Hemicoreia na infância: revisão da literatura com relato de caso / Hemichorea in childhood: literature review with case report

O diagnóstico etiológico de quadros coréicos é amplo e algumas características da apresentação auxiliam no raciocínio diagnóstico, como o tempo de instalação do quadro (agudo/crônico), a distribuição corporal (focal/generalizada) e sintomas associados. Na infância, a principal causa da forma aguda é a coreia de Sydenham. Descrevemos o caso de uma paciente do sexo feminino de 13 anos que apresentou hemicoreia de instalação aguda relacionada a febre reumática, sendo a manifestação dimidiada atípica nesta condição.

There are numerous causes of chorea, and some characteristics of the presentation of this symptom help in the diagnosis reasoning, such as the onset time of the condition (acute/chronic), body distribution (local/generalized), and associated symptoms. In childhood, the main cause of acute chorea is Sydenham chorea. In childhood, the main cause of the acute form is Sydenham chorea. We report a case of a 13-year-old female patient who presented with acute onset hemichorea, being diagnosed with Sydenham's chorea.

Choreiform movements and basal ganglia calcification as a presentation of Fahr's disease

Basal ganglia calcifications may be a radiological finding in approximately 20% of the general population. When they are associated with neuropsychiatric and motor symptoms in an idiopathic form, they are known as Fahr's disease. They are termed "Fahr's syndrome" when they are secondary to an identifiable and potentially treatable cause. In this report, we present the clinical case of a 69-year-old woman with the onset of subacute chorea, with no other associated symptoms, in whom extensive basal ganglia calcifications were found on neuroimaging, due to which metabolic disorders were subsequently ruled out. The objective is to contribute to the characterization of the potential motor manifestations which would give rise to clinical suspicion. Due to its low incidence and the little information on this condition in the region, we want to encourage documentation of other cases and the process for ruling out other differential diagnoses, in order to obtain more information on its actual epidemiology and signs and symptoms in Colombia. (Acta Med Colomb 2022; 48. DOI:https://doi.org/10.36104/amc.2023.2635).

Dancing Feet Syndrome in Diabetes : Para-ballism and Para-chorea in a Diabetic Patient with Diaphragmatic Myoclonus

While movement disorders in Diabetes have long been recognized,the terminology of diabetic striatopathy is relatively more recent. Herein,we report a rare case of diabetic striatopathy in a 62-year-old woman with uncontrolled Type 2 Diabetes Mellitus who presented with Para-ballismus and Para-chorea along with diaphragmatic Myoclonus, a constellation of rarely reported before simultaneously in a hyperglycemic state.While these movement phenomena are extremely rare, the case also highlights they may persist even after acute control of hyperglycaemia, emphasizing on the need for achieving long term Glycemic control for its management

Systemic Lupus Erythematosus Combined with Chorea: Report of One Case and Literature Review / 中国医学科学院学报

Systemic lupus erythematosus combined with chorea is relatively rare in China,and there are no unified diagnostic criteria or specific ancillary tests.Therefore,it is confirmed by exclusionary clinical diagnosis.To improve the understanding of this disease among rheumatologists,we report the clinical data of a patient with systemic lupus erythematosus combined with chorea admitted to the Department of Rheumatology and Immunology in the First Affiliated Hospital of Jinan University in January 2022.Furthermore,we review the relevant literature in the past 10 years and summarize the clinical features of these cases.

Corea como primera manifestación de lupus eritematoso sistémico sin síndrome de anticuerpos antifosfolipídicos: reporte de caso y revisión de la bibliografía / Chorea as the first clinical manifestation of systemic lupus erythematosus without anti-phospholipid syndrome: case report and literature review

