Arnold Chiari Malformation, also known as Chiari type II malformation, is one of a group of brain malformations affecting the cerebellum. Named after Hans Chiari and Julius Arnold, the pathologists who first described the group of malformations. This condition is almosNormally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation. In Arnold Chiari Malformation, both cerebellar and brain stem tissue protrude into the foramen magnum and the cerebellar vermis (the nerve tissue that connects the two halves of the cerebellum) may be only partially complete or absent
t always associated with myelomeningocele, the most serious form of spina bifida
Dandy-Walker Syndrome (DWS) is a congenital (happening before birth) condition where the cerebellum does not develop normally. The cerebellum is an area at the back of the brain that controls movement and balance.
With DWS, parts of the cerebellum may never develop or may be very small. Other parts of the cerebellum become filled with fluid or develop small masses of fluid (cysts). In most cases, a build-up of fluid inside the skull causes the head to get bigger than it should.
Syringomyelia is a condition where a cyst, called a syrinx, develops in the spinal cord. It most commonly affects the lower cervical spine. It is often associated with abnormalities of the skull or spinal column. The majority of cases are linked to Chiari malformation type 1, where the cerebellar tonsils are displaced into the spinal canal. Symptoms vary depending on the location of the syrinx but can include pain, loss of sensation, muscle weakness or atrophy, and autonomic dysfunction. Diagnosis is made using imaging like MRI. Treatment involves surgery to decompress pressure on the spinal cord like laminectomy with the goal of resolving the syrinx.
1. Stroke is defined as a nontraumatic brain injury caused by occlusion or rupture of cerebral blood vessels that results in sudden neurological deficits.
2. The most common types of stroke are ischemic (85%) and hemorrhagic (15%). Ischemic strokes are further classified as thrombotic, embolic, or lacunar.
3. Major risk factors for stroke include hypertension, heart disease, diabetes, smoking, and older age. Location of brain injury determines the specific neurological symptoms, such as deficits on one side of the body for middle cerebral artery strokes.
Transverse myelitis is a rare neurological condition where the spinal cord becomes inflamed across its width. It is often caused by an autoimmune response following a viral infection. Symptoms depend on the level of spinal cord involvement and may include sensory changes, motor weakness, and sphincter disturbances. Diagnosis involves ruling out other causes and showing signs of spinal cord inflammation. The goals of physiotherapy are to improve strength, mobility, and independence through exercises and management of issues like spasticity and skin care.
This document provides an overview of syringomyelia, including its pathogenesis, pathology, classification, clinical features, and natural history. Syringomyelia is a condition characterized by fluid-filled cavities within the spinal cord. It most commonly affects the cervical and thoracic regions. Clinical features include pain and sensory loss. The natural history varies, but symptoms typically progress slowly over years, with some patients experiencing stabilization or spontaneous resolution in rare cases.
This document discusses spinal cord injuries, including the anatomy of the spine, mechanisms of injury, types of injury, assessment, management in the acute phase, and long term care. It notes that spinal cord injuries can cause paralysis, sensory deficits, and autonomic dysfunction depending on the level and completeness of injury. Initial management involves immobilization, minimizing secondary injury, and addressing airway, breathing and circulation problems. Definitive care includes early surgical fixation and rehabilitation to prevent complications like pressure sores, thrombosis, and psychological issues.
1) The document discusses various syndromes that can result from lesions or occlusions in different parts of the posterior circulation arteries that supply the brainstem and cerebellum.
2) Specific syndromes are described based on the location of the lesion, including PCA, vertebral artery, and basilar artery syndromes. Onset, signs and symptoms on both sides of the lesion are outlined.
3) Midbrain, pontine, and medullary syndromes are also detailed. Bilateral lesions causing Anton's syndrome and Balint's syndrome are mentioned. A variety of resulting neurological deficits are associated with different posterior circulation artery occlusions.
Arnold-Chiari malformation involves the downward displacement of brain tissue through the foramen magnum. It can cause a range of symptoms from neck pain to difficulty swallowing. Diagnosis involves imaging tests like MRI. Treatment may include decompression surgery to create more space in the skull as well as shunt procedures to drain excess spinal fluid for conditions like hydrocephalus. Management requires a multidisciplinary team and surgery often provides symptom relief.
The document discusses cerebellar ataxia, a disorder caused by damage to the cerebellum that controls coordination. It causes loss of coordination, balance problems, and slurred speech. The cerebellum coordinates muscle movement and is located in the hindbrain. Causes include viruses, alcohol, tumors, and toxins. Symptoms are diagnosed through neurological exams, imaging scans, and lab tests. Treatment focuses on physical therapy, assistive devices, and treating any underlying causes to ease symptoms and improve quality of life. Prevention involves vaccinations to reduce risk of viral infections that can lead to cerebellar ataxia.
Syringomyelia is a condition where a cyst, called a syrinx, develops in the spinal cord. It most commonly affects the lower cervical spine. It is often associated with abnormalities of the skull or spinal column. The majority of cases are linked to Chiari malformation type 1, where the cerebellar tonsils are displaced into the spinal canal. Symptoms vary depending on the location of the syrinx but can include pain, loss of sensation, muscle weakness or atrophy, and autonomic dysfunction. Diagnosis is made using imaging like MRI. Treatment involves surgery to decompress pressure on the spinal cord like laminectomy with the goal of resolving the syrinx.
1. Stroke is defined as a nontraumatic brain injury caused by occlusion or rupture of cerebral blood vessels that results in sudden neurological deficits.
2. The most common types of stroke are ischemic (85%) and hemorrhagic (15%). Ischemic strokes are further classified as thrombotic, embolic, or lacunar.
3. Major risk factors for stroke include hypertension, heart disease, diabetes, smoking, and older age. Location of brain injury determines the specific neurological symptoms, such as deficits on one side of the body for middle cerebral artery strokes.
Transverse myelitis is a rare neurological condition where the spinal cord becomes inflamed across its width. It is often caused by an autoimmune response following a viral infection. Symptoms depend on the level of spinal cord involvement and may include sensory changes, motor weakness, and sphincter disturbances. Diagnosis involves ruling out other causes and showing signs of spinal cord inflammation. The goals of physiotherapy are to improve strength, mobility, and independence through exercises and management of issues like spasticity and skin care.
