Neuropathology : official journal of the Japanese Society of Neuropathology
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Flail arm (FA) syndrome, a minor subtype of amyotrophic lateral sclerosis (ALS), is characterized by progressive weakness and upper girdle wasting, but the associated pathological changes remain unclear. A 59-year-old man was admitted to our hospital with a 3-year history of upper girdle weakness. Bulbar symptom and gait disturbance gradually developed, and he was clinically diagnosed with FA syndrome. ⋯ The histological findings were consistent with typical ALS, including Bunina bodies and Lewy body-like and skein-like inclusions. Cytoplasmic vacuoles were found in the remaining anterior horn motor neurons of the lumbar spinal cord. This is a unique autopsy case with a long-standing clinical course that suggests that FA syndrome is an atypical form of ALS.