@trinexpharmacy.com
Does Over-Masturbation Contribute to Chronic Prostatitis.pptxwalterHu5
In some case, your chronic prostatitis may be related to over-masturbation. Generally, natural medicine Diuretic and Anti-inflammatory Pill can help mee get a cure.
These lecture slides, by Dr Sidra Arshad, offer a simplified look into the mechanisms involved in the regulation of respiration:
Learning objectives:
1. Describe the organisation of respiratory center
2. Describe the nervous control of inspiration and respiratory rhythm
3. Describe the functions of the dorsal and respiratory groups of neurons
4. Describe the influences of the Pneumotaxic and Apneustic centers
5. Explain the role of Hering-Breur inflation reflex in regulation of inspiration
6. Explain the role of central chemoreceptors in regulation of respiration
7. Explain the role of peripheral chemoreceptors in regulation of respiration
8. Explain the regulation of respiration during exercise
9. Integrate the respiratory regulatory mechanisms
10. Describe the Cheyne-Stokes breathing
Study Resources:
1. Chapter 42, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 36, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 13, Human Physiology by Lauralee Sherwood, 9th edition
pathology MCQS introduction to pathology general pathology
171 neurologic presentations of systemic vasculitides
1. Neurologic
Presentations of
Systemic Vasculitides
Alireza Minagar, MD, FAAN
a,
*, Marjorie Fowler, MD
b
,
Meghan K. Harris, MD
a
, Stephen L. Jaffe, MD
a
Vasculitis or angiitis refers to a group of inflammatory disorders of the blood vessels
that cause structural damage to the affected vessel, including thickening and weak-
ening of the vessel wall, narrowing of its lumen, and, usually, vascular necrosis.1
Proposed pathogenetic mechanisms for development of vasculitis include immune
complex formation and deposition in the vessel wall; invasion of endothelial cells
and perivascular spaces and alteration of endothelial cell functions by infectious
microorganisms, underlying neoplastic or autoimmune process (such as systemic
lupus erythematosus), or toxins; granuloma formation; presence of autoantibodies,
such as antiendothelial cell antibodies or antineutrophil cytoplasmic antibodies
(ANCA); and orchestrated action of cellular and molecular immune responses
involving proinflammatory cytokines and adhesion molecules.2–4
The overall annual incidence of the systemic vasculitides (excluding giant cell arter-
itis) is approximated at 39 per 1,000,000.5
Systemic vasculitis is classified according
to vessel size and histopathologic and clinical features. Vasculitides with small vessel
involvement typically include Henoch-Scho¨ nlein purpura and cryoglobulinemia. Poly-
arteritis nodosa and Wegener granulomatosis are small- and medium-sized vessel
vasculitides, whereas temporal arteritis (TA) and Takayasu arteritis involve large
vessels. Because of the complex and heterogeneous nature of vasculitic syndromes
and diffuse organ distribution, diagnosis and treatment of these disorders pose
a formidable challenge to clinicians. The authors of this article review the neurologic
presentations of the major systemic vasculitides.
a
Department of Neurology, Louisiana State University Health Sciences Center, 1501 Kings
Highway, Shreveport, LA 71130, USA
b
Department of Pathology, Louisiana State University Health Sciences Center, 1501 Kings
Highway, Shreveport, LA 71130, USA
* Corresponding author.
E-mail address: aminag@lsuhsc.ed (A. Minagar).
KEYWORDS
Vasculitis Arteritis Cryoglobulinemia Purpura
Neurol Clin 28 (2010) 171–184
doi:10.1016/j.ncl.2009.09.015 neurologic.theclinics.com
0733-8619/09/$ – see front matter ª 2010 Published by Elsevier Inc.
2. TEMPORAL ARTERITIS
TA, also known as giant cell arteritis (GCA), is a systemic disease that almost exclu-
sively affects individuals older than 50 years, with a mean age of onset of 70 years
and a range of 50 to more than 90 years of age. Its estimated occurrence is 15 to
25 per 100,000 individuals.6,7
TA may cause permanent blindness in one or both
eyes in 25% to 50% of affected individuals; therefore, timely diagnosis and aggressive
treatment is of obvious significance. Women are affected more frequently,8
and it is
more common in Caucasians than African Americans. Postmortem studies have
demonstrated that TA is more frequent than is clinically apparent.9
TA primarily, but
not exclusively, affects the cranial branches of the arteries that originate from the
arch of the aorta. Transmural inflammation of the affected arteries and intimal hyper-
plasia cause luminal occlusion. The initial presentation of TA may be insidious or
sudden. Certain presentations, such as visual loss and neurologic impairments, are
usually acute, whereas systemic manifestations, such as fatigue, anorexia, weight
loss, arthralgia, myalgia, low-grade fever, anemia, and leukocytosis, may be present
weeks or months before the time of clinical diagnosis. Alterations of mental status
characterized by delusional thinking, confusion, insomnia, lack of attention, and
cognitive decline may occur with TA. Many patients complain of headache with a lanci-
nating quality, commonly localized to the areas along the affected arteries of the scalp.
