Well descriptive power point presentation for fresh neurosurgery residents across the world with very basic knowledge of Chiari malformations and its types and principles of the management and management of its associations.
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 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.
This document discusses the anatomy, imaging, classification, and treatment of various cervical vertebral anomalies. It begins with an overview of the normal anatomy of the atlantoaxial joint and landmarks seen on imaging. It then discusses various congenital and acquired bony and soft tissue anomalies that can occur in this region, including platybasia, basilar invagination, occipitalization of the atlas, and atlantoaxial dislocation. Imaging criteria and classifications for these conditions are provided. Common associated findings like Chiari malformation and syringomyelia are also mentioned. The document concludes with sections on clinical presentation and evaluation of these cervical vertebral anomalies.
1. The cardinal signs of cerebellar dysfunction are ataxia, tremor, hypotonia, and asthenia. Tests include finger-nose coordination, rapid alternating hand movements, and gait.
2. Cerebellar lesions can cause dysarthria, scanning speech, nystagmus, and impaired eye movements like dysmetric saccades.
3. Clinical exams test arm and leg coordination through tasks like finger tapping, heel-shin rubbing, and tandem walking. Hypotonia is shown through floppy posture and pendular reflexes. Strength and endurance may also be reduced.
Wallenberg syndrome, also known as lateral medullary infarction, is caused by occlusion of the posterior inferior cerebellar artery, which supplies blood to the lateral medulla. This leads to vertigo, abnormal eye movements, Horner's syndrome on one side, ataxia of the limb on the same side, and dissociated sensory loss. The condition is usually due to atherosclerosis but can also result from traumatic vertebral artery dissection. MRI and MRA are used to diagnose the infraction and rule out arterial dissection.
The Dandy-Walker complex is a rare congenital brain malformation characterized by cystic dilation of the 4th ventricle and partial or complete absence of the cerebellar vermis. It ranges from mega cisterna magna to Dandy-Walker variant to Dandy-Walker malformation based on severity. Associated anomalies are common. The cause is unknown but likely involves abnormal development of the hindbrain. Treatment involves managing hydrocephalus, often with shunt procedures. Prognosis depends on severity and associated anomalies, with higher mortality and developmental delays in more severe forms.
This document discusses the anatomy, landmarks, measurements, common anomalies, syndromes, and injuries of the craniovertebral junction. It begins with a brief description of the craniovertebral junction's development and components. It then outlines several key anatomical landmarks and measurements used to evaluate the region on imaging. The remainder of the document details various congenital anomalies, developmental abnormalities, syndromes, and acquired conditions that can affect the craniovertebral junction.
The craniovertebral junction (CVJ) refers to the occiput, atlas, axis, and supporting ligaments. It is a transition zone between the mobile cranium and spinal column. The CVJ encloses important neural and vascular structures.
Anatomically, the CVJ includes bony structures like the occiput, atlas, and axis along with their articulations and connecting ligaments. It also has muscles, neural elements like the medulla and lower cranial nerves, lymphatics, arteries and veins. Congenital anomalies of the CVJ can occur due to malformations during embryological development.
Radiological evaluation of the CVJ involves measurements and angles on X
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 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.
This document discusses the anatomy, imaging, classification, and treatment of various cervical vertebral anomalies. It begins with an overview of the normal anatomy of the atlantoaxial joint and landmarks seen on imaging. It then discusses various congenital and acquired bony and soft tissue anomalies that can occur in this region, including platybasia, basilar invagination, occipitalization of the atlas, and atlantoaxial dislocation. Imaging criteria and classifications for these conditions are provided. Common associated findings like Chiari malformation and syringomyelia are also mentioned. The document concludes with sections on clinical presentation and evaluation of these cervical vertebral anomalies.
1. The cardinal signs of cerebellar dysfunction are ataxia, tremor, hypotonia, and asthenia. Tests include finger-nose coordination, rapid alternating hand movements, and gait.
2. Cerebellar lesions can cause dysarthria, scanning speech, nystagmus, and impaired eye movements like dysmetric saccades.
3. Clinical exams test arm and leg coordination through tasks like finger tapping, heel-shin rubbing, and tandem walking. Hypotonia is shown through floppy posture and pendular reflexes. Strength and endurance may also be reduced.
