Chronic Acquired Demyelinating Polyneuropathy
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Chronic acquired demyelinating polyneuropathy (CADP) variants include chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), and distal acquired demyelinating symmetric neuropathy (DADS). Each condition has unique clinical features, electrodiagnostic tests, and responses to treatment that help distinguish them as part of the heterogeneous group of immune-mediated demyelinating disorders of the peripheral nervous system.
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Motor neuron diseases
Upper motor neurons convey impulses for voluntary motor activity and exert control over lower motor neurons, which directly innervate skeletal muscle. Upper motor neuron cell bodies are located in the motor cortex and premotor areas. Their axons form tracts that project to lower motor neurons in the brainstem and spinal cord. Lower motor neuron cell bodies are located in the brainstem and spinal cord. Damage to upper motor neurons results in spasticity and hyperreflexia, while lower motor neuron damage causes weakness, atrophy, fasciculations and hyporeflexia. Amyotrophic lateral sclerosis is a motor neuron disorder characterized by both upper and lower motor neuron degeneration.
Lambert Eaton Myasthenic Syndrome (LEMS)
A 49-year-old female presented with 10 months of progressive muscular weakness, mostly in the lower limbs. She also reported intense muscular pain, weight loss, dry mouth, and reduced libido. Testing found reduced reflexes that improved with exertion. Electrodiagnostic studies showed reduced compound muscle action potentials that increased with stimulation. Muscle biopsy showed type II fiber atrophy. She tested positive for calcium channel antibodies. The diagnosis was Lambert-Eaton myasthenic syndrome (LEMS), a rare autoimmune disorder caused by antibodies against calcium channels.
Friedreich’s Ataxia
FA is a very rare, genetic, recessive disease, affecting 1/50,000 people.
Originates from mutations in the “coding” of the mitochondria.
Discovered by Nicholaus Friedreich in the early 1860’s.
Both parents must have the dominant trait for a 25% chance of an offspring possessing the disease.
Not necessarily a disease that kills you, but eventually a wheelchair and regular assistance will be required.
Onset before age 20-25 year.
Approach to myopathy
The document provides guidance on evaluating patients presenting with suspected muscle disease. It outlines the key goals of determining the site of lesion, cause, and available treatments. Symptoms like weakness, fatigue, myalgia and their patterns are discussed to help determine underlying conditions. The temporal evolution, including acute vs chronic onset and progression, helps differentiate between genetic, inflammatory and metabolic myopathies. Considering symptom precipitants and distributions can provide clues to diagnose specific myopathies based on pattern recognition of proximal, distal or other muscle group involvement.
coma
This document discusses coma and disorders of consciousness. It defines coma as a state of unresponsiveness and unconsciousness, and notes that coma can be a medical emergency requiring intervention. The document outlines different levels of arousal from alert to coma and describes conditions like encephalopathy, locked-in syndrome, and persistent vegetative state. Causes of impaired consciousness discussed include alcohol, epilepsy, intoxication, trauma, infection, stroke, and hypoxia-ischemia. The Glasgow Coma Scale for assessing coma is also summarized.
Wilson's disease
Wilson's disease is caused by a genetic defect resulting in excessive copper deposition in tissues. It impairs the liver's ability to excrete copper into bile. Clinical features vary widely and include hepatic, neurological or psychiatric symptoms. Diagnosis involves low serum ceruloplasmin, high urinary copper, and Kayser-Fleisher rings on eye exam. Treatment includes copper-restricting agents like penicillamine or trientine to increase copper excretion, or zinc to reduce copper absorption. Liver transplantation may be considered for severe liver disease. With lifelong treatment, prognosis is good if Wilson's disease is diagnosed and managed early.
Myoclonus
Myoclonus is characterized by brief, involuntary muscle contractions or inhibitions. It can be classified anatomically based on its physiological origin in the cortex, subcortex, or periphery. Clinically, myoclonus is classified as physiological, essential, epileptic, or secondary. Treatment involves addressing the underlying cause, with anti-seizure medications often used for cortical or cortical-subcortical myoclonus, and benzodiazepines or botulinum toxin injections for other types.
Motor neuron disease - Etiology, Pathogenesis, Clinical Features, Classificat...
