Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis presenting as acute neurological emergencies.

Background The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) is a self-limited benign disorder of unclear pathogenesis, with diverse clinical manifestations. Cases We report two unusual presentations of this entity. The first case developed a catastrophic picture, characterized by acute elevation of intracranial pressure, necessitating emergency life support. The second case presented with hyperacute-onset mixed aphasia and facial droop, masquerading as acute ischemia of the middle cerebral artery territory. Both patients made full recoveries. Conclusion The possibility of HaNDL should be considered in individuals presenting with unusual patterns of headaches and transient neurological symptoms. Our report will expand the spectrum of this disorder, which will help physicians not only to recognize the unusual manifestations of this rare disorder, but also consider potential therapeutic interventions.

Related or not? Development of spontaneous Creutzfeldt-Jakob disease in a patient with chronic, well-controlled HIV: A case report and review of the literature.

BACKGROUND: We report a novel case of a rare disease: spontaneous Creutzfeldt-Jakob disease in a patient with well-controlled HIV. We explore the relationship between spontaneous Creutzfeldt-Jakob disease and HIV. CASE REPORT: A 66-year-old man with long-standing, well-controlled HIV infection presented with 3 months of progressive, subacute neurocognitive decline. His symptoms included conceptual apraxia, apathy, memory impairment, and gait disturbance, and were initially attributed to depressive "pseudo-dementia." Unfortunately, the patient's symptoms rapidly progressed and he ultimately succumbed to his illness. Autopsy confirmed the clinical diagnosis of spontaneous Creutzfeldt-Jakob disease. DISCUSSION: This case highlights spontaneous Creutzfeldt-Jakob disease as a rare terminal illness in the setting of well-controlled chronic HIV. To our knowledge, this is the first report of a patient with chronic and previously well-controlled HIV infection dying from a prion disease. Despite the very different epidemiology and pathophysiology of HIV and spontaneous Creutzfeldt-Jakob disease, this case does raise questions of whether certain host genetic factors could predispose to both conditions, albeit currently, there is no clear causal link between HIV and spontaneous Creutzfeldt-Jakob disease.

Ondansetron-related hemorrhagic posterior reversible encephalopathy syndrome (PRES) following gastric bypass.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiographic syndrome formally recognized in 1996, which describes specific changes noted on neuroimaging thought to be related to impaired cerebral blood flow autoregulation and endothelial dysfunction. We report a case of PRES in the setting of increased ingestion of ondansetron; complicated by hemorrhagic transformation and refractory intracranial hypertension. We hypothesize an association of 5-HT3 antagonism and PRES. FINDINGS: This is a case study report; with review of previously published literature through PubMed search. We describe the case of a 25 year old man following bariatric surgery who increased his ingestion of ondansetron, taking up to 40 tablets/day due to excessive nausea and vomiting. The patient was hospitalized for progressively more severe headache of 1 week's duration. Computed tomography (CT) revealed bilateral cerebral edema in the parietal and occipital lobes in the setting of elevated blood pressure (BP). Three days into his admission, following improvement in his BP with oral anti-hypertensive but continued use of the ondansetron, the patient developed near complete blindness. CT head imaging revealed progression of the posterior cerebral edema and intraparenchymal hemorrhage. He was admitted to our ICU and despite supportive treatment, his neurological examination worsened while CT head imaging findings remained stable. Invasive multimodality monitoring revealed elevated intracranial pressure. The patient was aggressively treated and after a prolonged hospitalization and rehabilitation course, made a significant recovery. CONCLUSION: This case highlights a very rare potential neurological complication of ondansetron, a commonly used medication. We hypothesize an underlying association between PRES and 5-HT3 antagonism, via the latter's potential role in endothelial dysfunction. Prompt recognition and treatment of PRES is essential, in order to prevent secondary cerebral injury and the associated potentially grave consequences.