Wallenberg syndrome, also known as lateral medullary infarction, is caused by occlusion of the posterior inferior cerebellar artery, which supplies blood to the lateral medulla. This leads to vertigo, abnormal eye movements, Horner's syndrome on one side, ataxia of the limb on the same side, and dissociated sensory loss. The condition is usually due to atherosclerosis but can also result from traumatic vertebral artery dissection. MRI and MRA are used to diagnose the infraction and rule out arterial dissection.
The Dandy-Walker complex is a rare congenital brain malformation characterized by cystic dilation of the 4th ventricle and partial or complete absence of the cerebellar vermis. It ranges from mega cisterna magna to Dandy-Walker variant to Dandy-Walker malformation based on severity. Associated anomalies are common. The cause is unknown but likely involves abnormal development of the hindbrain. Treatment involves managing hydrocephalus, often with shunt procedures. Prognosis depends on severity and associated anomalies, with higher mortality and developmental delays in more severe forms.
This document discusses the anatomy, landmarks, measurements, common anomalies, syndromes, and injuries of the craniovertebral junction. It begins with a brief description of the craniovertebral junction's development and components. It then outlines several key anatomical landmarks and measurements used to evaluate the region on imaging. The remainder of the document details various congenital anomalies, developmental abnormalities, syndromes, and acquired conditions that can affect the craniovertebral junction.
The craniovertebral junction (CVJ) refers to the occiput, atlas, axis, and supporting ligaments. It is a transition zone between the mobile cranium and spinal column. The CVJ encloses important neural and vascular structures.
Anatomically, the CVJ includes bony structures like the occiput, atlas, and axis along with their articulations and connecting ligaments. It also has muscles, neural elements like the medulla and lower cranial nerves, lymphatics, arteries and veins. Congenital anomalies of the CVJ can occur due to malformations during embryological development.
Radiological evaluation of the CVJ involves measurements and angles on X
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.
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.
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.
Dandy-Walker Malformation: Classification and ManagementDr. Shahnawaz Alam
Dandy-Walker malformation is a congenital brain abnormality where the cerebellum and fourth ventricle are abnormally developed. It ranges in severity from mild vermian hypoplasia to more severe presentations with cyst formation. Treatment involves managing hydrocephalus with ventriculoperitoneal shunting, though some mild cases require no treatment. Complications can include herniation of brain structures if only certain areas are shunted, so simultaneous dual shunting of the lateral ventricles and fourth ventricle cyst may be preferable. Long term outcomes depend on severity but can include normal intelligence if the malformation is mild without other brain anomalies.
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.
This document discusses sensory ataxia, including its causes, clinical presentation, and differential diagnosis. Sensory ataxia results from disorders of the cerebellum, vestibular system, or sensory pathways. It is characterized by incoordination without significant weakness. Key findings include impaired proprioception, positive Romberg's sign, pseudoathetosis, and loss of vibration or joint position sense. Causes include peripheral neuropathies, paraneoplastic disorders, infections like HIV, autoimmune conditions like Sjogren's syndrome, and medications like chemotherapy. Dorsal root ganglionopathies commonly underlie sensory ataxia and can be assessed through nerve conduction studies and lumbar puncture. Distinguishing sensory
This document provides information on myelopathy and spinal cord lesions:
- Myelopathy describes any neurological deficit related to the spinal cord, and can cause quadriplegia, paraplegia, sensory deficits, and autonomic disturbances. It can be inflammatory (myelitis) or vascular in nature (vascular myelopathy).
- Cervical spondylotic myelopathy is the most common form of myelopathy. Lesions of the spinal cord can be compressive (intramedullary or extramedullary) or non-compressive.
- Different levels of spinal cord lesions present with distinct symptoms, such as sensory losses in specific dermatomes or weaknesses of particular muscles. Features help
1. A 32-year-old woman presented with progressive weakness of all four limbs over two months. Examination showed moderate weakness equally in all limbs without wasting, normal tone and reflexes, and no sensory issues, consistent with ALS.
2. An 18-year-old presented with sudden onset weakness of all four limbs for three days. Examination found weakness, diminished tone and reflexes, and vibration sense loss in the toes, consistent with transverse myelitis.
