1/6. Serial brain SPECT images in a case of Sydenham chorea.BACKGROUND: The pathophysiological nature of Sydenham chorea (SC) has been presumed to be an autoimmune-mediated inflammatory process. Positron emission tomography in SC has revealed a striatal hypermetabolism that might explain the transient neuronal dysfunction. However, any focal hyperperfusion in the striatum or its related structures has not been demonstrated in previous single photon emission computed tomographic (SPECT) imaging studies, which raised a concern about the pathogenesis of the striatal hypermetabolism. OBJECTIVE: To investigate the cerebral perfusion patterns of the subcortical structures by using serial technetium Tc 99m-ethyl cysteinate dimer SPECT in a case of SC, which may provide a clue for the pathophysiological mechanisms. DESIGN: A case report and serial SPECT studies. CASE PRESENTATION: A girl aged 4 years 3 months showed severe generalized choreic movements with concomitant signs of acute pharyngitis. Results of a laboratory study taken 7 days after the onset of chorea showed elevated antistreptolysin O titer, c-reactive protein levels, and erythrocyte sedimentation rate. Other laboratory data, throat culture, echocardiography, brain magnetic resonance imaging, and electroencephalography did not reveal any abnormalities. Five days after treatment with haloperidol and penicillin, the chorea began to improve slowly, and completely resolved in 2 months. RESULTS: Three serial SPECT images and semiquantitative analysis of cerebral perfusion were obtained. Cerebral perfusion in the striatum and thalamus was markedly increased bilaterally during the stage of active chorea and then returned nearly to its baseline level during the convalescent phase. These cerebral perfusion patterns were concordant with semiquantitative analysis. CONCLUSIONS: Hyperperfusion in both the striatum and thalamus in our patient may reflect the subcortical inflammatory processes in SC. The unequivocal SPECT findings in our patient are difficult to reconcile with the negative findings of previous SPECT studies but may suggest the heterogeneity of the perfusion patterns in SC.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/6. A combined pattern of movement disorders resulting from posterolateral thalamic lesions of a vascular nature: a syndrome with clinico-radiologic correlation.We report a series of seven patients in whom a combined pattern of complex movement disorders restricted to one upper extremity emerged as a result of posterolateral thalamic lesions of vascular origin. This disorder was mainly characterized by choreiform and dystonic movements associated with variable, rhythmic, alternating movements of low frequency (myorhythmia). All cases showed, on computed tomography scan and/or magnetic resonance imaging, focal lesions involving the posterolateral quadrant of the thalamus. review of similar cases reported with identical clinico-radiologic features allows us to conclude that it is possible to establish an accurate anatomoclinical correlation based on the clinical phenomenology, even before imaging studies are performed, in these cases. The opposite is not entirely possible, however, because lesions in the same quadrant of the thalamus are often associated with different patterns of abnormal movements or present without abnormal movements.- - - - - - - - - - ranking = 4keywords = nature (Clic here for more details about this article) |
3/6. phenytoin-induced choreoathetosis in patients with severe myoclonic epilepsy in infancy.We describe three patients with severe myoclonic epilepsy in infancy (SME) who suffer from choreoathetosis due to the adverse effect of phenytoin. Choreoathetosis appeared when these patients were 8, 19, and 21 years old, 2 days to 6 months after increasing the phenytoin dosage. Choreoathetosis disappeared when the phenytoin dosage was decreased. The two elder patients experienced episodic and rather paroxysmal onset of long-lasting choreoathetosis, requiring the differential diagnosis from degenerative disease. In one of the patients, an ictal SPECT revealed decreased perfusion in the basal ganglia contralateral to the unilateral choreoathetosis. polypharmacy, including carbamazepine and zonisamide, may have facilitated the onset of choreoathetosis. phenytoin-induced choreoathetosis in the patients with SME is an important differential diagnosis among degenerative disorders involving involuntary movements. The episodic and paroxysmal nature of this movement disorder can delay its diagnosis and effective treatment. patients with SME appear to be particularly vulnerable to this side effect of phenytoin, indicating the possible involvement of basal ganglia in the pathophysiology of this type of epilepsy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/6. Senescent cell antigen, band 3, and band 3 mutations in cellular aging.As part of our ongoing studies on mechanisms of cellular aging, we searched for "experiments of nature" that might provide insights into the process of normal cellular aging. Our search for band 3 protein alterations resulted in the discovery of three different ones. A band 3 alteration that results from an addition to band 3 does not alter red cell lifespan or produce clinical disease. In contrast, two band 3 alterations that are associated with band 3 aging and/or degradation are characterized by shortened red cell lifespan and clinical diseases.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/6. Kinesigenic choreoathetosis due to brain injury.This uncommon paroxysmal movement disorder featuring attacks of dystonic spasm with athetoid posturing is usually of idiopathic nature, often familial and starting in childhood. There are a few reported examples due to cerebral pathology, and only four previous cases due to brain trauma. We report here a 21 year old man with left-sided motor spasms, choreoathetoid in type, which were clearly caused by a right frontal penetrating injury with contusion and haemorrhage. The attacks were relieved by phenytoin therapy. In this case, it seems that the post-traumatic paroxysmal kinesigenic choreoathetosis may be a form of reflex epilepsy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/6. phenytoin-induced dystonia and choreoathetosis in two retarded epileptic children.Similar movement disorders developed in two 8-year-old retarded children while they were receiving phenytoin. seizures subsequent to a diphtheria-pertussis-tetanus immunization had developed in each child at 1 to 2 months of age. A static encephalopathy ensued, characterized by mental retardation, ataxia, spasticity, and a mixed seizure disorder. Intermittent dystonia and choreoathetosis developed insidiously while serum phenytoin concentrations were in the therapeutic range. Sustained dystonia and choreoatheosis developed 2 hours after an oral provocation with phenytoin. The baseline abnormalities on the electroencephalogram remained unchanged during the choreoathetosis. Recognizable metabolic abnormalities known to be associated with similar movement disorders were excluded. It was concluded from these studies that the movement disorder is secondary to phenytoin and can occur at therapeutic serum concentrations. phenytoin is a central anticholinergic agent and a central stimulant of serotonin, and may induce movement disorders as a result of altering these neurotransmitters in the brain. The variable expression of these movement disorders may relate to the nature of the preexisting striatal insult.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |