A 62‐year‐old man with a family history of dementia, showing dementia and parkinsonism, presented with personality change and behavioral abnormality

Abstract

The patient was a 62-year-old man. His mother, maternal uncle and grandfather had suffered from dementia. At the age of 57, the patient developed hypobulia, apathy and mental irritability, and showed disinhibited and stereotypical behaviors. At the age of 58, he showed personality change, poor understanding, restless and aimless walking, perseveration and echolalia as well as memory disturbance and disorientation. There was amnestic aphasia but no apraxia or agnosia. A CT scan and MRI disclosed localized symmetrical atrophy of the frontal and temporal lobes. Thereafter, he became mutistic and developed bradykinesia, rigidity of the extremities and impairment of vertical ocular pursuit movement. Sucking and grasp reflexes were positive with forced crying. He also revealed increased deep tendon reflexes in the extremities and bilateral Babinski signs. At the age of 59, he showed an oral tendency and could no longer walk. At the age of 60, he became bedridden, and tube feeding was started because of pseudobulbar palsy. At the terminal stage, he had repeated episodes of bronchopneumonia and died with akinetic mutism after a disease duration of about 5 years.