Discinesia paroxística primaria kinesiogénica y no kinesiogénica: Reporte de 3 casos y revisión de la literatura / Paroxysmal Kinesiogenic Dyskinesia and Paroxysmal Non-Kinesiogenic Dyskinesia: Three Case Reports and a Review

Las discinesias paroxísticas son un grupo de entidades consistentes en paroxismos de diversos movimientos anormales de corta duración asociados o no a factores precipitantes. Suele afectar a sujetos jóvenes y la prevalencia es desconocida. La fisiopatología es incierta; se han identificado ciertas mutaciones que expliquen su origen. Clínicamente se pueden manifestar como ataques paroxísticos de movimientos de tipo coreoatetósico, distónicos o balísticos de corta duración y con preservación de la conciencia. Los estudios electrofisiológicos y de imagen suelen ser normales. Este grupo de trastornos del movimiento hacen parte del diagnóstico diferencial de las crisis epilépticas. El pronóstico suele ser bueno y el tratamiento es sintomático con anticonvulsivantes. Se presentarán tres casos de dos tipos de trastornos paroxísticos del movimiento y revisión de la literatura.

Paroxysmal dyskinesias are a group of entities consisting of paroxysms of diverse abnormal movements of short duration, associated or not with precipitating factors. It usually affects young subjects and its prevalence is unknown. The pathophysiology is uncertain; some mutations have been identified that explain their origin. Clinically, they can manifest as paroxysmal attacks of choreoathetosis, dystonic or ballistic movements of short duration and with preservation of consciousness. Electrophysiological and imaging studies are usually normal. This group of movement disorders are part of the differential diagnosis of epileptic seizures. Prognosis is usually good and the treatment is symptomatic with anticonvulsants. Three cases of two types of paroxysmal movement disorders and a review of the current literature are presented.

Distúrbio de movimento por conta de estado hiperosmolar não cetótico em idosa: relato de caso / Movement disorder caused by hyperosmolar hyperglycemic state in an older adult: a case report

O diabetes e suas complicações constituem as principais causas de mortalidade precoce na maioria dos países. O envelhecimento da população e a crescente prevalência da obesidade e do sedentarismo, além dos processos de urbanização, são considerados os principais fatores responsáveis pelo aumento da incidência e da prevalência do diabetes mellitus (DM) em todo o mundo. Este relato de caso objetiva descrever a presença de distúrbio do movimento em idoso por conta do estado hiperosmolar não cetótico. A combinação de hemicoreia-hemibalismo, hiperglicemia não cetótica e envolvimento dos gânglios da base em exames de imagem é considerada uma síndrome única. Os distúrbios do movimento em estado hiperosmolar não cetótico apresentam resposta terapêutica satisfatória com o uso de neurolépticos e controle glicêmico adequado. A escassez de trabalhos publicados proporciona subdiagnósticos clínico e laboratorial, interferindo no prognóstico e no acompanhamento dos pacientes.

Diabetes mellitus (DM) and its complications constitute the leading causes of early mortality in most countries. Population aging and the growing prevalence of obesity and sedentary lifestyles, in addition to spreading urbanization, are considered the main drivers of the increasing incidence and prevalence of DM worldwide. This case report describes the acute onset of movement disorder in an older woman secondary to hyperosmolar hyperglycemic state (HHS). The combination of hemichorea­hemiballismus, HHS, and evidence of basal ganglia involvement on neuroimaging is considered a unique syndrome. Movement disorders secondary to HHS respond satisfactorily to administration of neuroleptic agents and proper glycemic control. The lack of published studies on this pathologic entity may lead to clinical and laboratory underdiagnosis, with negative impacts on patient prognosis and follow-up.

Como a coréia de Sydenham é tratada no Rio de Janeiro? / How Sydenham's chorea is treated in Rio de Janeiro?

