Unraveling Nonketotic Hyperglycemia Hemichorea-Hemiballismus Syndrome: A Case Report of Diagnosis and Management.

Nonketotic hyperglycemia hemichorea-hemiballismus syndrome (NHH) is an uncommon neurological condition linked to poorly managed diabetes mellitus (DM). It presents with spontaneous, erratic movements that impact just one side of the body. Our case of NHH was of a 76-year-old female with uncontrolled type 2 DM, ischemic heart disease, and dilated cardiomyopathy. Despite previous treatment for similar symptoms, the patient developed left-sided choreo-ballistic movements. Despite difficulties obtaining clear magnetic resonance imaging (MRI) due to involuntary movements, the image revealed T1 hyperintense signals in the right lentiform nucleus and subtle signals in the left lentiform nucleus and external capsule. Management included insulin, tetrabenazine, haloperidol, lorazepam, and other adjunctive therapies, resulting in symptom resolution by the fourth day. This case underscores the importance of considering NHH in patients with uncontrolled DM presenting with abnormal movements, highlighting the challenges in imaging due to involuntary movements and emphasizing the need for aggressive glycemic control and treatment strategies.

Recovery of nonketotic hyperglycaemic hemichorea -hemiballismus due to acute ischemic stroke in the contralateral supplementary motor area: a case report and literature review.

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.

A Case of Non-ketotic Hyperglycemic Hemichorea and Fahr Syndrome.

Non-ketotic hyperglycemic hemichorea (NHH) denotes acute hemichorea or hemiballism in patients with poorly controlled diabetes with striatal abnormalities seen on brain MRI. Here, we describe a case with diabetes mellitus and primary hypoparathyroidism who developed NHH with bilateral chorea due to the abrupt stopping of her diabetic regimen. She presented with subacute and progressive bilateral asymmetric chorea. Over the prior six months, she stopped following her diabetic regimen. Brain imaging showed features of diffuse brain calcifications suggestive of Fahr syndrome. Extensive blood investigations including genetic testing for causes of basal ganglia calcifications were unremarkable. Treatment with tetrabenazine and resumption of her diabetes medications slowly improved her chorea. This case highlights the importance of interpreting imaging findings in the context of the nature and time course of the chorea presentation. In addition, it emphasizes a systematic approach to interpreting diffuse brain calcifications with the appropriate investigations.

Further learning of clinical characteristics and imaging manifestations of nonketotic hyperglycemic hemichorea.

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.

A Case Report and Literature Review of Nonketotic Hyperglycemic Hemichorea.

Nonketogenic hyperglycemic hemichorea, also recognized as diabetic striatopathy, is a rare manifestation of diabetes mellitus. The diagnosis of nonketotic hyperglycemic hemichorea is usually made through imaging along with a corresponding clinical picture. Early identification and treatment can lead to complete resolution of the symptoms and better patient outcomes. Here we present a 49-year-old female patient, with a past medical history of poorly controlled type 2 diabetes mellitus and prior left index finger amputation as a complication of her diabetes, who presented for evaluation of a two-week duration of sudden-onset left upper extremity choreiform movements.

Combined thalamic and pallidal deep brain stimulation in diabetic hemiballism/hemichorea.

Hemiballism/hemichorea (HH) is a hyperkinetic movement disorder observed mostly in older adults with cerebrovascular diseases. Although the symptoms improve without any treatment, lesioning or DBS (deep brain stimulation) may be rarely required to provide symptomatic relief for patients with severe involuntary movements. HH is a rare complication of uncontrolled diabetes. There are only a few reported cases of diabetic HH that have been surgically treated. Thus, herein, we have reported the case of a 75-year-old female with type-II diabetes mellitus that presented with disabling involuntary limb movements of the left side, despite being treated conservatively for six months. DBS targeting the globus pallidus internus (GPi) and ventral intermediate (Vim) thalamic nucleus was performed. Complete resolution of symptoms was achieved with a combined stimulation of the thalamic Vim nucleus (at 1.7 mA) and GPi (at 2.4 mA). The combined stimulation of the Vim nucleus and GPi effectively resolved the diabetes-induced HH symptoms in our patient. Thus, although certain conclusions cannot be drawn due to the rarity of the surgically treated patients with HH, the combined stimulation is a novel treatment option for resistant HH.

