Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic inflammatory neuropathy. CIDP is diagnosed according to the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria
https://www.youtube.com/watch?v=AYEhL1LdONY
CIDP is a chronic acquired demyelinating neuropathy. It has two patterns - a continuous progressive course over months to years or a relapsing course with partial recovery between episodes. Diagnosis requires documentation of demyelination through electrodiagnostic testing, CSF analysis and sometimes nerve biopsy. Treatment includes corticosteroids, IVIG and plasmapheresis, which are effective in around 50-70% of patients. Corticosteroids are usually the first line treatment, starting with high dose prednisone and slowly tapering as response occurs.
This document describes a case of a 32-year-old male who presented with weakness in all four limbs that progressed over a week. Based on examination and investigations, he was diagnosed with recurrent quadriparesis likely due to an autoimmune etiology of chronic inflammatory demyelinating polyneuropathy (CIDP) associated with systemic lupus erythematosus (SLE). He showed improvement in symptoms with intravenous steroids and plasma exchange.
This document provides diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP), including:
1) Clinical criteria for typical and atypical CIDP with inclusion/exclusion factors.
2) Definite, probable, and possible electrophysiological criteria involving compound muscle action potential tests.
3) Supportive diagnostic criteria including cerebrospinal fluid analysis, MRI findings, nerve conduction studies, and nerve biopsy results.
It also outlines inclusion/exclusion criteria and supportive criteria specifically for diagnosing pure sensory CIDP without motor involvement.
This document discusses the approach to transverse myelitis (TM). Some key points:
1. TM is an acute inflammatory disorder of the spinal cord that presents with weakness, sensory alterations, and bowel/bladder dysfunction. Symptoms progress to maximum deficit within 4 hours to 21 days.
2. It can be idiopathic or secondary to conditions like multiple sclerosis or systemic lupus erythematosus (SLE). Diagnosis involves ruling out other causes and detecting spinal cord inflammation.
3. Initial treatment involves high-dose steroids and possibly plasma exchange. Patients with underlying autoimmune diseases may require additional immunotherapy. Prognosis depends on factors like severity and speed of progression, with most recovery
Neurology 2nd investigation of neurological diseaseRamiAboali
This document discusses various neurological investigations and presenting problems. It provides details on neuroimaging techniques like CT, MRI, EEG and lumbar puncture. It also describes nerve conduction studies and evoked potentials. Common presenting problems covered include syncope, coma, and alterations in behavior like delirium and dementia. Causes, diagnosis, and management are discussed for syncope and coma. The document is a reference for neurology that outlines different testing and clinical presentations.
Post-traumatic epilepsy (PTE) is defined as recurrent seizures occurring after traumatic brain injury (TBI). TBI accounts for 10-20% of epilepsy cases. Risk factors for early PTE include GCS <10, intracranial hematoma, and seizures within 24 hours of injury. Risk factors for late PTE include penetrating injury, intracranial hematoma, early PTE, and age over 35. Temporal lobes are the most common localization. Standard anticonvulsants are used to treat established PTE but prophylaxis is ineffective at preventing late PTE. Surgery may be considered for refractory late PTE if the seizure focus is well-localized.
Neuromyelitis optica (NMO), also known as Devic's disease, is an inflammatory disorder characterized by demyelination of the optic nerve and spinal cord. It is associated with antibodies against aquaporin-4 (AQP4-IgG). The disease predominantly affects women and typically presents with episodes of optic neuritis and transverse myelitis. MRI often shows long extensive lesions of the spinal cord. Treatment involves high-dose steroids for acute attacks and immunosuppressants like mycophenolate mofetil or rituximab to prevent relapses. Distinguishing NMO from multiple sclerosis is important for treatment, as some therapies effective for MS may worsen NMO.
The document describes a case of a 14-year old boy who developed abnormal movements and weakness following treatment for snake bite with anti-venom serum (ASV). MRI later revealed lesions in the basal ganglia. He was diagnosed with acute disseminated encephalomyelitis (ADEM) caused by an immune response to the anti-venom. Steroid treatment improved his symptoms and he recovered fully after 3 months. ADEM is an inflammatory demyelinating condition that can be triggered by infections or vaccines and presents with polyneurological symptoms.
CIDP is a chronic acquired demyelinating neuropathy. It has two patterns - a continuous progressive course over months to years or a relapsing course with partial recovery between episodes. Diagnosis requires documentation of demyelination through electrodiagnostic testing, CSF analysis and sometimes nerve biopsy. Treatment includes corticosteroids, IVIG and plasmapheresis, which are effective in around 50-70% of patients. Corticosteroids are usually the first line treatment, starting with high dose prednisone and slowly tapering as response occurs.
This document describes a case of a 32-year-old male who presented with weakness in all four limbs that progressed over a week. Based on examination and investigations, he was diagnosed with recurrent quadriparesis likely due to an autoimmune etiology of chronic inflammatory demyelinating polyneuropathy (CIDP) associated with systemic lupus erythematosus (SLE). He showed improvement in symptoms with intravenous steroids and plasma exchange.
This document provides diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP), including:
1) Clinical criteria for typical and atypical CIDP with inclusion/exclusion factors.
2) Definite, probable, and possible electrophysiological criteria involving compound muscle action potential tests.
3) Supportive diagnostic criteria including cerebrospinal fluid analysis, MRI findings, nerve conduction studies, and nerve biopsy results.
It also outlines inclusion/exclusion criteria and supportive criteria specifically for diagnosing pure sensory CIDP without motor involvement.
This document discusses the approach to transverse myelitis (TM). Some key points:
1. TM is an acute inflammatory disorder of the spinal cord that presents with weakness, sensory alterations, and bowel/bladder dysfunction. Symptoms progress to maximum deficit within 4 hours to 21 days.
2. It can be idiopathic or secondary to conditions like multiple sclerosis or systemic lupus erythematosus (SLE). Diagnosis involves ruling out other causes and detecting spinal cord inflammation.
3. Initial treatment involves high-dose steroids and possibly plasma exchange. Patients with underlying autoimmune diseases may require additional immunotherapy. Prognosis depends on factors like severity and speed of progression, with most recovery
Neurology 2nd investigation of neurological diseaseRamiAboali
This document discusses various neurological investigations and presenting problems. It provides details on neuroimaging techniques like CT, MRI, EEG and lumbar puncture. It also describes nerve conduction studies and evoked potentials. Common presenting problems covered include syncope, coma, and alterations in behavior like delirium and dementia. Causes, diagnosis, and management are discussed for syncope and coma. The document is a reference for neurology that outlines different testing and clinical presentations.
Post-traumatic epilepsy (PTE) is defined as recurrent seizures occurring after traumatic brain injury (TBI). TBI accounts for 10-20% of epilepsy cases. Risk factors for early PTE include GCS <10, intracranial hematoma, and seizures within 24 hours of injury. Risk factors for late PTE include penetrating injury, intracranial hematoma, early PTE, and age over 35. Temporal lobes are the most common localization. Standard anticonvulsants are used to treat established PTE but prophylaxis is ineffective at preventing late PTE. Surgery may be considered for refractory late PTE if the seizure focus is well-localized.
