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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A young woman who underwent gastric bypass surgery for morbid obesity had intractable nausea and vomiting for several weeks postoperatively, leading to poor intake and excessive weight loss. In the ninth postoperative week, she became confused and off balance and reported blurred and double vision. Examination disclosed slow saccades, nystagmus, and impaired abduction of both eyes as well as memory loss and ataxia. Visual acuity was slightly subnormal, and ophthalmoscopy disclosed a thickened and telangiectatic peripapillary nerve fiber layer with retinal hemorrhages. MRI showed high T2 and FLAIR signal in the dorsomedial thalamus and mamillary bodies bilaterally, substantiating a clinical diagnosis of Wernicke encephalopathy (WE). After thiamine treatment, visual acuity returned to normal and eye movements and alignment almost completely normalized. Fundus abnormalities eventually regressed. Although the ocular motor findings of WE have been well documented, the ophthalmoscopic findings have not. Resembling the findings in Leber hereditary and toxic optic neuropathies, they may represent manifestations of impaired mitochondrial function in retinal ganglion cells and capillaries. Recognition that these ophthalmoscopic findings may occur in WE is important to avoid procedures such as lumbar puncture that may delay urgent treatment with thiamine.
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PMID:Peripapillary nerve fiber layer thickening, telangiectasia, and retinal hemorrhages in wernicke encephalopathy. 2018 9

A 75-year-old man with diabetic nephropathy treated with hemodialysis visited to a medical office because of slight fever, and received intravenous glucose infusion without any vitamins. Thereafter, he noticed gait disturbance and began to tell inconsistent stories. He was admitted to our hospital due to aggravation of these symptoms. On admission, he was disoriented and not able to sit by himself because of severe truncal ataxia without weakness. He had also gaze direction nystagmus. Based on clinical features, we considered him as having Wernicke's encephalopathy (WE) and treated him with 100 mg thiamine per day. The thiamine supply diminished these symptoms soon. Plasma thiamine level prior to the administration was 7 ng/ml, which confirmed the diagnosis. MRI did not disclose any abnormalities frequently seen in WE. WE is a life-threatening disease, and 'early detection, early cure' is important for recovering without sequelae. The thiamine deficiency is often seen in dialysis patients because of dietary restrictions as well as its loss during dialysis. This case gives us the caution; when hemodialysis patients present acute/subacute gait disturbance and/ or abnormal mental state, we should consider WE. Furthermore, high-risk patients, such as elderly patients under hemodialysis may need some supplement including thiamine even at preclinical stage.
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PMID:[Wernicke encephalopathy in a non-alcoholic patient with diabetic nephropathy under hemodialysis]. 2059 67

Wernicke's encephalopathy (WE) is caused by thiamine (vitamin B1) deficiency and most commonly found in individuals with chronic alcoholism and malnutrition. Clinically, its key features are mental status disorders and oculomotor abnormalities as well as stance and gait ataxia. The diagnosis of WE is frequently missed although delay of appropriate treatment can lead to death or Korsakoff's amnestic syndrome. It is therefore crucial in suspected cases of WE, not to await confirmation of diagnosis, but immediately administer high-dose intravenous thiamine and simultaneously treat magnesium deficiency. Alcoholics at risk of WE should on admission receive immediate prophylactic therapy with parenteral thiamine.
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PMID:[Wernicke's encephalopathy in chronic alcoholics]. 2121 96

The purpose of this pictorial essay is to present the computed tomography (CT) and magnetic resonance imaging (MRI) findings of Wernicke's encephalopathy, a rare, severe, acute neurological syndrome due to thiamine (vitamin B1) deficiency, associated with high morbidity and mortality. The classical clinical triad, which includes ocular signs, altered consciousness and ataxia, can be found in only one-third of patients. Although chronic alcoholic patients are the most commonly affected, Wernicke's encephalopathy may complicate malnutrition conditions in nonalcoholic patients, in whom it is greatly underestimated. CT and above all MRI of the brain play a fundamental role in diagnosing the condition and ruling out other diseases. MRI is the most sensitive technique and is required in all patients with a clinical suspicion of Wernicke's encephalopathy. Medial thalami, mamillary bodies, tegmentum, periaqueductal region, and tectal plate are typical sites of abnormal MRI signal. The dorsal medulla, red nuclei, cranial nerve nuclei, cerebellum, corpus callosum, frontal and parietal cerebral cortex are less common sites of involvement although they are more frequently affected in nonalcoholic patients. Paramagnetic contrast material may help to identify lesions not otherwise visible.
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PMID:CT and MRI of Wernicke's encephalopathy. 2122 66

Wernicke's encephalopathy is caused by thiamine deficiency, and is characterized by acute mental confusion, ataxia, and ophthalmoplegia. It is also a rare neurologic complication of hematopoietic stem cell transplantation (HSCT). However, because of its rare incidence, Wernicke's encephalopathy can easily be overlooked in HSCT patients, and a few misleading steps in the early stage of the disease may result in permanent neurologic disability or even mortality. We recently encountered a case of Wernicke's encephalopathy in a patient who underwent allogeneic HSCT. Based on our own experience and previously published documents, we suggest early radiologic surveillance and treatment for patients with findings compatible with Wernicke's encephalopathy following HSCT.
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PMID:Wernicke's encephalopathy following allogeneic hematopoietic stem cell transplantation. 2125 32

