Search

Rui Wang,1,2 Meng Li,2 Yao Wang,1 Chao Zhi Tang,3 Yuan Chen Wang4 1Department of Neurology, Henan Rongjun Hospital, Xinxiang City, Henan Province, People’s Republic of China; 2Department of Neurology, The Fifth Clinical College of Xinxiang Medical College, Xinxiang City, People’s Republic of China; 3College of Life Science, Henan Normal University, Xinxiang City, Henan Province, People’s Republic of China; 4School of Clinical Medicine, Zhengzhou University, Zhengzhou City, Henan Province, People’s Republic of ChinaCorrespondence: Rui Wang, Email 2360062620@qq.comObjective: To explore the effect of infection on hyperglycemic hemichorea.Methods: The clinical data of 11 patients with hyperglycemic hemichorea admitted to the Affiliated People’ s Hospital of Xinxiang Medical College and the Second People’s Hospital of Xinxiang were retrospectively analyzed, including gender, age, clinical symptoms, imaging features, blood glucose, glycated hemoglobin, infection indicators, and treatment conditions.Results: Eleven patients had acute or sub-acute onset, including 9 females and 2 males, with an average age of 74.55 years. Nine patients presented with unilateral limb involuntary movement and 2 patients presented with bilateral limb involuntary movement. Imaging findings of 9 patients showed abnormalities in the basal ganglia region. Random blood glucose levels were all elevated at admission, with an average blood glucose value of 26.20 mmol/l. Urine ketone bodies were positive in 2 patients, inflammatory indexes were elevated in 7 patients, symptoms of hypoglycemic treatment and anti-infection treatment in 10 patients disappeared, and symptoms of 1 patient disappeared after improved microcirculation treatment.Conclusion: The disease tends to occur in middle-aged and elderly women, and the blood glucose may fluctuate significantly during the onset. Infection may lead to the occurrence and development of the disease. Active control of blood sugar, inflammation and improvement of brain metabolism can effectively control symptoms and prevent recurrence.Keywords: hyperglycemia, infection, hemichorea, clinical analysis

Objective: To summarize the clinical characteristics and imaging manifestations of patients with nonketotic hyperglycemic hemichorea (NH‐HC) and to explore the possible pathogenesis, diagnosis. and treatment of the disease in order to improve the understanding of this disease and avoid misdiagnosis. Methods: Retrospective analysis was performed on the case data of five patients with NH‐HC admitted to our hospital in recent years. The patients were treated in the department of endocrinology, department of neurology, and department of neurosurgery in our hospital, respectively. Meanwhile, relevant literatures were consulted for further learning. Results: NH‐HC is usually presented as a triad of nonketotic hyperglycemia, lateral chorea, and typical imaging manifestations of head magnetic resonance imaging or computed tomography, but the clinical manifestations are not the same, and imaging features may also be different, presenting a diversified trend in clinical practice. All five patients were given glucose‐lowering drugs and improved with or without combination of drugs to control symptoms of chorea. Conclusion: NH‐HC is a rare complication of diabetes, characterized by hyperglycemia and hemichorea. How to identify the extreme situation and make fast judgment is a top priority. Timely and correct control of blood glucose is the key to the treatment, and when necessary, application of dopamine receptor antagonists in patients with combination therapy can accelerate improvement of the clinical symptoms. The prognosis of NH‐HC is good, the clinician should strengthen comprehensive understanding of this disease to avoid missed diagnosis or misdiagnosis and enable patients to get more timely and effective treatment. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) can present with movement disorders, among which chorea is closely associated with antiphospholipid (aPL) antibodies. Brain imaging results obtained in patients with chorea are generally inconsistent with the clinical manifestation of chorea; moreover, medical tests for hemichorea, which are expected to reveal distinct localization, may show negative findings. Herein, we present a case of a 15‐year‐old girl with SLE who had a history of left cerebral infarction; tests revealed elevated aPL levels, and she developed recurrent left hemichorea 2 years later. Brain magnetic resonance imaging (MRI) results revealed no acute lesions during each episode of involuntary movements, and an MRI perfusion scan failed to provide an explanation for the asymmetric presentation. Although various hypotheses have been proposed regarding the mechanism underlying the occurrence of chorea, some scenarios still remain unexplained. Further investigation on the pathophysiology of chorea in SLE may be warranted to clarify its prognosis. [ABSTRACT FROM AUTHOR]

Abstract Objective To summarize the clinical characteristics and imaging manifestations of patients with nonketotic hyperglycemic hemichorea (NH‐HC) and to explore the possible pathogenesis, diagnosis. and treatment of the disease in order to improve the understanding of this disease and avoid misdiagnosis. Methods Retrospective analysis was performed on the case data of five patients with NH‐HC admitted to our hospital in recent years. The patients were treated in the department of endocrinology, department of neurology, and department of neurosurgery in our hospital, respectively. Meanwhile, relevant literatures were consulted for further learning. Results NH‐HC is usually presented as a triad of nonketotic hyperglycemia, lateral chorea, and typical imaging manifestations of head magnetic resonance imaging or computed tomography, but the clinical manifestations are not the same, and imaging features may also be different, presenting a diversified trend in clinical practice. All five patients were given glucose‐lowering drugs and improved with or without combination of drugs to control symptoms of chorea. Conclusion NH‐HC is a rare complication of diabetes, characterized by hyperglycemia and hemichorea. How to identify the extreme situation and make fast judgment is a top priority. Timely and correct control of blood glucose is the key to the treatment, and when necessary, application of dopamine receptor antagonists in patients with combination therapy can accelerate improvement of the clinical symptoms. The prognosis of NH‐HC is good, the clinician should strengthen comprehensive understanding of this disease to avoid missed diagnosis or misdiagnosis and enable patients to get more timely and effective treatment.