INTRODUÇÃO: A coréia de Sydenham é a principal causa de coréia adquirida na infância no Brasil. Assim, torna-se relevante saber como os médicos tratam os pacientes coréicos no nosso meio. OBJETIVO: Descrever a prática médica informada em coréia de Sydenham entre pediatras. MÉTODO: Estudo observacional descritivo seccional realizado por entrevistas feitas entre pediatras de emergência e especialistas dos hospitais públicos do Município do Rio de Janeiro. RESULTADOS: 74 por cento dos entrevistados referiu não usar escalas de gravidade; somente 81 por cento dos médicos fazem uso regular de penicilina benzatina; 64 por cento referem iniciar tratamento farmacológico; 28,3 por cento usam apenas o haloperidol para o tratamento. CONCLUSÃO: As escalas de gravidade não são usadas rotineiramente no atendimento de pacientes coréicos; há tendência à prescrição irregular de penicilina entre médicos mais jovens; o haloperidol é a droga mais prescrita entre os entrevistados.

BACKGROUND: Sydenham's chorea is the most common cause of acquired childhood chorea in Brazil. Thus it is relevant to know how physicians treat those patients. OBJECTIVE: To describe the practice patterns of Sydenham's chorea among pediatricians. METHOD: A descriptive study was undertaken using a questionnaire among specialists and emergency pediatricians who work in public hospitals of Rio de Janeiro district. RESULTS:74 percent of the physicians informed not to use any severity scale; 81 percent informed to always prescribe benzatine penicillin; 64 percent informed to begin pharmacological treatment for all patients; and Haloperidol was the most remembered drug among all physicians. CONCLUSION: Physicians do not use routinely severity scales in follow-up of choreic patients; there is a tendency of irregular prescription of benzatine penicillin by younger doctors; and 28.3 percent prescribe only haloperidol.

Hemichorea resulting from single enhancing computed tomography lesion.

A single small enhancing computerized tomographic lesion is a common finding in Indian patients with seizures, particularly focal seizures. A small single enhancing computed tomography lesion also presents with varied non-epileptic manifestations viz. focal neurological deficits, episodic vascular headache, syndrome of increased intracranial pressure, etc. Here we present a case of hemichorea resulting from single enhancing CT lesion. A 12-year-old female presented with acute onset abnormal movements involving right side of body. A clinical diagnosis of right hemichorea was made and patient was subjected to neuroimaging along with other investigations. CT scan showed a single ring-enhancing lesion with perifocal edema in left thalamic area suggestive of neurocysticercosis. Patient was treated with albendazole, steroids and haloperidol therapy. Patient showed marked improvement at follow-up after one month. Repeat CT scan revealed resolution of lesion. This case has been reported because hemichorea in young female is usually caused by post-streptococcal infection and it is quite rare presentation of small ring enhancing lesion.

Cerebral blood flow abnormalities in children with Sydenham's chorea: a SPECT study.

Functional imaging studies in patients with Sydenhams chorea have suggested brain perfusional abnormalities. In this study, we aimed to investigate the cerebral perfusion patterns of the cortical/subcortical structures by using Tc-99m hexamethylpropylenamine oxime single photon emission computed tomography in children with Sydenhams chorea, accompanied with magnetic resonance imaging and cranial Doppler studies. Brain MRI and Doppler studies of the all cases were normal. In the brain SPECT study, six patients were determined to have cerebral perfusion abnormalities. Although six patients responded well to the therapy, two girls who had hypoperfusion in the right frontal region as well as hypo- / hyperperfusion in the basal ganglia did not respond to therapy. While the chorea form of the patients who have cerebral perfusional abnormality was generalized, the clinical picture of the patients with normal cerebral perfusion was in the form of hemichorea.

Paroxysmal kinesigenic dyskinesia manifestation of hyperthyroidism.

Sporadic paroxysmal kinesigenic dyskinesia (PKD) secondary to thyrotoxicosis is an extremely rare entity. A 36-year-old female presented with the features of PKD. Her investigations revealed thyrotoxicosis. Her dyskinesia did not respond to carbamazepine but remitted with the anti-thyroid drug, neomercazole. Perhaps hyperthyroidism-related PKD is a result of a metabolic disturbance of the basal ganglia circuits rather than a permanent and irreversible change.

