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Motor Neuron Disease

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    disease affecting motor neurons
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  • Where are the Anterior Horn cells
    B
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  • The anterior horn cells affect which of the following?  A. Thought processing, B. sensation to the face and limbs, C. movements of the upper and lower extremities, D. Coordination and balance
    movement of the extremities
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  • Which motor neuron lesion (upper, lower, central) is affected by marked muscle wasting and atrophy, Flaccid hypotonic muscles, Diminished or absent DTRs, Muscle cramps Fasciculations
    lOWER MOTOR NEURON LESION
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  • What tract does ALS affect ( be specific)
    Lateral Corticospinal tract
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  • Which condition affects: Both UMNs + LMNs,  onset later in life,  Spares sensory, vision, hearing and autonomic functions, Intact  Cognition ?
    ALS
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  • Which condition starts in one limb,  spreads to adjacent myotomes and eventually spread to contralateral limb, then bulbar, and respiratory muscles?
    ALS
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  • Which variant of ALS presents initally with bulbar symptoms (Dysarthria, drooling, gagging, dysphagia, choking) and “handkerchief sign”?
    progressive bulbar palsy
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  • Which ALS Variant presents with Pure LMN symptoms, distal limb wasting and weakness, fasciculations, cramps, reduced reflexes in weak limbs long clinical course, slow progression to prox limb muscles. No sensory symptoms
    Progressive Muscle Atrophy
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  • Which of the following are NOT a usual electrodiagnostic fining in ALS?  A. Tongue fasculation,B. PSW in thoracic paraspinals,C. absent right tibial motor NCS, D. absent left sural NCS
    Absent left sural nerve
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  • Which of the following EMG/NCS findings can help predict prognosis? PSW in FDI, reduced CMAP in tibial, absent ulnar study, fasciculations in gastroc.
    reduced CMAP amplitude
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  • Which of the following EMG findings is seen in ALS: A. increased and early MUAP recruitment,B. PSW with polyphasic potentials, C. reduced MUAP amplitudes (<0.5 mv) , D. reduced radial SNAP amplitude
    PSW and polyphasic potentials
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  • Which condition is often confused with Progressive Muscle Atrophy(PMA) , Predominant LMN symptoms, slowly progressive ; 2:1 (M:F); onset before 5th decade Progressive, asymmetric weakness, often of distal UE first
    Multifocal Motor neuropathy
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  • Which of following NOT a characteristic of post polio syndrome?   A. Previous history of poliomyelitis,  B.Partial to complete recovery of function, C. Stability for approximately 5 years, D. New onset of neuromuscular symptons
    stability of 5 years (should be 15 years)
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  • How does Kennedy's disease ( SMA) differ from ALS on NCS testing?   Kennedy's dz has reduced SNAP amplitude, Kennedy's affects mostly female, Kennedy's presents in children :
    Kennedy's disease presents with low amplitude SNAPs due to DRG involvement
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