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Washington University Experience | NEURODEGENERATION | NBIA1 (PKAN, Hallervorden-Spatz Dz) | 11A0 Case 11 History
Case 11 History (AANP 2014 DSS Case 1) ---- The patient was a 72-year-old woman hospitalized for management of pleural and pericardial effusions and shock complicating chronic rheumatoid arthritis. As the patient was encephalopathic on admission, neurologic evaluation was limited. Per her family and primary care provider, the patient had a history of depression and possible wide-based gait, but no other overt neurological symptoms. She was employed as a substitute teacher and living independently. MRI of the head without gadolinium contrast was obtained, showing generalized cerebral and cerebellar atrophy, in addition to susceptibility-weighted imaging signal loss involving the bilateral substantia nigra and striatum, suggesting increased iron. She expired ten days after admission following withdrawal of life support.
