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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 16A0 Case 16 History
Case 16 History ---- The patient was a 61yo female whose case was referred from an outside location for pathologic evaluation. She carried a diagnosis of progressive supranuclear palsy vs. corticobasal degeneration. Seven years prior to death she had increasing difficulty performing responsible work as a bookkeeper. She also had problems reading due to “jumps” in her vision but no diplopia. From that point on she had difficulty going downstairs because of an inability to look down. Two years later she reported gait disturbances, mainly characterized by stiffness and unsteadiness. One year after that, she started to have increasing falls, one with ankle fracture. She reported non-rhythmic involuntary movements of the left upper extremity which worsened over the ensuing months. The patient's husband also noted side to side head movements. She had bouts of uncontrolled laughing. The patient became more indifferent to emotional stimuli during this period. Three years prior to admission she noted occasional urinary incontinence and needed more help in her activities of daily living. She reported no swallowing disturbances or numbness. The general neurologic exam was significant for normal short-term memory with mild impairment of long-term memory. The patient had decreased verbal fluency, abnormal alternating movements and apathy but no perseverations. Formal psychometric exam denoted a mildly abnormal cognitive function, possibly suggestive of a very mild dementia. Cranial nerves were remarkable for abnormal extraocular movements with ho upward gaze either voluntarily or with pursuit. There was only five degree (minimal) voluntary or pursuit downgaze. There was no vertical optokinetic and decreased horizontal optokinetic nystagmus. Strength was normal. Tone showed cogwheel rigidity. There was clasp-knife tone in the left upper extremity. There was a frontal lobe release reflex of a snout but no grasp or palmomental reflexes. There is no myoclonus tremor dystonia or dyskinesias, but there was complex uncontrolled non-rhythmic movements of the left upper extremity which increased with activity. There was incoordination of the left upper extremity more severe than with the right. Gait testing showed small steps, narrow-based and unstable with loss of associated movements. MRI showed right parieto-occipital atrophy more than the left side and also generalized mild atrophy. Clinical impression was supranuclear vertical gaze palsy, gait disturbances, and parkinsonism, but also with alien hand syndrome, left hemisensory neglect, and apraxias of the left upper extremity. The alien hand syndrome and left upper extremity abnormalities are consistent with corticobasal ganglionic degeneration, whereas the other symptoms are suggestive of progressive supranuclear palsy. There is no further history available thereafter until the autopsy.