Resumen ANTECEDENTES: La corea gravídica es un padecimiento poco común, caracterizado por movimientos espontáneos irregulares, no predecibles, de duración breve, que cambian de localización sin secuencia definida, y que poseen la particularidad de ser involuntarios. En la actualidad, su incidencia se estima en 1 caso por cada 2275 embarazos. CASO CLÍNICO: Paciente de 30 años, 45 kg, talla 1.65 m, IMC 16.5, tensión arterial 110-78 mmHg, frecuencia cardiaca de 136 lpm, frecuencia respiratoria de 19 rpm, saturación de oxígeno 98%. Inició con movimientos coreicos a las 12 semanas de embarazo y titulación de anticuerpos antinucleares 1:320, anticuerpos anti-ADN de 150 UI/mL, C3 en 126 mg/dL y C4 en 31 mg/dL. Se estableció el diagnóstico de lupus eritematoso sistémico con base en los criterios de EULAR/ACR. A las 32 semanas tuvo amenaza de parto pretérmino y progresión, a pesar de la útero-inhibición, preeclampsia con criterios de severidad y manifestaciones neuropsiquiátricas con exacerbación de los movimientos coreicos. La resonancia magnética no reportó hallazgos patológicos y se descartó el síndrome de anticuerpos antifosfolipídicos secundario. Luego de la finalización del embarazo remitieron los movimientos involuntarios característicos de la corea gravídica. CONCLUSIÓN: El control prenatal en el primer trimestre es fundamental para un de-senlace favorable de las pacientes; el inicio temprano de la inmunoterapia es decisivo en los desenlaces perinatales pues disminuye la morbilidad y mortalidad asociadas. La corea es una manifestación clínica incapacitante, de ahí la importancia de identificar su causa e iniciar el tratamiento oportuno.

Abstract BACKGROUND: Chorea gravidarum is a rare condition characterised by irregular, unpredictable, spontaneous movements of short duration, changing location without defined sequence, and with the peculiarity of being involuntary. Currently, its incidence is estimated at 1 case per 2275 pregnancies. CLINICAL CASE: Patient aged 30 years, 45 kg, height 1.65 m, BMI 16.5, blood pressure 110-78 mmHg, heart rate 136 bpm, respiratory rate 19 rpm, oxygen saturation 98%. She started with choreic movements at 12 weeks of pregnancy and titration of antinuclear antibodies 1:320, anti-DNA antibodies 150 IU/mL, C3 at 126 mg/dL and C4 at 31 mg/dL. The diagnosis of systemic lupus erythematosus was established based on EULAR/ACR criteria. At 32 weeks she had threatened preterm labour and progression, despite utero-inhibition, preeclampsia with severity criteria and neuropsychiatric manifestations with exacerbation of choreic movements. MRI reported no pathological findings and secondary antiphospholipid antibody syndrome was ruled out. After termination of pregnancy the involuntary movements characteristic of chorea gravidarum subsided. CONCLUSION: Prenatal monitoring in the first trimester is essential for a favourable patient outcome; early initiation of immunotherapy is crucial for perinatal outcomes as it decreases associated morbidity and mortality. Chorea is a disabling clinical manifestation, hence the importance of identifying its cause and initiating timely treatment.

Hemidistonía y hemicorea en un paciente pediátrico con déficit del transportador de glucosa de tipo 1 / Hemidystonia and hemichorea in a pediatric patient with glucose transporter type 1 deficiency

El síndrome de deficiencia del transportador de glucosa cerebral de tipo 1 es una enfermedad neurometabólica rara en pediatría. Existe un fenotípico clásico (85 %) y otro no clásico (15 %). Ambos fenotipos se asocian con hipoglucorraquia. Se identifican múltiples mutaciones en el gen SLC2A1. El tratamiento es la terapia cetogénica. Se presenta un varón que comenzó a los cuatro años con hemicorea y hemidistonía medicado con anticonvulsivantes sin respuesta clínica, por lo que consultó nuevamente a los seis años. Con sospecha diagnóstica de síndrome de déficit de glut-1 atípico se realizó punción lumbar; el diagnóstico se confirmó por la presencia de hipoglucorraquia. Inmediatamente después de iniciar la dieta cetogénica, el paciente no presentó más movimientos anormales durante los siguientes 8 años hasta la actualidad, ya cumplidos los 14 años.

Glucose transporter type 1 deficiency syndrome is a rare pediatric neurometabolic disorder. There are two phenotypes: the classical phenotype (85%) and the non-classic (15%). Both phenotypes are associated with hypoglycorrhachia. Multiple mutations are described in the SCL2A1 gene. The treatment is the ketogenic diet. We report a case of a four-year-old male patient who started with hemichorea and hemidystonia and was medicated with drugs for seizures without clinical response, that's why his parents made another pediatric consultation at his six-year-old. With the suggestive clinical findings of glucose transporter type 1 deficiency syndrome the lumbar puncture was made confirming the diagnosis. Immediately after starting the ketogenic diet the patient stopped making abnormal movements up to the moment when he is fourteen years old, eight years after.