This document provides an overview of syringomyelia, including its pathogenesis, pathology, classification, clinical features, and natural history. Syringomyelia is a condition characterized by fluid-filled cavities within the spinal cord. It most commonly affects the cervical and thoracic regions. Clinical features include pain and sensory loss. The natural history varies, but symptoms typically progress slowly over years, with some patients experiencing stabilization or spontaneous resolution in rare cases.
This document discusses spinal cord injuries, including the anatomy of the spine, mechanisms of injury, types of injury, assessment, management in the acute phase, and long term care. It notes that spinal cord injuries can cause paralysis, sensory deficits, and autonomic dysfunction depending on the level and completeness of injury. Initial management involves immobilization, minimizing secondary injury, and addressing airway, breathing and circulation problems. Definitive care includes early surgical fixation and rehabilitation to prevent complications like pressure sores, thrombosis, and psychological issues.
1) The document discusses various syndromes that can result from lesions or occlusions in different parts of the posterior circulation arteries that supply the brainstem and cerebellum.
2) Specific syndromes are described based on the location of the lesion, including PCA, vertebral artery, and basilar artery syndromes. Onset, signs and symptoms on both sides of the lesion are outlined.
3) Midbrain, pontine, and medullary syndromes are also detailed. Bilateral lesions causing Anton's syndrome and Balint's syndrome are mentioned. A variety of resulting neurological deficits are associated with different posterior circulation artery occlusions.
Arnold-Chiari malformation involves the downward displacement of brain tissue through the foramen magnum. It can cause a range of symptoms from neck pain to difficulty swallowing. Diagnosis involves imaging tests like MRI. Treatment may include decompression surgery to create more space in the skull as well as shunt procedures to drain excess spinal fluid for conditions like hydrocephalus. Management requires a multidisciplinary team and surgery often provides symptom relief.
The document discusses cerebellar ataxia, a disorder caused by damage to the cerebellum that controls coordination. It causes loss of coordination, balance problems, and slurred speech. The cerebellum coordinates muscle movement and is located in the hindbrain. Causes include viruses, alcohol, tumors, and toxins. Symptoms are diagnosed through neurological exams, imaging scans, and lab tests. Treatment focuses on physical therapy, assistive devices, and treating any underlying causes to ease symptoms and improve quality of life. Prevention involves vaccinations to reduce risk of viral infections that can lead to cerebellar ataxia.
Tuberculous infection of the central nervous system (CNS) can occur via hematogenous spread or direct extension from a local infection. It most commonly manifests as tuberculous meningitis or tuberculomas. Tuberculous meningitis involves thick exudate in the subarachnoid space and can lead to hydrocephalus or ischemic infarcts. Tuberculomas appear as ring-enhancing lesions on imaging. Pott's disease is spinal tuberculosis that causes vertebral body collapse and kyphosis. Management involves antituberculous medications for at least 6-9 months.
A 42-year-old male was admitted to the emergency department after a motor vehicle accident with back pain and inability to move his lower limbs. Examination revealed absent sensation and paralysis below T12 with MRI showing a lesion at T11. He was diagnosed with complete T11 paraplegia and underwent surgery. Rehabilitation focused on preventing complications like pressure sores, infections, and maintaining mobility. Spinal cord injuries cause various impairments depending on level and severity, and patients require long-term management of physical, psychological, and social impacts on quality of life.
Stroke (cerebrovascular accident [CVA]) is the sudden loss of neurological function caused by an interruption of the blood flow to the brain.
Impairments may resolve spontaneously as brain swelling subsides (reversible ischemic neurological deficit), generally within 3 weeks.
Residual neurological impairments are those that persist
longer than 3 weeks and may lead to lasting disability
This document discusses spinal dysraphism, which refers to congenital anomalies resulting from failed fusion of the dorsal spinal elements. It describes different types including spina bifida occulta, meningocele, myelomeningocele, myelocystocele, and lipomeningocele. Meningocele involves a mass composed of CSF, meninges, and skin, but no neural elements. Myelomeningocele contains neural tissue and is usually associated with neurological deficits. Myelocystocele and lipomeningocele involve fatty tissue herniating through spinal defects. Radiological imaging plays an important role in diagnosis, with ultrasound, CT, MRI, and myelography discussed.
This document provides information about transverse myelitis (TM), including its definition, etiology, presentation, diagnosis, treatment, and prognosis. Some key points:
- TM is defined as acute spinal cord inflammation and injury that causes neurological deficits. It can be post-infectious, post-vaccination, or associated with MS.
- Presentation involves varying degrees of motor weakness, sensory alterations, and autonomic dysfunction below a certain spinal level. Up to half of idiopathic cases follow a respiratory or gastrointestinal illness.
- Diagnosis involves ruling out other causes through MRI, CSF analysis showing inflammation, and clinical features meeting criteria. Treatment begins with high-dose steroids to reduce inflammation,
This document defines and describes different types of movement disorders including chorea, athetosis, and ballism. It discusses the etiology and characteristics of various forms of chorea including vascular chorea, autoimmune chorea, drug-induced chorea, metabolic chorea, and infectious chorea. The roles of structures like the globus pallidus and subthalamic nucleus in causing hyperkinetic movements are mentioned. Treatment options vary depending on the underlying cause but may include medications, surgery, or management of any precipitating conditions.
The document discusses various spinal cord syndromes classified as either complete or incomplete cord syndromes. It provides details on complete cord transection which results in paralysis, loss of sensation, and autonomic dysfunction below the level of injury. Brown-Sequard syndrome and central cord syndrome are discussed as examples of incomplete cord syndromes characterized by mixed upper and lower motor neuron findings on one or both sides of the body. The document also covers syndromes involving specific regions of the spinal cord including conus medullaris, cauda equina, and anterior spinal artery syndromes.
1) The document discusses various causes of compressive myelopathy including spondylosis, herniated discs, spinal stenosis, and tuberculosis. It describes the clinical features and treatments for different levels of involvement in the cervical and lumbar spine.
