Beware Of The Cortical Ribboning: The Spectrum - Experience From A Case Series

INTRODUCTION: Creutzfeldt-Jakob disease (CJD) is an uncommon, fatal, genetic, transmissible and degenerative, brain disorder. It affects one person in every one million in the United States. A definite diagnosis can be made by histopathological confirmation which is often not possible for various reasons. Therefore, great caution is needed before the diagnosis is offered.MATERIALS AND METHODS: Patients with radiological diagnosis of CJD in the past 2 years were assessed clinically, reassessed at 1-month, and results are discussed.RESULTS: Four out of the 12 patients seen had nonprion cause. Hyperacute onset with coma, nonprogressive changes in imaging and electroencephalography, were seen in those who improved. The presence or absence of 14-3-3 in cerebrospinal fluid was not useful in either confirming or excluding the diagnosis in a given case.CONCLUSION: All patients with cortical ribboning need correlation with clinical features and follow-up to confirm or exclude prion disease.