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.

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.

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.

Dance of the Sugar: Two Case Reports of Chorea Associated With Nonketotic Hyperglycemia.

Hyperglycemia-induced chorea/ballism is a rare clinical entity that often occurs in the setting of nonketotic hyperglycemia due to poor glycemic control in elderly patients with a diagnosis of type 2 diabetes mellitus (DM). This condition is typically characterized by hemichorea/hemiballism and unique brain imaging findings in the contralateral basal ganglia. Treatment involves the correction of blood glucose, and most cases resolve without additional therapy. Here we report two cases of this condition in which patients with type 2 DM presented with nonketotic hyperglycemia and typical neuroimaging findings. Although rare, clinicians should be aware of this condition in patients with diabetes who present with sudden abnormal movements since its prompt diagnosis and treatment often lead to a favorable outcome.

Hemichorea-Hemiballism in A 50-Year-Old Man With Newly Diagnosed Diabetes.

OBJECTIVE: Hyperglycemia may cause acute central nervous system dysfunction manifesting as agonizing involuntary movements due to insult to the basal ganglia. We report a case of hemichorea-hemiballism (HCHB) in a patient with diabetes. METHOD: Clinical assessment of the patient was performed, along with laboratory tests and brain imaging. RESULTS: The patient was a 50-year-old man with newly detected diabetes with persistent involuntary movement of the right upper and lower limbs for few weeks. The involuntary movement was nonrhythmic, nonpatterned, purposeless, and often jerky with variable amplitude and frequency, sometimes wild and flailing in the form of hemichorea with a ballistic component (HCHB). He had a history of poor compliance to prescribed oral antidiabetic drugs. At presentation, although he was hemodynamically stable, random capillary blood glucose level was 18 mmol/L and glycated hemoglobin A1 level was 15.1% (141.5 mmol/mol). Clinical examination did not reveal any focal deficit or positive Babinski sign. There was a hyperintensity in the left basal ganglia region in T1-weighted magnetic resonance imaging (MRI) of the brain, which was iso-to-hyperintense in T2-weighted image and fluid-attenuated inversion recovery sequence. There was no restriction of diffusion on the diffusion-weighted image or blooming on gradient echo sequences, indicating absence of infarction or hemorrhage. Control of hyperglycemia resulted in disappearance of the involuntary movement within 1 month. CONCLUSION: While there are many differential diagnoses for HCHB, the clinical scenario suggests hyperglycemia as the underlying cause in this patient. This case reiterates that multiple central nervous system manifestations may be attributable to diabetes.

Clinical and Neuroimaging Features in a Patient with Non-Ketotic Hyperglycemia.

Hemichorea-hemiballism (HC-HB) is a spectrum of involuntary flinging and flailing, non-patterned, irregular movements involving one side of the body. A rare dysfunction of glucose metabolism leading to a state of non-ketotic hyperglycemia (NKH) is thought to be a cause of these symptoms. In previous case studies, imaging findings have been in the basal ganglia as hyperintense lesions on magnetic resonance imaging (MRI) or hyperdensities on computerized tomography (CT). This case is unique due to abnormal findings in the MRI T2/fluid-attenuated inversion recovery (FLAIR) sequence in areas not previously reported-the thalamus and midbrain/pons. As in other NKH cases, the patient improved both clinically and radiologically. In patients with uncontrolled diabetes and abnormal movements, monitoring of blood glucose is imperative as it can lead to recognition of HC-HB. Other etiologies, including stroke, neoplasm, demyelination, and inflammatory processes, have uncertain prognoses with unfavorable outcomes. The prognosis for NKH is usually favorable, and thus important to identify.

Clinical features of hemichoreahemiballism: A stroke-related movement disorder.

We examined pathogenesis and clinical features of three hemichorea-hemiballism (HCHB) cases. We studied their age, magnetic resonance imaging results, vascular risk factors, management, and outcomes. One man and two women (aged 74-86 years) demonstrated acute onset of HCHB, lasting for at least several months. Patients had one or more vascular risk factors, including hypertension and diabetes. All patients presented subacute or old infarction in the basal ganglia with contralateral symptoms. We administered clonazepam (0.5-1 mg/day), haloperidol (0.375-0.75 mg/day), or both as necessary and observed symptom-control. Vascular lesions in the basal ganglia were a contributing factor. Symptoms were controlled using pharmacotherapy with gamma-aminobutyric acid-agonist (clonazepam) or anti-dopaminergic (haloperidol) medication.

