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Hemichorea-hemiballismus (HCHB) is a rare manifestation of hyperglycaemic hyperosmolar state caused by contralateral lesion in basal ganglia. A 74-year-old, known diabetic and hypertensive woman presented with one-week history of high-grade fever and loss of consciousness associated with involuntary movement of the right upper and lower limbs for 10 hours prior to presentation. Physical examination revealed pyrexia, tachycardia and altered sensorium. Blood glucose was 53.8 mmol/l, hemoglobin A1c (Hb A1c) 9.9% and brain computed tomography (CT) scan showed cerebral atrophy with bilateral basal ganglia hyperdensities. Escherichia coli was cultured from the urine. She did well on treatment with soluble insulin, rehydration and intravenous ceftriaxone. HCHB is a rare complication seen in patients with poorly controlled diabetes mellitus. This report highlights the reversibility of the disease with prompt diagnosis and appropriate insulin treatment. HCHB should be distinguished from other intracranial pathologies.
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@#Hemichorea after a striatal lesion of the ipsilateral side of the cerebral hemisphere is a very rare condition. A 72-year-old female presented with right-sided bradykinesia and left-sided hemichorea after a left striatal hemorrhage. In a shape analysis of the brain conducted via MRI, her damaged left basal ganglia was severely contracted whereas her right dorsolateral putamen surface was deformed and hypertrophied. Excessive compensation of the contralateral hemisphere may be a possible mechanism of ipsilateral hemichorea in this case.
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@#INTRODUCTION: Nonketotic hyperglycemia among type 2 diabetic patients have recently been documented to cause the rare movement disorder called Hemichorea-hemiballism syndrome which is a hyperkinetic movement disorder presenting as a continuous, non-patterned, involuntary movements caused by a basal ganglia dysfunction. METHODS: A 76-year-old male with a known history of hypertension and no history of stroke and diabetes presented with a 10-day history of increasingly persistent involuntary movements of the right extremities. On admission, the patient was conscious with stable vital signs and unremarkable neurologic findings except for the involuntary flailing movements of the right extremities. Diagnostic testing revealed first documentation of hyperglycemia with brain MRI changes on T1 hyperintensity signals on the basal ganglia and T2/FLAIR weighted imaging showing mixed hypointense and hyperintense signals which is a classical MRI finding in patients with HC-HB syndrome caused by nonketotic hyperglycemia. The patient was treated for diabetes and was maintained on anti-dopaminergic medications for the uncontrollable involuntary movements. After five months, resolution of the hemiballism-hemichorea syndrome was noted after appropriate treatment. CONCLUSION: This case report highlights hemichoreahemiballism syndrome in a newly diagnosed type 2 diabetic patient who had normal glucose levels at presentation. The prompt recognition and correction of uncontrolled newly diagnosed diabetes and administration of anti-dopamine agents lead to a rapid improvement of symptoms, less neurologic sequelae and an overall favorable prognosis.
Subject(s)
Chorea , Dyskinesias , Hyperglycemia , Basal Ganglia Diseases , Diabetes Mellitus, Type 2 , Basal GangliaABSTRACT
Hyperglycemia-induced hemichorea (HGHC) is a rare but characteristic hyperkinetic movement disorder involving limbs on one side of the body. In a 75-year-old woman with a left-sided HGHC, conventional brain MR imaging showed very subtle T1-hyperintensity and unique gadolinium enhancement in the basal ganglia contralateral to movements. Multi-parametric MRI was acquired using pulse sequence with quantification of relaxation times and proton density by multi-echo acquisition. Myelin map was reconstructed based on new tissue classification modeling. In this case report of multi-parametric MRI, quantitative measurement of myelin change related to HGHC in brain structures and its possible explanations are presented. This is the first study to demonstrate myelin loss related to hyperglycemic insult in multi-parametric quantitative MR imaging.
