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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of progressive supranuclear palsy (PSP) are reported in two men (49 and 75years old) who for one and four years respectively had sudden falling while walking. Rigidity of the neck was an carly feature that prgressed to involve the upper trunk while "subcortical dementia", dysarthria and dysphagia appeared. They had a complete paralysis of vertical eye movements and slow horizontal voluntary eye movements. Oculocephalic reflexes were intact. On caloric stimulation vestibulo-ocular responses were present but only slow saccadic eye movements were observed. With surface electrodes eye movements were studied during the REM phase of sleep. Our patients had both vertical and horizontal eye movements during paradoxal sleep. This findings is in keeping with a supranuclear ophtalmoplegia, and may help in antemorten diagnosis of PSP.
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PMID:Progressive supranuclear palsy: report of two cases (author's transl). 18 May 89

We report a 67-year-old female with progressive supranuclear palsy (PSP) who dramatically improved when given L-threo-3,4-dihydroxyphenylserine (L-DOPS). This patient developed dysarthria, lack of facial expression, and slowness at age 64. She was admitted to a local hospital, diagnosed as having parkinsonism and treated with antiparkinsonian drugs. Despite this treatment, she had difficulty in turning over in bed and standing up from a seat, and began to fall backward at age 65. One year later, she had trouble in walking due to frequent falls and became bedridden. The patient was admitted to our hospital in July 1991 under treatment with 20 mg/200 mg of carbidopa/L-dopa and 4 mg of trihexyphenydyl hydrochloride per day. Neurological examination revealed masked face, pseudobulbar palsy, and dystonic rigidity of the neck and upper trunk. Eye movements were normal except for impaired vertical saccades and convergence inability. Deep tendon reflexes were generally brisk and the plantar responses were flexor bilaterally. Tests of pulsion showed that her postural reflex was markedly disturbed, especially in retropulsion. Her gait showed severe unsteadiness. Neuropsychological tests showed intellectual impairment, frontal lobe dysfunction, and memory disturbance. Computed tomography showed an atrophic midbrain with prominent enlargement of ambient and quadrigeminal plate cisterns. Single photon emission computed tomography (SPECT) using 123-I-isopropyl-iodoamphetamine demonstrated marked frontal hypoperfusion. L-DOPS was administered at a dose of 100 mg per day and gradually increased up to 600 mg per day over a period of five weeks, while carbidopa/L-dopa and trihexyphenidyl hydrochloride were continued as on admission.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of progressive supranuclear palsy dramatically improved with L-threo-3,4-dihydroxyphenylserine]. 142 39

Low body weight is a characteristic symptom of Parkinson's disease (PD), but the mechanism is unknown. To determine whether bulbar involvement is responsible for the weight loss, we compared the mean body weight (MBW), height, and body mass index (BMI) of 281 patients with PD and 86 patients with progressive supranuclear palsy (PSP). Although the patients with PSP had significantly worse dysphagia and dysarthria than the patients with PD, their MBW and BMI were not appreciably different. We conclude that bulbar dysfunction is not the reason for weight loss in PD.
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PMID:Low body weight in Parkinson's disease. 156 33

Language functions were studied in 6 patients with clinically diagnosed progressive supranuclear palsy who conformed to the characteristic pattern of 'subcortical dementia'. Dysarthria, reading difficulties and disturbances of handwriting were present in all patients. Some patients showed additional deficits including visual dyslexia, constructional dysgraphia and an increased rate of self-corrections and misnamings in object confrontation naming. In most instances, the naming errors referred to an object visually similar to the target object, suggesting that visual misperception is the major cause of the naming disorder. It is concluded that a variety of language impairments may develop secondary to other neurological and neuropsychological changes in progressive supranuclear palsy.
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PMID:Language functions in progressive supranuclear palsy. 206 60