3. A 15-year-old girl reported right abdominal pain and right leg weakness for six months without bladder issues. Exam found spastic right leg weakness, absent lower abdominal reflex, and right lower sensation loss, consistent with a right
A detailed description of benign paroxysmal positional vertigo (BPPV): the symptoms, causes, diagnosis, and treatment methods.For more information, please visit www.everydayhearing.com
1. Spinal shock is a temporary loss of spinal reflex activity below the level of spinal cord injury that occurs immediately after severe spinal cord injury.
2. It is demonstrated by a loss of muscle tone, reflexes, and sensation below the level of injury and can last from hours to weeks depending on the severity and level of injury.
3. Spinal shock results from the loss of descending facilitation from the brain to the spinal cord below the level of injury and goes through phases of areflexia, initial reflex return, hyperreflexia, and eventually spasticity as the spinal cord recovers over time.
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.
Dr Ahmad Taha - Fetal Medicine Unit, Orient Hospital. Presented in Syrian Society of Obstetricians and Gynaecologists Annual Meeting 2015 - Dama Rose Hotel.
This document discusses dysphagia (difficulty swallowing) in pseudobulbar palsy. It begins by defining bulbar palsy and pseudobulbar palsy, noting that bulbar palsy involves lower motor neuron lesions affecting bulbar muscles, while pseudobulbar palsy involves upper motor neuron lesions. It then describes the anatomy and physiology of swallowing, including the four phases. It discusses the causes, signs, and treatments of dysphagia. Key assessment tools mentioned include a video swallow study and 3-ounce water swallow test. The document provides an overview of dysphagia for health professionals.
The craniovertebral junction refers to the occiput, atlas, and axis vertebrae and their articulations and ligaments. It is a complex anatomical region forming the transition between the skull and cervical spine. Common craniovertebral junction anomalies include occipitalization of the atlas, basilar invagination, atlantoaxial dislocation, and dens dysplasia. These anomalies can be developmental, post-traumatic, or acquired. Imaging studies including X-rays, CT, MRI are used to classify and evaluate the anomalies. Treatment may involve surgery such as fusion if the anomaly causes spinal cord compression.
This document summarizes neurological manifestations of human immunodeficiency virus (HIV). It discusses how HIV can affect both the central nervous system and peripheral nervous system. Some key points:
- Up to 50% of HIV patients experience clinically apparent neurological disease. Common manifestations involve the brain, meninges, spinal cord, peripheral nerves, and muscles.
- Direct effects of HIV include infection of cells like macrophages, monocytes, microglia, and possibly astrocytes. Indirect effects include opportunistic infections and neoplasms.
- Common CNS manifestations include HIV encephalopathy/dementia, space-occupying lesions, encephalitis, and stroke-like syndromes. Common peripheral
Intramedullary spinal cord lesions occur within the spinal cord itself while extramedullary lesions occur outside the spinal cord. Intramedullary lesions typically cause symmetrical symptoms that affect motor and sensory functions diffusely down the spinal cord, often with early sphincter involvement. In contrast, extramedullary lesions usually cause asymmetrical symptoms, with local or radicular pain and early upper motor neuron signs but later lower motor neuron signs and sensory involvement in the affected segment.
This document discusses various syndromes that can result from strokes in different areas of the brainstem. It begins with an overview of brainstem anatomy and blood supply. It then describes in detail the clinical presentations of medial and lateral midbrain syndromes, various pontine syndromes including medial and lateral inferior pontine syndromes, and medial and lateral medullary syndromes. Case examples are provided to illustrate the different neurological deficits that can occur based on the location of the brainstem stroke.
Damage to spinal cord structures can cause various impairments depending on the location and extent of the lesion. A complete transection of the spinal cord at L1 would cause ipsilateral loss of light touch and proprioception below the lesion, upper motor neuron signs ipsilaterally below, and contralateral loss of pain and temperature sensation. Epiconus syndrome from a lesion at L4-S2 is associated with weakness of hip and knee flexion and ankle movement with sensory loss from L4-S5 and loss of bladder and bowel control. Cutaneous reflexes and other reflexes have localizing value in myelopathy patients.
