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

Progressive spinocerebellar degeneration was identified in six children with chronic cholestatic liver disease and attributed to severe vitamin E deficiency. In addition to areflexia, ataxia, dysmetria, and diminished vibratory and position sense, three patients had pigmentary retinopathy. Abnormalities were present on electromyography, nerve conduction studies, and electroretinography. Because the vitamin E deficiency was not corrected by oral administration of massive doses of vitamin E, vitamin E was administered by the intramuscular route. With doses of 50 to 100 mg of vitamin E every three to seven days, over a 32-month interval (range, 15 to 44 months), vitamin E deficiency and abnormal red blood cell peroxide hemolysis were corrected. Other than discomfort and occasional edema at the site of injection, there were no side effects of parenteral vitamin E therapy. In several other studies intramuscular vitamin E therapy has produced significant neurologic improvement in patients with similar characteristics. In this study clinical progression of spinocerebellar degeneration was arrested but improvement could not be demonstrated despite adequate vitamin E replacement.
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PMID:Intramuscular vitamin E repletion in children with chronic cholestasis. 346 1

Forty female out-patients undergoing therapeutic abortion participated in a double-blind study comparing flunitrazepam 0.05 mg . kg-1 with thiopentone 6.0 mg . kg-1 as induction agents for general anaesthesia. Induction time, as measured by the time to loss of lid reflex and voluntary speech, was not only significantly longer in patients receiving flunitrazepam, but also much more variable and imprecise than with thiopentone. The Steward recovery room scores and psychomotor drawing test results revealed that recovery was significantly slower in the flunitrazepam group. Anterograde amnesia was observed in all patients who had received flunitrazepam and in one patient who had received thiopentone. No retrograde amnesia was found in either group. Flunitrazepam produced postoperative drowsiness, sedation, ataxia and nausea while with thiopentone discomfort from surgery and discomfort at the intravenous injection site were the main complaints. Because of the slowness of induction with flunitrazepam and marked individual variation, we do not feel that this drug can be considered a suitable agent for routine induction of general anaesthesia.
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PMID:Comparison of flunitrazepam and thiopentone for induction of general anaesthesia. 610 7

Side effects of carbamazepine (CBZ), valproate (VPA) and clonazepam (CZP) are rare during long-term use but rather common and usually transient during the early phases of treatment. The usual side effects of CBZ are drowsiness, dizziness, and diplopia, which are dose dependent in long-term use, but CBZ does not seem to cause cognitive disturbances, as do phenobarbital and phenytoin. Other reactions to CBZ may include leukopenia, hyponatremia, disturbances of vitamin D metabolism and fortunately rarely, agranulocytosis and hepatitis. Use of VPA can lead to gastrointestinal discomfort, weight gain, hair loss, tremor and sedation, but these side effects are rather uncommon, mild, and transient during VPA monotherapy. Potentially hazardous reactions such as hepatitis and pancreatitis have occurred in a few patients on VPA, generally with multidrug therapy. Some of the side effects are dose related. They infrequently lead to withdrawal of VPA. Side effects limited to initiation of CZP therapy include drowsiness, ataxia, and behavioral changes; they are usually transient but can lead to dose reduction or even withdrawal of the drug. Except for development of tolerance, CZP seems to be practically free of long-term side effects.
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PMID:Side effects of carbamazepine, valproate and clonazepam during long-term treatment of epilepsy. 642 98

Complications from mydriatic and cycloplegic drugs are rare compared with their extensive use. Adverse effects are often related to dosage or other factors. The ocular complications include increased intraocular pressure, pigmentation of the conjunctiva and cornea, pigment in the anterior chamber, lacrimal duct blockage, macular edema, corneal endothelium damage, hyperemia, allergy, discomfort, and blurred vision. The systemic complications are those common to sympathomimetic and parasympatholytic drugs and include tachycardia, hypertension, headache, faintness. pallor, trembling, excessive sweating, palpitations, arrhythmias, confusion, hallucinations, drowsiness, ataxia, flushed skin, high fever, dysarthria, thirst, dry mouth, convulsions, disorientation, nervousness, coma, and death. An understanding of all possible side effects is of paramount importance to those using these drugs in the treatment of anticholinesterase poisoning. This review is intended as a ready reference to the adverse effects of mydriatic and cycloplegic drugs.
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PMID:Mydriatic and cycloplegic drugs: a review of ocular and systemic complications. 703 29

Chiari malformation, also called Arnold-Chiari deformities, are rare hindbrain herniations that may present in children or adults. The most common symptoms include headache, syncope, disordered eye movement, sensory loss, weakness, and cerebellar features such as ataxia. Dysphagia occurs in 5-15% of patients, although only a few reports describe dysphagia as the only presenting symptom. We report a case of a 27-year-old woman who presented with a three-year history of dysphagia, chest pain, and weight loss. Esophageal manometrics revealed markedly disordered esophageal motility and gastroesophageal reflux. Her symptoms failed to respond to high doses of omeprazole, prokinetics, and eventually surgical fundoplication. The subsequent onset of neurological symptoms led to the diagnosis of Chiari type I malformation. Following posterior craniotomy with decompression, her dysphagia and chest discomfort completely resolved. Repeat esophageal manometrics revealed complete resolution of prior abnormalities.
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PMID:Esophageal dysphagia as the sole symptom in type I Chiari malformation. 861 24

