Case 238 -- Rigidity, Decreased Ocular Movements, and Dementia

Contributed by Kar-Ming Fung1, MD, PhD, Mark S Forman1, MD, PhD, Brett Cucchiara2, MD, Elizabeth ZuLinska3 MD, Maria Wallis-Crespo3, MD, Ehud Lavi1, MD
1 Department of Pathology and Laboratory Medicine, Division of Neuropathology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania. 2 Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania. 2 Department of Pathology, Pennsylvania Hospital, Philadelphia, Pennsylvania.
Published on line in July 2000


A 70 year-old right-handed, retired obstetrician without significant past medical history presented to a neurologist in 1993 with a four year history of cognitive decline. He first noted trouble playing the piano, had difficulty writing his name, and had loss of vocabulary. This progressed to mild memory loss, right side weakness and increasing difficulty in expressing himself, though, his comprehension and reading abilities were reportedly normal. He did not complain about his symptoms. He also had several falls including fractured shoulder after a fall down a flight of stairs. At the time of his presentation, he had become wheelchair bound due to an increasingly unsteady gait.

His general examination was unremarkable. On neurologic examination, his answers were laconic but he was oriented to place and time. He had difficulty with short term memory, design-copying, and drawing objects. His interpretation of proverbs was concrete. Examination of cranial nerves was normal. Motor examination revealed asymmetric spastic weakness, greater on the right, without fasciculations or atrophy. Sensation was intact. Reflexes were brisk throughout and there was bilateral ankle clonus and Babinski signs. Coordination exam revealed slow but otherwise unremarkable movements. Routine blood tests including RPR, B12, ESR and TSH were all normal. Brain MRI scans on three separate occasions in the preceding years were unremarkable. At that time, no treatment was initiated.

Four years later, he was admitted to the hospital with a urinary tract infection. Neurological examination at that time revealed him to be awake but mute, and unable to follow simple verbal commands. Ocular motility was limited with no vertical or left gaze but preservation of conjugate gaze to the right. Motor examination revealed asymmetrical weakness with increased muscle tone, greater on the right. Reflexes were brisk with bilateral Babinski signs. He died a year and a half later and an autopsy limited to the brain was performed.




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