Case Of Chorea Research Articles

Chorea associated with gabapentin use in an elderly man

Background: Chorea is a hyperkinetic movement disorder characterized by irregular, flowing, nonstereotyped, random, involuntary movements. Huntington disease (HD) and drug-induced chorea account for >50% of adult-onset cases. Chorea associated with gabapentin, an anticonvulsant, has not been well documented. Objective: The purpose of this article was to report a case of chorea that developed in an elderly man being treated with gabapentin for severe anxiety. Case summary: A 75-year-old white man (height, 165.1 cm; weight, 65.8 kg; body mass index, 19.6 kg/m2) with anxiety disorder not otherwise specified was admitted to a geriatric medicine psychiatric unit in Connecticut because of worsening symptoms of anxiety affecting his cognitive ability. On evaluation, the patient had choreiform movements involving the neck, trunk, upper and lower extremities, and tongue. The patient reported that symptoms began after taking gabapentin 300 mg PO TID (prescribed by his geriatrician) for the treatment of anxiety. The patient had been taking gabapentin for >1 month when the symptoms first appeared. There was no known family history of HD, and patient workup was unremarkable for other conditions (eg, vascular disease of the brain, progressive dementia, infectious and metabolic disorders) that might present with chorea. The chorea lasted for ~4 months and resolved within 2 days after gabapentin discontinuation. Conclusion: This article reports a case of chorea in an elderly patient who was receiving gabapentin for the treatment of anxiety. After gabapentin discontinuation, the chorea resolved completely, indicating a probable adverse drug reaction.

A Case of Chorea as the Initial Manifestation of SLE Triggered by Estrogen

Neuro log ica l com plications of system ic lupus erythem atosus (SLE) are re lative ly com m on, but chorea as the in itia l m an ifestation o f SLE unm asked by exogenous estrogen is very rare. A -46 -year o ld right handed wom an presented w ith genera lized chorea s ince 2 weeks ago. Her m edica l records revea led that the chorea had appeared w ith in one m onth after estrogen m ed ication. The laboratory test revea led pos itive antinuc lear antibody (ANA ), pos itive anti-dsDNA and pos itive anti-h is tone antibody. A fter d iscontinuation o f estrogen, her chore ic m ovem ent was not d im in ished. W e report a case of new ly d iagnosed SLE attribute to chorea wh ich triggered by estrogen. Journal of Movement Disorders 1(2):86-89, 2008

Sydenham’s chorea

Sydenham's chorea (SC) is a manifestation of acute rheumatic fever (ARF). Although the incidence of SC has declined significantly, particularly in developed areas, it remains the most common cause of acute chorea in children worldwide. Recent data show that SC accounts for almost all cases of chorea in children in the United States. As there is no specific laboratory marker of this condition, the diagnosis relies on a careful clinical history and laboratory assessment to rule out alternative causes. Morbidity is primarily related to cardiac lesions in ARF. It is recommended that all patients with suspected SC submit to a cardiologic evaluation. Neurologic features encompass motor signs, among which chorea is the most prominent, and nonmotor symptoms such as obsessive-compulsive behavior and attention-deficit/hyperactivity disorder. The first-line treatment of SC is valproic acid. Patients who do not respond to this drug or who present with severe chorea (particularly chorea paralytica, in which the muscle tone is so decreased that patients are bedridden) should be treated with risperidone. Other dopamine receptor-blocking drugs, such as haloperidol, may also be useful. There is growing evidence that immunosuppressive treatment, such as intravenous methylprednisolone followed by a tapering course of oral prednisone, is effective. This option has been used in patients who failed to respond to (or did not tolerate) the previously mentioned therapies. Plasmapheresis and intravenous immunoglobulin are regarded as experimental treatments. Obsessive-compulsive behavior associated with SC is not usually as severe as in other conditions such as Tourette's syndrome. Finally, because at least 20% of patients with SC experience recurrent attacks of ARF, they carry a high risk of developing severe cardiac lesions. These patients require prophylaxis against streptococcus infection, using penicillin or sulfa drugs.

Uncommon neurological manifestations of hemolytic anemia: A report of two cases

Neurological complications of hemolytic anemias are rather uncommon. We are reporting two cases of hemolytic anemia presenting as chorea and recurrent ischemic stroke. The first one is a case of chorea in a patient with sickle cell trait. Reviewing the literature we could find only one case report of chorea in sickle cell disease disease. The second is a case of recurrent ischemic stroke in hereditary spherocytosis. We could trace two reports on a Medline search, though their association was less certain.