Motor neuron disease - Etiology, Pathogenesis, Clinical Features, Classificat...Chetan Ganteppanavar
This document discusses motor neuron diseases, including amyotrophic lateral sclerosis (ALS). It provides details on the classification, symptoms, signs, diagnosis, prognosis, and management of ALS and related conditions. Key points include that ALS is characterized by the degeneration of both upper and lower motor neurons, leading to muscle weakness, atrophy, and fasciculations. Diagnosis involves finding signs of both upper and lower motor neuron involvement. Prognosis is typically worse if onset is bulbar or simultaneous in multiple limbs. Treatment focuses on managing symptoms while no treatments have been proven to slow disease progression.Recommended
Motor neuron diseases
Upper motor neurons convey impulses for voluntary motor activity and exert control over lower motor neurons, which directly innervate skeletal muscle. Upper motor neuron cell bodies are located in the motor cortex and premotor areas. Their axons form tracts that project to lower motor neurons in the brainstem and spinal cord. Lower motor neuron cell bodies are located in the brainstem and spinal cord. Damage to upper motor neurons results in spasticity and hyperreflexia, while lower motor neuron damage causes weakness, atrophy, fasciculations and hyporeflexia. Amyotrophic lateral sclerosis is a motor neuron disorder characterized by both upper and lower motor neuron degeneration.
Lambert Eaton Myasthenic Syndrome (LEMS)
A 49-year-old female presented with 10 months of progressive muscular weakness, mostly in the lower limbs. She also reported intense muscular pain, weight loss, dry mouth, and reduced libido. Testing found reduced reflexes that improved with exertion. Electrodiagnostic studies showed reduced compound muscle action potentials that increased with stimulation. Muscle biopsy showed type II fiber atrophy. She tested positive for calcium channel antibodies. The diagnosis was Lambert-Eaton myasthenic syndrome (LEMS), a rare autoimmune disorder caused by antibodies against calcium channels.
Friedreich’s Ataxia
FA is a very rare, genetic, recessive disease, affecting 1/50,000 people.
Originates from mutations in the “coding” of the mitochondria.
Discovered by Nicholaus Friedreich in the early 1860’s.
Both parents must have the dominant trait for a 25% chance of an offspring possessing the disease.
Not necessarily a disease that kills you, but eventually a wheelchair and regular assistance will be required.
Onset before age 20-25 year.
Approach to myopathy
The document provides guidance on evaluating patients presenting with suspected muscle disease. It outlines the key goals of determining the site of lesion, cause, and available treatments. Symptoms like weakness, fatigue, myalgia and their patterns are discussed to help determine underlying conditions. The temporal evolution, including acute vs chronic onset and progression, helps differentiate between genetic, inflammatory and metabolic myopathies. Considering symptom precipitants and distributions can provide clues to diagnose specific myopathies based on pattern recognition of proximal, distal or other muscle group involvement.
coma
This document discusses coma and disorders of consciousness. It defines coma as a state of unresponsiveness and unconsciousness, and notes that coma can be a medical emergency requiring intervention. The document outlines different levels of arousal from alert to coma and describes conditions like encephalopathy, locked-in syndrome, and persistent vegetative state. Causes of impaired consciousness discussed include alcohol, epilepsy, intoxication, trauma, infection, stroke, and hypoxia-ischemia. The Glasgow Coma Scale for assessing coma is also summarized.
Wilson's disease
Wilson's disease is caused by a genetic defect resulting in excessive copper deposition in tissues. It impairs the liver's ability to excrete copper into bile. Clinical features vary widely and include hepatic, neurological or psychiatric symptoms. Diagnosis involves low serum ceruloplasmin, high urinary copper, and Kayser-Fleisher rings on eye exam. Treatment includes copper-restricting agents like penicillamine or trientine to increase copper excretion, or zinc to reduce copper absorption. Liver transplantation may be considered for severe liver disease. With lifelong treatment, prognosis is good if Wilson's disease is diagnosed and managed early.
Myoclonus
Myoclonus is characterized by brief, involuntary muscle contractions or inhibitions. It can be classified anatomically based on its physiological origin in the cortex, subcortex, or periphery. Clinically, myoclonus is classified as physiological, essential, epileptic, or secondary. Treatment involves addressing the underlying cause, with anti-seizure medications often used for cortical or cortical-subcortical myoclonus, and benzodiazepines or botulinum toxin injections for other types.
