Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The most common cause of chorea-ballismus (CB) is a vascular lesion; it is also associated with nonketotic hyperglycaemia in diabetes mellitus (DM) and may be the first manifestation of this disorder. We describe the CT, MRI and proton MR spectroscopy (1H-MRS) of CB in eight patients. Six had hemichorea-hemiballismus (HC-HB) and two bilateral CB. Single-voxel (SV) 1H-MRS was performed using point-resolved spectroscopy (PRESS). Voxels were positioned in the basal ganglia of the patients and control subjects. PRESS was also used to obtain spectroscopic imaging (1H-MRSI) of the slice of interest in two patients. CT showed a slightly dense striatum in all the patients with CB, and T1-weighted images revealed high signal. The CB correlated well with the neuroimaging findings. SV 1H-MRS showed the mean (+/- SD) N-acetylaspartate (NAA)/ creatine (Cr) ratio to be 1.45 +/- 0.19 in HC-HB and 1.82 +/- 0.06 on the opposite normal side (P = 0.01). The choline (Cho)/ Cr ratio was 1.3 +/- 0.12 in HC-HB and 1.11 +/- 0.13 on the opposite normal side (P = 0.005). A lactate peak was seen in seven patients. The NAA/Cr ratio was 1.44 +/- 0.15 in bilateral CB and 1.74 +/- 0.16 in the controls (P = 0.017); the Cho/Cr ratios were 1.36 +/- 0.1 and 1.19 +/- 0.07 (P = 0.015). The low NAA/Cr suggests neuronal loss or damage and the high Cho/Cr probably indicates gliosis. The presence of lactate may suggest mild ischaemia due to acute vascular events during hyperglycaemia and underlying chronic focal cerebrovascular diseases in DM.
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PMID:In vivo proton MR spectroscopy of chorea-ballismus in diabetes mellitus. 1151 79

We describe a case of extrapontine myelinolysis in a child presenting with chorea and parkinsonian symptoms after treatment for hyponatremic dehydration. Although both extrapontine myelinolysis (EPM) in childhood and extrapyramidal presentation of EPM are very rare in children, the patient presented with pure extrapyramidal symptoms. Besides basal ganglia involvement and sparing brain stem, MRI demonstrated involvement of the amygdala bilaterally hitherto never reported in EPM patients. While single voxel proton spectroscopy (MRS) of the basal ganglia at the beginning was normal, the follow-up MRS showed moderate to severe NAA and mI decrease. Diffusion-weighted imaging abnormalities appeared relatively late during the disease course, also an unexpected finding. In addition, there were apparent increases in apparent diffusion coefficient (ADC) values of the affected basal ganglia instead of diffusion restriction.
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PMID:Extrapontine Myelinolysis in a Child: a Rare Case with MRI, DWI and MRS Follow-up. 2425 59