Neuromyelitis optica (NMO), also known as Devic's disease, is an inflammatory disorder characterized by demyelination of the optic nerve and spinal cord. It is associated with antibodies against aquaporin-4 (AQP4-IgG). The disease predominantly affects women and typically presents with episodes of optic neuritis and transverse myelitis. MRI often shows long extensive lesions of the spinal cord. Treatment involves high-dose steroids for acute attacks and immunosuppressants like mycophenolate mofetil or rituximab to prevent relapses. Distinguishing NMO from multiple sclerosis is important for treatment, as some therapies effective for MS may worsen NMO.
The document describes a case of a 14-year old boy who developed abnormal movements and weakness following treatment for snake bite with anti-venom serum (ASV). MRI later revealed lesions in the basal ganglia. He was diagnosed with acute disseminated encephalomyelitis (ADEM) caused by an immune response to the anti-venom. Steroid treatment improved his symptoms and he recovered fully after 3 months. ADEM is an inflammatory demyelinating condition that can be triggered by infections or vaccines and presents with polyneurological symptoms.
neuromyelitis optica spectrum disorder Dr. Musa AtarzadehMusa Atazadeh
1. The document discusses the diagnosis and diagnostic criteria for Neuromyelitis Optica Spectrum Disorder (NMOSD) according to the 2015 AAN criteria.
2. The diagnosis involves assessing for core clinical characteristics, compatible neuroimaging findings, and testing for AQP4-IgG antibodies.
3. Certain clinical presentations and neuroimaging patterns can raise red flags and suggest alternative diagnoses rather than NMOSD. Repeating AQP4-IgG testing over time or in the CSF may also be considered in some cases.
This document provides an overview of neuromyelitis optica (NMO), including its clinical presentation, pathogenesis, diagnostic criteria, treatment, and biomarkers. Some key points:
- NMO predominantly targets the optic nerve and spinal cord, often causing severe vision loss or paralysis. It was previously considered a variant of multiple sclerosis but is now recognized as a distinct condition.
- The discovery of antibodies against aquaporin-4 helped establish NMO as a separate autoimmune disease, with these antibodies detected in around 70-80% of cases.
- In addition to optic neuritis and transverse myelitis, NMO can involve other areas of the CNS and cause a range of neurological and non-
This document provides an overview of neuromyelitis optica spectrum disorders (NMOSD). It discusses the epidemiology, clinical features, diagnostic criteria, investigations, neuroimaging findings, and treatments for NMOSD. Key points include that NMOSD predominantly affects the optic nerves and spinal cord, is strongly associated with antibodies against the aquaporin-4 protein, and treatments involve high-dose steroids, plasma exchange, or intravenous immunoglobulins for acute exacerbations. The diagnostic criteria were revised in 2015 to incorporate aquaporin-4 antibody testing and distinguish NMOSD from multiple sclerosis.
Mononeuritis multiplex is a peripheral neuropathy involving damage to two or more noncontiguous nerves. It can be caused by various systemic conditions like diabetes, vasculitis, infections, and rheumatological disorders. The document discusses the clinical presentation, diagnostic evaluation, management, and treatment of mononeuritis multiplex.
1. The document describes two clinical case discussions involving patients presenting with neurological symptoms.
2. The first case involves a 24-year-old male with involuntary movements for 8 months, found to have subacute sclerosing panencephalitis confirmed by MRI and CSF analysis.
3. The second case involves an 18-year-old female with ptosis, difficulty swallowing and speaking, diagnosed with myasthenia gravis confirmed by positive acetylcholine receptor antibodies and response to prostigmin testing.
Mitochondrial DNA (mtDNA) encodes proteins that are essential components of the oxidative phosphorylation (OXPHOS) system located in the inner mitochondrial membrane. Defects in mtDNA or nuclear genes involved in mitochondrial functions can cause a wide range of mitochondrial diseases. MtDNA is maternally inherited and mutations can be transmitted from mother to offspring. Common mitochondrial diseases include Chronic Progressive External Ophthalmoplegia (CPEO), Kearns-Sayre Syndrome (KSS), MELAS, MERRF, and Leber Hereditary Optic Neuropathy (LHON). These diseases have varying clinical features depending on the mutation and often affect the brain, muscles, and eyes.
This document provides an overview of the management of multiple sclerosis (MS). It describes MS as a chronic inflammatory demyelinating disease of the central nervous system that predominantly affects women aged 20-40. The main phenotypes discussed are relapsing-remitting MS, clinically isolated syndrome, secondary progressive MS, and primary progressive MS. Diagnosis involves dissemination of lesions in time and space based on clinical symptoms and MRI findings. Treatment aims to reduce relapse rates and disability progression through disease-modifying therapies like interferons, glatiramer acetate, fingolimod, natalizumab, and dimethyl fumarate. Symptomatic treatments are also discussed.
This document discusses several types of autoimmune encephalitis, including Hashimoto's encephalopathy, NMDA encephalitis, and limbic encephalitis. Hashimoto's encephalopathy is defined by the presence of thyroid peroxidase antibodies in patients with encephalitis that responds to steroids. It can cause non-specific neurological symptoms. NMDA encephalitis commonly affects young women and can cause psychiatric issues, decreased consciousness, and movement disorders. Diagnosis involves detecting NMDA receptor antibodies in CSF or serum. Limbic encephalitis involves inflammation of limbic structures and is associated with antibodies against proteins like LGI1; it typically causes memory loss, behavioral changes and seizures in older patients.
Pediatrics notes about "Acute flaccid paralysis". These notes were published in 2018.
You can download them from
- Telegram: https://t.me/pediatric_notes_2018
- Mediafire: http://www.mediafire.com/folder/u5u60m184t9z7/Pediatric_Notes_2018
Congenital myasthenic syndrome (CMS) is a rare heterogeneous group of genetic disorders that affect neuromuscular transmission. CMS can present at birth or in early infancy with symptoms like ptosis, external ophthalmoplegia, proximal limb weakness, and respiratory difficulties. Diagnosis involves neurophysiology testing showing a decremental response on repetitive nerve stimulation and increased jitter on single fiber EMG. Genetic testing and muscle biopsy can help identify the underlying cause, which may include defects in presynaptic, synaptic, or postsynaptic proteins. Treatment involves cholinesterase inhibitors, potassium channel blockers, beta-2 agonists, or open channel blockers depending on the CMS subtype. Supportive care like respiratory
This presentation provides an overview of demyelinating diseases, focusing on multiple sclerosis (MS). It defines demyelinating diseases as those that cause myelin destruction while sparing other nervous system elements. MS is described as an autoimmune, inflammatory demyelinating disease of the central nervous system (CNS) that is more common in women. The presentation covers the pathology, clinical features, investigations, and treatment approaches for MS.
