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)

Patients with cerebellar disease may exhibit tremulous phonation as part of their dysarthria. The results of an acoustic analysis of cerebellar voice tremor in a patient with hereditary ataxia and presenting with a purely cerebellar syndrome are reported. Analysis included computation of speech intensity contours, fundamental frequency contours, and spectral parameters from sustained productions of vowels and voiceless fricatives. Fundamental frequency contours during sustained phonation of vowels showed rhythmic oscillations at a rate of about 3 Hz. No concomitant periodicity could be detected for the parameters characterising voiceless fricative production. The results indicate an impairment of phonatory control in relation to the maintenance of a constant isometric activity of the internal laryngeal muscles. Cerebellar voice tremor may therefore be classified as a form of postural tremor.
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PMID:Cerebellar voice tremor: an acoustic analysis. 201 Jul 64

Distinct chronic posttraumatic syndromes, ascribed to neurological deficits of patients suffering severe head injuries and being in prolonged coma, are much less frequently encountered in the literature than acute traumatic syndromes. The major components of the posttraumatic midbrain syndrome, resulting from compressive necrosis or vascular infarction at the midbrain level, are ipsilateral cerebellar signs (the predominant one being intention tremor), contralateral pyramidal signs (the predominant one being a spastic-dystonic hemiparesis), dysarthria, and mild to moderate intellectual impairment. Significant bilateral cerebellar dysfunction following head injury, without pyramidal, extrapyramidal, or pseudobulbar signs, constitutes a posttraumatic cerebellar syndrome. Its most disabling component, namely posttraumatic intention tremor, may be alleviated by thalamotomy. Following severe closed head injury, an infrequently encountered posttraumatic entity of dystonic hemiplegia or hemiparesis, which may be alleviated by thalamotomy, can occur, but does not have a specific neuroanatomical basis. Intention tremors following severe head injuries, rarely associated with hydrocephalus and without other significant cerebellar findings, can develop as a dysfunction of the cerebellofugal outflow system. While chronic posttraumatic syndromes can be complex and difficult to treat, cerebellar stimulation has been utilized ipsilaterally to modulate limb spasticity, and bilateral ventrolateral cryothalamectomies staged 4-6 months apart have been successful in alleviating severe (intractable) intention tremors.
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PMID:Rehabilitative neurosurgery: posttraumatic syndromes. 262 1

We report on 4 cases of perennial hepatic encephalopathy and review similar published cases. The neurological picture consists of a cerebellar syndrome, both static and kinetic, dysarthria, choreo-athetoid abnormal movements and mental deterioration. Symptoms are permanent and usually worsen progressively. Some patients may present with a myelopathy, either isolated or combined with an encephalopathy. Relevant anatomical alterations, either encephalic or spinal, may be observed similarly in several varieties of liver disease, but in every case the role of portocaval shunts, whether spontaneous or surgically performed, appears essential. Altered results of laboratory studies, such as EEG or ammonemia, are described. Histological changes include a peculiar sort of hyperplasia of the protoplasmic astrocytes, along with a certain amount of neuronal loss. Surmised pathological mechanisms and applied therapy are briefly reviewed. For an appraisal of therapeutic results, perennial hepatic encephalopathies should be set apart from both the acute varieties and the usual chronic variety with its succession of recurrent exacerbations and remissions.
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PMID:[Chronic hepatic encephalopathies. Acquired cerebral degeneration not due to Wilson's disease]. 630 20

Twenty-four patients with leukemia or lymphoma refractory to conventional chemotherapy were given a course of systemic, high-dose cytosine arabinoside (3 gm/m2 every 12 hours for twelve doses). Four patients developed cerebellar degeneration during treatment. Ataxia of gait and limb movements, dysarthria, and nystagmus appeared five to seven days after the first dose, worsened over the next two to three days, and then remained stable for two to six days. Incomplete improvement occurred over the following one to two weeks. Postmortem examination disclosed loss of Purkinje cells in the depths of cortical sulci with relative preservation of those at the crests of folia and those in the most posterior inferior portions of the cerebellum. Other patients developed a mild, reversible cerebellar syndrome over the same time course as that of the irreversible disorder. Manifestations ranged from nystagmus alone to dysarthria and unsteadiness of gait without limb ataxia. We conclude that cytosine arabinoside at this dosage causes a cerebellar degeneration with characteristic clinical and pathological features. Among the present patients with refractory hematological malignancies, such degeneration occurred with an incidence of 16.7%, more than twice that reported in previous series.
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PMID:Cerebellar degeneration caused by high-dose cytosine arabinoside: a clinicopathological study. 665 Dec 39

