Acute chorea: case series from the emergency room of a Brazilian tertiary-level center / Coreia aguda: série de casos de pronto atendimento de um centro terciário brasileiro

ABSTRACT Background: Chorea is a movement disorder characterized by random, brief and migratory involuntary muscle contractions. It is defined as acute when present within hours to days. Three main causes for this scenario have emerged as most likely: vascular, toxic-metabolic and inflammatory. Objectives: To identify the prevalence of the main etiologies and major clinical findings of acute chorea in the emergency room of a tertiary-level referral center; and to suggest an approach for guiding the diagnostic workup and clinical management. Methods: We retrospectively reviewed the clinical aspects and neuroimaging data of 10 patients presenting with acute chorea at the neurological emergency room of our hospital from 2015 to 2019. Results: Stroke was the most common etiology (50% of the cases). All of them were ischemic. It was noteworthy that only one case demonstrated the classical ischemic topographic lesion at the contralateral subthalamic nuclei. Regarding nonvascular etiologies, nonketotic hyperglycemia was the major cause, followed by drug-related chorea. One patient showed inflammatory etiology, which was probably Sydenham chorea reactivation. Conclusion: Acute chorea is an uncommon and challenging problem at the emergency room, often associated with potentially treatable causes. We suggest that use of the acronym DANCE (Diagnosis of chorea, Acute stroke protocol, Normal glucose levels, Check neuroimaging, Exposure to drugs) could form a potential initial approach in the evaluation, in order to emphasize causes that require prompt proper management (e.g. thrombolysis).

RESUMO Introdução: Coreia é um distúrbio do movimento caracterizado por contrações musculares caóticas, migratórias, aleatórias e involuntárias. Usualmente, define-se como coreia aguda quando presente dentro de horas a dias. Neste cenário, três causas emergem como as mais comuns: vascular, tóxico-metabólica e inflamatória. Objetivos: O objetivo deste estudo foi identificar a prevalência das principais etiologias e os principais achados clínicos de coreia aguda na sala de emergência de um centro de referência terciário, a fim de sugerir uma abordagem para orientar a investigação diagnóstica e o manejo na emergência. Métodos: Revisamos retrospectivamente os dados clínicos e de neuroimagem, de 2015 a 2019, de 10 pacientes com coreia aguda na sala de emergência neurológica de um hospital terciário. Resultados: A etiologia mais comum foi o acidente vascular cerebral (AVC) (50% dos casos). Todos os AVCs foram isquêmicos e apenas um se apresentou como isquemia clássica do núcleo subtalâmico contralateral. Em relação às causas não vasculares, a hiperglicemia não cetótica demonstrou ser a principal, seguida pela coreia relacionada a medicamentos. Um paciente apresentou etiologia inflamatória, por provável reativação da coreia de Sydenham. Conclusão: A coreia aguda é um problema incomum e desafiador na sala de emergência, muitas vezes associado a causas potencialmente tratáveis. Nós sugerimos o acrônimo DANCE (Diagnosis of chorea, Acute stroke protocol, Normal glucose levels, Check neuroimaging, Exposure to drugs) para auxiliar na abordagem como primeiro passo na sala de emergência, a fim de enfatizar causas que requerem tratamento imediato e adequado (por exemplo, trombólise).

Hemicorea como presentación clínica de hemorragia mesencefálica / Hemichorea as clinical presentation of hemorrhagic ictus

Paciente masculino, hipertenso con inicio súbito de trastornos de movimientos hipercineticos, involuntarios, continuos e irregulares, de la cabeza y hemicuerpo izquierdo. Cursó con afectación de diversos segmentos corporales, principalmente la porción distal de las extremidades, que disminuyen significativamente durante el sueño y se exacerban con emociones, stress o alta concentración. Este trastorno puede tener múltiples causas: genéticas, degenerativas, cerebrovasculares, metabólicas, endocrinas, tóxicas y medicamentosas; en este caso no hay historia de consumo de tóxicos o medicamentos, ni tampoco, datos de causas metabólicas como hiper o hipoglicemia, hipercalcemia significativa o hipernatremia o hiponatremia, entre otras. Se descartó la etiología degenerativa por el curso agudo de la enfermedad. Las imágenes tomográficas fueron compatibles con hemorragia mesencefálica, lo cual motiva esta presentación por lo infrecuente de los trastornos del movimiento involuntario tras un ictus, aunque está descrita en la bibliografía(AU)

We describe a male patient, with hypertension, who consulted for sudden onset of continuous hemichoreic movements of the head and left side of the body. These movements diminished during the sleep and exacerbated with emotions, stress or alertness. Usually this clinical presentation can be due to genetic, degenerative, cerebrovascular events, metabolic causes, medications, toxic substances and several electrolytic disturbances. None of the later were found in this patient, and degenerative origin was not an option due to the sudden onset of his symptoms. The brain cat-scan showed a mesencephalic hemorrhage, which is infrequent with this clinical presentation(AU)

Coreia: uma manifestação rara da arterite de Takayasu / Chorea: A rare manifestation of Takayasu's arteritis

RESUMORelatamos um caso de uma menina com coreia recorrente e diagnóstico de arterite de Takayasu. Esta manifestação clínica foi relatada em apenas um paciente com tal vasculite na faixa etária pediátrica.

