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)