09-11-2017, 09:40 AM
Muscle Weakness + Foot Drop + Fasciculation. 5 -10 % of cases are familial (autosomal dominant).
Infantile presentation is call SPINAL MUSCULAR ATROPHY (PERONEAL MUSCULAR ATROPHY - LOU GHERING DISEASE)
This is a progressive motor neuron disease that presents w/ a combination of Lower and Upper Motor Neuron Findings.
The median survival after Diagnosis is 3 to 5 years, with death usually occuring from respiratory failure.
Hint in USMLE, Pt with MOTOR DYSFUNCTION (PROGRESSIVE WEAKNESS) but NO SENSORY DYSFUNCTION W/ NORMAL GAIT
Infantile presentation is call SPINAL MUSCULAR ATROPHY (PERONEAL MUSCULAR ATROPHY - LOU GHERING DISEASE)
This is a progressive motor neuron disease that presents w/ a combination of Lower and Upper Motor Neuron Findings.
The median survival after Diagnosis is 3 to 5 years, with death usually occuring from respiratory failure.
Hint in USMLE, Pt with MOTOR DYSFUNCTION (PROGRESSIVE WEAKNESS) but NO SENSORY DYSFUNCTION W/ NORMAL GAIT