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.

Late-onset Chorea with a Rare but Identifiable Cause.

Chorea is a very commonly encountered movement disorder; it has various etiologies, and it can have autoimmune, vascular, degenerative, or paraneoplastic etiology. Our patient had acute onset chorea and a strong history of smoking, which made us suspect first vascular followed by paraneoplastic cause. After ruling out common vascular and metabolic causes, his whole body positron emission tomography (PET) scan revealed a mass in the right upper lobe, a biopsy revealed a small cell carcinoma lung and a paraneoplastic panel showed antibodies positive for collapsin response mediator protein 5 antigen (CRMP-5/CV2); the patient was started on immunomodulation, chemotherapy with the variable response, he succumbed to a cardiac event after treatment.

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.

Chorea and polycythaemia vera.

We report two patients with chorea associated with polycythaemia vera, in whom the haematocrit and haemoglobin were within the reference range. Polycythaemia vera is potentially easily treatable and so is important to consider in people developing late-onset chorea.

Investigation of the factors associated with hemichorea/hemiballismus in post-stroke patients.

Classical knowledge highlights the role of lesions of the subthalamic nuclei (STN) in the pathophysiology of hemichorea/hemiballismus (HH). However, the published reports indicate various other lesion regions in the majority of post-stroke cases with HH. Ergo, we aimed to investigate the significance of the lesion site and clinical features for developing HH in post-stroke patients. Overall, we retrospectively scanned all the patients with stroke who were hospitalized between 01/06/2022 and 31/07/2022 in our neurology clinic. The data regarding the demographic features, comorbidities, stroke etiologies, and laboratory findings, including serum glucose and HBA1C were retrospectively recruited using the electronic-based medical record system. The cranial magnetic resonance imaging (MRI) and computed tomography images have been systematically evaluated for the presence of lesions in localizations that are previously associated with HH. We conducted comparative analyses between patients with and without HH to reveal the discrepancies between groups. The logistic regression analyses were also performed to reveal the predictive values of some features. Overall, the data of 124 post-stroke patients were analyzed. The mean age was 67.9 ± 12.4 years (F/M = 57/67). Six patients were determined to develop HH. The comparative analyses between patients with and without HH revealed that the mean age tended to be higher in the HH group (p = 0.08) and caudate nucleus involvement was more common in the HH group (p = 0.005). Besides cortical involvement was absent in all subjects developing HH. The logistic regression model revealed the presence of a caudate lesion and advanced age as factors associated with HH. We found that the caudate lesion was a crucial determinant of the occurrence of HH in post-stroke patients. With the significance of the other factors of increased age and cortical sparring, we observed differences in the HH group may be investigated also in future-related studies on larger groups.

Diagnostic Uncertainties: Chorea.

Chorea is a hyperkinetic movement disorder with a multitude of potential etiologies, both acquired and inherited. Although the differential diagnosis for new-onset chorea is extensive, there are often clues in the history, exam, and basic testing that can help to narrow the options. Evaluation for treatable or reversible causes should take priority, as rapid diagnosis can lead to more favorable outcomes. While Huntington's disease is most common genetic cause of chorea, multiple phenocopies also exist and should be considered if Huntington gene testing is negative. The decision of what additional genetic testing to pursue should be based on both clinical and epidemiological factors. The following review provides an overview of the many possible etiologies as well as a practical approach for a patient presenting with new-onset chorea.

Hemichorea secondary to isolated temporal infarction with severe middle cerebral artery stenosis: a case report and review of literature.

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

Cognitive decline as the main manifestation of diabetic striatal disease but without involuntary movements: a case report.

Diabetic striatopathy (DS) is a rare central nervous system complication of diabetes mellitus, characterized mainly by non-ketotic hyperglycemia and lateralized involuntary movements. Patients with diabetic striatopathy manifested solely by subacute cognitive decline were rarely reported. In this paper, we report a patient with DS who presented solely with subacute cognitive decline without involuntary movements, and cranial CT showed bilateral high density in the basal ganglia. In contrast, SWI showed microhemorrhages in the right caudate nucleus head. After one week of treatment, including glycemic control, the patient showed significant improvement in cognitive function, while a repeat cranial CT showed improved hyperdensity in the right basal ganglia region. 1 month later, at telephone follow-up, the patient's symptoms did not recur.

Diabetic striatopathy: a case report.

Diabetic striatopathy, a rare condition also known as hyperglycemic nonketotic hemichorea, is characterized by chorea or hemiballismus and distinctive basal ganglia abnormalities visible on neuroimaging. We present the case of an 86-year-old woman with diabetic striatopathy exhibiting hemichorea. She had a history of poorly controlled type 2 diabetes and presented with involuntary movements of her left limb along with facial expressions suggestive of chorea. Laboratory tests confirmed hyperglycemia, with an elevated hemoglobin A1c level. Neuroimaging revealed T1-hyperintensity in the right basal ganglia. The patient was diagnosed with diabetic striatopathy and responded well to intensive insulin therapy with a rapid resolution of symptoms.

