Lambert–Eaton myasthenic syndrome ( lems)
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Mrs. Johnson, a 45-year-old woman, presented with leg weakness, difficulty climbing stairs and dropping eyelids as her main symptoms. Her symptoms also included dry eyes, dry mouth, dizziness, swallowing problems, blurred vision, and erectile dysfunction. The main cause of her condition was determined to be cancer, as her symptoms developed after being attacked by Lambert-Eaton myasthenic syndrome, a rare autoimmune disorder where cancer cells affect the connection between nerves and muscles.
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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.
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS), AKA "Lou Gehrig's Disease," is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
NEUROMYOTONIA : A COMPLETE REVIEW
Acquired neuromyotonia is an inflammatory disorder characterized by abnormal nerve impulses from peripheral nerves that cause continuous muscle fiber activity and symptoms like muscle stiffness, cramping and weakness. It has several potential causes including an unknown acquired cause, paraneoplastic syndrome related to cancer, or a hereditary form. Diagnosis involves identifying continuous muscle contractions and abnormal electrical muscle activity on electromyography. Treatment focuses on using anti-convulsant drugs to stop abnormal nerve impulses or plasma exchange to provide short-term relief from symptoms.
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.
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,
Myasthenic crisis
This document discusses myasthenia crisis, which is a life-threatening condition defined as weakness from myasthenia gravis that requires intubation or delays extubation after surgery. It presents information on the epidemiology, pathophysiology, clinical presentation, diagnosis, and management of myasthenia crisis. Key aspects of management include admission to the intensive care unit, assessment of respiratory function, elective intubation if needed, rapid immunomodulating therapy, careful weaning, and monitoring for complications.
Motor neuron disease - Etiology, Pathogenesis, Clinical Features, Classificat...
Motor neuron disease - Etiology, Pathogenesis, Clinical Features, Classificat...Chetan Ganteppanavar
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This document discusses peripheral neuropathy, which refers to diseases that affect nerves outside the brain and spinal cord. It covers the types of peripheral nerves and classifications of neuropathies. Some key causes of peripheral neuropathy discussed include diabetes, nutritional deficiencies like B1/B12 deficiency, alcoholism, infections like HIV and leprosy, and certain drugs. Symptoms, clinical presentations, investigations, and treatment approaches for different types of peripheral neuropathies are also summarized.
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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.
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS), AKA "Lou Gehrig's Disease," is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
NEUROMYOTONIA : A COMPLETE REVIEW
Acquired neuromyotonia is an inflammatory disorder characterized by abnormal nerve impulses from peripheral nerves that cause continuous muscle fiber activity and symptoms like muscle stiffness, cramping and weakness. It has several potential causes including an unknown acquired cause, paraneoplastic syndrome related to cancer, or a hereditary form. Diagnosis involves identifying continuous muscle contractions and abnormal electrical muscle activity on electromyography. Treatment focuses on using anti-convulsant drugs to stop abnormal nerve impulses or plasma exchange to provide short-term relief from symptoms.
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.
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,
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This document discusses myasthenia crisis, which is a life-threatening condition defined as weakness from myasthenia gravis that requires intubation or delays extubation after surgery. It presents information on the epidemiology, pathophysiology, clinical presentation, diagnosis, and management of myasthenia crisis. Key aspects of management include admission to the intensive care unit, assessment of respiratory function, elective intubation if needed, rapid immunomodulating therapy, careful weaning, and monitoring for complications.
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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.
The hereditary motor sensory neuropathies
A 14-year-old girl presented with progressive weakness and deformities in her legs and hands since age 4. Examination found claw hands, foot drop, diminished sensation, and absent reflexes. She was diagnosed with Charcot-Marie-Tooth disease, a genetic disorder causing peripheral nerve damage. It results in muscle wasting and weakness over time. While most common in adults, it can begin in childhood. There is currently no cure, but treatment focuses on managing symptoms like foot drop and providing ankle support.
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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.
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.
Hereditary motor and sensory neuropathy
Hereditary motor and sensory neuropathy (HMSN), also known as Charcot-Marie-Tooth disease, is the most common inherited neuromuscular disorder. It is characterized by slowly progressive distal weakness and muscle atrophy in the legs and later in the hands. There are two major types - one with slow nerve conduction velocities associated with demyelinating neuropathy, and one with relatively normal nerve conduction velocities associated with axonal degeneration. Most families demonstrate autosomal dominant inheritance with a 50% chance of offspring being affected if a parent has the disease.
