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)

We studied the application of proton magnetic resonance spectroscopy (1H-MRS) in a case of corticobasal degeneration. A 73-year-old woman was referred to our hospital for dysarthria and clumsiness in right arm movement. Intelligence was normal and neither ideational apraxia or ideomotor apraxia were observed. Her speech was slurred and extremely slow. Neurological findings showed a limitation of ocular upward gaze movement, impaired smooth pursuit in the horizontal gaze, rigidity, limb kinetic apraxia and cortical sensory disturbance in the right arm. Magnetic resonance imaging (MRI) showed diffuse brain atrophy, especially in the left fronto-parietal cortex around the central sulcus. A positron emission tomography (PET) study showed diffuse decrement of cerebral blood flow, dominantly in the left hemisphere. The decrease in the uptake of 18F-Fluoro-deoxyglucose also revealed glucose hypo-metabolism, especially in the left frontal and parietal lobe. 1H-MRS by the multivoxel method showed a decrease in N-acetylaspartate (NAA)/Creatine to 1.0 in the left basal ganglia and temporal lobe, compared to the values between 1.4 and 1.7 in the right basal ganglia. These findings indicated that 1H-MRS might detect neuronal loss or degeneration when MRI showed minimal cortical atrophy. This study, the first application of 1H-MRS in a case of corticobasal degeneration, showed that this method was useful for the evaluation of pathophysiological changes in corticobasal degeneration.
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PMID:[A lateralized reduction of NAA in a case of corticobasal degeneration (CBD): application of proton magnetic resonance spectroscopy (1H-MRS)]. 962 63

Using proton magnetic resonance spectroscopy (1H-MRS) and single photon emission CT (SPECT), the cerebellum of patients with olivopontocerebellar atrophy (OPCA) and of age-matched control subjects was studied. A spectrum was collected from a 27 cm3 (3 x 3 x 3 cm) voxel in the cerebellum containing white and gray matters in order to measure the distribution and relative signal intensities of N-acetylaspartate (NAA), creatine (Cre) and choline (Cho). In the cerebellum of the patients with OPCA, mean NAA/Cre ratios for OPCA patients were significantly decreased compared with normal control subjects (OPCA, 1.01 +/- 0.247; controls, 1.526 +/- 0.144: p < 0.001). Mean NAA/Cho ratios for OPCA patients were slightly decreased (OPCA, 1.285 +/- 0.228; controls 1.702 +/- 0.469: p < 0.06). Cho/Cre ratios valued in the cerebellum of OPCA patients were not significantly different from those in normal controls (OPCA, 0.793 +/- 0.186; controls, 0.946 +/- 0.219). The ratio of RI count in the cerebellum to that in the occipital lobe was significantly decreased in OPCA patients (OPCA, 0.947 +/ 0.096; controls, 1.06 +/- 0.063: p < 0.01). Cerebellar signs were assessed including gait ataxia, limb ataxia, dysarthria, saccadic pursuit, and nystagmus separately or in combination. In patients with more severe ataxic gait and dysarthria. MRS revealed slightly lowered NAA/Cre ratio. There was no significant correlation between NAA/Cre ratio and severity of other clinical signs. The MRS and SPECT findings give a confirmative evidence of hypofunction in cerebellum of patients with OPCA.
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PMID:[Proton magnetic resonance spectroscopy and single photon emission CT in patients with olivopontocerebellar atrophy]. 974 73

We report a 49-year-old previously healthy woman with acute onset of decrease in attention, dysarthria and ataxia, accompanied by drowsiness. On admission, there were cloudness of consciousness, hallucination and left hemiparesis. Cerebrospinal fluid study revealed a cell count of 1/mm3, and the cytology was class I with a slight increase in protein. MRI of the brain performed on admission showed multiple gadolinium-enhanced lesions with a T2 weighted high intensity area in the cerebral white matter. At first the patient was diagnosed as acute disseminated encephalomyelitis (ADEM), and treated with methylprednisolon pulse therapy. Soon after, she showed transient clinical improvement, but her condition soon worsened. MR spectroscopy revealed elevated choline peak, decreased NAA peak and lactate peak, which indicated a neoplastic lesion. The brain biopsy disclosed diffuse intravascular lymphoma (IVL). MRS was useful in the differential diagnosis of IVL from ADEM.
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PMID:[MR spectroscopy findings of a case of intravascular malignant lymphoma: usefulness for differential diagnosis]. 1288 28

We reported a Japanese case of spinocerebellar ataxia type 6 (SCA6) with episodic ataxia type 2 (EA2) phenotype. A 28-year-old woman was admitted to our hospital because of episodic unsteadiness of gait and dysarthria for 4 years. Neurological examination revealed truncal ataxia and dysarthria during attacks, but no abnormal findings in interictal phases. A brain MRI showed no obvious cerebellar atrophy, whereas proton MR spectroscopy (1H-MRS) disclosed decrease of the N-acetylaspartate/ceatine (NAA/Cr) ratio in the cerebellar hemisphere. We identified the expanded 22 CAG repeats without a missense mutation in the CACNA1A gene. After one year from the discharge, her gait ataxia became gradually obvious even in the interictal phase. To our knowledge, although a few foreign papers had reported the SCA6 cases with EA2 phenotype, there is no particular report on such cases in Japan. 1H-MRS, in addition to CAG repeats analysis, might enable us to differentiate SCA6 from EA2, because the latter showed no decrease of NAA/Cr ratio in cerebellar hemisphere according to the previous reports.
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PMID:[A case of spinocerebellar ataxia type 6 with its initial symptom of episodic ataxia-like phenotype]. 1648 24

The prevalence of pathological laughing and crying in multiple sclerosis (MS) is 10%. It has been speculated that the anatomical lesion responsible for the pathological laughing is located in the pontine base, prefrontal cortex, and cerebellum. We report an 18-year-old male patient presenting with pathological laughing and hypomania. In his neurological examination, he had a euphoric effect with ataxic walking and dysarthria speech. He had a bilateral conjugated gaze limitation, with a prominent bilateral horizontal nystagmus on left gaze, dysmetria, dysdiadokokinesia, and remarkable dysfunction in a heel-to-shin test on the left. The IgG index in cerebrospinal fluid was normal with an oligoclonal band was present. In cranial MRI, there was a lesion on central pons which was hypointense in T1 images with contrast enhancement and hyperintense in T2 and flair images. Also another lesion in right brachium pontis which did not contrast enhancement but was hyperintense on T2 and flair images was present. There was an elevation of myoinositol/creatine ratio and choline and a reduction of NAA in proton MR spectroscopy. MR spectroscopic evaluation of the patient demonstrated the demyelination process. There has been no report of patients in whom pathological laughter was the presenting symptom of clinically isolated brainstem syndrome.
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PMID:Pathological laughing as a manifestation in a clinically isolated brainstem syndrome: a case report. 1882 40