Hemichorea-Hemiballism Syndrome Caused by Nonketotic Hyperglycemia in a Newly Diagnosed Diabetes Mellitus Type 2 Patient with Euglycemia at Presentation

@#INTRODUCTION: Nonketotic hyperglycemia among type 2 diabetic patients have recently been documented to cause the rare movement disorder called Hemichorea-hemiballism syndrome which is a hyperkinetic movement disorder presenting as a continuous, non-patterned, involuntary movements caused by a basal ganglia dysfunction. METHODS: A 76-year-old male with a known history of hypertension and no history of stroke and diabetes presented with a 10-day history of increasingly persistent involuntary movements of the right extremities. On admission, the patient was conscious with stable vital signs and unremarkable neurologic findings except for the involuntary flailing movements of the right extremities. Diagnostic testing revealed first documentation of hyperglycemia with brain MRI changes on T1 hyperintensity signals on the basal ganglia and T2/FLAIR weighted imaging showing mixed hypointense and hyperintense signals which is a classical MRI finding in patients with HC-HB syndrome caused by nonketotic hyperglycemia. The patient was treated for diabetes and was maintained on anti-dopaminergic medications for the uncontrollable involuntary movements. After five months, resolution of the hemiballism-hemichorea syndrome was noted after appropriate treatment. CONCLUSION: This case report highlights hemichoreahemiballism syndrome in a newly diagnosed type 2 diabetic patient who had normal glucose levels at presentation. The prompt recognition and correction of uncontrolled newly diagnosed diabetes and administration of anti-dopamine agents lead to a rapid improvement of symptoms, less neurologic sequelae and an overall favorable prognosis.

Pure Cortical Stroke Causing Hemichorea-Hemiballismus.

BACKGROUND: Movement disorders including hemichorea-hemiballism as the initial presentation of an acute ischemic stroke are uncommon. Structures outside of the deep subcortical areas such as the subthalamic nucleus or basal ganglia are rarely involved. CASE REPORT: We report a case of a 72-year-old man with vascular risk factors who presented with acute onset right-sided hemichorea-hemiballism. Metabolic-, infectious-, and toxic-related conditions were ruled out, his EEG was without epileptiform changes. An MRI confirmed an acute ischemic stroke in the parieto-occipital region without any subcortical structures involved. Atrial Fibrillation was later discovered during his hospitalization and was treated appropriately. CONCLUSIONS: Although rare, strokes outside of the subthalamic nucleus can result in hemichorea-hemiballism.

Diabetic Hemichorea-hemiballism after Prompt Improvement in Hyperglycemia.

We herein report a case of hemichorea-hemiballism in an 85-year-old man diagnosed with diabetes at 76 years of age. After a one-year interruption in treatment, he was treated with a low-calorie diet, linagliptin, and nateglinide. Over 51 days, his HbA1c level decreased from 15.8% to 7.7%. After a prompt improvement in his hyperglycemia, he began experiencing involuntary movements in the right upper and lower extremities. T1-weighted magnetic resonance imaging showed a high signal intensity in the left lens nucleus. The patient was diagnosed with diabetic hemichorea-hemiballism and received haloperidol (1 mg/day) as treatment.

Irreversible Hemichorea-Hemiballism in a Case of Nonketotic Hyperglycemia Presenting as the Initial Manifestation of Diabetes Mellitus.

BACKGROUND: Hemichorea-hemiballism (HCHB) is a hyperkinetic movement disorder with features of both chorea and ballism occurring on the same side. CASE REPORT: We present a case of HCHB due to nonketotic hyperglycemia (NKH) that was the initial presentation of diabetes and was irreversible clinically even after 6 months of optimal blood sugar control. DISCUSSION: Although HCHB due to hyperglycemia is a potentially reversible condition in the majority of patients, prolonged uncontrolled hyperglycemia may cause ischemic insult and persistent symptoms. Hyperglycemia should always be kept in the list of differentials while dealing with patients who are newly diagnosed with HCHB.

Movement disorders and stroke.

