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)

A 51-year-old woman, with progressive gait disturbance and dysarthria, had been diagnosed as Menzel-type spinocerebellar degeneration. Later, she developed dystonic posture of upper limbs and bulging eyes. She was diagnosed as Machado-Joseph disease from neurological findings, which consisted of cerebellar signs, pyramidal tract signs and extrapyramidal tract signs and peripheral neuropathy. She died suddenly of unknown origin. Her illness lasted about 13 years. Neuropathological findings showed moderate neuronal loss with gliosis in the subthalamic nucleus, globus pallidus, substantia nigra, dentate nucleus, oculomotor and hypoglossal nucleus and anterior horn. Positron emission tomography (PET) using 15O steady state inhalation technique revealed reduction of cerebral blood flow and cerebral metabolic rate of oxygen in not only cerebellum but also cerebral cortex. These findings are different from typical PET findings of spinocerebellar degeneration.
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PMID:[A case of Machado-Joseph disease--cerebral blood flow and cerebral metabolic rate of oxygen]. 129 Nov 71

With the great progress in the imaging technique of cerebro-cerebellar perfusion and metabolism, it has been revealed that supratentorial cerebrovascular disorders often cause some reduction of contralateral cerebellar blood flow and metabolism. The phenomenon, termed crossed cerebellar diaschisis (CCD), is interpreted to be brought about by transneuronal deactivation of cerebellum via the corticopontocerebellar pathway and usually not to accompanied by limb-incoordination. We have experienced 2 cases presenting ataxia clinically and CCD in positron emission tomography (PET) which are thought to be caused by the interruption of two distinctive neural pathways, the corticopontocerebellar and cerebellorubrothalamic pathways. Case 1 was a 34-year-old housewife with cerebral infarction which magnetic resonance imaging disclosed in the left parietal cortex and subcortical white matter. She showed mild right-sided hemiataxia featured by dysarthria, hypermetria, dysrhythmia, decomposition, dysdiadochokinesis and rebound phenomenon. Cheirooral syndrome, a hand-pronation sign and defects of combined sensation were present on the right but not accompanied by any disturbances of deep sensation. PET with 15O-labeled CO2 and O2 demonstrated the left frontoparietooccipital and contralateral cerebellar hypoperfusion and hypometabolism. Case 2 was a 69-year-old female suffering from aftereffects of old thalamic hemorrhage. Neurological examination revealed moderate degree of right-sided hemiataxia suggesting a cerebellar type of dysfunction like in case 1. Muscle power and sensory system were well-preserved. Brain CT revealed a small and restricted low density area in the left posterolateral thalamus indicating destruction of the Vim nucleus. PET study confirmed reduced blood flow and oxygen metabolism in the left thalamus and contralateral cerebellar hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical and etiological study of crossed cerebellar diaschisis. Report of two cases]. 261

Two anaesthetic techniques were compared in a randomized trial of 60 ASA I and II women admitted for diagnostic dilatation and curettage of the uterus. Group I had fentanyl 2.5 micrograms/kg and thiopentone 2 mg/kg i.v. Supplementary thiopentone 50 mg was given every 15 s until loss of the eyelid reflex. Anaesthesia was maintained with nitrous oxide in oxygen 2/1 together with supplementary thiopentone 50 mg as required. Group II had midazolam 0.1 mg/kg i.v. and a supplementary 0.025 mg/kg every second min until sleep or dysarthria, followed by paracervical blockade with 1% mepivacaine 10 ml on each side of the portio. There was no significant depression of the cardiovascular system. The working conditions for the gynaecologist were good in both groups. The midazolam technique gave just as good amnesia as did general anaesthesia, and there was a high degree of patient satisfaction in both groups. In the thiopentone group there was a significant depression of the respiratory rate and a significantly higher frequency of adverse effects (nausea and vomiting) as compared to the midazolam group. It is concluded that paracervical blockade combined with midazolam, titrated i.v., until sleep or dysarthria, is a recommendable anaesthetic technique for diagnostic dilatation and curettage.
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PMID:Midazolam combined with paracervical blockade compared to general anaesthesia for curettage of the uterus. 306 45

This study describes the clinical course of a 31 year old woman who developed multiple anaerobic brain abscesses six days after tonsillectomy, followed by hemoparesis and dysarthria. In spite of craniotomy, repeated punctures and drainage of pus and high dose local and systemic antibiotics, there was an obvious deterioration in the patient's condition. Hyperbaric oxygen therapy was tried as a last resort. The patient improved quickly, and six months after the tonsillectomy seems to be neurologically symptomfree.
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PMID:[Hyperbaric oxygen therapy of anaerobic brain abscesses following tonsillectomy]. 398 Feb 50

A 62-year-old man developed recurrent TIAs presenting as mild unconsciousness, dysarthria and weakness of the right upper extremity lasting for 15 to 20 minutes. He was found to have severe iron deficiency anemia (hemoglobin: 5.5-5.9g/dl; hematocrit: 18.4-19.5%) which insidiously developed through the chronic bleeding from the gastric ulcer. He had slight hypertension (184/86mmHg), but no orthostatic hypotension. DSA and MR angiography showed severe stenosis at the origin of the bilateral internal carotid arteries and of the left vertebral artery. There was also hypoplasia of the right vertebral artery. Blood circulation detected by 123I-IMP-SPECT was markedly decreased in the whole brain and in the right hemisphere of the cerebellum. TIA was, however, completely disappeared following to the recovery of anemia. The present case suggested that the presence of severe anemia accelerated the occurrence of hemodynamic TIA (regional cerebral anemic hypoxia), which is probably the consequence of the reduced oxygen-transporting capacity of the blood.
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PMID:[Hemodynamic TIA associated with severe anemia--a case report]. 799 47