2) Imaging techniques like MRI, CT myelogram, and X-rays are used to identify compression of the spinal cord or nerve roots from conditions like herniations, osteophytes, and tuberculosis lesions.
3) Surgical intervention may be indicated for moderate to severe myelopathy, progressive neurological deficits, or failure of conservative treatment. The goal is to decompress the spinal cord and relieve compression.
Still's disease, sometimes referred to as Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by the classic triad of persistent high spiking fevers, joint pain and a distinctive salmon-colored bumpy rash.
Cerebellar dysfunction can cause ataxia, a lack of muscle coordination. The cerebellum controls fine movements and posture. Damage to the cerebellum disrupts these functions. Ataxia affects walking, limb movements, speech, and eye movements. It is diagnosed through clinical exams like finger-nose and Romberg tests. The causes include lesions, tumors, or injuries to the cerebellum. While incurable, treatment focuses on easing symptoms to improve quality of life.
This document discusses Duchenne muscular dystrophy (DMD), a genetic disorder characterized by progressive muscle degeneration and weakness. It is caused by mutations in the dystrophin gene leading to absence of the dystrophin protein. The document outlines the clinical presentation and stages of DMD from early childhood to late stages requiring wheelchair use. It also discusses diagnostic testing, management including cardiac and nutritional support, genetic counseling and potential future treatments like exon skipping drugs.
This document summarizes several types of chest wall deformities. It describes pectus excavatum, or funnel chest, which is characterized by a depression of the sternum. It affects about 1 in 400 births and is more common in boys. Pectus carinatum, or pigeon chest, involves a protrusion of the sternum. The document also discusses Poland's syndrome, cleft sternum, ectopia cordis (where the heart is located outside the chest), and thoracic deformities seen in diffuse skeletal disorders like Jeune's syndrome and Jarcho-Levin syndrome. It provides details on symptoms, causes, evaluation, and surgical repair options for each condition.
Guillain-Barré syndrome (GBS) can be described as a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes.
Although the classic description of GBS is that of a demyelinating neuropathy with ascending weakness, many clinical variants have been well documented in the medical literature.
This document discusses arachnoiditis, an inflammatory disease of the arachnoid membrane surrounding the spinal cord. It develops in three stages: initial nerve inflammation, scar tissue formation causing nerve adhesion, and complete nerve root encapsulation. Causes include trauma from surgery, chemical exposure, and infection. Symptoms include pain, paresthesia, sensory loss, and muscle weakness. Diagnosis involves MRI or CT myelography. Treatment includes corticosteroids, NSAIDs, rhizotomy for pain, and decompression surgery to remove cysts. Physical therapy techniques like exercises, heat, TENS, and neural mobilization can help manage symptoms.
This document describes Chiari malformation, specifically Chiari type I and II. It discusses the presentation, diagnosis, and treatment of a 34-year-old female patient presenting with progressive arm weakness, shoulder pain, and headaches exacerbated by coughing. Examination found reduced sensitivity in her arms. MRI confirmed Chiari type I malformation with tonsillar herniation. The patient underwent posterior fossa decompression surgery, with improvement in symptoms at 6-month follow up. Chiari malformations cause hindbrain herniation and obstruction of CSF flow, often requiring surgery to enlarge the posterior fossa.
Neurosyphilis is an infection of the nervous system caused by the bacterium Treponema pallidum, which causes syphilis. It typically develops after many years of untreated syphilis. Symptoms vary depending on the areas affected but may include mental deterioration, paralysis, meningitis, tabes dorsalis resulting in girdle pain and joint damage, and ocular symptoms. Treatment involves intravenous penicillin, but neurosyphilis can still cause permanent damage. Nursing care focuses on maintaining patient health, safety, and independence through measures like seizure precautions, skin care, physiotherapy, and partner screening.
This document defines peripheral vascular disease and describes the differences between peripheral arterial and venous disorders. It lists various risk factors for PVD like age, smoking, hypertension, and diabetes. Tests for assessing arterial insufficiency include ankle brachial index, treadmill testing, and skin perfusion pressure measurement. Tests for venous insufficiency include venous filling time, Homan's sign, and Trendelenburg test. Signs and symptoms of arterial disease include pale skin, absent pulses, and painful ulcers. Venous disease presents with warm skin, edema, and less painful ulcers. Grading scales assess severity of venous disease based on symptoms and ulcer characteristics.
Chiari malformations are a group of hindbrain abnormalities involving the rhombencephalon and cerebrospinal fluid junction. There are four main types of Chiari malformations described. Chiari type I involves tonsillar herniation below the foramen magnum without brainstem herniation. Chiari type II involves herniation of the brainstem, cerebellar vermis, and fourth ventricle and is associated with myelomeningocele. Chiari type III is an occipital encephalocele with similar anomalies to type II. Chiari type IV involves cerebellar hypoplasia without herniation. Clinical features of Chiari I can include headache, numbness, and
This document discusses various congenital abnormalities including neural tube defects. It begins by defining congenital abnormalities as defects present at birth or in early life. It then discusses specific defects like anencephaly, microcephaly, and megalencephaly in more detail, covering their causes, symptoms, diagnosis, and management. It also covers other central nervous system abnormalities like septal-optic dysplasia, diastematomyelia, polymicrogyria, encephalocele, hydrocephalus, and several types of spina bifida. The document provides an overview of major congenital abnormalities and their characteristics.
Unit 5 Child with Congenital Disorders.pptxRenitaRichard
Congenital anomalies refer to structural or functional abnormalities present at birth. This document discusses several common congenital anomalies including spina bifida, meningocele, hydrocephalus, cerebral palsy, and cleft lip and cleft palate. For each condition, the document defines it, discusses causes, signs and symptoms, diagnosis, treatment, and potential complications. Surgeries are often needed to repair defects, while other treatments may include shunts, braces, physical therapy, or speech therapy depending on the condition. Managing congenital anomalies requires a multidisciplinary care approach.