Nonketotic hyperglycemia hemichorea and hemiballismus: a case report.

BACKGROUND: Diabetic striatopathy, also known as hyperglycemic hemichorea-hemiballismus, is a rare movement disorder associated with nonketotic hyperglycemia in patients with poorly controlled diabetes mellitus. The pathophysiology is not fully elucidated but may involve hyperviscosity, ischemia, and alterations in basal ganglia neurotransmitters. CASE PRESENTATION: We present a case of a 64-year-old Asian female patient with longstanding poorly controlled type 2 diabetes mellitus who developed abrupt-onset right-sided hemichorea-hemiballismus. Laboratory results showed hyperglycemia without ketoacidosis. Neuroimaging revealed left putaminal hyperdensity on computed tomography and T1 hyperintensity on magnetic resonance imaging. With insulin therapy and tetrabenazine, her movements improved but persisted at 1-month follow-up. DISCUSSION: This case illustrates the typical features of diabetic striatopathy, including acute choreiform movements contralateral to neuroimaging abnormalities in the setting of nonketotic hyperglycemia. While neuroleptics may provide symptomatic relief, prompt glycemic control is critical given the risk of recurrence despite imaging normalization. CONCLUSION: Diabetic striatopathy should be recognized as a rare disorder that can occur with poorly controlled diabetes. Further study of its pathophysiological mechanisms is needed to better guide management. Maintaining tight glycemic control is essential to prevent recurrence of this debilitating movement disorder.

Hyperglycemia-Induced Complete Left-Sided Hemiballismus Due to Uncontrolled Diabetes in a 70-Year-Old Female: A Case Report.

This report details the presentation of a 72-year-old female with left-sided continuous non-rhythmic involuntary movements persisting for two months. The movements affected the left side of her face, arm, and leg. The patient had a history of multiple hyperglycemic episodes and diabetic ketoacidosis. This report investigates the basal ganglia's involvement in hemiballismus, a movement disorder possibly linked to the patient's hyperglycemia. It discusses the complex management of hyperglycemia-induced hemiballismus and the need for more research to understand the underlying mechanism and optimal treatment strategies.

Hyperglycemia-induced hemichorea-hemiballismus syndrome - a systematic review.

Nonketotic hyperglycemia may occur as a cause of chorea in patients with chronic decompensated diabetes. Because it is rare and consequently poorly studied, diagnosis and treatment can be delayed. Therefore, our objective was to summarize clinical and radiological features, as well as treatments performed, from previously reported cases to facilitate adequate management in clinical practice. We searched MEDLINE/PubMed, EMBASE, Cochrane, CINAHL, Web of Science, Scopus, and LILACS databases for studies published before April 23, 2021. We included case reports and case series of adults (aged ≥ 18 years) that described hyperglycemic chorea with measurement ofglycated hemoglobin (HbA1c) and cranial magnetic resonance imaging (MRI). Studies were excluded if participants were pregnant women, aged < 18 years, and had no description of chorea and/or physical examination. We found 121 studies that met the inclusion criteria, for a total of 214 cases. The majority of the included studies were published in Asia (67.3%). Most patients were women(65.3%) aged > 65 years (67.3%). Almost all patients had decompensated diabetes upon arrival at the emergency department (97.2%). The most common MRI finding was abnormalities of the basal ganglia (89.2%). There was no difference in patient recovery between treatment with insulin alone and in combination with other medications. Although rare, hyperglycemic chorea is a reversible cause of this syndrome; therefore, hyperglycemia should always be considered in these cases.

Hemichorea in patients with temporal lobe infarcts: Two case reports.

