[Parietal amyotrophy (Silverstein's syndrome). A new case. Letter].
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Biomedical subjects
Publications and source records attributed to J Vaamonde-Gamo.
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INTRODUCTION: Paroxysmal movement disorders are of sudden onset, paroxystic or intermittent, with normality or at least absence of abnormal movements between episodes. They are uncommon conditions with high familial incidence. The commonest form is paroxystic dystony. Paroxystic ataxia and paroxystic tremor are much rarer. DEVELOPMENT: Paroxystic dystony is seen clinically as paroxystic episodes of variable duration, consisting of dystonic movements and postures which present spontaneously or are triggered by certain stimuli or situations. There are different types: kinesgenic paroxystic choreoathetosis, dystonic paroxystic choreoathetosis, intermediate paroxystic choreoathetosis and nocturnal paroxystic choreoathetosis. The paroxystic ataxias are characterized by recurrent episodes of ataxia in the absence of any known metabolic defect. In type 1 episodic ataxia there are also episodes of ataxia and myochemical changes. The gene responsible for this condition has been found on the short arm of chromosome 12. Type 2 episodic ataxia is characterized by its excellent response to acetazolamide. The gene responsible is found on the short arm of chromosome 19. CONCLUSION: Very few cases of paroxystic tremor have been described.
INTRODUCTION: Dementia with Lewy bodies (DLB) is one of the main differential diagnosis of Alzheimer's disease (AD). In DLB there is 40-70% loss of striatal dopamine and the loss of dopaminergic cell is accompanied by loss of the dopamine transporter. The loss of dopaminergic neurons in DLB can be confirmed in vivo with I-FP-CIT (DaT-SCAN), a pre-synaptic dopamine transporter marker. There are no changes in DaT-scan in AD compared with controls. AIM. The use of DaT-SCAN for the differential diagnosis between AD and DLB. CASE REPORTS: We use the DaT-SCAN to study the nigrostriatal pathway in 6 patients with dementia and moderate parkinsonism. The Mini-Mental Test and the Unified Parkinson's Disease Rating Scale, motor part only (UPDRS), were performed. A SPECT scan was carried out 3 to 4 hours after administration of 185 MBq FP-CIT (ioflupane) a dopaminergic presynaptic ligand. With occipital cortex used as a radioactivity uptake reference, ratios for the caudate nucleus and the anterior and posterior putamen of both hemispheres were calculated. All scans were also rated by a simple visual method. All patients had dementia with moderate fluctuations in cognitive function, parkinsonian syndrome and hallucinations. DaT-SCAN was normal in 2 patients and pathological in 4. CONCLUSION: The sensitivity and specificity of the DLB criteria vary markedly. FP-CIT SPECT may be a new tool in the differential diagnosis between DLB and AD.
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