RESUMO
Nonketotic hyperglycemia may occur as a cause of chorea in patients with chronic decompensated diabetes. Because it is rare and consequently poorly studied, diagnosis and treatment can be delayed. Therefore, our objective was to summarize clinical and radiological features, as well as treatments performed, from previously reported cases to facilitate adequate management in clinical practice. We searched MEDLINE/PubMed, EMBASE, Cochrane, CINAHL, Web of Science, Scopus, and LILACS databases for studies published before April 23, 2021. We included case reports and case series of adults (aged ≥ 18 years) that described hyperglycemic chorea with measurement ofglycated hemoglobin (HbA1c) and cranial magnetic resonance imaging (MRI). Studies were excluded if participants were pregnant women, aged < 18 years, and had no description of chorea and/or physical examination. We found 121 studies that met the inclusion criteria, for a total of 214 cases. The majority of the included studies were published in Asia (67.3%). Most patients were women(65.3%) aged > 65 years (67.3%). Almost all patients had decompensated diabetes upon arrival at the emergency department (97.2%). The most common MRI finding was abnormalities of the basal ganglia (89.2%). There was no difference in patient recovery between treatment with insulin alone and in combination with other medications. Although rare, hyperglycemic chorea is a reversible cause of this syndrome; therefore, hyperglycemia should always be considered in these cases.
Assuntos
Coreia , Discinesias , Hiperglicemia , Humanos , Coreia/etiologia , Coreia/tratamento farmacológico , Coreia/diagnóstico por imagem , Hiperglicemia/complicações , Discinesias/etiologia , Discinesias/tratamento farmacológico , Discinesias/diagnóstico por imagem , Imageamento por Ressonância Magnética , Feminino , Síndrome , Masculino , IdosoRESUMO
Sydenham's chorea is an autoimmune chorea emerging after a group A beta-hemolytic streptococcal (GABHS) infection, i.e. a rheumatic chorea with or without the presence of carditis or arthritis. The disorder, defined by the presence of chorea, is also associated with cognitive and behavioral symptoms, including emotional lability, anxiety, depressive and obsessive-compulsive symptoms. The authors review the pathophysiology, clinical characteristics, and available evidence on therapeutic strategies, the latter including the secondary prevention of GABHS infections, reduction of chorea, and immune modulation. Sydenham's chorea has been regarded as a model for pediatric autoimmune neuropsychiatric disorders, however, the field is marked by conflicting results and controversies. Regarding therapeutics, there are limited high-quality interventional studies and the selection of treatment strategy often relies on the clinician's experience. A serial treatment algorithm is presented based upon the severity of clinical presentation and response to symptomatic pharmacotherapy.
Assuntos
Doenças Autoimunes , Coreia , Transtorno Obsessivo-Compulsivo , Transtornos de Ansiedade , Criança , Coreia/tratamento farmacológico , HumanosRESUMO
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.
Assuntos
Humanos , Masculino , Adolescente , Adulto Jovem , Coreia/diagnóstico , Coreia/tratamento farmacológico , Carbamazepina/uso terapêutico , Eletroencefalografia/métodos , Anticonvulsivantes/uso terapêuticoRESUMO
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.
Assuntos
Humanos , Feminino , Idoso , Coreia/tratamento farmacológico , Coreia/diagnóstico por imagem , Hiperglicinemia não Cetótica/complicações , Discinesias/tratamento farmacológico , Discinesias/diagnóstico por imagem , Complicações do Diabetes , Psicotrópicos/uso terapêutico , Diabetes Mellitus/fisiopatologia , Hipoglicemiantes , Transtornos dos Movimentos/diagnósticoAssuntos
Coreia/diagnóstico por imagem , Idoso de 80 Anos ou mais , Antidiscinéticos/uso terapêutico , Coreia/tratamento farmacológico , Coreia/etiologia , Haloperidol/uso terapêutico , Humanos , Coma Hiperglicêmico Hiperosmolar não Cetótico/complicações , Masculino , Tomografia Computadorizada por Raios XAssuntos
Humanos , Masculino , Idoso de 80 Anos ou mais , Coreia/tratamento farmacológico , Tomografia Computadorizada por Raios X , Coreia/etiologia , Coreia/diagnóstico por imagem , Antidiscinéticos/uso terapêutico , Haloperidol/uso terapêutico , Coma Hiperglicêmico Hiperosmolar não Cetótico/complicaçõesAssuntos
Coreia/diagnóstico , Adulto , Coreia/tratamento farmacológico , Coreia/genética , Coreia/terapia , Feminino , Humanos , Hidroxiureia/uso terapêutico , Janus Quinase 2/genética , Imageamento por Ressonância Magnética , Mutação , Inibidores da Síntese de Ácido Nucleico/uso terapêutico , FlebotomiaRESUMO
OBJECTIVE: This study aimed to report the case of a patient with paroxysmal nonkinesigenic dyskinesias and Fahr syndrome who had a marked response to carbamazepine. METHODS: We present the case of a 57-year-old female patient with episodes of paroxysmal choreoathetoid dyskinesias in the oromandibular region and distal region of upper and lower extremities, with fluctuating dystonic postures in the same distribution; duration was variable ranging from 30 minutes to 3 hours. Laboratory studies were consistent with primary hyperparathyroidism with bilateral brain calcifications. RESULTS: Treatment with low doses of carbamazepine was successful.
