Project description:A patient with rapidly changing mental status responded to treatment with intramuscular or IV thiamine but not oral dosages, suggesting that presentation of thiamine deficiency can be highly variable, which can complicate the correct diagnosis and treatment.
Project description:Background and objectivesLesions of the cerebellar flocculus cause enduring downbeat nystagmus (DBN) with unrelenting oscillopsia. Unlike most patients with DBN, the flocculus is structurally spared in nonalcoholic Wernicke encephalopathy (nWE) with chronic DBN. The objective was to study the effects of alcohol in nWE.MethodsWe recorded eye movements of a unique patient with nWE under controlled alcohol consumption who said his oscillopsia disappeared with a few drinks of alcohol.ResultsHis DBN was markedly diminished by alcohol (by 77.4%), although he remained alert with normal saccades.DiscussionThis striking observation may be caused by the differential effect of alcohol on the perihypoglossal complex and the paramedian tract neurons, which control the level of activity in the flocculus, with opposite (inhibition and excitation, respectively) effects. The finding suggests new ideas about the treatment and pathophysiology of DBN with a structurally intact cerebellum.
Project description:Graft-versus-host disease (GVHD) is an immune triggered process leading to severe immune dysregulation and organ dysfunction until death and it is one of the worst medical complications after a transplant. Patients with GVHD may have several neurological alterations: during this acute severe phase there is coexistence of various and nonspecific neurological symptoms. We are reporting a case of a 53 year old woman with severe GVHD after bone marrow transplant with acute neurological signs and symptoms. MRI study showed findings consistent with Posterior reversible encephalopathy syndrome and Wernicke encephalopathy.
Project description:Wernicke encephalopathy (WE) is an acute life-threatening neurological condition caused by thiamine (vitamin B1) deficiency. Patients with WE often present with a triad of symptoms consisting of ophthalmoplegia, gait ataxia, and mental confusion. If WE is not treated in a timely manner, it can lead to serious complications such as confusion, coma, or death. Although alcohol abuse is the most commonly reported cause of WE, nonalcoholic causes-although rare-do exist. Herein, we present the case of a nonalcoholic woman with medullary infarctions who presented with intractable vomiting. Her clinical state subsequently progressed to include ophthalmoplegia and gait ataxia. A diagnosis of WE was suspected based on her clinical presentation; this was confirmed by brain magnetic resonance imaging (MRI) and the finding of decreased serum thiamine levels. Brain magnetic resonance imaging demonstrated the complete resolution of abnormal hyperintensities during a follow-up visit, 6 months after treatment.
Project description:Non-encephalopathic presentations of CNS thiamine deficiency may be difficult to diagnose. We describe neuro-otologic findings of Wernicke syndrome in 5 patients with vestibular manifestations. Diagnosis was confirmed by low serum levels, response to replacement, and brain MRI to exclude other causes. All had bilaterally abnormal horizontal head impulse vestibulo-ocular reflex (VOR) responses and pathologic gaze-evoked nystagmus, without encephalopathy. After thiamine replacement, 4 had total resolution of vestibular and oculomotor findings. Novel findings included 2 patients whose VOR function improved within minutes of IV repletion and 1 whose recovery was documented by serial quantitative recordings. Early diagnosis of Wernicke by examining vestibular reflexes and prompt IV treatment might prevent encephalopathy and other neurologic or systemic complications of thiamine depletion.
Project description:Background and purposeWernicke encephalopathy is a severe neurologic disorder that results from a dietary vitamin B1 deficiency. It is characterized by changes in consciousness, ocular abnormalities, and ataxia. This study was undertaken to analyze and compare findings on MR imaging and neurologic symptoms at clinical presentations of patients with Wernicke encephalopathy with and without a history of alcohol abuse.Materials and methodsA multicenter study group retrospectively reviewed MR brain imaging findings, clinical histories, and presentations of 26 patients (14 female, 12 male) diagnosed between 1999 and 2006 with Wernicke encephalopathy. The age range was 6-81 years (mean age, 46 .6+/-19 years).ResultsFifty percent of the patients had a history of alcohol abuse, and 50% had no history of alcohol abuse. Eighty percent showed changes in consciousness, 77% had ocular symptoms, and 54% had ataxia. Only 38% of the patients showed the classic triad of the disease at clinical presentation. At MR examination, 85% of the patients showed symmetric lesions in the medial thalami and the periventricular region of the third ventricle, 65% in the periaqueductal area, 58% in the mamillary bodies, 38% in the tectal plate, and 8% in the dorsal medulla. Contrast enhancement of the mamillary bodies was statistically positively correlated with the alcohol abuse group.ConclusionsOur study confirms the usefulness of MR in reaching a prompt diagnosis of Wernicke encephalopathy to avoid irreversible damage to brain tissue. Contrast enhancement in the mamillary bodies is a typical finding of the disease in the alcoholic population.
