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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., (© 2024 The Authors. Journal of Diabetes published by Ruijin Hospital, Shanghai Jiaotong University School of Medicine and John Wiley & Sons Australia, Ltd.)

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., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

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., (© 2022. Fondazione Società Italiana di Neurologia.)

Objective: To describe chorea/ballism triggered by a hyperglycemic event., Methods: We used the electronic records system at Mayo Clinic-Rochester to identify patients diagnosed with chorea or ballism from January 1st, 2000 through December 31st, 2014. Each record was reviewed to confirm chorea/ballism. From these cases we selected those that developed chorea/ballism within a month after a hyperglycemic episode (blood glucose >300 mg/dL). Clinical, laboratory, and imaging findings were analyzed., Results: Of the 596 chorea cases, we identified 7 patients (5 women) whose chorea was preceded by a hyperglycemic episode (range 3-30 days) during 15 years of surveillance, including new-onset diabetes in four cases. Median age was 80 years (range, 53-86). The chorea/ballism was unilateral in 6/7 cases and half of these unilateral cases had contralateral putamen T1-hyperintensity on brain MRI. After glucose correction, the chorea resolved within one week without recurrence in only one case. Among the 6 cases with persistent chorea, it was controlled with dopamine blocking/depleting medications., Conclusions: Chorea triggered by hyperglycemia is a rare complication of diabetes, with only seven cases identified at our tertiary medical center during 15 years of surveillance. This comprised about 1% of all chorea cases at our center during this time. Hyperglycemic chorea primarily developed in later life, with new-onset diabetes in the majority (4/7). Although MRI putamen T1-hyperintensity is reportedly typical, it was only seen in 3/6 cases. This MRI appearance may be mistaken for a hemorrhagic stroke, given the usual unilateral presentation. The chorea was controlled with dopamine blocking/depleting medications., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: Monoballismus is rarely seen clinically, but when observed, it is usually a manifestation of an acute cerebrovascular accident (CVA). We report a case of monoballismus observed in a patient without evidence of a CVA., Case Report: We observed a case of monoballismus in a 60-year-old diabetic patient who had not had a stroke. The movement disorder resolved with improvement of the patient's hyperglycemia. Nonketotic hyperglycemia is an uncommon cause of ballismus. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergency physicians should include the movement disorder of ballismus among the potential clues that a patient may be suffering an acute CVA. However, noncerebrovascular causes of ballismus exist. The movements manifest by a patient with ballismus should also lead the physician to consider the possibility not only of a CVA, but also neuroleptic malignant and serotonin syndromes., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Hemichorea-hemiballism (HC-HB) in uncontrolled diabetes mellitus is an uncommon manifestation of hyperglycemia. The pathophysiology of hyperglycemic HC-HB is not well understood. A previous report showed increased intracortical inhibition in the motor cortex in a patient with diabetes with HC-HB. The objective of this study is to investigate motor cortex excitability in patients with hyperglycemic HC-HB. We hypothesized that intracortical inhibition measured with transcranial magnetic stimulation, which likely reflects the excitability of cortical γ-aminobutyric acid (GABA)ergic circuits, would be impaired in patients with hyperglycemic HC-HB. We studied 15 patients with mean age 71.5 years (range, 48-94 y) and 12 age-matched healthy subjects. The motor cortex contralateral to the hemichorea was tested. Transcranial magnetic stimulation measures included motor evoked potential, recruitment curve, GABAA mediated short interval intracortical inhibition, intracortical facilitation, and GABAB mediated silent period duration and long interval intracortical inhibition. No significant difference was found in motor threshold, recruitment curve response, short interval intracortical inhibition, or intracortical facilitation in both rest and active conditions between patients with hyperglycemic HC-HB and normal subjects. However, long interval intracortical inhibition was significantly increased during muscle activation but not at rest in patients with hyperglycemic HC-HB. The silent period duration is also increased in patients with hyperglycemic HC-HB. We concluded that long interval intracortical inhibition and silent period are increased in the motor cortex contralateral to the hemichorea in hyperglycemic HC-HB, but only during muscle activation. Hemichorea-hemiballism may be associated with increased GABAB receptor-mediated inhibitory activity in the motor cortex., (© 2014 International Parkinson and Movement Disorder Society.)

Hemichorea, due to contralateral basal ganglia dysfunction, is well described in patients with hyperglycaemia but the underlying mechanism remains poorly understood. We present clinical and radiological data that support ipsilateral vascular compromise to the basal ganglia in such cases. We suggest vascular evaluation in patients with unilateral neurological phenomena secondary to suspected systemic or metabolic disorders.

