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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 12A0 Case 12 History
Case 12 History ---- The patient was an 80yo female with a past medical history of progressive supranuclear palsy, hypothyroidism status post thyroidectomy, hypertension, atrial fibrillation, remote history of cerebral vascular accident, cardiomyopathy, status post pacemaker and defibrillator placement. She first presented to the Neurology Clinic in 10/2014 for evaluation of gait disorder that began approximately 5 years prior. Her slowly progressive symptoms that became rather abruptly worse in 2012 when she started having inexplicable falls. Her voice had become low and difficult to understand two years prior to presentation. She occasionally choked on cold liquids. She had impulsivity, poor judgement, inappropriate laughing and crying. She had been using a walker until May 2014, but has been basically wheelchair bound since that time. The physical examination showed hypophonia with mild dysarthria, marked speech latency and slow speech. She had abnormal extraocular movement with no down gaze, very limited upgaze, and nearly full horizontal gaze to the left. She also had mild ophthalmoplegia to the right. There was no nystagmus or square wave jerks noted. Motor exam showed full strength with asymmetric rigidity detectable only in the left arm. The right upper extremity and legs had normal tone and no cogwheeling. At that time, her clinical diagnosis was progressive supranuclear palsy (PSP). The mild asymmetric rigidity could suggest corticobasal degeneration (CBD); however, there were no other signs of asymmetry to strongly support a CBD diagnosis. Since then, she continued to have cognitive decline and motor deterioration. She was last evaluated in the neurology clinic in 11/2016. At that time, she required full assistance with all activities of daily living except feeding herself. She had minimal spontaneous movement with some spontaneous speech. She also had frequent near choking episodes especially when drinking water. She was admitted in 10/2018 to an OSH for severe dehydration and hypernatremia. During admission she received intravenous fluids and had mild pyuria that was treated empirically with cefepime and then ceftriaxone. During the morning following admission, the patient developed a cough and had low oxygen saturations. However, chest x-rays on admission and after the onset of cough were negative for pulmonary infiltrates or heart failure. During her hospital stay, the patient required increasing amounts of oxygen and her lungs became increasingly congested. Goals of care were redirected towards comfort. The patient expired in 10/2018.