This document summarizes key information about multifocal motor neuropathy (MMN):
1) MMN is a rare, purely motor neuropathy characterized by asymmetric motor deficits predominantly affecting the upper limbs, with diagnostic clues including conduction block and anti-GM1 antibodies.
2) Clinical features include distal weakness without sensory loss, and electrophysiology shows motor conduction block. Treatment involves intravenous immunoglobulin which provides benefit for most patients.
3) The pathophysiology likely involves autoimmune attack mediated by IgM antibodies against the ganglioside GM1, disrupting paranodal function and conduction. While chronic, MMN has a relatively benign prognosis with treatment.
references:
Phases and Phenotypes of Multiple Sclerosis By Orhun H. Kantarci, MD.
Diagnosis of Multiple Sclerosis By Jiwon Oh, MD, PhD, FRCPC
Nature Reviews | Disease Primers
Multiple sclerosis Massimo Filippi1,2*, Amit Bar- Or3, Fredrik Piehl4,5,6, Paolo Preziosa1,2, Alessandra Solari7, Sandra Vukusic8 and Maria A. Rocca1,2
Dr. Shubham Garg discusses neuromyelitis optica (NMO), an autoimmune condition where antibodies attack aquaporin-4 in the central nervous system. NMO predominantly affects women and has a median age of onset of 32-41 years. Key clinical features include transverse myelitis, typically longitudinally extensive, and severe optic neuritis. Treatment involves high-dose steroids for acute attacks and immunosuppressants like azathioprine to reduce relapse rates. Prognosis is generally worse than multiple sclerosis due to risk of cumulative disability, though relapse rates can be lowered with appropriate treatment.
This document provides an overview of various demyelinating diseases of the central nervous system. It begins by defining demyelinating diseases as those involving disruption of myelin, which forms an insulating sheath around axons. It then classifies and describes several specific diseases, including acute disseminated encephalomyelitis (ADEM), inflammatory demyelinating pseudotumor, multiple sclerosis (MS), neuromyelitis optica, central pontine myelinolysis, HIV encephalopathy, progressive multifocal leukoencephalopathy (PML), and others. For each disease, it discusses clinical features, magnetic resonance imaging (MRI) findings, differential diagnoses, and pathology where relevant.
Paraneoplastic syndrome (PNS) is the term used to refer to the disorders that accompany the benign or the malignant tumors and are not caused by mass effect or invasion / metastasis.
These disorders are triggered by an immune system response to neuronal proteins expressed by the tumor(onconeural proteins).
These PNS also occur due to substances secreted by the neoplasm itself.
This document discusses peripheral neuropathy and provides guidance on evaluating and diagnosing peripheral nerve disorders. It defines peripheral neuropathy as disorders affecting the peripheral nervous system, which can involve sensory nerves, motor nerves, or both. The document outlines that peripheral neuropathies can be classified based on whether they primarily affect the cell body, myelin, or axon. It also lists common causes of peripheral neuropathy like diabetes, paraproteinemia, alcohol misuse, and vitamin B12 deficiency. The document provides guidance on clinical assessment, laboratory and electrodiagnostic testing, skin or nerve biopsy, and treatment approaches for peripheral neuropathy.
A 20-year-old male presented with headaches, double vision, difficulty swallowing and nasal regurgitation over the past 1-3 weeks. On examination, he had neck stiffness and multiple cranial nerve palsies. Imaging showed diffuse pachymeningeal enhancement and bilateral papilledema. Cerebrospinal fluid analysis was consistent with basal meningitis. He was started on antitubercular treatment but developed severe anemia and splenomegaly, and was ultimately diagnosed with acute lymphocytic leukemia involving the central nervous system.
Valproic acid is an anticonvulsant medication used to treat epilepsy and bipolar disorder. It works by increasing brain levels of the inhibitory neurotransmitter GABA. Common side effects include nausea, vomiting, tremors, and weight gain. It can cause serious liver toxicity and birth defects, so risks must be considered. The dosage is individualized based on a person's medical condition, age, and other factors. Therapeutic drug monitoring is important to ensure blood levels remain within the target range for maximum benefit and minimum harm.
MOGAD is an inflammatory demyelinating disease of the CNS associated with antibodies targeting myelin oligodendrocyte glycoprotein (MOG). It can present with optic neuritis, acute disseminated encephalomyelitis (ADEM)-like attacks, brainstem syndromes, cerebral cortical encephalitis, or myelitis. MRI often shows large or longitudinally extensive lesions of the optic nerve, brain, or spinal cord. Pathology demonstrates perivenous demyelination, MOG-dominant myelin loss, and predominant CD4+ T-cell inflammation. MOGAD has a more heterogeneous clinical and radiological presentation compared to other CNS demyelinating diseases like multiple s
neuromyelitis optica spectrum disorder Dr. Musa AtarzadehMusa Atazadeh
1. The document discusses the diagnosis and diagnostic criteria for Neuromyelitis Optica Spectrum Disorder (NMOSD) according to the 2015 AAN criteria.
2. The diagnosis involves assessing for core clinical characteristics, compatible neuroimaging findings, and testing for AQP4-IgG antibodies.
3. Certain clinical presentations and neuroimaging patterns can raise red flags and suggest alternative diagnoses rather than NMOSD. Repeating AQP4-IgG testing over time or in the CSF may also be considered in some cases.
This document provides an overview of neuromyelitis optica (NMO), including its clinical presentation, pathogenesis, diagnostic criteria, treatment, and biomarkers. Some key points:
- NMO predominantly targets the optic nerve and spinal cord, often causing severe vision loss or paralysis. It was previously considered a variant of multiple sclerosis but is now recognized as a distinct condition.
- The discovery of antibodies against aquaporin-4 helped establish NMO as a separate autoimmune disease, with these antibodies detected in around 70-80% of cases.
- In addition to optic neuritis and transverse myelitis, NMO can involve other areas of the CNS and cause a range of neurological and non-
This document provides an overview of neuromyelitis optica spectrum disorders (NMOSD). It discusses the epidemiology, clinical features, diagnostic criteria, investigations, neuroimaging findings, and treatments for NMOSD. Key points include that NMOSD predominantly affects the optic nerves and spinal cord, is strongly associated with antibodies against the aquaporin-4 protein, and treatments involve high-dose steroids, plasma exchange, or intravenous immunoglobulins for acute exacerbations. The diagnostic criteria were revised in 2015 to incorporate aquaporin-4 antibody testing and distinguish NMOSD from multiple sclerosis.
Mononeuritis multiplex is a peripheral neuropathy involving damage to two or more noncontiguous nerves. It can be caused by various systemic conditions like diabetes, vasculitis, infections, and rheumatological disorders. The document discusses the clinical presentation, diagnostic evaluation, management, and treatment of mononeuritis multiplex.
1. The document describes two clinical case discussions involving patients presenting with neurological symptoms.
2. The first case involves a 24-year-old male with involuntary movements for 8 months, found to have subacute sclerosing panencephalitis confirmed by MRI and CSF analysis.