Tuberculous infection of the central nervous system (CNS) can occur via hematogenous spread or direct extension from a local infection. It most commonly manifests as tuberculous meningitis or tuberculomas. Tuberculous meningitis involves thick exudate in the subarachnoid space and can lead to hydrocephalus or ischemic infarcts. Tuberculomas appear as ring-enhancing lesions on imaging. Pott's disease is spinal tuberculosis that causes vertebral body collapse and kyphosis. Management involves antituberculous medications for at least 6-9 months.
A 42-year-old male was admitted to the emergency department after a motor vehicle accident with back pain and inability to move his lower limbs. Examination revealed absent sensation and paralysis below T12 with MRI showing a lesion at T11. He was diagnosed with complete T11 paraplegia and underwent surgery. Rehabilitation focused on preventing complications like pressure sores, infections, and maintaining mobility. Spinal cord injuries cause various impairments depending on level and severity, and patients require long-term management of physical, psychological, and social impacts on quality of life.
Stroke (cerebrovascular accident [CVA]) is the sudden loss of neurological function caused by an interruption of the blood flow to the brain.
Impairments may resolve spontaneously as brain swelling subsides (reversible ischemic neurological deficit), generally within 3 weeks.
Residual neurological impairments are those that persist
longer than 3 weeks and may lead to lasting disability
This document discusses spinal dysraphism, which refers to congenital anomalies resulting from failed fusion of the dorsal spinal elements. It describes different types including spina bifida occulta, meningocele, myelomeningocele, myelocystocele, and lipomeningocele. Meningocele involves a mass composed of CSF, meninges, and skin, but no neural elements. Myelomeningocele contains neural tissue and is usually associated with neurological deficits. Myelocystocele and lipomeningocele involve fatty tissue herniating through spinal defects. Radiological imaging plays an important role in diagnosis, with ultrasound, CT, MRI, and myelography discussed.
This document provides information about transverse myelitis (TM), including its definition, etiology, presentation, diagnosis, treatment, and prognosis. Some key points:
- TM is defined as acute spinal cord inflammation and injury that causes neurological deficits. It can be post-infectious, post-vaccination, or associated with MS.
- Presentation involves varying degrees of motor weakness, sensory alterations, and autonomic dysfunction below a certain spinal level. Up to half of idiopathic cases follow a respiratory or gastrointestinal illness.
- Diagnosis involves ruling out other causes through MRI, CSF analysis showing inflammation, and clinical features meeting criteria. Treatment begins with high-dose steroids to reduce inflammation,
This document defines and describes different types of movement disorders including chorea, athetosis, and ballism. It discusses the etiology and characteristics of various forms of chorea including vascular chorea, autoimmune chorea, drug-induced chorea, metabolic chorea, and infectious chorea. The roles of structures like the globus pallidus and subthalamic nucleus in causing hyperkinetic movements are mentioned. Treatment options vary depending on the underlying cause but may include medications, surgery, or management of any precipitating conditions.
The document discusses various spinal cord syndromes classified as either complete or incomplete cord syndromes. It provides details on complete cord transection which results in paralysis, loss of sensation, and autonomic dysfunction below the level of injury. Brown-Sequard syndrome and central cord syndrome are discussed as examples of incomplete cord syndromes characterized by mixed upper and lower motor neuron findings on one or both sides of the body. The document also covers syndromes involving specific regions of the spinal cord including conus medullaris, cauda equina, and anterior spinal artery syndromes.
1) The document discusses various causes of compressive myelopathy including spondylosis, herniated discs, spinal stenosis, and tuberculosis. It describes the clinical features and treatments for different levels of involvement in the cervical and lumbar spine.
2) Imaging techniques like MRI, CT myelogram, and X-rays are used to identify compression of the spinal cord or nerve roots from conditions like herniations, osteophytes, and tuberculosis lesions.
3) Surgical intervention may be indicated for moderate to severe myelopathy, progressive neurological deficits, or failure of conservative treatment. The goal is to decompress the spinal cord and relieve compression.
Still's disease, sometimes referred to as Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by the classic triad of persistent high spiking fevers, joint pain and a distinctive salmon-colored bumpy rash.
Cerebellar dysfunction can cause ataxia, a lack of muscle coordination. The cerebellum controls fine movements and posture. Damage to the cerebellum disrupts these functions. Ataxia affects walking, limb movements, speech, and eye movements. It is diagnosed through clinical exams like finger-nose and Romberg tests. The causes include lesions, tumors, or injuries to the cerebellum. While incurable, treatment focuses on easing symptoms to improve quality of life.
This document discusses Duchenne muscular dystrophy (DMD), a genetic disorder characterized by progressive muscle degeneration and weakness. It is caused by mutations in the dystrophin gene leading to absence of the dystrophin protein. The document outlines the clinical presentation and stages of DMD from early childhood to late stages requiring wheelchair use. It also discusses diagnostic testing, management including cardiac and nutritional support, genetic counseling and potential future treatments like exon skipping drugs.
This document summarizes several types of chest wall deformities. It describes pectus excavatum, or funnel chest, which is characterized by a depression of the sternum. It affects about 1 in 400 births and is more common in boys. Pectus carinatum, or pigeon chest, involves a protrusion of the sternum. The document also discusses Poland's syndrome, cleft sternum, ectopia cordis (where the heart is located outside the chest), and thoracic deformities seen in diffuse skeletal disorders like Jeune's syndrome and Jarcho-Levin syndrome. It provides details on symptoms, causes, evaluation, and surgical repair options for each condition.
Guillain-Barré syndrome (GBS) can be described as a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes.
Although the classic description of GBS is that of a demyelinating neuropathy with ascending weakness, many clinical variants have been well documented in the medical literature.
This document discusses arachnoiditis, an inflammatory disease of the arachnoid membrane surrounding the spinal cord. It develops in three stages: initial nerve inflammation, scar tissue formation causing nerve adhesion, and complete nerve root encapsulation. Causes include trauma from surgery, chemical exposure, and infection. Symptoms include pain, paresthesia, sensory loss, and muscle weakness. Diagnosis involves MRI or CT myelography. Treatment includes corticosteroids, NSAIDs, rhizotomy for pain, and decompression surgery to remove cysts. Physical therapy techniques like exercises, heat, TENS, and neural mobilization can help manage symptoms.
