Severe Chorea Research Articles

Chorea in a patient with cerebral palsy: Treatment with levetiracetam

We report on the case of an adult cerebral palsy patient who developed severe chorea coincident with a febrile illness from a nonstreptococcal infection. The chorea improved markedly with the use of levetiracetam (LEV, Keppra).

Pain in Parkinson's Disease.

Pain is reported by nearly 50% of patients with Parkinson's disease. In some patients, it can be more debilitating than the motor deficits. In order to identify the appropriate treatment strategy for each patient, it is useful to categorize pain syndromes as follows: 1) low DOPA (end of dose wearing off, diphasic, or early morning) painful states are associated with inadequate levels of dopamine receptor stimulation; 2) high DOPA (peak dose) painful states occur at times of maximum levodopa efficacy; and 3) many patients report pain that has no obvious relation to dopaminergic medications or may even be caused by other conditions. Low DOPA painful states are best treated by trying to provide more continuous dopaminergic stimulation and thereby reduce or prevent the number and duration of "off" periods. Adding or increasing the dose of direct-acting dopamine receptor agonists or of catechol-o-methyl transferase inhibitors is the best first-line strategy. Other approaches include increasing the frequency of immediate-release levodopa preparations or using controlled-release preparations. More invasive approaches should be considered only when simpler methods fail. These include deep brain stimulation to the pallidum or the subthalamic nucleus, or direct duodenal continuous infusion of levodopa in patients who are unable to undergo surgery. Pain associated with excessive dopaminergic stimulation usually is a result of dystonia or severe chorea. Reduction of levodopa is the first step in attempting to diminish high DOPA states, followed by reduction or cessation of other dopaminergic agents such as selegiline, catechol-o-methyl transferase inhibitors, or direct-acting dopamine receptor agonists. Adding amantadine can reduce chorea significantly and it should be tried if the potential and actual side effects are tolerable to the patient. Deep brain stimulation is a good final option if medication adjustments are ineffective. Nonspecific pains of Parkinson's disease can be difficult to treat. The effective use of central pain suppressant or analgesics is anecdotal and difficult to verify. In untreated early disease, generalized pain or pain related to joint or muscle immobility may be reduced by effective treatment of the underlying Parkinson's disease.

Rate and correlates of weight change in Huntington’s disease*

Objective: To determine the rate and correlates of weight change in a large, well characterised sample of patients with Huntington’s disease followed at 44 sites by the Huntington Study Group....

Genetics of movement disorders and ataxia

Genetic disorders of the central nervous system have a propensity to cause movement disorders or ataxia, as a part of the phenotype, or sometimes as the main phenotypic manifestation. The...

Acute chorea due to ondansetron in an obstetric patient

This case describes a 34-year-old female who developed severe chorea following intravenous administration of ondansetron after elective caesarean section under spinal anaesthesia. A course of benztropine led to a decrease in severity of the reaction which resolved on the fifth postoperative day. Ondansetron is believed to cause extrapyramidal reactions through actions on 5HT 3 receptors in the basal ganglia. Whereas isolated case reports of extrapyramidal reactions to ondansetron do exist, they have typically been associated with higher doses and have resolved within hours. We report the lengthiest extrapyramidal reaction to ondansetron yet described.

Chorea in juvenile primary antiphospholipid syndrome: CASE REPORT

Chorea was observed in a 12-year-old girl with primary antiphospholipid syndrome (APS). She developed severe chorea in a few weeks. On immunosuppressive treatment, including high doses of glucocorticoids and cyclophosphamide, she had a rapid clinical recovery. Single photon emission computed tomography (SPECT) of the brain showed decreased circulation in the basal ganglia and in the medial parts of both temporal lobes. One month after treatment, SPECT was completely normalised. APS in children has a variety of clinical manifestations, and should be suspected in cases of unexplained thromboembolic disease or obscure neurological symptoms.

Movement disorders in association with herpes simplex virus encephalitis in children: a review.