Subject(s)
Aged , Female , Humans , Basal Ganglia , Brain , Classification , Extremities , Gadolinium , Hyperglycemia , Hyperkinesis , Magnetic Resonance Imaging , Movement Disorders , Myelin Sheath , Protons , RelaxationABSTRACT
Resumen La diabetes mellitus es una enfermedad de alta prevalência, así como también lo son sus complicaciones agudas. Una de ellas es el estado hiperosmolar no cetósico que raramente se asocia con alteraciones involuntarias del movimiento. Sin embargo, existe una condición que asocia alteraciones glucémicas con desórdenes del movimiento, denominado síndrome de corea hiperglicemia y ganglios basales, de la cual presentamos un caso y hacemos revisión de la literatura.
Abstract Diabetes mellitus is a disease of high prevalence, as well as its acute complications. One of them is the nonketotic hyperosmolar state that is rarely associated with involuntary changes in movement. However, there is a condition that associates glycemic alterations with movement disorders, called chorea hyperglycemia basal ganglia syndrome, of which we present a case and review the literature.
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Objective:To investigate the pathophysiology,clinical manifestation and neuroimaging characteristics and therapeutic experiences for hemichore associated with non-ketotic hyperglycemia (HC-NH).Methods:Clinical data of three patients with HC-NH from Xiangya Hospital,Central South University were analyzed retrospectively,and the related literature was reviewed.Results:The core clinical features of HC-NH were characterized by acute/subacute onset of hemichorea with non-ketotic hyperglycemia in the elderly females.Radiologic findings associated with HC-NH were characterized by hyperattenuation on computed tomographic (CT) scans and hyperintensity on Tl-weighted magnetic resonance imaging (MRI) at unilateral basal ganglion region.Blood glucose control was the foundation of treatment.Dopamine receptor antagonists and benzodiazepine sedative were helpful in controlling hemichorea.Conclusion:Hemichorea-hemiballismus is a rare complication of nonketotic hyperglycaemia in elderly type 2 diabetes.It is associated with contralateral striatal radiological abnormality and typically T1 hyperintensity on MRI.The pathophysiology of HC-NH is not clear.The prognosis of HC-NH is favorable.Antidiabetic drugs combined with dopamine receptor antagonists can effectively relieve the hemichorea symptoms.
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La corea es un trastorno hiperquinético del movimiento. Puede producirse por una gran variedad de enfermedades genéticas, infecciosas, neurodegenerativas, metabólicas, autoinmunes, estructurales o secundarias a fármacos. Es una rara manifestación de lesiones vasculares cerebrales isquémicas o hemorrágicas, afectando habitualmente un hemicuerpo y formando parte del clásico síndrome lacunar de hemicorea-hemibalismo. Presentamos el caso de un paciente de sexo masculino de 67 años de edad con factores de riesgo cardiovasculares, que se presentó con un accidente cerebrovascular a forma de hemicorea vascular. Si bien dicha presentación está descrita en toda la bibliografía, es importante por su baja frecuencia lo cual hace que la mayoría de los médicos clínicos nunca hayan estado en contacto con pacientes portadores de dicha entidad.
Chorea is a hyperkinetic movement disorder. It can be caused by a great variety of conditions: genetic, infectious, degenerative, metabolic, autoimmune, structural or related to drugs. A rare manifestation of brain vascular lesions, either isquemic or hemorrhagic, it generally affects one side of the body as part of the classic hemichorea-hemibalism lacunar syndrome. We present the case of a 67 year old male with risk factors for cardiovascular disease, admitted with the diagnosis of vascular hemichorea as the manifestation of a stroke. Although such presentation is described in most of our bibliography, it is important because of its low prevalence and, consequently, the fact that most general physicians have never had contact with patients suffering from this entity.