Yumi Otaka,1 Yukinori Harada,1 Norio Sugawara,2 Taro Shimizu,1 Norio Yasui-Furukori2 1Department of Diagnostic and Generalist Medicine, Dokkyo Medical University, School of Medicine, Tochigi, Japan; 2Department of Psychiatry, Dokkyo Medical University, School of Medicine, Tochigi, JapanCorrespondence: Norio Yasui-Furukori, Department of Psychiatry, Dokkyo Medical University, School of Medicine, Mibu, Shimotsuga, Tochigi, 321-0293, Japan, Tel +81-282-86-1111, Fax +81-282-86-5187, Email furukori@dokkyomed.ac.jpBackground: Diabetic chorea is a rare complication of diabetes mellitus for which head MRI is the most common diagnostic imaging modality. Cases have been reported where CT and/or MRI findings are inconsistent or clinical symptoms and imaging findings do not appear simultaneously. We aimed to compile the cases in which imaging findings appeared on MRI retests and to examine in a systematic review whether temporal differences in the appearance of imaging findings correlate with clinical characteristics.Case Presentation: An 80-year-old man with type 2 diabetes mellitus came to a hospital with abnormal movements of the left upper and lower extremities. Two days after the first visit, his symptoms flared up, and his head MRI showed an old cerebral infarction and no new lesion. On day 14, he retested T1-weighted imaging and showed a high signal in the right putamen, which was considered diabetic chorea. Blood glucose was controlled with insulin, and the involuntary movements disappeared.Methods: PubMed and ICHUSHI were searched to identify patients with diabetic chorea who had undergone MRI retests. Patients grouped by the temporal change in the presence/absence of imaging findings were compared on age, sex, duration of diabetes mellitus, blood glucose level, HbA1c level, side of involuntary movement, time to first MRI, and follow-up MRI.Results: Of the 64 cases analyzed, 43 (67.2%) were female. The mean age was 69.0 years. 16 (25.0%) had worsening findings upon MRI retesting, 37 (57.8%) had improvement, and 10 (15.6%) had unchanged findings. There were no significant differences in age, sex, mean blood glucose level or HbA1c at onset among the groups.Conclusion: There was no association between the pattern of appearance of imaging findings over time and clinical characteristics, including glucose levels. If initial MRI findings are negative, MRI retesting after a certain time may help diagnose diabetic chorea.Keywords: hyperglycemia, hemichorea, hemiballismus, diabetes, movement disorders

Due to their immunocompromised state, recipients of hematopoietic stem cell transplants (HSCTs) are at a higher risk of opportunistic infections, such as that of toxoplasmosis. Toxoplasmosis is a rare but mortal infection that can cause severe neurological symptoms, including confusion. In immunosuppressed individuals, such as those with acquired immunodeficiency syndrome (AIDS), toxoplasmosis can cause movement disorders, including hemichorea–hemiballismus. We present the case of a 54-year-old Caucasian male with a history of hypertension and JAK-2-negative primary myelofibrosis who underwent an allogeneic peripheral blood stem cell transplant from a related donor. After the development of acute changes in mental status, left-sided weakness, and left-sided hemichorea–hemiballismus post-transplant, the patient was readmitted to the hospital. Subsequent testing included an magnetic resonance imaging (MRI) of the brain, which revealed multiple ring-enhancing lesions around the thalami and basal ganglia, as well as a cerebrospinal fluid tap that tested positive for toxoplasmosis. The patient was initially treated with intravenous clindamycin and oral pyrimethamine with leucovorin. The completion of treatment improved the patient's mental status but did not improve his hemichorea–hemiballismus. This case illustrates an uncommon complication associated with central nervous system (CNS) toxoplasmosis in stem cell transplant recipients. Due to its rarity, cerebral toxoplasmosis in immunocompromised patients often remains undetected, particularly in HSCT patients who are immunosuppressed to improve engraftment. Neurological and neuropsychiatric symptoms due to toxoplasmosis may be misidentified as psychiatric morbidities, delaying appropriate treatment. Polymerase chain reaction (PCR) assays offer methods that are sensitive and specific to detecting toxoplasmosis and provide opportunities for early intervention. [ABSTRACT FROM AUTHOR]

Non-ketotic hyperglycaemic hemichorea-hemiballismus (NHHH) is often secondary to middle-aged and elderly diabetic people with poor-controlled diabetes; Fahr's disease (FD) is another rare neurological disorder characterized by abnormal calcified deposits in the brain that control movement. We described a rare case of NHHH combined with a heterozygous mutation (SLC20A2) resulting in one family with FD. The patient has a 30-day history of involuntary choreic movements of the left limbs and left face. In addition, he had a bit of speech slurred and walked unsteadily. He was diagnosed with type 2 diabetes mellitus two months ago. Over the past two months, he had noticed that urination, appetite, and water volume increased obviously and weight loss drastically. Other problems such as dizziness, headache, difficulty swallowing, nausea, and vomiting did not occur. T1- weighted MRI indicts characteristic contralateral basal ganglia hyper-intensity. During hospitalization, he was injected insulin and oral haloperidol. And the clinical symptoms improved, but parkinsonism symptoms emerge soon after discharge. The parkinsonism symptoms were gradually improved after adjusting medications. Combined with the subsequent genetic test results, we attribute it to NHHH with FD. It is relatively rare that NHHH or FD is both presents. We should use antipsychotics with caution in these patients to avoid parkinsonism symptoms. [ABSTRACT FROM AUTHOR]

Hemichorea caused by cerebral infarction in the centrum semiovale is a rare condition that can often be misdiagnosed. In this case report, we present a rare case of a 66-year-old man who experienced involuntary movement in his left limbs due to acute cerebral infarction in the centrum semiovale. The patient did not have any obvious inducements for the hemichorea. In this case, the treatment approach followed the guidelines for treating acute cerebral infarction, combined with the use of dopamine receptor blockers. The involuntary movements gradually improved and completely remitted after 5 days of treatment, with no relapse within the following 6 months. To summarize, this case report highlights the rare occurrence of hemichorea caused by cerebral infarction in the centrum semiovale. Prompt recognition and appropriate treatment are essential to prevent misdiagnosis and ensure optimal management of the condition. [ABSTRACT FROM AUTHOR]