Sydenham's chorea: clinical and evolutive characteristics

CONTEXT: During the last 12 years we have observed an increase in the frequency of Sydenham's chorea in our country. We have observed that some of our patients have presented recurrence of the chorea despite regular treatment with benzathine penicillin. OBJECTIVE: The aim of our study was to evaluate clinical and evolutive characteristics of Sydenham's chorea in a group of patients followed in our Pediatric Rheumatology Unit. TYPE OF STUDY: Retrospective study. SETTING: Section of Pediatric Rheumatology - Discipline of Allergy, Clinical Immunology and Rheumatology - Department of Pediatrics - UNIFESP - EPM. PARTICIPANTS: Two hundred and ninety patients with rheumatic fever followed between 1986 and 1999. METHODS: We reviewed the records of 290 patients with rheumatic fever followed between 1986 and 1999. All patients were diagnosed according to the revised Jones criteria (1992). We included 86 patients that presented Sydenham's chorea as one of the major criteria (one or more attacks) and evaluated their clinical and evolutive characteristics as well the treatment. RESULTS: Fifty-five patients were girls and 31 were boys. The mean age at onset was 9.7 years and mean follow-up period was 3.6 years. The 86 Sydenham's chorea patients presented 110 attacks of chorea. We observed isolated chorea in 35 percent of the patients, and 25 (29 percent) presented one or more recurrences. We included only 17 of the 25 patients for further analysis, with a total of 22 recurrences of which 14 were attacks of chorea, because it was not possible to precisely detect the interval between attacks in the other patients. The approximate interval between the attacks ranged from 4 to 96 months. In 71 percent of the patients there was no failure in the secondary prophylaxis with benzathine penicillin, which was performed every 3 weeks. CONCLUSION: Despite the regular use of secondary benzathine penicillin prophylaxis, children with rheumatic fever have a high risk of Sydenham's chorea recurrence

Movimentos involuntários anormais como primeira manifestaçäo do lupus eritematoso sistêmico: relato de caso / Involuntary movement disorders as first manifestation of systemic lupus erythematosus: case report

Relatamos o caso de uma paciente, de 36 anos de idade, que desenvolveu quadro de coréia após dois meses do início do uso de anticoncepcional oral, acompanhando-se, posteriormente, de trombocitopenia, úlcera mucosa em cavidade oral, artrite, positividade para os anticorpos antinuclear (FAN), anti-DNA e anti-Sm, preenchendo critérios para lúpus eritematoso sistêmico, segundo o Colégio Americano de Reumatologia. A pesquisa para os anticorpos anticoagulante lúpico e anticardiolipina (IgG e IgM) foi negativa. A paciente foi tratada com prednisona, fenitoína, fenobarbital e clonazepam, obtendo melhora clínica e laboratorial. Discutimos a ocorrência da coréia e outros movimentos anormais como primeira manifestação do lúpus eritematoso sistêmico, sua relação com os anticoncepcionais orais e os anticorpos antifosfolípides.

Role of vitamin E in rheumatic chorea.

Rheumatic chorea is the sole neurologic manifestation of rheumatic fever. It is a debilitating illness lasting for weeks to months. Drugs like diazepam, haloperidol, chlorpromazine take four to six weeks for functional improvement and can cause serious side effects. The authors investigated the role of Vitamin E in reducing rheumatic chorea. A case series of patients of rheumatic chorea were administered Vitamin E in the dose 50 IU daily for fifteen days. The various clinical signs of rheumatic chorea were scored with MAIMS score (Modified Abnormal Involuntary Movement Scale score) which is used for tardive dyskinesia. No other drug for abnormal movements was used. In all the 4 patients who received vitamin E, there was remarkable change by 7th day and almost complete functional improvement by 14th day. Vitamin E is safer than the conventional drugs used for chorea in children. It was found effective in this case series. Its role needs further evaluation by a double-blind randomized controlled trial.