Wernicke's encephalopathy is an acute neurological syndrome due to thiamine deficiency, which is characterized by a typical triad of mental status changes, oculomotor dysfunction and ataxia. Despite the fact that Wernicke's encephalopathy, in developed countries, is frequently associated with chronic alcoholism, there have been a number of published cases associating this encephalopathy with parenteral feeding without vitamin supplementation. Diagnosis is primarily a clinical one, and can be supported by laboratory tests and imaging studies; treatment should start as soon as possible, for the morbidity and mortality (almost 20%) associated with this syndrome is high. Thiamine supplementation, along with other vitamins, is recommended for patients in risk of developing this syndrome.
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PMID:Wernicke's encephalopathy induced by total parental nutrition. 2151 77

Wernicke-Korsakoff syndrome (also called Wernicke's encephalopathy) is a potentially fatal, neuropsychiatric syndrome caused most frequently by thiamine deficiency. The three classic symptoms found together are confusion, ataxia and eyeball manifestations. Memory disturbances can also be symptoms. Wernicke's encephalopathy mainly results from alcohol abuse, but also from malnutrition, cancer, chronic dialysis, thyrotoxicosis and, in well-founded cases, encephalopathy associated with autoimmune thyroid disease (EAATD). The coexistence of many factors makes a proper diagnosis difficult, delays appropriate treatment and consequently reduces the chance of complete recovery. We present the case of a 53 year-old female with Wernicke's encephalopathy caused by chronic malnutrition, surgical operation, as well as thyrotoxicosis. She received treatment with intravenous thiamine administration and also anti-thyroid treatment which caused satisfactory regression of her neurological symptoms.
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PMID:Wernicke-Korsakoff syndrome in the course of thyrotoxicosis - a case report. 2152 81

Wernicke's syndrome, caused by thiamine deficiency, is most commonly associated with alcoholism but can also occur in patients who are malnourished or have malabsorption of nutrients for other reasons. Since the classic triad of encephalopathy, nystagmus and ataxia occurs simultaneously in only 10-33% of cases, a high index of suspicion is needed in any patient with confusion and memory loss. In this case report, we present a 56-year-old female patient with metastatic colon cancer complicated with enterocutaneous fistula. She developed Wernicke's encephalopathy precipitated by 5-fluorouracil infusion. Replacement with thiamine rapidly reversed her neurologic symptoms and signs.
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PMID:Wernicke's Encephalopathy in Colon Cancer. 2153 79

Imaging techniques including computed tomography (CT), magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT), and positron emission tomography (PET) have been widely applied to the investigation of patients with acute or chronic ataxias. Fundamentally, CT has a role in the emergency evaluation of the patient with acute ataxia to ascertain brainstem or cerebellar hemorrhage and to exclude a mass lesion in the posterior cranial fossa. Conventional MRI is the most frequently performed imaging investigation in patients with ataxia. It can support the diagnosis of acute cerebellitis and Wernicke encephalopathy by revealing T2 signal changes with a typical distribution. In patients with inherited or sporadic chronic ataxia it reveals three fundamental patterns of atrophy of the brainstem, cerebellum, and spinal cord which match the gross neuropathological descriptions. These are represented by olivopontocerebellar atrophy (OPCA), cortical cerebellar atrophy (CCA), and spinal atrophy (SA). A substantial correspondence exists among these patterns of atrophy shown by MRI and the etiological classification of inherited or acquired chronic ataxias. This, along with demonstration of T2 signal changes characteristic of some diseases, makes conventional MRI potentially useful for the diagnostic work-up of the single patient, especially in the case of a sporadic disease. Non-conventional MR techniques including diffusion MR, spectroscopy, and functional MR have been used in patients with acute or chronic ataxia, but their exact role in the evaluation of the single patient is not established yet. They are currently investigated as potential tools to monitor progression of neurodegeneration in chronic ataxia and to serve as "surrogate markers" in clinical trials. Several radiotracers have been utilized in combination with SPECT and PET in patients with ataxia. Perfusion SPECT can reveal cerebellar blood flow abnormalities early in the course of cerebellitis. It has also been utilized to investigate perfusion of the brain in several inherited or sporadic chronic ataxic diseases, contributing to improved understanding of the pathophysiology of these conditions. Recently, perfusion SPECT has been tested as a "surrogate marker" to verify the effects of newly developed therapies in patients with a variety of chronic ataxias. Whole-body FDG-PET is recommended in patients with suspected paraneoplastic cerebellar degeneration to detect the primary malignancy. Brain FDG-PET has provided important information on the pathophysiology of several acquired and inherited conditions. PET and SPECT with radiotracers able to assess the nigrostriatal system or the density of D2 dopamine receptors in the striatum are increasingly used in patients with adult-onset sporadic ataxia for the differential diagnosis between multiple system atrophy in which overt striatal abnormalities are found and idiopathic late-onset cerebellar ataxia in which no abnormality is detected.
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PMID:Magnetic resonance and nuclear medicine imaging in ataxias. 2182 82

A 67-year old male was brought to the hospital by his family because he had been suffering from somnolence, bradypsychia and gait disturbance for one week. He lived alone, reported an ethanol intake higher than 100-120 g/day. His diet was limited in quality and amount. The physical examination showed stigmata of chronic liver disease. The neurological exam revealed right-side cerebellar tremor, bilateral dysmetria and gait ataxia as well as hyporeflexia in the lower limbs. He was diagnosed of Wernicke encephalopathy. How should this patient be evaluated and treated?
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PMID:[Wernicke encephalopathy in alcoholic patients]. 2252 55


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