Need for Swift Diagnosis of Primary Angiitis of Central Nervous System: A Case With Focal Motor Seizures of Hand Progressing to Aphasia

Primary angiitis of the central nervous system (PACNS) is a rare disorder and difficult to diagnose. The time period from the presentation of the patient until a diagnosis can be protracted, which may result in further progression of the disease and poor patient outcomes. It has immensely varied symptomatology, further confounding swift diagnosis. We present a case of a 33-year-old male who had focal motor seizures with intact awareness of the right upper limb for three weeks prior to presenting to our hospital with acute right hemiparesis. One month into hospital admission, the patient developed complete motor aphasia while being investigated for the cause of multiple ischemic brain infarcts. The patient underwent immunosuppression along with plasmapheresis and pulse steroid therapy started prophylactically on suspicion of PACNS, which was subsequently confirmed by brain biopsy. Disease remission was achieved with rituximab.