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Objective: Creutzfeldt–Jakob disease is a rapidly progressive spongiform encephalopathy. The E200K mutation is found in a majority of genetically transmitted Creutzfeldt–Jakob disease cases. Methods: We describe the case and associated neuroimaging of an E200K-129M gene-mutation-related fatal spongiform encephalopathy with resultant clinical insomnia and thalamic changes. Results: A 46-year-old Caucasian male presented with, who was well until 2 months prior to admission, a rapidly progressive dementia followed by a change in personality with auditory and visual hallucinations. His wife noted progressively worsening jerking and other limb movements and that he kept his eyes open overnight and was “awake” at all hours. Magnetic resonance imaging, electroencephalogram and initial cerebrospinal fluid analysis were essentially non-diagnostic. Positron emission topography revealed severe bilateral thalamic hypometabolism. Posthumous cerebrospinal fluid analysis revealed abnormal PrP 27-30 protein. Autopsy confirmed prion disease and presence of the E200K-129M mutation. Conclusion: This report highlights that positron emission topography imaging may help diagnose E200K-129M mutation-related spongiform encephalopathy. In cases of non-diagnostic magnetic resonance imaging, electroencephalogram and cerebrospinal fluid studies, early positron emission topography may help in the workup of rapidly progressive dementia.

Objective: Apnea test is required as part of the brain death examination. The duration of the apnea test is variable but typically requires 8–10 min. Prolonged apnea tests have been reported in the setting of hypothermia. Here, we describe a case of prolonged duration of apnea test secondary to a phenomenon called cardiac ventilation. Methods: The patient presented in coma with brainstem areflexia after having an intracerebral hemorrhage resulting in subfalcine, central, uncal, and tonsillar herniations. Confounding variables were excluded. Brain death testing was performed, and she was found to have brainstem areflexia. Pre-requisites for apnea test were then met. Results: Apnea testing, however, was prolonged at 110 min. When reconnected to ventilator, it was noted that she had small (30–35 cc) tidal volumes at a rate of her heart rate without respiratory effort. Ancillary testing with four-vessel cerebral angiogram confirmed cerebral circulatory arrest. Conclusions: To our knowledge, this is the longest reported case of apnea testing during brain death testing. Variables known to cause a delay in the rise of carbon dioxide (PaCO2) levels were excluded. We suspect the hyperdynamic cardiac state caused cardiac ventilations resulting in slow increase in carbon dioxide levels.

Cerebral air embolism is a dreaded complication of invasive medical procedures. The mainstay of therapy for patients with cerebral air embolism has been hyperbaric oxygen therapy, high flow oxygen therapy, and anticonvulsants. We present a novel therapeutic approach for treatment of cerebral air embolism causing large vessel occlusion, using endovascular aspiration. Our patient developed a cerebral air embolism following sclerotherapy for varicose veins. This caused near total occlusion of the superior division of the M2 segment of the right middle cerebral artery. Symptoms included unilateral paralysis, unintelligible speech, and hemianopia; National Institutes of Health Stroke Scale (NIHSS) on presentation was 16. The air embolism was treated using a distal aspiration technique. Angiography following aspiration showed Thrombolysis in Cerebral Infarction 2B reperfusion. Following aspiration, the patient was re-examined; NIHSS at that time was 4. At 1 month follow-up, the modified Rankin Scale score was 1 and NIHSS was 1. Treatment of cerebral air embolism is discussed.

INTRODUCTION Creutzfeldt-Jakob disease (CJD) is a rapidly progressive dementia with a median survival of 2-14 months. The diagnosis can only be made accurately by biopsy/autopsy. However, this is not always feasible or desirable. Thus, diagnostic criteria have been proposed by UCSF, European MRI-CJD Consortium, and WHO. We will compare these criteria. PATIENTS AND METHODS Retrospective study of 31 patients (average age of 69.2 years) between 2003 to 2010 by ICD9 codes 046.1, 046.11, and 046.19. RESULTS All patients presented with rapidly progressive dementia (mean duration of 4.25 months). Pyramidal and extrapyramidal findings, myoclonus, cerebellar changes, akinetic mutism, and visual disturbances were observed in 6.5-48.4%. Five had periodic pattern on EEG. CSF biomarker 14-3-3 was positive in 11. Tau was positive in 6. Neuron specific enolase was positive in 9. By consensus (kappa = 0.62), MRI was “typical” of CJD in 23 with cortical ribboning (n = 16), basal ganglia hyperintensity (n = 15), or combination (n = 8). By WHO criteria, which does not include neuroimaging, CJD was diagnosed in 10, but 14 if any CSF biomarker was used (p = NS). The UCSF criteria, which does not include CSF biomarkers, diagnosed 18 cases, and the European MRI-CJD Consortium, which includes neuroimaging and CSF biomarkers but with less neurological signs, diagnosed 23 cases (p < 0.05 and p < 0.001, respectively). CJD-mimics included urosepsis, neurosarcoidosis, idiopathic left temporal lobe epilepsy, alcohol intoxication, central nervous system vasculitis, viral encephalitis, and non-Hodgkin's lymphoma. CONCLUSION This study illustrates the variability in diagnosing CJD and emphasizes the diagnostic utility of neuroimaging. It also highlights false-positives that occur with neuroimaging.