Human: Thank you for the summary. Can you provide a 3 sentence summary that focuses
Chiari malformations are a group of hindbrain malformations where brain tissue herniates through the foramen magnum. Chiari type I is the most common, characterized by cerebellar tonsil herniation of >5mm through the foramen magnum. Patients may be asymptomatic or experience headaches, neck pain, or syringomyelia. Treatment involves decompression surgery. Chiari type II involves brainstem herniation and is associated with myelomeningocele. It causes a small posterior fossa and "banana-shaped" cerebellum. Chiari type III is an encephalocele of the posterior fossa contents through the foramen magnum.
This document summarizes several neural tube closure disorders including Chiari malformations, encephaloceles, anencephaly, spinal dysraphism, and tethered cord syndrome. It describes the anatomy, clinical presentation, and treatment for each condition. Neural tube closure occurs during early gestation and disorders result from failed closure of the brain and spinal cord. The most common types are Chiari malformations, spinal dysraphism including meningoceles and myelomeningoceles, and encephaloceles. Presentation depends on the specific region and tissues involved. Treatment involves surgical repair when possible to address neurological deficits and complications.
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.
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.
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.
Dandy-Walker Malformation: Classification and ManagementDr. Shahnawaz Alam
Dandy-Walker malformation is a congenital brain abnormality where the cerebellum and fourth ventricle are abnormally developed. It ranges in severity from mild vermian hypoplasia to more severe presentations with cyst formation. Treatment involves managing hydrocephalus with ventriculoperitoneal shunting, though some mild cases require no treatment. Complications can include herniation of brain structures if only certain areas are shunted, so simultaneous dual shunting of the lateral ventricles and fourth ventricle cyst may be preferable. Long term outcomes depend on severity but can include normal intelligence if the malformation is mild without other brain anomalies.
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.
This document discusses sensory ataxia, including its causes, clinical presentation, and differential diagnosis. Sensory ataxia results from disorders of the cerebellum, vestibular system, or sensory pathways. It is characterized by incoordination without significant weakness. Key findings include impaired proprioception, positive Romberg's sign, pseudoathetosis, and loss of vibration or joint position sense. Causes include peripheral neuropathies, paraneoplastic disorders, infections like HIV, autoimmune conditions like Sjogren's syndrome, and medications like chemotherapy. Dorsal root ganglionopathies commonly underlie sensory ataxia and can be assessed through nerve conduction studies and lumbar puncture. Distinguishing sensory
This document provides information on myelopathy and spinal cord lesions:
- Myelopathy describes any neurological deficit related to the spinal cord, and can cause quadriplegia, paraplegia, sensory deficits, and autonomic disturbances. It can be inflammatory (myelitis) or vascular in nature (vascular myelopathy).
- Cervical spondylotic myelopathy is the most common form of myelopathy. Lesions of the spinal cord can be compressive (intramedullary or extramedullary) or non-compressive.
- Different levels of spinal cord lesions present with distinct symptoms, such as sensory losses in specific dermatomes or weaknesses of particular muscles. Features help
1. A 32-year-old woman presented with progressive weakness of all four limbs over two months. Examination showed moderate weakness equally in all limbs without wasting, normal tone and reflexes, and no sensory issues, consistent with ALS.
2. An 18-year-old presented with sudden onset weakness of all four limbs for three days. Examination found weakness, diminished tone and reflexes, and vibration sense loss in the toes, consistent with transverse myelitis.
3. A 15-year-old girl reported right abdominal pain and right leg weakness for six months without bladder issues. Exam found spastic right leg weakness, absent lower abdominal reflex, and right lower sensation loss, consistent with a right
A detailed description of benign paroxysmal positional vertigo (BPPV): the symptoms, causes, diagnosis, and treatment methods.For more information, please visit www.everydayhearing.com
1. Spinal shock is a temporary loss of spinal reflex activity below the level of spinal cord injury that occurs immediately after severe spinal cord injury.
2. It is demonstrated by a loss of muscle tone, reflexes, and sensation below the level of injury and can last from hours to weeks depending on the severity and level of injury.
3. Spinal shock results from the loss of descending facilitation from the brain to the spinal cord below the level of injury and goes through phases of areflexia, initial reflex return, hyperreflexia, and eventually spasticity as the spinal cord recovers over time.
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.
Dr Ahmad Taha - Fetal Medicine Unit, Orient Hospital. Presented in Syrian Society of Obstetricians and Gynaecologists Annual Meeting 2015 - Dama Rose Hotel.