Stroke may be associated with different types of movement disorders, such as hyperkinetic syndromes (hemichorea-hemiballism, unilateral asterixis, limb-shaking, dystonia, tremor, myoclonus) and hypokinetic syndromes (especially vascular parkinsonism). However, movement disorders are rare and transient in acute stroke and, as a permanent consequence, are more often delayed. While ischemic and hemorrhagic strokes can happen at any level of the frontal-subcortical motor system, they can be explained most of the time by a dysfunction in the basal ganglia motor circuit. However, only brain MRI allows the involved structure(s) to be precisely located, and each syndrome is specific to the type of lesion. Treatment is above all symptomatic. Only limb-shaking syndrome requires urgent surgical treatment because of the low-perfusion hemodynamic state. The functional prognosis depends on the type of movement disorder.

Reply #2 to: Glycemic Choreoballism.

In Response To: Lee D, Ahn, TB. Glycemic choreoballism. Tremor Other Hyperkinet Mov. 2016; 6. doi: 10.7916/D8QJ7HNF Original Article: Roy U, Das SK, Mukherjee A, et al. Irreversible hemichoreahemiballism in a case of nonketotic hyperglycemia presenting as the initial manifestation of diabetes mellitus. Tremor Other Hyperkinet Mov. 2016; 6. doi: 10.7916/D8QZ2B3F.

A case of Hemichorea-Hemiballism Induced by Acute Infarction of Bilateral Corona Radiata and Cortex.

Movement disorders are a recognized complication of stroke. Here we present a case of hemichorea-hemiballism (HCHB) after stroke. Basal ganglia and thalamus are typically recognized as sites responsible for HCHB. The MRI scan showed acute infarction which was unexpectedly present in both sides of corona radiate and cortex, but not in basal ganglia. This cortical HCHB could have evolved due to hypoperfusion of basal ganglia undetectable at the MRI scan or due to interruption of excitatory connections from the cerebral cortex to basal ganglia.

Hemichorea-hemiballism in a nonketotic diabetic patient.

Hemichorea-hemiballism can be the solely presentation of a wide range of non-neurological clinical pictures, such as metabolic or hydro-electrolyte derange-ments. Hemichorea-hemiballism as the first presentation of type 2 diabetes mellitus has been described. The case depicted herein reinforces this association highlighting that especially in elder patients with new-ly diagnosed hemichorea-hemiballism, non-ketotic hyperglycemia should promptly be recognized.

Two Cases of Hemichorea Associated with Nonketotic Hyperglycemia in Type 2 Diabetes Mellitus Patient

Hemichorea-hemiballism is a rare complication of nonketotic hyperglycemia in type 2 diabetes mellitus (T2DM). It can be complicated in long-standing type 1 diabetes mellitus or T2DM, and has been described as a presenting symptom of new-onset diabetes. Rapid correction of diabetic ketoacidosis may also cause the delayed hemichorea. Although hyperglycemic hemiballism rarely causes generalized chorea due to bilateral basal ganglia involvement, patients typically present with hemichorea developing over days to months in the setting of elevated serum glucose. On T1-weighted brain magnetic resonance imaging and computed tomography scan a high signal intensity lesion at the basal ganglia is characteristic. After the correction of hyperglycemia, the movements generally disappear within hours, but atypical cases with delayed onset after the resolution of hyperglycemia, unremitting severe movements, and late recurrence are also reported. We report two cases of female T2DM patients who presented with hemichorea. One patient presented with hemichorea in nonketotic hyperglycemia, and the other with delayed onset hemichorea after the resolution of hyperglycemia.

Hemichorea-hemiballism syndrome: A look through susceptibility weighted imaging.

Hemichorea-hemiballism syndrome (HCHB) is a relatively rare cause of unilateral chorea in diabetic patients and is due to non ketotoic hyperglycaemia. Characteristic magnetic resonance (MR) findings include T1 hyperintensity in the contralateral putamen without any significant signal alteration on other conventional MR sequences. We report susceptibility weighted imaging (SWI) findings in a case of HCHB syndrome.

Lesions in basal ganglia in a patient with involuntary movements as a first sign of diabetes - case report and review of the literature.

We present a case of unilateral hyperdensity of the lentiform and caudate nucleus on CT with hyperintesity on T1-weighted images on MRI in a 71-year-old woman with hemichorea-hemiballism and recently diagnosed diabetes.