The headache associated with TA is persistent and usually unilateral. Indeed, develop-
ment of a new headache or prominent alteration of the pattern of a chronic headache
in an elderly patient is the most frequent symptom of TA and should alert the physician
to the possibility of this diagnosis. Diffuse tenderness of the scalp or face which stems
from the inflammatory ischemic changes associated with widespread arteritis of the
extracranial arteries, presents along with the headache, and is usually present over
the temporal or occipital arteries. Jaw pain and claudication and oral mucosa or scalp
ulceration can also be presentations of TA.
Other neurologic presentations of TA, which occur in at least 30% of affected indi-
viduals, consist of peripheral neuropathy, transient ischemic attack, and stroke.10
Peripheral neuropathy in patients with TA can occur as a mono- or polyneuropathy
and may affect upper or lower extremities. Although the median nerve is the most
commonly affected peripheral nerve, other nerves, such as ulnar, radial, tibial, sciatic,
spinal, and trigeminal nerve, may also be involved in the pathologic process. On elec-
tromyographic and nerve conduction studies, one observes abnormalities of action
potential amplitudes and conduction velocities of the affected nerves, indicative of
axonal damage and demyelination. Angiographic and neuropathologic examinations
reveal that diffuse arteritis of the vessels supplying the damaged nerves plays a signif-
icant role in pathogenesis of the peripheral neuropathy in TA.
Cranial neuropathies are also significant presentations of TA. The optic nerve is the
most frequently affected cranial nerve; and based on clinical observations over the
past 3 decades, up to 23% of affected patients develop permanent visual loss
because of anterior ischemic optic neuropathy (AION).11,12
Indeed, visual loss is the
most significant symptom of TA and may be temporary or permanent, monocular or
binocular. Transient visual loss experienced with TA is similar to that in patients with
atherosclerotic cerebrovascular disease, except in rare instances of TA, where it
may alternate between the two eyes. In patients with TA, the most common site of
optic nerve involvement is the anterior optic nerve, resulting from posterior ciliary
arteritis. Less frequently, ophthalmoscopic examination may reveal retrobulbar optic
neuropathy without papillitis. Patients with acute AION develop an altitudinal visual
field defect. Other ocular complications of TA include uni- or bilateral central retinal
Minagar et al172
3. artery occlusion. Although most of these patients develop sudden and permanent
visual loss, some may experience transient visual loss in 1 eye before the visual
loss becomes permanent. Compromise of the blood circulation through the
ophthalmic or central retinal arteries due to inflammation or thrombosis in TA seems
to cause the retinal ischemia. Vasculitis of the vessels supplying the optic chiasm is
associated with bitemporal visual field defects, whereas vasculitis of the vascular
system of the retrochiasmal visual sensory pathway can cause homonymous visual
field defects. Some patients with bilateral blindness due to TA may experience visual
hallucinations originating from loss of visual input to the cerebral cortex.
Patients with TA may develop diplopia caused by vasculitis affecting the ocular
motor system. The oculomotor nerve is commonly affected, and the presence of
a combination of ophthalmoparesis and ptosis raises the possibility of oculomotor
nerve ischemic infarction. In such cases, paralysis usually does not affect the pupil.
Other ocular abnormalities that may be observed in patients with TA include uni- or
bilateral internuclear ophthalmoplegia and pupillary disturbances, such as tonic pupils
and light-near pupillary dissociation.
Other neurologic presentations of TA, although fairly uncommon, include vertigo
and hearing loss, tinnitus, transient hemianesthesia of the tongue, lingual paralysis,
and facial pain. Between 10% and 20% of patients with TA have carotid bruits that
are commonly bilateral and indicate carotid artery involvement. Almost 40% of these
patients develop some form of ischemic ocular or cerebral syndrome.
Histopathologically, TA shows transmural inflammation of the media, intima, and
adventitia. Lymphocytes, macrophages, and multinucleated giant cells are present
in the arterial wall in a patchy distribution. Arterial narrowing is thought to be caused
by mural hyperplasia resulting in occlusive ischemia (Fig. 1 A and B).13,14
Similar
inflammatory changes are observed in the related disease, polymyalgia rheumatica
(PR). Symptoms of PR are more widespread and systemic than TA and include
pain, fever, malaise, stiff muscles, and weight loss. There is a significant relationship
between the 2 diseases, and it has been proposed that they may be different symp-
tomatic manifestations of the same underlying disease process.15
Almost 50% of
patients with TA present with PR and 10% of patients with PR develop TA.16
A diagnosis of TA should be considered in any patient older than 50 years who pres-
ents with new-onset headache, jaw claudication, ear pain, scalp tenderness, unex-
plained fever, anemia, double vision, and visual loss. Patients with TA usually have
normochromic normocytic anemia, markedly high erythrocyte sedimentation rate
(ESR40 mm/h), and elevated C-reactive protein. On 4-vessel cerebral angiography,
vasculitic changes appear as alternating stenotic segments or complete occlusion of
the affected vessels. Biopsy of the temporal artery is performed to confirm the diag-
nosis before aggressive treatment. However, the pitfall of this procedure, due to the
patchy nature of the underlying inflammation, is that biopsy achieves a sensitivity of
up to 70%, and thus, a considerable number of patients with TA may have a negative
biopsy result. Based on the 1990 American College of Rheumatology17
criteria for
classification of TA, at least three of the following 5 items must be present (sensitivity
93.5%, specificity 91.2%):
1. Age of onset greater than 50 years
2. New-onset headache or localized head pain
3. Temporal artery tenderness to palpation or reduced pulsation
4. ESR greater than 50 mm/h
5. Abnormal arterial biopsy (necrotizing vasculitis with granulomatous proliferation
and infiltration)
Neurologic Presentations of Systemic Vasculitides 173
4. Once a clinician suspects a diagnosis of TA in an elderly patient on clinical grounds,
treatment should be initiated to save the patient’s vision. The mainstay of treatment is
systemic corticosteroids. Treatment begins immediately with prednisone at a dosage
of 40 mg/day to 80 mg/day. This high dose treatment plan is maintained for up to
a month and then prednisone dosage should be tapered by 50% during one month
and then after slowly reduced by no more than 1 mg/month. The oral prednisone
should be tapered slowly to prevent relapses, with the tapering periods as long as 2
years. To lessen corticosteroid-associated osteoporosis, concurrent treatment with
alendronate should be initiated.