Non-Ketotic Hyperglycaemic (NKH) Hemichorea-Hemiballismus Syndrome/Diabetic Striatopathy (DS) is a rare neurological complication of diabetes mellitus. Hemichorea can present as a manifestation of diabetes mellitus or occur in patients with poorly controlled diabetes. This case report highlights the importance of differentiating this syndrome from other intracranial pathologies, as adequate glycaemic control can lead to complete resolution of the symptoms. The authors present a case of a 65-year-old female who presented to the Emergency Department (ED) with a sudden onset of involuntary movements in the left upper and lower limbs for 30 minutes. The patient was conscious and oriented, with a General Random Blood Sugar (GRBS) of 502 mg/dL, and ketone levels of 0.1 mmol/L (normal value: less than 0.6 mmol/L). The Computed Tomography (CT) scan revealed high attenuation in the right basal ganglia, and the Magnetic Resonance Imaging (MRI) of the brain indicated T1 hyperintensity in the right basal ganglia. The patient was managed with insulin infusion and hydration. Involuntary movements completely resolved after blood sugar levels were controlled with insulin. Hyperglycaemia should be considered as a differential diagnosis in patients presenting to the ED with hemichorea.

Abstract Background Hemichorea typically results from a contralateral subthalamic nuclei (STN) lesion, although it has been reported in the cortex in a minority of cases. However, to our best knowledge, there are no documented cases in literature of hemichorea occurring as a secondary condition to an isolated temporal stroke. Case presentation We present a case of an elderly female who sustained a sudden onset of hemichorea in her right extremities, predominantly in the distal region, lasting over a period of two days. Brain diffuse weighted image (DWI) demonstrated a high signal in the temporal region, while magnetic resonance angiography (MRA) revealed severe stenosis of the middle cerebral artery. During the symptomatic phase, computed tomography perfusion (CTP) revealed delayed perfusion in the left middle cerebral artery territory, characterized by the time-to-peak (TTP) measure. Based on the results of her medical history and laboratory tests, we were able to rule out the possibility of infectious, toxic, or metabolic encephalopathy. Her symptoms gradually improved with antithrombotic and symptomatic treatment. Conclusions It is important to recognize and consider acute onset hemichorea as an initial symptom of stroke to avoid misdiagnosis and delays in appropriate treatment. Further research on temporal lesion that lead to hemichorea is warranted to gain a better understanding of the underlying mechanisms.

Introduction: Cerebrovascular disease often presents with “negative” symptoms such as weakness with reduced movement of body parts or sensory loss. Rarely do “positive” symptoms such as abnormal movements manifest in acute stroke, with hemichorea being a very rare manifestation.  Case Report: This is a case report of a 62-year-old chronic smoker with no known past medical history who presented with choreatic movements of his arm and leg. Magnetic resonance imaging of the brain showed changes consistent with an infarct in the right centrum semiovale. He was treated with dual antiplatelets and was noted to have subsequent improvement in symptoms.  Conclusion: Recognition and awareness of stroke presenting as movement disorders in the emergency department can help prevent delays in diagnosis and treatment.

Background: Hemichorea and hemiballismus are rare movement disorders caused by central nervous system toxoplasmosis. Objective: To describe the case of a male patient with treatmentnaïve HIV who initiated with right hemichorea and hemiballismus. Case report: We present a 29-year-old male with a history of untreated HIV. He presented to the emergency room with rightsided hemichorea and hemiballismus. The neurological examination was unremarkable, except for the presence of abnormal movements characterized by non-rhythmic, large-amplitude, violent and occasionally choreiform movements in the right side of his body. Brain MRI revealed a ringenhancing lesion in the left basal nuclei, raising suspicion of CNS toxoplasmosis. Further serologic tests confirmed the diagnosis. Treatment was initiated with trimethoprim-sulfamethoxazole, pyrimethamine/clindamycin and antiretrovirals. Haloperidol, aripiprazole, and clonazepam were administered to alleviate abnormal movements. Subsequent MRI scans showed radiological improvement, and the patient was discharged. Conclusion: Hemichorea-hemiballismus is a neurological manifestation unfrequently related to CNS toxoplasmosis. Prompt initiation of antiretroviral therapy and antimicrobial treatment is crucial to improve patient outcomes. [ABSTRACT FROM AUTHOR]

Context: Hemichorea associated with nonketotic hyperglycemia (HC-NH) is a rare diabetic complication for which the pathogenesis remains unclear. Objective: This study reported 16 cases of HC-NH to improve the understanding of the disease and avoid misdiagnosis and missed diagnosis. Methods: Data of 16 patients with HC-NH in a single center from 2000 to 2021 were analyzed retrospectively, and the relevant literature was reviewed. Results: The participants (8 men and 8 women) had a mean age of 67.6 ± 16.4 years. Bilateral limbs were involved in 2 cases, and the others had hemichorea (6 in the left side and 8 in the right side). The average random blood glucose level was 17.51 ± 7.67 mmol/L, and the glycated hemoglobin A1c level was 11.9%±3.1% at admission. Eleven patients had a history of diabetes, and the other 5 patients were diagnosed with new-onset diabetes mellitus, but no remarkable differences were observed in the presentation or treatment of chorea. Ketonuria was detected in 7 patients. The basal ganglia (putamen, globus pallidus, and caudate nucleus) of 9 cases had typical hyperdensity on computed tomography and/or hyperintensity signals from magnetic resonance imaging. The chorea symptoms of 15 patients improved within 5.0 ± 1.9 days after treatment. Conclusion: This study provides additional valuable information about the clinical and neuroimaging features of HC-NH. We hypothesize that chronic ischemia of the basal ganglia due to cerebral atherosclerosis combined with hyperglycemia is associated with HC-NH. [ABSTRACT FROM AUTHOR]

Hyper-/hypoglycemic states are rare but well-established causes of hyperkinetic movements, including chorea and ballismus, usually associated with brain lesions in the basal ganglia. We report a case of hemichorea-hemiballismus (HCHB) syndrome that developed after a severe hypoglycemic episode in a 71-year-old man with poorly controlled type 2 diabetes mellitus. Uncommonly, brain MRI showed contralateral cortical-subcortical T2 and T2-FLAIR-hyperintense frontoparietal lesions, with cingulate gyrus involved, while the basal ganglia were unaffected. In patients with hypoglycemic encephalopathy associated with cortical lesions, the long-term prognosis is usually poor. Nevertheless, in our patient, the dyskinesias and the cerebral lesions progressively regressed by achieving good glycemic control. After four and 12 months, the patient’s neurological examination was normal. To our knowledge, this is the first evidence of hypoglycemic etiology of cortical HCHB syndrome, supporting recent theories that cortical circuitries may independently contribute to the pathogenesis of chorea and ballismus. This is also the first report of cingulate gyrus involvement in hypoglycemic encephalopathy. Finally, this case may indicate that a subset of patients with cortical lesions due to hypoglycemia could present a good clinical outcome, likely depending on the size of the lesions and the duration and severity of the hypoglycemic episode.