3. The second case involves an 18-year-old female with ptosis, difficulty swallowing and speaking, diagnosed with myasthenia gravis confirmed by positive acetylcholine receptor antibodies and response to prostigmin testing.
Mitochondrial DNA (mtDNA) encodes proteins that are essential components of the oxidative phosphorylation (OXPHOS) system located in the inner mitochondrial membrane. Defects in mtDNA or nuclear genes involved in mitochondrial functions can cause a wide range of mitochondrial diseases. MtDNA is maternally inherited and mutations can be transmitted from mother to offspring. Common mitochondrial diseases include Chronic Progressive External Ophthalmoplegia (CPEO), Kearns-Sayre Syndrome (KSS), MELAS, MERRF, and Leber Hereditary Optic Neuropathy (LHON). These diseases have varying clinical features depending on the mutation and often affect the brain, muscles, and eyes.
This document provides an overview of the management of multiple sclerosis (MS). It describes MS as a chronic inflammatory demyelinating disease of the central nervous system that predominantly affects women aged 20-40. The main phenotypes discussed are relapsing-remitting MS, clinically isolated syndrome, secondary progressive MS, and primary progressive MS. Diagnosis involves dissemination of lesions in time and space based on clinical symptoms and MRI findings. Treatment aims to reduce relapse rates and disability progression through disease-modifying therapies like interferons, glatiramer acetate, fingolimod, natalizumab, and dimethyl fumarate. Symptomatic treatments are also discussed.
This document discusses several types of autoimmune encephalitis, including Hashimoto's encephalopathy, NMDA encephalitis, and limbic encephalitis. Hashimoto's encephalopathy is defined by the presence of thyroid peroxidase antibodies in patients with encephalitis that responds to steroids. It can cause non-specific neurological symptoms. NMDA encephalitis commonly affects young women and can cause psychiatric issues, decreased consciousness, and movement disorders. Diagnosis involves detecting NMDA receptor antibodies in CSF or serum. Limbic encephalitis involves inflammation of limbic structures and is associated with antibodies against proteins like LGI1; it typically causes memory loss, behavioral changes and seizures in older patients.
Pediatrics notes about "Acute flaccid paralysis". These notes were published in 2018.
You can download them from
- Telegram: https://t.me/pediatric_notes_2018
- Mediafire: http://www.mediafire.com/folder/u5u60m184t9z7/Pediatric_Notes_2018
Congenital myasthenic syndrome (CMS) is a rare heterogeneous group of genetic disorders that affect neuromuscular transmission. CMS can present at birth or in early infancy with symptoms like ptosis, external ophthalmoplegia, proximal limb weakness, and respiratory difficulties. Diagnosis involves neurophysiology testing showing a decremental response on repetitive nerve stimulation and increased jitter on single fiber EMG. Genetic testing and muscle biopsy can help identify the underlying cause, which may include defects in presynaptic, synaptic, or postsynaptic proteins. Treatment involves cholinesterase inhibitors, potassium channel blockers, beta-2 agonists, or open channel blockers depending on the CMS subtype. Supportive care like respiratory
This presentation provides an overview of demyelinating diseases, focusing on multiple sclerosis (MS). It defines demyelinating diseases as those that cause myelin destruction while sparing other nervous system elements. MS is described as an autoimmune, inflammatory demyelinating disease of the central nervous system (CNS) that is more common in women. The presentation covers the pathology, clinical features, investigations, and treatment approaches for MS.
This document summarizes key information about multifocal motor neuropathy (MMN):
1) MMN is a rare, purely motor neuropathy characterized by asymmetric motor deficits predominantly affecting the upper limbs, with diagnostic clues including conduction block and anti-GM1 antibodies.
2) Clinical features include distal weakness without sensory loss, and electrophysiology shows motor conduction block. Treatment involves intravenous immunoglobulin which provides benefit for most patients.
3) The pathophysiology likely involves autoimmune attack mediated by IgM antibodies against the ganglioside GM1, disrupting paranodal function and conduction. While chronic, MMN has a relatively benign prognosis with treatment.
references:
Phases and Phenotypes of Multiple Sclerosis By Orhun H. Kantarci, MD.
Diagnosis of Multiple Sclerosis By Jiwon Oh, MD, PhD, FRCPC
Nature Reviews | Disease Primers
Multiple sclerosis Massimo Filippi1,2*, Amit Bar- Or3, Fredrik Piehl4,5,6, Paolo Preziosa1,2, Alessandra Solari7, Sandra Vukusic8 and Maria A. Rocca1,2
Dr. Shubham Garg discusses neuromyelitis optica (NMO), an autoimmune condition where antibodies attack aquaporin-4 in the central nervous system. NMO predominantly affects women and has a median age of onset of 32-41 years. Key clinical features include transverse myelitis, typically longitudinally extensive, and severe optic neuritis. Treatment involves high-dose steroids for acute attacks and immunosuppressants like azathioprine to reduce relapse rates. Prognosis is generally worse than multiple sclerosis due to risk of cumulative disability, though relapse rates can be lowered with appropriate treatment.
This document provides an overview of various demyelinating diseases of the central nervous system. It begins by defining demyelinating diseases as those involving disruption of myelin, which forms an insulating sheath around axons. It then classifies and describes several specific diseases, including acute disseminated encephalomyelitis (ADEM), inflammatory demyelinating pseudotumor, multiple sclerosis (MS), neuromyelitis optica, central pontine myelinolysis, HIV encephalopathy, progressive multifocal leukoencephalopathy (PML), and others. For each disease, it discusses clinical features, magnetic resonance imaging (MRI) findings, differential diagnoses, and pathology where relevant.
Paraneoplastic syndrome (PNS) is the term used to refer to the disorders that accompany the benign or the malignant tumors and are not caused by mass effect or invasion / metastasis.
These disorders are triggered by an immune system response to neuronal proteins expressed by the tumor(onconeural proteins).
These PNS also occur due to substances secreted by the neoplasm itself.
This document discusses peripheral neuropathy and provides guidance on evaluating and diagnosing peripheral nerve disorders. It defines peripheral neuropathy as disorders affecting the peripheral nervous system, which can involve sensory nerves, motor nerves, or both. The document outlines that peripheral neuropathies can be classified based on whether they primarily affect the cell body, myelin, or axon. It also lists common causes of peripheral neuropathy like diabetes, paraproteinemia, alcohol misuse, and vitamin B12 deficiency. The document provides guidance on clinical assessment, laboratory and electrodiagnostic testing, skin or nerve biopsy, and treatment approaches for peripheral neuropathy.
A 20-year-old male presented with headaches, double vision, difficulty swallowing and nasal regurgitation over the past 1-3 weeks. On examination, he had neck stiffness and multiple cranial nerve palsies. Imaging showed diffuse pachymeningeal enhancement and bilateral papilledema. Cerebrospinal fluid analysis was consistent with basal meningitis. He was started on antitubercular treatment but developed severe anemia and splenomegaly, and was ultimately diagnosed with acute lymphocytic leukemia involving the central nervous system.