This document describes Chiari malformation, specifically Chiari type I and II. It discusses the presentation, diagnosis, and treatment of a 34-year-old female patient presenting with progressive arm weakness, shoulder pain, and headaches exacerbated by coughing. Examination found reduced sensitivity in her arms. MRI confirmed Chiari type I malformation with tonsillar herniation. The patient underwent posterior fossa decompression surgery, with improvement in symptoms at 6-month follow up. Chiari malformations cause hindbrain herniation and obstruction of CSF flow, often requiring surgery to enlarge the posterior fossa.
Neurosyphilis is an infection of the nervous system caused by the bacterium Treponema pallidum, which causes syphilis. It typically develops after many years of untreated syphilis. Symptoms vary depending on the areas affected but may include mental deterioration, paralysis, meningitis, tabes dorsalis resulting in girdle pain and joint damage, and ocular symptoms. Treatment involves intravenous penicillin, but neurosyphilis can still cause permanent damage. Nursing care focuses on maintaining patient health, safety, and independence through measures like seizure precautions, skin care, physiotherapy, and partner screening.
This document defines peripheral vascular disease and describes the differences between peripheral arterial and venous disorders. It lists various risk factors for PVD like age, smoking, hypertension, and diabetes. Tests for assessing arterial insufficiency include ankle brachial index, treadmill testing, and skin perfusion pressure measurement. Tests for venous insufficiency include venous filling time, Homan's sign, and Trendelenburg test. Signs and symptoms of arterial disease include pale skin, absent pulses, and painful ulcers. Venous disease presents with warm skin, edema, and less painful ulcers. Grading scales assess severity of venous disease based on symptoms and ulcer characteristics.
Chiari malformations are a group of hindbrain abnormalities involving the rhombencephalon and cerebrospinal fluid junction. There are four main types of Chiari malformations described. Chiari type I involves tonsillar herniation below the foramen magnum without brainstem herniation. Chiari type II involves herniation of the brainstem, cerebellar vermis, and fourth ventricle and is associated with myelomeningocele. Chiari type III is an occipital encephalocele with similar anomalies to type II. Chiari type IV involves cerebellar hypoplasia without herniation. Clinical features of Chiari I can include headache, numbness, and
This document discusses various congenital abnormalities including neural tube defects. It begins by defining congenital abnormalities as defects present at birth or in early life. It then discusses specific defects like anencephaly, microcephaly, and megalencephaly in more detail, covering their causes, symptoms, diagnosis, and management. It also covers other central nervous system abnormalities like septal-optic dysplasia, diastematomyelia, polymicrogyria, encephalocele, hydrocephalus, and several types of spina bifida. The document provides an overview of major congenital abnormalities and their characteristics.
Unit 5 Child with Congenital Disorders.pptxRenitaRichard
Congenital anomalies refer to structural or functional abnormalities present at birth. This document discusses several common congenital anomalies including spina bifida, meningocele, hydrocephalus, cerebral palsy, and cleft lip and cleft palate. For each condition, the document defines it, discusses causes, signs and symptoms, diagnosis, treatment, and potential complications. Surgeries are often needed to repair defects, while other treatments may include shunts, braces, physical therapy, or speech therapy depending on the condition. Managing congenital anomalies requires a multidisciplinary care approach.
(1) Spina bifida is a birth defect where part of the spinal cord and its meninges are exposed through an opening in the backbone. (2) It results from the neural tube failing to close properly during early pregnancy. (3) Spina bifida can range from mild to severe depending on factors like the size and location of the defect and presence of complications such as hydrocephalus or Chiari malformation.
This document provides information about cerebral palsy, including:
(1) It is a motor function disorder caused by permanent brain damage present at birth or shortly after.
(2) The most common types are spastic cerebral palsy (stiff muscles) and athetoid cerebral palsy (uncontrolled movements).
(3) Treatment aims to improve symptoms through physical therapy, bracing, medication, botulinum toxin injections, and sometimes surgery. The goal is improving quality of life and function rather than curing the underlying brain damage.
Hydrocephalus is a condition where cerebrospinal fluid builds up in the brain, increasing pressure inside the skull. It can be congenital, acquired after birth from injuries or infections, or develop later in life. Symptoms depend on age but include headaches, nausea, vision issues, and cognitive or mobility problems. Treatment involves surgically inserting a shunt to drain fluid from the brain ventricles to other body areas. Complications can arise from shunt malfunctions or progression of the condition if left untreated.
Spina bifida is a birth disorder that involves the incomplete development of the spine. In the first month of pregnancy, a special set of cells forms the “neural tube;” the top of the tube becomes the brain, and the remainder becomes the spinal cord and structures around it. In spina bifida, the neural tube doesn’t close completely and some of the bones of the spine do not close in the back. This can result in an opening anywhere along the spine and may cause damage to the spinal cord and nerves.There are four types of spina bifida: occulta, closed neural tube defects, meningocele, and myelomeningocele. The symptoms of spina bifida vary from person to person, depending on the type and level of involvement. Most cases are mild and do not require special treatment. The more serious cases involve nerve damage.
Occulta is the mildest and most common form in which one or more bones of the spinal column (vertebrae) are malformed. The name “occulta,” which means “hidden,” indicates that a layer of skin covers the opening in the bones of the spine. It usually shows no symptoms and is often found by accident on an x-ray or similar test.
Closed neural tube defects are a diverse group of disorders in which the spine may have malformations of fat, bone, or the membranes (the meninges) that cover the spinal cord. Many of these neural tube defects require surgery in childhood. People with this type of spina bifida may have weakness of the legs and trouble with bowel and bladder control. These issues may change or progress as children grow. It is important to have close communication with doctors to minimize these changes as much as possible.