Background: Hemichorea typically results from a contralateral subthalamic nuclei (STN) lesion, although it has been reported in the cortex in a minority of cases. However, to our best knowledge, there are no documented cases in literature of hemichorea occurring as a secondary condition to an isolated temporal stroke. Case presentation: We present a case of an elderly female who sustained a sudden onset of hemichorea in her right extremities, predominantly in the distal region, lasting over a period of two days. Brain diffuse weighted image (DWI) demonstrated a high signal in the temporal region, while magnetic resonance angiography (MRA) revealed severe stenosis of the middle cerebral artery. During the symptomatic phase, computed tomography perfusion (CTP) revealed delayed perfusion in the left middle cerebral artery territory, characterized by the time-to-peak (TTP) measure. Based on the results of her medical history and laboratory tests, we were able to rule out the possibility of infectious, toxic, or metabolic encephalopathy. Her symptoms gradually improved with antithrombotic and symptomatic treatment. Conclusions: It is important to recognize and consider acute onset hemichorea as an initial symptom of stroke to avoid misdiagnosis and delays in appropriate treatment. Further research on temporal lesion that lead to hemichorea is warranted to gain a better understanding of the underlying mechanisms. [ABSTRACT FROM AUTHOR]

Non-Ketotic Hyperglycaemic (NKH) Hemichorea-Hemiballismus Syndrome/Diabetic Striatopathy (DS) is a rare neurological complication of diabetes mellitus. Hemichorea can present as a manifestation of diabetes mellitus or occur in patients with poorly controlled diabetes. This case report highlights the importance of differentiating this syndrome from other intracranial pathologies, as adequate glycaemic control can lead to complete resolution of the symptoms. The authors present a case of a 65-year-old female who presented to the Emergency Department (ED) with a sudden onset of involuntary movements in the left upper and lower limbs for 30 minutes. The patient was conscious and oriented, with a General Random Blood Sugar (GRBS) of 502 mg/dL, and ketone levels of 0.1 mmol/L (normal value: less than 0.6 mmol/L). The Computed Tomography (CT) scan revealed high attenuation in the right basal ganglia, and the Magnetic Resonance Imaging (MRI) of the brain indicated T1 hyperintensity in the right basal ganglia. The patient was managed with insulin infusion and hydration. Involuntary movements completely resolved after blood sugar levels were controlled with insulin. Hyperglycaemia should be considered as a differential diagnosis in patients presenting to the ED with hemichorea. [ABSTRACT FROM AUTHOR]

Hyper-/hypoglycemic states are rare but well-established causes of hyperkinetic movements, including chorea and ballismus, usually associated with brain lesions in the basal ganglia. We report a case of hemichorea-hemiballismus (HCHB) syndrome that developed after a severe hypoglycemic episode in a 71-year-old man with poorly controlled type 2 diabetes mellitus. Uncommonly, brain MRI showed contralateral cortical-subcortical T2 and T2-FLAIR-hyperintense frontoparietal lesions, with cingulate gyrus involved, while the basal ganglia were unaffected. In patients with hypoglycemic encephalopathy associated with cortical lesions, the long-term prognosis is usually poor. Nevertheless, in our patient, the dyskinesias and the cerebral lesions progressively regressed by achieving good glycemic control. After four and 12 months, the patient's neurological examination was normal. To our knowledge, this is the first evidence of hypoglycemic etiology of cortical HCHB syndrome, supporting recent theories that cortical circuitries may independently contribute to the pathogenesis of chorea and ballismus. This is also the first report of cingulate gyrus involvement in hypoglycemic encephalopathy. Finally, this case may indicate that a subset of patients with cortical lesions due to hypoglycemia could present a good clinical outcome, likely depending on the size of the lesions and the duration and severity of the hypoglycemic episode. [ABSTRACT FROM AUTHOR]

Background: Anti-IgLON5 disease is an autoimmune encephalopathy with sleep disturbances as a hallmark in the majority of reported cases. Additional clinical symptoms are heterogenous and include movement disorders, bulbar dysfunction, autonomic disorders, and neurocognitive impairment. Case: Here, we report the case of an 87-year-old woman presenting with isolated progressive hemichorea. An extensive diagnostic work-up revealed antibodies against IgLON5 in the serum. Neither history nor polysomnography (PSG) unveiled signs and features of sleep dysfunction typically reported in anti-IgLON5 disease. Literature Review: In an extensive literature review we identified twelve other studies reporting about patients with confirmed anti-IgLON5 disease and chorea as extrapyramidal movement disorder in their clinical phenotype. Subsequently, clinical characteristics of these patients were carefully evaluated. Conclusions: Our results support the diversity of clinical phenotypes in anti-IgLON5 disease, adding isolated hemichorea to the spectrum of presenting symptoms. As sleep-related disorders are often not the leading reason for consultation and only revealed by PSG examination, we suggest that screening for antibodies against IgLON5 should be considered in patients presenting with unexplained movement disorders, including isolated hemichorea. [ABSTRACT FROM AUTHOR]

Abstract Background Chorea hyperglycemia basal ganglia syndrome is a rare disorder causing involuntary movements in both upper and lower limbs. Uncontrolled hyperglycemia causes neurotransmitter derangements in basal ganglia resulting in involuntary movements. These cases pose different challenges to anaesthesiologist in both general and regional anaesthesia. Case presentation Here, we discuss a case of chorea hyperglycemia basal ganglia syndrome with lower limb gangrene posted for knee amputation done under spinal anaesthesia. Our patient had recurrent choreiform movements due to non-compliance to diabetic medications. Strict glycemic control always aids in reduction of choreiform movements along with improved wound healing. Conclusions Tight glycemic control and good anesthetic plan in perioperative period can reduce morbidity in patients affected with chorea hyperglycemia basal ganglia syndrome.

