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Chronic Neurologic Disorders CH 234

Chronic Neurologic Disorders CH 234. November 3, 2009. Amyotrophic Lateral Sclerosis. Aka ALS or Lou Gehrig disease Rapidly progressive muscle atrophy and weakness Degeneration of both upper and lower motor neurons Causing varying degrees of spasticity, hyper-reflexia, and muscle paralysis

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Chronic Neurologic Disorders CH 234

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  1. Chronic Neurologic Disorders CH 234 November 3, 2009

  2. Amyotrophic Lateral Sclerosis • Aka ALS or Lou Gehrig disease • Rapidly progressive muscle atrophy and weakness • Degeneration of both upper and lower motor neurons • Causing varying degrees of spasticity, hyper-reflexia, and muscle paralysis • Sensory and cognitive function is usu. Spared • Progressive respiratory muscle weakness • 50% die in 3 yrs of diagnosis

  3. ALS in the ED • Acute respiratory failure: may need intubated (ABG not reliable tool to assess impending failure) • Aspiration pneumonia • Choking episodes • Trauma from extremity weakness

  4. Myasthenia Gravis • Autoimmune disease • Acetylcholine receptor antibodies impair receptor fxn • “great imitator” • General weakness, esp proximal muscle groups • Ptosis and diplopia first and later dysphagia • Symptoms worse with prolonged use, improves with rest • Usu. no sensory, reflex or cerebellar deficit

  5. Myasthenia Gravis • Myasthenic crisis: respiratory failure from weakness • May be drug induced or related to a thymoma • Dx with edrophonium chloride, EMG and serum studies • Tx is with cholinergic inhibitor such as pyridostigmine (do not miss doses) • Most significant ED complication is need for intubation • Avoid depolarizing or nondepolarizing paralytic agents as they persist in the system 3x as long

  6. Tensilon Test • Determines disease exacerbation vs excessive cholingeric drug therapy • Epdrophonium (tensilon) is given. If the patient has resolution of weakness, then it is considered a positive test and due to exacerbation • Negative if muscle fasciculations, resp depression or cholingeric symptoms occur with Tensilon • A positive result should be followed with longer acting Neostigime

  7. Multiple Sclerosis • Motor, sensory, visual, and cerebellar dysfunction caused by CNS myelin destruction • May be relapsing remitting, relapsing progressive or chronically progressive • Seen most commonly in women in 20-30 who live in the north and are white • Diagnosed by MRI (multiple plaques on brain and spinal cord that may resolve and reform in other areas), LP and clinical signs/symptoms

  8. Multiple Sclerosis • Optic neuritis is presenting sx in 30% • Symptoms may worsen with heat • Plethora of signs including gait disturbance, weakness, incontinence, dementia, vertigo,etc • High dose methylprednisolone shortens exacerbations • Long term therapy by neurology with interferon-B and immunosuppressive meds

  9. Lambert-Eaton Myasthenic Syndrome • Autoimmune disorder causes fluctuating weakness and fatigue esp of the proximal limb muscles • Show improvement with sustained or repeated exercise, Lambert sign • c/o myalgias, muscle stiffness, paresthesia, metallic tase and muscarinic insufficiency (dry mouth) • Assoc with older men who smoke and have lung CA • Supportive tx

  10. Parkinson Disease • Chronic neurodegernerative disease • Extrapyramidal movement disorder: resting tremor, cogwheel rigidity, bradykinesias or akinesia and impaired postural reflexes • Reduced number of functional dopaminergic receptors in substantia nigra with presence of Lewy bodies • Tx with anticholinergics; levodopa or carbidopa; and dopamine receptor agonists ie bromocriptine

  11. Polio Myelitis and Postpolio Syndrome • Neurotropicenterovirus that causes paralysis through motor neuron destruction, muscle denervation, and atrophy • 80% are asymptomatic, 1-2% get major illness with neurologic abnl • Most will resolve in 1 yr but 20% will develop bulbar polio (speech, swallowing, facial muscle, and extraocular muscle dysfunction) • Postpolio syndrome ~30yrs after the initial and affects muscles/joints intially spared • Consider polio if acute febrile illness, aseptic meningitis and asymmetric flaccid paralysis with loss of reflexes and normal sensation

  12. Sources • Tintinalli’s 6th edition

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