Meningocele occurs when the meninges protrude through the spine and cause a sac of spinal fluid on the back. This fluid is typically only around the brain and spine, but a problem with the bony covering over the spine allows it to poke out. The malformation contains no nerves and may or may not be covered by a layer of skin. Individuals with meningocele may have minor symptoms.Myelomeningocele is the most severe form of spina bifida. A portion of the spinal cord or nerves are exposed in a sac through an opening in the spine that may or may not be covered by the meninges. The opening can be closed surgically while the baby is in utero or shortly after the baby is born. Most people with myelomeningocele experience changes in brain structure, leg weakness, and bladder and bowel dysfunction.
Myelomeningocele is often called a "snowflake condition" because no two people with the condition are the same. Typically, if the opening in the spine is lower down the back, the person will experience less symptoms. People with myelomeningocele require close follow-up with physicians throughout their childhood and lifespan to maximize their function and prevent complications like kidney failure.Complications of spina bifida may include:
Abnormal sensation or paralysis, which mostly occurs with closed neural tube defects and myelomenin
Cerebral palsy (CP) is a group of disorders that affect movement and posture due to damage to the developing brain. It was first identified by William Little in the 19th century. While difficult births were originally blamed, Sigmund Freud suggested other prenatal factors were involved. CP can be caused by problems before, during, or after birth. The majority of cases are congenital. Risk factors include preterm birth, low birthweight, infections during pregnancy, and brain injuries. Treatment focuses on improving mobility, function, and development through physical, occupational, and speech therapies. While there is no cure, early intervention can greatly improve quality of life.
ARNOLD CHIARI MALFORMATION-STRUCTURAL DEFECTS IN BRAIN AND SPINAL CORDManjumam2
Arnold-Chiari malformation is a structural defect where the cerebellum, which controls balance, protrudes through the opening at the base of the skull. There are four types with varying severity, from type I where only the cerebellar tonsils extend through, to type IV with an underdeveloped cerebellum. Common symptoms include headaches, neck pain, dizziness, weakness, and difficulty swallowing. Diagnosis involves medical history, neurological exam, and an MRI to identify the extent of herniation. Treatment depends on the type but may include surgery to repair other issues, shunt placement, decompression, and physiotherapy to help manage symptoms.
Neural tube defects are birth defects that result from the failure of the neural tube to close properly during early embryonic development. The most common types are spina bifida, anencephaly, and encephalocele. Prevention focuses on women getting adequate folic acid before and during early pregnancy. Treatment depends on the specific type of defect but may involve surgery to repair the opening and protect the exposed tissues, management of any related conditions like hydrocephalus, and lifelong care for resulting physical, neurological, and functional impairments. Prognosis depends on the severity of the defect and can include permanent disability.
Hydrocephalus is an abnormal buildup of cerebrospinal fluid in the brain that increases pressure inside the skull. It can be congenital, developing before birth due to infections, genetic factors, or issues with brain development. It can also be acquired after birth due to head injuries, tumors, or infections. The excess fluid causes brain tissue to be compressed and brain damage over time. Spina bifida is a birth defect where the spinal column does not fully close, leaving part of the spinal cord and meninges exposed. Both conditions require lifelong management, often involving surgical insertion of shunts to drain fluid and prevent further brain damage.
Hydrocephalus is the buildup of fluid in the cavities (ventricles) deep within the brain. The excess fluid increases the size of the ventricles and puts pressure on the brain.
Microcephaly is a medical condition where the head circumference is more than two standard deviations smaller than average for age and sex because the brain has not developed or grown properly. It can be present at birth or develop in the first few years of life. The severity of associated mental retardation is related to the severity of microcephaly, with a 33% risk of developing mental retardation for head circumferences 2-3 SD below average and 62% for over 3 SD below average. Microcephaly can be classified as primary/genetic or secondary/environmental based on its cause. The prognosis depends on any accompanying conditions, with microcephaly generally resulting in reduced life expectancy and poor chances of normal brain development.
Spina bifida is a birth defect where the spinal cord fails to develop properly, leaving an opening in the vertebrae. It occurs when the neural tube fails to close fully during early embryonic development. There are three main types: spina bifida occulta, meningocele, and myelomeningocele. Myelomeningocele is the most severe form and often results in paralysis and loss of sensation below the level of the defect. Risk factors include family history, obesity, fever during early pregnancy, and folic acid deficiency. Treatment involves surgery to close the opening and address any related issues like hydrocephalus. Lifelong management focuses on rehabilitation, preventing infections and complications, and addressing issues with mobility,
73807 usx-2104 bowel conditions in children webinarIdespiran
This document discusses various conditions in children that can lead to bowel dysfunction, including spinal cord injuries, sacral agenesis, necrotizing enterocolitis (NEC), Ehlers-Danlos syndromes (EDS), and spina bifida. It provides details on the symptoms and impacts of each condition, such as neurogenic bowel from spinal cord injuries depending on the level of injury. It also discusses challenges children face in managing bowel issues, like negative quality of life impacts and potential lack of treatment due to lack of knowledge.
This document discusses several pediatric neurological disorders including hydrocephalus, spina bifida, and bacterial meningitis. Hydrocephalus is an abnormal buildup of cerebrospinal fluid in the brain ventricles which can cause increased intracranial pressure. It is usually treated with surgical insertion of a shunt system to drain fluid from the brain. Spina bifida is a birth defect involving incomplete closing of the spinal column which can range from mild to severe. Bacterial meningitis is a medical emergency caused by bacterial infection of the meninges and cerebrospinal fluid, with symptoms including fever, irritability, and neck stiffness.
The document discusses several congenital malformations including anencephaly, microcephaly, megalencephaly, septo-optic dysplasia, diastomatomyelia, polymicrogyria, encephalocele, and spina bifida. It provides details on causes, clinical presentations, diagnostic evaluations, and management for each condition. Anencephaly is a neural tube defect where the brain and skull are absent. Microcephaly is a small head size due to abnormal brain development. Megalencephaly is an abnormally large brain. Septo-optic dysplasia involves optic nerve and pituitary abnormalities. Spina bifida is an incomplete closing of the spine that can range from mild
Spina bifida is a birth defect where the backbone and spinal canal do not close before birth, causing part of the spinal cord to protrude out of the back. It affects around 1 in 800 infants and can range from a mild condition where the spine does not fully close to more severe cases where parts of the spinal cord and membranes protrude out. Symptoms include issues with bladder/bowel control, paralysis, and hydrocephalus in many cases. Prenatal screening and folic acid supplementation can help reduce risks, while surgery after birth and lifelong treatment can address symptoms and complications.