Diabetic striatopathy is a rare clinico-radiological phenomenon characterized by hemichorea/hemiballismus, hyperglycemia, hyperosmolarity in the absence of ketoacidosis. It is due to lesion in contralateral basal ganglia which is evident as T1 hyperintensity in MRI brain study. Here we are reporting a case of hemichorea /hemiballismus which was associated with hyperglycemia. Our patient improved significantly once blood glucose was controlled adequately. As it carries good prognosis, we are reporting this rare case to illustrate the importance of recognition of this rare syndrome.

Involuntary movements following aneurysm surgery are rare. Here, we report the case of a patient who developed left hemichorea following surgical clipping of a right internal carotid artery bifurcation aneurysm. The movement disorder resulted from an acute infarct involving the head of the caudate nucleus. The patient responded well to symptomatic treatment, with complete resolution of chorea. The occurrence of new-onset involuntary movements following aneurysm surgery should raise the suspicion of infarcts involving basal ganglia structures. [ABSTRACT FROM AUTHOR]

Diabetic chorea is a rare movement disorder associated with diabetes mellitus. We report the case of a patient that benefited from pimozide and died of pancreatic cancer. A 70-year-old woman presented with pollakiuria and involuntary movements of left limbs since three months. Laboratory tests revealed high serum levels of glycemia and glycated haemoglobin. She was admitted to internal medicine department and discharged one week later: insulin was administered with normalization of blood glucose levels and the involuntary movements gradually disappeared. Three weeks later she was admitted to neurological department due to the recurrence of the involuntary movements. Glycemia and other routine laboratory tests were normal. Neurological examination showed choreic movements involving left limbs. MRI showed a hyperintensity on T1- and T2-weighted sequences of right putamen and caudate nucleus head. Haloperidol was administered without improvement, it was successively substituted with tetrabenazine and the patient was discharged with an unvaried clinical picture. Two months later tetrabenazine was discontinued because of inefficacy and pimozide was started. The choreic movements considerably diminished after few days. Four months later, a pancreatic cancer was diagnosed and the patient died in the same month. Clinical and radiological features were suggestive of diabetic chorea. Our patient benefited exclusively from pimozide, it could be reasonable to use pimozide in resistant form and also propose it as first choice treatment. Another important element is the diagnosis of pancreatic cancer some months after chorea onset: a causal link could exist. [ABSTRACT FROM AUTHOR]

Background: Diabetic striatopathy is defined as a state of hyperglycemia associated with chorea/ballism, striatal hyperdensity at CT, or hyperintensity at T1-weighted MRI. It is considered a rare complication of uncontrolled diabetes but prevalence data are scarce.Objectives: Characterize diabetic striatopathy prevalence in the population afferent to the largest teaching hospital in Genova (Liguria, Italy) and investigate the role of glycated hemoglobin level in predicting the risk.Methods: Data were retrospectively obtained from general population undergoing blood sampling for glycated hemoglobin and resulting with HbA1c values ≥ 8%, from January 2014 to June 2017. Brain neuroimaging of those who underwent at least a brain CT or MRI was examined in search of findings compatible with diabetic striatopathy and clinical information was collected. Logistic regression was used to predict the risk of diabetic striatopathy based on age and HbA1c values.Results: Subjects with uncontrolled diabetes were 4603. Brain neuroimaging was available in 1806 subjects and three patients with diabetic striatopathy were identified, all of them reporting choreic movements. The prevalence of hemichorea due to diabetic striatopathy was therefore 3 cases out of 1806 (0.16%) in our population. Hepatic and hypoxic encephalopathies were the conditions most frequently mimicking diabetic striatopathy. Odds ratio of diabetic striatopathy and HbA1c level was significantly correlated (p = 0.0009).Conclusions: To the best of our knowledge, this study is the first to evaluate the prevalence of diabetic striatopathy in Italy. High HbA1c values may have a role in predicting diabetic striatopathy. [ABSTRACT FROM AUTHOR]

Objective: To explore the clinical manifestations, diagnosis, treatment, and pathogenesis of diabetic striatopathy (DS) to improve the understanding of the disease and avoid misdiagnosis or underdiagnosis.Methods: The clinical, laboratory, and imaging data of 6 patients (5 Asian females and 1 Asian male) with diabetic striatum were analyzed retrospectively, and the related literature was reviewed.Results: All 6 patients showed hyperglycemia, 5 patients presented with involuntary movement of unilateral limbs, and 1 with unilateral limb numbness. Besides, 5 patients (except case 3) underwent MRI examinations that showed hyperintensity in unilateral caudate and lentiform nucleus on T1-weighted images. And all 6 patients who underwent brain CT examinations showed hyperdensity or isodensity in unilateral caudate and lentiform nucleus. None had a family history of similar abnormal movements. After blood glucose control and symptomatic support treatment, the symptoms of all patients improved to various degrees, and reexaminations showed that the lesions gradually disappeared.Conclusion: Diabetic striatal disease is a rare complication of diabetes mellitus, the result of a combination of different pathogenesis. It is characterized by hyperglycemia, hemichorea, and contralateral striatal T1WI hyperintensity or CT hyperdensity. Both ketosis and nonketotic hyperglycemic hemichorea have typical imaging manifestations. The prognosis is excellent when this disease is detected early, and the lesions can be gradually absorbed and dissipated with glycemic control. [ABSTRACT FROM AUTHOR]