Valproic acid is an anticonvulsant medication used to treat epilepsy and bipolar disorder. It works by increasing brain levels of the inhibitory neurotransmitter GABA. Common side effects include nausea, vomiting, tremors, and weight gain. It can cause serious liver toxicity and birth defects, so risks must be considered. The dosage is individualized based on a person's medical condition, age, and other factors. Therapeutic drug monitoring is important to ensure blood levels remain within the target range for maximum benefit and minimum harm.
MOGAD is an inflammatory demyelinating disease of the CNS associated with antibodies targeting myelin oligodendrocyte glycoprotein (MOG). It can present with optic neuritis, acute disseminated encephalomyelitis (ADEM)-like attacks, brainstem syndromes, cerebral cortical encephalitis, or myelitis. MRI often shows large or longitudinally extensive lesions of the optic nerve, brain, or spinal cord. Pathology demonstrates perivenous demyelination, MOG-dominant myelin loss, and predominant CD4+ T-cell inflammation. MOGAD has a more heterogeneous clinical and radiological presentation compared to other CNS demyelinating diseases like multiple s
Treatment of chronic inflammatory demyelinating polyneuropathyMohamadAlhes
This document summarizes treatment options for chronic inflammatory demyelinating polyneuropathy (CIDP). The main treatments are immunoglobulin therapy (IVIG or SCIG), corticosteroids, and plasmapheresis. IVIG and plasmapheresis provide equivalent short-term benefits but most patients require ongoing intermittent treatment. Corticosteroids can induce remission but have significant side effects with long-term use. Treatment must be tailored to the individual patient based on disease severity and response to initial therapies.
Clozapine is an atypical antipsychotic drug used to treat treatment-resistant schizophrenia. It carries serious risks of agranulocytosis (low white blood cell count), thromboembolism (blood clots), and myocarditis (heart inflammation).
For agranulocytosis, the risk is highest in the first 18 weeks and patients must receive weekly blood monitoring during this period. Thromboembolism risk is increased in the first 3 months and can be reduced through encouraging exercise and hydration. Myocarditis typically occurs within the first 8 weeks and patients should be monitored for symptoms like chest pain and fever.
Due to these serious risks, clozapine is generally only
Atrial fibrillation is the most common type of cardiac arrhythmia. It is the leading cardiac cause of stroke
https://www.youtube.com/watch?v=4pSobW-a6gQ&list=PL2XcrMWxPBLQyEdWnJuO4qSI2m-WTrglH&pp=gAQBiAQB
1) Patients suffering from subarachnoid hemorrhage (SAH) are at risk of developing acute cardiopulmonary complications within the first week, such as cardiac dysfunction, pulmonary edema, and pneumonia.
2) Approximately 30% of SAH patients experience neurogenic myocardial stunning within the first week, caused by a catecholamine surge, which can lead to reduced cardiac output and negatively impact cerebral perfusion.
3) Pulmonary complications occur in 20% of SAH patients and increase the risks of vasospasm and further medical issues. They require careful monitoring and management of fluids to prevent overloading.
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by features of accelerated aging. It is caused by a mutation in the LMNA gene which leads to production of a defective protein called progerin. Children with HGPS appear normal at birth but start exhibiting aging-related problems at a very early age. They experience severe failure to thrive, loss of body fat and hair, stiff joints, and cardiovascular complications leading to death usually in their teens. The only approved treatment is lonafarnib which works by inhibiting progerin production and has shown benefits in improving growth and reducing disease severity. A multidisciplinary care approach is needed to manage the various health issues associated with H
1. Psychotropic drugs can cause weight gain through several mechanisms including antagonism of 5-HT2A/2C receptors, antihistaminic effects, and inducing insulin resistance.
2. Drugs that antagonize 5-HT2A/2C receptors or have antihistaminic effects include certain antidepressants and antipsychotics. Drugs known to cause insulin resistance and significant weight gain include olanzapine, clozapine, and quetiapine.
3. The extent of weight gain varies by drug, with clozapine and olanzapine associated with mean weight increases of 5-12kg in the first year, compared to 2.5-5kg for qu
This document provides an overview of subarachnoid hemorrhage (SAH). It discusses the anatomy and histology of cerebral vasculature, epidemiology of SAH including risk factors and causes, classification of cerebral aneurysms, grading scales for SAH severity, clinical presentation, investigations including imaging and lumbar puncture, and laboratory tests. The epidemiology section notes that SAH accounts for 1-6% of strokes, has a high case fatality rate of up to 51%, and is most commonly caused by rupture of a cerebral aneurysm. Clinical presentation involves sudden, severe headache as the most common symptom along with possible neurological deficits, while investigations include noncontrast CT, CTA, MRI, lumbar puncture,
Fabry disease is a rare genetic disorder caused by deficient activity of the enzyme alpha-galactosidase A. This results in accumulation of globotriaosylceramide and related molecules in the body's cells. It is an X-linked recessive condition affecting males more severely than females. Symptoms involve the skin, eyes, kidneys, heart, and nervous system. Diagnosis is confirmed through enzyme testing or genetic analysis. Treatment includes enzyme replacement therapy with agalsidase beta, agalsidase alfa, or pegunigalsidase alfa. Migalastat is a pharmacological chaperone drug that can also be used. Management of organ-specific complications is also important.
This document discusses diagnosing and differentiating between primary and secondary headache disorders. It begins by explaining that headache is a common symptom neurologists evaluate and can be caused by many underlying diseases. The challenges are that primary headaches are prevalent, so secondary headaches may co-occur with primary types. Various classification systems and criteria for primary vs. secondary headaches are reviewed. Causes of secondary headaches like subarachnoid hemorrhage, cerebral vasoconstriction syndrome, spontaneous intracranial hypotension, and idiopathic intracranial hypertension are then discussed in detail, including related symptoms, imaging findings, diagnostic criteria and management considerations.
Natalizumab is a monoclonal antibody approved to treat relapsing-remitting multiple sclerosis. It works by blocking leukocyte migration across the blood-brain barrier. While effective at reducing MS relapses, it carries a risk of progressive multifocal leukoencephalopathy due to John Cunningham virus reactivation. PML is a rare brain infection that can cause severe disability or death. The risk of PML from natalizumab increases with treatment duration over 24 months, presence of anti-JCV antibodies, and prior immunosuppressant use. Discontinuing natalizumab may lead to PML immune reconstitution inflammatory syndrome, worsening neurologic symptoms.
1) Stroke is the fifth leading cause of death in the USA, with approximately 85% being ischemic strokes, over half occurring in the MCA territory.
2) The MCA arises from the internal carotid artery and supplies blood to regions of the frontal, parietal, and temporal lobes. Occlusions in different segments of the MCA result in variable neurological deficits.
3) Occlusion of the upper MCA division causes hemiplegia and sensory loss on the contralateral side of the body, as well as visual field defects. Occlusion of the lower division can cause visual field defects or aphasic disturbances depending on whether the left or right hemisphere is involved.
