Freiburg Neuropathology Case Conference

Excerpt

A 62-year-old female patient presented with an acutely deteriorating paraparesis which had evolved slowly over 6 months. On admission, she showed complete paraplegia, new cortical blindness and psychomotor retardation. Diagnostic imaging revealed multiple cerebral lesions. Extensive cerebrospinal fluid (CSF) analyses showed no evidence of an infectious origin, but revealed identical oligoclonal bands in serum and CSF denoting a systemic disease in combination with blood–brain barrier disruption. The laboratory workup for vasculitis was inconspicuous. Elevated levels of serum soluble interleukin 2 receptor and neopterin pointed to sarcoidosis, but were regarded as non-specific. Due to pneumonia, the patient required antibiotic treatment and was temporarily transferred to the intensive care unit. A stereotactic biopsy was performed under general anesthesia. The initial postoperative course was uneventful; however, with dexamethasone treatment a slight improvement of the cortical blindness occurred. The initial histopathological diagnosis was small-vessel vasculitis. Consequently, the patient was treated with cyclophosphamide and prednisolone, and transferred to a rehabilitation hospital. Further manifestations of vasculitis were not found. After histopathological determination of the final diagnosis the patient was retransferred from the rehabilitation hospital. She showed a further improvement of the cortical blindness. After initiation of the definitive treatment there was a marked improvement of the cortical blindness, whereas the paraparesis remained unchanged. …