Stroke-like presentation of autoimmune chorea with positive anti-Yo and anti-MOG antibodies: a case report.

With the in-depth study of autoimmune encephalitis, more and more antibody combinations and clinical manifestations appear in our sights, enriching the spectrum of autoimmune encephalitis. Here, we report a case of a 58-year-old male patient with sudden involuntary movement of the left limb. The brain MRI was normal. CSF analysis showed slightly elevated protein (548.38 mg/L) and normal cell count(1.00 10^6/L). No tumors were detected by the whole-body PET-CT. Positive anti-Yo and anti-MOG antibodies were found in the blood. So we considered the diagnosis of autoimmune chorea with positive anti-Yo and anti-MOG antibodies, after immunoglobulin shock and methylprednisolone shock therapy were used, the patient's involuntary movement gradually disappeared. This is the first case of autoimmune encephalitis with both anti-Yo and anti-MOG antibodies, and stroke-like chorea is also rare. This case enriches the clinical presentation of double antibody-associated encephalitis.

Nonketotic Hyperglycemic Hemichorea in an Elderly Male: A Case Report.

BACKGROUND: Nonketotic hyperglycemic hemichorea is a rare complication of diabetes mellitus seen in the emergency department. It is most commonly reported in elderly women, predominantly of Asian race, with poorly controlled diabetes mellitus. Patients present with a triad of nonketotic hyperglycemia, hemichorea, and contralateral basal ganglia abnormality on imaging. Its exact pathophysiology is still not known. However, it has a very good prognosis with early diagnosis and treatment. CASE REPORT: We report a case of hemichorea involving the right upper and lower limbs due to nonketotic hyperglycemia. The patient's symptoms resolved after normalization of blood glucose. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Nonketotic hyperglycemic hemichorea should be included in the differential of a patient presenting with chorea and high blood glucose levels. It has an excellent prognosis with both symptoms and imaging abnormalities typically resolving completely with restoration of normoglycemia.

Antiphospholipid-related chorea.

Chorea can be associated with autoimmune diseases such as antiphospholipid syndrome and has been associated with the isolated presence of antiphospholipid antibodies (aPL). Chorea is a rare neurological manifestation of antiphospholipid syndrome. The pathophysiological mechanisms underlying aPL-related chorea are still debated. One postulated mechanism is aPL or other autoantibody binding to brain-blood vessel endothelium, resulting in endothelial dysfunction secondary to a proinflammatory cascade, with sequalae of inflammation and local microthrombosis. Another postulated mechanism considers immune-mediated attack (aPL or antibasal ganglia antibodies) against specific basal ganglia epitopes. Here, we report a patient with isolated aPL-related chorea that followed a relapsing-remitting course. We highlight the role of brain metabolic imaging with fluorodeoxy glucose positron-emission tomography in the diagnostic workup of chorea and the challenges in the practical management of aPL-related chorea with symptomatic treatments.

Is diabetic striatopathy the culprit of seizures in a patient with ketotic hyperglycemia-induced hemichorea-hemiballismus?

BACKGROUND: Diabetic striatopathy is a rare neurological manifestation of nonketotic hyperglycemia that presents with contralateral hemichorea-hemiballismus. Presentation with concurrent seizures is rarely reported. CLINICAL PRESENTATION: We report a case of diabetic striatopathy presenting with focal and generalized tonic-clonic seizures (GTCS) with right hemichorea-hemiballismus induced by a ketotic hyperglycemic state. Head MRI showed high T1-weighted signal intensity in the left lentiform nucleus with no significant diffusion restriction or postcontrast enhancement. The patient's condition gradually improved, with seizure control on AEDs. Hemichorea-hemiballismus significantly improved with adequate blood sugar control and resolved with low-dose haloperidol. CONCLUSIONS: Diabetic striatopathy presenting with hemichorea-hemiballismus and concurrent GTCS has been reported previously in two cases; however, it has never been reported in ketotic hyperglycemia. To the best of our knowledge, we herein report the first case report of focal and generalized seizures in a ketotic hyperglycemic state and mesial temporal sclerosis.

Treatment-resistant diabetic chorea manifesting with psychiatric symptoms: a case report.

