Magnetic resonance imaging of mesenrhombencephalitis
Introduction
Mesenrhombencephalitis is a rare inflammatory disorder involving the brainstem and cerebellum. Initially described by Bickerstaff and Cloake [1] in 1951, the disease process classically presents with areflexia, ataxia, and ophthalmoplegia. It may be life-threatening and is very difficult to diagnose clinically. Laboratory findings are not uncommonly normal. The etiology is often undetermined; however, viruses are implicated in the majority of cases, with herpes simplex being the most common causative agent [2], [3], [4]. Several case reports and small series have described the magnetic resonance (MR) findings of mesenrhombencephalitis [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13]. We describe the clinical, laboratory and MR findings in five patients with this rare inflammatory process.
Section snippets
Materials and methods
Five patients with the clinical and imaging findings of brainstem encephalitis were identified. These included three males and two females ranging in age from 1 to 25 years (mean age, 15.4 years). Medical records were reviewed for presenting signs and symptoms. Laboratory data reviewed included total and differential white blood cell counts; blood cultures; cerebrospinal fluid (CSF) analyses, CSF bacterial, fungal, and viral cultures; viral antibody titers; and other serology when available.
Results
Presenting signs and symptoms in this group of patients included altered mental status, confusion, ataxia, dizziness, unilateral weakness, numbness, headache, memory loss, fever, aphasia, and dysphasia.
CSF analysis in four patients was normal. One patient (Case 1) had abnormal CSF analysis, which revealed 10 white blood cells, 44 polymorphonuclear cells, and 56 monocytes/mm3, protein level of 27 mg/dl, and a glucose level of 140 mg/dl. Brain biopsy was performed in this patient, which showed
Discussion
Mesenrhombencephalitis is serious, uncommon illness originally described by Bickerstaff and Cloake [1], which is very difficult to diagnose clinically. Patients typically present with symptoms of areflexia, ataxia, and ophthalmoplegia [1]. Other presenting signs and symptoms include altered mental status, fever, headache, memory loss, and weakness. The etiology is frequently undetermined; however, viruses are thought to be the cause in most cases, with herpes simplex as the most common [2], [3]
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