The Huntington's disease-like syndromes: what to consider in patients with a negative Huntington's disease gene test

Huntington's disease (HD), which is caused by a triplet-repeat expansion in the IT15 gene (also known as huntingtin or HD), accounts for about 90% of cases of chorea of genetic etiology. In recent years, several other distinct genetic disorders have been identified that can present with a clinical picture indistinguishable from that of HD. These disorders are termed Huntington's disease-like (HDL) syndromes. So far, four such conditions have been recognized, namely disorders attributable to mutations in the prion protein gene (HDL1), the junctophilin 3 gene (HDL2), and the gene encoding the TATA box-binding protein (HDL4/SCA17), and a recessively inherited HD phenocopy in a single family (HDL3), the genetic basis of which is currently poorly understood. These disorders, however, account for only a small proportion of cases with the HD phenotype but a negative genetic test for HD, and the list of HDL genes and conditions is set to grow. In this article, we review the most important HD phenocopy disorders identified to date and discuss the clinical clues that guide further investigation. We will concentrate on the four so-called HDL syndromes mentioned above, as well as other genetic disorders such as dentatorubral-pallidoluysian atrophy, neuroferritinopathy, pantothenate-kinase-associated neurodegeneration and chorea-acanthocytosis.

Choreatische Bewegungsstörungen

The differential diagnosis of chorea includes a growing number of rare diseases. This article gives hints on clinical differences and possible laboratory investigations which may help to identify the underlying disease. The majority of hereditary chorea cases are caused by Huntington's disease. Different courses of disease can be distinguished depending on age at disease onset. The diagnosis can be confirmed genetically. Predictive testing is also possible but should be applied with caution only following internationally accepted guidelines. Our knowledge about treatment of chorea is limited, and studies have focused on the use of neuroleptics only. Their value is often outweighed by serious side effects. All efforts to find disease-modifying therapies for Huntington's disease had negative outcomes so far. To face these therapeutic limitations, the European Huntington's Disease Network was formed as a platform supporting the development and undertaking of clinical studies in Huntington's disease to improve care for these patients.

An Australian guideline for rheumatic fever and rheumatic heart disease: an abridged outline

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are diseases of poverty. They occur at world-record rates in Indigenous Australians, yet individual cases are often poorly managed, and most jurisdictions with high rates of these diseases do not have formal control strategies in place. New Australian guidelines formulated in 2005 by the National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand for diagnosis and management of ARF and RHD are a valuable resource for clinicians and policymakers. Key recommendations of the guidelines include: New diagnostic criteria for ARF in high-risk populations, including Indigenous Australians, which include echocardiographic evidence of subclinical valvular disease, and polyarthralgia or aseptic monoarthritis as major manifestations. Clear guidance about treatment of ARF. Non-steroidal anti-inflammatory drugs should be withheld until the diagnosis is confirmed, and corticosteroids may be an option in severe acute carditis. Most cases of chorea do not require medication, but use of carbamazepine or sodium valproate is recommended if medication is needed. Clear guidance about dose, dosing frequency and duration of secondary prophylaxis. Benzathine penicillin G is the preferred medication for this purpose. Establishment of a coordinated control program for all regions of Australia where there are populations with high prevalence of ARF and RHD. Key elements and indicators for evaluation are recommended. Active screening and legislated notification of ARF and RHD, where possible. Development of a structured care plan for all patients with a history of ARF or with established RHD, to be recorded in the patient's primary health care record.

Mouvements choréiques résolutifs sous traitement anti-rétroviral au cours d’une encéphalite à VIH

Infection of the central nervous system with human immunodeficiency virus (HIV) can be associated with movement disorders. A case of chorea during HIV encephalitis which responded well to antiretroviral therapy is reported. Choreic movements disappeared with a decrease of MRI lesions observed in basal ganglia. The efficacy of anti-retroviral therapy in choreic movements, a rare syndrome with HIV encephalitis, can be underlined.

Camillo Golgi: A Clinical Pathologist

Camillo Golgi opened new avenues in histology and neurobiology as well as in clinical neurology and internal medicine. This is demonstrated by investigations Golgi performed during 1861–1876 on the etiology and pathology of mental diseases, on the neuropathology of Huntington's chorea (Golgi provided the first detailed description at the microscopic level of pathological changes in the basal ganglia and cerebral cortex of one case of chorea), on meningiomas and cerebral gliomas. In the period following these investigations, Golgi focused especially on infectious diseases. He pursued fundamental studies on malaria (which remain among the most important and original of his contributions) on rabies, as well as on smallpox and influenza. Thus, Camillo Golgi should be remembered for his discoveries of the black reaction and the Golgi apparatus, as well as for the modern impulse he was able to give to clinical neurology and internal medicine.

Hypermetabolism of basal ganglia in chorea associated with antiphospholipid antibodies demonstrated by F-18 FDG

A brain FDG PET study was performed on a 21-year-old woman with subacute chorea of unknown origin. Associated with her chorea, she had abnormal levels of antiphospholipid antibodies. She had none of the classical features of SLE nor primary antiphospholipid syndrome. The images showed high F-18 FDG uptake in the basal ganglia, while the brain MRI and EEG were normal. An association between chorea and antiphospholipid antibodies had been demonstrated before, with normal brain CT, MRI, 123IMPSPECT and cerebral angiography. The report suggests the advantage of FDG PET in imaging of unexplained cases of chorea associated with antiphospholipid antibodies.