Neuroacantocitose: relato de caso / Neuroacanthocytosis: a case report

Relatamos o caso de um paciente de 45 anos com neuroacantocitose. O paciente apresenta crises parciais complexas como automatismos e crises generalizadas tônico-clônicas, assim com distúrbios do movimento caracterizados por coréia do tronco e membros superiores, e discinesia orofacial. Os exames complementares revelam acantocitose de 11 por cento, eletrencefalograma com foco irritativo no lobo temporal direito, creatino fosfoquinase sérica de 101 U/L e imagem de ressonância magnética com redução volumétrica e hiper-intensidade do sinal no núcleo caudado e putâmen bilateralmente.

Corea inducido por el uso de anticonceptivos orales en dos casos / Chorea induced by oral contraceptives in 2 cases

We report two women using oral contraceptives, aged 17 and 33 years old, who presented with hemichorea. In both patients all other possible causes of chorea were discarded and the disease disappeared when contraceptives were discontinued. Four months later, the 33 years old patient used again oral contraceptives and chorea reappeared. This rare complication of contraceptive use has been previously reported in young and mainly nulliparous women

Coreoatetosis en un paciente con síndrome de inmunodeficiencia adquirida: a propósito de un caso / Chorea in a patient with AIDS: about a case

Se presenta el caso de un paciente con enfermedad avanzada debida al virus de la inmunodeficiencia humana tipo-1 (HIV-1), de 30 años de edad, sexo masculino, que ingresa a Sala 10 del Hospital Francisco J. Muñiz presentando cefalea y movimientos involuntarios en miembro superior izquierdo, con tomografía computada de cerebro (TAC) que reveló múltiples lesiones compatibles con toxoplasmosis cerebral

Sydenham's chorea.

OBJECTIVE: To study the clinical profile in patients with Sydenham's chorea. DESIGN: Prospective. SETTING: Medical college and hospital. SUBJECTS: Sixty cases with Sydenham's chorea, between 1988 and 1994, were studied. Of these 36 were girls; the mean age at presentation was 11.1 yr (range 7-16 yr). RESULTS: Female predominance was apparent only after 9 years of age. There was a high familial incidence for both chorea and rheumatic fever. Generalized chorea was seen in 40 and hemichorea in 20 patients. The chorea lasted from 5 to 40 days; 13 patients had recurrent episodes. Gait disturbances, dysarthria and weakness were common. Six patients had co-existing heart disease, 4 had arthritis and one had subcutaneous nodules. A raised ESR and positive ASO titer were seen in 37 and 20 patients respectively. CONCLUSIONS: There was an increased incidence of Sydenham's chorea after 9 years of age in girls possibly suggesting the influence of female sex hormones. A high incidence of neurologic manifestations were noted. Acute phase reactants were raised in almost half the patients. Sodium valproate, whenever used, was effective in controlling the chorea.

Paroxysmal kinesigenic choreoathetosis

Paroxysmal kinesigenic choreoathetosis (PKC) is characterized by short paroxysms of focal or generalized involuntary movement induced by sudden movements, and is a well-known disease in the neurologic literature, but only 4 cases have been reported in Korea. The purpose of the presentation is to clarify the clinical features of PKC in Korea. We clinically analyzed 20 patients with PKC between 1986 and 1994 at Yongdong Severance Hospital, Yonsei Medical Center, with a minimum of a 1 to 2 year follow-up period. There were 14 men and 6 women. The age at onset of the condition ranged from 8 to 17 years (mean, 13.1 years). Six patients (30%) had a family history of the condition and the mode of inheritance was suggestive of an autosomal recessive pattern. The involuntary movements seemed to be dystonic rather than choreoathetonic upon a mild attack, and the paroxysms were precipitated by sudden movements. The attacks occurred on one or both sides, and were often associated with dysarthria, upward gaze and sensory aura. Consciousness was never lost. Their duration were usually 10 to 30 seconds, and never more than two minutes. All laboratory tests including electroencephalographic and neuroimaging studies showed no abnormality. All patients responded well to diphenylhydantoin. PKC is not rare in Korea and has a benign course.