Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a patient who experienced a postoperative Wernicke encephalopathy 8 days after a left hepatectomy performed for metastasis related to a rectal cancer. During the six months before surgery the patient lost 10 kg of weight (15%). Moreover, in the postoperative period the patient received exclusively 5% dextrose solution intravenously. On the 8th postoperative day, an alteration of consciousness, a vertical nystagmus and an ataxia led to consider the diagnosis of thiamine deficiency that was then established by the decrease in the transcetolase activity of the red blood cells. Vitamin B1 supply improved the clinical status rapidly and completely. This observation allows to review aetiologies and clinical forms of thiamine shortage. In addition, it stresses the detection of exposed patients and the prevention methods.
 ...
PMID:[Postoperative encephalopathies: thiamine deficiency, an unrecognized etiology]. 1123 77

We report the case of a 54-year-old alcoholic female patient who was hospitalized for neurologic alterations along with a severe hyponatremia (plasma Na+: 97 mEq/l). She suffered from potomania and was given, a few days before admission, a thiazide diuretic for hypertension. A careful correction of plasma Na+ levels was initiated over a 48-hour period (rate of correction < 10 mEq/l/24h) in order to avoid brain demyelination. After a 2-day period of clinical improvement, her neurologic condition started to deteriorate. By the 5th day of admission, she became tetraplegic, presented pseudobulbar palsy, ataxia, strabism, extrapyramidal stiffness and clouding of consciousness. Scintigraphic and MRI investigations demonstrated pontine and extrapontine lesions associated with Gayet-Wernicke encephalopathy. After correction of ionic disorders (hyponatremia, hypokaliemia) and vitamin B (thiamine) deficiency, the patient almost completely recovered without notable disabilities. This case illustrates that profound hyponatremia, in a paradigm of slow onset, can be compatible with life. It also demonstrates that demyelinating lesions, usually considered as a consequence of a too fast correction of hyponatremia, may occur despite the strict observance of recent guidelines. There is increasing evidence to suggest that pontine swelling and dysfunction may sometimes occur in alcoholic patients even in absence of disturbance in plasma Na+ levels. It is therefore of importance, while managing a hyponatremic alcoholic patient, to identify additional risk factors (hypokaliemia, hypophosphoremia, seizure-induced hypoxemia, malnutrition with vitamin B deficiency) for brain demyelination and to correct them appropriately.
 ...
PMID:Central and extrapontine myelinolysis in a patient in spite of a careful correction of hyponatremia. 1177 Aug 3

We present a case of a 25-year-old woman with drowsiness, nystagmus, severe ataxia and areflexia, which developed six weeks after admission to an obstetric clinic for hyperemesis gravidarum. She had been treated with intravenous dextrose and electrolyte solutions and antiemetics. Magnetic resonance imaging (MRI) performed on the fifth day of her neurologic symptoms showed increased intensity in both thalami, periaqueductal grey matter, the floor of the fourth ventricle and superior cerebellar vermis in T2 weighted and FLAIR images. Clinical signs and MRI findings were consistent with the diagnosis of Wernicke's encephalopathy. On the third day of thiamine replacement, neurologic signs improved dramatically In addition to our case, we review 29 previously reported cases of Wernicke's encephalopathy associated with hyperemesis gravidarum, and emphasize the importance of thiamine supplementation to women with prolonged vomiting in pregnancy especially if they are given intravenous or parenteral nutrition.
 ...
PMID:Wernicke's encephalopathy due to hyperemesis gravidarum: an under-recognised condition. 1178 26

A report of 33 years old female with Wernicke's encephalopathy is presented. The disease was secondary to hyperemesis gravidarum, started from the 6th week of pregnancy. Neurological symptoms as nystagmus, headache, vertigo, disturbance of consciousness and ataxia are described, as well as difficulties in finding out the right diagnosis. We present the therapy with vitamin B1. The subsequent course of the pregnancy was uncomplicated and resulted in a birth--by caesarean section of a healthy male infant, weighted 2790 grams. However, four months later after the delivery, the patient is not yet completely recovered and still demands thiamine supplementation.
 ...
PMID:[Wernicke's encephalopathy due to hyperemesis gravidarum]. 1453 43

Wernicke's encephalopathy is a serious neurologic disorder caused by vitamin-B1 or thiamine deficiency. The classical triad of clinical symptoms described by Wernicke (gait ataxia, ophthalmoplegia, and confusion) are found in only a third of patients upon initial examination. Typical findings upon MR imaging in patients with Wernicke's encephalopathy are well documented, with signal intensities in the medial thalami and periaqueductal regions of the midbrain. We report a case of Wernicke's encephalopathy revealing an unusual contrast enhancement. It is therefore important to note that the acute stage of Wernicke's encephalopathy may be associated with an intense contrast enhancement upon MR-imaging reflecting the disruption of the blood-brain barrier and inflammatory processes caused by thiamine deficiency. As a consequence from the guideline for managing Wernicke's encephalopathy by the Royal College of Physicians early B-vitamin treatment in suspected is recommended cases.
 ...
PMID:Wernicke's encephalopathy: unusual contrast enhancement revealed by magnetic resonance imaging. 1464 3