Serotonin syndrome occurs when there is too much serotonin in the brain and can range from mild to severe symptoms. Mild symptoms include nervousness, nausea, diarrhea and dilated pupils. Moderate symptoms add agitation, muscle twitching and sweating. Severe symptoms involve confusion, high blood pressure, fever and seizures. Serotonin syndrome is caused by drugs that inhibit serotonin reuptake or metabolism including antidepressants, pain medications, herbal supplements and illegal drugs. Treatment depends on severity from stopping the causing medication for mild cases to intensive care admission for severe cases and may include sedation, IV fluids and controlling heart rate and fever.
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- Treatment involves immunomodulation with pyridostigmine, corticosteroids, immunosuppressants like azathioprine and mycophenolate, IVIG, or plasma exchange.
- Diagnosis is based on symptoms, serologic testing for acetylcholine receptor (AChR) or muscle-specific kinase (MuSK) antibodies, and electrodiagnostic testing showing decremental response on repetitive nerve stimulation.
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Overall life span (LS) was 1671.7±1721.6 days and cumulative 5YS reached 62.4%, 10 years – 50.4%, 20 years – 44.6%. 94 LCP lived more than 5 years without cancer (LS=2958.6±1723.6 days), 22 – more than 10 years (LS=5571±1841.8 days). 67 LCP died because of LC (LS=471.9±344 days). AT significantly improved 5YS (68% vs. 53.7%) (P=0.028 by log-rank test). Cox modeling displayed that 5YS of LCP significantly depended on: N0-N12, T3-4, blood cell circuit, cell ratio factors (ratio between cancer cells-CC and blood cells subpopulations), LC cell dynamics, recalcification time, heparin tolerance, prothrombin index, protein, AT, procedure type (P=0.000-0.031). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and N0-12 (rank=1), thrombocytes/CC (rank=2), segmented neutrophils/CC (3), eosinophils/CC (4), erythrocytes/CC (5), healthy cells/CC (6), lymphocytes/CC (7), stick neutrophils/CC (8), leucocytes/CC (9), monocytes/CC (10). Correct prediction of 5YS was 100% by neural networks computing (error=0.000; area under ROC curve=1.0).
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4. CIDP should be considered in patients presenting
with a progressive or relapsing-remitting
polyneuropathy involving both motor and sensory
nerves along with areflexia, particularly when
weakness predominates and affects proximal and
distal muscles simultaneously and symmetrically.
The diagnosis can be more difficult in patients
presenting with atypical CIDP variants
5. The age of onset is younger (mean 29 years) in
those who had a relapsing course than in those
experiencing a chronic progressive course (mean
51 years).
A history of preceding infection is found in less
than 10%.
The clinical features that distinguish CIDP from
axonal peripheral neuropathies are the prominence
of muscle weakness and the early involvement of
upper extremity and proximal muscles as well as
distal muscles.
6. Axonal polyneuropathies are characterized by
predominantly distal weakness, deep tendon
reflexes are globally reduced or absent in CIDP,
whereas only the ankle reflexes are diminished in
typical axonal polyneuropathies.
The features of CIDP point to the multifocal or
generalized nature of the disease even at early
stages of the illness.
Children have a more precipitous onset and more
prominent gait abnormalities
Pregnancy is associated with relapses, occurring
mainly in the 3rd trimester and postpartum period
7. Additional findings are
1. Postural tremor of the hands,
2. Enlargement of peripheral nerves,
3. Papilledema and facial or bulbar weakness.
4. Rarely, respiratory failure requiring mechanical
ventilation or autonomic dysfunction
5. Massive nerve root enlargement, causing
myelopathy or symptomatic lumbar stenosis
6. CIDP may be associated with a relapsing
multifocal demyelinating CNS disorder
resembling MS with CNS demyelination
8. Elements that raise suspicion for an alternative
diagnosis include:
1. The presence of weakness in the respiratory
muscles.
2. Clear asymmetric pattern of weakness.
3. Severe tremor, ataxia, or muscle weakness at
disease onset
4. Prominent autonomic dysfunction
5. Prominent pain
6. No improvement after one or more effective
therapies (eg, glucocorticoids, IVIG)
9. CIDP is a syndrome with many underlying causes.
1) CIDP is more than 10 times more frequent in
patients with IDDM and NIDDM,
2) HIV-1 infection, Chronic active hepatitis,
3) SLE, Inflammatory bowel disease.
4) Monoclonal gammopathy of undetermined
significance (MGUS),
5) Hematological malignancies (including
Waldenström macroglobulinemia [WM], multiple
myeloma, POEMS syndrome, and Castleman
disease)
6) Lymphomas
7) Melanoma or after therapy by vaccination with
melanoma
10. CIDP may occasionally be drug induced.
1. Tacrolimus,
2. Macrolide antibiotic
3. TNF-α antagonists, including etanercept,
infliximab, and adalimumab
4. Interferon-alpha
The neuropathic manifestations in these patients
may start as early as 2weeks after initiation of
therapy or as late as 16months
12. Electrodiagnostic testing
Electrophysiologic features of CIDP are those of
peripheral nerve demyelination:
1. Partial conduction block (drop in CMAP
amplitude or area of more than 20%)
2. Conduction velocity slowing
3. Prolonged distal motor latencies
4. Delay or disappearance of F waves
5. Temporal dispersion (increase in the CMAP
duration of more than 15%); suggest an
acquired process.
13.
14. Needle EMG is consistent with polyradiculopathy
pattern with fibrillation potentials and large MUAPs
with reduced recruitment involving multiple
myotomes with or without involvement of the
paraspinal muscles
15. It is difficult to determine conduction slowing in
patients who have severe axonal loss.
Evaluating nerves that supply more proximal and
less severely denervated muscles is helpful in
finding the conduction abnormalities.
Extensive studies of the 4limbs improve the
electrodiagnostic yield.
Cranial nerve conduction studies; showing
conduction slowing is useful for diagnosis.
16. Distal acquired demyelinating symmetric
neuropathy; NCS showed symmetrical and
uniform slowing of distal latencies more than
conduction velocities with rare conduction
blocks.
MADSAM (multifocal asymmetric
demyelinating sensory and motor) neuropathy;
NCS demonstrating focal conduction block or
severe slowing of nerve conduction.
Sensory nerve root involvement is suggested by
abnormal somatosensory evoked responses,
17. Electrodiagnostic criteria for CIDP
These criteria are applied by testing the median,
ulnar (stimulated below the elbow), peroneal
(stimulated below the fibular head), and tibial
nerves on one side of the body.
