NEUROMUSCULAR AND PERIPHERAL NERVE DISORDERS

Steven Arkin M.D. St. Lukes Hospital Kansas City, MO

Case 1: 72 year old diabetic woman

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2 years progressive numbness and tingling in the feet Occasional sharp foot pain, especially at night Staggers frequently Mild weakness, sometimes trips on rugs Insulin requiring for 10 years slowed nerve conductions with low amplitudes

Peripheral Neuropathy
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Weakness or sensory loss or both based on nerve injury Generally distal symptoms, legs before arms, but there are exceptions Mostly symmetrical but can be asymmetric or focal Small fiber - diminished pain/temperature, preserved strength, reflexes Large fiber - loss position, vibration touch/pressure, areflexia

“dying back” Reversing the inciting event leads to regeneration Insidious onset. slow recovery Stocking glove sensory and motor loss .Axonal Neuropathy       Metabolic changes within the axon Failure of axonal transport leads to distal nerve degeneration Degeneration than proceeds proximally at a slow pace .

slowed conduction. elevated CSF protein .Demyelinating Neuropathy  Primary destruction of the myelin sheath      Axons remain intact throughout or degenerate later Muscle generally does not denervate Faster onset/recovery than axonal Mild sensory loss with prominent muscle weakness which can start proximally Absent reflexes.

Guillain Barre. B12 Infection . B6.HIV Immunologic .Neuropathy .Charcot Marie Tooth Monoclonal Gammopathy Neoplastic . Porphyria Deficiencies . Uremia.Drugs and Environmental Toxins Metabolic . Chronic inflammatory Collagen vascular Hereditary .Diabetes. Endocrine.Etiology          Toxic .Thiamine.direct infiltration or paraneoplastic disorder .

“pupil sparing” Focal entrapments or ischemic neuropathies Amyotrophy .proprioception. distal weakness later Cranial nerve dysfunction. feet before hands Absent reflexes early. especially extraocular. vibration and light touch Small fiber .impaired pain/temperature Autonomic .cardiovascular and GI (gastroparesis) Most commonly presents with slow onset numbness and tingling then pain in distal extremities.asymmetrical proximal leg weakness and pain .Diabetic Neuropathy         Large fiber .

3 white blood cells Respiratory: Forced vital capacity 2 Liters .Case 2: 24 year old man        4 weeks progressive weakness both legs Hands and feet ache early in the course No bowel or bladder symptoms Preceded by mild respiratory illness Exam: prominent leg and mild hand weakness. no reflexes Lumbar puncture: elevated protein.

ophthalmoparesis in 10% .blood pressure lability and bradycardia or tachycardia Cranial nerve abnormalities .1      Weakness.Guillain Barre Syndrome .facial diparesis. usually preceded by distal paresthesia 67% have previous URI or GI infection Impaired breathing. areflexia. requiring intubation in 25-50% Autonomic dysfunction may be prominent .

Guillain Barre Syndrome .ophthalmoparesis.DVT prophylaxis. areflexia . ataxia.plasma exchange or IV immunoglobulin 5% mortality. early intubation Limit complications . 10% severe residual Poor prognosis .clinical history/exam. early physical therapy Miller .advanced age. CSF and EMG Elevated spinal fluid protein without increased white count Conduction slowing or block on EMG Treat acutely .Fisher variant .2         Diagnosis . cardiopulmonary monitoring.

slowed conduction velocities Treat . plasma exchange.steroids. IV Immunoglobulin . evolving > 8 weeks Rarely involves cranial nerves Follows infection in only 30% Progressive (66%) or stepwise/progressive (34%) Increased spinal fluid protein.Chronic Inflammatory Demyelinating Polyneuropathy       Sensory and motor deficits.

Taxol Other drugs . ddI. thallium. lead.Phenytoin.Metronidazole.Cis-Platinum.Hexane. ddC Chemotherapy .partly due to nutritional deficiency (thiamine) . Vincristine. arsenic Alcohol . Disulfiram. Dapsone Environmental .Toxic Neuropathies      Antimicrobials . Nitrofurantoin.

Methotrexate .weekly oral dosing . allow > 3 months to work .vasculitic. chronic inflammatory.for neuropathies associated with IgM or IgG proteins.Guillain Barre.Azathioprine .Plasma exchange .Neuropathy Treatment .1  Immune modulation .4 gm/kg/d x 5 days .Steroids . 0.steroid sparing. chronic inflammatory . Guillain Barre.Intravenous Immunoglobulin . 3-5 days .

Phenytoin. serotonin reuptake inhibitors .mexilitine.Antidepressants . capsaicin.Anticonvulsants .2  Symptomatic for pain . carbamazepine. gabapentin .Neuropathy Treatment .tricyclics. some low dose neuroleptics .Others .

Associated Neuropathy   Early onset .CSF protein elevation WITH increased white cells and seropositivity Late onset .Acute inflammatory demyelinating neuropathy .multiple mononeuropathies with cranial nerves involved .chronic distal sensory polyneuropathy .Chronic inflammatory demyelinating neuropathy .CMV polyradiculoneuropathy .HIV .

paraneoplastic. immunofixation.Chronic inflammatory demyelinating . glucose.BUN/Cr.Inherited . ESR Of the rest: .DM.13% .still idiopathic . dysproteinemia .42% .24% . toxic.Neuropathy Workup   Routine evaluation yields diagnosis in 50% of cases . B12. thyroid.21% .

positive Tinel’s at wrist.Case 3: 34 year old man       2 years numbness in both hands Numbness increased while preparing PowerPoint slides Pain/tingling awaken him at night Shaking the hands relieves the symptoms No proximal complaints. Phalen’s sign . legs normal Exam: weak thumb abduction.