This document discusses dysphagia (difficulty swallowing) in pseudobulbar palsy. It begins by defining bulbar palsy and pseudobulbar palsy, noting that bulbar palsy involves lower motor neuron lesions affecting bulbar muscles, while pseudobulbar palsy involves upper motor neuron lesions. It then describes the anatomy and physiology of swallowing, including the four phases. It discusses the causes, signs, and treatments of dysphagia. Key assessment tools mentioned include a video swallow study and 3-ounce water swallow test. The document provides an overview of dysphagia for health professionals.
The craniovertebral junction refers to the occiput, atlas, and axis vertebrae and their articulations and ligaments. It is a complex anatomical region forming the transition between the skull and cervical spine. Common craniovertebral junction anomalies include occipitalization of the atlas, basilar invagination, atlantoaxial dislocation, and dens dysplasia. These anomalies can be developmental, post-traumatic, or acquired. Imaging studies including X-rays, CT, MRI are used to classify and evaluate the anomalies. Treatment may involve surgery such as fusion if the anomaly causes spinal cord compression.
This document summarizes neurological manifestations of human immunodeficiency virus (HIV). It discusses how HIV can affect both the central nervous system and peripheral nervous system. Some key points:
- Up to 50% of HIV patients experience clinically apparent neurological disease. Common manifestations involve the brain, meninges, spinal cord, peripheral nerves, and muscles.
- Direct effects of HIV include infection of cells like macrophages, monocytes, microglia, and possibly astrocytes. Indirect effects include opportunistic infections and neoplasms.
- Common CNS manifestations include HIV encephalopathy/dementia, space-occupying lesions, encephalitis, and stroke-like syndromes. Common peripheral
Intramedullary spinal cord lesions occur within the spinal cord itself while extramedullary lesions occur outside the spinal cord. Intramedullary lesions typically cause symmetrical symptoms that affect motor and sensory functions diffusely down the spinal cord, often with early sphincter involvement. In contrast, extramedullary lesions usually cause asymmetrical symptoms, with local or radicular pain and early upper motor neuron signs but later lower motor neuron signs and sensory involvement in the affected segment.
This document discusses various syndromes that can result from strokes in different areas of the brainstem. It begins with an overview of brainstem anatomy and blood supply. It then describes in detail the clinical presentations of medial and lateral midbrain syndromes, various pontine syndromes including medial and lateral inferior pontine syndromes, and medial and lateral medullary syndromes. Case examples are provided to illustrate the different neurological deficits that can occur based on the location of the brainstem stroke.
Damage to spinal cord structures can cause various impairments depending on the location and extent of the lesion. A complete transection of the spinal cord at L1 would cause ipsilateral loss of light touch and proprioception below the lesion, upper motor neuron signs ipsilaterally below, and contralateral loss of pain and temperature sensation. Epiconus syndrome from a lesion at L4-S2 is associated with weakness of hip and knee flexion and ankle movement with sensory loss from L4-S5 and loss of bladder and bowel control. Cutaneous reflexes and other reflexes have localizing value in myelopathy patients.
Human: Thank you for the summary. Can you provide a 3 sentence summary that focuses
Chiari malformations are a group of hindbrain malformations where brain tissue herniates through the foramen magnum. Chiari type I is the most common, characterized by cerebellar tonsil herniation of >5mm through the foramen magnum. Patients may be asymptomatic or experience headaches, neck pain, or syringomyelia. Treatment involves decompression surgery. Chiari type II involves brainstem herniation and is associated with myelomeningocele. It causes a small posterior fossa and "banana-shaped" cerebellum. Chiari type III is an encephalocele of the posterior fossa contents through the foramen magnum.
This document summarizes several neural tube closure disorders including Chiari malformations, encephaloceles, anencephaly, spinal dysraphism, and tethered cord syndrome. It describes the anatomy, clinical presentation, and treatment for each condition. Neural tube closure occurs during early gestation and disorders result from failed closure of the brain and spinal cord. The most common types are Chiari malformations, spinal dysraphism including meningoceles and myelomeningoceles, and encephaloceles. Presentation depends on the specific region and tissues involved. Treatment involves surgical repair when possible to address neurological deficits and complications.
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.