Project description:BackgroundWernicke-Korsakoff Syndrome (WKS) is a neuropsychiatric syndrome caused by thiamine deficiency. Cancer predisposes to thiamine deficiency through various mechanisms. Although many case reports exist on nonalcoholic WKS in cancer, larger qualitative studies are lacking.MethodRetrospective study of patients admitted to a cancer hospital and diagnosed with WKS during routine care on a psychiatric consultation service. Only patients with at least 1 additional supporting feature (magnetic resonance imaging findings, low serum thiamine concentrations, or response to treatment) were included. Data pertaining to demographics, risk factors, phenomenology, and outcomes were abstracted from medical records by chart review.ResultsIn all, 18 patients were included. All patients developed WKS during cancer treatment. Hematologic malignancy, gastrointestinal tract tumors, low oral intake, and weight loss were common risk factors. All patients presented with cognitive dysfunction, most commonly impaired alertness, attention, and short-term memory. All were diagnosed by operational criteria proposed by Caine et al., 1997 (where 2 of the following are required: nutritional deficiency, ocular signs, cerebellar signs, and either altered mental status or mild memory impairment). Few exhibited Wernicke's classic triad. Diagnostic and treatment delay were common. Only 3 patients recovered fully.ConclusionNonalcoholic WKS can occur during cancer treatment and manifests clinically as delirium. Diagnosis should be made using operational criteria, not Wernicke's triad. Most patients were not underweight and had normal serum concentration of vitamin B12 and folate. A variety of mechanisms might predispose to thiamine deficiency and WKS in cancer. Given the high frequency of residual morbidity, studies should focus on decreasing diagnostic and treatment delay.
Project description:BACKGROUND:Little is known regarding paediatric medical emergency calls to Danish Emergency Medical Dispatch Centres (EMDC). This study aimed to investigate these calls, specifically the medical issues leading to them and the pre-hospital units dispatched to the paediatric emergencies. METHODS:We performed a retrospective, observational study on paediatric medical emergency calls managed by the EMDC in the Region of Southern Denmark in February 2016. We reviewed audio recordings of emergency calls and ambulance records to identify calls concerning patients ≤ 15 years. We examined EMDC dispatch records to establish how the medical issues leading to these calls were classified and which pre-hospital units were dispatched to the paediatric emergencies. We analysed the data using descriptive statistics. RESULTS:Of a total of 7052 emergency calls in February 2016, 485 (6.9%) concerned patients ≤ 15 years. We excluded 19 and analysed the remaining 466. The reported medical issues were commonly classified as: "seizures" (22.1%), "sick child" (18.9%) and "unclear problem" (12.9%). The overall most common pre-hospital response was immediate dispatch of an ambulance with sirens and lights with a supporting physician-manned mobile emergency care unit (56.4%). The classification of medical issues and the dispatched pre-hospital units varied with patient age. DISCUSSION:We believe our results might help focus the paediatric training received by emergency medical dispatch staff on commonly encountered medical issues, such as the symptoms and conditions pertaining to the symptom categories "seizures" and "sick child". Furthermore, the results could prove useful in hypothesis generation for future studies examining paediatric medical emergency calls. CONCLUSION:Almost 7% of all calls concerned patients ≤ 15 years. Medical issues pertaining to the symptom categories "seizures", "sick child" and "unclear problem" were common and the calls commonly resulted in urgent pre-hospital responses.