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

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

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]

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]

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.

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]

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]

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]

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.

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.

Background: Hyperglycemic-hemichorea is a well-established clinical entity which leads to signal changes on brain MRI. We are reporting a case of hyperglycemic-hemichorea where the DaT scan showed reduced uptake bilaterally. Case Report: A 57-year-old female was seen in the clinic for hemichorea due to hyperglycemia. Her brain magnetic resonance imaging (MRI) showed increased T1 signal intensity in bilateral lenticular nuclei and the DaT scan showed reduced uptake on both sides. Discussion: This case highlights the importance of performing a DaT scan in the correct clinical context, as an abnormality on brain MRI can lead to false-positive DaT scan results.

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. [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. Significance: 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. [ABSTRACT FROM AUTHOR]

Objective: To explore the clinical manifestation, diagnosis, therapy, and mechanism of hemichorea associated with non-ketotic hyperglycemia (HC-NH) so as to enhance awareness and avoid misdiagnosis or missed diagnosis of the disease. Methods: A case of HC-NH was reported and reviewed in terms of the clinical features, diagnosis and treatment. Results: Hemichorea associated with non-ketotic hyperglycemia is a rare complication of diabetes mellitus, which is commonly seen in elderly women with poorly-controlled diabetes. The condition is characterized by non-ketotic hyperglycemia, unilateral involuntary choreiform movements, and contralateral basal ganglia hyper-intensity by T1-weighted MR imaging or high density on CT scans. Blood glucose control is the basal treatment, in combination with dopamine receptor antagonists and benzodiazepine sedative, in controlling hemichorea. Conclusion: In clinical practice, the possibility of unilateral chorea should be considered for diabetic patients with poor blood glucose control. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Lateralized involuntary movements consistent with hemichorea-hemiballism (HCHB) may appear following the development of contralateral haemorrhagic or ischaemic lesions of the basal ganglia, particularly the striatum (caudate nucleus and putamen). This condition is called vascular HCHB, but the same symptoms can be caused by a completely different striatal lesion. Glycaemic HCHB may occur in patients with uncontrolled hyperglycaemia: basal ganglia hyperdensity is seen on brain CT, while increased T1 signal intensity and reduced susceptibility-weighted imaging (SWI) and gradient-echo sequences (T2*-GRE) are detected on MRI. Case description: An 83-year-old man with multiple vascular risk factors and uncontrolled chronic hyperglycaemia was admitted for ischaemic stroke presenting with dysarthria and mild left hemiparesis. No involuntary movements were reported at admission. The emergent brain CT scan was negative for vascular acute lesions, while a mild bilateral hyperdensity of the striata was detectable. Involuntary movements on the left side of the body, consistent with HCHB, appeared 27 days later. The alterations on brain CT completely disappeared after 3 months. On brain MRI, the T1 signal alterations resolved after 10 months, while SWI and T2*-GRE sequences showed persisting alterations after 2 years. Discussion: Detailed brain imaging demonstrated evolution of striatal alterations of glycaemic HCHB before the appearance of involuntary movements and during the following 2 years. The association between ischaemic stroke and glycaemic HCHB favours the hypothesis that chronic hyperglycaemia more likely determines striatal alterations and the clinical picture of HCHB when vascular hypoperfusion also occurs.

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. [ABSTRACT FROM AUTHOR]

Non-ketotic hyperglycaemic hemichorea is a rare syndrome characterized by a triad of hemichorea, hyperglycemia, and peculiar imaging abnormalities. Regression of symptoms is typically associated with normalisation of serum glucose levels and plasma osmolality. An 85 year-old female was admitted to the neurology clinic with left-sided involuntary movements. Magnetic resonance imaging of brain demonstrated hyperintensity on T1 weighted images. She was treated with clonazepam (12. 5 mg/ day) for symptomatic therapy. Hyperglycaemia was controlled using a combined therapy with insulin aspart and insülin glargine. The patient showed prompt improvement in her involuntary movements in a day. Acute or subacute onset of unilateral involuntary movements should suggest non-ketotic hyperglycaemic hemichorea, the recognition of which may result in prompt control of the symptoms by normalization of blood glucose. [ABSTRACT FROM AUTHOR]

