Neuropathies & Myopathies Dr. Dinesh T, Junior resident 1 Jipmer physiologist 06/06/2022
Nov 12, 2014
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Neuropathies & Myopathies
Dr. Dinesh T,
Junior resident
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Neuropathies
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Definition
Damage to nerves which may be caused either by diseases or trauma to the nerve or as a component of systemic illness
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• The neuropathy is a symptom of another disorder
• In most common forms of polyneuropathy, the nerve fibers most distant from the brain and the spinal cord malfunction first.
• Pain and other symptoms often appear symmetrically
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• The peripheral nerves include:Cranial nerves
(with the exception of the second)
Spinal nerve roots Dorsal root ganglia Peripheral nerve trunks and their terminal
branches Peripheral autonomic nervous system
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Symptoms in neuropathy
A wide array of symptoms can occur when nerves are damaged
o Paresthesia o Sensitivity to touch,
PositivePins and needlesTinglingBurning
NegativeNumbnessDeadnessAs if wearing shocks and walk
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In chronic course symptoms worse, muscle wasting, paralysis, or gland dysfunction
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Neuropathy - Signs
• Distal sensory loss• Distal weakness and
atrophy• Decreased or absent
reflexes– Ankle jerks lost first
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Various classifications
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Etiological Classification of neuropathies
Hereditary Neuropathies– Hereditary motor and sensory
neuropathy – type I– HMSN – Type II– Dejerine – Sottas Neuropathy HMSN- type III– HSMN – type IV– HSMN – type V
Inflammatory neuropathies• Immune mediated
o Guillain-Barré syndromeo Chronic inflammatory demyelinating
polyradiculoneuropathy• Infectious
– Leprosy – Diphtheria– Varicella – zoster
• Acquired metabolic and toxic neuropathies– Peripheral neuropathy in adult onset Diabetes– Metabolic and nutritional peripheral neuropathies – Neuropathies associated with malignancy– Toxic neuropathies
• Traumatic neuropathies
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Medications Causing Neuropathies
o Axonal
Vincristine Paclitaxel Nitrous oxide Colchicine IsoniazidHydralazine Metronidazole Pyridoxine Didanosine Lithium Alfa interferon Dapsone
Phenytoin Cimetidine Disulfiram Chloroquine Ethambutol Amitriptyline
o Demyelinating Amiodarone Chloroquine Suramin Gold
o Neuronopathy Thalidomide Cisplatin Pyridoxine
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Pathophysiological classification
• Motor , sensory, or autonomic• Mononeuropathy , polyneuropathy or
mononueritis multiplex• Focal, multifocal or symmetric • Proximal or distal • Axonal, demyelinating or both• Acute, sub acute or chronic
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• Some neuropathies may affect all three types of nerves, others primarily affect one or two types.
• Predominately motor neuropathy• Predominately sensory neuropathy• Sensory-motor neuropathy• Autonomic neuropathy
• Impaired function and symptoms depend on the type of nerves that are damaged.
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• Mononeuropathy involve damage to only one nerve
• When multiple nerves supplying one limb are affected-called polyneuropathy.
• Two or more isolated nerves in separate areas of the body are affected-called mononeuritis multiplex
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o Focal neuropathies include common compressive neuropathies such as carpal tunnel syndrome, ulnar neuropathy ,peroneal neuropathy
oMultifocal neuropathy suggests a mononeuritis multiplex that may be caused, for example, by vasculitis or diabetes
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Axonal degeneration
• Primary destruction of the axon with secondary degeneration of its myelin sheath
• Generalized abnormality in the neuron cell body- neuronopathy
• Abnormality in the axon - axonapathy
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Segmental demyelination
Dysfucntion of Schwann cell or damage to the myelin sheath
Denuded axon provide signal for remyelination
Precursor cells within endoneurium replace injured cells
Cells proliferate and engulf axon.= remyelination in time
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Neurophysiological classification
• Uniform demyelinating sensorimotor poly neuropathy• Segmental demyelinating, motor more than sensory
neuropathy• Axonal , motor more than sensory polyneuropathy• Axonal sensory polyneuropathy• Axonal mixed sensorimotor polyneuropathy• Mixed axonal and demyelinating sensorimotor
polyneuropathy
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Uniform demyelinating sensorimotor poly neuropathy
• Hereditary motor sensory neuropathy- type I, III,IV
• Leucodystrophies• Tangier disease• Cockayne syndrome• Congenital cerebrotendinous xanthomatosis• Congenital hypomyelinating neuropathies
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HMSN I (Charcot- Marie- Tooth I)
• HSMN I – AD is the most common hereditary neuropathy.