Corea gravídica secundaria a síndrome antifosfolipídico: reporte de un caso y revisión de la bibliografía / Chorea gravidarum secondary to Antiphospholipid Syndrome: Report of a case and review of the literature

Resumen ANTECEDENTES: La incidencia de la corea gravídica es de 1 caso por cada 2275 embarazos. El diagnóstico diferencial se establece con distintas enfermedades neurológicas. El tratamiento de la causa subyacente implica la curación espontánea del cuadro clínico. OBJETIVO: Reportar el caso de una paciente que inició con corea gravídica secundaria a síndrome antifosfolipídico y revisar la bibliografía al respecto. CASO CLÍNICO: Paciente de 37 años, con 28 semanas de embarazo, que consultó por alteraciones del comportamiento. Los estudios de laboratorio reportaron anticuerpos antifosfolipídicos y anti-beta 2 glicoproteína elevados en dos determinaciones separadas por 12 semanas, con ANCA y ANA negativos. En la resonancia magnética cerebral no se informaron hallazgos patológicos. Se estableció el diagnóstico de corea gravídica, secundaria a síndrome antifosfolipídico. La reacción al tratamiento con anticoagulantes y corticosteroides fue satisfactoria; en la actualidad, la paciente permanece asintomática y sin tratamiento. CONCLUSIONES: Las pruebas analíticas y de neuroimagen son útiles para establecer el origen de la corea. El tratamiento con anticoagulantes se indica para prevenir eventos trombóticos en pacientes con corea secundaria a síndrome antifosfolipídico. La eficacia de los corticosteroides en el control de los síntomas neurológicos es variable; no obstante, la paciente del caso tuvo una reacción favorable y desaparición completa de los síntomas.

Abstract BACKGROUND: Chorea gravidarum has an estimated incidence of 1:2275 pregnancies. The differential diagnosis of this entity characterized by involuntary spontaneous movements together with behavioral alterations but be made with different neurological diseases. Generally, treatment of the underlying cause involves spontaneous cure of the condition. OBJETIVES: To report the case of a patient who debuted with a chorea gravidarum secondary to antiphospholipid syndrome and to review the literature. CASE DESCRIPTION: 37-years-old patient, 28 weeks pregnant, who consulted for behavioral alterations. Laboratory test showed elevated anti phospholipid and anti-beta 2 glycoprotein antibodies in two determinations 12 weeks apart, with negative ANCA and ANA. There was no pathological finding in brain magnetic resonance. She was diagnosed with corea gravidarum secondary to antiphospholipid syndrome. She was treated effectively with anticoagulants and corticosteroids, and is currently asymptomatic and without treatment. CONCLUSIONS: Analytical and neuroimaging tests are helpful in establishing the etiology of chorea. anticoagulants are indicated to prevent thrombotic events in chorea secondary to antiphospholipid syndrome. Corticosteroids have variable efficacy in the control of neurological symptoms, although in our case complete remission of the symptoms will be overcome.

Estriatopatía diabética como complicación poco frecuente de la diabetes. Reporte de casos

Resumen: La estriatopatía diabética se refiere a un síndrome clínico- radiológico dado por corea-balismo y aumento de señal estriatal en la imagen, ya sea tomografía o resonancia magnética de cráneo. Si bien no existen estudios epidemiológicos, se estima una prevalencia menor a 1/100.000 hab. Clásicamente se ha documentado en mujeres añosas, descendencia asiática, con diabetes tipo 2 y estado hiperglicémico hiperosmolar no cetósico. Pero en los últimos años se ha reportado en gran variedad de pacientes diabético. Se caracteriza por ser reversible, con el tratamiento específico de la hiperglicemia. Algunos pacientes requieren de uso de otras drogas para tratamiento sintomático mientras se logra la euglicemia. Se han reportado casos de persistencia de movimientos y atrofia del caudado, cuando no se realiza un diagnóstico y tratamiento adecuados y oportunos. La recurrencia llega a ser del 4 al 20%, por lo que es importante establecer un seguimiento cercano de estos pacientes. Presentamos dos casos de estriatopatía diabética, una mujer de 72 años y un hombre de 73 años, ambos se presentaron con hemicorea aguda, pero con diferente evolución y tratamiento.