Background & objectives: Hyperosmolar hyperglycaemic state (HHS) is a medical emergency, but there is a paucity of studies reporting the spectrum of neurological manifestations of HHS. We, therefore, report the neurological spectrum, triggering factors and outcome of HHS in general neurology practice. Methods: The records of the patients with HHS were extracted from computerized hospital information system and those managed currently were prospectively included. The demographic, clinical manifestations, duration of diabetes and precipitating events such as infection, stress and stroke were noted. Patients with HHS were categorized into seizure, movement disorder and encephalopathy groups. Their electroencephalography, magnetic resonance imaging (MRI) findings and outcome were noted. Results: There were 17 patients with HHS (age range 40 and 75 yr) and seven were females. Seven patients were diabetic for five years, one for four years, one for one year and four were diagnosed after the occurrence of HHS. Four patients had epilepsia partialis continua persisting for 72-360 h, one patient had focal seizures and his MRI revealed T2 hyperintensity in frontal region in one patient and cerebellar vermian hyperintensity in another. All the five patients improved, but two had neurological deficits on discharge. Nine patients had encephalopathy which was precipitated by stroke in six patients, urinary infections in two and meningitis in one. Three females had hemichorea-hemiballismus syndrome, which was triggered by infections. Abnormal movements lasted 5-10 days and responded to correction of hyperosmolarity. Nine out of 17 patients improved completely whereas the remaining eight had partial recovery, these patients had stroke, ventilator-related complications or meningoencephalitis. Interpretation & conclusions: The most common presentation of HHS was encephalopathy (9) followed by seizure (5) and hemichorea-hemiballismus syndrome (3) which responded to the correction of hyperosmolar state. [ABSTRACT FROM AUTHOR]

Diabetic hemichorea-hemiballism with non-ketotic hyperglycemia is usually a benign syndrome. Here, we report a 78-year-old woman with persistent hemichorea (HC) for longer than 1 year with a recurrence after rapid correction of hyperglycemia. Following the disappearance of the characteristic T1 hyperintensity at 3 months after onset, an MRI demonstrated T2* hypointensity and atrophic changes in the contralateral striatum, suggesting irreversible neuronal loss and some vascular proliferation. The electrophysiological examination using transcranial magnetic stimulation revealed significantly shorter cortical silent periods (CSPs) on the contralateral primary motor cortex (M1), possibly in relation to cortical hyperexcitability. We have applied 10 daily sessions of low-frequency repetitive transcranial magnetic stimulation (rTMS) over the contralateral M1 to reduce the hyperexcitability. The HC was suppressed during and for several minutes after rTMS with prolongation of CSPs. After rehabilitation therapy, the patient was able to walk independently with a walker. We suggest that the combination of low-frequency rTMS and rehabilitation therapy may be a possible choice in medically refractory involuntary movements.

Background: Chorea can be caused by a variety of diseases, including neurodegenerative disorders, vascular events, toxic-metabolic states, and immunologic and infectious diseases. We describe a patient who presented with hemichorea as the initial manifestation of Diabetes Mellitus (DM) and responded partially to the glycemic control. Case report: A 63-year-old, healthy Hispanic man with no prior history of medical illness presented with subacute onset, gradually progressive hemichorea of 6 weeks' duration. On evaluation, he was found to have non-ketotic hyperglycemia with high serum glucose (328 mg/dL), elevated hemoglobin A1C (9.9%), and absent ketones. Magnetic Resonance Imaging of the brain demonstrated hyper intense signals in bilateral basal ganglia on T1W images. He was diagnosed to have DM. Despite optimal glycemic control with insulin, the patient continued to have hemichorea at 3 months follow-up and required haloperidol for control of the involuntary movements. Significance: Involuntary movements, particularly hemichorea, can be a manifestation and rarely be a presenting sign of DM. [ABSTRACT FROM AUTHOR]

Hemichorea hemiballismus (HCHB) is a rare and debilitating presentation of hyperglycemia and subcortical stroke. Early identification, proper assessment and management of HCHB can lead to complete symptom relief. We describe a case of HCHB presenting to a palliative care setting. A 63-year-old diabetic and hypertensive lady, with history of ovarian cancer presented to Palliative Medicine outpatient clinic with two days history of right HCHB. Blood investigations and brain imaging showed high blood sugar levels and lacunar subcortical stroke. Blood sugar levels were controlled with human insulin and Aspirin. Clopidogrel and Atorvastatin were prescribed for the management of lacunar stroke. HCHB reduced markedly post-treatment, leading to significant reduction in morbidity and improvement in quality of life. The symptoms completely resolved within one week of starting the treatment and the patient was kept on regular home and outpatient follow up for further monitoring. Acute palliative care (APC) approach deals with the management of comorbidities and their complications along with supportive care. Prompt assessment and management of such complications lead to better patient outcomes. [ABSTRACT FROM AUTHOR]