TAKAYASU ARTERITIS
Takayasu arteritis, also called pulseless disease, is a systemic necrotizing vasculitis
that may result in postinflammatory stenosis or occlusion of the entire aorta, the abdom-
inal aorta, the ascending aorta, or just the aortic arch and its proximal branches. The
innominate, common carotid, and subclavian arteries and the celiac, mesenteric, renal,
pulmonary, iliac, and coronary arteries are frequently affected. Currently, the exact
cause and cure for Takayasu disease are unknown. Takayasu disease occurs world-
wide, most of the patients are women, and the age of onset is between 10 and 30 years.
It seems that hypoperfusion of the affected organ due to the underlying arterial occlu-
sive process causes the clinical manifestations of Takayasu disease. For example,
Fig. 1. Neuropathology of TA. (A) Early lesion of a large muscular artery. Necrosis, inflamma-
tion and giant cell formation can be noted immediately adjacent to the internal elastic
lamina, which is undergoing degenerative changes. There is some intimal proliferation
(hematoxylin and eosin [HE], original magnification Â100). (B) In this more advanced
lesion, there is complete segmental destruction of the internal elastic lamina and virtually
the entire media. Marked intimal proliferation has nearly occluded the lumen. Few inflam-
matory cells remain (HE, original magnification Â50). (Courtesy of William E. Ballinger,
MD, Gainesville, FL; From Nadeau SE. Neurologic manifestations of systemic vasculitis.
Neurol Clin 2002 Feb;20(1):123–50; with permission.)
Minagar et al174
5. systemic hypertension occurs because of narrowing of the aorta, renal artery stenosis,
decreased elasticity of the aortic wall, or a combination of these effects.
Systemic presentations of Takayasu disease are divided into 3 clinical stages: (1)
the acute stage, characterized by nonspecific systemic manifestations, such as
low-grade fever, fatigue, malaise, anorexia, nocturnal sweating, and widespread
aching; (2) the stage of acute inflammation of the affected vessels, characterized by
the presence of vascular bruits, diminished or absent pulses, and tenderness; and
(3) the chronic obliterative phase due to vascular occlusion, identified by decreased
pulses, development of vascular bruits, claudication, and even development of
ischemic ulcers in affected extremities. Frequently, neurologic involvement occurs
and sometimes, this may be the initial presentation of the disease process. Occlusion
of the vertebral or carotid arteries may cause ischemic stroke; and patients may
present with headache, syncope, and blurred vision. Patients with Takayasu disease
may also develop intracranial aneurysms, with symptoms originating from either
ruptured or unruptured aneurysms. Pathologically, Takayasu disease manifests with
granulomatous inflammation of the vessel adventitia and the outer part of the media,
with infiltration of lymphocytes, plasma cells, histiocytes, and multinucleated giant
cells. Diagnosis of Takayasu disease rests on clinical suspicion in any young patient,
particularly women, with clinical manifestations of vascular ischemia and the presence
of bruits, decrease or absence of pulses, ischemic ulcers, or a combination of these
findings. The American College of Rheumatology 1990 criteria for the classification
of Takayasu arteritis is presented in Box 1.18
Aortic angiography confirms the diag-
nosis and one may observe occlusion or dilatation of vascular segments, stenotic
segments, aneurysm formation, and increased collateral circulation. Treatment of
Box 1
1990 criteria for the classification of Takayasu arteritisa
1. Age at disease onset less than 40 years
Development of symptoms or findings related to Takayasu arteritis at 40 years or younger
2. Claudication of extremities
Development and worsening of fatigue and discomfort in muscles of one or more
extremities while in use, especially the upper extremities
3. Decreased brachial artery pulse
Decreased pulsation of one or both brachial arteries
4. Blood pressure (BP) difference greater than10 mm Hg
Difference of more than 10 mm Hg in systolic blood pressure between arms
5. Bruit over subclavian arteries or aorta
Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta
6. Arteriogram abnormality
Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large
arteries in the proximal upper or lower extremities, not due to arteriosclerosis,
fibromuscular dysplasia, or similar causes; with changes usually focal or segmental.
a
For purposes of classification, a patient shall be said to have Takayasu arteritis if at least 3 of
these 6 criteria are present. The presence of any 3 or more criteria yields a sensitivity of 90.5%
and a specificity of 97.8%. BP 5 blood pressure (systolic; difference between arms). (Data from
Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for
the classification of Takayasu arteritis. Arthritis Rheum 1990;33(8):1129–34.)
