Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Wernicke encephalopathy and Korsakoff psychosis are two facets of the same disease with well-determined cerebral lesions caused by thiamine deficiency. The disease occurs mainly in alcoholics, but other conditions (malabsorption or severe malnutrition) also predispose to the risk of Wernicke-Korsakoff syndrome. The incidence in Denmark is unknown. In the period 1.1.1979-31.12.1988, 24 patients (18 men and six women) were discharged from Rigshospitalet with the diagnosis Wernicke encephalopathy or Korsakoff psychosis. This represents about 0.05% of all admissions in the period. Eighteen out of the 24 cases (75%) were admitted in the past three years (1986-88). The mean age was 55 years. Twenty patients admitted alcohol abuse. The presenting symptoms and the patients' complaints showed great variety and were often related to other alcoholic complications, which could mask the disease. The classic symptom combination: eye movement abnormalities- ataxia and disorders of consciousness were found in seven patients (29%). Sixteen patients had disorders of consciousness or orientation. All the patients were treated with thiamine. The eye-movement disorder has recovered in eight out of ten known cases (80%), nystagmus--in six out of seven cases (86%) while ataxia, disorders of orientation and confabulation recovered in about 50% of cases. The average duration of hospitalisation was 50 days. Altogether nine patients died during the observation period. The condition is most probably underdiagnosed and the traditional diagnostic criteria are considered too rigid. The diagnosis should be considered in alcoholics who present even only one of the classical symptoms and in patients with alcohol dementia. Thiamine should be given on wide indications.
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PMID:[Wernicke-Korsakoff syndrome at the Rikshospitalet in 1979-1988. A retrospective study]. 192 15

Thiamin is one of the marginally adequate nutrients in the Australian diet. The incidence and prevalence of Wernicke-Korsakoff syndrome in this country may be the highest in the world. Homeless men could be at risk for low intakes of thiamin in association with irregular high alcohol intakes. A sample of 107 homeless men from two hostels and one clinic for homeless persons in inner Sydney was investigated for nutritional status; their thiamin status is reported here. By means of 24-hour recall methods, their mean dietary thiamin intake--0.76 mg per day--was less than the National Health and Medical Research Council's recommended dietary intake of 1.1 mg per day; at 0.076 mg/MJ it was even less than the minimal requirement of 0.08 mg/MJ. It was much lower than the mean intake of 1.38 mg per day that was found in the 1983 National Dietary Survey of adults and the distribution of thiamin intakes in this study was skewed positively, with the largest intake being in the range of 0-0.1 mg per day. On clinical examination we found a high prevalence of signs that were consistent with thiamin deficiency. Twenty-four per cent of the subjects showed three-or-more of the signs of the Wernicke-Korsakoff syndrome (ophthalmoplegia, nystagmus, ataxia, peripheral neuropathy and global confusion). In assaying for red-cell transketolase levels, this subgroup showed higher thiamin pyrophosphate effects than did the whole sample. Thirty-six per cent of the whole sample showed subnormal thiamin status by the thiamin pyrophosphate effect. Thus, in this sample, homeless men showed a high prevalence of dietary, biochemical and clinical features to indicate subclinical or early clinical thiamin deficiency.
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PMID:Thiamin status of a sample of homeless clinic attenders in Sydney. 204 82

The Wernicke-Korsakoff syndrome is induced by thiamine deficiency. It occurs mainly in subjects with chronic alcoholism. It begins with an acute phase (Wernicke's encephalopathy) and changes to a chronic phase, which is characterized by a symptomatology varying from subclinical findings to a fully developed Korsakoff psychosis. We examined otoneurologically eight patients with Wernicke-Korsakoff syndrome. The examination included: clinical status, caloric stimulation, optokinetic nystagmus, pure tone audiogram (air- and bone conduction), speech audiogram, and brain-stem auditory evoked potentials. The typical findings of the chronic phase are: gaze nystagmus and reduced gain of the optokinetic nystagmus. In five of seven patients OK gain was reduced by more than two standard-deviations. The other examinations including brain-stem potentials did not reveal any specific findings. The symptomatology of the acute phase is well known: nystagmus (mostly lateral gaze nystagmus) and reduced by caloric responses. Early diagnosis of a Wernicke-Korsakoff syndrome is of great importance since its treatment is simple and efficient. The results of a thorough neuro-otologic examination are of considerable diagnostic value, particularly during the chronic phase of the disease.
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PMID:[Neuro-otologic findings in the Wernicke-Korsakoff syndrome]. 685 27