Abstract: Hemiballism is a rare movement disorder characterized by a high amplitude movement of an entire limb or limbs on one side of the body. The acute development of hemiballismus is often caused by focal lesions in the contralateral basal ganglia and STN. Many etiologies exist for this rare disorder with vascular causes and nonketotic hyperglycemia being the most common. Clearer understanding of the pathophysiology of hemiballism has led to important insights into the function and interaction of structures within the basal ganglia. Newer models of basal ganglia function have been proposed based on the study of the pathophysiology of hemiballism. Prognosis is favorable for most patients with complete resolution with or without treatment. Medical and surgical treatments are often successful in reducing or completely ameliorating the movements in those patients with more severe movements. [Copyright &y& Elsevier]

Hyperglycemia-induced unilateral basal ganglion lesions occur mostly in Asian patients. A signal abnormality in the basal ganglion region is evident on these patients’ neuroimaging. Despite characteristic imaging findings and clinical manifestations, the underlying mechanism is still unclear. To clarify the underlying pathophysiology of unilateral basal ganglion lesions, we examined the [18F]-fluorodeoxyglucose (FDG) positron emission tomography (PET) findings in 3 patients with hyperglycemia. The PET studies were performed at 3 weeks, 5 weeks, and 7 months after clinical onset. The markedly reduced rates of cerebral glucose metabolism in the corresponding lesions on T1-weighted magnetic resonance images provided direct evidence of regional metabolic failure. We suggest that the metabolic derangements associated with hyperglycemia and vascular insufficiency contribute to regional metabolic failure in patients with poorly controlled diabetes mellitus. [ABSTRACT FROM AUTHOR]

Background: Chorea associated with non-ketotic hyperglycemia and high signal intensity lesions on T1-weighted brain magnetic resonance images (C-H-BG) is recognized as a unique syndrome that affects elderly women exclusively. However, its overall clinical features are unclear. Material and Methods: The literature describing patients with C-H-BG from 1985 to 2001 was reviewed using MEDLINE. Their clinical features and those of four patients with C-H-BG at this hospital were analyzed. Results: This study included 49 patients from the literature and four patients at this hospital. Their mean age at the onset was 71.1 years (range=22–92 years). Women were affected more frequently than men (men/women=17:30). The mean serum glucose level measured after the onset of chorea was 481.5 mg/dl (ranging from 169 to 1264), HbA1c level was 14.4% (ranging from 9.9 to 19.2), and the serum osmolarity was 305.9 mmol/kg (ranging from 291 to 335). Forty-seven patients developed hemichorea. Six patients developed bilateral chorea, and magnetic resonance imaging (MRI) showed bilateral basal ganglia lesions. MRI showed that putamen was involved in all cases (isolated putamen=31 patients, additional basal ganglia lesions=22 patients). None had lesions confined to the caudate nucleus or the globus pallidus. In all, except one, the anterior limb of the internal capsule was spared. Follow-up MRI studies were performed in 22 patients. In most, hemichorea improved along with the disappearance of the lesions. In 39 patients, chorea had ameliorated completely. The remaining 14 cases showed some improvement during the follow-up period. The chorea recurred in seven patients. Conclusion: C-H-BG is a benign disorder affecting the elderly. It affects men much more frequently than has been reported. The high signal intensity basal ganglia lesion on the T1-weighted brain MRI study was reversible, and correlated with the clinical improvement in chorea. [Copyright &y& Elsevier]

Hemichoreais a rare neurologic disorder due to oxidative stress leading to neurodegenerationof the dentate nuclei and striata. It is rarely observed in diabetes. One such case occurring in an adult female diabetic is described here.

Abstract: Background: Metabolic conditions, including hyperglycemia, can have various neurological presentations. Hemiballismus hemichorea is a rare manifestation reported to occur with severe hyperglycemia and is reversed in most cases with control of sugars. Case Report: We present a case of a patient with no known diabetes history who presented with uncontrolled jerky movements of one-half of her body, which resolved with achievement of euglycemia. Conclusions: Important differential diagnoses that need to be evaluated are discussed. [Copyright &y& Elsevier]

Hemichorea-hemiballism (HCHB) syndrome, which is most commonly related to non-ketotic hyperglycemia, is a rare type of chorea. Here, we present an unusual case of HCHB syndrome who was not a known case of diabetes. This case highlights the importance of recognising underlying non-ketotic hyperglycemia, as control of hyperglycemia is helpful in the quick relief of symptoms. [ABSTRACT FROM AUTHOR]