-what is hydrocephalus
-introduction of hydrocephalus
-embryonic development
-normal CSF circulation
-flow of CSF
-congenital hydrocephalus
-acquired hydrocephalus
-types of hydrocephalus
-communicating hydrocephalus
-non communicating hydrocephalus
-symptoms
-Crack pot sign
-Macewen Sunset sign
-testing and diagnosis of hydrocephalus
-medical management of hydrocephalus
-surgical management of hydrocephalus
-physiotherapy management of hydrocephalus
-recent advance in hydrocephalus
-hydrocephalus
physiotherapy
Microcephaly is a medical condition where the head circumference is more than two standard deviations smaller than average for age and sex because the brain has not developed properly or has stopped growing. It can be present at birth or develop in the first few years of life. Microcephaly can be classified as primary (genetic) or secondary (environmental/acquired). The prognosis depends on the severity and any associated conditions, with more severe microcephaly associated with higher risks of intellectual disabilities and developmental delays.
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3. Introduction
Arnold Chiari Malformation, also known as Chiari type II malformation, is one of
a group of brain malformations affecting the cerebellum. Named after Hans
Chiari and Julius Arnold, the pathologists who first described the group of
malformations. This condition is almost always associated with
myelomeningocele, the most serious form of spina bifida
4. Clinically Relevant Anatomy
Normally the cerebellum and parts of the brain stem sit in an indented space at
the lower rear of the skull, above the foramen magnum (a funnel-like opening
to the canal). When part of the cerebellum is located below the foramen
magnum, it is called a Chiari malformation. In Arnold Chiari Malformation, both
cerebellar and brain stem tissue protrude into the foramen magnum and the
cerebellar vermis (the nerve tissue that connects the two halves of
the cerebellum) may be only partially complete or absent
5. Clinically Relevant Anatomy
This condition has skull, dural, brain, spinal, and spinal cord manifestations,
including the downward displacement of the medulla, fourth ventricle, and
cerebellum into the cervical spinal canal, as well as elongation of the pons and
fourth ventricle, probably due to a relatively small posterior fossa
The brainstem is elongated and displaced into the opening of the base of the
skull and into the top of the spinal canal. It is often kinked. The brainstem,
cranial nerves and lower portion of the cerebellum may be stretched or
compressed. This means that any of the functions controlled by these areas
may be affected
6. Clinical Presentation
Many people with Arnold Chiari malformation have no obvious symptoms.
Compressive mechanisms are thought to cause symptoms, but it is likely that
stretching of abnormally placed cranial nerves may provoke symptoms. In
babies, the most common symptoms are a weak or absent cry, breathing
difficulties, including stridor, arching of the neck, failure to thrive and feeding or
swallowing difficulties. a weak cry, gagging or vomiting, arm weakness, a stiff
neck, developmental delays, and an inability to gain weight
Blockage of cerebrospinal fluid (CSF) flow may cause the formation of a syrinx,
which can lead to syringomyelia. Central cord symptoms such as hand
weakness, sensory loss, and, in severe cases, paralysis may occur.
7. Clinical Presentation
Common symptoms people with Chiari Type II malformations might experience
are:
• headaches (usually at the back of the head and often made worse by coughing, sneezing
or straining)
• neck pain
• dizziness and balance problems
• hearing loss and/or tinnitus
• muscle weakness and paralysis
• visual problems and nystagmus
• swallowing difficulties
• dysesthesia in the limbs
• problems with hand coordination and fine motor skills.
8. Diagnostic Procedures
The key tests for diagnosis of Arnold Chiari Malformation are MRI and CT
scans. An MRI will show the abnormal CSF flow and configuration and position
of the brain and spinal cord. Antenatal ultrasound imaging shows an
indentation of the frontal bone with an abnormally shaped cerebellum
9. Differential Diagnosis
• The main differential diagnosis is the type of Chiari malformation
• Chiari I malformation does not have a myelomeningocele.
• Chiari III has an occipital and/or high cervical encephalocele.
• Chiari IV has severe cerebellar hypoplasia without displacement of the
cerebellum through the foramen magnum.
10. Epidemiology
It used to be said that Chiari malformations are rare, but with routine use of MR
imaging, Chiari malformation is discovered with increasing frequency. Chiari II
is found in all children with myelomeningocele, although less than one-third
develop symptoms referable to this malformation
11. Physiotherapy Management
Physiotherapy can help with many of the symptoms caused by Arnold Chiari
Malformation:
• Vestibular Rehabilitation is indicated when patients have vestibular problems
• Conservative physiotherapy soft tissue techniques may be of benefit for
cervical pain
• Soft tissue techniques may be of benefit for headaches
12. Surgical Interventions
Treatment of patients with Arnold Chiari malformation is complex due to the
variety and variable severity of malformations, but some patients may require
any of the following surgical interventions : myelomeningocele repair and
management of neurogenic bladder
• ventricular shunting - hydrocephalus usually requires shunting and can
improve the cranial nerve and brainstem function
• craniovertebral decompression may be required in neonates with brainstem
dysfunction if hydrocephalus is not present, or if symptoms and signs do not
improve with shunting. Some older patients with hindbrain herniation or
syringohydromyelia may also benefit from ventricular shunts
13. Prognosis
Many with Type I CM do not show symptoms and do not know they have the
condition. Symptoms vary among individuals, Individuals who have severe
Chiari malformation and have undergone surgery see a reduction in their
symptoms, Even though paralysis may be permanent.
16. What is Dandy-Walker Syndrome?
• Dandy-Walker Syndrome (DWS) is a congenital (happening before birth)
condition where the cerebellum does not develop normally. The cerebellum is
an area at the back of the brain that controls movement and balance.
• With DWS, parts of the cerebellum may never develop or may be very small.