Abstract: Diabetic striatopathy refers to a clinical-radiological syndrome caused by chorea-ballismus and increased striatal signal in the image, either tomography or magnetic resonance imaging of the skull. Although there are no epidemiological studies, a prevalence of less than 1/100,000 inhabitants is estimated. It has classically been documented in elderly women, Asian descent, with type 2 diabetes and a non-ketotic hyperosmolar hyperglycemic state. But in recent years it has been reported in a wide variety of diabetic patients. It is characterized by being reversible, with specific treatment of hyperglycemia. Some patients require the use of other drugs for symptomatic treatment while euglycemia is achieved. Cases of persistence of movements and atrophy of the caudate have been reported, when an adequate and timely diagnosis and treatment are not performed. Recurrence reaches 4 to 20%, so it is important to establish a close follow-up of these patients. We present two cases of diabetic striatopathy, a 72-year-old woman and a 73-year-old man, both presented with acute hemichorea, but with different evolution and treatment.

Resumo: A estriatopatia diabética refere-se a uma síndrome clínico-radiológica causada por coreia-balismo e aumento do sinal estriatal na imagem, seja na tomografia ou na ressonância magnética do crânio. Embora não existam estudos epidemiológicos, estima-se uma prevalência inferior a 1/100.000 habitantes. Tem sido documentada classicamente em mulheres idosas, descendentes de asiáticos, com diabetes tipo 2 e estado hiperglicêmico hiperosmolar não cetótico. Mas nos últimos anos tem sido relatado em uma ampla variedade de pacientes diabéticos. Caracteriza-se por ser reversível, com tratamento específico da hiperglicemia. Alguns pacientes requerem o uso de outras drogas para tratamento sintomático enquanto a euglicemia é alcançada. Casos de persistência dos movimentos e atrofia do caudado têm sido relatados, quando um diagnóstico e tratamento adequado e oportuno não são realizados. A recorrência atinge 4 a 20%, por isso é importante estabelecer um acompanhamento próximo desses pacientes. Apresentamos dois casos de estriatopatia diabética, uma mulher de 72 anos e um homem de 73 anos, ambos com hemicoréia aguda, mas com evolução e tratamento diferentes.

Balismo e Coreia em idoso propiciado por estado hiperglicêmico não cetótico: Relato de Caso / Ballism and chorea in older man propitiated by a non-ketotic hyperglycemic state: Case Report

O diabetes mellitus é uma doença endócrino-metabólica de grande prevalência mundial que pode culminar, quando descompensada, com diversas complicações, entre elas alterações neurológicas. Este relato de caso tem como objetivo descrever a presença de balismo e coreia em membros superiores bilateralmente propiciados pelo estado hiperglicêmico não cetótico em paciente com diabetes mellitus sem tratamento. Devido à agilidade em procurar por atendimento médico assim que se iniciaram os sintomas, e o correto manejo intra-hospitalar, nenhuma alteração foi visualizada em exame de imagem. No entanto, foram descartadas quaisquer outras causas que justificassem o quadro. Após o controle glicêmico adequado, houve melhora clínica progressiva e desaparecimento de movimentos involuntários.

Diabetes mellitus is an endocrine metabolic disease of great worldwide prevalence that can culminate, when decompensated, with several complications, including neurological changes. This case report aims to describe the presence of ballism and chorea in the upper limbs bilaterally caused by the non-ketotic hyperglycemic state in a patient with untreated diabetes mellitus. Due to the agility in seeking medical care as soon as the symptoms started, and the correct intra-hospital management, no changes were seen in the imaging scan. However, any other causes that justified the situation were ruled out. After adequate glycemic control, there was progressive clinical improvement and disappearance of involuntary movements.

Síndrome antifosfolípido no trombótico: manifestaciones inflamatorias hematológicas y neurológicas / Non thrombotic antiphospholipid syndrome: neurologic and hematologic inflammatory manifestations

RESUMEN El síndrome antifosfolípido (SAF) es una entidad caracterizada por la presencia de anticuerpos antifosfolípidos y características clínicas como la pérdida fetal recurrente y la trombosis venosa o arterial. El diagnóstico del SAF se realiza extrapolando criterios de clasificación que incluyen criterios clínicos y de laboratorio, con manifestaciones trombóticas en su gran mayoría. Sin embargo, hay una gran variedad de manifestaciones no trombóticas de la enfermedad no incluidas en los criterios de clasificación. Dichas manifestaciones "extra-criterio" son aquellas que, debido a su frecuencia y a la relevancia que tienen para la toma de decisiones, son hallazgos de valor. Entre ellas, destacan características inflamatorias en dominios como el hematológico, de las cuales la principal es la trombocitopenia. De las diferentes manifestaciones neurológicas, debido a su frecuencia o gravedad, resaltan la migraña, la epilepsia, la demencia y otras graves como la corea y la mielitis. En la presente revisión se reseña la información disponible sobre las manifestaciones de dos dominios frecuentes y relevantes "extra-criterio" del síndrome antifosfolípido: las hematológicas y las neurológicas.