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This document discusses athetosis and dystonia. It defines athetosis as irregular, slow writhing movements, often of the extremities and fingers. Dystonia is defined as an abnormal sustained muscle contraction causing twisting movements and abnormal postures. The document describes the clinical presentations and patterns of movement seen in athetosis. It discusses the potential pathophysiology of athetosis involving lesions in the frontal lobes, parietal lobes, and putamen. Causes of athetosis in children and adults are provided. Dystonia is similarly defined and classified. Potential pathology, types, hereditary forms, and secondary causes of dystonia are outlined in detail.
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
Disorders of the neuromuscular junction
Disorders of the neuromuscular junction include Myasthenia gravis, Lambert-Eaton myasthenic syndrome, Botulism, Tetanus, Strychnine intoxication, Organophosphates poisoning and neuromyotonia. Pharmacology of the NMJ is also reviewed in brief.
Tabes dorsalis
Tabes dorsalis is a progressive degeneration of nerve cells and fibers in the spinal cord that carry sensory information to the brain, caused by untreated syphilis. It is characterized by sensory deficits, loss of coordination, and diminished reflexes. The disease progresses through preataxic, ataxic, and paralysis stages. Clinical features include loss of sensation, Argyll Robertson pupils, dementia, hypotonicity, loss of coordination, and trophic ulcers. Treatment involves antibiotics, steroids, pain medications, exercises, and splinting to manage symptoms.
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This document provides information about motor neuron disease (MND), including its types, clinical features, pathology, diagnosis, and treatment. It discusses four main types of MND: amyotrophic lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy, and primary lateral sclerosis. The pathology of MND involves degeneration of motor neurons in the brain and spinal cord. Diagnosis is based on involvement of upper and lower motor neurons and ruling out other conditions. Treatment focuses on exercises, positioning, bracing, and psychological support to maintain function and prevent complications as the disease progresses.
Approach to peripheral neuropathy
This document discusses peripheral neuropathy and provides guidance on evaluating and diagnosing peripheral nerve disorders. It defines peripheral neuropathy as disorders affecting the peripheral nervous system, which can involve sensory nerves, motor nerves, or both. The document outlines that peripheral neuropathies can be classified based on whether they primarily affect the cell body, myelin, or axon. It also lists common causes of peripheral neuropathy like diabetes, paraproteinemia, alcohol misuse, and vitamin B12 deficiency. The document provides guidance on clinical assessment, laboratory and electrodiagnostic testing, skin or nerve biopsy, and treatment approaches for peripheral neuropathy.
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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.
Polyneuropathy
Peripheral neuropathy refers to damage to peripheral nerves. There are three main types: mononeuropathy affecting a single nerve, mononeuritis multiplex affecting multiple nerves asymmetrically, and polyneuropathy affecting multiple nerves concurrently and symmetrically. Polyneuropathy can be classified as axonopathy, myelinopathy, or neuronopathy depending on whether the axons, myelin sheaths, or neurons are affected. Symptoms and signs include both negative symptoms like numbness and weakness as well as positive symptoms like tingling and pain. Evaluation involves taking a history and examining for patterns of onset, progression, fluctuations, and other systemic diseases. Diagnosis involves nerve conduction studies and sometimes nerve biopsies. Treatment focuses
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This document provides information about late responses in nerve conduction studies, including F-waves, H-reflexes, and axon reflexes. It discusses the mechanisms, characteristics, and clinical applications of each response. The F-wave results from antidromic stimulation of motor neurons and evaluates more proximal nerve segments. The H-reflex is a monosynaptic stretch reflex that is reliably measured in the lower limbs. Axon reflexes occur due to terminal nerve branching and suggest reinnervation. Late responses provide valuable information about radiculopathies and plexopathies that cannot be observed with distal nerve studies alone.
Multiple sclerosis
Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system characterized by breakdown of the myelin sheath covering nerve axons. It affects over 400,000 people in the US and more than 2.1 million worldwide. Genetic factors, autoimmunity, infection, vitamin D levels, and loss of protective childhood infections may play a role in MS etiology. Clinically, MS presents with a variety of neurological symptoms depending on the location of lesions in the brain and spinal cord, including visual, motor, sensory and cognitive impairments. Disease courses include relapsing-remitting MS, secondary progressive MS, primary progressive MS and progressive-relapsing MS.
