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)

Epithelial cysts in the posterior fossa are very rare, and only 22 cases have been reported in literatures. We report a case of epithelial cyst occurring on the anterior surface of the brain stem. A 31-year-old man was admitted to our hospital complaining of right hemiparesis and dysarthria. Plain CT showed a heterogeneous (isodense-hyperdense) mass in front of the pons, and was not enhanced after injection of contrast medium. As for findings of MRI, the tumor revealed as heterogeneous (isointense-hyperintense) mass on T1-weighted image and also heterogeneous (hypointense-hyperintense) mass on T2-weighted image. We removed partially the tumor which content was like yogurt. The histological findings of the cyst wall revealed a pseudostratified columnar ciliated epithelium.
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PMID:[Epithelial cyst occurring on the anterior surface of the brain stem--case report and review of the literatures]. 129 17

A case with cortico-basal degeneration was reported with special reference to the immunohistochemical study. A 59-year-old housewife noted tremor and clumsiness of her left hand. On the initial examination she showed the hyperreflexia of the upper extremities and jaw jerk, parkinsonian symptoms such as Myerson sign, parkinsonian gait and rigidity in the left arm. She showed pronounced forced grasping in the left hand. At the age of 60 she showed a WAIS scale with verbal IQ of 99 and performance below the scale. She could not copy hand postures. Tremor was aggravated by action or anxiety, more prominent on the left hand. There was some incoordination on the finger-nose testing of left arm and on the knee-heel testing of both legs. She also showed homolateral dyskinesia. She had a left Babinski sign and sensory testing was normal. A CT scan showed slightly enlarged ventricles (Fig. 1). At the age of 61 she could not understand simple requests and speak few words spontaneously, showing severe dysarthria. There were palilalia and motor impersistence. CT scan showed more widening of the lateral ventricles. At the age of 62, she had lingual dyskinesia and tapping on her upper lip provoked myoclonic jerk on her arms. She died of pneumonia at the age of 65 years, 6 years from the onset. The brain weighed 1190 g. There were bilateral old subdural hematomas on the right parietal and occipital lobe and the left parietal lobe. There was atrophy of frontal and superior parietal region.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Widespread tau abnormality in a case of cortico-basal degeneration]. 129 53

Speech motor changes in six patients with focal unilateral upper motor neuron (UUMN) lesions were characterised using a variety of techniques including listener judgements, speech acoustic analyses, electromyographic and strength measures. Listener judgements of speech understand-ability, intelligibility and dysarthric features indicated mild dysarthria. Diadochokinesis, electromyographic and strength measures corroborated these observations. The findings of this study delineate the characteristics of the dysarthria associated with unilateral upper motor neuron lesions and have important implications for identifying potential upper motor neuron components of other neurogenic disorders of speech and language.
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PMID:Dysarthria associated with focal unilateral upper motor neuron lesion. 130 85

We report the results of a single-subject multiple baseline across behaviours experiment of a 74-year-old woman presenting with Parkinson's disease. Her speech was typical of a hypokinetic dysarthria. The main features of her dysarthric speech were: a restriction in the modulation of fundamental frequency, an inappropriate pitch level and a rate disturbance. Three measures of prosody were operationally defined as follows: (1) linguistic modulation of fundamental frequency, (2) mean fundamental frequency and (3) rate. Treatment focused on ameliorating these aspects employing a multiple baseline design. Measures during and post-therapy documented improvement for each of these three aspects of prosody. Independent judges were also more capable of understanding her speech and her speech prosody after therapy. Upon follow-up measures 10 weeks later, most of the improvement was maintained.
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PMID:The effects of speech and language therapy for a case of dysarthria associated with Parkinson's disease. 130 95

Thirty-six parkinsonian patients were selected by age of onset of over 70 and a minimum of five years duration of illness. The mean age of onset was 73.5 years and 30 patients were still alive after a mean of 7.2 years. We found that late onset Parkinson's disease has a relatively benign course with more "axial symptoms" especially dysarthria, freezing and postural instability. Dyskinesias and fluctuations are rare and dementia occurs in few patients in spite of their old age.
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PMID:Some clinical aspects of late onset parkinsonism. 132 Apr 90