BACKGROUND: Hemichorea and other hyperkinetic movement disorders are uncommon presentations of stroke and are usually secondary to deep infarctions affecting the basal ganglia and thalamus. Therefore, temporal ischemic lesions causing hemichorea are rare. We report the cases of two patients with acute ischemic temporal lobe infarct strokes that presented as hemichorea. CASE SUMMARY: Patient 1: An 82-year-old woman presented with a 1-mo history of involuntary movement of the left extremity, which was consistent with hemichorea. Her diffusion-weighted imaging (DWI) revealed an acute ischemic stroke that predominantly affected the right temporal cortex, and magnetic resonance angiography of the head showed significant stenosis of the right middle cerebral artery (MCA). Treatment with 2.5 mg of olanzapine per day was initiated. When she was discharged from the hospital, her symptoms appeared to have improved compared with those previously observed. Twenty-seven days after the first admission, she was readmitted due to acute ischemic stroke. Computed tomography perfusion showed marked hypoperfusion in the right MCA territory. An emergency transfemoral cerebral angiogram was performed and showed severe stenosis in the M1 segment of the right MCA. After percutaneous transluminal angioplasty was successfully performed, abnormal movements or other neurologic problems did not occur. Patient 2: A 76-year-old man was admitted to our hospital for a 7-d history of right-upper-sided involuntary movements. DWI showed an acute patchy ischemic stroke in the left temporal lobe without basal ganglia involvement. Subsequent diffusion tensor imaging confirmed fewer white matter fiber tracts on the left side than on the opposite side. Treatment with 2.5 mg of olanzapine per day improved his condition, and he was discharged. CONCLUSION: When acute hemichorea suddenly appears, temporal cortical ischemic stroke should be considered a possible diagnosis. In addition, hemichorea may be a sign of impending cerebral infarction with MCA stenosis.

Characterization of Diabetic Striatopathy With Repeated Follow-Up Using Multiple Imaging Studies.

Diabetic striatopathy is a rare condition with a prevalence of less than one in 100,000. Herein, we report a case of diabetic striatopathy exacerbated by hyperglycemia and hypoglycemia, with repeated follow-up with multiple imaging studies. This case suggested that putamen neuronal loss and dysfunction, gliosis, and ischemia are associated with diabetic striatopathy pathophysiology. In addition, striatal hyperintensity on T1-weighted MRI images was more pronounced after symptom remission when evaluated several times over a short period. Therefore, clinicians should be aware that even if MRI findings are normal in the very early stages of the onset of diabetic striatopathy, repeating MRIs at intervals may reveal typical findings.

Hemichorea-hemiballismus associated with a case of cerebral toxoplasmosis in a hematopoietic stem cell transplant recipient.

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.

A rare neurological manifestation of diabetes mellitus-Hemichorea-hemiballismus in a patient with diabetic striatopathy: A case report.

Diabetic striatopathy is a rare neurological complication of diabetes mellitus that presents with sudden onset hemichorea or hemiballismus and is associated with hyperglycemia and striatal abnormality, either by hyperdensity on non-contrast computer tomography or hyperintensity on T1-weighted magnetic resonance imaging. Here we report a 55-year-old female, from Sri Lanka, who presented with involuntary movements of the left upper and lower limbs. Her past medical history included diabetes mellitus and she was on warfarin 5 mg daily for a mechanical mitral and tricuspid valve replacement. The random blood sugar on admission was 462 mg/dL and the last INR was 3.03. While hemiballismus has multiple etiologies, intracranial hemorrhage would be the main differential in a patient on anticoagulation. Other differentials include drug-induced dyskinesia, metabolic abnormalities, and autoimmune etiologies. Hemiballismus in the presence of high blood glucose should always raise the suspicion of diabetic striatopathy. The non-contrast computed tomography of the brain showed hyperdensity in the right-side caudate nucleus, lentiform nucleus, and globus pallidus which is a characteristic of diabetic striatopathy but could have been mistaken for an intracranial hemorrhage. The involuntary movements improved with glucose control and treatment with clonazepam and tetrabenazine. This case highlights the potential for misdiagnosis of diabetic striatopathy as an intracranial hemorrhage in a patient on warfarin, which can lead to delays in appropriate management and erroneous omission of warfarin. Early recognition and treatment of diabetic striatopathy can lead to significant improvement in the quality of life.