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Scleroderma
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Dystonia
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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.
The hereditary motor sensory neuropathies
A 14-year-old girl presented with progressive weakness and deformities in her legs and hands since age 4. Examination found claw hands, foot drop, diminished sensation, and absent reflexes. She was diagnosed with Charcot-Marie-Tooth disease, a genetic disorder causing peripheral nerve damage. It results in muscle wasting and weakness over time. While most common in adults, it can begin in childhood. There is currently no cure, but treatment focuses on managing symptoms like foot drop and providing ankle support.
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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.
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.
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.
Hereditary motor and sensory neuropathy
Hereditary motor and sensory neuropathy (HMSN), also known as Charcot-Marie-Tooth disease, is the most common inherited neuromuscular disorder. It is characterized by slowly progressive distal weakness and muscle atrophy in the legs and later in the hands. There are two major types - one with slow nerve conduction velocities associated with demyelinating neuropathy, and one with relatively normal nerve conduction velocities associated with axonal degeneration. Most families demonstrate autosomal dominant inheritance with a 50% chance of offspring being affected if a parent has the disease.
Athetosis and dystonia
This document discusses athetosis and dystonia. It defines athetosis as irregular, slow writhing movements, often of the extremities and fingers. Dystonia is defined as an abnormal sustained muscle contraction causing twisting movements and abnormal postures. The document describes the clinical presentations and patterns of movement seen in athetosis. It discusses the potential pathophysiology of athetosis involving lesions in the frontal lobes, parietal lobes, and putamen. Causes of athetosis in children and adults are provided. Dystonia is similarly defined and classified. Potential pathology, types, hereditary forms, and secondary causes of dystonia are outlined in detail.
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
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Disorders of the neuromuscular junction include Myasthenia gravis, Lambert-Eaton myasthenic syndrome, Botulism, Tetanus, Strychnine intoxication, Organophosphates poisoning and neuromyotonia. Pharmacology of the NMJ is also reviewed in brief.
Tabes dorsalis
Tabes dorsalis is a progressive degeneration of nerve cells and fibers in the spinal cord that carry sensory information to the brain, caused by untreated syphilis. It is characterized by sensory deficits, loss of coordination, and diminished reflexes. The disease progresses through preataxic, ataxic, and paralysis stages. Clinical features include loss of sensation, Argyll Robertson pupils, dementia, hypotonicity, loss of coordination, and trophic ulcers. Treatment involves antibiotics, steroids, pain medications, exercises, and splinting to manage symptoms.
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This document provides information about motor neuron disease (MND), including its types, clinical features, pathology, diagnosis, and treatment. It discusses four main types of MND: amyotrophic lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy, and primary lateral sclerosis. The pathology of MND involves degeneration of motor neurons in the brain and spinal cord. Diagnosis is based on involvement of upper and lower motor neurons and ruling out other conditions. Treatment focuses on exercises, positioning, bracing, and psychological support to maintain function and prevent complications as the disease progresses.
Approach to peripheral neuropathy
This document discusses peripheral neuropathy and provides guidance on evaluating and diagnosing peripheral nerve disorders. It defines peripheral neuropathy as disorders affecting the peripheral nervous system, which can involve sensory nerves, motor nerves, or both. The document outlines that peripheral neuropathies can be classified based on whether they primarily affect the cell body, myelin, or axon. It also lists common causes of peripheral neuropathy like diabetes, paraproteinemia, alcohol misuse, and vitamin B12 deficiency. The document provides guidance on clinical assessment, laboratory and electrodiagnostic testing, skin or nerve biopsy, and treatment approaches for peripheral neuropathy.
Spondyloarthropathy
This document discusses spondyloarthritis (SpA), a group of inflammatory diseases that share features like axial joint inflammation, asymmetric oligoarthritis, and enthesitis. The main types of SpA are ankylosing spondylitis, psoriatic arthritis, undifferentiated spondyloarthritis, and reactive arthritis associated with inflammatory bowel disease. SpA is strongly associated with the HLA-B27 gene. Clinical features include inflammatory back pain, peripheral arthritis, enthesitis, dactylitis, and eye and bowel inflammation. Diagnosis involves assessing clinical features, lab tests like elevated CRP/ESR and HLA-B27 status, and imaging of the sacroiliac joints and spine
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.