Nonketotic hyperglycemia-induced hemichorea is a rare condition of type 2 diabetes. It is characterized by hyperglycemia with the symptom traced to the basal ganglion like hemichorea or hemiballism, with the hyperintensity within basal ganglion presented in computed tomography (CT) or hyper signal in T1-weighted magnetic resonance image (MRI). It was also confirmed with a relatively better prognosis in that the symptoms of these patients could be relieved after the alleviation of hyperglycemia. However, the exact pathophysiology or mechanism of this condition currently was unclear. Besides, the duration of improvement in tomography as far was varied. In the present study, we reported an elderly female patient who tested with nonketotic hyperglycemia (random blood glucose of fingertips was 19 mmol/L or 342 mg/dL, blood ketone was 0.1 mmol/L) with the symptoms of dysphoria and mild chorea of left low limb, the MRI and CT showed contralateral striatopathy. Her condition achieved alleviation after the normalization of blood glucose. We subsequently rechecked her MRI in arterial spin labeling sequence which showed the hypoperfusion in the right basal ganglion rather than the opposite. Therefore, we suppose the hyperglycemia could induce temporary hypoperfusion in the basal ganglion associated with motor dysfunction which is manifested by hemichorea or hemiballism.

Abstract Background Hemichorea is usually caused by contralateral deep structures of brain. It rarely results from acute cortical ischemic stroke and that caused by ipsilateral brain lesions is even rarer. Case presentation A 64-year-old female presented with acute obtuseness and left-sided hemichorea. She had a history of right frontal lobe surgery and radiotherapy due to brain metastasis from lung cancer 8 years ago. MRI revealed acute left frontal lobe infarction in addition to an old right frontal lobe lesion. 18FDG PET-CT showed hypometabolism in the left frontal lobe and hypermetabolism in the right basal ganglia region and central sulcus. The choreatic movement remitted after antipsychotic treatment. Conclusion The mechanism of hemichorea after ipsilateral cortical infarction is poorly understood. We assume both previous contralateral brain lesion and recent ipsilateral ischemic stroke contributed to the strange manifestation in this case.

Diabetic striatopathy is a rare clinico-radiological phenomenon characterized by hemichorea/hemiballismus, hyperglycemia, hyperosmolarity in the absence of ketoacidosis. It is due to lesion in contralateral basal ganglia which is evident as T1 hyperintensity in MRI brain study. Here we are reporting a case of hemichorea/hemiballismus which was associated with hyperglycemia. Our patient improved significantly once blood glucose was controlled adequately. As it carries good prognosis, we are reporting this rare case to illustrate the importance of recognition of this rare syndrome. [ABSTRACT FROM AUTHOR]

Abstract Background Despite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF. Case presentation A previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine. The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs. Conclusions SC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease.

Background: Chorea hyperglycemia basal ganglia syndrome is a rare disorder causing involuntary movements in both upper and lower limbs. Uncontrolled hyperglycemia causes neurotransmitter derangements in basal ganglia resulting in involuntary movements. These cases pose different challenges to anaesthesiologist in both general and regional anaesthesia. Case presentation: Here, we discuss a case of chorea hyperglycemia basal ganglia syndrome with lower limb gangrene posted for knee amputation done under spinal anaesthesia. Our patient had recurrent choreiform movements due to non-compliance to diabetic medications. Strict glycemic control always aids in reduction of choreiform movements along with improved wound healing. Conclusions: Tight glycemic control and good anesthetic plan in perioperative period can reduce morbidity in patients affected with chorea hyperglycemia basal ganglia syndrome. [ABSTRACT FROM AUTHOR]

Hemidystonia, unilateral limb choreoathetosis, unilateral limb tremor, faciobrachial dystonia, and myoclonus have been reported in the literature. Keywords: Rasmussen's encephalitis; Hemichorea; Movement disorder EN Rasmussen's encephalitis Hemichorea Movement disorder 542 545 4 05/13/22 20220501 NES 220501 Movement disorders in Rasmussen's encephalitis (RE) are rarely described. Hyperkinetic movement disorders except myoclonus3 have been rarely described in RE because the more predominant presentation of seizures overshadows subtle movement disorders; however, a thorough understanding of movement disorders in RE is important because these may be the only presentations of a patient with RE. [Extracted from the article]

Teaching Point: Unilateral choreiform movements combined with contralateral hyperdense lentiform and/or caudate nucleus on computed tomography is suggestive for non-ketotic hyperglycemia, warranting further metabolic workup.

Objective: We describe an unusual case of diabetic hemichorea hemiballismus (diabetic HCHB) with symptoms resistant to traditional therapy and exacerbated by hypoglycemia. Case Presentation: A 62-year-old woman with a 3-year history of noninsulin dependent type 2 diabetes presented with left-sided, involuntary, “jerking” movements. History included inconsistent metformin use, peripheral vascular disease, hypertension, and hyperlipidemia. Physical exam was documented as chorea of the left upper and lower extremity. Blood glucose was 776 mg/dL (82-115 mg/dL), and head computed tomography scan was read as asymmetric hyperattenuation of the right lentiform nucleus. The chorea dissipated within 48 hours of basal, bolus insulin and maintenance of blood glucose from 140 to 180 mg/dL. Hyperintensities were not documented on magnetic resonance imaging 4 days later. The patient presented twice in the following weeks for increasing frequency of chorea and hypoglycemia of 62 mg/dL and 40 mg/dL. Repeat magnetic resonance imaging was read as right-sided basal ganglia hyperintensities. Short courses of haloperidol, alprazolam, and tizanidine and a 2-week course of olanzapine yielded no improvement in chorea. Two weeks of tetrabenazine did improve the chorea; however, residual weakness and gait dysfunction persisted. Discussion: The differential diagnosis for chorea includes hereditary and acquired forms. Diabetic HCHB is a rare, acquired, metabolic form that occurs in older, female, type 2 diabetics with poor glucose control. The patient experienced exacerbations of chorea in the setting of hypoglycemia. Conclusion: Glycemic control is important in the long-term management of diabetic HCHB, and this case demonstrates hypoglycemia as a potential cause for resistant cases.