A 2-year-old girl with reducing body myopathy was reported. She had no family history of neuromuscular disease. She developed normally with no delay in milestones during infancy. She had no muscle weakness or hypotonia up to 2 years of age when she received mumps vaccination. Three days after the injection, she was first noticed to have limb muscle weakness. The muscle weakness progressed rapidly with increasing difficulty in gait and raising the upper arms, particularly the left. Four months later, she had difficulty in keeping her head up and could no longer climb the stairs. On physical examination, she had proximal dominant generalized muscle weakness, with a preferential neck muscle involvement. She walked waddlingly and stood up with Gowers' maneuver. Facial and ocular muscles were intact. No dysarthria, dysphagia or respiratory difficulty was noted. EMG showed myopathic pattern. Serum creatine kinase level was moderately elevated to 739 IU/l. In the biopsied left biceps muscle, there was marked variation in fiber size, but no apparent necrotic or regenerating fibers. The most striking feature was the presence of numerous eosinophilic inclusions which reduced nitroblue tetrazolium (NBT) and were, therefore, stained dark with menadione-linked alpha-glycerophosphate dehydrogenase even without the substrate of menadione, showing the histochemical characteristics of "reducing" body. The bodies were predominantly seen in fibers with disorganized intermyofibrillar networks and with high acid phosphatase activity. On electron microscopy, the reducing bodies consisted of fine granular material with the similar electron density to the chromatin granules and were located mostly around the degenerated nuclei, suggesting the nuclear degeneration playing a role in forming the reducing bodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Reducing body myopathy--a case report]. 132 Oct 16

A thirty-two year old female had chronic progressive external ophthalmoplegia (CPEO), exertional fatigue, dysarthria, dysphagia, and bilateral hearing impairment. Histochemical stains, obtained from the right vastus lateralis, showed ragged-red fibers and wide-spread abnormalities in the number, size, and the structure of mitochondria under electronomicroscopic examination. A biochemical analysis showed a low activity of NADH-cytochrome C reductase, NADH dehydrogenase and a normal activity of succinate cytochrome C reductase and cytochrome C oxidase. This data suggests a specific defect in the NADH dehydrogenase of complex I (NADH CoQ reductase). We believe that this is the first biochemically defined mitochondrial myopathy reported in Taiwan and provides additional evidence for the existence of biochemical heterogeneity in mitochondrial disorders of CPEO.
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PMID:Chronic progressive external ophthalmoplegia with NADH-CoQ reductase deficiency: report of a case. 132 93

The authors systematized the descriptions of different ataxic degenerative syndromes associated with the development of pyramidal symptomatology. Demonstrated the genetic pleomorphism of hereditary spastic ataxias, described the clinical features characteristic of different types of inheritance of spastic ataxias. Based on the authors' observations and analysis of the reported data the following criteria for the diagnosis "spastic ataxia" are suggested: autosomal dominant inheritance type, with the onset on the 3rd-4th decade of life, cerebellar ataxia and dysarthria coupled with tendinous hyperreflexia and the rise of the muscular tone by the spastic type.
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PMID:[Clinical polymorphism and genetic heterogeneity of hereditary spastic ataxia]. 133 96

We describe three brothers with type 3 GM1 gangliosidosis presenting as dystonia. The ages of the patients when examined were 28, 31, and 33. They had developed dysarthria with facial grimacing since early childhood. The common neurological sign was generalized dystonia. Both dystonic postures and dystonic movements resulting from varying degrees of fixed rigidity of each muscle involved did not disappear when the patients were lying or sitting relaxed. There was no correlation between the severity of dystonia and the residual activities of acid beta-galactosidase. Magnetic resonance imaging (MRI) showed bilaterally symmetric high intensity lesions only in the putamen on T2-weighted and proton density images. Selective putaminal changes on MRI may be the lesions most responsible for symptomatic dystonia in this disorder.
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PMID:Type 3 GM1 gangliosidosis: characteristic MRI findings correlated with dystonia. 133 95

Three cases of later onset metachromatic leukodystrophy in one family were reported. The brother is 32 years old, younger sister is 35 years old and the elder sister is 39 years old, who were normal as child without any family history of neurological disease. The three cases began their illness at about the age of 30 years old with dysarthria, progressive dementia, motor disturbance and numbness in the extremities. CT scan showed low density in the white matter of frontal, parietal lobes and around the ventricle. The written reports of CT scan were lacunar infarction in brother, multiple lacunar infarction in younger sister and Binswanger's disease in elder sister. Nerve biopsy showed myelinated fibers were decreased in number. Metachromatical materials were seen in and around the Schwann cell cytoplasm. Metachromatical materials were lamillar inclusions in granular matrix by electromicroscopic examination. So nerve biopsy is easy, safe and effective method to diagnose the uncertain pathogenic leukoence-phalopathy.
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PMID:[Later onset metachromatic leukodystrophy diagnosed by nerve biopsy]. 133 86


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