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This document discusses motor neuron diseases, including amyotrophic lateral sclerosis (ALS). It provides details on the classification, symptoms, signs, diagnosis, prognosis, and management of ALS and related conditions. Key points include that ALS is characterized by the degeneration of both upper and lower motor neurons, leading to muscle weakness, atrophy, and fasciculations. Diagnosis involves finding signs of both upper and lower motor neuron involvement. Prognosis is typically worse if onset is bulbar or simultaneous in multiple limbs. Treatment focuses on managing symptoms while no treatments have been proven to slow disease progression.Transverse myelitis
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.
Transverse myelitis
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,
Neuromyelitis optica spectrum disorders
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Non-Compressive Myelopathy
This document discusses various causes of non-compressive myelopathy, including infectious, inflammatory, vascular, metabolic, degenerative, and physical agent causes. It covers specific conditions such as transverse myelitis, multiple sclerosis, spinal infarction, vascular malformations, vitamin B12 deficiency, and radiation myelopathy. Treatment approaches are mentioned for some conditions.
Compressive Myelopathy
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.
Approach to a patient with stroke
The document defines various types of strokes and transient ischemic attacks. It discusses the epidemiology, risk factors, clinical features, investigations, and management of strokes. The main types are ischemic and hemorrhagic strokes. Investigations include brain imaging like CT scan and MRI to identify the type of stroke and underlying causes. Treatment focuses on minimizing brain damage, preventing complications, rehabilitation, and reducing the risk of recurrence.
Spinal cord syndromes
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.
approach to polyneuropathy
This document provides an overview of the approach to polyneuropathy. It begins by defining peripheral neuropathy and classifying it as mononeuropathy, multiple mononeuropathy, or polyneuropathy. It then discusses the anatomical classification and role of nerve fibers. The main etiologies of polyneuropathy are described including metabolic, toxic, vitamin deficiencies, inflammatory, infectious, hereditary, neoplastic, and idiopathic causes. The pathophysiology involves axonal degeneration, demyelination, and Wallerian degeneration. Key features that help differentiate axonal from demyelinating neuropathies are outlined. The document concludes by discussing investigations, treatment approaches, and references.
Congenital myopathy
This document provides an overview of congenital myopathies and congenital muscular dystrophies. It defines congenital myopathies as muscle disorders presenting in infancy with generalized muscle weakness and hypotonia. Several types of congenital myopathies are described based on their histopathological features, including nemaline myopathy, central core disease, centronuclear myopathy, and congenital fiber type disproportion. The clinical features, investigations, pathology, genetics, and management are discussed for each type. Congenital muscular dystrophies are also briefly introduced.
Disorders of autonomic nervous system
This document discusses disorders of the autonomic nervous system. It begins by describing the anatomy and neurotransmitters of the sympathetic and parasympathetic nervous systems. It then discusses various tests used to evaluate autonomic function, including heart rate variation with deep breathing, the Valsalva maneuver, sudomotor function tests, and orthostatic blood pressure recordings. The document concludes by describing specific autonomic disorders like Parkinson's disease, spinal cord lesions, autoimmune autonomic neuropathy, and postural orthostatic tachycardia syndrome.
Hepatic encephalopathy
Hepatic encephalopathy is a brain dysfunction caused by liver disease or portosystemic shunts. Gut-derived toxins such as ammonia cross the blood-brain barrier and cause neurological or psychiatric abnormalities. Treatment involves removing precipitating causes, lowering ammonia levels through antibiotics, lactulose, and BCAA supplementation, and correcting nutritional deficiencies. For refractory or recurrent cases, transplantation may be required.
Chronic inflammatory demyelinating Polyradiculoneuropathy
Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is a rare neurological disorder characterized by progressive weakness and impaired sensory function caused by damage to the peripheral nervous system. It has an incidence rate of 1-2 per 100,000 people and predominantly affects males over 50 years old. CIDP is diagnosed based on progressive muscle weakness, reduced reflexes, nerve conduction studies showing demyelination, elevated CSF protein, and nerve biopsy with signs of demyelination. Treatment involves intravenous immunoglobulin, plasma exchange, steroids, or other immunosuppressants, with around 50% of patients experiencing improvement with therapy.