Congenital malformations of the brain abdul finalabduljelil nejmu
This document provides an overview of congenital malformations of the brain. It discusses normal brain development and then covers various types of malformations including disorders of neurulation (neural tube defects like anencephaly and cephaloceles), disorders of regionalization (holoprosencephaly, septo-optic dysplasia, corpus callosum dysgenesis, and Dandy-Walker malformation), and Chiari malformations (types I-III and variants). For each type of malformation, the document describes key imaging features, clinical presentation, associated conditions, and differential diagnosis.
The document discusses craniovertebral junction anomalies including definitions, classifications of bony and soft tissue anomalies, Arnold-Chiari malformation types, clinical presentations, investigations, and treatments. Major anomalies discussed include platybasia, occipitalization, basilar invagination, dense dysplasia, atlanto-axial disease, Arnold-Chiari malformations, syringomyelia, and syringobulbia. Clinical features, investigations using MRI/CT, and surgical treatments are described for various conditions.
Improved transcranial motor evoked potentials after craniovertebral decompres...Anurag Tewari MD
Surgical strategies towards the treatment of patients with symptomatic Chiari II malformations
(CIIM) are favorable. Despite immediate evaluation and treatment with CSF shunt revision
surgery, a significant population of CIIM patients requires hindbrain decompression. There is
growing evidence for the utility of intraoperative electrophysiological studies, particularly
combinatorial assessment with SSEPS and Tc-MEPs in spinal surgeries for brainstem
compression and myelopathy, but scarce in the pediatric CIIM and myelodysplasia literature.
Here, we report our use of a departmental IONM safety checklist and its efficacy in two cases of
infants presenting with progressive brainstem dysfunction and long-tract signs CIIM hindbrain
decompression.
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.
This document discusses various congenital anomalies that affect the central nervous system, including neural tube defects (NTDs) such as spina bifida occulta, meningocele, meningomyelocele, encephalocele, and anencephaly. It describes the embryology of neural tube formation and risk factors for NTDs such as folic acid deficiency. The document also discusses craniosynostosis, which is the premature fusion of skull sutures, and the various types including plagiocephaly, scaphocephaly, and trigonocephaly. It notes treatment may involve surgery to correct skull growth abnormalities.
This document provides a detailed overview of MRI findings for various spinal pathologies. It discusses preferred imaging sequences and protocols for evaluating the spine, bone marrow, nerves, and cerebrospinal fluid flow. Key points include:
- MRI is the preferred modality for identifying soft tissue and bone marrow abnormalities in the spine. T2-weighted, STIR, and gadolinium-enhanced sequences are often used.
- Dynamic, diffusion, and spectroscopy imaging can provide functional and physiological spinal information. Positioning patients in flexion and extension can identify disc abnormalities.
- Degenerative changes, disc herniations, fractures, infections, and tumors are described. Characteristic MRI patterns help differentiate benign from malignant lesions
a comprehensive presentation on the subject of spinal dysraphism and spina bifida and its neurosurgical management as well as the management of its various other types
The document discusses various causes of central vertigo including:
1. Wallenberg syndrome which is caused by occlusion of the posterior inferior cerebellar artery and presents with nausea, vomiting, nystagmus, ataxia and other neurological signs.
2. Brainstem or cerebellar infarcts which present with abrupt vertigo and accompanying neurological symptoms depending on location and size of infarct.
3. Cerebellar hemorrhage which is a neurosurgical emergency that can cause sudden onset headache, vertigo and vomiting.
4. Other causes discussed include multiple sclerosis, central nervous system tumors, acoustic neuromas, neurodegenerative disorders, epilepsy, familial atax
chiari or arnold chiari malformations, various types and pathophysiology, radiological and clinical presentation of the types, signs symptoms, investigations and treatment of these malformations both conservative and surgical. considerations and controversiies in management of chiari malformation associated with various conditions.
This document discusses various bony and soft tissue anomalies of the craniocervical junction that can cause compression of the brainstem and spinal cord. It outlines different classification systems and measurement lines used to evaluate these anomalies on imaging. Common anomalies discussed include platybasia, basilar invagination, occipito-atlantal fusion, Arnold-Chiari malformation and syringomyelia. Clinical features and approaches to management are also summarized.