ABSTRACTThe case of a girl with recurring chorea and a Takayasu's arteritis diagnosis is reported. This clinical manifestation has been reported in only one patient with this vasculitis in the pediatric group.

Hemicorea vascular: caso clínico y revisión / Vascular hemichorea: case report and review

La corea es una rara complicación de las lesiones vasculares cerebrales isquémicas o hemorrágicas y aunque las manifestaciones clínicas son variables, habitualmente se afecta un único hemicuerpo, en cuyos casos la lesión se encuentra en el hemisferio cerebral contralateral. A pesar de que la remisión espontánea es la norma, el tratamiento sintomático en ocasiones es requerido. El caso es un paciente masculino de 58 años de edad, con hipertensión arterial sin tratamiento, obesidad tipo II, consumo de 6 cajetillas de cigarrillos al año e ingesta moderada de alcohol. El paciente inició enfermedad actual tres días previos a acudir al servicio de emergencias, cuando presentó movimientos involuntarios incesantes en miembro superior izquierdo y pie ipsilateral, persistiendo durante el día y observables durante la noche, sin limitación del movimiento voluntario. El examen físico no presentó alteraciones adicionales a los movimientos coreiformes; exámenes pertinentes de laboratorio no alterados. Se realizó resonancia magnética de cerebro revelando hiperintensidad en región talámica posterolateral derecha consistente con enfermedad cerebrovascular isquémica. Se indicó tratamiento sintomático y para patologías de base. El caso es importante por la baja prevalencia y escasos estudios publicados actualizados inherentes al origen vascular de la hemicorea para diagnóstico, pronóstico y tratamiento.

Chorea rarely complicates ischemic or hemorrhagic cerebral vascular lesions. Clinical symptoms usually involve one side of the body while the injury is situated on the contralateral cerebral hemisphere. Spontaneous remission is the norm, but sometimes symptomatic treatment is required. A 58-year-old male patient who suffers from untreated high blood pressure, type II obesity, smokes 6 packs of cigarettes per year and has a moderate intake of alcohol is presented. The patient’s recent history began three days before he appeared at the Emergency Department. His symptoms were ceaseless, involuntary movements in his left arm and foot during day and night with no restriction of voluntary movements. Physical examination and laboratory tests revealed no other findings. Magnetic resonance imaging of the brain showed hyperintensity in the right posterolateral thalamic region consistent with ischemic cerebrovascular disease. Symptomatic therapy was indicated and his underlying conditions were addressed. The importance of this case lies on the low prevalence as well as the scarcity of publications regarding vascular causes of hemichorea, including diagnosis, therapy and prognosis.

Corea y eritrocitosis de altura: reporte de caso / Chorea and high altitude erythrocytosis: a case report

La corea es un trastorno del movimiento que tiene a la eritrocitosis como causa poco frecuente. La eritrocitosis o poliglobulia es el aumento de la masa eritrocitaria absoluta y su causa primaria más frecuente es la policitemia vera. Entre las causas secundarias adquiridas figura la eritrocitosis por hipoxia central, como la que ocurre en los habitantes de grandes alturas. La corea es una manifestación neurológica rara de la eritrocitosis, se presenta en 0,5 a 2% de estos pacientes. Se reporta el caso de un paciente de sexo masculino de 71 años de edad que presentó corea generalizada, de inicio súbito, a predominio orolinguofacial y de extremidades inferiores, secundario a eritrocitosis adquirida, que mejoró tras la disminución del hematocrito mediante sesiones sucesivas de flebotomías.

Chorea is a movement disorder which is rarely caused by erythrocytosis. Erithrocytosis or polycythemia is the augmentation of the absolute erythrocytic mass and its most common primary cause is polycythemia vera. Some of the secondary causes are erythrocytosis by central hipoxia occurring in people who live in the highlands. Chore is a rare neurological manifestation of erythrocytosis occurring in 0.5 to 2% of these patients. There was a report of a 71 year-old male patient with generalized chorea of sudden onset predominant in mouth, tongue and face and lower limbs, secondary to acquired erythrocytosis, which improved after the number of erythrocytes decreased through successive sessions of phlebotomy.

An Interesting Case of Thyrotoxicosis with Choreoathetosis - A Rare Association.

Thyrotoxicosis may present with spectrum of movement disorders. Though tremor is most frequently associated, chorea has also been reported rarely. A rare case of thyrotoxicosis in a young female presenting with choreoathetotic movement is reported here. The choreoathetotic movement in this case was attributed to thyrotoxicosis based on clinical and biochemical criteria after exclusion of other causes.

Relapse of herpes simplex encephalitis presenting as choreoathetosis.