Guillain-Barré syndrome -Dr Sajith Sebastian
Guillain-Barré syndrome is an acute inflammatory demyelinating polyneuropathy that is the most common cause of acute flaccid paralysis. It has an annual incidence of 0.6 to 4 cases per 100,000 people and often follows a bacterial or viral infection. Campylobacter jejuni infection is the most commonly identified precipitant. Guillain-Barré syndrome results from an autoimmune attack on the peripheral nervous system that causes muscle weakness and loss of reflexes. Treatment involves supportive care along with plasmapheresis or intravenous immunoglobulin to stop the immune response. Most patients recover fully but some are left with permanent neurological deficits.
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Motor neuron diseases are a group of rare neurodegenerative disorders that progressively cause nerves in the brain and spinal cord to lose function, affecting voluntary muscle movement. The most common type in adults is amyotrophic lateral sclerosis (ALS), which affects both upper and lower motor neurons. There is no cure for motor neuron diseases, so treatment focuses on managing symptoms and maximizing quality of life through supportive care, rehabilitation, and FDA-approved drugs that may slow progression. Prognosis depends on the specific type of motor neuron disease and age of onset, with some forms being fatal and others non-fatal but still impacting quality of life over time.
Motor neuron disease
Motor neuron disease (MND) refers to conditions characterized by degeneration of upper and lower motor neurons. Amyotrophic lateral sclerosis (ALS) is the most common form of MND and involves both upper and lower motor neurons. ALS is clinically defined based on involvement of motor neurons and includes features such as muscle weakness, atrophy, fasciculations, and stiffness. The pathology of ALS involves degeneration and death of motor neurons in the brain, brainstem, and spinal cord leading to muscle denervation and atrophy. While the cause of ALS is largely unknown, factors such as oxidative stress, protein aggregation, mitochondrial dysfunction, and glutamate excitotoxicity are hypothesized to contribute to motor neuron de
Myopathies
This document discusses various types of myopathies (disorders affecting muscle). It defines myopathies and distinguishes them from other causes of muscle weakness. It then describes different categories of myopathies including inflammatory myopathies (such as polymyositis and dermatomyositis), muscular dystrophies (such as Duchenne, Becker, limb-girdle, facioscapulohumeral), congenital myopathies, metabolic myopathies, and others. For each type, it discusses inheritance, clinical features, diagnostic criteria, and treatment when available.
Polymyalgia rheumatica
This document discusses polymyalgia rheumatica (PMR), a clinical syndrome characterized by severe aching and stiffness in the neck, shoulder, and pelvic areas. It affects people over age 50, especially whites and females. Symptoms include morning stiffness and pain in proximal muscles that is worse with inactivity. Treatment involves corticosteroids like prednisone to reduce inflammation over 2-4 years as the average length of the disease is 3 years and relapses are common if steroids are tapered too quickly.
Approach to myopathy
This document provides an overview of the approach to evaluating and diagnosing myopathies. It discusses the types of muscle fibers and symptoms that may be present in patients with myopathies. The evaluation involves assessing temporal evolution, distribution of weakness, family history, and laboratory/diagnostic testing including CK levels, EMG, and muscle biopsy. Causes of myopathies include genetic, acquired, inflammatory/immune, and those associated with other systemic illnesses. Specific myopathies discussed include central core disease, nemaline myopathy, and centronuclear myopathy.
Still's disease
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.
Tardive Dyskinesia
Tardive dyskinesia is a delayed onset movement disorder caused by dopamine receptor-blocking agents. It affects 20-50% of patients treated with neuroleptics long-term and is characterized by involuntary movements, especially of the face. Diagnosis involves ruling out other causes and observing symptoms for at least a month after discontinuing the offending drug. Management focuses on withdrawing the causal medication, though symptoms often persist long-term.
Original publications
This document lists 48 original publications by JC Möller and collaborators related to Parkinson's disease, multiple system atrophy, restless legs syndrome, and other movement disorders. The publications cover a range of topics including experimental pain sensitivity, clinical decision making tools for chronic pain patients, roles of cortical and pallidal brain frequencies, association between sleep and dyskinesia in an animal model of Parkinson's, and several genetic studies related to various movement disorders.
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Transverse myelitis
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.
