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)

Paroxysmal symptoms are described in 14 patients with undoubted or suspected multiple sclerosis (MS). In seven of the patients the paroxysms were the first symptom of the disease, although only one has developed definite MS so far. The clinical features have been compared with 153 patients previously reported in the literature which has been reviewed, with special reference to 36 in whom paroxysmal symptoms were the initial manifestations of MS. Attention has been focused on paroxysmal symptoms of brain stem and spinal cord origin of the following types: paroxysmal dysarthria and ataxia, diplopia, tonic seizures, paroxysmal akinesia, paroxysmal sensory disturbances and pains. Examples of each type have been reported as the first symptoms of MS with remissions ranging from less than one to 21 years before other manifestations of MS have developed.
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PMID:Paroxysmal symptoms as the first manifestations of multiple sclerosis. 737 30

A patient suffering from bilateral thalamic infarction in the region supplied by the paramedian arteries sparing the internal capsules underwent acoustic analysis of sentence utterances. The results were compared with the findings obtained in parkinsonian subjects, in patients with upper motor neuron lesions, and in normal subjects. Acoustic measurements revealed increased pitch, monotonous speech, rough voice quality, and normal speech tempo concomitant with articulatory impreciseness in terms of incomplete closure productions. This constellation resembled parkinsonian dysarthria. Damage to the thalamic projection area of the pallidal efferents, therefore, seems to be the most probable cause of the patient's speech disorders. In parkinsonian subjects stereotactical lesions of this structure ameliorate rigor, but not akinesia. Thus, our patient's speech deficits, and by analogy the corresponding parkinsonian dysarthric disturbances, may be considered akinetic signs.
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PMID:Dysarthria in bilateral thalamic infarction. A case study. 833 76

Posteroventral pallidotomy (PVP) was carried out in 86 patients with Parkinson's disease, who presented marked bradykinesia, freezing of gait and postural defect associated with rigidity and tremor in 82 patients (bradykinesia type), and similar gait and postural problems with minimum signs of rigidity and tremor in 4 (pure akinesia type). The stereotactic coordinates of Leksell's device were calculated from MRI and conventional ventriculography. The final target was defined by microelectrode techniques in the basal ganglia. The microrecording study revealed a very high background activity in the internal pallidum in patients of the bradykinetic type, however, a much lower pallidal activity in patients of the pure akinesia type. Fifty-eight patients underwent unilateral PVP, and 28 underwent bilateral surgery. Following PVP, rigidity tremor and poor reciprocal movements were significantly improved especially in the contralateral extremities. The most dramatic findings were the reversal of akinetic symptoms and wearing-off phenomena. The patients were followed up for 3-30 months (mean = 8) after surgery. Of the 82 bradykinesia type patients, good result were obtained in 48 (58%), fair results in 26 (32%), and minor improvement or no change in 8 (10%). In all the 4 patients of the pure akinesia type, recurrence of the akinetic symptoms occurred after a temporal improvement lasting a few days to 3 month after surgery. There was worst dysarthria in 3 patients, hemiparesis in 1 and partial motor aphasia in 1. The visual field problem was not complicated in any patients. These findings suggest that akinetic symptoms in PD are implicated in overactive pallidal outputs with putative GABAergic modulator by excessively inhibiting pedunculopontine nucleus activity (midbrain locomotor and posture regions) as well as thalamic activity. Partial interruption of the pallidal efferents eliminates the akinetic symptoms by disinhibitory effects on the target structures. The pathology of PD of the pure akinesia type is supposedly in the brainstem and should be excluded from indication of pallidotomy.
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PMID:Surgical control of akinesia in Parkinson's disease. 879 Oct 23

Progressive supranuclear palsy (PSP) is a distinct clinicopathological syndrome described by Steele, Richardson and Olszewski in 1964. Its clinical features include supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, nuchal dystonia, and dementia. The neuropathological changes are characteristic and include cell loss, gliosis, and neurofibrillary degeneration in the basal ganglia, brain stem and cerebellum. But, all these clinical features are not present in the early stage and diagnosis of PSP is sometimes difficult. Atypical presentation of PSP includes the case without ophthalmoplegia, with markedly dementia, or pure akinesia. Pure akinesia presents freezing of gait, handwriting and speech without rigidity or tremor, and can be the initial and early symptom-complex of PSP.
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PMID:[Progressive supranuclear palsy]. 901 35

We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the later. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.
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PMID:Multiple system atrophy: a review of 203 pathologically proven cases. 908 71

We studied the clinical features and molecular genetics of a family, afflicted with a form of atypical parkinsonism, originating from the Madeira Islands of Portugal. We examined four affected individuals and reviewed clinical information on one other affected family member. Mean age at onset was 31 years. Parkinsonism (akinesia, rigidity, gait disturbance) was the most prominent feature in advanced disease. Levodopa responsiveness with peak-dose dyskinesia was present in one individual. Initial symptoms and other clinical features were variable and included other extrapyramidal signs (dystonia, action tremor of the limbs and bulbar muscles, synkinesis), ophthalmologic abnormalities (ptosis, slow saccades, progressive external ophthalmoplegia, hypometric saccades, saccadic pursuit movements), speech abnormalities (dysarthria, hypernasality), cortical impairment (dementia, frontal lobe dysfunction, palilalia, perseveration), minor cerebellar signs (dysmetria, gait ataxia), pyramidal abnormalities (spasticity, hyperreflexia), and peripheral nervous system abnormalities (propioceptive loss, areflexia, distal weakness, atrophy). The length of trinucleotide repeats in the MJD1 gene was in the normal range for all affected individuals.
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PMID:Atypical parkinsonism in a family of Portuguese ancestry: absence of CAG repeat expansion in the MJD1 gene. 915 59