• CMT-I A chromosome 17p11 , CMT-IB chromosome 1q22 , CMT-IC 16p13, chromosome , CMT-IX chromosome Xq13.1
• Slowly progressive distal weakness • Foot deformity, areflexia , distal sensory loss• Upper limb ataxia, tremor, peripheral n
hypertrophy
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Neurophysiological features
• Conduction velocity less than 25% of lower limit• Median motor forearm conduction< 38 m/s• Uniform NCV changes in adjacent nerves• Absence of conduction block and temporal
dispersion• F response• Needle EMG shows minimal fibrillations in distal
muscles.
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Electrophysiological studies ( NCS ) showo Uniform slowing of NCVo Similar NCV slowing in adjacent nerveso Absence of conduction block and temporal
dispersiono Prolongation of F response commensurate with
NCV slowing
Uniform demyelinating sensorimotor poly neuropathy-
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Segmental demyelinating motor more than sensory neuropathy
o Acute inflammatory demyelinating poly radiculo neuropathy AIDP
o Chronic inflammatory demyelinating poly radiculo neuropathy CIDP
o Multifocal motor neuropathy o Paraproteinemiao HIV neuropathyo Lyme diseaseo Diphtheria o Penicillamine
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AIDP
o The prototypeo Distal paresthesia with symmetric weaknesso Distal areflexiao Variants are pure motor, pure sensory, autonomic,
relapsing, and Miller fisher typeso Cranial nerves esp facial n and bulbar may be involvedo Respiratory muscles are severely involved in about 25
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Pathophysiology of GBS
• Pathological findings include inflammatory and demyelinating changes.
• Monocytes and macrophages appear to attack myelin sheaths.
• Myelinated fibers show segmental demyelination during the first few days. Segmental remyelination occurs subsequently.
• The lesions have a perivenular distribution
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Chronic inflammatory demyelinatingpolyneuropathy
• Chronic progressive or relapsing neuropathy, motor > sensory.
• Electrophysiology: slow conduction velocity & conduction block
• Pathology: segmental demyelination and remyelination, onion bulbs, fibrosis and little or no lymphocytic
infiltration of tissue.
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Segmental demyelinating motor more than sensory neuropathy
Nerve conduction studies o Slowing of motor and sensory conduction
velocityo Prolongation of terminal latencyo Conduction blocko Dispersion and prolonged or absent F waves
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Axonal, motor more than sensory neuropathy
• Axonal type of GBS• Acute intermittent porphyria• HSMN type II , V• Toxic neuropathies such as lead, dapsone • Paraneoplastic syndrome • Metabolic – hypoglycemia• Critical care neuropathy
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• Distal symmetric weakness and wasting with minimal sensory loss
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Axonal,motor more than sensory neuropathy
• Nerve conduction studies• Reduced CMAP amplitude• NCV is normal• SNAP amplitudes are also decreased• Fibrillations appear in distal muscles
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Sensory axonal polyneuropathy
• Diabetic neuropathy• Carcinomatous sensory neuropathy• HSMN type I- IV• Friedrich ataxia• Abetalipoproteinemia • Toxins - cisplatin• Pyridoxine overdosage• Vt –E neuropathy• Malabsorption• Acromegaly,
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Diabetic neuropathy
• Onset of neuropathy depends upon the duration of illness
• 50% diabetics have peripheral neuropathy of which 80% have had the illness for >15 years
• Distal symmetric sensory or sensorimotor, autonomic, focal or multifocal asymmetric
• Symmetric neuropathy involves distal sensory , motor nerves .