Polyneuropathy
Peripheral neuropathy refers to damage to peripheral nerves. There are three main types: mononeuropathy affecting a single nerve, mononeuritis multiplex affecting multiple nerves asymmetrically, and polyneuropathy affecting multiple nerves concurrently and symmetrically. Polyneuropathy can be classified as axonopathy, myelinopathy, or neuronopathy depending on whether the axons, myelin sheaths, or neurons are affected. Symptoms and signs include both negative symptoms like numbness and weakness as well as positive symptoms like tingling and pain. Evaluation involves taking a history and examining for patterns of onset, progression, fluctuations, and other systemic diseases. Diagnosis involves nerve conduction studies and sometimes nerve biopsies. Treatment focuses
Late Responses (F-wave and H.Reflex)
This document provides information about late responses in nerve conduction studies, including F-waves, H-reflexes, and axon reflexes. It discusses the mechanisms, characteristics, and clinical applications of each response. The F-wave results from antidromic stimulation of motor neurons and evaluates more proximal nerve segments. The H-reflex is a monosynaptic stretch reflex that is reliably measured in the lower limbs. Axon reflexes occur due to terminal nerve branching and suggest reinnervation. Late responses provide valuable information about radiculopathies and plexopathies that cannot be observed with distal nerve studies alone.
Multiple sclerosis
Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system characterized by breakdown of the myelin sheath covering nerve axons. It affects over 400,000 people in the US and more than 2.1 million worldwide. Genetic factors, autoimmunity, infection, vitamin D levels, and loss of protective childhood infections may play a role in MS etiology. Clinically, MS presents with a variety of neurological symptoms depending on the location of lesions in the brain and spinal cord, including visual, motor, sensory and cognitive impairments. Disease courses include relapsing-remitting MS, secondary progressive MS, primary progressive MS and progressive-relapsing MS.
Multiple sclerosis rehab
This document discusses rehabilitation principles for multiple sclerosis (MS). It begins by defining MS as a chronic, progressive disease of the central nervous system characterized by demyelination of the brain and spinal cord. It then covers the epidemiology, pathogenesis, subtypes, common symptoms and signs, diagnosis using the McDonald criteria, disease severity as measured by EDSS, disease-modifying therapies, and approaches to managing common issues like gait impairment and fatigue through rehabilitation and exercise.
Scleroderma
Scleroderma is a multisystem collagen vascular disease characterized by fibrosis of the skin and internal organs. It results from endothelial cell injury, fibroblast activation, and immune system involvement. The two main types are limited cutaneous systemic sclerosis, which affects the skin of the hands and face, and diffuse cutaneous systemic sclerosis, which has more extensive skin involvement. Major organ manifestations include pulmonary fibrosis, gastrointestinal tract abnormalities, heart and kidney involvement. Treatment focuses on preventing further organ damage through immunosuppression and addressing specific organ system complications.
Dystonia
Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive movements and postures. It is caused by excessive force in the primary muscles used for movement along with overflow activation of additional muscles. Dystonia can have various etiologies and is often initiated or worsened by voluntary action. Treatment may involve botulinum toxin injections to reduce muscle overactivity.
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Neuromuscular Junction Disease
Neuromuscular junction diseases interfere with the transmission of signals from nerves to muscles and can be acquired or inherited. Myasthenia gravis is an acquired autoimmune disorder where antibodies induce acetylcholine receptor deficiency at the neuromuscular junction, causing weakness that fluctuates with activity. Symptoms are tested using drugs like edrophonium, and treatment includes anticholinesterases, immunosuppressants, thymectomy, and plasmapheresis. Lambert-Eaton myasthenic syndrome is another autoimmune condition where antibodies affect calcium channels, and is associated with lung cancer. Certain drugs can also induce myasthenic syndrome symptoms.