A 64-year-old male patient of amyotrophic lateral sclerosis (ALS) with frozen gait, axial rigidity and supranuclear upper gaze palsy was reported. We have followed this patient more than four years. He was well until November 1982, when he noticed weakness of left arm. In March 1983, he noticed hypogeusia and in July, he developed dysarthria and frozen gait. On admission, he was alert and oriented. Neurological examination revealed dysarthria, dysphagia and muscular weakness and atrophy in bilateral upper extremities, dominantly in left side. He showed remarkable frozen gait, retropulsion and could not walk. Brain CT showed mild dilatation of the third ventricle. In August 1988, he showed tongue atrophy, and weakness and atrophy of the extremities progressed during these four years. He also showed axial rigidity and frozen gait. Brain CT showed severe third ventricular dilatation and atrophy of tegmentum of the midbrain and cerebellum that were compatible with progressive supranuclear palsy (PSP). Six months later, he developed upper gaze palsy. From these findings, we concluded that this patient had a quite unique clinical features of both ALS and PSP.
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PMID:[A case of amyotrophic lateral sclerosis associated with clinical features of progressive supranuclear palsy]. 259 46

Progressive supranuclear palsy (PSP) is a syndrome of supranuclear ophthalmoplegic palsy, pseudobulbar palsy, rigidity of the limbs, nuchal dystonia, and dementia in which the gaze palsy is the hallmark of the disease. Most neurologists are reluctant to consider the diagnosis unless visual problems exist. Since the earliest complaints of PSP are said to be variable and subtle, accurate diagnosis is often delayed and initial pathologic changes of the disease not well studied. Two patients came to autopsy with dementia, gait disturbances, and/or dysarthria but no eye findings by history or physical exams. Symptoms had been attributed to metastatic cancers. At autopsy prominent globose neurofibrillary tangles with variable cell loss, microglial nodules, and neuronophagia were found in the locus ceruleus, third cranial nerve complex, nucleus supratrochlearis, nucleus centralis superior, and nucleus basalis of Meynert with mild pallor of the globus pallidus, mild cell loss in the dentate nucleus of the cerebellum, and sparing of the superior colliculus. The diagnosis of early PSP was made. These cases serve to 1) detail the more limited neuropathologic changes in early PSP, 2) reemphasize that the earliest clinical symptoms of PSP are not gaze palsies, and 3) remind clinicians to consider PSP in their differential diagnosis in patients with gait disturbances, dementia, and/or dysarthria, and 4) document PSP in association with carcinoma in two cases.
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PMID:Early progressive supranuclear palsy: pathology and clinical presentation. 272 Oct 44

We surveyed neurologists and chronic care facilities in and near two New Jersey counties with a combined population of 799,022, regarding cases of progressive supranuclear palsy. All suspected cases were examined personally, using rigid criteria. The prevalence ratio was 1.39/100,000. A total of 50 New Jersey cases yielded median intervals to onset of requiring gait assistance, 3.1 years; visual symptoms, 3.9 years; dysarthria, 3.4 years; dysphagia, 4.4 years; requiring wheelchair, 8.2 years; and death, 9.7 years.
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PMID:Prevalence and natural history of progressive supranuclear palsy. 338 18

Ten male patients with parkinsonism secondary to Parkinson's disease or progressive supranuclear palsy had clinical neurological, speech, and acoustical speech evaluations. In addition, seven of the patients were evaluated by x-ray computed tomography (CT) and (F-18)-fluorodeoxyglucose (FDG) positron emission tomography (PET). Extensive variability of speech features, both clinical and acoustical, were found and seemed to be independent of the severity of any parkinsonian sign, CT, or FDG PET. In addition, little relationship existed between the variability across each measured speech feature. What appeared to be important for the appearance of abnormal acoustic measures was the degree of overall severity of the dysarthria. These observations suggest that a better understanding of hypokinetic dysarthria may result from more extensive examination of the variability between patients. Emphasizing a specific feature such as rapid speaking rate in characterizing hypokinetic dysarthria focuses on a single and inconstant finding in a complex speech pattern.
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PMID:Clinical and acoustical variability in hypokinetic dysarthria. 349 Apr 98

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

In this case study, a 59-year-old male with progressive supranuclear palsy and hypokinetic dysarthria wore a small, solid state, battery operated, delayed auditory feedback device to reduce speech rate and to aid speech intelligibility. Time series measurements were made from tape recordings taken at the beginning of treatment and again after three months of daily wearing of the device. Measures of speech rate, intensity, and overall intelligibility indicate that when the instrument is worn, the subject's speech is slowed, vocal intensity increases, and intelligibility is markedly improved. The subject and his family report satisfaction with the instrument. The application of delayed auditory feedback to the treatment of communication disorders is discussed.
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PMID:DAF as instrumental treatment for dysarthria in progressive supranuclear palsy: a case report. 644 31


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