Neurologic Presentations of Systemic Vasculitides 175
6. Takayasu disease with systemic corticosteroids, prednisone 1 mg/kg/d for 3 months,
is followed by a gradual tapering regimen based on suppression of the systemic mani-
festations. Takayasu disease does have a prolonged, relapsing, and remitting course;
in cases of unsuccessful treatment with corticosteroids, treatment with cytotoxic
agents, such as cyclophosphamide or methotrexate, should be considered. Certain
patients may benefit from surgical procedures, such as bypass of the obstructed
arteries or percutaneous transluminal angioplasty.
WEGENER GRANULOMATOSIS
Wegener granulomatosis (WG) is a vasculitic disorder of small- and medium-sized
vessels that produces necrotizing granulomatous lesions of the upper and/or lower
respiratory tract and kidney (necrotizing glomerulonephritis), and disseminated vascu-
litis. WG develops in association with antineutrophil cytoplasmic antibodies (ANCA)
and is a member of the family of ANCA-associated vasculitides (AAV).1
It is more
common in men, and the age range varies from 8 to 80 years, with a mean age of
40 years. In many patients, WG initially presents with granulomatous lesions of the
upper respiratory tract. Systemic manifestations of WG consist of chronic sinusitis,
chronic rhinitis, nasal ulceration, epistaxis, upper respiratory serosanguineous
discharge, serous otitis media, cough and hemoptysis, and dyspnea. Other presenta-
tions include fever, weight loss, anorexia, and arthralgia. Clinically, ocular involvement
presents with orbital pseudotumor, scleritis, and uveitis, and renal involvement mani-
fests with proteinuria, hematuria, and renal failure.
At any stage of the disease process, up to 50% of patients develop neurologic
complications affecting both peripheral and central nervous systems. Almost half of
those with neurologic complications suffer from recurrent mononeuropathies, mono-
neuritis multiplex, or symmetric polyneuropathy. Patients with CNS involvement
present with cranial neuropathies, most frequently cranial nerves II, VI, and VII and
nonspecific evidence of cerebral vasculitis, such as hemiparesis, seizures, aphasia,
and visual field defects. Ischemic strokes, encephalopathy, granulomatous basilar
meningitis, pachymeningitis, and myelitis are other CNS manifestations of WG that
occur less frequently.
Although the exact cause of WG remains unknown, existing evidence indicates
involvement of hypersensitivity mechanisms. Diagnosis of WG can be based on the
combination of the clinical picture along with the presence of classical antineutrophil
cytoplasmic antibodies (C-ANCA) in the serum and the demonstration of necrotizing
vasculitis on histopathologic examination. On neuroimaging, the spectrum of brain
computed tomographic (CT) and magnetic resonance (MR) abnormalities in WG
include dural thickening and enhancement, and abnormal signals within the brainstem
and white matter (Fig. 2 A, B, and C).19
Before the 1960s, WG was a lethal disorder and
most patients succumbed to the progressive renal insufficiency. Administration of
systemic corticosteroids may be associated with clinical improvement in only some
of the treated patients but does reduce the overall mortality rate. Various immunosup-
pressive agents, such as cyclophosphamide, azathioprine, methotrexate, chlorambu-
cil, and nitrogen mustard, have been used for treatment of corticosteroid failures. Of
these, cyclophosphamide has improved the prognosis for these patients significantly.
In many instances, patients are treated with a combination of prednisone and cyclo-
phosphamide. However, one should recognize that although this combination
improves 5-year prognosis of the treated patients remarkably, the cumulative toxicity
and side effects of these medications over several years are fairly high, and even under
this therapeutic regimen, between 10% and 50% of patients relapse.20
The regimen of
Minagar et al176
7. prednisone and cyclophosphamide should be continued until systemic presentations
improve, and then it should be slowly tapered. The therapeutic response can be
measured by serial clinical assessment and a declining pattern of the ESR, serum
C-reactive protein, and C-ANCA.
CRYOGLOBULINEMIA
Cryoglobulins are serum proteins that precipitate at 4
C and re-dissolve after warming
to 37
C, and they occur in patients with various clinical conditions. These serum
proteins are mainly immunoglobulins and are usually composed of either IgG or
IgM. Alternatively, they can be mixed, being composed of 2 different immunoglobu-
lins, most commonly a combination of IgG and IgM. Cryoglobulins, which have a single
monoclonal immunoglobulin, are most commonly associated with lymphoproliferative
disorders, such as multiple myeloma, lymphomas and leukemias. Mixed cryoglobulins
are frequently detected in patients with connective tissue diseases, systemic vascu-
litic syndromes, and infections such as hepatitis C viral infection; and this condition
is called essential mixed cryoglobulinemia (EMC).