• Decreased sensation, loss of pain sensation – ulcer
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Diabetic neuropathy
• Predominant pathology is axonal neuropathy.• In chronic cases segmental demyelination also seen
Pathophysiology –• Loss of small myelinated fibers and unmyelinated
fibers. But large fibers can also be affected.• Endoneurial arterioles show thickening, hyalination,
intense PAS positivity in the walls and extrensive reduplication basement membrane
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Sensory axonal polyneuropathy
• Nerve conduction studies • Diminished or absent SNAP amplitude in
the setting of normal motor nerve conduction velocity
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Axonal type of mixed sensorimotor neuropathy
• Nutritional deficiencies (vitamin deficincy, alcoholism)• Metabolic ( diabetic, uraemia, liver disease,
amyloidosis)• Connective tissue disorders (rheumatoid arthritis,
SLE, PAN)• Multiple myeloma• Carcinoma• Cryoglobunemia• Heavy metals – lead, arsenic, gold, mercury• Drugs- metronidazole, phenytoin etc
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• Paresthesia and dyesthesia of feet and distal legs
• Wasting is marked• Loss of ankle reflex • Pathophysiology – evidence of
degeneration of distal portion of axons
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Axonal type of mixed sensorimotor neuropathy
Nerve conduction studies• Reduced or absent SNAP • CMAP amplitude decreases and motor
conduction velocity also decrease in later stage
EMG• Fibrillations and positive sharp waves are
prominent in distal muscles.• Temporal dispersion on proximal stimulation is
not found as in demyelinating neuropathies
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Mixed axonal loss and demyelinating neuropathy
• Diabetes • Uremia• Paraproteinemia
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• Paresthesia, dyesthesia or numbness• Reduced vibration and two point
discrimination• Pathophysiology – segmental
demyelination and remyelination along with axonal degeneration
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Mixed axonal loss and demyelinating neuropathy
• Nerve conduction studies • Reduced or unrecordable CMAP, SNAP or
both• Moderate to severe slowing of NCV with
temporal dispersion of CMAP
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Clinical examination
• Thorough history and physical examination is needed.
• Cranial nerve examination • Motor , sensory, autonomic nervous system
examination• Fundus examination• Lymphadenopathy , hepatomegaly or
splenomegaly, and skin lesions
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Lab tests:
• CBC, electrolytes, ESR• Fasting serum glucose, glycosylated hemoglobin, blood
urea nitrogen, creatinine,• Liver , kidney,, thyroid function studies• Inflammatory markers, • Total protein level • Vit D, B12, • Cytology• CSF • Urinalysis • Nerve biopsy
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Electrophysiologic studies
• EMG and nerve conduction studies (NCS) are often the most useful initial laboratory studies in the evaluation of a patient with peripheral neuropathy
• Confirm the presence of a neuropathy • Provide information as to the type of fibers involved
(motor, sensory, or both), the pathophysiology (axonal loss versus demyelination) and a symmetric versus asymmetric or multifocal pattern of involvement.
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• The limitations of EMG/NCS. – There is no reliable means of studying proximal
sensory nerves. – NCS results can be normal in patients with small-fiber
neuropathies– Lower extremity sensory responses can be absent in
normal elderly patients.
• EMG/NCS are not substitutes for a good clinical examination.
Electrophysiologic studies
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Treatment
• The goal of treatment is to manage the underlying condition causing the neuropathy and repair damage, as well as provide symptom relief.