SUMMARY Antiphospholipid syndrome (APS) is an entity defined for the presence of antiphospholipid antibodies and events such as recurrent fetal loss and arterial or venous thrombosis. APS diagnosis is made through classification criteria, including laboratory criteria and clinical manifestations (most of them being thrombotic manifestations). Nonetheless, there is a wide variety of non-thrombotic manifestations of APS, which are not included in classification criteria. These "noncriteria" manifestations are important for decision making, based on their frequency or clinical relevance. Among them, some domains stand out: the hematologic domain, being thrombocytopenia the main manifestation; and the neurological domain with different manifestations such as migraine, epilepsy, dementia and severe manifestations, such as chorea and myelitis. Therefore, in the present article, information concerning two of the most common "non-criteria" domains (neurological and hematological) of APS is reviewed.

Hemibalismo en el síndrome hiperglucémico hiperosmolar no cetósico

RESUMEN Se trata de una paciente del sexo femenino de 71 años de edad con diagnóstico de diabetes mellitus tipo 2 en tratamiento irregular con antidiabéticos orales, que acude al Servicio de urgencias por movimientos anormales del hemicuerpo derecho, interpretados como hemibalismo con componentes coreicos, de 3 días de evolución. En el laboratorio se detecta hiperglicemia no cetósica y en la tomografía de cráneo se evidencia hiperdensidad en ganglios caudado y lenticular lado izquierdo. Tras el adecuado control de la glicemia y dosis bajas de haloperidol la paciente evolucionó favorablemente, con disminución de los movimientos anormales.

ABSTRACT This is a 71-year-old female patient with a diagnosis of type 2 diabetes mellitus undergoing irregular treatment with oral antidiabetics, who came to the emergency department due to abnormal movements of the right hemibody, interpreted as hemiballism with choreic components, with 3 days. of evolution. Nonketotic hyperglycemia is detected in the laboratory and hyperdensity in the caudate and lenticular ganglia on the left side is evidenced on the skull tomography. After adequate glycemic control and low doses of haloperidol, the patient evolved favorably, with a decrease in abnormal movements.

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).

Hemichorea associated with ketotic hyperglycemia: two cases report and literature review / 中华神经科杂志

Hemichorea associated with ketotic hyperglycemia is a lateral chorea caused by hyperglycemic ketosis, which is very rare clinically, and has not been reported at home and abroad in elderly patients with type 2 diabetes. Two cases with hemichorea associated with ketotic hyperglycemia were reported. One case was an 86-year-old female with primarily diagnosed diabetes and unilateral limb involvement. The other case was an 85-year-old man with chronic poor glycemic control and bilateral limb involvement. In order to improve the understanding of this disease, the clinical and imaging manifestations of this disease were analyzed in combination with relevant literature.

Subthalamic Nucleus: Neuroanatomical Review

Discovered in 1865 by Jules Bernard Luys, the subthalamic nucleus is a set of small nuclei located in the diencephalon, inferior to the thalamus and superior to the substantia nigra, that can be visualized in a posterior coronal section. Histologically, it consists of neurons compactly distributed and filled with a large number of blood vessels and sparse myelinated fibers. This review presents an analysis of this anatomical region, considering what is most recent in the literature. Subthalamic neurons are excitatory and use glutamate as the neurotransmitter. In healthy individuals, these neurons are inhibited by nerve cells located in the side globus pallidus. However, if the fibers that make up the afferent circuit are damaged, the neurons become highly excitable, thus causing motor disturbances that can be classified as hyperkinetic, for example ballism and chorea, or hypokinetic, for example Parkinson disease (PD). The advent of deep brain stimulation has given the subthalamic nucleus great visibility. Studies reveal that the stimulation of this nucleus improves themotor symptoms of PD.

Chorea as the first presenting sign of rheumatic fever in a 12-year-old female child

Rheumatic chorea (RC*) is a movement disorder seen in young children and adolescents with a recent history of incompletely treated group A beta-hemolytic streptococcal (GABHS) pharyngitis. Although, it rarely presents as the first manifestation of the rheumatic fever, physicians should be aware of the disease, so that early diagnosis and prompt treatment may lead to elimination of the pathogen and prevent further disease progression. We present a case of a 12-year-old female child who presented with only RC as the first clinical sign.

