Cranial neuropathies, confusion, and ataxia— Challenges for diagnosis and therapy

Abstract

Introduction: The differential diagnosis of patients presenting with multiple cranial neuropathies, ataxia, and altered mentation is broad and includes immunologic, infectious, vasculitic and metabolic conditions. Primary considerations are Bickerstaff’s brainstem encephalitis (BBE), the Miller Fisher syndrome (MFS), Wernicke’s encephalopathy and botulism. The initial workup may be unrevealing. Timely treatment is imperative and unnecessary treatment can be associated with serious adverse reactions. Sensitivity to the decisions needed in such patients is therefore important. Case report: A 58-year-old male presented with symptoms of altered mental status, blurred vision, dysphagia and dysarthria, impaired pupillary responses to light, facial diplegia, ataxia, and decreased tendon reflexes after an episode of a self resolving diarrheal disease. Primary initial diagnostic concerns were Bickerstaff’s brainstem encephalitis (BBE), Miller Fisher syndrome (MFS), Wernicke’s encephalopathy and botulism. Initial work-up including cerebrospinal fluid analyses, imaging studies, and an electrodiagnostic examination did not provide information helpful for narrowing this differential. The patient was treated with botulinum antitoxin, thiamine and intravenous immune globulin (IVIG) before the results of specialized tests were available. The patient’s clinical condition improved. Retrospectively, the patient was diagnosed as BBE. Conclusion: This case emphasizes the difficulties in distinguishing between BBE, MFS, and botulism as well as demonstrating the complexities of treating such patients.