Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The correcting action of tropatepine hydrochloride upon the extrapyramidal effects induced by neuroleptics has been studied in 32 acute psychotic states. A satisfying result has been obtained with daily doses of 10 to 20 mg in 30 cases (excellent results: 20, good results: 10 patients). The action upon dyskinetic crises is particularly good and quickly obtained, avoiding a recourse to an i.m. corrector in 7 cases out of 8. The akineto-hypertonic syndrome has been efficiently corrected in 18 cases out of 23. The hyperkinetic manifestations (akathisia, tasikinesia) reacted favourably in 8 cases out of 11, dysarthria in 7 cases out of 11. The lessening of tremor was less satisfying (8 cases out of 15); same thing for the dryness of the mouth (7 cases out of 16) and disorder of visual accomodation (6 cases out of 12). The clinical tolerance was excellent. With the used doses, we found neither inconvenient atropinic effect, nor disorder of the vigilance, nor pharmacodependence. The one daily administration of a 10 mg tablet turned out to be sufficient in 19 cases. This possibility of only one administration a day appeared to us as a great advantage.
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PMID:[Clinical study of a new agent correcting the syndrome of neuroleptic impregnation in acute psychotic states]. 109 47

Two patients with disulfiram-(Antabuse-)induced encephalopathy exhibited paranoid ideas, disorientation, impaired memory, ataxia, dysarthria, snout and grasp reflexes, and abnormal electroencephalograms. The first patient developed symptoms on two occasions, each time after disulfiram administration. The second patient experienced a generalized seizure followed by fulminant psychosis three weeks after starting disulfiram therapy. Spinal fluid examination in the latter patient revealed a low homovanillic acid (HVA) level. Since disulfiram inhibits dopamine oxidation, disulfiram-induced encephalopathy may be related to excess dopaminergic activity in the central nervous system.
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PMID:Disulfiram-induced encephalopathy. 125 49

We analyzed 71 patients (45 males and 26 females) with Wilson's disease (WD) who were seen at our hospital from 1979 through 1990. The mean age at onset was 18.1 +/- 6.5 years, with 17.0 +/- 6.6 years for males and 20.2 +/- 5.7 years for females. The mean age at the time of diagnosis was 21.0 +/- 6.3 years. Hepatic WD was the most frequent mode of presentation in childhood with a mean age of 15.5 +/- 6.0 years, while neurologic WD tended to occur in adolescence with a mean age of 21.0 +/- 8.9 years. The ages of onset were 12.5 +/- 0.5 years for renal WD and 25.3 +/- 2.4 years for psychiatric WD. The common initial symptoms were neurologic and hepatobiliary. In addition, hematologic and renal disorders were also common during evaluation. The neurologic findings at the time of diagnosis were tremors (66.2%), dysarthria (56.3%), gait disturbances (46.5%), dystonia (42.3%) and decreased facial expressions (40.8%). Less frequent but notable neurologic presentations were psychosis (11.3%), epileptic seizures (5.6%) and hypokalemic periodic paralysis (1.4%). When compared with two previous large Chinese series, the present data show a male preponderance, an earlier age of onset for males and higher incidences of hepatic, hematologic and renal involvement. The possible reasons for the discrepancies between the present study and previous Chinese series are discussed.
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PMID:Wilson's disease: clinical analysis of 71 cases and comparison with previous Chinese series. 135 28

Neurologic manifestations, afflicting up to 70% of SLE patients, include psychosis, seizures, chorea, neuropathies, and stroke. MRI is useful in evaluation of lupus patients and several reports have documented cerebral atrophy or focal hyperintensities. We report an unusual MRI appearance in a 56-year-old woman with SLE, diagnosed on the basis of pleuritis, lymphopenia, anti-DNA antibodies, and neurologic involvement. She reported recent onset of Raynaud's phenomenon and generalized macular rash. She presented after two months of gradual deterioration with memory loss, flattened affect, dysphagia, dysarthria, anomia, and somnolence, without focal neurologic signs. Investigations included elevated ESR, reduced complement, normal CSF without oligoclonal bands, negative viral serology, normal hormone and vitamin levels, normal renal and hepatic function. Neuropsychologic testing showed widespread impairment (WAIS-R: FSIQ-63; WMS-69; DRS-98; RCPM-14; WAB AQ-78.8). CT was normal but MRI showed strikingly symmetric, confluent hyperintensities extensively involving cerebral and cerebellar white matter on T1 and T2 weighted scans. Basal ganglia and subependymal and subcortical white matter were spared. Treated with prednisone, the patient made a gradual, but incomplete, recovery. These MRI findings may reflect widespread vasculopathy or direct immunologic brain insult with or without immunologic blood-brain barrier disruption.
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PMID:Dementia with leukoencephalopathy in systemic lupus erythematosus. 191 71