Fosphenytoin is a water-soluble disodium phosphate ester of phenytoin that is converted in plasma to phenytoin. Fosphenytoin is compatible with most common i.v. solutions and can be administered safely through the i.m.route. An additional safety factor is the absence of propylene glycol in the fosphenytoin formulation. Propylene glycol is used as a vehicle in the i.v. phenytoin preparation and by itself may produce serious cardiovascular complications. Studies of the pharmacokinetics, safety, and tolerance of i.v. fosphenytoin have demonstrated that fosphenytoin produces phenytoin plasma concentrations similar to those achieved with oral and i.v. phenytoin, but without significant cardiovascular effects and only minimal discomfort at the injection site. Aside from local reactions, the most common adverse events associated with fosphenytoin have been pruritus and reactions typical of phenytoin (e.g., dizziness, somnolence, and ataxia). Fosphenytoin represents a significant advance in the treatment of patients with seizures who require parenteral therapy.
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PMID:Intravenous administration of fosphenytoin: options for the management of seizures. 864 9

In order to clarify the significance of motion sickness in the vestibular system, we compared the findings of experimental motion sickness between different kinds of subjects, some of which were already reported. Subjects were healthy adults, healthy children between the ages of 4 and 15 years, and patients with congenital and acquired labyrinthine loss. They were asked to walk while wearing horizontally and vertically reversing goggles. Equilibrium ataxia as well as motion sickness were evoked by horizontal reversal, but not by vertical reversal in healthy subjects. Kindergarten children exhibited severe ataxia, but little nausea. The frequency of severe ataxia decreased during growth, inversely as the frequency of nausea syndrome increased. Although a patient with acquired loss became severely ataxic, a patient with congenital loss did not show any ataxia at all. The present study suggests that vestibular cues are indispensable to the ego-spatial relationship in the brain, and once the ego-spatial relationship becomes inadequate, discomfort acts as a safety device to brake uncontrollable actions. Then, perception of the outer world may automatically adjust voluntary actions by affecting motor commands. The importance of visual cues for representing an alternative framework may differ between congenital and acquired labyrinthine loss.
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PMID:The significance of motion sickness in the vestibular system. 917 23

Fosphenytoin (Cerebyx), is a water soluble prodrug that is rapidly and completely converted to phenytoin. This study reports the injection-site tolerance and safety of intramuscular fosphenytoin (> 10 mg/kg doses) in 60 patients requiring a phenytoin loading dose. Patients received injections at single or multiple sites with volumes ranging from 4 to 30 ml per injection site. The majority of patients had no irritation (erythema, swelling, tenderness, bruising) or complaints of discomfort related to fosphenytoin injection either after injection (95%) or at follow-up (88%). Irritation, when reported, was mild in all cases. Forty of 60 patients (67%) reported transient side effects, primarily involving the central nervous system, such as nystagmus, dizziness or ataxia, which are commonly associated with phenytoin therapy. All patients received prescribed doses; no patient had an injection(s) stopped due to intolerance or side effects. No serious adverse events occurred with intramuscular fosphenytoin. In this study, intramuscular fosphenytoin was demonstrated to be a safe and well tolerated, and in many instances, a preferable alternative to other means of phenytoin loading.
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PMID:Intramuscular fosphenytoin (Cerebyx) in patients requiring a loading dose of phenytoin. 933 83

Six 20-24-month-old heifers received ketamine (KE), 2% lidocaine (Li), or ketamine and 2% lidocaine (KELI) into the first intercoccygeal (Co(1)-Co(2)) epidural space while standing. Heart rate, arterial blood pressure (systolic, diastolic, and mean), respiratory rate, rectal temperature, analgesia, sedation, and ataxia were determined before treatment and at predetermined intervals. Subjective scoring was used to assess pain and discomfort in reproductive manipulations. The duration of analgesia was 35+/-14 min for Li, 48+/-11 min for KE, and 35+/-7 min for KELI (P>0.05). Motor block determined by KE, Li, or KELI treatments was restricted to tail, external genital organs (vulva and vagina), anus, and rectum, with little interference in the cervix or uterus. It was concluded that epidural ketamine, 2% lidocaine, or their combination produced satisfactory analgesia without discomfort or ataxia and facilitated reproductive manipulations.
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PMID:Effects of caudal epidural ketamine and/or lidocaine on heifers during reproductive procedures: a preliminary study. 1964 56

Patient-based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ-5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient-reported health status was compromised in patients of all genotypes (EQ-5D visual analogue scale (EQ-VAS) mean 61.45 +/- 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ-VAS variance in the whole sample and might be extrapolated to other SCA genotypes.
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PMID:Self-rated health status in spinocerebellar ataxia--results from a European multicenter study. 2017 83


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