During testing, limb temperature should be no
less than 33°C at the palm and no less than 30°C
at the external malleous
18. Definite CIDP
At least one of the following:
1. MDL prolongation ≥50% above ULN in 2nerves,
2. Reduction of MCV ≥30% below LLN in 2nerves,
3. Prolongation of F-wave latency ≥20% above ULN
in 2nerves (≥50% if amplitude of distal negative
peak CMAP <80% of LLN),
4. Absence of F-waves in 2nerves (if these nerves
have distal negative peak CMAP amplitudes ≥20%
of LLN) + ≥1 other demyelinating parameter in ≥1
other nerve,
19. 5. MCB: ≥30% reduction of the proximal relative to
distal negative peak CMAP amplitude, excluding
the tibial nerve, and distal negative peak CMAP
amplitude ≥20% of LLN in 2nerves; or in 1nerve
+ ≥ 1 other demyelinating parameter except
absence of F-waves in ≥1 other nerve,
6. Abnormal temporal dispersion: >30% duration
increase between the proximal and distal negative
peak CMAP (at least 100% in the tibial nerve) in
≥2 nerves,
7. Distal CMAP duration (interval between onset of
the 1st negative peak and return to baseline of the
last negative peak) prolongation in ≥1 nerve + ≥1
other demyelinating parameter in ≥1 other nerve
20. Probable CIDP
≥30% amplitude reduction of the proximal
negative peak CMAP relative to the distal,
excluding posterior tibial nerve, if the distal
negative peak CMAP is ≥20% of LLN, in two
nerves plus at least one other demyelinating
parameter (meeting any of the definite criteria)
one other nerve
Possible CIDP
As in 'Definite CIDP but in only one nerve
21. Laboratory studies
1. Fasting serum glucose and/or oral glucose
tolerance test, HbA1c
2. CBC, LFT, TFT, Serum calcium and creatinine
3. Serum protein electrophoresis (SPEP) and
Immunofixation
4. Serum free light chain (FLC) assay, or 24-hour
urine protein electrophoresis (UPEP) and
Immunofixation
5. CRP, ANAS, Angiotensin-converting enzyme
22. Laboratory studies
7. Hepatitis panel, HIV antibody, Borrelia burgdorferi
serology
8. Chest radiograph
9. Skeletal survey and vascular endothelial growth
factor (VEGF) if a monoclonal gammopathy is
found
10.Evaluation for inherited neuropathies as CMT or
transthyretin (TTR) familial amyloid
polyneuropathy (FAP)
11.Testing for anti-MAG is recommended if an IgM
gamopatny is identified. Neurofascin and contactin 1
(CNTN1) antibody testing
23. Lumbar puncture
CSF analysis is recommended in patients with
suspected CIDP particularly in whom the clinical and
electrophysiologic findings are inconclusive.
CSF protein is elevated (45 mg/dLl and WBC is
normal) in over 80%. An elevated CSF protein level
in patients with DM should be attributed to CIDP if
>100mg/dl.
24. Lumbar puncture
An increased CSF WBC of >10 cells/mm³ should
suggest a diagnosis other than CIDP as infection,
inflammation or neoplasm. An exception to this
general rule is that patients with HIV infection may
have pleocytosis, although the CSF WBC count in
patients with CIDP and HIV infection is generally
<50/mm²
25. Neuroimaging
MRI with gadolinium of the spinal cord, spinal
roots, cauda equina, brachial plexus, lumbosacral
plexus, and other nerve to look for enlarged or
enhancing nerves.
MRI is usually reserved for atypical cases, often
when clinical and electrophysiologic findings are
focal (as multifocal CIDP), and to rule out other
causes of neuropathy and infiltrative pathology.
26. Neuroimaging
MRI is important if chronic immune sensory
polyradiculopathy (CISP) or chronic immune
sensorimotor polyradiculopathy (CISMP) are being
considered, as these are rare variants with clinical
features that overlap with structural. infectious, and
infiltrative causes of polyradiculopathy.
In brachial plexus MRI, nerve enlargement or
enhancement is seen in approximately 40%-30% of
patients with CIDP.
27.
28. (A)T2 showing lack of regular fluid-isointense signal, due to
swollen cauda equina fibers (arrows).
(B)T1, Diffuse cauda equina enhancement (arrows) is
depicted, indicating inflammation.
(C)T1, Enlarged and enhancing root fibers are shown, exiting
the neuroforamen (arrows).
29. Nerve ultrasound
When appropriate expertise is available,
neuromuscular ultrasound can also be used to
detect nerve hypertrophy in patients with
acquired and hereditary forms of chronic
demyelinating neuropathies. Although the
findings are not specific for CIDP, they may help
indicate regions of involvement
30. Median nerve
A, Cross-sectional area of the median nerve measuring 8 mm
B, Cross-sectional image at 7 cm proximal to the antecubital fossa,
measuring 21 mm.
C, Longitudinal image demonstrating focal enlargement and
hypoechogenicity.
31. Nerve biopsy
Typically, the sural nerve is biopsied, but other
nerves include the superficial peroneal, superficial
radial, and gracilis motor nerve.
Limitation of nerve biopsy is suboptimal sensitivity
and specificity. CIDP is a multifocal disorder, and
motor nerve fibers tend to be more affected than
sensory nerves, the inflammatory component of
CIDP may not be prominent and thus may not be
apparent on biopsy.
32. Nerve biopsy
Nerve biopsy can provide solid evidence of
demyelination. In addition biopsy reveals other
neuropathies that mimic CIDP, as those due to
amyloidosis, sarcoidosis and vasculitis .
Electron microscopy and teased fiber analysis of
nerve biopsy specimens is highly desirable
34. AIDP
There is a temporal continuum between AIDP and
CIDP. The time course of progression and the
occurrence of relapses are used to distinguish
between these entities:
GBS commonly reaches its nadir within 3-4 weeks
but does not progress beyond 8 weeks.
CIDP continues to progress or has relapses for
greater than 8weeks.
35. AIDP
Subacute inflammatory demyelinating
polyneuropathy (SIDP) is the term used by some
authors for disease that reaches its nadir between
4-8weeks.
GBS is typically monophasic, but up to two
relapses in the first 8weeks from onset can occur.
3 or more relapses in the first 8weeks is highly
suggestive of acute CIDP. Relapses closer to the
eight-week time period is more suggestive of
CIDP.
36. AIDP
Observation of the patient over time can clarify
whether the clinical course is that of AIDP or
CIDP, and therapeutic interventions are likely to
be initiated before a patient reaches a specific
time point that distinguishes between these
entities. As an example, some patients with CIDP
have a subacute onset resembling that seen in
GBS, and CIDP is recognized only after relapses
or progression occur over the ensuing few
months.
37. AIDP
Clinical features that distinguish AIDP from CIDP.
1. The onset of GBS is usually easily identified,
while the onset of CIDP is typically less clear.
2. Antecedent events are more frequent with GBS
than in CIDP. 70% of AIDP cases are preceded by
an infectious illness, vaccination, or surgery by 3-
4 weeks prior to the onset of clinical symptoms.
The antecedent event prior to CIDP in no more
than 30%
38. AIDP
3. Prominent sensory signs (ie, sensory ataxia
and impaired vibration and pinprick
sensation).
4. Cranial nerve involvement is more common
in GBS.
5. The need for ventilator support favors GBS .
6. Autonomic involvement in the form of labile
hypertension, heart rhythm disorders, and
gastrointestinal dysmotility also favors GBS.