Footloose (footloose), footloose.

A 78-year-old woman without past relevant medical history presented to the emergency department for acute transient dysarthria. NIHSS was 0/42. Neurological examination revealed chorea-like movements over the left limbs, especially the foot. No other neurological signs were present. CT perfusion showed right cortical hypoperfusion due to right M2 occlusion, basal-ganglia perfusion was normal. Brain MRI revealed a small focus of restricted diffusion in the right insula, sparing basal ganglia. Based on the neuroimaging features and clinical correlation, despite the NIHSS score, we decided to treat the patient with alteplase, after iv-thrombolysis hyperkinetic movements ceased completely. Brain-MRI performed 72 h after symptom onset confirmed a confined insular ischemic lesion without the involvement of deep gray matter structures. Hyperkinetic movement disorders, such as hemichorea hemiballismus, are rare presentations of stroke, basal ganglia are mainly involved even if the insular cortex has been described too. Clinical decision on whether to treat ischemic stroke does not include movement disorders. Our case underscores NIHSS limitations in clinical practice.

A case of hemichorea associated with nonketotic hyperglycaemia: A new magnetic resonance spectroscopy (MRS) finding and possible future implications.

BACKGROUND: Diabetic Striatopathy (DS) is a rare complication of a poor-controlled Diabetes Mellitus consisting of sudden onset of movement disorders. To date, there is still poor knowledge about the pathogenesis. CASE: We describe a 79 year old men affected by sudden onset hemichoreic movements whose cause was a non-ketotic hyperglycaemia diagnosed despite the normal blood glucose levels thanks to brain CT and magnetic resonance imaging. Then, we introduce a new magnetic resonance spectroscopy (MRS) finding never described until today which allowed us to produce a new pathogenetic theory of a phenomenon still without definitive explanations. LITERATURE REVIEW: We performed a review of DS cases using the Medline database and we extracted main data regarding imaging findings. CONCLUSIONS: Thanks to our MRS we show new imaging findings never described until today, with a new pathogenetic explanation, since all the causative hypotheses produced during the past years have never found evidence.

Recurrent hemichorea in an adolescent with systemic lupus erythematosus and previous ipsilateral cerebral infarction.

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.

Diabetic striatopathy: an updated overview of current knowledge and future perspectives.

PURPOSE: Diabetic striatopathy (DS) is a rare complication of poorly controlled diabetes mellitus (DM), characterized by hyperglycemia associated with chorea/ballism and characteristic reversible basal ganglia abnormalities on computed tomography (CT) and/or magnetic resonance imaging (MRI). We propose a narrative review of the literature on this topic, currently unknown to most, and about which physicians should be aware. We intend to summarize, critically review, and take to mean the evidence on this disorder, describing its typical features. METHODS: We searched Pubmed for English-language sources using the following keywords in the title and the abstract: diabetic striatopathy, hyperglycemic non-ketotic hemichorea/hemiballism, chorea/hemichorea associated with non-ketotic hyperglycemia, diabetic hemiballism/hemichorea, chorea, hyperglycemia, and basal ganglia syndrome. We collected scientific articles, including case reports, reviews, systematic reviews, and meta-analyses from the years 1975 to 2023. We eliminated duplicate, non-English language or non-related articles. RESULTS: Older Asian women are more frequently affected. Suddenly or insidiously hemichorea/hemiballism, mainly in the limbs, and high blood glucose with elevated HbA1c in the absence of ketone bodies have been observed. Furthermore, CT striatal hyperdensity and T1-weighted MRI hyperintensity have been observed. DS is often a treatable disease following proper hydration and insulin administration. Histopathological findings are variable, and no comprehensive hypothesis explains the atypical cases reported. CONCLUSION: DS is a rare neurological manifestation of DM. If adequately treated, although treatment guidelines are lacking, the prognosis is good and life-threatening complications may occur occasionally. During chorea/hemiballism, we recommend blood glucose and HbA1c evaluation. Further studies are needed to understand the pathogenesis.