Myasthenia gravis
Myasthenia gravis is an autoimmune disorder characterized by varying degrees of weakness in voluntary muscles. It occurs when antibodies block or damage receptors at the neuromuscular junction, preventing normal muscle contraction in response to nerve impulses. Risk factors include female gender under 40, male gender over 60, and other autoimmune disorders. Symptoms include drooping eyelids, blurred or double vision, weakness in the face, and generalized weakness that worsens with activity. Diagnostic tests include the edrophonium test, blood tests for antibodies, repetitive nerve stimulation, and pulmonary function tests. Treatment involves cholinesterase inhibitors, corticosteroids, immunosuppressants, and in some cases plasmapher
Effects of LEMS on P/Q type Calcium Channels
The document discusses a study examining which calcium channel subtypes may compensate for the loss of P/Q-type calcium channels in tottering (tg) mice treated with plasma from Lambert-Eaton Myasthenic Syndrome (LEMS) patients. The study found increased expression of the N-type (α1B) and L-type (α1C) calcium channel subunits in the tg mice treated with LEMS plasma, suggesting these subtypes may compensate for the loss of the P/Q-type (α1A) channels. More trials are needed to confirm these results and calculate standard errors.
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Este documento describe tres patologías del sistema nervioso periférico: la neuropatía, el síndrome de Guillain-Barré y la miastenia gravis. La neuropatía es la forma más común de daño al sistema nervioso periférico y afecta principalmente las piernas y los pies. El síndrome de Guillain-Barré daña los nervios y causa debilidad muscular y parálisis ascendente. La miastenia gravis es una enfermedad autoinmune que causa debilidad muscular que empeora con la actividad y
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The neuromuscular junction disorders including myasthenia gravis
The document discusses the neuromuscular junction and myasthenia gravis. It describes the key parts of the neuromuscular junction including the pre-synaptic membrane, synaptic cleft, post-synaptic membrane, and contractile apparatus. It then discusses the pathophysiology of myasthenia gravis, including how antibodies decrease the number of available acetylcholine receptors and impair neuromuscular transmission. Common symptoms of myasthenia gravis are also summarized, such as weakness and fatigability of muscles that typically first involve the eyes, face, and throat.
Debilidad muscular ok
El documento describe varios tipos de miopatías inflamatorias. Se caracterizan por debilidad muscular, aumento de enzimas musculares y hallazgos inflamatorios en la biopsia muscular. Incluyen la polimiositis, dermatomiositis y miositis de cuerpos de inclusión, que se diferencian por sus manifestaciones cutáneas y patrones de afectación muscular.
DEBILIDAD MUSCULAR
El documento describe diferentes trastornos del sistema motor como la debilidad muscular, la hemiparesia, el desequilibrio, la ataxia de extremidades y los trastornos de la marcha. Estos trastornos pueden ser causados por disfunciones en la corteza cerebral, los ganglios basales, el cerebelo o la médula espinal. Se detallan varios tipos específicos de debilidad muscular, hemiparesia, alteraciones del equilibrio, ataxia y trastornos de la marcha como la marcha festinante, en tijeras
Neuromuscular Disorders
This document discusses several neuromuscular disorders including Amyotrophic Lateral Sclerosis (ALS), Multiple Sclerosis (MS), Spinal Cord Injury, Huntington's Chorea, and Spinal Muscle Atrophy. It provides details on ALS such as the pathophysiology, signs and symptoms, course of the disease, etiology, epidemiology, diagnosis, treatment, and complications. Comparisons are made between ALS and other disorders such as MS, Spinal Cord Injury, Huntington's Chorea, and Spinal Muscle Atrophy.
Enfermedades inmunitarias del sistema nervioso y del ojo
Este documento describe varias enfermedades inmunitarias del sistema nervioso y del ojo. Explica la esclerosis múltiple, una enfermedad desmielinizante crónica del sistema nervioso central causada por una respuesta inmune contra la mielina. También describe el síndrome de Guillain-Barré, una polineuropatía aguda inflamatoria que causa debilidad muscular progresiva. Por último, analiza la miastenia gravis y el síndrome miasténico de Lambert-Eaton, dos trastornos autoinmunes
Botulismo
O documento resume as causas, sintomas, diagnóstico e tratamento do botulismo, uma doença neuroparalítica grave causada pela ingestão de alimentos contaminados com a toxina produzida pela bactéria Clostridium botulinum. Os principais sintomas incluem visão dupla, fotofobia, ptose palpebral e tonturas. O diagnóstico é feito através de exames clínicos e detecção da toxina ou bactéria. O tratamento envolve procurar um médico imediatamente e seguir as in
Síndrome de Eaton Lambert. Patología de la placa motora y su tratamiento
El documento describe el síndrome de Eaton-Lambert (LEMS), un trastorno de la transmisión neuromuscular causado por autoanticuerpos contra los canales de calcio presinápticos. Esto causa una disminución en la liberación de acetilcolina y debilidad muscular proximal fluctuante, a menudo asociada con cáncer de pulmón de células pequeñas. El diagnóstico se basa en la clínica, pruebas electrofisiológicas que muestran facilitación muscular después del ejercicio y detección de anticuerpos.