We report a case of herpes simplex virus (HSV) encephalitis (HSE) in an 11-year-old boy who recovered with acyclovir therapy but developed relapse after 2 weeks. Choreoathetosis was the presenting feature of relapse. Response to antiinflammatory treatment was excellent. To the best of our knowledge, this is the first case of HSE relapse presenting with choreoathetosis reported from India. We describe the patient and review the literature on HSE and HSE relapse.

Coréia de Syndenham: uma dança entre o Streptococcus, o sistema imune e os núcleos da base / Syndenham's chorea: a dance involving the Streptococcus the immune system and the basal ganglia

A coréia de Syndenham (CS) é a manifestação da febre reumática e consiste em movimentos involuntários anormais que se assemelham a uma "dança". Em sua etiopatogênese, várias evidências apontam para mecanismos auto-imunes desencadeados por uma infecção na orofaringe pelo Streptococcus ß-hemolítico do grupo A. Esses processos imunes interferem no funcionamento dos núcleos da base, gerando desde o quadro motor de coréia até os distúrbios psiquiátricos associados à CS.

Severe childhood autosomal recessive muscular dystrophy, mental subnormality and chorea.

Severe childhood autosomal recessive muscular dystrophy (SCARMD) is characterized by a severe Duchene muscular dystrophy like phenotype. Most such cases represent alpha or gamma sarcoglycanopathies. Mental subnormality is very uncommon and other central nervous system deficits have not been documented in patients with SCARMD. We report a brother and sister with the SCARMD phenotype, who additionally had static mental subnormality and choreiform movements. Work-up for sarcolgycan genes, dystrophin gene and known causes of mental retardation and chorea was normal.

Síndromes neuropsiquiátricas pós-estreptocócicas / Post-streptococcal neuropsychiatric syndromes

Nesta revisão narrativa, o nosso objetivo foi descrever as síndromes neuropsiquiátricas pós estreptocócicas e discuti-las à luz das evidências científicas atuais sobre os possíveis mecanismos patogenéticos envolvidos. Nos últimos anos, uma série de distúrbios do movimento, como tiques, distonia, parkinsonismo, e transtornos psiquiátricos, como o transtorno obsessivo-compulsivo (TOC) e o transtorno da hiperatividade com déficit de atenção (THDA), vem sendo considerada parte do espectro das manifestações pós-estreptocócicas. O termo PANDAS (acrônimo do inglês: pediatric autoimmune neuropsychiatric disorder associated with streptococcus) foi inclusive cunhado para descrever um subgrupo de pacientes com TOC e tiques que inibe flutuação clínica dos sintomas associada a infecção estreptocócica. Entretanto a análise crítica das evidências clinicolaboratoriais não apóia esse espectro ampliado das manifestações pós-estreptocócicas. Apenas na coréia de Sydenham há evidências consistentes de patogênese mediada por processo auto-imune pós-estreptocócico.

In this narrative review, our objective was to describe the post-streptococcal neuropsychiatric syndromes and to discuss the possible pathogenetic mechanisms involved in their clinical expression. Recently, several movement disorders, such as tics, dystonia, and parkinsonism, and psychiatric disorders like obsessive-compulsive disorders (OCD) and attention deficit hyperativity disorder (ADHD), are being considered part of a putative spectrum of post-streptococcal infection disorder. The term PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcus) has been introduced to describe a subset of patients with these diagnoses in which onset of symptoms or symptom exacerbations are related to streptococcal infection. However the critical analysis of clinical and laboratory data does not support the hypothesis of an extended spectrum of post-streptococcal neuropsychiatric disorders. Only for Sydenham chorea there is consistent evidence for a post-streptococcal autoimmune mediated pathogenesis.

Chorea and microphonia as presenting feature of SLE.

Systemic lupus erythematosus (SLE) is a multisystem immunologic disease. Renal and neurological manifestations are frequently seen in childhood SLE. Out of the neurological features movement disorders are uncommon. A case of SLE is presented here with unusual neurological manifestation i.e. chorea associated with microphonia.

Chorea Following Acute Carbon Monoxide Poisoning

The clinical cases of 6 patients suffering with chorea after acute carbon monoxide (CO) poisoning were reviewed. There were 2 men and 4 women, and the age at onset ranged from 11 to 60 (mean 33.0) years. All the patients except one were associated with mild delayed CO encephalopathy. The latency period between CO poisoning and the onset of chorea was 10 to 30 (mean 21.7) days. The duration of chorea after CO poisoning was 14 to 90 (mean 39.8) days. The brain CT findings were bilateral low- density lesions in the basal ganglia and/or in the white matter of the cerebral cortex, and there was no correlation between the lesion sites on the imagings and the development of chorea. Neuroleptic agents alleviated the chorea and the patients did not relapse after neuroleptic agents were halted.

Chorea due to nonketotic hyperglycemia.

A 62 year old diabetic and hypertensive male presented with sudden onset generalized chorea. Investigations revealed uncontrolled diabetes with absent ketones and normal serum osmolality. Achievement of euglycemia with insulin therapy abolished the involuntary movements completely within a day. The direct effect of hyperglycemia causing striatal neuronal dysfunction could be the pathogenesis of the chorea in our patient.