Hemicorea de larga duración asociada a trastorno metabólico

RESUMEN La corea asociada a la hiperglucemia representa un síndrome neurológico poco frecuente, mucho más común entre las mujeres asiáticas mayores. La fisiopatología sigue siendo controvertida y poco comprendida. Sin embargo, se han propuesto teorías como el aumento de la sensibilidad del receptor de dopamina, el infarto del núcleo estriado, el aumento del metabolismo del ácido gamma-aminobutírico y una alteración de la barrera hematoencefálica. Informamos que una mujer de 75 años de edad, de raza indígena, fue ingresada en el Departamento de emergencias debido a movimientos aleatorios, involuntarios y continuos severos en su brazo y pierna derecha que comenzaron 3 días antes, acompañados de síntomas osmóticos (polidipsia y poliuria). El nivel de glucosa capilar al ingreso fue 470,5 mg/dL, sin rastros de cetona en sangre u orina, mientras que el valor de hemoglobina glicosilada fue 15,4%. La resonancia magnética nuclear reportó hiperintensidad en T1 del núcleo lenticular izquierdo predominantemente a nivel del putamen sin otras alteraciones. Describimos los aspectos de la larga duración de los síntomas a pesar del tratamiento y el control metabólico adecuado.

ABSTRACT Chorea associated with hyperglycemia represents a rare neurological syndrome, much more common among older Asian women. The pathophysiology remains controversial and poorly understood. However, theories have been proposed such as increased dopamine receptor sensitivity, striated nucleus infarction, increased gamma-aminobutyric acid metabolism and an alteration of the blood-brain barrier. We report that a 75-year-old woman, of indigenous race, was admitted to the Emergency Department due to severe random, involuntary and continuous movements in her right arm and leg that began 3 days earlier, accompanied by osmotic symptoms (polydipsia and polyuria). The level of capillary glucose at admission was 470.5 mg/dL, without traces of ketone in blood or urine, while the value of glycosylated hemoglobin was 15.4%. Nuclear magnetic resonance imaging reported hyperintensity in T1 of the left lenticular nucleus predominantly at the putamen level without other alterations. We describe aspects of the long duration of symptoms despite adequate treatment and metabolic control.

Chorea gravidarum: Case report and review of the literature / Corea gravídica: reporte de caso y revisión de la literatura

RESUMEN Objetivos: presentar un caso de corea gravídica y hacer una revisión sistemática de la literatura pu- blicada sobre el tratamiento y pronóstico materno- fetal de la entidad. Materiales y métodos: se presenta el caso de una paciente de 16 años, primigesta, que ingresa a un hospital público de tercer nivel de complejidad, con 8 semanas de gestación, con clínica de 3 días de evo- lución caracterizada por movimientos involuntarios de la cabeza y las extremidades, con hiperreflexia en miembro inferior derecho. Antecedente de Corea de Sydenham. Se da tratamiento con antisicóticos y benzodiacepinas para la sintomatología y etiológico con penicilina benzatínica y control del cuadro a las 14 semanas. Suspendió el tratamiento a las 35 semanas. Parto normal a las 39 semanas. Se realizó una búsqueda en la base de datos de Medline vía PubMed, UptoDate, Medscape y Google Académico empleando los términos: "Pregnancy" and "Chorea Gravidarum". La búsqueda se limitó a reportes y series de casos o artículos de revisión, desde el año 2000 al 2019. Resultados: se encontraron siete reportes de casos y una revisión de tema. El tratamiento se realizó con haloperidol benzodiacepinas y clorpromacina en cuatro de los siete casos. En uno de los dos casos con antecedentes de corea de Sydenham se utilizó penicilina. El pronóstico materno-fetal fue bueno en seis de siete casos, en un caso hubo restricción de crecimiento intrauterino. Conclusión: el tratamiento de la corea gestacional principalmente es expectante y su finalidad es disminuir la sintomatología, el pronóstico materno- fetal es bueno.