Transverse myelitis
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,
Neuromyelitis optica spectrum disorders
This document provides an overview of neuromyelitis optica spectrum disorders (NMOSD). It discusses the epidemiology, clinical features, diagnostic criteria, investigations, neuroimaging findings, and treatments for NMOSD. Key points include that NMOSD predominantly affects the optic nerves and spinal cord, is strongly associated with antibodies against the aquaporin-4 protein, and treatments involve high-dose steroids, plasma exchange, or intravenous immunoglobulins for acute exacerbations. The diagnostic criteria were revised in 2015 to incorporate aquaporin-4 antibody testing and distinguish NMOSD from multiple sclerosis.
Non-Compressive Myelopathy
This document discusses various causes of non-compressive myelopathy, including infectious, inflammatory, vascular, metabolic, degenerative, and physical agent causes. It covers specific conditions such as transverse myelitis, multiple sclerosis, spinal infarction, vascular malformations, vitamin B12 deficiency, and radiation myelopathy. Treatment approaches are mentioned for some conditions.
Compressive Myelopathy
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.
Approach to a patient with stroke
The document defines various types of strokes and transient ischemic attacks. It discusses the epidemiology, risk factors, clinical features, investigations, and management of strokes. The main types are ischemic and hemorrhagic strokes. Investigations include brain imaging like CT scan and MRI to identify the type of stroke and underlying causes. Treatment focuses on minimizing brain damage, preventing complications, rehabilitation, and reducing the risk of recurrence.
Spinal cord syndromes
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.
approach to polyneuropathy
This document provides an overview of the approach to polyneuropathy. It begins by defining peripheral neuropathy and classifying it as mononeuropathy, multiple mononeuropathy, or polyneuropathy. It then discusses the anatomical classification and role of nerve fibers. The main etiologies of polyneuropathy are described including metabolic, toxic, vitamin deficiencies, inflammatory, infectious, hereditary, neoplastic, and idiopathic causes. The pathophysiology involves axonal degeneration, demyelination, and Wallerian degeneration. Key features that help differentiate axonal from demyelinating neuropathies are outlined. The document concludes by discussing investigations, treatment approaches, and references.
Congenital myopathy
This document provides an overview of congenital myopathies and congenital muscular dystrophies. It defines congenital myopathies as muscle disorders presenting in infancy with generalized muscle weakness and hypotonia. Several types of congenital myopathies are described based on their histopathological features, including nemaline myopathy, central core disease, centronuclear myopathy, and congenital fiber type disproportion. The clinical features, investigations, pathology, genetics, and management are discussed for each type. Congenital muscular dystrophies are also briefly introduced.
Disorders of autonomic nervous system
This document discusses disorders of the autonomic nervous system. It begins by describing the anatomy and neurotransmitters of the sympathetic and parasympathetic nervous systems. It then discusses various tests used to evaluate autonomic function, including heart rate variation with deep breathing, the Valsalva maneuver, sudomotor function tests, and orthostatic blood pressure recordings. The document concludes by describing specific autonomic disorders like Parkinson's disease, spinal cord lesions, autoimmune autonomic neuropathy, and postural orthostatic tachycardia syndrome.
Hepatic encephalopathy
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Chronic inflammatory demyelinating Polyradiculoneuropathy
Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is a rare neurological disorder characterized by progressive weakness and impaired sensory function caused by damage to the peripheral nervous system. It has an incidence rate of 1-2 per 100,000 people and predominantly affects males over 50 years old. CIDP is diagnosed based on progressive muscle weakness, reduced reflexes, nerve conduction studies showing demyelination, elevated CSF protein, and nerve biopsy with signs of demyelination. Treatment involves intravenous immunoglobulin, plasma exchange, steroids, or other immunosuppressants, with around 50% of patients experiencing improvement with therapy.
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Guillain-Barré syndrome is an acute inflammatory demyelinating polyneuropathy that is the most common cause of acute flaccid paralysis. It has an annual incidence of 0.6 to 4 cases per 100,000 people and often follows a bacterial or viral infection. Campylobacter jejuni infection is the most commonly identified precipitant. Guillain-Barré syndrome results from an autoimmune attack on the peripheral nervous system that causes muscle weakness and loss of reflexes. Treatment involves supportive care along with plasmapheresis or intravenous immunoglobulin to stop the immune response. Most patients recover fully but some are left with permanent neurological deficits.