Movement disorders have been reported in association with herpes simplex virus (HSV) encephalitis in adults and children. The cause of this complication is unclear, although a number of proposed aetiologies have been suggested, including inadequate antiviral therapy1–3, a side effect of anticonvulsants4, or a possible postinfectious mechanism5–8. A child who presented with severe chorea associated with relapse of HSV encephalitis prompted us to review the clinical features, management, and outcomes of this condition.

Chorea in fifty consecutive patients with rheumatic fever

Rheumatic fever (RF) remains a significant public health problem in developing countries. Although its incidence has been declining in developed areas, several outbreaks of RF have been identified in the United States since 1985. To study the prevalence and clinical features of Sydenham's chorea (SC), a well-recognized expression of RF, we examined 50 consecutive patients with RF seen at our Pediatric Cardiology Clinic. Chorea was rated on a scale of 0-4 (0 = no chorea; and 4 = severe chorea, patient unable to walk). Chorea, active or in remission, occurred in 13 patients (26%). The male-to-female ratio was 1.08 among the total population and 0.86 among the SC group. The mean age of the 50 patients at onset of RF was 8.4 years and the standard deviation (SD) 3.1 years. Carditis was diagnosed in 84% of patients with and without chorea, but arthritis was more common in the latter group (83.8%) than in the SC group (30.8%). The mean age at onset of SC was 9.2 years (SD 2.7). Involuntary movement, noticed by nine patients, was the most common initial symptom. Chorea went into remission in eight patients after 9.9 months (SD 13.4). Chorea was rated 3 in ten patients, 1 in two patients, and 4 in one patient. Among the five patients with active SC, three had hemichorea and two had generalized chorea. We recognized abnormal saccades in 80% of patients with active chorea, whereas just 13.5% of patients without chorea displayed this abnormality. SC remains an important cause of chorea in countries plagued by RF.

The syndrome of autosornal recessive pontocerebellar hypoplasia, microcephaly, and extrapyramidal dyskinesia (pontocerebellar hypoplasia type 2)

The syndrome of autosomal recessive pontocerebellar hypoplasia, microcephaly, severely impaired mental and motor development, and extrapyramidal dyskinesia is a distinct system degeneration, previously designated pontocerebellar hypoplasia type 2 (PCH-2). To further characterize its clinical and neuroimaging features, we compiled data from 10 nonrelated pedigrees. Six pedigrees were Dutch, two Swedish, and two German. All 16 patients showed an identical profile of virtually absent developmental milestones, early-onset severe chorea, and microcephaly together with pontocerebellar hypoplasia. Family distribution supports autosomal recessive transmission. The present data support the PCH-2 phenotype as a distinct neurogenetic entity.

Severe evening dyskinesias in advanced Parkinson's disease: Clinical description, relation to plasma levodopa, and treatment

We report four patients with Parkinson's disease who had an unusual pattern of severe chorea and dystonia in the evenings only. The temporal pattern of abnormal movements and simultaneous monitoring of plasma levodopa and clinical state were consistent with dyskinesias associated with subtherapeutic (low dopa dyskinesias) rather than peak concentrations of levodopa (high dopa dyskinesias). In two patients, addition of a direct-acting dopamine receptor agonist was helpful in ameliorating this complication of antiparkinson therapy.

Pallidal GABA and chorea in Huntington's disease

Neurochemical correlates of chorea in Huntington's disease were studied using striatal and pallidal tissue taken post mortem from patients with mild and severe chorea. While GABA was decreased in all these areas in Huntington's disease, patients with mild chorea had significantly less GABA in the medial pallidum than did those with severe chorea. There was no relationship between the degree of chorea and concentrations of dopamine or its metabolite. Thus the chorea of Huntington's disease may relate to the balance of residual GABAergic innervation between specific areas of the basal ganglia, consistent with primate models of dyskinesias.