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Objective To study the clinical manifestations and neuroimaging characteristics of patients with hemichorea-hemiballismus (HC-HB) induced by nonketotic hyperglycemia. Methods The clinical data, laboratory findings and neuroimaging findings of 5 patients with HC-HB induced by nonketotic hyperglycemia who were treated in Changhai hospital of Second Military Medical University were retrospectively analyzed. The 5 patients included 4 females and 1 male, ranging 65-83 years old and averaging (76. 6±7. 2) years old. Results All the five patients presented an acute onset, with four of them having chorea or ballismus involuntary movement in unilateral limbs and face and one having generalized chore. The highest levels of blood glucose in patients at onset were 18. 6-44. 6 mmol/L (averaging[26. 6±10. 5] mmol/L), with negative urine ketone. T1-weighted imaging showed hyperintensity in contralateral basal ganglia in 4 cases and in bilateral basal ganglia in 1 case, with no edema or mass effect; most T2-weighted imaging was of isointensity. Increased protein levels and normal number of cells were observed in 3 cases in cerebrospinal fluid examination, and two of three cases had increased IgG index or 24 h intrathecal synthesis rate, which was relieved by effective control of blood glucose combined with pharmacotherapy, such as haloperidol. Conclusion Nonketotic hyperglycemia and HC-HB are the characteristics of HC-HB induced by nonketotic hyperglycemia, with hyperintensity on T1-weighted MRI imaging in the contralateral basal ganglia or in bilateral basal ganglia occasionally. Early diagnosis and proper treatment of those patients can achieve good prognoses.
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Hemichorea after cortical infarction has rarely been reported. We report a female patient in which hemichorea of the right extremities developed following a left temporo-parietal infarction. An acute infarction in the territory of the inferior division of the left middle cerebral artery was evident in diffusion-weighted imaging, but the basal ganglia, thalamus, and brainstem appeared normal. Her choreic movement gradually improved after administering haloperidol, and the hemichorea disappeared after 4 days.
Subject(s)
Female , Humans , Basal Ganglia , Brain Stem , Chorea , Extremities , Haloperidol , Infarction , Middle Cerebral Artery , ThalamusABSTRACT
Objective:To summarize the clinicopathologia and imaging features of Hemiballism-hemichorea induced by hyperglycemia and increase the understanding of the disease and improve the accuracy of diagnosis. Methods:The imaging images and clinical manifestation of 5 cases Hemiballism-hemichorea induced by hyperglycemia were analyzed retrospectively. The disease pathogenesis and mechanism relating to the Characteristic neuroradiological findings was obtained by literature review. Results:The common clinical manifestation of Hemiballism-hemichorea induced by hyperglycemia is unilateral limb involuntary movements. CT scans typically show an area of hyperdensity in the basal ganglia and The characteristic finding on the T1-weighted MRI is high signal intensity in the contralateral putamen. Conclusion: Hemiballism-hemichorea induced by hyperglycemia is an unusual but important differential diagnosis in patients with particular neuroradiological findings as prompt diagnosis and treatment of hyperglycemia has an excellent prognosis. The correct diagnosis could be received through integrated the patient's past medical history, presentation, laboratory and imaging inspection into account.
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Hemiballismus-hemichorea is an uncommon disorder. In the past, patients developing hemiballismus and hemichorea were considered to have poor prognosis with high morbidity and even mortality at times. While majority of patients of hemiballismus go into spontaneous remission, some severe cases require prolonged treatment. We here describe two such cases of which one presented to us with post stroke hemiballismus, unresponsive to monotherapy and the other was an HIV positive patient with unresponsive hemichorea secondary to CNS toxoplasmosis. Both these patients responded well when combination therapy was instituted and had normal recovery.
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Chorea is a major manifestation of acute RF and is the only evidence of RF in approximately 20% of cases. We report on a 15-year-old boy who presented with transient right side involuntary jerky movements, apical systolic murmur, sinus bradycardia, arthralgia, elevated antistreptolysin O titer and ESR, who was diagnosed with acute rheumatic fever and improved with haloperidol, prednisolone, digoxin, aspirin and furosemide and was given benzathine penicillin prophylaxis for future RF. Patient is faring well in follow up visits. We present our case because of its rarity.