Motor neuron disease.pptx new
Motor neuron diseases are a group of rare neurodegenerative disorders that progressively cause nerves in the brain and spinal cord to lose function, affecting voluntary muscle movement. The most common type in adults is amyotrophic lateral sclerosis (ALS), which affects both upper and lower motor neurons. There is no cure for motor neuron diseases, so treatment focuses on managing symptoms and maximizing quality of life through supportive care, rehabilitation, and FDA-approved drugs that may slow progression. Prognosis depends on the specific type of motor neuron disease and age of onset, with some forms being fatal and others non-fatal but still impacting quality of life over time.
Motor neuron disease
Motor neuron disease (MND) refers to conditions characterized by degeneration of upper and lower motor neurons. Amyotrophic lateral sclerosis (ALS) is the most common form of MND and involves both upper and lower motor neurons. ALS is clinically defined based on involvement of motor neurons and includes features such as muscle weakness, atrophy, fasciculations, and stiffness. The pathology of ALS involves degeneration and death of motor neurons in the brain, brainstem, and spinal cord leading to muscle denervation and atrophy. While the cause of ALS is largely unknown, factors such as oxidative stress, protein aggregation, mitochondrial dysfunction, and glutamate excitotoxicity are hypothesized to contribute to motor neuron de
Myopathies
This document discusses various types of myopathies (disorders affecting muscle). It defines myopathies and distinguishes them from other causes of muscle weakness. It then describes different categories of myopathies including inflammatory myopathies (such as polymyositis and dermatomyositis), muscular dystrophies (such as Duchenne, Becker, limb-girdle, facioscapulohumeral), congenital myopathies, metabolic myopathies, and others. For each type, it discusses inheritance, clinical features, diagnostic criteria, and treatment when available.
Polymyalgia rheumatica
This document discusses polymyalgia rheumatica (PMR), a clinical syndrome characterized by severe aching and stiffness in the neck, shoulder, and pelvic areas. It affects people over age 50, especially whites and females. Symptoms include morning stiffness and pain in proximal muscles that is worse with inactivity. Treatment involves corticosteroids like prednisone to reduce inflammation over 2-4 years as the average length of the disease is 3 years and relapses are common if steroids are tapered too quickly.
Approach to myopathy
This document provides an overview of the approach to evaluating and diagnosing myopathies. It discusses the types of muscle fibers and symptoms that may be present in patients with myopathies. The evaluation involves assessing temporal evolution, distribution of weakness, family history, and laboratory/diagnostic testing including CK levels, EMG, and muscle biopsy. Causes of myopathies include genetic, acquired, inflammatory/immune, and those associated with other systemic illnesses. Specific myopathies discussed include central core disease, nemaline myopathy, and centronuclear myopathy.
Still's disease
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.
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Chronic inflammatory demyelinating Polyradiculoneuropathy
Chronic inflammatory demyelinating Polyradiculoneuropathy
Similar to Chronic Acquired Demyelinating Polyneuropathy
Tardive Dyskinesia
Tardive dyskinesia is a delayed onset movement disorder caused by dopamine receptor-blocking agents. It affects 20-50% of patients treated with neuroleptics long-term and is characterized by involuntary movements, especially of the face. Diagnosis involves ruling out other causes and observing symptoms for at least a month after discontinuing the offending drug. Management focuses on withdrawing the causal medication, though symptoms often persist long-term.
Original publications
This document lists 48 original publications by JC Möller and collaborators related to Parkinson's disease, multiple system atrophy, restless legs syndrome, and other movement disorders. The publications cover a range of topics including experimental pain sensitivity, clinical decision making tools for chronic pain patients, roles of cortical and pallidal brain frequencies, association between sleep and dyskinesia in an animal model of Parkinson's, and several genetic studies related to various movement disorders.
Steroid Myopathy
Steroid myopathy is a non-inflammatory toxic myopathy typically caused by fluorinated glucocorticoids. It primarily affects postural muscles in the proximal regions. Clinical manifestations include muscle wasting, weakness, and fatigability that can present acutely or chronically. Diagnosis involves assessing clinical history, electromyography, laboratory assays, imaging, and muscle biopsy showing type 2 fiber atrophy without necrosis. Treatment focuses on reducing steroid dose when possible or switching to non-fluorinated steroids, improving nutrition, and physical activity.
