Pick's Disease
A 60 year old male is brought in by his wife who states that he has recently developed behavioral and personality changes including lack of interest in everyday activities and bouts of unexplained anger. The wife also states that he has developed difficulty in speaking during the past 3-4 months. Significant past history includes a partial gastrectomy for a "benign" tumor, partial thyroidectomy for "goiter", and he was treated for gonorrhea 40 years ago while he was in the Navy. His wife also says he is a moderate drinker (1-2 beers per day). Physical exam reveals normal pulse and blood pressure. A neurological exam reveals equal and bilateral muscle stretch reflexes, and normal muscle tone and strength in all extremities; no sensory deficits, and intact cranial nerves; prominent snout reflex, and glabelar reflex and definite impaired cognitive function including paraphrasic errors, poor abstraction and inability to subtract serial sevens (7 from 100).
Laboratory studies reveal normal CBC (no macrocytes), glucose, electrolytes, liver function studies, thyroid studies, serum B-12 and serum folate levels. A VDRL is negative.
You schedule an MRI scan of his head and neck in 3 days. Unfortunately, the patient goes home and dies suddenly the next day and the coroner orders an autopsy.
1. The following image represents the patient’s brain. Based on the above history and this image, this patient’s most likely diagnosis is:
A. Wernicke-Korsakoff Syndrome
B. Alzheimer's Disease
C. Parkinson Disease
D. Progressive supranuclear palsy
E. Pick Disease
F. Prion disease (Creutzfeldt-Jakob Disease)
Correct Answer: E
2. The morphologic finding in this patient include which of the following:
A. Hemorrhagic necrosis of mamillary bodies
B. Global cerebral atrophy
C. Loss of pigment in the substantia nigra
D. Hydrocephalus, obliterative endarteritis and gliosis.
E. Frontal and temporal lobe atrophy
Correct Answer: E
3. Complications in patients with this diagnosis include:
A. Aspiration pneumonia, urinary tract infection
B. Hepatic failure
C. Ataxia and Charcot joints
D. Rigidity and tremors
Correct Answer: A