Cervical spondylotic myelopathy and Kennedy syndrome mimicking amyotrophic lateral sclerosis

A 62 year old male patient presented with a longstanding history of slowly progressive limb weakness, speech, and swallowing difficulties. In 1983 a diagnosis of amyotrophic lateral sclerosis had been made. At that time his physical examination showed tongue atrophy with fibrillations, proximal limb weakness, and brisk lower limb tendon reflexes. Electromyography showed abnormal “spontaneus activity” with fibrillations and positive sharp waves in muscles of all limbs. His further medical and family history was unremarkable. In January 2000 neurological examination showed mild facial weakness, marked atrophy and fibrillations of the tongue, severe dysarthria and dysphagia, atrophy, and weakness of …