Clinically, EMC, which affects mainly middle-aged women, presents with purpura,
arthralgia, weakness, and mixed cryoglobulinemia. Patients with EMC develop cuta-
neous vasculitis and progressive renal insufficiency because of glomerulonephritis.
Similar to many other hypersensitivity vasculitides, EMC seems to occur due to
Fig. 2. Neuroimaging findings of Wegener’s granulomatosis- (A) Contrast-enhanced axial CT
scan of brain at the level of the tentorium of a 35-year-old male patient with new-onset head-
ache, which demonstrates dural thickening with contrast enhancement (arrows). (B) Contrast-
enhanced coronal T1-weighted MR image (500/11/1) of a 41-year-old man with severe daily
headaches, which reveals dural enhancement (arrows). (C) T2-weighted axial MR image
(2500/80/2) of a 56-year-old man with multiple cranial neuropathies, which shows hyperin-
tense signal throughout much of the pons. (From Provenzale JM, Allen NB. Wegener granulo-
matosis: CT and MR findings. AJNR Am J Neuroradiol 1996;17:785–92; with permission.)
Neurologic Presentations of Systemic Vasculitides 177
8. deposition of immune complexes in small vessels. In any patient with the combination
of hypocomplementemia, cutaneous vasculitis, and elevated liver function tests,
a diagnosis of mixed cryoglobulinemia must be considered. A vasculitic motor and
sensory peripheral neuropathy commonly occurs in patients with EMC at some point
during their disease course. Sensory-motor mononeuritis multiplex is another form of
neuropathy that may be present in these patients. CNS complications of mixed cryo-
globulinemia are rare and include diffuse encephalopathic syndromes with focal signs,
seizures, myelopathy, and sometimes, ischemic stroke.21–24
The pathogenetic mech-
anisms of neuropathy include immune-mediated demyelination and nerve ischemia
due to occlusion of the vasa nervorum by cryoglobulins or vasculitis.
HENOCH-SCHO¨ NLEIN PURPURA
Henoch-Scho¨ nlein purpura (HSP) is one of the most common forms of systemic
vasculitis seen in children, and it is recognized by a combination of nonthrombocyto-
penic purpura, arthralgia, and abdominal pain. Certain conditions, such as malignan-
cies and infections, and certain drugs are associated with HSP. Systemic complaints
include fever, malaise, and edema; nephritis occurs in 40% of affected patients.
Although the underlying vasculitis is leukocytoclastic, the exact cause of HSP remains
unknown. Neurologic manifestations of HSP consist of headache, encephalopathy,
intracranial and intraparenchymal hemorrhage, nonhemorrhagic vasculitic involve-
ment of cerebral tissue, peripheral neuropathy, and entrapment neuropathy due to
focal edema. The presence of elevated serum IgA or IgA deposits in the vascular
wall indicate that HSP is due to IgA-mediated inflammation. In 1990, the classification
criteria for HSP were proposed by the American College of Rheumatology (Table 1).25
The clinical diagnosis is further supported by detection of leukocytoclastic vasculitis
on skin biopsy and IgA immunofluorescence in small vessels of skin and renal
glomeruli. Differential diagnosis of HSP includes infectious endocarditis, systemic
lupus erythematosus, meningococcemia, disseminated intravascular coagulation,
and Waldenstro¨ m hyperglobulinemic purpura. Although there is no specific treatment
for HSP, these patients benefit from supportive therapy and brief courses of
corticosteroids.
Table 1
1990 criteria for classification of Henoch-Scho¨ nlein purpura
Criterion Definition
Probable purpura Slightly raised ‘‘palpable’’ hemorrhagic skin lesions, not related
to thrombocytopenia
Age %20 years Patient 20 years or younger at onset of first symptoms
Bowel angina Diffuse abdominal pain, worse after meals, or the diagnosis of
bowel ischemia, usually including bloody diarrhea
Wall granulocyte on biopsy Histologic changes showing granulocytes in the walls of
arterioles or venules
For purposes of classification, a patient shall be said to have Henoch-Scho¨ nlein purpura if at least 2
of these 4 criteria are present. The presence of any 2 or more criteria yields a sensitivity of 87.1%
and a specificity of 87.7%. (Data from Mills JA, Michel BA, Bloch DA, et al. The American College of
Rheumatology 1990 criteria for the classification of Henoch-Scho¨ nlein purpura. Arthritis Rheum
1990;33(8):1114–21.)
Minagar et al178
9. NERVOUS SYSTEM VASCULITIS ASSOCIATED WITH DRUG ABUSE
Drugs, particularly sympathomimetics, have been associated with several neurologic
disorders, including cerebral ischemic infarcts and intracerebral and subarachnoid
hemorrhage. The most commonly abused drugs that are associated with these neuro-
logic complications include cocaine, heroin, ephedrine, phenylpropanolamine, and
amphetamines. These illicit drugs may be taken orally or intravenously or may be
snorted. Ischemic or hemorrhagic complications usually affect younger individuals.