Assuntos
Anticonvulsivantes/uso terapêutico , Doenças dos Gânglios da Base/tratamento farmacológico , Calcinose/tratamento farmacológico , Carbamazepina/uso terapêutico , Coreia/tratamento farmacológico , Doenças dos Gânglios da Base/complicações , Doenças dos Gânglios da Base/diagnóstico por imagem , Calcinose/complicações , Calcinose/diagnóstico por imagem , Coreia/complicações , Coreia/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-IdadeRESUMO
Chorea may be secondary to hyperosmolar nonketotic hyperglycemia, but such situation has rarely been described in adolescents, particularly as the initial and single manifestation of type 1 diabetes. We describe a case of a previously healthy 14-year-old girl with sudden onset of choreic movements on her left upper and lower limbs. Brain computed tomography (CT) and magnetic resonance imaging (MRI) revealed an area of hyperdensity/hyperintensity affecting the right striatum. Blood glucose was 349 mg/dL. Despite adequate glucose control, the involuntary movements persisted and haloperidol, later substituted with valproate, was prescribed, with satisfactory but not complete resolution of the chorea. In 2 other occasions, when the patient had an infection and subsequent hyperglycemia, the chorea relapsed. Although not common, hyperglycemia must be considered in the differential diagnosis of acute hemichorea-hemiballismus in children and adolescents, particularly because it is a potentially reversible cause.
Assuntos
Coreia/fisiopatologia , Diabetes Mellitus Tipo 1/fisiopatologia , Discinesias/fisiopatologia , Adolescente , Antibacterianos/efeitos adversos , Antibacterianos/uso terapêutico , Encéfalo/patologia , Coreia/diagnóstico , Coreia/tratamento farmacológico , Coreia/patologia , Diabetes Mellitus Tipo 1/diagnóstico , Diabetes Mellitus Tipo 1/tratamento farmacológico , Diabetes Mellitus Tipo 1/patologia , Diagnóstico Diferencial , Discinesias/diagnóstico , Discinesias/tratamento farmacológico , Discinesias/patologia , Feminino , Infecção Focal/tratamento farmacológico , Infecção Focal/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Doenças da Unha/tratamento farmacológico , Doenças da Unha/fisiopatologia , Recidiva , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Sydenham's chorea is associated with dysfunction of fronto-striatal circuits induced by cross-reactive antibodies to group A ß-hemolytic streptococcus. High susceptibility of extrapyramidal effects of neuroleptics in patients with Sydenham's chorea suggests underlying nigro-striatal dysfunction. OBJECTIVE: To study the presence of parkinsonism in patients with a history of Sydenham's Chorea. METHODS: We used the UFMG Sydenham's Chorea Rating Scale (USCRS) and the Unified Parkinson's Disease Rating Scale (UPDRS) part III, respectively, to determine the presence of chorea and parkinsonian symptoms and signs in 25 adults with a history of previous Sydenham's Chorea currently without chorea or use of anti-choreic drugs. RESULTS: Bradykinesia was found in 64% of subjects. There was a statistically significant correlation between bradykinesia and hemichorea (-0.412; p = 0.036) and bradykinesia and generalized chorea (0.412; p = 0.036). There was no correlation between bradykinesia and use of anti-choreic drugs. CONCLUSIONS: Bradykinesia is common in patients with Sydenham's Chorea in remission. This finding suggests an immune-mediated dysfunction of the nigro-striatal system.