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)

Scopoletin has been isolated and identified in gari, a cassava food consumed in Nigeria (West Africa). Its levels in gari and cassava flour is not altered by post processing treatments such as sundrying, refrigeration and storage. Scopoletin has also been identified as an active principle in the traditional herbal infusion of the fruit of Tetrapleura tetraptera TAUB used in the ethnopharmacology of West Africa. It is a potent hypotensive and non-specific spasmolytic agent. These pharmacological effects of Scopoletin are probably the underlying factors in the slowly developing tropical neuropathy characterised by optic atrophy, nerve deafness and ataxia endemic among populations subsisting on cassava diets such as gari. Hitherto, these toxicities were attributed to cyanogenic glucosides (cyanide) present in cassava.
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PMID:Coumarin compounds in cassava diets: 2 health implications of scopoletin in gari. 192 93

Ataxic hemiparesis (AH) is a clinical entity in which minimal pyramidal weakness is associated with same-sided motor ataxia. It may be caused by a lesion of the pons or of cerebro-cerebellar and corticospinal fibers in other areas. Associated symptoms help in the clinical localization of a syndrome that has to be differentiated from lobar ataxias (frontal, parietal lobe), sensory ataxia accompanying spinal cord, corticospinal weakness and ataxic neuropathy. We report 3 cases of AH caused by a lesion in the contralateral cerebral cortex: 2 were cases of postictal seizure while the third patient had left ataxic hemiparesis, left focal motor seizures and cortical memory loss. All 3 patients recovered within 7 days. AH as a postictal phenomenon has not previously been reported.
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PMID:Reversible postictal ataxic hemiparesis. 201 27

Effects of organophosphorus esters (OPs) inducing delayed neuropathy in the adult hen have traditionally been evaluated by assessment of morphology and function of nerve and muscle in the rear limbs of animals exposed. In this study, organophosphorus-induced delayed neuropathy (OPIDN), including neuromuscular function and histology, were studied in vivo using sciatic nerve, tibial nerve and gastrocnemius muscle in anesthetized hens that had been administered phenyl saligenin phosphate (PSP), 2.5 mg/kg by intramuscular injection. In addition, OPIDN was examined in vitro using the biventer cervicis nerve and muscle of the same adult hens. Both nerve-muscle preparations were used for construction of strength duration curves (SDC) on days 4-5, 7-8, and 15-16 after PSP; the biventer cervicis preparation was also used 21-22, 37 and 64 days after PSP administration. Histological examination was done at these same time periods. SDC revealed significant increases in excitability thresholds for preparations from hens receiving PSP only compared to preparations from control hens, or compared to preparations from hens treated with PSP and either nifedipine (1 mg/kg intramuscularly for 5 days), or verapamil (7 mg/kg intramuscularly for 4 days), with treatment beginning 24 hours before administration of PSP. Ataxia, which appeared 7-10 days after hens were given PSP, was less pronounced in hens given PSP plus either calcium channel blocker than in hens given PSP alone. Whether treatment was initiated before or after PSP, verapamil, a phenylalkylamine, reduced sensitivity of the biventer cervicis muscle to acetylcholine-induced stimulation. The dihydropyridine, nifedipine, was less effective at reducing muscle sensitivity to acetylcholine post-exposure than when used as a pretreatment. Lesions were extensive in the biventer cervicis nerve after PSP administration and modification by treatment with calcium channel blockers was evident.
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PMID:Modification of phenyl saligenin phosphate-induced delayed effects by calcium channel blockers: in vivo and in vitro electrophysiological assessment. 208 84

A 23 year old male developed abdominal pain, diarrhoea, and vomiting several hours after poisoning with acute triphenyltin intoxication in a suicide attempt. Severe ataxia, dysmetria, nystagmus, and blurring of vision soon supervened. Disturbance of consciousness and confusion developed 12 days later and lasted for two months. A delayed sensorimotor polyneuropathy was shown by electrophysiological studies to be due to axonal degeneration and demyelination. The neuropathy rapidly recovered after consciousness was regained.
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PMID:Acute triphenyltin intoxication: a case report. 750 Jan 18

A New Zealand family with 3 members affected by late onset hereditary cerebellar ataxia is reported, distinguished by the development of near global thermoanalgesia. Because proprioception and autonomic function were preserved, and ataxia only slowly worsened, this proved to be a benign syndrome. The pattern of sensory loss indicated a 'length-dependent' neuropathy. Evaluation of sural nerve biopsy revealed a marked loss of axons, especially of those with diameters 1-7 microns and 0.2-1.5 microns. It therefore seemed likely that impairment of pain and temperature in the syndrome resulted from a loss of primary sensory afferents. It is concluded that this family represents a previously unrecognized variant and that the spectrum of hereditary ataxias needs to be widened to include an associated severe but selective loss of pain and temperature sensation.
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PMID:Benign hereditary cerebellar ataxia with extensive thermoanalgesia. 216 79

An 11-year-old girl was found to have severely reduced beta-galactocerebrosidase activity as evidence of late-onset globoid cell leukodystrophy, while her mother had almost normal enzyme activity in circulating white blood cells. Clinically, the patient showed a remitting course marked by seizures, ataxia, white-matter disease on computed tomographic scan, and reduced conduction velocities of peripheral nerves. Symptoms improved somewhat around the age of 10 years. Two sural nerve biopsies, performed 6 years apart, disclosed a demyelinating neuropathy. By electron microscopy, membrane-bound vacuolar lysosomes in Schwann cells of myelinated axons, unlike the typical needlelike inclusions seen in classic infantile globoid cell leukodystrophy, were present in both specimens. Thus, clinical, morphologic, and biochemical data in this patient--and her mother--emphasize, compared with past reports on late-onset globoid cell leukodystrophy, considerable variation in the nosologic spectrum of late-onset globoid cell leukodystrophy and conspicuous differences from classic infantile globoid cell leukodystrophy.
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PMID:Late-onset globoid cell leukodystrophy: unusual ultrastructural pathology and subtotal beta-galactocerebrosidase deficiency. 217 71