A 35-year-old woman developed upbeating nystagmus while recovering from presumed Wernicke-Korsakoff syndrome. The upbeating nystagmus changed to downbeating nystagmus with convergence. The slow-phase velocity of the downbeating nystagmus varied with the convergence effort. We believe that this unusual nystagmus pattern implies convergence input to the supranuclear centers for vertical gaze.
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PMID:Upbeat nystagmus changing to downbeat nystagmus with convergence. 719 14

Wernicke's encephalopathy and Korsakoff's psychosis represent a continuum of the same pathologic process. The etiology is an absolute deficiency of thiamine rather than a direct toxic effect of alcohol. The triad of Wernicke's encephalopathy--global confusional state, ophthalmoplegia and nystagmus, and ataxia--is occasionally seen in chronic alcoholics and is often attenuated by immediate thiamine treatment. The triad of Korsakoff's psychosis--memory loss, learning deficits and confabulation--may be seen in either the acute or the long-term care setting.
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PMID:The Wernicke-Korsakoff syndrome. 742 67

A 25-year-old woman suffered from hyperemesis gravidarum when she was seven weeks pregnant. Since her vomiting continued, she received intravenous dextrose and electrolytes without thiamine in a hospital. One month later, she developed gait disturbance, followed by confusion and dysarthria. On admission to our department, she was confusional and had ataxic dysarthria. Spontaneous and gaze evoked nystagmus was present. Limb coordination was bilaterally ataxic. Based on her clinical course and symptoms, she was diagnosed as having Wernicke's encephalopathy. From the admission day, intravenous infusion of vitamin B1 (600 mg/day) was started. A few days later, her consciousness and limb ataxia began to improve. However, truncal ataxia and polyneuropathy became evident. Eight weeks after onset, she developed Korsakoff's psychosis such as anterograde and retrograde amnesia, disorientation and confabulation. We administered large amounts of corticosteroid (methylprednisolone 500 mg/day) in order to reduce brain edema or stabilize the impaired blood-brain barrier. Soon after, her psychosis began to improve gradually. She recovered remarkably from the psychosis, but she was left with persistent nystagmus, mild ataxic gait and polyneuropathy. The present case suggests that corticosteroid may have the beneficial effect on Wernicke-Korsakoff syndrome.
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PMID:[Beneficial effect of steroid pulse therapy on Wernicke-Korsakoff syndrome due to hyperemesis gravidarum]. 795 22

A female alcoholic presented with Wernicke's encephalopathy subsequent to administration of diazepam and glucose (without thiamine) for treatment of withdrawal seizures. Nystagmus and cerebellar ataxia quickly resolved when administered thiamine, although severe global amnesia consistent with Korsakoff's syndrome persisted. Magnetic resonance imaging (MRI) revealed infarction of the right temporal lobe with hippocampal atrophy, but no lesions of thalamus or atrophy of mammillary bodies. Positron emission tomography (PET) confirmed decreased cerebral metabolic rates for glucose (CMRglu) in the right temporal lobe corresponding to MRI findings, but also significant metabolic asymmetry of dorsal thalamus, i.e. reduced CMRglu in left versus right. This patient is unique in that neuroradiological findings revealed intact mammillary bodies and suggest asymmetrical dysfunctions (structural right temporal and functional left diencephalic) to produce her profound amnesia.
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PMID:Severe global amnesia presenting as Wernicke-Korsakoff syndrome but resulting from atypical lesions. 868 98

