A 53-year-old, right-handed woman presented with a 3-year history of difficulty in expressing herself properly and understanding spoken language.
History of present illness:
Her family reported that she had felt disoriented in familiar places and was unable to recall recent events or remember names of family members. They noticed behavioral changes consisting of irritability and aggressiveness. The patient became increasingly depressed and anxious and developed a left-sided clumsiness and a mild symmetric tremor mainly affecting the hands.
Eight months before admission, the patient had myoclonic jerks in the left arm. During the 3 months before admission, she developed trouble with reading and writing. Shortly thereafter, she was unable to perform activities of daily living without help. Her family denied any history of falls during the course of her disease.
She had previously been treated with rivastigmine patches at another institution, without clinical improvement.
There was no history of alcohol abuse or smoking.
The patient's father had a spastic paraparesis of unknown origin.
What is the most likely diagnosis? What more do you want to know?
(Stay tuned for more clues tomorrow morning.)