[A case of amyotrophic lateral sclerosis presenting as motor aphasia in its early stage]

A 76-year-old right-handed woman complained of speech disturbance and difficulity of singing was admitted to our hospital. Examination showed motor aphasia and mild cognitive impairment. After she was discharged, dementia and weakness of the extremities had rapidly progressed. She was readmitted eight month after the first visit, when she was almost abulic, her skeletal and bulbar muscles were remarkably atrophic, and hyperreflexia of the extremities were seen. Electromyographcal study showed neurogenic pattern. These findings suggest amyotrophic lateral sclerosis (ALS) with dementia. Pathological findings were atrophy at the anterior horn of the spinal cord. The brain was diffusely atrophic. The extent of degenerative change was not lateralized. This case is a discriminative type of ALS with dementia, that its first symptom is motor aphasia.