Carpal Tunnel Syndrome . burning in hands and fingers Pain in anterior wrist . relieved by shaking Weakness in thenar muscle group with atrophy late Tinel’s . tingling.forced flexion of wrist reproduces symptoms .tapping wrist elicits symptoms Phalen’s .1        Entrapment of median nerve at the wrist Numbness.might radiate into anterior forearm Pain/paresthesias prominent at night.

underlying cause. splint.diabetes .pregnancy .clinical. surgery . injection.2     Bilateral in 40% Associated with the following .myxedema .rheumatoid arthritis and other collagen vascular disease Diagnosis . slow nerve conduction across wrist Treat .Carpal Tunnel Syndrome .frequent overuse of the wrist or direct injury .

knee at fibular head Tibial .elbow (cubital tunnel) or wrist Radial .wrist drop (Saturday night palsy) Femoral .hip Lateral femoral cutaneous .foot drop .ankle (tarsal tunnel) .Other Common Entrapment Neuropathies       Ulnar .meralgia paresthetica Peroneal .

loss of nasolabial fold on the right.Case 4: 57 year old woman     Awoke with right facial weakness . normal taste. intact corneal reflex .unable to smile properly or close her right eye Pain behind the right ear Decreased sensation right side of face with sense of swelling Exam: unable to close right eye.

taste abnormal (25%). abnormal tearing (10%). hyperacusis .Bell’s Palsy -1       Lower AND upper facial weakness Weakness severe in 67% Progression to plateau of weakness in days to 2 weeks Rarely bilateral Dysesthesias on the same side of the face as weakness Other symptoms .

80-85% recover completely Treat .Bell’s Palsy .Lyme.2      Preceding viral illness in 60% Possible association with herpes virus Untreated. TB.prednisone 1mg/kg/d for 5-10 days. then quickly taper .with or without Acyclovir 2000mg/d for 5 days Exclude other causes . sarcoid. syphilis . neoplasm.

hyperactive reflexes. widespread fasciculations.Case 5: 38 year old man       6 months progressive weakness in the right leg Twitching in upper and lower extremities Occasional cramps in the right calf No sensory or bladder symptoms Starting to choke on solid food Exam: Isolated right leg weakness. extensor plantar responses .

fasciculations Upper motor neuron .1        Onset generally asymmetric weakness Lower motor neuron . sensation.facial weakness. dysarthria. spastic dysphonia pseudobulbar affect .weakness. Babinski Bulbar dysfunction .hyperreflexia. atrophy. dyphagia.Amyotrophic Lateral Sclerosis . bowel/bladder function .excess or inappropriate crying/laughing Rarely presents with diaphragmatic weakness Preserved mentation.

slows progression .2      Survival 3-5 years Incidence 1-3/100.Riluzole .cramping. dysphagia. respiratory compromise Treat .decreases glutamate release .Amyotrophic Lateral Sclerosis .000 Familial in 5-10% Symptomatic treatment .

Case 6: 71 year old man        3 months intermittent unilateral ptosis and diplopia 8 weeks intermittent dysphagia for liquids. especially with the evening cocktail Exam reveals proximal leg more than arm weakness Normal sensation and reflexes Decremental response to repetitive nerve stimulation Positive edrophonium test using ptosis as objective finding Acetylcholine receptor antibody positive .

then proximal legs/arms Bulbar/respiratory generally later Remains solely ocular in 16% If to proceed to generalized. 87% occur in 1st year .Myasthenia Gravis .1        Autoimmune dysfunction of nicotinic acetylcholine receptor at postsynaptic region of neuromuscular junction Fluctuating weakness of VOLUNTARY muscles Most commonly begins with ocular dysfunction ptosis/diplopia Also neck weakness.

Edrophonium (tensilon) test .positive in 85-90%.CT chest .positive with thymoma .corrects objective deficit .Repetitive nerve stimulation .Acetylcholine receptor antibody . thymoma in 15% Diagnostic testing: .decremental response . more likely if generalized weakness .screen for thymoma .antistriatal muscle antibody .Myasthenia Gravis .2   Thymic hyperplasia in 65% .

quinine.Acetylcholinesterase inhibitor .steroid sparing.pyridostigmine .Myasthenia Gravis .IV Immunoglobulin .best response for remission in young woman with hyperplasia . allow 3 months for effect Avoid aminoglycosides. quinidine.may initially worsen .Azathioprine .Thymectomy .alternative to plasma exchange .Alternate day prednisone .Plasma exchange .for crisis and pre-op thymectomy . procainamide .3   Treatment .

especially oat cell lung Dx .underlying malignancy .Lambert-Eaton Syndrome       Weakness improves with effort Autonomic dysfunction Defective presynaptic release of acetylcholine at neuromuscular junction Associated with malignancy .3.immunosuppression .4 .diaminopyridine increases acetylcholine release .EMG shows facilitated (incremental) response to repetitive nerve stimulation Treat .

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