Botulismo
O documento resume as principais informações sobre o botulismo, doença causada pela toxina produzida pela bactéria Clostridium botulinum. Apresenta os tipos de botulismo, sintomas, tratamento com antitoxina e a importância da prevenção por meio de boas práticas de conservação de alimentos.
Myasthenia gravis
Myasthenia Gravis is an autoimmune neuromuscular disorder characterized by muscle weakness and fatigability. It is caused by antibodies that block acetylcholine receptors at the neuromuscular junction, preventing muscle contraction. Symptoms vary widely and can include weakness of the eye muscles, facial muscles, limbs, and respiratory muscles. Diagnosis involves physical exams, blood tests to detect antibodies, and electrodiagnostic tests. Treatment options include acetylcholinesterase inhibitors, immunosuppressants, plasmapheresis, intravenous immunoglobulin, and thymectomy.
Botulismo
El Clostridium botulinum es una bacteria anaerobia que produce la neurotoxina botulínica, la cual causa el botulismo al ser ingerida. El botulismo se presenta con síntomas como dificultad para hablar, debilidad muscular progresiva y parálisis. Se trata con antitoxina y soporte respiratorio para prevenir un paro respiratorio.
Neuro Muscular Disorders
In this slideshow, we covered most of neuromuscular disorders which might face you in medicine in general and in pediatrics in particular.
We hope if you find this slideshow helpful for your seeking of this subject.
Cheers,
Neuromuscular junction
This document provides an overview of the neuromuscular junction. It defines the neuromuscular junction as the junction between the terminal branch of a nerve fiber and a muscle fiber. It describes the structure of the neuromuscular junction including the presynaptic and postsynaptic membranes separated by a synaptic cleft. It explains the process of neuromuscular transmission from the release of acetylcholine to the destruction of acetylcholine and the development of end plate potentials. It discusses neuromuscular blockers and stimulators and their uses. It also covers motor units, recruitment of motor units, and some applied physiology concepts like myasthenia gravis and Eaton-Lambert syndrome.
Miastenia gravis
1) El documento describe la historia de la miastenia gravis, desde las primeras descripciones clínicas en el siglo XVII hasta la identificación de los anticuerpos contra los receptores de acetilcolina en 1973. 2) Se detalla el descubrimiento de que los inhibidores de la acetilcolinesterasa producían mejoría en los pacientes, llevando a la sugerencia de que la debilidad se debía a una alteración en la función de la placa motora. 3) También se mencionan contribuciones como la descripción de la hiperplasia
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The neuromuscular junction disorders including myasthenia gravis
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Enfermedades inmunitarias del sistema nervioso y del ojo
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Síndrome de Eaton Lambert. Patología de la placa motora y su tratamiento
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Lambert–Eaton myasthenic syndrome ( lems)
- 1. Lambert-Eaton Myasthenic Syndrome Presented by Samit Islam Rudra
- 2. Mrs Johnson : 45years old
- 3. Symptoms Leg Weakness Climbing stairs difficulty Dropping Eyelids Dry Eyes Dry Mouth
- 4. Symptoms (cont.) Dizziness Swallowing problem Lifting Object Blurred Vision Erectile DIsfunction
- 5. After being attacked by LEMS
- 7. Main reason : Cancer cells
- 8. Epidemiology • LEMS is estimated to affect 4 to 10 cases per million people. • Europe :2,000 to 4,000 patients per million. • United States : 1,200 to 3,100 patients per million.
- 10. Mrs Johnson with the Doctors
- 11. We hope Mrs Johnson will come back to her normal life .