Forty-five patients with Wilson's disease (WD) were prospectively studied: 27 had neurological deficits, 12 hepatic signs, and 6 were asymptomatic. Kayser-Fleischer rings occurred in 23 of the neurological patients and in only 4 of the hepatic patients. Neurological features were extremely variable with respect to frequency and severity. Most frequent were dysdiadochokinesis (25 patients), dysarthria (23), bradykinesia (17), and posture tremor (14). Fifteen, mainly long-term treated patients, presented with rather discrete neurological abnormalities which predominantly consisted of dysarthria and various forms of tremor. Eight patients had a parkinsonian type of neurological WD associated with signs of an organic mood syndrome. Three patients were predominantly hyperkinetic, presenting with dystonic and choreatic movements. In 1 patient, ataxia was the predominant neurological feature. There was a clear-cut correlation between the severity of neurological impairment and the restriction in functional capacity. Nine patients were not able to engage in salaried employment or were retired. Psychiatric symptoms and behavioural disorders were common, varying from mild personality and psychological disturbances to severe psychiatric illness resembling psychotic disorders and major depressive syndromes. Significant mental deterioration was not found in the patients. Disturbances of mood were observed in 12 patients, all of whom had neurological abnormalities. There was a history of an attempted suicide in 7 patients, and a history of an organic delusional syndrome in 3.
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PMID:Neurological and neuropsychiatric spectrum of Wilson's disease: a prospective study of 45 cases. 191 12

Three patients with adult-onset neuronal-ceroid lipofuscinosis (Kufs' disease) are presented. Two cases were familial (autosomal recessive inheritance) and 1 case was possibly sporadic. The main clinical feature was progressive deterioration of cognitive functions. In 2 siblings, aged 37 and 41, dementia was associated with personality and behaviour changes, suggesting a psychotic disorder and with dysarthria and tic-like dyskinetic movements. In a third patient, dementia was only associated with an asymptomatic pigmentary retinal degeneration. CT scan revealed diffuse cerebral atrophy in all cases. Diagnosis was established by brain biopsy in 2 cases. Autofluorescence emission and absorption spectra from the abnormal pigment was studied and was not found contributive.
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PMID:[Dementia and psychiatric disorders in Kufs disease]. 229 Oct 38

A prospective neuropsychiatric study of 31 consecutive subjects with Wilson's disease is reported. Exploratory factor analysis yielded four factors: neurological, cognitive, hepatic/depressive and psychiatric. Significant associations were found between a 'psychopathic' personality factor and neurological and dysarthria scores. The d' and beta coefficients of a signal-detection memory task dissociated: the former correlated only with Mini-Mental State and Benton visuospatial task; and the latter with depressive symptoms. Using discriminant function analysis, there was efficient classification of 'psychopathic' personality by dysarthria, and of individual depressive symptoms by disturbance of gait. Biochemical markers of hepatic dysfunction were significantly associated with certain depressive symptoms. No evidence emerged to support the putative association with schizophrenia-like psychosis.
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PMID:Wilson's disease: a prospective study of psychopathology in 31 cases. 259 16

Three patients from two families had an unusual phenotypical variant of late-onset hexosaminidase-A deficiency. The clinical picture was dominated by spinal motor neuron involvement mimicking juvenile-onset spinal muscular atrophy. Atypical features included prominent muscle cramps, postural and action tremor, recurrent psychosis, incoordination, corticospinal and corticobulbar involvement, and dysarthria. The presence of these atypical features in patients whose lower motor neuron involvement would otherwise be consistent with juvenile-onset spinal muscular atrophy should raise the suspicion of the presence of hexosaminidase-A deficiency and GM2 gangliosidosis that can be proved by appropriate enzyme assays.
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PMID:Hexosaminidase-A deficiency presenting as atypical juvenile-onset spinal muscular atrophy. 293 15

Reported is a 19-year-old manic-depressive patient who developed persistent dysarthria with coexisting apraxia while on a combination of high dose haloperidol and lithium carbonate. The speech disability occurred as a solitary symptom in a patient with normal serum lithium levels and no other signs or symptoms of lithium toxicity and persisted after lithium was discontinued and the neuroleptic changed. There were several factors which favored an association between the speech disability and the drug therapy. These included improvement during a drug-free trial: the absence of a prior history of a speech problem; the patient's marked psychotic state and anxiety: and the high dosage of haloperidol.
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PMID:Persistent dysarthria with apraxia associated with a combination of lithium carbonate and haloperidol. 680 52

A case of acute mania in a 50-year-old woman who had received hemodialysis treatment for 2 1/2 years is described. The patient's presentation with secondary manic and associated psychotic features was the initial symptom of the syndrome of progressive dialysis encephalopathy (PDE). Shortly thereafter, an abnormal EEG tracing was obtained. Several months later she developed dysarthria and muscular twitchings and EEG changes persisted, a symptom complex characteristic of PDE. The appearance of psychiatric symptoms among patients receiving chronic dialysis treatment may herald the onset of PDE.
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PMID:Secondary mania as a presentation of progressive dialysis encephalopathy. 682 59


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