Transient Hemichorea-hemiballism Induced by a Combination of Postprandial Hypotension and Severe Stenosis of the Innominate Artery Concomitant with Left Carotid Occlusion.

Hemichorea-hemiballism (HCHB) due to transient ischemic attacks (TIAs) is rare. An 83-year-old woman had repeated episodes of right-sided HCHB for 3 months. Magnetic resonance (MR) angiography demonstrated occlusion of the left carotid and middle cerebral arteries and severe stenosis of the innominate artery, and 24-hour ambulatory blood pressure monitoring showed a blood pressure decrease of >20 mmHg after each meal. We speculated that HCHB developed as TIAs due to hemodynamic failure in the left cerebral hemisphere, caused by a combination of severe stenosis of the innominate artery concomitant with occlusion of the left carotid and middle cerebral arteries as well as postprandial hypotension.

Clinical and imaging analysis of diabetic striatopathy / 实用放射学杂志

Objective To investigate the clinical manifestation and imaging features of diabetic striatopathy(DS).Methods A retrospective analysis was conducted on the clinical,laboratory,and imaging data of 8 patients with DS,which was then summarized in conjunction with relevant literature.Results Random blood glucose(8.39-24.80 mmol/L)and glycated hemoglobin(HbA1c)(9.0%-21.50%)were elevated in 8 patients.One case had positive urine ketone bodies(++),while 7 cases had negative urine ketone bodies(-).Hemichorea was present in 7 cases,while 1 case did not exhibit hemichorea.A total of 7 cases showed unilateral striatum T1WI hyperintensity on MRI,and 8 cases showed iso/hyper-density on CT scans.Following blood glucose control and other related treatments,involuntary movement disappeared in 2 cases,and symptoms improved in 5 cases.Conclusion DS mainly occurs in diabetic patients with poorly controlled blood glucose,presenting with typical clinical manifestation and neuroimaging features.It manifests exclusively in the contralateral striatum of the affected limb.The diagnosis should be based on a combination of clinical,laboratory,and imaging findings to prevent missed or misdiagnoses.

Hyperglycemia-induced hemichorea-hemiballismus syndrome - a systematic review

ABSTRACT Nonketotic hyperglycemia may occur as a cause of chorea in patients with chronic decompensated diabetes. Because it is rare and consequently poorly studied, diagnosis and treatment can be delayed. Therefore, our objective was to summarize clinical and radiological features, as well as treatments performed, from previously reported cases to facilitate adequate management in clinical practice. We searched MEDLINE/PubMed, EMBASE, Cochrane, CINAHL, Web of Science, Scopus, and LILACS databases for studies published before April 23, 2021. We included case reports and case series of adults (aged ≥ 18 years) that described hyperglycemic chorea with measurement of glycated hemoglobin (HbA1c) and cranial magnetic resonance imaging (MRI). Studies were excluded if participants were pregnant women, aged < 18 years, and had no description of chorea and/or physical examination. We found 121 studies that met the inclusion criteria, for a total of 214 cases. The majority of the included studies were published in Asia (67.3%). Most patients were women (65.3%) aged > 65 years (67.3%). Almost all patients had decompensated diabetes upon arrival at the emergency department (97.2%). The most common MRI finding was abnormalities of the basal ganglia (89.2%). There was no difference in patient recovery between treatment with insulin alone and in combination with other medications. Although rare, hyperglycemic chorea is a reversible cause of this syndrome; therefore, hyperglycemia should always be considered in these cases.