Stiff Person Syndrome
This document discusses Stiff Person Syndrome (SPS), a rare autoimmune neurological disorder. It provides information on the epidemiology, pathophysiology, clinical manifestations, diagnosis, variants, treatment and prognosis of SPS. SPS is often misdiagnosed or underdiagnosed and is characterized by painful muscle rigidity and spasms. It involves autoimmunity related to glutamic acid decarboxylase or other synaptic proteins. Treatment involves diazepam and other GABAergic drugs, which can provide relief from symptoms.
Neuropsychiatric Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect the central nervous system and cause neuropsychiatric symptoms. Diagnosing neuropsychiatric SLE (NPSLE) is challenging due to its diverse manifestations. Genetic, autoantibody, and cytokine factors may contribute to NPSLE pathogenesis by disrupting the blood-brain barrier and causing inflammatory changes. Treatment involves immunosuppression but outcomes depend on the specific neuropsychiatric symptoms.
Hereditary Diffuse Leukoencephalopathy with Axonal Spheroids
Hereditary Diffuse Leukoencephalopathy with Axonal Spheroids (HDLS) is a rare, autosomal dominant, neurodegenerative disease characterized by white matter changes and neuroaxonal spheroids in the brain. It typically presents in mid-life with personality changes, cognitive decline, and motor impairments. Magnetic resonance imaging shows white matter lesions spreading from the periventricular areas. The disease progresses rapidly over 6-11 years, leading to death within about a decade of onset. The pathology involves primary axonal damage and secondary demyelination or vice versa. An accurate diagnosis currently requires brain histology as the causal genes remain unknown.
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Septic encephalopathy is characterized by diffuse brain dysfunction that occurs in patients with sepsis without direct evidence of intracranial infection or metabolic causes unrelated to the infectious process. It is a common complication among critically ill patients, occurring in 9-71% of cases. Diagnosis involves assessing for altered mental status in the setting of sepsis through EEG, biomarkers of brain injury, and neuroimaging while excluding other potential causes. Management focuses on early treatment of the underlying sepsis through organ support and intensive care, with adjunctive therapies like EEG monitoring and antipsychotics potentially helping to guide care and symptoms.
Electrodiagnostic Evaluation of Amyotrophic Lateral Sclerosis
The document summarizes key points about electrodiagnostic evaluation of amyotrophic lateral sclerosis (ALS):
- Nerve conduction studies and needle electromyography are used to detect lower motor neuron involvement and rule out other disorders like multifocal motor neuropathy. Nerve conduction studies examine motor and sensory nerves while needle EMG assesses denervation and reinnervation in weak muscles.
- Nerve conduction studies in ALS may show normal or decreased compound muscle action potentials and prolonged distal motor latencies. Needle EMG reveals widespread acute denervation through fasciculations and chronic denervation seen as increased duration and amplitude of motor unit potentials.
- A thorough clinical exam is needed before electrodiagnostic testing
Autoimmune movement disorders
This document discusses autoimmune movement disorders, which can mimic neurodegenerative or metabolic conditions. Autoimmune syndromes are rarely isolated and accompanying clinical signs help with diagnosis. A detailed history and examination can reveal red flags to guide diagnosis, as timely identification is important given these conditions are treatable. The document then examines the clinical approach and various autoimmune movement disorders like cerebellar ataxia, chorea, dystonia, myoclonus, parkinsonism, paroxysmal movement disorders, stiff person spectrum disorders, tics, tremor, and sleep behavior disorders. It covers their characteristics, potential antibodies, investigations including imaging and antibody testing, management with drug therapy, and variable response and prognosis.
Todd's Paralysis
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Chronic Acquired Demyelinating Polyneuropathy
- 1. Chronic Acquired Demyelinating Polyneuropathy A CIDP variants Ade Wijaya, MD – August 2018
- 2. Chronic Aquired Demyelinating Polineuropathy • CIDP • MMN • MADSAM • DADS
- 3. History 1890 first case reccurent neuritis published by Eichhorst 1958, Austin announced that this kind of neuropathy responsive to steroid Eichhorst H. Beitrage zur pathologie der nerven und muskeln.Cor Kl Schweiz Aertze. 1890;20:59–71. Austin JH. Recurrent polyneuropathies and their corticosteroids treatment. Brain. 1958;8:157–92.