Two patients of ataxic sensory neuropathy associated with silicosis were studied. Case 1 is a 53-year-old (in 1979) man who was a stonecutter for 40 years and diagnosed as silicosis in 1973. Case 2 is a 64-year-old (in 1984) man who was a glasscutter for 30 years and had been treated for silicosis from 1980 to 1982. Both patients developed dysesthesias in the hands, feet and face asymmetrically and gait ataxia over a few months. Vibratory and joint position senses were profoundly diminished but were accompanied by only mildly decreased pain and temperature sensations. Their muscle power was almost unchanged. Both had absent muscle power was almost unchanged. Both had absent muscle stretch reflexes. Sensory nerve conduction velocities were absent and motor nerve studies were almost normal. Nerve biopsy in case 2 showed a severe loss of large myelinaed fibers, and no inflammatory infiltrates and onion bulb formations. Although these findings suggested the carcinomatous neuropathy, we could not find any malignancy. Both patients had elevated polyclonal gamma-globulin levels and rheumatoid factors and, in case 2 an increase of IgG in serum. Cerebrospinal fluid showed an albumino-cytogenic dissociation and steroid therapy was successful in both patients. Case 1 died of pneumonia in 1989. Though an autopsy was not performed, his condition had continued to improve without signs of malignancy during 10 years. The condition of case 2 has also continued to improve, although ataxias remain.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Two cases of ataxic sensory neuropathy associated with silicosis]. 217 42

Two brothers, 31 and 25 years of age, with distal hereditary motor neuropathy (distal HMN) and choroideremia are reported. Their parents were second cousins. During the first decade, their visual acuity gradually diminished with night blindness, accompanied by weakness of the legs. From the third decade, they noticed weakness of the hands. The neurological signs of both cases could be summarized as follows: bilateral pes cavus, Charcot-Marie-Tooth type distal atrophy and weakness of the four limbs, no sensory deficit, hyperreflexia in the upper limbs and knees, absent ankle jerks and plantar reflexes, and severe visual impairment due to choroideremia. In addition to these signs, the younger patient showed nystagmus and limb kinetic ataxia suggesting cerebellar involvement. Muscle biopsies and EMG studies revealed chronic neurogenic changes. MCV's in the median nerves were normal, but the extensor digitorum brevis muscles were totally denervated. SCV's, the amplitude of sensory action potentials and sural nerve biopsies were normal. In both patients, elevated CSF protein without pleocytosis and high level of serum IgA & E were demonstrated. Associations of choroideremia and abnormal laboratory findings as noted in our cases have not been so far reported in distal HMN, but we suppose these disorders may be genetically related.
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PMID:[Two siblings of distal hereditary motor neuropathy with choroideremia]. 226 99

Indices of organophosphorus (OP)-induced delayed neuropathy (OPIDN) in the hen model have traditionally been restricted to the early inhibition of neuropathy target esterase (NTE) and ataxia with associated pathological changes in hind limb peripheral nerve which occur more than 7 days after OP exposure. The biventer cervicis nerve-muscle preparation was used to evaluate OPIDN in adult hens at various time periods after treatment with either the protoxicant tri-o-tolyl phosphate (TOTP), 360 mg/kg po, or the active congener phenyl saligenin phosphate (PSP), 2.5 mg/kg im. NTE activity was 21 and 48% of control for TOTP and PSP, respectively, 4 days after administration. Clinical signs were notable by 10 days and progressed in severity to paralysis by 21 days. Partial clinical recovery was evident at 37 days. Denervation hypersensitivity of biventer cervicis muscle to acetylcholine (ACh) was evident as early as 4 days following TOTP or PSP treatment. The sensitivity to ACh was greatest 21 days after OP administration, with partial recovery at 37 days. Strength-duration curves (SDC) of preparations from OP-treated hens showed an increase in excitability thresholds and elevated rheobase with shorter chronaxie than did preparations from controls as early as 4 days following treatment with either compound. SDC at 37 days indicated partial reinnervation. Peripheral nerve myelinated fiber degeneration and regeneration consistent with these physiological changes was seen on histopathological examination. This study suggests that the biventer cervicis nerve-muscle preparation may prove useful for detection of functional and morphological changes that occur during the interval between NTE inhibition and appearance of clinical deficits.
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PMID:Use of the biventer cervicis nerve-muscle preparation to detect early changes following exposure to organophosphates inducing delayed neuropathy. 237 92

In 23 Lewis rats 2 forms of neuropathy both with atactic gait were studied by morphometric analysis of walking tracks. In one group of animals experimental allergic neuritis (EAN) was induced which leads to both sensory and motor dysfunction. Clinical symptoms are atactic gait and hindlimb paresis. In another group pyridoxine (vitamin B6) neuropathy was induced which is a purely sensory neuropathy clinically presenting with gait ataxia, too. Track analysis is a simple method that requires no visualisation process and produces significant and reproducible data. The findings were compared with the clinical scores and electrophysiological data. In EAN, toe spreading was impaired early, and at a later stage stride width, print length and outward rotation of the hind feet changed. Pyridoxine-induced neuropathy produced only an increase in stride width. Track analysis correlated well with clinical grading and electrophysiological recordings. We propose track analysis as a reliable and accurate indicator of neuropathy symptoms in rats.
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PMID:Measurement of atactic and paretic gait in neuropathies of rats based on analysis of walking tracks. 238 37


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