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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 patients 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.
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Middle Aged , Chorea/diagnosis , Chorea/etiology , Vascular Diseases/complications , Functional Laterality , Magnetic Resonance Imaging , Prognosis , Tomography, X-Ray ComputedABSTRACT
Hemichorea-hemiballism is a rare complication of nonketotic hyperglycemia in type 2 diabetes mellitus (T2DM). It can be complicated in long-standing type 1 diabetes mellitus or T2DM, and has been described as a presenting symptom of new-onset diabetes. Rapid correction of diabetic ketoacidosis may also cause the delayed hemichorea. Although hyperglycemic hemiballism rarely causes generalized chorea due to bilateral basal ganglia involvement, patients typically present with hemichorea developing over days to months in the setting of elevated serum glucose. On T1-weighted brain magnetic resonance imaging and computed tomography scan a high signal intensity lesion at the basal ganglia is characteristic. After the correction of hyperglycemia, the movements generally disappear within hours, but atypical cases with delayed onset after the resolution of hyperglycemia, unremitting severe movements, and late recurrence are also reported. We report two cases of female T2DM patients who presented with hemichorea. One patient presented with hemichorea in nonketotic hyperglycemia, and the other with delayed onset hemichorea after the resolution of hyperglycemia.
Subject(s)
Female , Humans , Basal Ganglia , Brain , Chorea , Diabetes Mellitus, Type 1 , Diabetes Mellitus, Type 2 , Diabetic Ketoacidosis , Dyskinesias , Glucose , Hyperglycemia , Magnetic Resonance Imaging , RecurrenceABSTRACT
BACKGROUND: This paper synthesizes two unique cases of an adult Filipino patient presenting with involuntary unilateral extremity movements which upon further workup revealed cerebral toxoplasmosis as their primary etiology, further revealing their immunocompromised states. To our best knowledge there is limited available data on cerebral toxoplasmosis in our local setting. RATIONALE AND OBJECTIVES: The Filipino population is not spared from the pervasive global upsurge of AIDS cases as well as HIV-related infections. The aim of this report is to document the clinical features and diagnostics of two patients with cerebral toxoplasmosis presenting with unilateral involuntary extremity movements to heighten the level of awareness of Filipino physicians on the need to further explore possible etiologies of common neurological signs and symptoms as well as document additional cases of HIV-related infection in our country. CASE DESCRIPTION: The first case is a 30 year old male with no comorbidities who presented with fever and sudden onset of choreoathetoid movements of the left extremities. MRI revealed a rim enhancing nodule in the left supraorbital frontal lobe and in the middle cerebral peduncle. The second case is a 40 year old male, known hypertensive, initially diagnosed to have pulmonary tuberculosis, who presented with sudden onset of focal left arm myoclonic jerks. MRI revealed a rim enhancing cortical nodule on the right precentral gyrus. Both lesions were consistent with characteristics of toxoplasmosis. Both patients also showed positive serological titers for toxoplasmosis and had low CD4 T-cell count on flow cytometry. Both patients were treated with high dose trimethoprim-sulfamethoxazole and were further worked up for HIV-related infections. CONCLUSION: Majority of Filipino patients are still not provided with the opportunity to be diagnosed and treated for HIV-related infections, with one reason being the low index of suspicion for such cases. There is still limited amount of data available locally regarding patients presenting with Cerebral Toxoplasmosis, and that common neurological signs and symptoms as presented in this report should provide the much needed enlightenment in a physician's clinical eye to entertain such etiologies.