We present an interesting [18F]fluoro-2-deoxyglucose positron emission tomography (FDG-PET) imaging finding in a patient with ocular flutter and cerebellar ataxia as part of anti-Ma 1/2 antibody-mediated paraneoplastic syndrome associated with a testicular seminoma. He had a typical anterior mesial temporal hyperintensity on magnetic resonance imaging (MRI) without gadolinium enhancement. In addition, his FDG-PET images showed increased deep cerebellar and inferior rectus and superior oblique ocular muscles FDG uptake. This case is the first to visualize in vivo the possible underlying neuropathological mechanism of ocular flutter associated with cerebellar nuclei on functional imaging.

Objective We described a case of cerebral venous sinus thrombosis in a patient taking multiple supplements as part of a naturopathic anti-aging regimen. Methods Case report. Results The patient presented with a thalamic infarct associated with a thrombus in the vein of Galen. He reported no previous history of endocrinopathy and no known hypercoagulability risk factors. He was treated with therapeutic anticoagulation resulting in improvement. Diagnostic workup revealed hyperthyroidism and gonadotrophic deficiency attributed to significant supplement medicine usage. Conclusion This case highlights a potential risk of dietary supplements. Use of these supplements may be a risk factor for idiopathic cerebral venous thrombosis.

Serotonin syndrome is a potentially life-threatening condition described in all age groups. The diagnosis is clinically determined by either the Sternbach or the Hunter criteria. These criteria are based on recent serotonergic agent use and physical examination findings, including tremor, hyperreflexia, myoclonus, muscle rigidity, diaphoresis, nystagmus, or hyperthermia. The syndrome is an adverse reaction in which a drug is used alone or in combination with another drug resulting in increased centrally available serotonin. One such drug that has been shown to cause serotonin syndrome is the serotonergic-noradrenergic receptor reuptake inhibitor (SNRI) venlafaxine, which is commonly used for major depressive disorder (MDD). We report in this article serotonin syndrome caused by the combination of venlafaxine with a calcineurin inhibitor in two cases of transplant patients diagnosed with MDD. One patient was immunosuppressed with tacrolimus while the other was immunosuppressed with cyclosporine.

Background: Central pontine myelinolysis (CPM) is a demyelinating disorder most frequently described in alcoholism and malnourishment after rapid correction of hyponatremia affecting the pontine base. We

Rabies encephalitis is a uniformly fatal disease with several hypothesis proposed to explain pathogenesis and mortality. One such theory premises deficiency of neuronal nitric oxide synthetase deficiency causing selective cerebral vasospasm. We present transcranial Doppler findings in a case of rabies that did not show imaging evidence of vasospasm. Transcranial Doppler in two published cases have reported occurrence of global vasospasm. Our case showed no sonographic vasospasm in middle cerebral arteries but consistently increased distal resistance indices likely due to cerebral edema. While vasospasm may occur in rabies, cerebral edema secondary to disease or pharmacological interventions confounds transcranial Doppler readings. Cerebral angiogram and multimodality monitoring is encouraged.

A 54-year-old woman with 4 months of vomiting after gastric fundoplication presented with confusion, generalized weakness, anterograde amnesia, and ophthalmoplegia. Brain MRI showed hyperintensity in the posteromedial thalami (figure, A) and periaqueductal area (figure, B). Serum thiamine was 22 nmol/L (normal 70–180 nmol/L). Supplementation resulted in rapid improvement of ophthalmoplegia but slow improvement of confusion. Anterograde amnesia persisted despite 2 weeks of supplementation.

Objective: To identify inpatient variables that best differentiate acute strokes from stroke-mimics Background Identifying and triaging in-hospital strokes can be difficult but do represent a valuable treatment opportunity. We sought to systematically analyze symptoms, signs, and other characteristics of inpatient strokes with the hypothesis that focal neurological symptoms/deficits would best predict true strokes whereas altered mental status predict stroke-mimics. Design/Methods: We retrospectively analyzed prospectively collected inpatient stroke calls over one year (n=93) and retrospectively analyzed systematically collected data focusing on presenting symptoms, signs, stroke risk factors, and nursing unit location. We excluded patients admitted to a neurology service. Chi-square (categorical variables), t-test (continuous) and binary multiple logistic regression (multivariate analysis) were used to compare strokes versus stroke-mimics. Results: Fifty-seven (mean age 69.6 years; 54% female) of the 93 patients had a confirmed new stroke with 42.1% having an NIHSS 2 of model = 0.322]. 51.7% of the true strokes had atrial fibrillation at the time of stroke and 6.9% within 72 hours. Total NIHSS and laboratory values did not predict true stroke. Conclusions: The majority of acute inpatient stroke calls are for patients with true stroke. Most true stroke patients had atrial fibrillation, a complaint or finding of arm weakness, an NIH1b score of 0, and normal mental status. Disclosure: Dr. Newey has nothing to disclose. Dr. Wisco has nothing to disclose. Dr. George has nothing to disclose. Dr. Gebel has nothing to disclose.

A 43-year-old woman with a 2-year history of neuromyelitis optica (NMO; typical imaging [figure 1] and …