Cytomegalovirus ventriculoencephalitis (CMV-VE) is a devastating opportunistic infection seen most frequently in patients with AIDS. The authors describe eight patients with AIDS and CMV-VE, who developed the clinical features of the Wernicke-Korsakoff syndrome, including impaired memory, confabulation, nystagmus, ophthalmoplegia, and ataxia. CMV-VE is perhaps a more frequent cause of the Wernicke-Korsakoff syndrome than traditional associations.
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PMID:Cytomegalovirus ventriculoencephalitis presenting as a Wernicke's encephalopathy-like syndrome. 1113 96

A 10-year-old girl with M2 acute myeloid leukemia underwent an unrelated cord blood transplantation in refractory first relapse. On day +13, after 48 hours with fever, she showed a measles-like rash, and on day +15, she began experiencing neurologic symptoms (headache, tremors, weakness, nystagmus, mild confusion, speaking, taste, and behavior disturbances, and focal seizures). She also had amnesia for recent events with disability to learn, mimicking Wernicke-Korsakoff syndrome. Computed tomography of the brain and cerebrospinal fluid (CSF) and electroencephalogram were nonspecific. We found human herpesvirus 6 (HHV-6) DNA in CSF and cytomegalovirus in bronchoalveolar lavage using polymerase chain reaction techniques. Treatment with ganciclovir and foscarnet was effective, with total resolution of symptoms.
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PMID:Herpesvirus-6 encephalitis complicated by Wernicke-Korsakoff syndrome in a pediatric recipient of unrelated cord blood transplantation. 1190 11

Beri-Beri is caused by vitamin B1 (thiamine) deficiency. Thiamine is essential for carbohydrate metabolism and the generation of energy. Depending on age and calorie intake, 1-1.5mg/day are required with a 50% increase during pregnancy and lactation. Fever and increased muscular activity will also increase thiamine requirements (storage in muscles is limited, and reserves are quickly depleted). The sources of thiamine are meat, the outer layer of cereal grains and pulses, nuts, and leafy vegetables. The vitamin is lost during milling and processing and during excessive cooking. Beri-beri takes 2 forms: wet beri-beri which has a high output biventricular failure with edema associated with profound peripheral vasodilation and tachycardia (this also occurs in an acute fulminating form known as shoshin beri-beri) and dry beri-beri with symptoms of peripheral neuropathy with taxia, weakness, paraesthesia, and patchy sensory loss with areflexia. In this form, foot and/or wrist drop may occur. Thiamine deficiency can also produce Wernicke-Korsakoff psychosis characterized by vomiting, horizontal nystagmus, ophthalmoplegia, memory loss, and confabulation. Wet beri-beri is a medical emergency treated by intravenous administration of thiamine for several days. 38 patients (27 men and 11 women) were identified with beri-beri in urban Banjul in the Gambia. 14 had wet beri-beri, 11 a mixed presentation, and 13 dry beri-beri. Most of the patients were disabled for many months. Risk factors were pregnancy, alcohol consumption, fever, exercise, diabetes, and dysentery. 4 of the patients died (2 were in the last trimester of pregnancy). The staple diet in urban areas of the Gambia is imported, polished white rice in a groundnut- or oil-based sauce with fish and vegetables such as peppers, onions, and tomatoes. Meat is too expensive for the urban poor, and fruit and vegetable consumption is highly seasonal and income-dependent. There is little chance that this diet will be changed for the 46% of the population who live in urban areas. It is likely that a substantial proportion of the population has subclinical thiamine deficiency and are at risk of beri-beri. Since thiamine added to imported rice will be destroyed by traditional means of cooking, adding the vitamin to wheat flour may be an appropriate public health measure.
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PMID:Beri-beri: "Endemic amongst urban Gambians". 1231 72


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