A brain MR imaging case-controlled study of individuals who were cocaine dependent
demonstrated the presence of multiple, small, silent, subcortical ischemic lesions.26
Cerebral angiography in many cases of illicit drug use has demonstrated vasculitic
changes of CNS vessels, including widespread segmental narrowing of the affected
vessels with a beading pattern. Some of these illicit drugs are capable of causing
vasospasm, and thus, the mechanism behind vascular insufficiency within the CNS
may be a vasculopathy rather that true vasculitis. A compounding issue in making
a clear-cut diagnosis of illicit drug-induced CNS vasculitis is that there are usually
multiple exposures to multiple illicit substances and coexisting infections present,
such as syphilis, hepatitis C, and AIDS. Treatment of these patients rests on identifi-
cation and discontinuation of the offending drug, search for other concurrent infec-
tions, particularly human immunodeficiency virus (HIV), control of hypertension
when present, and supportive treatment. Administration of systemic corticosteroids
or immunosuppressive therapy is not recommended unless diagnosis of CNS vascu-
litis is supported by histopathologic examination.
MEDICATION-INDUCED VASCULITIS
Vasculitic syndromes may occur in association with use of prescribed drugs and the
most common drug-associated vasculitis is usually restricted to the skin. This form of
skin vasculitis presents with a maculopapular or vesicular rash, and, less frequently, as
palpable purpura. Rarely, vasculitis is widespread and presents with fever; arthralgia;
evidence of cardiac, hepatic, or renal dysfunction; and eosinophilia. The pathophysi-
ology of this form of vasculitis is hypothesized to involve the attachment of the drug to
proteins, generating haptens that are able to elicit potent immune responses. Neuro-
logically, the central and peripheral nervous system and muscles may be involved. The
list of offending drugs that may cause vasculitis contains several general types, anti-
biotics being a major type. The penicillins are probably the best known members of
this group. Treatment of drug-related vasculitis begins with immediate discontinuation
of the offending drug followed by use of corticosteroids (prednisone 1 mg/kg/d for up
to 30 days).
POLYARTERITIS NODOSA
Polyarteritis nodosa (PAN), also called periarteritis nodosa, is a rare inflammatory
necrotizing vasculitis that affects small- to medium-sized arteries. Although it is often
idiopathic, it can be associated with other disorders, such as cryoglobulinemia,
leukemia, arthritis, Sjogren syndrome, hepatitis C, hepatitis B, and HIV infection.27
PAN is a systemic disease and may affect any organ; however, the skin, kidney,
peripheral nerves, and gastrointestinal tract are most commonly involved. The annual
incidence of PAN is about 0.2 to 0.7 per 100,000 and it affects men more than
women.28
PAN is more common in individuals 40 to 60 years of age. PAN affects
the central and peripheral nervous systems. The classification scheme proposed by
the American College of Rheumatology for PAN is presented in Table 2.29
Neurologic Presentations of Systemic Vasculitides 179
10. Between 23% and 53% of patients with PAN develop CNS pathology. CNS involve-
ment is less common than that of the peripheral nervous system and usually presents
late in the course of disease. The two most frequent central neurologic pictures of PAN
include diffuse encephalopathy and focal or multifocal disturbances of the brain or
spinal cord. Diffuse encephalopathy associated with PAN presents with rapid decline
in level of consciousness and with seizures. Focal neurologic deficits, which may
occur suddenly, are due to ischemic stroke affecting the cerebral cortex, brainstem,
or cerebellum. Patients with PAN may develop anterior optic neuropathy and posterior
(retrobulbar) optic neuropathy; and they tend to experience acute and painless visual
loss. These forms of optic neuropathy stem from vasculitis-induced ischemia in the
distribution of the posterior ciliary arteries, or inflammation of the optic nerve. Cranial
nerve III, IV, VI, VII, and VIII involvement rarely occurs. Rarely, patients with PAN
may experience intracranial hemorrhage. Mononeuritis multiplex is common in the
context of PAN and presents with asymmetric motor and sensory findings that mainly
Table 2
American College of Rheumatology 1990 criteria for the classification of Polyarteritis Nodosa
Criterion Definition
(1) Weight loss 4 kg Loss of 4 kg or more body weight since
illness began, not due to dieting or
other factor
(2) Livedo reticularis Mottled reticular pattern over the skin of
portions of the extremities or torso
(3) Testicular pain or tenderness Pain or tenderness of the testicles, not
due to the infection, trauma, or other
causes
(4) Myalgias, weakness, or polyneuropathy Diffuse myalgias (excluding shoulder and
hip girdle) or weakness of muscles or
tenderness of leg muscles
(5) Mononeuropathy or polyneuropathy Development of mononeuropathy,
multiple mononeuropathies, or
polyneuropathy
(6) Diastolic BP90 mm Hg Development of hypertension with
diastolic BP 90 mm Hg
(7) Elevated blood urea nitrogen or
creatinine
Elevation of blood urea nitrogen 40 mg/
dL (14.3 mmol/L) or creatinine 1.5/dL
(132 mmol/L), not due to dehydration or
obstruction
(8) Hepatitis B virus Presence of hepatitis B surface antigen or
antibody in serum
(9) Arteriographic abnormality Arteriogram showing aneurysm or
occlusion of the visceral arteries, not
due to arteriosclerosis, fibromuscular
dysplasia, or other noninflammatory
causes
(10) Biopsy of small- or medium-sized artery
containing polymorphonuclear cells
Histologic changes showing the presence
of granulocytes and mononuclear
leukocytes in the artery wall
For classification purposes, a patient with vasculitis is diagnosed with PAN if at least 3 criteria of the
above criteria are met. (Data from Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American
College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis
Rheum 1990;33(8):1088–93.)