Chorea and high antiphospholipid antibodies: probable primary antiphospholipid syndrome

A young man presented with generalized chorea as the first manifestation of probable primary antiphospholipid syndrome. He was well till 3 months before admission when he started to have involuntary, choreiform movements involving all extremities, the head and the bulbar muscles. Apart from these movements his physical examination was otherwise unremarkable. Laboratory investigations revealed mild thrombocytopenia, high partial thromboplastin time (PTT) only partially corrected by the addition of normal plasma, false positive syphilis serology, weakly positive antinuclear antibody and a high level of IgG anticardiolipin antibodies. Brain magnetic resonance imaging (MRI) showed multiple scattered small areas of high signal intensity on T2 weighted image in the area of centrum semiovale bilaterally. The patient was started on aspirin and prednisone with rapid symptomatic improvement. Despite the difficulty in proving the association between chorea and the high antiphospholipid antibodies, chorea appears in this case to be the initial symptom of primary antiphospholipid syndrome and we suggest screening for antiphospholipid antibodies in unexplained cases of chorea.

Chorea and polycythaemia.

An analysis is presented of 35 cases of chorea as a symptom of polycythaemia. This analysis reveals: (a) whereas polycythaemia occurs predominantly in males (3:2), polycythaemic chorea (PC) occurs predominantly in females (5:2), at a real ratio of female:male = 4:1, the prevalence being 1-2.5% of polycythaemic patients; (b) PC manifests predominantly after the age of 50 (8 cases before, 27 after 50 years), making polycythaemia the first disorder to be considered in cases of so-called 'senile' chorea; (c) PC is generalised, with predominant involvement of faciolingual and brachial muscles, and associated with muscular hypotonia; (d) PC may last from periods of weeks to years, usually responds to haloperidol, venesection or 32P-treatment, but may persist, or recur with treatment, or remit spontaneously, and (e) no relationship exists between the choreatic syndrome and (the rare finding of) a small infarct in the caudate nucleus. The cause of the choreatic syndrome in polycythaemia is presumably to be explained as a neostriatal hyperviscosity syndrome producing venous stasis, reduced brain blood flow and impaired tissular O2/glucose metabolism. The state of dopaminergic hyperactivity is presumably enhanced by relatively increased neostriatal catecholestrogens. The hypothesis of polycythaemic excess of dopamine-laden platelets releasing excess of dopamine in the neostriatum needs to be confirmed by laboratory evidence of platelet counts.

A case of chorea due to polycythaemia vera

The history of a 76 years-old woman suffering from chorea caused by polycythaemia vera is reported. Initial treatment with repeated phlebotomy followed by intravenous administration of P 32 led to the disappearance of both the polycythaemia and the chorea. Since abnormal CBF in the region of the basal ganglia has been postulated to be responsible for this syndrome, a dynamic CT examination was performed, but no significant abnormalities were found.

Increased dopamine receptor sensitivity after estrogen treatment using the rat rotation model.

Estrogen was administered to male rats that had received unilateral injections of 6-hydroxydopamine into the striatum. Following this treatment, their duration of rotation increased in response to amphetamine. Estrogen treatment resulted in a corresponding increase in the number of striatal dopamine receptors. Therefore, both behavioral and biochemical evidence suggests that striatal dopamine function is influenced by peripherally administered estrogens. These results are relevant to the clinical cases of chorea associated with elevated concentrations of estrogen, which occur in pregnancy and during oral contraceptive use.

Clinical features of chorea associated with systemic lupus erythematosus

Neurologic complications of systemic lupus erythematosus (SLE) are common, but chorea is rare. Three cases of chorea associated with SLE are presented as well as a review of 28 cases from the world literature. Chorea may be the first and at times the only sign prior to the establishment of diagnosis of SLE. Under such conditions the establishment of the correct diagnosis is difficult, and the chorea may be attributed to a more common etiology. Also the SLE-related chorea may recur in the same subject during the course of the disease. Finally, the chorea associated with SLE can be brought under control by use of haloperidol (Haldal ®).