- 4. CIDP • Proximal and distal weakness • Large fiber sensory loss • Elevated cerebrospinal fluid (CSF) protein content • Demyelinating changes nerve conduction studies or nerve biopsy • Response to immunomodulating treatment. Dimachkie, M. M., Barohn, R. J., & Katz, J. (2013). Multifocal motor neuropathy, multifocal acquired demyelinating sensory and motor neuropathy, and other chronic acquired demyelinating polyneuropathy variants. Neurologic clinics, 31(2), 533-555.
- 5. Multifocal Motor Neuropathy (MMN) • First demonstrated by parry and colleagues, at 1988 • M:F = 3:1 • Onset mostly on 5th decade (range: 1st – 8th decade) Parry GJ, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle Nerve. 1988;11:103–107.
- 7. Multifocal Acquired Demyelinating Sensory and Motor (MADSAM) Neuropathy • Lewis-Summer Syndrome • Similar to MMN with sensory involvement • Up to 50 % develop to CIDP • Cranial neuropathies are reported Saperstein DS, Amato AA, Wolfe GI, Katz JS, Nations SP, Jackson CE, Bryan WW, Burns DK, Barohn RJ. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve. 1999;22:560–566. Lewis RA, Sumner AJ, Brown MJ, Asbury AK. Multifocal demyelinating neuropathy with persistent conduction block. Neurology. 1982;32:958–964. Oh SJ, Claussen GC, Dae SK. Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate variant of chronic inflammatory demyelinating polyneuropathy. J Periph Nerv Syst. 1997;2:362–369.
- 8. Distal Acquired Demyelinating Symmetric (DADS) Neuropathy • DADS-M & DADS-I • DADS-M related to M protein; resistant to immunotherapy (more common) • DADS-I (idiopathic) responsive to immunotherapy (less common) • Men • 6th decade or later Katz JS, Saperstein DS, Gronseth G, Amato AA, Barohn RJ. Distal acquired demyelinating symmetric (DADS) neuropathy. Neurology. 2000;54:615–620.
- 9. Dimachkie, M. M., Barohn, R. J., & Katz, J. (2013). Multifocal motor neuropathy, multifocal acquired demyelinating sensory and motor neuropathy, and other chronic acquired demyelinating polyneuropathy variants. Neurologic clinics, 31(2), 533-555.
- 10. Dimachkie, M. M., Barohn, R. J., & Katz, J. (2013). Multifocal motor neuropathy, multifocal acquired demyelinating sensory and motor neuropathy, and other chronic acquired demyelinating polyneuropathy variants. Neurologic clinics, 31(2), 533-555.
- 11. Dimachkie, M. M., Barohn, R. J., & Katz, J. (2013). Multifocal motor neuropathy, multifocal acquired demyelinating sensory and motor neuropathy, and other chronic acquired demyelinating polyneuropathy variants. Neurologic clinics, 31(2), 533-555.
- 13. Less Common CADP Variants • Chronic inflammatory sensory polyradiculopathy (CISP) with normal NCS but abnormal somatosensory evoked potentials • Chronic ataxic neuropathy with ophthalmoparesis, M protein, cold agglutinins and disialosyl ganglioside antibodies (CANOMAD) Sinnreich M, Klein CJ, Daube JR, Engelstad J, Spinner RJ, Dyck PJ. Chronic immune sensory polyradiculopathy: a possibly treatable sensory ataxia. Neurology. 2004;63(9):1662–1669. Willison HJ, O'Leary CP, Veitch J, Blumhardt LD, Busby M, Donaghy M, Fuhr P, Ford H, Hahn A, Renaud S, Katifi HA, Ponsford S, Reuber M, Steck A, Sutton I, Schady W, Thomas PK, Thompson AJ, Vallat JM, Winer J. The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies. Brain. 2001 Oct;124(Pt 10):1968–77.
- 14. SUMMARY • CADP are a heterogenous group of immune-mediated neuromuscular disorders affecting myelin that include MMN, MADSAM, DADS and other less common variants. • Each of these entities has distinctive laboratory and electrodiagnostic features that aid in their diagnosis, clinical characteristics are of paramount importance in diagnosing specific conditions and determining the most appropriate therapies