Background: We report a 74-year-old lady with hemichorea of 6 months duration due to severe carotid stenosis without cerebral infarction. Electroencephalography and single-photon emission computed tomography studies suggested hemispheric abnormality. Hemichorea resolved following carotid endarterectomy. Discussion: Hemichorea is the commonest movement disorder following acute stroke. It is rarely reported in association with severe carotid stenosis without evident infarction. In the absence of corresponding structural abnormalities on magnetic resonance imaging in hemichorea, vascular imaging is critical in establishing the diagnosis. Hemichorea in setting of carotid or vascular stenosis is often resistant to medical treatment and best responds to reperfusion. Conclusion: Hemichorea due to critical hemispheric ischemia is a well-described rare entity that best responds to reperfusion strategies. Awareness is essential to identify this entity and manage appropriately. [ABSTRACT FROM AUTHOR]

Asymmetric chorea unrelated to structural lesions is typically due to systemic etiologies, such as metabolic, autoimmune, or other inflammatory disorders. This is an editorial commenting on a paper by Batot C, Chea M, Zeidan S, et al. Clinical and radiological follow up of Pfizer-BioNTech COVID-19 vaccine-induced hemichorea-hemiballismus. Tremor and Other Hyperkinetic Movements; 2022; 12(1). DOI: https://doi.org/10.5334/tohm.688. A 90-year-old patient is reported who developed hemichorea shortly after his second vaccination against COVID-19. Hypometabolism was noted in the contralateral striatum. This case provides potential insights and raises questions about mechanisms of immunemediated hemichorea. [ABSTRACT FROM AUTHOR]

Background: Hemichorea-hemiballismus is a rare hyperkinetic movement disorder. Case Report: A 90-year-old male developed left hemichorea-hemiballismus after his second dose of the Pfizer-BioNTech COVID-19 vaccine. A wide range of investigations including magnetic resonance imaging did not reveal an alternative cause. [18F]- fluorodeoxyglucose-positron emission tomography (FDG-PET) showed increases in right putamen fixation compared to the left side. The patient showed significant improvement after five days of intravenous corticosteroids, with a normal FDG-PET. Discussion: This hemichorea-hemiballismus case shows dynamic restoration of putamen metabolism mirroring clinical evolution after administration of corticosteroids, suggesting an autoimmune COVID-19 vaccine-induced reaction. [ABSTRACT FROM AUTHOR]

Background: Chorea can be due to a large number of etiologies. Unilateral chorea is classically related to a contralateral structural lesion, e.g. of the putamen or subthalamic nucleus, however, based upon personal impressions, we have observed that systemic disease, in particular metabolic or autoimmune conditions, can also lead to a unilateral or markedly asymmetric presentations. We sought to investigate this impression by reviewing the literature. Methods: A PubMed search was conducted using the terms asymmetric" AND "chorea" OR "hemichorea" OR "unilateral" AND "chorea" OR "monochorea" OR "right greater than left" AND "chorea" OR "left greater than right" AND "chorea" OR "right more than left" AND "chorea" OR "left more than right" AND "chorea" as well as "hemiballismus" NOT "stroke" NOT "infarct" NOT "dyskinesia. A total of 243 sources were felt to meet criteria and were reviewed. Results: The most common etiology of reported hemi- or asymmetric chorea was diabetic non-ketotic hyperglycemic hemichorea/hemiballismus. Other common diagnoses were Sydenham's disease, antiphospholipid syndrome and drug-induced chorea. The vast majority of patients with hemi- or asymmetric chorea had acquired rather than genetic, degenerative or congenital causes. Conclusion: Despite the potential limitations of our literature review, the evidence presented here supports the observation that the vast majority of asymmetric or unilateral chorea presentations are due to acquired causes, and in this situation an exhaustive search for reversible etiology should be undertaken. However, presentation with symmetric, generalized chorea does not exclude reversible causes, and investigations should address these in addition to genetic and neurodegenerative etiologies. [ABSTRACT FROM AUTHOR]

Introduction: Chorea, hyperglycemia, basal ganglia syndrome (CHBG) is a rare neurological complication of nonketotic hyperglycemia which occurs more often in elderly female patients with undiagnosed or poorly controlled diabetes mellitus. Case report: We present the case of a 82-year old female, diagnosed with type 2 diabetes mellitus, but untreated, admitted with non-ketotic hyperglycemia and hemiballistic movements in her left limbs, with acute onset, that changed to choreic movements and then disappeared. Brain magnetic resonance imaging (MRI) showed characteristic hyper intensity in the right (contralateral) putamen on the T1-weighted images. Other secondary causes for ballismus and chorea were excluded. Hemiballismus/hemichorea non-ketotic hyperglycemic basal ganglia (CHBG) syndrome was considered and blood glucose was lowered using insulin. As symptomatic treatment, Haloperidol was started but, due to adverse effects, it was stopped and Clonazepam was associated. The movement disorders disappeared in two weeks after glycemic control. Signifi cance: Movement disorders, like chorea and/or ballismus (hemichorea/hemiballismus) can be a marker of uncontrolled known diabetes mellitus or a presenting sign for an undiagnosed diabetes mellitus. The chorea hyperglycemia basal ganglia (CHBG) syndrome is rare and likely undiagnosed but, being aware of it’s existence is of high importance, as normalising blood sugar values severe neurological complications can be avoided.

Introduction: There remains uncertainty about the mechanism and specific location of the relative cortex with nonketotic hyperglycaemic hemichorea-hemiballismus (HC-HB). This paper aims to analyse the relationship between the disappearance of HC-HB and the supplementary motor area (SMA) infarction in a patient who recovered following an acute ischemic stroke., Case Presentation: An 83-year-old female patient with diabetes mellitus presenting with severe and refractory involuntary movement after hypoglycaemic therapy was referred to an outpatient neurosurgery department for further intervention. Laboratory, magnetic resonance imaging (MRI) and computed tomography (CT) neuroimaging and physical examinations were performed. After a diagnosis of HC-HB was confirmed, the patient received hypoglycaemic therapy and haloperidol; however, there was no significant improvement. Brain MRI T1-weighted images and CT scans showed high signal intensity involving the bilateral putamen nucleus. CT perfusion and CT angiography showed a hypo-perfusion in the SMA of the right hemisphere without significant vascular occlusion. Then, aspirin and clopidogrel were administered, and the patient's left leg presented slight involuntary movement three days later. Interestingly, her involuntary movement disappeared again on the second day after the discontinuation of antiplatelet therapy. She was discharged three days later, and her symptoms did not recur during a follow-up for three months., Conclusions: The SMA dysfunction caused by the acute infarction could terminate or reset the pathological neural path-way of nonketotic hyperglycaemic HC-HB and contribute to the disappearance of the involuntary movement on the contralateral side. The SMA may be a selective intervention target for patients with refractory nonketotic hyperglycaemic HC-HB.