Other parts of the cerebellum become filled with fluid or develop small
masses of fluid (cysts). In most cases, a build-up of fluid inside the skull
causes the head to get bigger than it should.
17. What is Dandy-Walker Syndrome?
• DWS can occur by itself, or along with other types of birth defects. Children
with DWS may also have heart problems, face or limb defects or problems
with vison and hearing. In rare cases, DWS may not be diagnosed until late
childhood or adulthood. DWS can cause delays in both motor and mental
development. The severity of symptoms and long-term outcomes are
different for each child. Some children need a lot of medical care at birth but
go on to live normal lives with few or no long-term effects. Other children may
lose the ability to move parts of their body (paralysis), seizures and problems
with thinking and learning.
• The long-term survival rate for infants with isolated DWS is very good. If
DWS appears with other genetic conditions, outcomes can vary. Early
treatment to support physical and mental development improves a child’s
chances of having the best quality of life possible for their condition
18. What Causes Dandy-Walker Syndrome?
• No one is sure what causes DWS. Scientists do know that certain genetic conditions or other
outside factors may increase the risk of a baby having the disease.
• DWS begins during the first month of pregnancy. As the baby’s cerebellum starts to develop
abnormally, fluid begins to build up. During the pregnancy, malformations and fluid in the
cerebellum continue to grow. The fluid build-up and problems in the brain structure can cause
many neurological and physical symptoms seen at birth and later in childhood.
• No one knows what causes DWS. There are likely many factors that could be a part of its
development. A number of different genetic syndromes are associated with DWS. Certain medical
problems in the mother, such as diabetes, have been associated with the development of DWS.
There is some research that says a baby’s risk for developing DWS increases if the mother is
exposed to certain types of infections or medicines while she is pregnant
19.
20.
21. What are the Signs and Symptoms of Dandy-Walker
Syndrome?
• Too much fluid in the brain
(called hydrocephalus)
• Signs of increased pressure from fluid build-
up in the brain, including irritability
and vomiting (usually seen in older children)
• Large head (called macrocephaly)
• Delayed crawling and walking
• Balance problems
• Stiff muscles
• Trouble with fine motor skills (picking up small
objects, writing)
• Vision problems
• Seizures
• Abnormal breathing
• Jerky eye or neck movements
• Delay in mental development
• Agenesis of the corpus callosum
22. What are the Types of Dandy-Walker Syndrome?
• Malformation. This is the most severe type of DWS. It is usually seen as part of a group with
congenital birth defects. With Dandy-Walker Malformation, some parts of the cerebellum are not
there. Others are enlarged with fluid-filled cysts. Most babies have hydrocephalus and
macrocephaly and need surgery to place a shunt (a hollow tube) into the brain. This will help the
remove extra fluid. These babies are at great risk for having long-term neurological and
developmental problems.
• Variant. This is the mildest form of DWS. It has a wide range of symptoms and outcomes. Babies
with the Dandy-Walker Variant may have less fluid build-up inside their brain. As a result, they will
have less damage to structures in the cerebellum. Babies with this type of DWS are less likely to
need surgery to remove extra fluid from their brains. However, many may still need surgery. Some
children with this type of DWS will go on to have normal intelligence and physical development.
Others may need many surgeries to remove fluid inside the skull. They will also need life-long
physical therapy.
23. How is Dandy-Walker Syndrome Diagnosed?
A variety of prenatal (before birth) tests can help your doctor diagnose DWS:
• Ultrasound: This type of imaging test that can show problems with how your baby’s brain is
forming
• Fetal MRI (magnetic resonance imaging): Non-invasive imaging test to get a clear, more
detailed image of your baby’s organs.
24. Other Tests
• Some congenital conditions are caused by changes in DNA, or a baby’s genetic makeup. The doctors may
suggest genetic testing to find out more about your baby’s DNA either during pregnancy or after birth.
Recommended tests may include:
• Cell-Free fetal DNA testing: A screening test where a sample of your blood is taken to look for copies of fetal
(baby) DNA. A positive result means that there could be problems with the baby’s DNA. It should be confirmed
with another test. A normal result does not mean that there are no underlying genetic problems. Other testing
can be done before or after birth depending on your needs and the needs of the baby.
• Amniocentesis: A medical procedure in which a small amount of amniotic fluid is taken and studied to screen
for genetic problems. This test is usually done in the middle of the second trimester. An amniocentesis can be
used to confirm findings on the cell-free fetal DNA test.
• Testing the baby: After birth, a sample of your baby’s blood is taken to look for chromosome problems. This is
called either a “karyotype” (basic chromosome study), or microarray (a more detailed look at smaller sections
of DNA).
25.
26. Care During Your Pregnancy
Your doctors will watch your pregnancy closely. If the malformation on your baby’s head is very
large, you may need to have a C-section. You will deliver your baby at a hospital that is prepared
to care for high-risk babies. You should discuss your plans for labor and delivery with your
obstetrician. Contact your doctor right away if you have any concerns
27. Treatment
There is no cure for DW and treatment usually involves treating the associated
symptoms. Often, a shunt may be placed when hydrocephalus is diagnosed. A
shunt will help drain excess fluid in the brain to reduce swelling and intracranial
pressure. A compilation of multiple affects may shorten life span. Medications are
often prescribed to control seizures.
28. How is Dandy-Walker Syndrome Treated?
• Although Dandy-Walker syndrome can interfere with everyday life, milder cases don’t always require
treatment—it depends on the severity of the developmental and coordination issues the person is
experiencing.
• Some common treatments for Dandy-Walker syndrome include:
• The surgical implantation of a shunt in the skull: If your child experiences increased intracranial pressure,
doctors may recommend inserting a shunt (a small tube) in the skull to help alleviate pressure. This tube will
drain fluid from the skull and carry it to other areas of the body, where it can be safely absorbed.
• Various therapies: Special education, occupational therapy, speech therapy, and physical therapy can help
your child manage the issues linked to Dandy-Walker syndrome. Talk to your pediatrician about the therapies
that will be most effective for your child.
• Survival and prognosis depend on the severity of the malformation and the presence of other congenital
defects.