Surgical treatments for PD and ET are promising. Medial Pallidotomy, the surgical lesioning of the pallidum, often improves symptoms of long-standing PD. We enrolled twenty-seven late stage PD patients for unilateral medial pallidotomy who were then assessed by the Core Assessment Program for Intracranial Transplantation (CAPIT) protocol. One year after surgery persistent improvement was seen contralateral to the lesion in the following features: drug-induced dyskinesias (92%), akinesia (38%), rigidity (51%), and tremor (42%). Complications included transient dysarthria (7 patients), facial weakness (9 patients), limb weakness (1 patient), swallowing problems (4 patients) and intracerebral haemorrhage (1 patient). Thalamic DBS may improve tremor in PD and ET patients. Therefore, we enrolled fifteen patients (9 PD and 6 ET patients) with disabling tremor, unresponsive to medication. They were assessed by the United Parkinson's Disease Rating Scale (UPDRS) and the Tremor Rating Scale (for PD and ET patients, respectively). Three months after surgery, limb tremor contralateral to stimulation improved by 71% in PD patients and 76% in ET patients. Complications included transient paresthesias (all), confusional state (1 patient) and intracerebral bleed (1 patient). Unilateral medial pallidotomy safely improves some Parkinsonian symptoms contralateral to the lesion. Thalamic DBS may effectively and safely improve contralateral limb tremor in PD and ET.
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PMID:Surgical interventions in the treatment of Parkinson's disease (PD) and essential tremor (ET): medial pallidotomy in PD and chronic deep brain stimulation (DBS) in PD and ET. 929 83

Several symptom complexes in multiple sclerosis (MS) are found in unusual circumstances but are characteristic of the disease. Most of these are amenable to treatment and will be confronted by the physiatrist treating patients who have MS. This article begins by addressing paroxysmal symptoms such as trigeminal neuralgia, paroxysmal dysarthria and ataxia, parathesia and pain, paroxysmal itching, and akinesia. Seizures, adventitious movements, fatigue, and complications related to pregnancy also are addressed.
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PMID:Multiple sclerosis potpourri. Paroxysmal symptoms, seizures, fatigue, pregnancy, and more. 989 8

Corticobasal degeneration(CBD) is a neurodegenerative disorder characterised clinically by apraxia, cortical sensory loss, alien limb, dementia, oculomotor abnormalities, dysarthria, postural instability, akinesia, rigidity, and pyramidal signs. Brain imaging may demonstrate greater abnormalities contralateral to the more affected side. We reported a case of corticobasal degeneration of which praxic impairments were improved by administration of amantadine. The patient was a 63-year-old right-handed woman. She showed marked dysfunction including rigidity, limb kinetic apraxia, cortical sensory loss, ideomotor apraxia, and dressing apraxia. A brain MRI scan revealed bilateral cortical atrophy centered in the postcentral gyrus, more pronounced in the left hemisphere than the right. A SPECT scan showed a decrease in blood flow in the temporo-parieto-occipital regions, more pronounced in the left hemisphere than the right. An EEG showed a diffuse slowness. L-dopa had no effect on the symptoms of rigidity, limb kinetic apraxia, cortical sensory loss, ideomotor apraxia, and dressing apraxia. By administration of amantadine, rigidity and cortical sensory loss did not improve, but some praxic impairments, such as dressing apraxia and ideomotor apraxia, and the EEG improved. Upon withdrawal of amantadine, the improved symptoms deteriorated. Amitriptyline did not improve the deteriorated symptoms. After amantadine was re-administered, the same praxic impairments and the EEG improved again. This suggested that administration of amantadine had some effect on certain praxic impairments and the EEG.
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PMID:[A case of corticobasal degeneration of which movemental disturbances were improved by administration of amantadine]. 1157 23

Surgical destruction of a portion of the ventrolateral nucleus of the thalamus is currently the procedure of choice for the treatment of incapacitating tremor and rigidity of parkinsonism. Seventy-three patients were treated by 105 thalamotomies at the University of Alberta Hospital and assessed one to four years later for improvement of function in everyday activities. Fifty-six patients were improved, 12 were unchanged, and five had died. Only two of the deaths were related to the operation. Paresis was permanent in only one patient. Twenty-five patients had bilateral operations and 22 of these showed improvement of function. Contraindications to operation include serious cardiovascular disease, mental deterioration, and those parkinsonian patients whose disability is chiefly due to akinesia, oculogyric crisis, dysphasia or dysarthria.
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PMID:RESULTS OF THALAMOTOMY FOR PARKINSON'S DISEASE. 1407 11


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