Cogan's Syndrome: A Rare Cause of Meningoencephalitis

We report a 14-year-old boy who presented with meningoencephalitis. Other features particularly auditory, vestibular, and ocular lead to the diagnosis of Cogan's syndrome. Treatment with prednisolone resulted in a rapid improvement and recovery of his hearing. Cogan's syndrome is a rare pr...

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Bibliographic Details
Published in:Journal of child neurology 2009-06, Vol.24 (6), p.753-757
Main Authors: Pysden, Karen S., Long, Vernon, Ferrie, Colin D., Leeds Teaching Hospitals NHS Trust
Format: Article
Language:English
Subjects:
Adolescent
Brain - drug effects
Brain - pathology
Diagnosis, Differential
Glucocorticoids - therapeutic use
Hearing Loss - drug therapy
Hearing Loss - etiology
Hearing Tests
Humans
Magnetic Resonance Imaging
Male
Meningoencephalitis - diagnosis
Meningoencephalitis - drug therapy
Meningoencephalitis - etiology
Prednisolone - therapeutic use
Vasculitis, Central Nervous System - complications
Vasculitis, Central Nervous System - diagnosis
Vasculitis, Central Nervous System - drug therapy
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Summary:We report a 14-year-old boy who presented with meningoencephalitis. Other features particularly auditory, vestibular, and ocular lead to the diagnosis of Cogan's syndrome. Treatment with prednisolone resulted in a rapid improvement and recovery of his hearing. Cogan's syndrome is a rare primary vasculitis, characterized by ocular, auditory, and vestibular symptoms, which can have significant morbidity and mortality. Presentation with a meningoencephailitic picture is unusual. Increased awareness of its clinical features among pediatricians and pediatric neurologists should lead to earlier diagnosis and increased recognition of the serious systemic manifestations. Early use of prednisolone can prevent hearing loss and can also be useful in treating the other vasculitic manifestations.
ISSN:0883-0738
1708-8283
DOI:10.1177/0883073808329533