ALS (AMYOTROPIC LATERAL SCLEROSIS)—(LOU GHERRIG’S Dz)
AN IDIOPATHIC MOTOR SYSTEMS Dz—THE MOST COMMON PROGRESSIVE MOTOR NEURON Dz
MAINLY (90%) SPORATIC, 10% AUTO D (HEREDITARY)
UPPER MOTOR NEURON FINDINGS IN THE LE’S
LOWER MOTOR NEURON FINDINGS IN THE UE’S—–SYMMETRIC WEAKNESS OF HANDS AND ARMS
INITIALLY FATIGUE—-THEN WEAKNESS—–FINALLY ATROPHY
CAN OFTEN PRESENT INITIALLY WITH BULBAR TYPE SYMPTOMS—-PND AND ILL DESCRIBED DYSPHAGIA
OCULAR MOTILITY IS SPARRED
TONGUE FASCICULATIONS
DYSPHAGIA (MAY BE HELPED BY BOTOX OR CP MYOTOMY)
VPI, HOARSENESS, DYSARTHRIA
EMOTIONAL LABILITY
50% DIE WITHIN 3-5 YEARS
DEMISE IS USUALLY DUE TO PULM INFXNS DUE TO LACK OF ADEQUATE PULM TOILET
TRAGIC AS DEMENTIA IS NOT ASSOC AND INTELLECT AND AWARENESS REMIAN INTACT
Dx:
Hx, PE, EMG
Rx:
SUPPORTIVE (TRH HAS BEEN TOUTED IN THE PAST AS HELPFUL)