Unraveling Diabetic Striatopathy: Clinical and Imaging Perspectives.

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.

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.

Botulinum neurotoxin as early treatment in acute-onset lesional hemiballism.

BACKGROUND: Hemiballism (HB) and hemichorea (HC) are the most frequent secondary movement disorders, usually caused by cerebrovascular diseases. In only a minority of cases, these involuntary movements are not self-limited, and they may severely compromise patients' quality of life, so that symptomatic treatments are required. Typical and atypical neuroleptics as well as tetrabenazine are considered therapies of choice. However, anecdotal reports of antiseizures medications and botulinum neurotoxin injection effectiveness have been described. METHODS: We described a case of severely disabling acute-onset lesional HB/HC, where high dosage of first- and second-line therapies was contraindicated due to patient's comorbidities. RESULTS: After botulin neurotoxin (BoNT) injections in his left upper limb muscles (biceps brachii, triceps brachii, teres major, and deltoid), the patient experienced gradual reduction of hyperkinetic movements. The gradual discontinuation of topiramate (TPM) did not worsen the clinical picture. DISCUSSION: The reduction of hyperkinetic movements led to rhabdomyolysis resolution as well as cutaneous injuries healing with renal function improvement, so that the patient was able to be eligible for rehabilitation, which was prevented by HB/HC itself. The clinical improvement was consistent with BoNT pharmacokinetic. The administration of BoNT early after the onset of lesional HB/HC remarkably modified the clinical management and drove toward comorbidities resolution and rehabilitation. CONCLUSION: The present case highlights the effectiveness of unconventional therapeutic options in disabling acute onset lesional HB/HC when first-line therapies are contraindicated. Particularly, this report may encourage BoNT application in the early stage of movement disorder emergencies.

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.

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.

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.

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 as a Delayed Manifestation of Hyperglycemia: A Case Report.

Hemichorea is characterized by involuntary, continuous, non-patterned movements on one side of the body. While it is most commonly caused by strokes, it can also be caused by metabolic derangements such as hyperglycemia. We present two patients who developed hemichorea in the setting of hyperglycemia. Our first patient had a history of uncontrolled diabetes mellitus and developed chorea 2 weeks following a hyperglycemic event. The second patient developed chorea while being on steroids for hip pain and was later diagnosed to have underlying diabetes mellitus. MRI showed hyperintensity in the contralateral lentiform nucleus in both cases. The chorea did not improve despite the correction of the hyperglycemia. Both patients were started on Tetrabenazine with significant improvement. Hyperglycemia-induced hemichorea might be underdiagnosed given the prevalence of diabetes mellitus in the population. Chorea can even be the presenting symptom of diabetes mellitus and in some cases is a delayed manifestation of hyperglycemia.

[Unilateral asterixis after hemiballism in a patient with acute cerebral infarction].

An-88-year-old right-handed female complained of repeated intermittent hemiballism in the right upper and lower extremities. She presented to our hospital with monoparesis and asterixis of the right arm, but not hemiballism. Brain MRI revealed acute disseminated cerebral infarctions in the middle cerebral artery watershed area of the left hemisphere, including the striatum and cortical areas. Occlusion of the left internal carotid artery was also detected. She was diagnosed as acute cerebral infarction and received intravenous infusion, after which her neurological symptoms gradually improved. We presumed that the intermittent hemiballism was related to dysfunction of the motor loop induced by circulatory insufficiency in the left striatum, and that unilateral asterixis might be induced by hemodynamic hypoperfusion in the left frontal lobe. The hemodynamic changes induced by occlusion of the left internal carotid artery might be associated with pathogenesis of these involuntary movements.

Prevalence of diabetic striatopathy and predictive role of glycated hemoglobin level.

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.

Hemiballism, a rare complication of coil embilization.

Thromboembolism is one of the major complications during coil embolization of an aneurysm, which usually causes familiar neurological deficits, such as, weakness, aphasia, etc. We report a rare complication by thromboembolism after coil embolization causing hemiballism. A 69-year-old female presented with unruptured posterior communicating artery aneurysm and was treated by coil embolization. After the procedure, the patient showed global aphasia and right hemiparesis, and there were small multiple, scattered infarctions on the left middle cerebral artery territory. The neurologic deficit subsided after five days, but hemballism occurred thereafter. There was no other medical history to explain the hemiballism. The patient was treated with clonazepam and antiepileptics and the hemiballism subsided at postoperative day 20. We report a case of rare complication, hemiballism, during coil embolization.

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.

Continuous subcutaneous insulin infusion and flash glucose monitoring in diabetic hemiballism-hemichorea.

A 71-year-old man without previous history of diabetes was hospitalized after suffering polyuria for 1 month and involuntary movement of the left arm for 1 week. His random serum glucose was 42.05 mmol/l and his hemoglobin A1C was 14% (129 mmol/mol). His serum osmolarity was normal and his urine ketone was negative. Cerebral CT revealed hyperdensity in the right basal ganglia. The patient was diagnosed with diabetic hemiballism-hemichorea (HH). Intravenous insulin was given and later shifted to continuous subcutaneous insulin infusion. During the hospital stay, insulin titration was guided mainly by flash glucose monitoring (FGM). Finger-prick glucose was occasionally checked to verify the accuracy of the FGM. Rapid correction of severe hyperglycemia was achieved without hypoglycemia. HH resolved within 1 week after euglycemia was achieved. This case emphasized the importance of being alert for HH as the initial presentation of diabetes and neuroimaging negative diabetic HH. In addition, interstitial glucose-monitoring technologies including continuous glucose monitoring and FGM can facilitate inpatient intensive insulin therapy in diabetic HH by avoiding hypoglycemia.

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.

Mirror movements induced by hemiballism due to putamen infarction: a case report and literature review.

Mirror movements (MMs), which are involuntary movements of one limb that synchronously mirror voluntary movements of the contralateral limb, are a relatively uncommon complication of strokes. Here we report what appears to be the first case of putamen infarction manifesting as MMs in one side of the body induced by contralateral hemiballism. MMs and hemiballism were nearly entirely eliminated after one week of clonazepam and haloperidol therapy. During the subsequent one year of standard ischemic stroke prevention measures, no further episodes of involuntary movement occurred. Our case and literature review highlight that acute stroke can manifest as hemiballism and MMs, which should be recognized as soon as possible to ensure timely management.

Hemiballism: Unusual clinical manifestation in three patients with frontoparietal infarct.

The term hemiballism-hemichorea refers to a movement disorder characterized by involuntary movements, often violent, described as uncontrollable jerking, flinging, flailing or kicking, involving proximal muscles of a limb and it is often associated with lesions in the subthalamic nucleus. In this report, we described three cases of hemiballism-hemichorea as the first manifestation of acute ischemic stroke with lesion in the frontoparietal region on brain MRI and no involvement of the subthalamic nucleus. One patient was treated with thrombolysis and recovered within one hour. The other patients recovered within 48 h from symptoms onset. The impairment of the recently described "hyperdirect way", in which the cortical signal reach directly the subthalamic nucleus, may underlie the symptoms. We support, with a clinical point of view, the role of the frontoparietal region in the genesis of the hemiballism-hemichorea. An acute onset of this symptom should lead to think to an acute stroke.