We report a rare association of amyotrophic lateral sclerosis (ALS) with in
cidental pulmonary and muscle sarcoidosis. A 63-year-old woman presented wi
th slowly progressive weakness and atrophy of the extremities starting from
the left leg. The biopsy of a small mass in the left gastrocnemius reveale
d a typical sarcoid nodule. She was treated with corticosteroid for possibl
e sarcoid neuromyopathy. In spite of the treatment, her clinical course was
relentlessly progressive and she died of bulbar palsy. Autopsy revealed a
loss of motor neurons in the anterior horn, vacuolar degeneration of the la
teral funiculus, and noncaseating granulomas in paratracheal lymph nodes an
d lungs. No granulomatous lesion or cellular infiltration was found in the
spinal cord.