ABSTRACT Objectives: To present a case of chorea gravidarum and conduct a review of the published literature on the treatment for this condition, and on maternal and fetal prognosis. Materials and methods: Case presentation of a 16-year-old primiparous patient admitted to a Level III public hospital at 8 weeks of gestation complaining of involuntary head and limb movements and right lower limb hyperreflexia lasting three days. The patient had a history of Sydenham chorea. Treatment with antipsychotics and benzodiazepines was given to manage symptoms, and with benzathine penicillin to address the etiology, achieving control at 14 weeks. Treatment was discontinued at 35 weeks and the patient went on to have normal delivery at 39 weeks. A search was conducted in the Medline via PubMed, UptoDate, Medscape and Google Scholar databases using the terms "Pregnancy and Chorea Gravidarum". The search was limited to case reports and case series or review articles published between 2000 al 2019. Results: Seven case reports and one review of the topic were found. In 4 of the 7 cases, treatment was based on haloperidol, benzodiazepines and chlorpromazine. Penicillin was used in one of two cases with a history of Sydenham chorea. Maternal and fetal prognosis was good in 6 of 7 cases, there was 1 case of intrauterine growth restriction. Conclusion: Treatment of gestation chorea is primarily expectant and the goal is to reduce symptoms. Maternal and fetal prognosis is good.

Discinesia paroxística não cinesiogênica: relato de caso / Paroxysmal non-kinesigenic dyskinesia: case report

Modelo do estudo: Relato de caso. Importância do problema e comentários: A discinesia paroxística não cinesiogênica é um tipo de discinesia paroxística. É caracterizada por movimentos involuntários unilaterais ou bilaterais, do tipo coreico, distônico, balístico ou misto. É uma desordem rara e o diagnóstico precoce é crucial para seu tratamento e melhoria na qualidade de vida do indivíduo. O presente estudo relata um caso de Discinesia Paroxística Não Cinesiogênica e seus achados clínicos, além de apresentar breve revisão da literatura (AU)

Study type: Case report. Relevance and comments: Paroxysmal non-kinesigenic dyskinesia is a type of paroxysmal dyskinesia. It is characterized by involuntary unilateral or bilateral movements, of the choreic, dystonic, ballistic or mixed type. It is a rare disorder and the early diagnosis is crucial for the treatment and improvement of the individual's quality of life. The present report illustrates a case of paroxysmal non-kinesigenic dyskinesia and clinical findings, as well as a brief review of the literature (AU)

Frecuencia De Casos Juveniles Con Enfermedad De Huntington En Población Mexicana / Frequency Of Juvenile Huntington´S Disease In A Mexican Population

Resumen Objetivo: El propósito de este estudio es determinar la prevalencia de casos juveniles en una muestra de sujetos mexicanos con enfermedad de Huntington (EH) confirmada molecularmente. Métodos: Se incluyeron pacientes con inicio clínico antes de los 21 años de edad que acudieron a la clínica de trastornos del movimiento del Instituto Nacional de Neurología y Neurocirugía. La información demográfica y clínica se obtuvo de la revisión de expedientes. Resultados: Se revisaron un total de 198 casos de pacientes con diagnóstico de EH, de los cuales el 6.5% (n=13) correspondió a formas juveniles. La media de edad para el inicio de síntomas fue de 17.8 ± 3.9 años. La puntuación media del UHDRS-motor fue de 46.2 ± 17.4 puntos. El síntoma motor predominante fue corea en el 53.8% de los casos. El 84.6% de los afectados presentó al menos una alteración neuropsiquiátrica. Conclusión: Se detectó que el fenotipo motor de estos pacientes fue dominantemente corea, contrario a lo reportado mundialmente hasta ahora, es decir, nuestro grupo presentó clínica motora atípica de EHJ.

Abstract Objective: The purpose of this study is to know the prevalence of juvenile cases in a sample of mexican subjects with confirmed Huntington Disease (HD). Methods: Patients with clinical debut before 21 years of age were included who attended at movement disorders clinic of the National Institute of Neurology and Neurosurgery. The demographic and clinical information was obtained from the review of files. Results: A total of 198 cases of patients diagnosed with HD were reviewed, of which 6.5% (n = .13) corresponded to juvenile forms. The mean age for the onset of symptoms was 17.8 ± 3.9 years. The mean score of the UHDRS-motor was 46.2 ± 17.4 points. The predominant motor symptom was chorea in (53.8%) of the cases. 84.6% of those affected presented at least one neuropsychiatric disorder. Conclusion: It was detected that the dominant motor phenotype of these patients was chorea compared to the world reports until now, accordingly to that, our group of juvenile HD shows atypical motor clinical.