[Bickerstaff's brainstem encephalitis -- an analysis of clinical and MRI findings].

BACKGROUND AND PURPOSE

Bickerstaff's brainstem encephalitis (BBE) is a very uncommon central nervous system disease. Its etiology remains unknown but it is postulated that BBE has an autoimmunologic origin. BBE is characterized by acute ophtalmoplegia, ataxia, pyramidal paresis and disturbance of consciousness. A special pattern of changes in magnetic resonance imaging (MRI) is characteristic of BBE. Lesions are located mainly in the brainstem. The aim of this paper is to analyze the clinical course and MRI changes in our own cases of BBE.

MATERIAL AND METHODS

We have analyzed medical records, clinical findings and MRI images of the patients with BBE diagnosis established in our Department of Neurology in the years 1998-2004.

RESULTS

During that period 4 cases of BBE were diagnosed with clinical sings of acute brainstem lesion and MRI imaging suggestive of BBE. In our patients we have observed ophtalmoplegia, ataxia, disturbances of consciousness and pyramidal signs. Two patients had a monophasic and 2 remitting-relapsing course of the disease. In all four cases we have seen on T2-weighted images large, irregular hyperintense lesions in the brainstem, especially in the pons, midbrain and medulla, but also in one case in the thalamus and vermis of the cerebellum. Patients were treated with steroids, plasmapheresis or acyclovir or a combination of these methods. We observed a complete recovery in two cases and significant improvement in the other two lasting more than two years confirming a relatively good prognosis in BBE.

CONCLUSIONS

BBE despite severe initial presentation usually has a good prognosis. MRI plays critical role in the diagnosis of BBE.