39. Chronic demyelinating neuropathies
There are several forms of chronic demyelinating
neuropayhies are distinct from CIDP on the basis of
clinical, electrodiagnostic, and therapeutic
differences. These include:
1) Multifocal motor neuropathy (MMN).
2) Distal acquired demyelinating symmetric
neuropathy (DADS) with monoclonal IgM
gammopathy and anti-myelin-associated
glycoprotein antibodies (anti-MAG).
40. Chronic demyelinating neuropathies
3. IgM-associated demyelinating neuropathies as
CANOMAD (chronic ataxic neuropathy with
ophthalmoplegia, IgM paraprotein, cold
agglutinins, and disialosyl antibodies).
CANOMAD is a chronic disorder with clinical
features similar to the Miller Fisher variant of
GBS. In CANOMAD, the GQ1b antibody is an
IgM antibody; in Miller Fisher syndrome, it is an
IgG antibody. Other IgM antibodies associated
with neuropathy include GD1a and GD1b, both
of which tend to cause a sensory-predominant
disorder.
41. Chronic demyelinating neuropathies
4) POEMS syndrome (osteosclerotic myeloma:
Polyneuropathy, Organomegaly, Endocrinopathy,
Monoclonal protein, Skin changes).
5) Demyelinating neuropathy associated with
medications such as tumor necrosis factor-alpha
blockers and checkpoint inhibitors
42. Chronic demyelinating neuropathies
CIDP is reported in association with a variety of
systemic illnesses as Lyme disease, hepatitis B or C,
HIV infection, SLE and other collagen vascular
disorders, thyroid disease, nephrotic syndrome,
solid organ or bone marrow transplantation, and
inflammatory bowel disease. The associations are
not necessarily causative, and in some cases, the
systemic illness is associated with increased risk for
more than one type of neuropathy
43. Genetic mimics of CIDP
Certain genetic disorders of peripheral nerve myelin
have characteristics that can mimic the clinical or
electrodiagnostic features of CIDP or its variants.
These include:
1) CMT disease, particularly CMT1A, adult-onset
CMT1B, CMT1X, and recessive cases as CMT4,
can cause multifocal, nonuniform slowing and
conduction block.
2) Hereditary neuropathy with liability to pressure
palsies, which causes conduction slowing at
compression sites.
44. Genetic mimics of CIDP
3) Transthyretin (TTR) familial amyloid
polyneuropathy (FAP) due to mutations in the TTR
gene is typically axonal but occasionally can
manifest as a demyelinating polyneuropathy with
features that overlap with CIDP, particularly
sporadic, late-onset (>50 years) forms of the
disease. Clues that may alert the clinician to the
possibility of TTR-FAP include prominent pain,
dysautonomia, distal upper limb motor deficits,
extension of small fiber sensory loss above the
wrist, and absence of ataxia. Sequencing of the TTR
gene can confirm the diagnosis. This is of particular
importance as new therapies for FAP are available.
45. Genetic mimics of CIDP
A careful family history and examination of parents
and siblings are important if these disorders are a
consideration, although absence of a family history
does not rule out a genetic cause. Appropriate
genetic testing should be considered in select
patients, particularly for peripheral myelin protein
22 (PMP22) gene duplication or deletion, connexin
32, and TTR.
47. Diagnosis
The following criteria support the diagnosis of the
classic form of CIDP:
1) Progression over at least two months
2) Weakness more than sensory symptoms
3) Symmetric involvement of arms and legs
4) Proximal muscles involved along with distal
muscles
48. Diagnosis
5. Widespread reduction or loss of deep tendon
reflexes
6. CSF protein without pleocytosis
7. Nerve conduction evidence of a demyelinating
neuropathy
8. Nerve biopsy evidence of segmental demyelination
with or without inflammation
9. Gait ataxia secondary to large fiber sensory loss
There is still no gold-standard set of diagnostic criteria
for demyelination, or for the diagnosis of CIDP and its
variants
49. EFNS/PNS criteria
The EFNS/PNS guideline defines CIDP as typical or
atypical. The diagnosis of CIDP is based upon
clinical, electrodiagnostic, and supportive criteria:
A. Clinical inclusion criteria for typical CIDP
require both of the following:
I. Chronically progressive or recurrent symmetric
proximal and distal weakness and sensory
dysfunction of all extremities, developing over
at least 2months: cranial nerves may be affected
II. Absent or reduced tendon reflexes in all
extremities
50. EFNS/PNS criteria
B. Clinical inclusion criteria for atypical CIDP require one of
the following, but otherwise as in typical CIDP. tendon
reflexes may be normal in unaffected limbs:
I. Predominantly distal (distal acquired demyelinating
symmetric neuropathy [DADS]) or
II. Asymmetric (multifocal acquired demyelinating sensory
and motor neuropathy [MADSAM), Lewis-Sumner
syndrome) or
III. Focal (eg, involvement of the brachial or lumbosacral
plexus or of one or more peripheral nerves in one upper or
lower limb); or
IV. Pure motor
V. Pure sensory (including chronic immune sensory
polyradiculopathy [CISP] affecting the central process of
the primary sensory neuron)
51. EFNS/PNS criteria
C. Clinical exclusion criteria:
I. Neuropathy probably caused by B. burgdorferi infection,
diphtheria, drug or toxin exposure
II. Hereditary demyelinating neuropathy
III. Prominent sphincter disturbance
IV. Diagnosis of multifocal motor neuropathy (MMN)
V. IgM monoclonal gammopathy with high titer antibodies to
myelin-associated glycoprotein (MAG)
VI. Other causes for a demvelinating neuropathy including
POEMS svndrome. osteosderotic myeloma, and diabetic
and nondiabetic lumbosacral radiculoplexus neuropathy;
peripheral nervous system lymphoma and amyloidosis may
occasionally have demyelinating features
52. EFNS/PNS criteria
Supportive criteria:
1) Elevated CSF protein with TLC <10/mm³, MRI showing
gadolinium enhancement and/or hypertrophy of the cauda equina,
lumbosacral or cervical nerve roots, or the brachial or
lumbosacral plexuses
2) Abnormal sensory electrophysiology in at least 1nerve:
I. Normal sural with abnormal median or radial SNAP amplitudes;
or
II. Conduction velocity <80% of LLN (<705 if SNAP amplitude
<80% of LLN; or
III. Delayed SSEPs without CNS disease
3) Clinical improvement following immunomodulatory treatment
4) Nerve biopsy showing unequivocal evidence of demyelination
and/or remyelination by electron microscopy or teased fiber
analysis
53. Koski criteria
The Koski criteria require the following:
A. Chronic polyneuropathy, progressive for at least eight
weeks
B. No serum paraprotein and no genetic abnormality,
and either.
I. Recordable CMAP in at least 75% of motor nerves
and either abnormal distal latency or abnormal motor
conduction velocity or abnormal F wave latency in
>50% of motor nerves; or
II. Symmetric onset or symmetric exam and weakness
in all four limbs and proximal weakness in at least
one limb