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Treatment
• Medical management– Analgesics . – antiepileptic drugs, including gabapentin, phenytoin,
and carbamazepine– some classes of antidepressants, including tricyclics
such as amitriptyline. – Mexiletine– local anesthetics such as lidocaine or topical patches
containing lidocaine – Codeine/oxycodone
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• Mechanical aids can help reduce pain and lessen the impact of physical disability.
– Hand or foot braces can compensate for muscle weakness or alleviate nerve compression.
– Orthopedic shoes can improve gait disturbances and help prevent foot injuries in people with a loss of pain sensation.
• If breathing becomes severely impaired, mechanical ventilation can provide essential life support.
Treatment
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• Surgical intervention often can provide immediate relief from mononeuropathies caused by compression or entrapment injuries.
– Repair of a slipped disk can reduce pressure on nerves where they emerge from the spinal cord; the removal of benign or malignant tumors can also alleviate damaging pressure on nerves.
– Nerve entrapment often can be corrected by the surgical release of ligaments or tendons.
Treatment
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Myopathies
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Definition
Neuromuscular disorders in which the primary symptom is muscle weakness due to dysfunction of muscle fiber
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Introduction
• Worldwide incidence of all inheritable myopathies is about 14%
• Overall incidence of muscular dystrophy is about 63 per 1 million.
• Worldwide incidence of inflammatory myopathies is about 5–10 per 100,000 people. More common in women
• Corticosteroid myopathy is the most common endocrine myopathy and endocrine disorders are more common in women
• Incidence of metabolic myopathies – increasing
Myopathy: symptoms
• Muscle pain and fatigue; exercise intolerance
• Proximal and symmetric weakness– Waddling gait; difficulty of rising from sitting,
climbing stairs; Gower’s sign– Hyperextension of the knee– Increased lordosis of the lumbar spine,
scoliosis– Contractures, tight Achilles tendons
• Myopathic face• Muscle atrophy; pseudohypertrophy• Myotonia• Tendon reflexes are normal or depressed
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Clinical examination
• Thorough clinical examination!• Observation – look for muscle
atrophy, deformities• Strength testing • Functional testing
– Stand up from a chair– Walk– Step up on a low stool
• REFLEXES and SENSATION
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Types of muscle diseases
• Hereditary muscle diseases– Denervation atrophy– Muscle dystrophies– Muscle channelopathies– Mitochondrial myopathies– Metabolic myopathies
• Acquired muscle diseases– Inflammatory myopathies– Endocrine and toxic myopathies– Infectious muscle diseases
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Myopathic Disorders
• Inflammatory Myopathies– Polymyositis– Dermatomyositis– Inclusion body myositis– Viral
• Muscular dystrophies– Duchenne muscular – Limb-girdle– Congenital– Fasioscapulohumeral – Oculopharyngeal – Emery – Dreifuss – Distal (Welander)
• Myotonic Syndromes– Myotonic dystrophy – Inherited– Schwarz-Jampel– Drug-induced
• Congenital myopathies– Central core disease– Nemaline myopathy– Myotubular– Fiber-type disproportion
• Metabolic myopathies– Glycogenoses– Mitochondrial– Periodic paralysis
• Endocrine myopathies– Thyroid– Parathyroid– Adrenal/steroid– Pituitary
• Drug-induced/toxic
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Diagnostic histological featuresof myopathies
• Absence of neurogenic abnormalities• Necrotic muscle fibers• Basophilic (regenerating) myofibers• Fibrosis of the endomysium• Special pathological features (inflammatory
cells, ragged red fibers etc.)
