Recurrent intracerebral hemorrhages due to central nervous system vasculitis: A neuropsychological case report

Abstract

Objective Primary angiitis of the central nervous system (PACNS) is a rare and devastating form of vasculitis that destroys the vessels of the brain and spinal cord, resulting in progressive and debilitating neurologic symptoms. The objective of the present study was to detail the diagnostic process of a case of a patient with PACNS who suffered from six intracerebral hemorrhages (ICHs). Method The patient was an African American woman with a history of recurrent ICHs of unclear etiology who received serial neuropsychological evaluations over the course of a 5-year period. Two comprehensive neuropsychological evaluations are included, as well as an overview of her clinical course, including differential diagnostic considerations and treatment planning. Results Neuropsychological assessment revealed marked deficits in visuospatial abilities and processing speed associated with her underlying neuropathology. Integrated review of her medical records indicated a probable diagnosis of PACNS as the likely etiology of her recurrent ICHs. Conclusions This study demonstrates the importance of differential diagnosis of low base-rate conditions, functional neuroanatomy and neurobehavioral phenomenology, serial assessment, and cognitive reserve in clinical neuropsychological practice.