Minagar et al180
11. affect the lower extremities, particularly the sciatic, peroneal, or tibial nerves. Less
commonly, the radial, cubital, and median nerves may be affected. The onset of
mononeuritis multiplex in these patients is usually sudden, whereas the distal
symmetric peripheral neuropathy in these patients develops slowly. Examination of
the cerebrospinal fluid may not reveal any diagnostic abnormalities.
Histopathologically, PAN manifests with focal but pan-mural necrotizing inflamma-
tory lesions that affect small- and medium-sized arteries (Fig. 3). Further examination
of these inflammatory lesions reveals disruption of the normal infrastructure of the
vessel wall, fibrinoid necrosis, and pleomorphic cellular infiltration with mainly
polymorphonuclear cells and a variable number of lymphocytes and eosinophils.
Diagnosis of PAN is made based on clinical suspicion when a patient presents with
constitutional symptoms, such as fever, chills, weight loss, fatigue, and multisystem
dysfunction. Anemia, elevated ESR, and evidence of thrombosis further support the
diagnosis of PAN. Brain CT and MR neuroimaging illustrates infarctions in the cortical
and subcortical regions of the cerebral hemispheres, basal ganglia, internal capsule,
brainstem, and cerebellum (Fig. 4 A and B).30
Arteriography may reveal the presence
of arterial saccular or fusiform aneurysms and narrowing and tapering of the affected
vessels. Histopathologic examination of affected tissue, such as skin, sural nerve,
muscle, or kidney, confirms the clinical diagnosis.
Treatment of PAN consists of the administration of systemic corticosteroids along
with cytotoxic agents, such as cyclophosphamide or azathioprine. If a patient does
not tolerate this regimen’s side effects, other therapeutic interventions that need to
be considered include plasma exchange, intravenous immune globulin, mycopheno-
late mofetil, and rituximab.
Fig. 3. A small muscular artery from the muscle of a patient with PAN, which illustrates the
proliferative phase in which neutrophils have been replaced by chronic inflammatory cells
and there is evidence of necrosis of the media, early intimal proliferation, and fibrosis
(HE, original magnification Â250). (Courtesy of William E. Ballinger, MD, Gainesville, FL;
From Nadeau SE. Neurologic manifestations of systemic vasculitis. Neurol Clin 2002
Feb;20(1):123–50; with permission.)
Neurologic Presentations of Systemic Vasculitides 181
12. MICROSCOPIC POLYANGIITIS
Microscopic polyangiitis (MPA) is another member of AAV that predominantly affects
small vessels (capillaries, venules, and arterioles). In MPA, granulomata are not
present. In MPA, ANCA is directed against myeloperoxidase.2
MPA is associated
with rapidly progressive focal segmental necrotizing glomerulonephritis. The average
age of onset is 50 years and men are more often affected than women. Savage and
colleagues31
observed that MPA may have an indolent course before clinical diag-
nosis. Systemic symptoms, such as arthralgia and hemoptysis, may be present
long before the explosive phase of the disease occurs. Peripheral neuropathy is
less common in MPA than PAN, whereas pulmonary involvement occurs more
frequently. Diagnostic workup for MPA reveals the presence of serum ANCA and
absence of surface hepatitis B antigen or antibody. Histopathologic examination of
a biopsied specimen from the kidney reveals the presence of focal segmental
thrombosing and necrotizing glomerulonephritis. Treatment of MPA is based on
immunosuppression with the combination of systemic corticosteroids and cyclophos-
phamide. Potential therapies under clinical investigation include intravenous immune
globulin and rituximab.
REFERENCES
1. Jayne D. Treatment of ANCA-associated systemic small-vessel vasculitis. APMIS
Suppl 2009;127:3–9.
2. Holle JU, Gross WL. ANCA-associated vasculitides: pathogenetic aspects and
current evidence-based therapy. J Autoimmun 2009;32(3–4):163–71.
Fig. 4. Case 4: 40-year-old man with multiple cerebral, cerebellar, and brain stem infarctions
related to PAN. (A) Axial T2-weighted (2200/80/0.75) MR image shows multiple sites of
hyperintense signal within the cerebellum (straight arrows) and pons (curved arrow). (B)
Axial T2-weighted (2200/80/0.75) MR image reveals bilateral parietal (solid arrows) and
left frontal (open arrows) areas of hyperintense signal within brain cortex. (From Provenzale
JM, Allen NB. Neuroradiologic findings in polyarteritis nodosa. AJNR Am J Neuroradiol
1996;17(6):1119–26; with permission.)