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Muscle dystrophies
• Hereditary myopathies, characterized by progressive weakness and muscle atrophy
• Genetic defect of proteins constituting the sarcolemma-associated cytoskeleton system
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Duchenne muscular dystrophy
• First described in 1881- dystrophin gene discovered in the early 1980's
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Duchenne muscular dystrophy• X-chromosome linked, recessive inheritance• 1 in 3500 live births,
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Clinical features
• Onset of weakness before age 5• Progressive weakness, proximal>distal,
and muscle wasting• Gower’s sign • Hypertrophy of calves,• psuedohypertrophy of deltoid, gluteal• Skeletal deformities• Cardiomyopathy• wheel chair dependence by the age of 12,
respiratory infection at 16-18 years. Fatal in the third decade
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Electrophysiology
• EMG changes – rate of muscle fiber destruction and extent of regeneration.
• Fiber loss-Low amplitude short duration MUPs,• Fiber degeneration- polyphasic MUPs • Necrosis - fibrillations with low amplitude and
short duration• Nerve conduction studies – generally normal
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• Elevated CPK levels to 20 to 100 folds• Variation in size and shape of muscle fibers and
small groups of necrotic and regenerating fibers- muscle biopsy.
• Absence of dystrophin gene in biopsied muscles or genetic defect analysis in WBCs
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Management
• No specific treatment• Physiotherapy• Aerobic exercise• Low intensity anabolic steroids• Prednisone supplements • Orthoses (orthopaedic appliances used for
support) • Orthopaedic surgery • Critical care
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Beckers muscular dystrophy
• Allelic defect in DMD gene.• 10 times less frequent than DMD• Better prognosis. Patient lives upto 40-50 years.• Mental retardation and heart failure can occur • Muscle biopsy – variable muscle fiber size with
aberrant large fibers. Endomysial fibrosis and fatty infiltration
• Patchy staining of DMD gene
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ge
Gene Clinical feature Pathophysiology
Fascioscapulohumeral MD - AD FSHMD1B Progressive muscular weakness and atrophy involving the face, scapular, proximal arm and peroneal muscles myopathic face,
Dystrophic myopathy with inflammatory infiltrates
Oculopharyngeal MD - AD PABP2 Ptosis and extra ocular muscle weakness
Dystrophic myopathy incl rimmed vacuoles
Emery – Dreifuss MD – X - linked EMD, LMNA Triad of early contracture, humero- peroneal weakness and cardiac conduction defects
Mild myopathic changes. Absent emerin by immunohistochemistry
Congenital –MD AR Laminin alpha 2
Neonatal hypotonia , muscle weakness
Variable fiber size and extensive endomysial fibrosis
Congenital –MD – Fukuyama type AR Fukutin Neonatal hypotonia and MR
Variable fiber size and extensive endomysial fibrosis. CNS malformation
Congenital –MD Walker – warnburg type
Protein o mannosyl transferase
Neonatal hypotonia and MR, ocular malformation
Variable fiber size and extensive endomysial fibrosis. CNS, ocular malformation
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ge
Gene Clinical feature Pathophysiology
Limb-girdle dystrophiesSarcoglycanopathies AR
Α, β, γ, δ sarcoglycans
α, β, γ, δ sarcoglycans
Starts between 2 and 20 yearsClinically indistinguishable from duchenne-dystrophyNo cardiac involvement
,
Normal dystrophin immunostaining, abnormal immunostaining with sarcoglycansGenetic defect analysis
Myotonic dystrophy AD CTG repeat expansion in a gene on chr. 19
• Myotonia: hyperexcitability of muscle membrane inability of quick muscle relaxation
• Progressive muscular weakness and wasting, most prominent in cranial and distal muscles
• Cataracts, frontal balding, testicular atrophy
• Cardiac abnormalities, mental retardation
Muscle biopsy showing mild myopathic changes and grouping of atrophic fast fibres
Myotonia congenita AD, AR Mucle cl geneAutosomal dominant form: Thomsen, autosomal recessive form: Becker
Myotonia (hyperexcitability of the muscle membrane): muscle stiffness and abnormal muscle relaxation, warm-up phenomenonHypertrophied muscles
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Inflammatory myopathies
PATHOPHYSIOLOGY• Polymyositis and inclusion body myositis (IBM) have
autoaggressive CD8 lymphocytes that appear to attack myofibers and suggest an autoimmune role.
