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)

Atypical forms of progressive multifocal leukoencephalopathy (PML) may simulate other disorders. A previously healthy 70-year-old female developed unsteadiness of gait, dysarthria, dementia and weakness leading to inanition and death from bronchopneumonia over a 43 month period. The diagnosis of PML was not suspected prior to death. Neuropathologic examination of the brain disclosed characteristic findings of PML-deep bilateral cerebral demyelinative foci with enlarged gemistocytic astrocytes and swollen oligodendrocytes containing intranuclear inclusions. Electron microscopy identified papova virus particles within these inclusions. An underlying source of immunosuppression was not identified either premortem nor at the time of autopsy. The prolonged clinical course, simulating that of a primary degenerative disease, and the lack of apparent immunocompromise are unusual features of PML and lend credence to the suggestions that variations in its expression and course are to be expected.
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PMID:Prolonged progressive multifocal leukoencephalopathy without immunosuppression. 369 Apr 32

In proportion to a rapid increase of dialysis patient, death caused by neurological complications is increasing annually among long-term hemodialyzed patients. A case of chronic subdural hematoma during long-term hemodialysis is presented. A 35-year-old male had undergone hemodialysis three times weekly for four years. He had marked changes in body-weight and blood pressure between hemodialysis. In March 1983, he had a headache, vomiting, and left hemiparesis. The CT scan showed a right subdural hematoma. He was admitted to our hospital 10 days later because of progressive hemiparesis and speech disturbance. The neurological examination showed left hemiparesis with sensory deficit and dysarthria. The CT scan showed an increase in the size of the subdural hematoma. Bleeding time was over 10 minutes. A right-sided burr hole was made and altered blood was removed and irrigated. After operation, headache and weakness rapidly subsided, but the next morning, attacks of convulsion occurred. The CT scan showed the rebleeding in the subdural space. After correcting the level of serum potassium by hemodialysis, a right parietal craniotomy was performed. Hematoma of about 100 g was removed and the capsule of the hematoma showed organized tissue histologically. Postoperatively, although attacks of convulsion occurred temporarily, he gradually improved. The levels of serum potassium and BUN were controlled by several treatments of hemodialysis. He was discharged with only mild hemiparesis. Subdural hematoma caused by hemodialysis is a very important complication. Chronic subdural hematoma is sometimes very difficult to differentiate from dysequilibrium syndrome or dialysis dementia. The CT scan is a very valuable examination to rule out subdural hematoma.
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PMID:[A case of chronic subdural hematoma in a hemodialyzed patient]. 372 77

A 58-year-old man recognized hoarseness and dysarthria followed by weakness of the left upper extremity. Examination five months later disclosed dementia which was manifested dominantly by changes in personality and behavior, but also by symptoms of amyotrophic lateral sclerosis. He became progressively weak, and his neck became rigid and extended for two months. He expired due to respiratory failure at the age of 60. At autopsy, the brain weighed 1120 g with atrophy of the temporal lobes. Microscopic examination revealed marked diminution of Betz cells with astrocytic proliferation in the motor cortex. There was a mild, localized spongy state in the upper layer in the frontal and temporal cortices. There were very few neurofibrillary changes and senile plaques, and no Pick's argentophilic bodies. Fibrillary gliosis was found in the white matter of the temporal lobes, external segment of the globus pallidus and the amygdaloid nucleus. The substantia nigra showed depletion of pigmented cells, free melanin pigment and reactive astrocytosis. A diminished number of motor neurons in the brain stem and spinal cord accompanied astrocytic gliosis, while the remaining cell contained many Bunina bodies. The pyramidal tracts showed mild degeneration bilaterally below the pyramis in the medulla. There are a number of cases in the literature whose pictures consist of amyotrophic lateral sclerosis, dementia and/or extrapyramidal symptoms. This case is identical to those cases. But in this case, the clinical and pathological features of amyotrophic lateral sclerosis are more dominant than in other cases.
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PMID:[An autopsy case of amyotrophic lateral sclerosis with dementia and neck extension]. 375 32

Thirty-one patients with Wilson's disease were evaluated with detailed neurologic and medical examinations. Mean age (+/- SD) at onset was 21 +/- 5 years and at examination was 28 +/- 6 years. Of the 90% of patients who were first treated with penicillamine, 31% deteriorated initially despite therapy, and half never recovered to pretherapy baseline. At the time of our evaluations, the most common neurologic findings were dysarthria (97%), dystonia (65%), dysdiadochokinesia (58%), rigidity (52%), gait and postural abnormalities (42%), and tremor (32%). Chorea and dementia were rare. Twenty-two patients underwent magnetic resonance imaging. All but one of the 19 symptomatic patients had abnormal scans. The three asymptomatic patients had normal scans. Most lesions were seen in the caudate, putamen, subcortical white matter, midbrain, and pons. Generalized brain atrophy was also common. Lesions were less common in the thalamus, cerebellar vermis, midbrain tegmentum, globus pallidus, red nucleus, and dentate nucleus. Dystonia and bradykinesia correlated with putamen lesions, and dysarthria correlated with both putamen and caudate lesions.
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PMID:Clinical assessment of 31 patients with Wilson's disease. Correlations with structural changes on magnetic resonance imaging. 382 91