Two cases (case 1, a 45-year-old man; case 2, a 68-year-old man) of superficial siderosis of the central nervous system are presented. Main neurological symptoms were anosmia, sensorineural deafness, dysarthria, ataxia, and pyramidal tract signs. Lumbar puncture revealed bloody cerebrospinal fluid (CSF) in both cases. In case 1, the CSF became watery clear after administration of hemostatic medicines. T2-weighted magnetic resonance images showed cerebellar atrophy and marginal hypointensity of the brainstem, cerebellum, and the entire spinal cord. T2-weighted images of the cranial nerves showed hypointensity of the VIII nerves which were clinically impaired as compared with normointensity of the VII nerves which presented no clinical symptom. These findings may reflect difference in the degree of hemosiderin depostion between the VII and VIII nerves. While case 1 had a borderline score of WAIS-R (IQ79), case 2 showed overt dementia (performance IQ65). Positron emission tomography showed that cerebral blood flow and cerebral oxygen metabolism were reduced in the basal temporal lobes in both cases.
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PMID:[Two cases of superficial siderosis of the central nervous system. Findings of the cerebrospinal fluid, magnetic resonance imaging and positron emission tomography]. 882 97

In the course of a decompression at flight level 280 (28,000 ft) in an altitude chamber flight, a 45-yr-old cabin air traffic controller developed sudden numbness in his left upper and lower extremities and, soon after, complete paralysis in the left side, dysarthria and left facial palsy. A presumptive diagnosis of arterial gas embolism (AGE) was made and hyperbaric oxygen therapy (HBO) was given after airevac of the patient to the closest compression facility. Complete resolution of the symptoms was obtained after treatment Table VI-A (extended), plus 3 consecutive HBO treatments (90 min of Oxygen at 2.0 ATA). AGE is a rare event in the course of regular altitude chamber flight and diagnosis should be done in the context of the barometric pressure changes and an acute cerebral vascular injury. Risk factors and follow-up diagnostic procedures are discussed.
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PMID:Neurological manifestation of arterial gas embolism following standard altitude chamber flight: a case report. 938 3

A 63 year-old-man, with an opercular infarct had a motor deficit with ataxic hemiparesis and cerebellar dysarthria. The motor deficit disappeared, but the cerebellar signs remained after three months. A voice instrumental analysis confirmed the cerebellar pattern of the dysarthria. CT and MRI showed the opercular lesion and the normality of the cerebellum and the pons. A volumic MRI analysis precised the localisation and the volume of the lesion. The oxygen consumption (CMRO2) was measured with PET and showed no cerebellar diaschisis. We propose that, in this case, the cerebellar dysarthria is the orofacial ataxic component of the hemiparesis. We suggest that it is related to disruption of the cortico-cerebellar tract, connecting the right orofacial frontal area of the primary motor cortex with the paravermal segment of the left cerebellar hemisphere.
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PMID:[Ataxic hemiparesis with cerebellar dysarthria due to an opercular lesion]. 977 22

A 49-year-old woman with 6 months history of body weight loss, muscle weakness, and dysarthria, was found with respiratory arrest and resuscitated in the morning of January 1999. An MRI brain scan revealed diffuse swelling and T2/FLAIR high signal intensity with mild Gadolinium enhancement in the lower pons and medulla oblongata. Although the histological diagnosis could not be obtained, glioma (astrocytoma) was suspected. In the morning of July 3rd she presented sweating and cyanosis. Her arterial oxygen saturation was 18%. When we asked her to breathe more, she kept breathing and oxygen saturation was normalized. However, she could not breathe at all when she fell asleep without stimulation. She was kept under respiratory support for 2 months. Her symptoms improved with fluctuating course after 70 Gy of radiation therapy. Ondine curse is one type of sleep apnea syndrome, defined as the selective disturbance of autonomous breathing. Surgical operation and stroke are the reported causes of this syndrome. Brainstem tumor is relatively common cause for children's Ondine curse. On the other hand, it rarely causes adult's Ondine curse as a main symptom.
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PMID:[A case of Ondine curse associated with a medullary tumor]. 1121 2

The protean manifestations of a novel maternally inherited point mutation of the mitochondrial genome are reported. The proband showed isolated, spastic paraparesis. A brother, who had suffered from a multisystem progressive disorder, ultimately died of cardiomyopathy. Another brother is healthy. The proband's mother showed truncal ataxia, dysarthria, severe hearing loss, mental regression, ptosis, ophthalmoparesis, distal cyclones, and diabetes mellitus. A muscle biopsy performed in the proband failed to show the morphological abnormalities typical of mitochondrial disorders; the activities of respiratory chain complexes were normal. However, complex I and IV activities were low in the muscle homogenate of the affected mother and brother. Sequence analysis of mtDNA showed a heteroplasmic mutation of the tRNA(Ile) gene (G4284A). The mutation load was approximately 55%, 80%, and 90% in the muscle mtDNA of the proband, his mother, and his affected brother, respectively. Mutation was undetected in the healthy brother, as well as in 100 control samples. Several cybrid clones containing homoplasmic mutant mtDNA from the proband showed significant reductions of complex IV activity and maximum oxygen consumption rate, compared with homoplasmic wild-type clones derived from the same subject.
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PMID:Novel heteroplasmic mtDNA mutation in a family with heterogeneous clinical presentations. 1178 91


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