Postoperative complications and nutritional deficits resulting from bariatric surgery can lead to severe vitamin-deficiency states, such as Wernicke's encephalopathy (WE). Patients with acute WE generally present with the classic clinical triad of inattentiveness, ataxia, and ophthalmoplegia. We describe a patient who presented with acute WE at 2 months after laparoscopic bariatric surgery. Initial MRI of the brain demonstrated the characteristic injuries of WE, and repeat imaging showed resolution after 4 months of thiamine supplementation, at which time the patient had normal gait but persistent memory deficits. Even with early recognition and aggressive therapy, acute WE commonly results in permanent disability due to the irreversible cytotoxic effects on specific regions of the brain. Since the clinical onset of acute WE follows a predictable time-course in post-bariatric surgery patients with malnutrition, we recommend prevention by administration of parenteral thiamine beginning at 6 weeks postoperatively in malnourished patients.
 ...
PMID:Acute Wernicke's encephalopathy following bariatric surgery: clinical course and MRI correlation. 1498 48

Wernicke encephalopathy consists of a triad of ophthalmoplegia, ataxia, and altered mental status. It is caused by thiamine deficiency and although it is commonly seen in alcoholics, patients undergoing gastric bypass surgery for morbid obesity could be a new group of patients to watch for. Florid papilloedema and optic neuropathy are not commonly seen but are still consistent with the diagnosis of Wernicke encephalopathy. The most striking feature remains the dramatic recovery after replacement of thiamine.
 ...
PMID:You are what you eat. 1596 92

Complexins are presynaptic proteins that bind to the SNARE complex where they modulate neurotransmitter release. A number of studies report changes in complexins in psychiatric (schizophrenia and depression) and neurodegenerative disorders (Huntington's disease, Wernicke's encephalopathy and Parkinson's disease). Here, we characterize the behavioural phenotype of Cplx1 knockout (Cplx1-/-) mice. Cplx1-/- mice develop a strong ataxia in the absence of cerebellar degeneration. Although originally reported to die within 2-4 months after birth, when reared using an enhanced feeding regime, these mice survive normally (i.e. >2 years). Cplx1-/- mice show pronounced deficits in motor coordination and locomotion including abnormal gait, inability to run or swim, impaired rotarod performance, reduced neuromuscular strength, dystonia and resting tremor. Although the abnormal motor phenotype dominates their overt symptoms, Cplx1-/- mice also show other behavioural deficits, particularly in complex behaviours. They have deficits in grooming and rearing behaviour and show reduced exploration in several different paradigms. They also show deficits in tasks reflecting emotional reactivity. They fail to habituate to confinement and show a 'panic' response when exposed to water. The abnormalities seen in the behaviour of Cplx1-/- mice reflect those predicted from the distribution of complexin I in the brain. Our data show that complexin I is essential not only for normal motor function in mice, but also for normal performance of other complex behaviours. These results support the idea that altered expression of complexins in disease states may contribute to the symptomatology of disorders in which they are dysregulated.
 ...
PMID:Profound ataxia in complexin I knockout mice masks a complex phenotype that includes exploratory and habituation deficits. 1600 Mar 19

A case of Wernicke's encephalopathy is reported. A 53-year-old alcoholic man was presented with acute confusion, upward gaze palsy of the left eye and gait ataxia for 5 days. The typical findings upon MRI of the brain showed multiple, scattered, small non-enhanced low signal intensity on T1WI, high signal intensity on T2WI involving at bilateral basal ganglias, and thalami, midbrain, pons, and periventricular regions with evidence of atrophic changes of brain and the mamillary bodies. After treatment with intravenous thiamine 100 mg/day, the left upward gaze palsy was fully recovered at the 8th day of treatment. The mental status and gait ataxia gradually improved.
 ...
PMID:Wernicke's encephalopathy: report of a case. 1608 30

Wernicke's encephalopathy (WE) is an acute neuropsychiatric condition due to thiamine deficiency (vitamin B1) most commonly associated with chronic alcohol abuse. WE is difficult to diagnose because the classical triad of signs (confusion, ataxia and ophthalmoplegia) occurs in only 10% of cases. The presentation is often one of a non-specific confusional state which may easily be attributed to intoxication, alcohol withdrawal or to a concurrent morbidity such as head injury. To improve the outcome, it is important to make a presumptive diagnosis of WE and treat the patients as soon as possible with high-dose parenteral thiamine. Patients with an alcohol problem associated with malnutrition should all be offered a preventive treatment with parenteral thiamine in view of the impaired oral thiamine absorption.
 ...
PMID:[Prevalence, prophylaxis and treatment of Wernicke encephalopathy. Thiamine, how much and how do we give it?]. 1611 48


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>