Primary vasculitis of the cns restricted to the brainstem
Abstract
Background: Primary or isolated vasculitis of the central nervous system (PVCNS) is a rare disease with an atypical clinical picture. It involves both small and mediumsized leptomeningeal, cortical and subcortical vessels. Etiology and pathogenesis are unknown. The first clinical sign is usually a headache and later in the disease virtually every neurological symptom and sign has been reported. The golden standard for diagnosis is a biopsy. High doses of corticosteroids and cyclophosphamide have been recommended for treatment.
Patient and methods: A case of a 61 years old woman with diabetes and hypertension is reported. She sudenly developed signs of focal ischemic brain stem lesion and had lesions visualised on MR in brain stem and both hemisferes. After temporary remission she developed similar signs five months later. She had died before the diagnostic procedures were performed. The diagnosis of PVCNS isolated to the brain stem was made post mortem.
Conclusions: The diagnostic and therapeutic challenges of PVCNS are big even for an experienced clinician. Diagnosis depends on a combination of clinical, imaging and histological features. Often the diagnosis is confirmed post mortem.
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