This document provides guidance on performing a newborn examination. It discusses examining the baby's history, vital signs, appearance, major body systems and reflexes. The examination is conducted in a warm, well-lit room and includes assessing temperature, heart rate, respiratory rate, blood pressure, color, muscle tone, reflexes, measurements and a full physical exam from head to toe. The exam evaluates the skin, fontanelles, eyes, ears, heart, lungs, abdomen, genitals, limbs and neurological function through assessing tone and primitive reflexes. The goal is to identify any abnormalities and ensure healthy development.
This document provides an overview of syringomyelia, including:
- It is a spinal cord cavity filled with cerebrospinal fluid, with a prevalence of 9 per 100,000 people.
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- Symptoms depend on the location and extent of the syrinx and can include sensory loss, weakness, pain, and autonomic dysfunction.
- Magnetic resonance imaging is the best way to diagnose and assess syringomyelia.
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2. Introductio
n• These comprise a group of abnormalities involving the
rhombencephalon (hindbrain) and the contents of the CV
junction.
• Presentlythere is no consensus regarding the precise
definition, classification, etiology and the surgical
management .
• Series of hindbrain anomalies
• Four types•
3. Definitions of Chiari
malformations• Chiari type I
tonsillar herniation below foramen magnum.
no associated brainstem herniation or supratentorial
anomalies. hydrocephalus uncommon.
• Chiari type II
caudal herniation of brainstem, cerebellar vermis, and fourth
ventricles. associated with myelomeningocele & intracranial
anomalies. hydrocephalus & syringohydromyelia common
• Chiari type III
occipital encephalocele with many of same intracranial anomalies seen
with type II malformation.
• Chiari type IV
hypoplasia / aplasia of cerebellum with no hindbrain herniation
7. Associated anomalies –
Chiari l• Skull
– Basilar skull and cv junction anomalies
(50%)
• Underdevelopment of supraocciput and
exocciput
• Shortening of supraocciput
• Shorter clivus
• Smaller and shallow posterior fossa
• Empty sella
• Platybasia
• Basilar impression
• Midline occipital keel
• Accessory occipital condyle
8. Associated anomalies –
Chiari l
• Spine
– Klippel-Feil deformity
– Atlantoaxial assimilation
– Retroflexion of odontoid
process
– Thickening of ligamentum
flavum
– Scoliosis
9. Associated anomalies –
Chiari l
• Ventricle and cistern
– Hydrocephalus (3-10%)
– Elongated 4th ventricle
– Retrocerebellar CSF space are
obliterated or diminished
10. Associated anomalies –
Chiari l
• Meninges
-- Elevated slope of tentorium
– Thickening of arachnoid at foramen magnum
– Constricting dural bands at level of
foramen magnum and posterior arch of
atlas
– Veils of arachnoid that obstruct fourth
ventricular outlet
15. Associated anomalies –
Chiari ll• Skull
– Craniolcunia or luckenschadel- copper beaten
appearance of calvaria
– Anterior scalloped frontal bone (lemon sign )
– Scalloping of pterous and jugular tubercle
– Enlarged FM
– Notched opisthion
– Elongated clivus with concavity
– Lower inion
– Basilar impression
– Assimilation of atlas
16. Chiari IISupratentorial
pathology• Luckenschadel or Lacunar skull
– result of abnormal radial growth of the skull, seen in upto 85% of
cases
– Focal areas of cortical thinning and scalloped appearance of the
skull
– most prominent at birth, may resolve with age
– not a result of raised ICP and hydrocephalus
19. Genetics of
Chiari
• Familial occurrence
• Concordance in twins and triplets
• Association with other genetic
disorder
– Spondyloepiphyseal dysplasia tarda
– Hadelu-Cheney syndrome
– Klippel-Fiel syndrome
– achondroplasia
20. Chiari
III• herniation of brainstem and cerebellum
into a posterior encephalocoel.
• Very rare; most severe form.
Management difficult.
• Differentiate from cervical myelomeningocoel.
• Severe neurological, developmental and
cranial nerve defects, seizures and
respiratory insufficiency.