A psychiatric follow-up of children with rheumatic fever and other chronic diseases

1. 1. Previous data [2] led to the theory that rheumatic fever originating during puberty might often produce nervous-system symptoms which mimic ‘functional’ mental disorder. The present psychiatric follow-up of children with rheumatic fever and other chronic diseases was undertaken to test this theory. 2. 2. Children first showing rheumatic symptoms during puberty (peak at ages 12 to 13) were found to have subsequent ‘functional’ psychiatric records about four times as often as children with rheumatic disease at other ages, or with non-rheumatic disease at any age. This trend occurred in a sample of New York children as well as in a sample of Colorado children, and was found among rheumatics without chorea as well as among chorea cases. 3. 3. Clinically non-rheumatic children, whose greatest known risk of rheumatic fever occurred at puberty, also showed an increased rate of psychiatric disorder. It was suggested that some of these cases actually acquired rheumatic fever at the time of risk, but that the classical rheumatic symptoms remained subclinical while the hypothesized symptom of rheumatic mental disorder occasionally became manifest (particularly among those affected at puberty). 4. 4. The type and extent of treatment suggested that more severe psychiatric disorders were encountered in the rheumatic groups than in the controls. Qualitatively, the chronic, insidious schizophrenias and the psychiatric disorders with antisocial implications predominated in the rheumatic groups.

Hemichorea due to metastatic lesion in the subthalamic nucleus.

Although the association of ballism and chorea with localized lesions in the subthalamic nucleus appears well established, 1-3 and experimental studies 4-7 have demonstrated that a similar form of dyskinesia can be produced by lesions in this nucleus in the rhesus monkey, relatively few cases of chorea or ballism due to metastatic lesions of the subthalamic nucleus have been reported. According to Whittier, 2 ballism most frequently occurs in hypertensive persons as a consequence of localized hemorrhage into the corpus Luysi (subthalamic nucleus). Only two reports 8,9 of ballism due to metastatic neoplasms (both primary in the breast) have been reported; this syndrome also has been reported in association with a discrete tubercle. 10 The current case was considered noteworthy not only because the dyskinesia appeared to be associated with a discrete metastasis but also because the chorea developed slowly, was somewhat intermittent in severity, and was exaggerated by the

Cortisone in Sydenham's chorea; report of two cases.

Beneficial effects of cortisone and pituitary adrenocorticotropic hormone (ACTH) on acute manifestations of rheumatic fever have recently been described.¹In particular, the effects on rheumatic polyarthritis and carditis have been studied. Since acute chorea is generally believed to be another manifestation of rheumatic fever,²we considered it of interest to determine the effects of cortisone in two cases of chorea that have recently come under our observation. To one of the patients pituitary adrenocorticotropic hormone was also administered. <h3>REPORT OF CASES</h3><h3>Case 1.—</h3> J. S., a 3 year old boy, was admitted to Beth Israel Hospital on June 18, 1950. Four weeks previously he had experienced a gradual onset of weakness of both arms and legs, associated with uncontrollable, purposeless movements of his limbs and face. Difficulty in swallowing and in speaking followed, and the patient was unable to sit or stand without support. Two weeks prior to

Post-Choreic Personality and Neurosis

In the relatively short period which has elapsed since the important findings about personality changes after encephalitis epidemica became known, those findings have been mainly responsible for the enormous advance in our knowledge of the sub-cortical direction of man's emotional and volitional life. This advance in psychopathology subsequently gave rise to the question whether similar sequelae could be found in the personality structure of those who had suffered from other forms of post-infectious encephalopathies. Chorea, which had already offered the puzzling problem of why the rheumatic virus becomes “neurotropic” in certain cases, was amongst the first to be considered, whereas 25 years ago no psychiatrist would have seen a special problem in the personality structure after choreic illness. The first investigations which dealt with entirely new problems in chorea minor were those of E. Straus, (1927), who found thathyperkinetic symptomsare frequently found, either in a generalized form, or as localized tics as sequelae of chorea minor, and of E. Guttmann (1927) and Schulz (1928), who both showed thathereditary predispositionof the brain plays an important role in persons who fall ill with chorea minor. Afollow-up studyof chorea cases with regard to personality development had not been undertaken until the present author's first publication on chorea (1934).

RESULTS OF TREATMENT OF ATHETOSIS BY SECTION OF EXTRAPYRAMIDAL TRACTS IN THE SPINAL CORD

CLASSIFICATION OF THE DYSKINESIAS Certain disorders characterized by abnormal involuntary muscular movements have been grouped together as of the extrapyramidal system, chiefly on the basis of the localization of the lesions observed at autopsy (Wilson, 1 Herz 2 ). They include athetosis, dystonia, torsion spasm, spasmodic torticollis, ballism, chorea, myoclonus and paralysis agitans. An important reason for excluding paralysis agitans from this list has already been given in a previous paper, 3 namely, that an extensive section of extrapyramidal motor pathways makes the tremor rigidity neither better nor worse. Further, recent pathologic investigations 4 have appeared to indicate that the alterations are by no means confined to the basal nuclei. No cases of chorea of either the Sydenham or the Huntington type have been sufficiently studied by modern physiologic methods to permit a comparison with the other diseases in the group. But the remaining syndromes in the list possess so