Creutzfeldt-Jakob Disease Presenting as Stroke: A Case Report and Systematic Literature Review.

BACKGROUND: Creutzfeldt-Jacob disease (CJD) is a human prion disease generally characterized by subacute changes in behavior and intellectual function, often followed by ataxia, vision changes, and myoclonus. Ten percent of cases may present atypically, both symptomatically and in respect to initial investigations. METHODS: We report a case of CJD mimicking acute stroke and review all similar cases in the magnetic resonance imaging era reported in English, identified through a PubMed and SCOPUS search. RESULTS: A 68-year-old woman presented with an acute left parietal syndrome, initially referred as a left middle cerebral artery territory stroke. Structural imaging was unremarkable and focal electroencephalogram changes suggested nonconvulsive status epilepticus. Subsequent clinical progression, with the development of cortical high signal on diffusion-weighted imaging and positive 14-3-3 protein in the cerebrospinal fluid, confirmed a diagnosis of CJD. Review of the literature identified 14 further cases mimicking both anterior and posterior stroke syndromes. CONCLUSIONS: CJD develops primarily within a population in whom stroke risk factors are common and represents a rare but important stroke mimic. Negative vascular imaging in elderly patients with apparent acute stroke syndromes should prompt diagnostic review including consideration of prion diseases.

Validation of a prognostic subarachnoid hemorrhage grading scale derived directly from the Glasgow Coma Scale.

BACKGROUND AND PURPOSE: A new Glasgow Coma Scale-based scale has been developed to predict patient outcome in subarachnoid hemorrhage by calculating cut-off points by which 2 consecutive categories corresponded to a statistically significant different outcome. We assessed the external validity of this Prognosis on Admission of Aneurysmal Subarachnoid Hemorrhage (PAASH) scale and compared it to the commonly used World Federation of Neurological Surgeons scale. METHODS: From our database of subarachnoid hemorrhage patients we retrieved data on all patients admitted between November 2000 and March 2006. By means of logistic regression, we calculated OR with corresponding 95% CI for poor outcome at 3 months for each category in comparison with the lowest category of both scales. Areas under the curve of the corresponding receiver operator characteristic curve were calculated. RESULTS: We included 537 patients. For the PAASH scale, OR ranged from 3.9 (95% CI, 2.4 to 6.2) to 84 (95% CI, 25 to 287) and increased more evenly than for the World Federation of Neurological Surgeons (WFNS) scale, with OR ranging from 2.3 (95% CI, 1.3 to 4.1) to 69 (95% CI, 31 to 157). Areas under the curve were 0.81 (95% CI, 0.77 to 0.84) for the PAASH and 0.82 (95% CI, 0.79 to 0.86) for the WFNS scale. CONCLUSIONS: Both PAASH and WFNS scales have a good discriminatory ability for patient prognosis. Because the OR of the PAASH increase more gradually, it is slightly preferable to the WFNS scale.