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Humans , Male , Adult , Acquired Immunodeficiency Syndrome , CD4-Positive T-Lymphocytes , Cerebral Peduncle , HIV Infections , Myoclonus , Toxoplasmosis, Cerebral , Trimethoprim, Sulfamethoxazole Drug Combination , Tuberculosis, Pulmonary , ChoreaABSTRACT
ObjectiveTo explore the clinical manifestations, pathogenesis, radiographic features and prognosis of Diabetic Hemichorea.Methods We conducted a retrospective analysis in 12 patients suffering Diabetic Hemichorea in terms of the clinical manifestations, radiographic features,laboratory results and treatment processeswho were hospitalized in our hospital from 2005 to 2010.Results Diabetic Hemichorea occurred mostly in the patients with diabetics with uncontrolled blood glucose level for a long term.Both ketosis and nonketosis patients can suffer this disease.Cranial CT and/or MRI imaging, especially those with high signal and clear border in MRIT1 WI but without edema shadow and mass effect, was of diagnostic value.The symptoms vanished 7 day to 4 years after the treatment.Conclusion Deepening the understanding of clinicians and imageological staff upon Diabetic Hemichorea can decrease the misdiagnosis and ensure timely treatment for this disease.
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Hemiballism is an uncommon neurological disorder characterized by uncontrollable movements of one lateral half of the body. We report a 56 years old male with a history of three weeks of polydipsia, polyuria and weight loss that, three days before consultation, started with hemiballism. A CAT scan without contrast showed a higher density in the lenticular nucleus and calcifications in caudate and lenticular nuclei. Diabetes was treated with regular insulin and hemiballism was controlled with neruoleptics. Ten days after admission a new CAT scan shows a partial regression of the lenticular lesion. After two months of follow up, the patient is asymptomatic.
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Humans , Male , Middle Aged , Diabetes Mellitus/physiopathology , Dyskinesias/etiology , Hyperglycemia/complications , Anti-Dyskinesia Agents/therapeutic use , Diabetes Mellitus/diagnosis , Dyskinesias/diagnosis , Dyskinesias/drug therapy , Hypoglycemic Agents , Haloperidol/therapeutic use , Hyperglycemia/drug therapy , Treatment OutcomeABSTRACT
@#Objective To investigate the related factors of hemichorea.Methods 15 patients with hemichorea were analyzed retrospectively,including their clinical features,characteristics of neuroimaging and treatment.They were followed up for 0.5～4 years.Results The serum glucose increase in 11 cases.The lacunar cerebral infarctions in the area of basal ganglion were found in 5 patients,and the microvessel lesions in the area of basal ganglion resulted from diabetes mellitus in 10 patients.13 patients accepted medicine treatment,11 cases improved,1 case was ineffective,1 case deteriorated.Conclusion The hemichorea mainly related with cerebrovascular disease.The hemichorea due to diabetes mellitus may related with the ischemic or hemorrhagic microvessel lesions.
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BACKGROUND: Hemichorea-Hemiballism (HCHB) can be caused by various diseases such as cerebrovascular disease, hyperglycemia, tumor, and inflammatory diseases. However, there are a few case studies using functional imaging such as single photon emission computed tomography (SPECT). METHODS: In this study, we included patients with HCHB. The patients with hyperglycemia over 250 mg/dl or high signal intensity on T1 weighted imaging were excluded. Clinical and neuroimaging characteristics of the patients were obtained and analyzed. RESULTS: We included 20 patients (M:F=12:8, mean age=67.1+/-15.3). Sixteen patients were presented with hemiballism and four with hemichorea. Six patients had no structural lesions causing HCHB. Subthalamic nucleus was the causative lesion in 6 patients. Other lesions associated with HCHB were basal ganglia, thalamus, and cortices. In a patient without structural lesion, anti-double stranded DNA antibody was detected. Brain SPECT showed not only perfusion abnormalities in the cases without structural lesions but also additional abnormalities in those with definite lesions. CONCLUSIONS: Various mechanisms were related to the development of HCHB. Functional imaging such as SPECT and immunological work-up is needed to investigate the underlying pathomechanism of HCHB.