Minagar et al182
13. 3. Pankhurst T, Savage CO, Little MA. Review article: leukocyte-endothelial dysre-
gulation in systemic small vessel vasculitis. Nephrology (Carlton) 2009;14(1):
3–10.
4. Ojeda VJ. Polyarteritis nodosa affecting the spinal cord arteries. Aust N Z J Med
1983;13:287–9.
5. Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis:
changing incidence or definition? Semin Arthritis Rheum 1995;25(1):28–34.
6. Hunder GG. Epidemiology of giant-cell arteritis. Cleve Clin J Med 2002;69(Suppl
2):SII79–82.
7. Baldursson O, Steinsson K, Bjo¨rnsson J, et al. Giant cell arteritis in Iceland. An
epidemiologic and histopathologic analysis. Arthritis Rheum 1994;37(7):1007–12.
8. Nordborg C, Nordborg E, Petursdottir V. Giant cell arteritis. Epidemiology,
etiology and pathogenesis. APMIS 2000;108(11):713–24.
9. Ostberg G. Temporal arteritis in a large necropsy series. Ann Rheum Dis 1971;
30(3):224–35.
10. Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med
2003;349(2):160–9.
11. Caselli RJ, Hunder GG, Whisnant JP. Neurologic disease in biopsy-proven giant
cell (temporal) arteritis. Neurology 1988;38(3):352–9.
12. Koorey DJ. Cranial arteritis. A twenty-year review of cases. Aust N Z J Med 1984;
14(2):143–7.
13. Eberhardt RT, Dhadly M. Giant cell arteritis: diagnosis, management, and cardio-
vascular implications. Cardiol Rev 2007;15(2):55–61.
14. Nadeau SE. Neurologic manifestations of systemic vasculitis. Neurol Clin 2002;
20(1):123–50.
15. Cantini F, Niccoli L, Storri L, et al. Are polymyalgia rheumatica and giant cell arter-
itis the same disease? Semin Arthritis Rheum 2004;33(5):294–301.
16. Epperly TD, Moore KE, Harrover JD. Polymyalgia rheumatica and temporal
arthritis. Am Fam Physician 2000;62(4):789–96, 801.
17. Hunder GG, Bloch DA, Michel BA, et al. The American College of rheumatology
1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;
33(8):1122–8.
18. Arend WP, Michel BA, Bloch DA, et al. The American College of rheumatology
1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;
33(8):1129–34.
19. Provenzale JM, Allen NB. Wegener granulomatosis: CT and MR findings. AJNR
Am J Neuroradiol 1996;17:785–92.
20. de Groot K, Reinhold-Keller E, Tatsis E, et al. Therapy for the maintenance
of remission in sixty-five patients with generalized Wegener’s granulomatosis.
Methotrexate versus trimethoprim/sulfamethoxazole. Arthritis Rheum 1996;39:
2052–61.
21. Abramsky O, Slavin S. Neurologic manifestations in patients with mixed cryoglo-
bulinemia. Neurology 1974;24(3):245–9.
22. Petty GW, Duffy J, Houston J 3rd. Cerebral ischemia in patients with hepatitis
C virus infection and mixed cryoglobulinemia. Mayo Clin Proc 1996;71(7):
671–8.
23. Reik L Jr, Korn JH. Cryoglobulinemia with encephalopathy: successful treatment
by plasma exchange. Ann Neurol 1981;10(5):488–90.
24. Ristow SC, Griner PF, Abraham GN, et al. Reversal of systemic manifestations of
cryoglobulinemia. Treatment with melphalan and prednisone. Arch Intern Med
1976;136(4):467–70.
Neurologic Presentations of Systemic Vasculitides 183
14. 25. Mills JA, Michel BA, Bloch DA, et al. The American College of rheumatology 1990
criteria for the classification of Henoch-Scho¨nlein purpura. Arthritis Rheum 1990;
33(8):1114–21.
26. Bartzokis G, Beckson M, Hance DB, et al. Magnetic resonance imaging evidence
of ‘‘silent’’ cerebrovascular toxicity in cocaine dependence. Biol Psychiatry 1999;
45(9):1203–11.
27. Lhote F, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis, and Churg-
Strauss syndrome: clinical aspects and treatment. Rheum Dis Clin North Am
1995;21:911–47.
28. Kurland LT, Hauser WA, Ferguson RH, et al. Epidemiologic features of diffuse
connective tissue disorders in Rochester, Minn., 1951 through 1967, with special
reference to systemic lupus erythematosus. Mayo Clin Proc 1969;44(9):649–63.
29. Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of rheuma-
tology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum
1990;33(8):1088–93.
30. Provenzale JM, Allen NB. Neuroradiologic findings in polyarteritis nodosa. AJNR
Am J Neuroradiol 1996;17(6):1119–26.
31. Savage CO, Winearls CG, Evans DJ, et al. Microscopic polyarteritis: presentation,
pathology and prognosis. Q J Med 1985;56(220):467–83.
Minagar et al184