• Treatment- well planned encephalocoel
closure
25. Chiari
I• HEADACHE
Most common symptom
(81%) Sub occipital
Radiation to vertex / neck / retro-
bulbar Heavy crushing / pressure
like
↑ed by physical exertion, Valsalva
maneuver, head dependency
and sudden changes In
position
26. Chiari
I• Spinal cord/ syrinx
– sensory
• Numbness: initial complaint, asymmetric, hands/arms
• Dissociated sensory loss: loss of pain and temp, preserved touch
and JPS
• Dysesthesia and proprioception disturbances – advanced stage
• Deep and boring/ itching/ burning
• C2 dysesthesia
• Interscapular pain
• All pain exacerbated by cough and sneeze
• Valsalva or severe cough may alter findings
• Charcot joints: <5% of patients
27. Chiari
I• Spinal cord/ syrinx
– Motor:
• Difficulty in performing fine motor tasks in
UL
• Weakness of hand and/or arm
• Wasting – distal and proximal
• Fasciculations
• Absent DTRs
• UMN lower extremities
• Horners’ – complete or partial
• Bowel and bladder control normal
• Occasionally LMN in lower extremities
28. Chiari
I• Brain stem/ CSF flow/ FM
– Cough headache
– Neck and arm pain – non dermatomal “deep and
boring”
– Down beat nystagmus
– Hoarse voice
– Palatal dysfunction
– Tong– fasciculations/ atrophy
– Dysphagia
– Hiccups
– Severe snoring
– Respiratory dysrhythmias
– Facial numbness
– Drop attacks
– Dysarthria
30. Chiari
II• Commonly presents in infancy, childhood and
adolescence
• May stabilize or improve after 6 to 12mths
• Risk of apneic attacks, dysphagia with aspiration
pneumonia,
life threatening vocal cord paralysis
• Leading cause of death in treated myelodysplastics
within first 2 yrs of life
36. Establishment of
Diagnosis
• X ray CVJ and cervical spine...
• Computed Tomography Scanning
(CT)
• Magnetic Resonance Imaging (MRI)
37. MR
I
• Investigation of choice to assess the
degree of tonsillar descent
• T2 weighted saggital MRI of the spine...
• Helps to screen the whole of the spine
and brain for any other associated
anomaly of the neuraxis or presence of
hydrocephalus
• Septations and flow voids within the
syrinx can be seen
41. PFD vs
PFDD• Durham and Fjeld-Olenec : meta-analysis of
studies that directly compare cohorts of pediatric
patients who underwent PFD with PFDD.
• Patients who undergo duraplasty are less likely
to require reoperation (2.1% vs. 12.6%) for
persistent or recurrent symptoms but are more
likely to suffer CSF- related complications
• No statistical difference in clinical outcomes
between the two groups, specifically with regard
to symptom improvement and syringomyelia
42. • clinical improvement were 65% in the PFD
patients and 79% in the PFDD patients
• Syrinx resolution :56% in the PFD patients and
87% in those undergoing PFDD.
43.
44. Selection of Surgical
Procedure
• Depends on whether chiari is associated
with syrinx or not.
• Also on the type and degress of tonsillar
descent.
• The key is to decompress the posterior fossa
and CVJ adequately, and to establish
normal CSF flow across the region of
formen magnum.
45. Various procedures
adopted
• FMD alone
• FMD with lax duroplasty
• FMD with arachnoid adhesiolysis
and lax duroplasty
• FMD with tonsillar resection, and lax
duraplasty
• FMD with any of the above and
additional removal of C2.
FMD = Suboccipital craniectomy encompassing the
foramen magnum rim and C1 posterior arch
54. Bleeding at
durotomy
• Cerebellar dura may have venous lakes
• Circular or occipital sinus may bleed
profusely
• Control
– Proceed slowly
– Bipolar
– Metal Clips
– Figure of 8 stiches
61. Recurrent or Unresolved Chiari
Failed procedure
• Causes:
– Inadequate decompression
• Bony
• Soft tissue
– Reformation of arachnoid scars
– Lack of CSF flow normalization despite
adquate soft tissue and bony
decompression
• Management
– Revision surgery
– Shunting of the syrinx
62. Follow
up• Chiari I
• Pts without a syrinx: follow up at 1, 6 &
12 months, then every 12 to 24 months
;no need of repeat imaging (if there is
symptomatic improvement)
• Pts with syrinx: follow up MRI in 6
to 12 months.No further imaging if
symptoms improve or syrinx
decreases in size significantly.