36 survivors of very severe traumatic childhood brain injury have been followed for a median period of 48 months after injury. One remains in a vegetative state and 35 have been discharged. 34 regained ambulation, and motor function became essentially normal in 14 of them. Ataxia and movement disorders were as common among the residua as was spasticity. Only one child remained aphasic, but 14 showed dysarthria. Six of the 36 showed major dementia and most of the rest showed very significant new impairment of cognitive and social function. This impairment was greater than expected from changes in the I.Q. Motor and speech function showed prolonged recovery while learning ability and social function did not. The overall degree of recovery seems not much better in these children than in adults.
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PMID:Survivors of severe traumatic brain injury in childhood. II. Late residual disability. 386 33

GM1 gangliosidosis is usually a pediatric disease caused by hereditary acid beta-galactosidase deficiency. There have been a few cases in adults. We saw a 51-year-old Japanese man with type 3 GM1 gangliosidosis who was manifesting dementia, dysarthria, gait disturbance, and limb rigidity. Radiologic studies showed platyspondylia, acetabular hypoplasia, and flattened femoral heads. Biochemical analysis revealed generalized acid beta-galactosidase deficiency.
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PMID:Type 3 (adult) GM1 gangliosidosis: case report. 392 60

We studied 30 necropsy cases of vascular dementia with a lacunar state. Manifestations included dementia, lack of volition, emotional lability, small-stepped gait, dysarthria, urinary incontinence, grasp reflex, pyramidal signs, paraplegia in flexion, and akinetic mutism. Pathologically, there was diffuse incomplete softening of white matter in all cases. Both lacunes and diffuse softening were found predominantly in the frontal lobes. The prominent clinical features were therefore frontal lobe symptoms, with good correlation between the symptoms and the distribution of pathologic lesions.
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PMID:Why do frontal lobe symptoms predominate in vascular dementia with lacunes? 395

Reviewing the literature since recognition of progressive supranuclear palsy (PSP) as a clinicopathological entity 20 years ago, the present state of knowledge is delineated. The etiology of PSP is still unknown. The clinical hallmarks are supranuclear palsy of vertical gaze, axial dystonia in extension and pseudobulbar palsy with marked dysarthria and dysphagia. Accessory features include subcortical dementia, mental, extrapyramidal, pyramidal and cerebellar symptoms. PSP is a disease of the presenium (average age at onset, 59.6 years) with a male preponderance (60% men). The onset is insidious with vague complaints of dysequilibrium (60%), mental changes (46%) and disturbed vision (21%), often preceding abnormal neurological findings. The important borderland and main differential diagnosis is parkinsonism. However, in PSP, responsiveness to antiparkinsonian agents is poor and progression is rapid and fatal within few years (average survival time, 5.7 years). Promising diagnostic tools at present include CT-scanning and neuro-otologic and -ophthalmologic examination. Neuropathological findings, confined to specific diencephalic, brainstem and cerebellar nuclei, include neurofibrillary tangles (ultrastructurally different from those seen in other CNS disorders), neuron loss and gliosis. The importance of research on neurocytochemistry, brain ultrastructure and immunology in the current investigation of PSP is outlined.
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PMID:Progressive supranuclear palsy--20 years later. 399 25

Olivopontocerebellar atrophy is a hereditary disorder that has variable clinical manifestations. Five types have been described, as well as a sixth that contains sporadic cases. This report describes a family with three affected members who demonstrate a composite of types III and V. Their features include progressive spasticity, ataxia, dementia, visual loss with retinal pigmentation, dysarthria, ophthalmoplegia, and chorea. This family might represent an additional category of the disease. In the two family members who developed chorea, baclofen resulted in marked improvement with abolition of the choreiform movements. Response has been sustained for several years in the mother and for eight months in the daughter. Neither has experienced any return of chorea while receiving treatment. When attempts were made to discontinue baclofen, choreiform movements returned promptly and with their original severity. Baclofen, a gamma-aminobutyric acid analogue, may be useful in the treatment of other forms of chorea as well.
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PMID:Olivopontocerebellar atrophy with dementia, blindness, and chorea. Response to baclofen. 402 7

Sixty-five cases of clinically diagnosed multiple lacunar state have been analysed. The clinical course of multiple lacunar state is usually progressive in nature, simulating degenerative diseases, in contrast with the mode of acute onset seen in the other vascular diseases. Average age at the initial visit was 63.8 year-old, and sex ratio showed marked male preponderance, being 12 for males and 1 for females. Gait disturbance and speech disturbance are the most frequent initial symptoms, followed by slow motion, emotional lability and swallowing difficulty. Neurological manifestations are dysarthria, short-stepped and apraxic gait, hyperreflexia, positive Babinski and Chaddock reflexes, minimal spasticity, dementia, positive palmo-mental reflex, emotional lability, fixed face, rigidity, bradykinesia, foot grasping, dysphagia, positive Myerson's sign and tremor, in the order described. The important point is that the rigidity is paratonic and the tremor is action or postural, not the cogwheel rigidity or resting tremor like Parkinson's disease. The appearance of pathological reflexes (Babinski and Chaddock reflexes) are quite important, especially Chaddock reflex, which can frequently become positive despite negative or equivocal Babinski reflex. Hypertension, especially longstanding in nature, is the major contributing factor in this disorder. CT scan showed the presence of one or more lacunes in 52 out of 65 cases (80.0%). The detection of lacune can be influenced by the quality of CT scan, and the high resolution CT scanner is greatly useful for that purpose. It is stressed that the detailed neurological and computed tomographic evaluations will make it possible to reach the clinical diagnosis and appropriate treatment of multiple lacunar state.
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PMID:[Clinical studies on multiple lacunar state]. 674 15


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