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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 6A0 Case 6 History
Case 6 History ---- The patient was a 64yo female with a history of Crohn's disease (status post ileocolectomy), cervical spondylosis (C5-7), depression, anxiety, and progressive supranuclear palsy. Her family history is notable for progressive muscular dystrophy in her first cousin and son. She first presented for a neurological evaluation in 2010, with complaints of speech slurring, difficulty "getting thoughts out," balance issues with several falls, relative increase in handwriting size, and relative loss of organizational skills. Neurologic examination and medical workup at this time was remarkable only for a positive anti-smooth muscle titer. In 2012, she returned for evaluation, again complaining of difficulty with daily organization and feeling easily overwhelmed by issues. She reported being easily distracted, losing focus, sleeping poorly and stopping working. She also reported abnormal speech prosody, intermittent tremor, shaky handwriting, and feeling unsteady on her feet. Her neurologic examination was significant for halting speech that fluctuated throughout the interview but normal gait and balance. There was no hummingbird sign on MRI, but there was a small brainstem with mild ventricular dilatation. Neuropsychological testing, which showed no evidence of dementia and suggested that her symptoms were due to deficits in integration rather than a structural lesion, possibly secondary to depression. Cognitive behavioral therapy with physical/occupational therapy targeting integration and Crohn's disease support group membership was recommended. In 2013, she returned with some improvement in tasks with therapy, but worsening gait and control of arm movements. At this time, neurological examination was significant for slow and mildly dysarthric speech, increased muscle tone and awkward movements of the right arm, hyperreflexia throughout, positive jaw jerk, positive shoulder jerk, positive Hoffman's and Babinski's signs bilaterally, non-sustained clonus with ankle-jerks (R>L), and stiff-legged gait with robotic arm swing. Subsequent follow-up (2013-2015) showed slowly progressive disease with increasing bradykinesia, postural instability, eye movement abnormalities, and spasticity, felt to be consistent with progressive supranuclear palsy. Sinemet was started to treat neck dystonia. She became wheelchair bound. She reported having involuntary jerking movements of arms (and sometimes legs) at night that caused sleep disturbance, choreiform dyskinesia, and dysphagia. By 2015, she was in an extended care facility and was on hospice in the last six months prior to death. Her oral intake decreased, and she expired in 1/2016
