Peripheral nerve and muscular

weakness/disorders
 Peripheral neuropathy
• Peripheral nerves are composed of sensory,
motor, and autonomic elements.
• Most are mixed carrying all the modalities
• Diseases can affect the cell body of a neuron or
its peripheral processes, namely the axons or the
encasing myelin sheaths
• Symptoms include
– fasciculations, weakness ,numbness dysesthesias
(burning/tingling),pain
– Autonomic dysfxn (orthostasis, bowel/bladder
retention/incontinence, impotence)
 etiology
• Demyelinating/inflammatory disease
– e.g acute like Guillen Barre syndrome,chronic
inflammatory polyradiculopathy
– Symmetrical proximal and distal weakness with
sensory loss
• Systemic diseases eg diabetes,drugs like isoniazid
etc produce symetrical distal sensory loss with or
without weakness
• Hereditary eg charcot marrie tooth
• Autoimmune disease eg rheumatoid,sjogrens
,SLE
 DIAGNOSIS
• complete blood count and (ESR),
• basic chemistries including serum electrolytes
and tests of renal and hepatic function,
• FBS, HbA1c,
• urinalysis, thyroid function tests
• B12, folate,
• rheumatoid factor, antinuclear antibodies (ANA),
• serum protein electrophoresis (SPEP), and urine
for Bence Jones protein
• Nerve or skin biopsy
• Medical conditions like uremea,liver disease
• HIV,HBV,HCV associated polyneuropathy
• Anti-retroviral associated neuropathy eg
didanosine
• In malignancy-paraneoplastic or infltration
• Other drugs-chemtherapy like
paclitaxel,antibiotics like
metronidazole,nitrafurnatoin,isoniazid
• Toxins eg lead,copper poisoning
• Nutritional neuropathies-VITE,B12,B1,B6,B3
 MANAGEMENT
• Diagnosis is by nerve conduction and
electromyelography
• Diagnosis and specific management eg proper control
of diabetes
• Replacement eg B12 in deficiency
• Removal of insult eg drugs
• Drugs for neuropathic pain
– Tricyclic antidepressants like armitrptilline 25mg
– Anticonvulsants eg gabapentin,pregabalin,carbamazepine,
– Opiod Analgesic-tramadol
– Other Antidepressant eg duloxetine etc
 GUILLAIN BARRE SYNDROME/GBS
• an acute, frequently severe, and fulminant
autoimmune polyradiculoneuropathy
• Precipitants:
– viral illness (EBV, CMV, HSV, HIV)
– , URTI (Mycoplasma),
– gastroenteritis (Campylobacter),
– Surgery
– older immunizations
 CLINICAL PRESENTATION
• Ascending paralysis over hours to days accompanied or
preceded by Sensory dysesthesias and numbness
• Respiratory failure requiring ventilatory assistance
occurs in 30%;
• Hypoactive then absent reflexes
• autonomic instability and arrhythmias occur in 50%
• Sensory loss eg pain,temperature is mild
• • Fisher variant: ophthalmoplegia, ataxia, areflexia;
associated with anti-GQ1b antibodies
• Clinical worsening plateaus at 4 weeks
 immunopathogenesis
• Some evidence that immune responses to non self
antigens (infectious agents, vaccines) misdirect to host
nerve tissue through a resemblance-of-epitope
(molecular mimicry) mechanism
• targets are likely to be glycoconjugates, specifically
gangliosides
• Autoantibodies are developed to various nerve
elements
• flaccid paralysis and sensory disturbance is conduction
block from demyelination.axon conduction remaon
intact thus recovery is quick
 diagnosis
• Diagnosis is largely clinical recognizing the
pattern of rapidly evolving paralysis with
areflexia, absence of fever or other systemic
symptoms, and characteristic antecedent
events
• CSF-marked high CSF protein i.e 100-
1000mg/dl without increased cells
• Electrodiagnosis-nerve conduction studies
 treatment
• Intravenous immunoglobulin or
immunopharesis
• Mechanical ventilation when needed
 Myasthenia gravis
• is a neuromuscular disorder characterized by weakness
and fatigability of skeletal muscles.
• decrease in the number of available acetylcholine
receptors (AChRs) at neuromuscular junctions due to
an antibody-mediated autoimmune attack.
• postsynaptic folds are flattened, or "simplified.
• Result of these is reduced neuromuscular transmission.
ACh is released normally but it produces small end-
plate potentials that may fail to trigger muscle action
potentials.
• Failure of transmission at many neuromuscular
junctions results in weakness of muscle contraction.
 Clinical features
• cardinal features are weakness and fatigability of muscles.
• Weakness increase with repeated use(fatigue) or late in the
day and may improve after rest/sleep
• Infections and systemic disorder can worsen
• Distribution of muscle weakness is disticnt with lid and
extrocular muscle early on with diplopia and ptosis are
common
• Weakness in chewing after prolonged effort eg meat
• Difficult in swallwoing,nasal voice,
• Limb involvement-proximal,maybe asymetric
• Reflexes are intact and so is sensation
 laboratory
• anti-AChR antibodies are detectable in the
serum of 85% of all myasthenic patients but in
only about 50% of patients with weakness
confined to the ocular muscles.
• Electrodiagnostic-electromyography.
• Anti-cholinesterase test also tensilon test.
 treatment
• Acetylcholinesterase pyridostigmine
• Thymomectomy
• Immunosuppressive
• Glucocorticoids-start low, titrate gradually