Chorea in polycythemia vera: a rare presentation of hyperviscosity

A 65-year-old woman developed severe chorea as the presenting symptom of the hyperviscosity syndrome together with polycythemia vera and an ovarian cyst. Whole blood viscosity was 69.5 centipoise at a shear rate of 1.1 s-1 (normal 12.0-22.0 centipoise). After four phlebotomies the chorea subsided completely, and the blood viscosity became normal. Hemoglobin level was stable at normal range and then increased to 18.0 g/dl with recurrence of the faciobuccal dyskinesia that disappeared promptly following the phlebotomies. This case emphasizes that in elderly patients with neurological disorders, such as chorea, polycythemia should be strongly considered as one of the treatable causes.

Looking Back at Psychiatry in General Practice

As students at the Durham medical school in Newcastle during the early thirties, we were taught that illness could be due to certain factors and these were listed. The malady could be caused by infection, acute or chronic, trauma, neoplasia. … there were many possibilities but at the very end was a sort of garbage bin for all the diseases which did not fit neatly into the organic syndromes; these were called hysteria or psychoneurosis. These functional cases were diagnosed in a purely negative way, by a process of exclusion. No attempt was made to explain possible reasons for these troubles and there were no suggestions as to how to treat them. We were led to believe that such people were no more than time-wasters, suffering from unworthy maladies. If neurosis in all its forms gave rise to disdain, psychosis produced overt panic, as exemplified in the following case history. A young woman of 20 was admitted to a medical ward in the late stages of pregnancy because she was suffering from severe Sydenham's chorea. She was so bad, she was constantly in danger of falling out of bed and she was quite unable to speak coherently. It must have been because of this she was deemed mad, and a respectable medical ward was certainly no place for a lunatic. Fear in both the medical and nursing staff had completely obliterated all feelings of compassion. She was certified and transferred to the asylum where she died the next day. It was well known that chorea gravis carried with it a bad prognosis and, after all, there were side wards. The poor lass should have been allowed to die in dignity in one of them.

Valproic acid in the treatment of Sydenham chorea

Severe cases of Sydenham chorea are still difficult to manage because optimal therapy has not been found. In this report severe chorea responded to valproic acid therapy after trials with diazepam and haloperidol had failed. The literature is reviewed and the possible mechanism of action of valproate in this movement disorder is discussed. Valproic acid is proposed as an effective treatment for severe cases of Sydenham chorea.

Neuronal storage disorder with chorea and curvilinear bodies.

A clinically unique case of ceroid-Iipofuscinosis is described with a juvenile onset, severe chorea that dominates the clinical picture, absence of retinal changes, and uniform intraneuronal inclusions consisting of curvilinear bodies. Cerebral biopsy was performed and ultrastructural and histochemi-cal studies were made. This case differs clinically from the classic late infantile type of nonglycolipid neuronal storage disorder and it differs clinically and ultrastructurally from the classic juvenile type with pigmentary degeneration of the retina. Several atypical variants without visual loss have been reported, but this case appears morphologically distinct from the few cases with a dystonic form recently described ultrastructurally. It supports the notion of nosologic heterogeneity among the nonglycolipid neuronal storage disorders.

Severe persistent chorea with phenothiazine therapy: report of a case.

Severe persistent chorea with phenothiazine therapy: report of a case.

A case of severe chorea gravidarum treated with promazine (sparine).

A case of severe chorea gravidarum treated with promazine (sparine).

Severe chorea gravidarum treated with corticotrophin: Forrest and Hales: p. 874

Severe chorea gravidarum treated with corticotrophin: Forrest and Hales: p. 874

Severe chorea gravidarum treated with corticotrophin.

Severe chorea gravidarum treated with corticotrophin.

A CASE OF SEVERE CHOREA ASSOCIATED WITH TAPEWORM, WITH REMARKS ON THE TREATMENT OF THIS DISEASE.

A CASE OF SEVERE CHOREA ASSOCIATED WITH TAPEWORM, WITH REMARKS ON THE TREATMENT OF THIS DISEASE.