The clinical syndrome of a 65-year-old patient with a slit-shaped right-sided meso-diencephalic lesion was analysed. A cerebellar syndrome with limb-kinetic ataxia, intention tremor and hypotonicity in all extremities as well as ataxic dysarthria was found. The disruption of the two cerebello-(rubro)-thalamic pathways probably explained the signs of bilateral cerebellar dysfunction. The uncrossed ascending limb of the right, and the crossed one of the left brachium conjunctivum may have been damaged by the unilateral lesion extending between caudal midbrain and dorsal thalamus.
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PMID:Bilateral cerebellar dysfunctions in a unilateral meso-diencephalic lesion. 724 Nov 66

While acute neurological symptoms complicating lithium therapy are well recognized, persistent neurologic dysfunction is uncommon. Regardless of the duration of symptoms, neuropathological correlates of lithium toxicity are few. We report the case of a 67-year-old man who developed lithium toxicity manifested by encephalopathy and coma followed by persistent dysarthria and ataxia. Autopsy revealed neuronal loss and gliosis in the cerebellar cortex and dentate nuclei; the cerebellar white matter exhibited prominent spongy change. Persistent cerebellar syndrome may occur with lithium intoxication and should be considered in the differential diagnosis of chronic cerebellar dysfunction.
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PMID:Neuropathologic correlates of persistent neurologic deficit in lithium intoxication. 799 83

Auditory-perceptual studies reported inconsistent data with respect to syllabic timing in cerebellar dysarthria, i.e. both reduced and increased variability of syllable durations. The present study performed a comprehensive analysis of syllabic timing during sentence utterances in 14 subjects with a pure cerebellar syndrome (CA). First, the CA patients presented with reduced speech tempo in terms of syllable and utterance durations. Second, a tendency for intrautterance syllabic isochrony, being an aspect of the percept of 'scanning speech', characterized a subgroup of patients with cerebellar dysarthria. Third, increased interutterance durational variability was found in three out of the five syllables considered. Thus, both reduced intrautterance and increased interutterance variability of syllable durations may pertain to cerebellar dysarthria. The latter dimension, however, seems to be more prominent.
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PMID:Speech rate and rhythm in cerebellar dysarthria: an acoustic analysis of syllabic timing. 817 15

To facilitate the study of cerebellar degenerative disorders, improved clinical diagnosis is needed. Cerebello-olivary atrophy is pathologically distinct, but until now its diagnosis has been thought to require postmortem examination. This condition was considered as a possible diagnosis in two patients from different families with dominantly inherited ataxia. The affected members of each family demonstrated a stereotyped, progressive, "pure" cerebellar syndrome, which began with gait ataxia followed years later by dysarthria and limb ataxia. The autopsy findings for the first patient's father revealed paleocerebellar and olivary atrophy, characteristic of cerebello-olivary atrophy. Magnetic resonance imaging (MRI) of the brain of both patients revealed medullary, vermian and, to a lesser extent, cerebellar hemispheric atrophy but a normal pons. Dominantly inherited cerebello-olivary atrophy was diagnosed in both patients. Characteristic clinical and MRI features thus permit a confident clinical diagnosis of dominantly inherited cerebello-olivary atrophy. Recognition of this entity during life should advance the classification of cerebellar degenerative disorders.
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PMID:Magnetic resonance imaging in the diagnosis of dominantly inherited cerebello-olivary atrophy: a clinicopathologic study. 850 32

Despite the involvement of cerebellar ataxia in a large variety of conditions and its frequent association with other neurological symptoms, the quantification of the specific core of the cerebellar syndrome is possible and useful in Neurology. Recent studies have shown that cerebellar ataxia might be sensitive to various types of pharmacological agents, but the scales used for assessment were all different. With the long-term goal of double-blind controlled trials-multicentric and international-an ad hoc Committee of the World Federation of Neurology has worked to propose a one-hundred-point semi-quantitative International Cooperative Ataxia Rating Scale (ICARS). The scale proposed involves a compartimentalized quantification of postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders, in order that a subscore concerning these symptoms may be separately studied. The weight of each symptomatologic compartment has been carefully designed. The members of the Committee agreed upon precise definitions of the tests, to minimize interobserver variations. The validation of this scale is in progress.
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PMID:International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. 909 50

Cerebellar disease affects a number of skilled movements, including those in speech. Ataxic dysarthria, the speech disorder that typically accompanies cerebellar disease, was studied by acoustic methods. Control subjects and subjects with ataxic dysarthria were recorded while performing a number of speaking tasks, including sustained vowel phonation, syllable repetition, monosyllabic word production (intelligibility test), sentence recitation, and conversation. Acoustic data derived from the speech samples confirmed the hypothesis that temporal dysregulation is a primary component of the speech disorder. The data also show that the nature of the disorder varies with the speaking task. This result agrees with observations on other motor systems in subjects with cerebellar disease and may be evidence of a dissociation of impairments. Suggestions are offered on the selection of measures for a given task and on the role of the cerebellum in the regulation of speaking.
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PMID:A speaking task analysis of the dysarthria in cerebellar disease. 919 89


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