• However,a major question exists about the etiology of IBM.
• Dermatomyositis is thought to be caused by auto antibodies, possibly targeting an antigen of the endothelium. Fiber injury may be caused by ischemia.
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Dermatomyositis Polymyositis Incusion body myositis
Sub acute progressive weakness
Sub acute progressive weakness
Slowly progressive weakness,
proximal>distal proximal>distal proximal and distal.
Children and adults, women
adults, women adults, mostly men
Characteristic rash and periorbital heliotrope.
Electromyogrammyopathic potentials, spontaneous
myopathic potentials, spontaneous
myopathic potentials, spontaneousactivity
Elevated serum creatine kinase activity.
Elevated serum creatine kinase activity
Mildly elevated serum creatine kinase or normal.
inflammatory myopathy affectingchiefly the perimysium with perifascicular atrophy.
inflammatory myopathy chiefly the endomysium
: inflammatory myopathy affectingchiefly the endomysium, but chronic and hasrimmed vacuoles and amyloid inclusions
Usually respond to glucocorticoids or IVGG.
respond to glucocorticoids does not respond to glucocorticoids.
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Polymyositis Inclusion body myositis Dermatomyositis
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Congenital myopathies
• Group of muscle disorders • Early onset• Slowly progressive• Hereditary • Generalised or proximal weakness and wasting• Hypotonia• Contractures• Normally or mildly elevated CPK • Normal or myopathic EMG
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Congenital myopathies
Central core disease Nemaline myopathy
Myotubular myopathy
Inheritence AD AD , AR XL, AD, AR
Gene RYR- 1 gene AD –NEM1 -TMP3AR- NEM2 - NEBAR- NEM3 - ACTAAR- NEM4 – TMP2AR- NEM5 - TNNT1AR- NEM7 - CFL2
XL – MTM1 AD – DNM2AR – BIN1
Clinical features Early onset hypotonia and weakness . Floppy infant . Associated skeletal deformities
Chilhood weakness .variable presentation. Floppy infant
Severe congenital hypotonia. Floppy infant .poor prognosis
Pathophysiology Cytoplasmic cores are distinct from surroundingSarcoplasm.
Aggregates of subsarcolemmal spindle shaped rods
Abundance of centrally located nuclei involving the majority of muscles
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Distal myopathies
• Welander distal myopathy• Miyoshi distal myopathy
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Metabolic myopathies
• Disorders of muscle energy metabolism• Disorders of lipid metabolism• Mitochontrial myopathies
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Mitochontrial myopathies
• Kearn – sayre syndrome• Myoclonic – epilepsy with ragged red fibers• Mitochontrial myopathy, lactic acidosis, stroke• Mitochontrial myopathies with recurrent
myoglobunuria• Mitochontrial DNA depletion syndrome• Progressive external opthalmoplegia and ragged
red fibers
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Endocrine myopathies
• Thyrotoxic myopathies• Cushing syndrome and steroid myopathy• Myopathy associated with oarathyroid disorders.
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Toxic myopathies
• Myotonic disorders• Necrotizing myopathies• Acute muscle necrosis• Mitochontrial myopathy• Hypokalemic myopathy• Inflammatory myopathy• Autophagic myopathy• Focal myopathy• Envenomation myopathy
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Muscle channelopathies
Nachannelopathies
Clchannelopathies
Cachannelopathies
Hyperkalemicperiodic paralysis
Myotoniacongenita(Thomsen andBecker type)
Malignanthyperthermia
Paramyotoniacongenita
Hypokalemicperiodic paralysis
Potassiumaggravatedmyotonia
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Treatment
o There is no single treatment for myopathy. o Treatment of the symptoms to specific cause-
targeting treatments. o Drug therapy o Physical therapy o Bracing for support, o Surgery o Massage
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Care !….the best rehabilitation method
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