Diabetic striatopathy (DS) is an acute hyperkinetic movement disorder arising from non-ketotic hyperglycemia. This condition predominantly affects females and is more common in the elderly, highlighting the interplay between diabetes, striatal pathology, and neurological movement disorders. DS is characterized by involuntary movements, such as hemichorea or hemiballism, and distinctive neuroimaging findings that can be mistaken for more common cerebrovascular events. In this case report, we describe a 67-year-old female with a history of poorly controlled type 2 diabetes mellitus who presented with the sudden onset of involuntary movements affecting her left upper and lower limbs. Clinical examination and laboratory investigations revealed hyperglycemia without ketosis. Neuroimaging via computed tomography (CT) of the brain identified a hyper density in the right lentiform nucleus, consistent with DS. The patient was treated with vesicular monoamine transporter 2 (VMAT) inhibitors, oral hypoglycemic agents, and insulin, resulting in marked symptom improvement over 10 days. This case underscores the importance of recognizing DS as a differential diagnosis in patients with hyperkinetic movement disorders and hyperglycemia. Proper diagnosis and management, including stringent glycemic control, are crucial for symptom resolution., Competing Interests: Human subjects: All authors have confirmed that this study did not involve human participants or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Nilofar et al.)

Background: Hemichorea is usually caused by contralateral deep structures of brain. It rarely results from acute cortical ischemic stroke and that caused by ipsilateral brain lesions is even rarer.Case Presentation: A 64-year-old female presented with acute obtuseness and left-sided hemichorea. She had a history of right frontal lobe surgery and radiotherapy due to brain metastasis from lung cancer 8 years ago. MRI revealed acute left frontal lobe infarction in addition to an old right frontal lobe lesion. 18FDG PET-CT showed hypometabolism in the left frontal lobe and hypermetabolism in the right basal ganglia region and central sulcus. The choreatic movement remitted after antipsychotic treatment.Conclusion: The mechanism of hemichorea after ipsilateral cortical infarction is poorly understood. We assume both previous contralateral brain lesion and recent ipsilateral ischemic stroke contributed to the strange manifestation in this case. [ABSTRACT FROM AUTHOR]

We report a case of a 74-year-old Japanese woman who, after left thalamic infarction, developed right hemichorea and its neglect. This rare finding was associated with ipsi- and contralateral brain perfusion changes, presumably reflecting de-afferentiation within the brain.

Kidney transplantation is the preferred renal replacement therapy for patients with end-stage kidney disease. However, these patients are vulnerable to multiple complications arising out of immunosuppression, comorbidities, and opportunistic infections. We report a case of hemichorea in a transplant recipient. This patient had concurrent polycythemia, new-onset diabetes after transplantation, and was on calcineurin-based immunosuppression. The magnetic resonance imaging ascribed her hemichorea to Non ketotic hyperglycemia (NKH) which recovered completely with prompt treatment of the hyperglycemia.

We describe an 83-year-old man who presented with acute ischemic stroke after an inguinal hernia operation. After mechanical thrombectomy, the patient regained full neurological function; however, 5 days later, he developed left hemichorea without radiological abnormalities at that time. His hemichorea was successfully treated with a small dose of haloperidol. A slight hyperintense signal was noted at the right putamen on T1-weighted imaging performed 43 days after the onset. This is the first report to highlight the development of delayed-onset hemichorea, despite the initial resolution of the neurological symptoms in a patient with acute ischemic stroke successfully treated with mechanical thrombectomy.

Abstract We describe a case of a 9‐year‐old child who presented with uncontrollable, involuntary movements associated with a recent streptococcal infection and echocardiographic evidence of valvulitis. These findings are consistent with the diagnosis of Sydenham's chorea, a rare but important movement disorder and one of the major “Jones criteria” for the diagnosis of acute rheumatic fever. Because of its rarity, patients with Sydenham's chorea often are misdiagnosed as having a behavioral or psychiatric illness. Early recognition and appropriate management can prevent the potential severe sequelae associated with acute rheumatic fever.

Isolated hemichorea (HC) in adults has a relatively restricted differential diagnosis including stroke of contralateral basal ganglia nuclei, nonketotic hyperglycemia, and basal ganglia toxoplasmosis in HIV infection. Hypoparathyroidism-related basal ganglia calcification can potentially cause neurological problems, including movement disorders, that are usually bilateral in keeping with bilateral symmetric lesions. We report a patient with video-documented isolated, adult-onset HC due to iatrogenic hypoparathyroidism and bilateral basal ganglia calcification. A 47-year-old woman presented with isolated adult-onset HC of 2 years' duration as the presenting and only neurological feature of hypoparathyroidism and bilateral extensive basal ganglia calcification, 20 years after thyroidectomy-induced hypoparathyroidism. Significant improvement in the unilateral hyperkinesia was noted after correction of hypocalcemia and hypoparathyroidism at 3 months. Isolated HC in adults is a rare presenting feature of hypoparathyroidism with bilateral basal ganglia calcification and is treatable with correction of the underlying metabolic abnormality. In all cases with a movement disorder and brain calcification, hypoparathyroidism should be actively sought as this treatable condition must not be missed.

Abstract Diabetes may cause late complications in the CNS but certain lesions may also occur acutely in hyperglycemia. We describe a case of hyperosmolar non‐ketotic syndrome and reversible hemichoreic dyskinesia with morphological changes in basal ganglia.

Background: Despite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF.Case Presentation: A previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine. The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs.Conclusions: SC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease. [ABSTRACT FROM AUTHOR]