We report a case of a 69-year-old man with treatment-resistant diabetic chorea presenting psychiatric symptoms. The right chorea lasted for 3 months and was refractory to control of diabetes mellitus or administration of haloperidol and benzodiazepines. Only administration of tiapride was efficacious. Magnetic resonance spectrometry and dopamine transporter-single photon emission computed tomography suggested that sustained ischemia at the striatum may lead to impaired expression of dopamine transporters, thereby resulting in deterioration in the indirect pathway. Tiapride inhibited dopamine D2 receptors, thereby restoring the function of the indirect pathway and resulting in improvement of diabetic chorea.

Successful Rituximab Therapy for Pediatric Antiphospholipid-Related Chorea: A Case Report and Review of the Literature.

Chorea is considered a nonthrombotic manifestation of the antiphospholipid syndrome, often preceding thrombotic events in children. It can be present in up to 5% of pediatric patients with antiphospholipid syndrome. Immunomodulatory treatment regimens seem to be successful in these patients, emphasizing the underlying immunological etiology. Corticosteroids are considered first-line treatment, but chorea tends to be therapy-resistant and guidelines about second-line therapy in children are solely based on small case studies. We present a case of a therapy-resistant chorea, successfully treated with rituximab. Furthermore, we give an overview of the existing literature concerning rituximab for the treatment of chorea in children. Our findings indicate that rituximab can be considered a safe option to treat antiphospholipid syndrome-related chorea in children.

Clinical and imaging features of diabetic striatopathy: report of 6 cases and literature review.

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.

Neuroimaging of Diabetic Striatopathy: More Questions than Answers.

BACKGROUND: Diabetic striatopathy (DS), coined as a generic term, has been defined as a hyperglycemic condition associated with either one of the two following conditions: chorea/ballism or striatal hyperdensity on computed tomography or striatal hyperintensity on T1-weighted magnetic resonance imaging. This review highlights those "gray areas," which need further exploration to understand better hyperglycemia-induced striatal changes and diverse movement disorder phenotypes associated with these changes. RESULTS AND DISCUSSION: We searched in PubMed and Google Scholar the terms "diabetes mellitus," "movement disorders," "diabetic striatopathy," "chorea," "hemichorea," "ballism," "hemichorea-hemiballism," and "neuroradiology" in various combinations (time range from 1980 to March 2022). We selected the publications about our topic of discussion. SUMMARY: Hemichorea-hemiballismus is the most commonly associated movement disorder in DS, and the putamen is the most frequently affected anatomical region. The exact pathophysiological mechanisms remain elusive. Clinical-radiological discordance is not rare. Complete reversal of symptoms with the resolution of the imaging findings is the most prevalent outcome in patients with DS. Dramatic improvement of chorea can be achieved by either insulin monotherapy or combination therapy of insulin and D2-blocker or, in some cases, even spontaneously. CONCLUSION: The term "diabetic striatopathy" is ambiguous and controversial. Pathological mechanisms behind clinical-radiological discordance in hyperglycemia-induced striatopathy need further exploration through well-designed studies. We propose a classification of DS that includes symptomatic DS (striatal neuroimaging lesions in association with a clinically evident movement disorder and hyperglycemia), clinically isolated DS (clinically evident movement disorders without striatal changes in neuroimaging), and radiologically isolated DS.

Pimozide and pancreatic cancer in diabetic chorea: a case report.

PURPOSE/AIM: 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. CASE REPORT: 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. CONCLUSION: 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.

An unexpected cause of chorea in an adolescent girl: systemic lupus erythematosus.

Involuntary movement disorders are rare in childhood. Hyperkinetic movement disorders including chorea stand as the leading cause. Although Sydenham chorea is the major diagnosis in most children and adolescents, appropriate differential diagnosis is fundamental for a final decision. A detailed and careful history as well as physical examination is the principal proceeding for accurate diagnosis. Herein, we report on an adolescent girl who was admitted to our hospital with chorea and subsequently diagnosed with systemic lupus erythematosus (SLE). Accompanying joint complaints in the patient's history, including growth retardation noticed during a physical examination and bicytopenia recognized in laboratory evaluation, increased the suspicion of SLE rather than Sydenham chorea in the patient. Central nervous system involvement defined as neuropsychiatric lupus presents wide clinical findings varying from stroke and seizures to psychosis and cognitive dysfunction. Although disease activity, persistently positive anticardiolipin antibodies, and lupus anticoagulant positivity are reported to be the most important risk factors in neuropsychiatric lupus, they are not always directly correlated. We present this patient in order to draw attention to the importance of physical examination and history in the differential diagnosis of chorea in childhood.

Huntington's disease: diagnosis and management.

Huntington's disease (HD) is an inherited neurodegenerative disease characterised by neuropsychiatric symptoms, a movement disorder (most commonly choreiform) and progressive cognitive impairment. The diagnosis is usually confirmed through identification of an increased CAG repeat length in the huntingtin gene in a patient with clinical features of the condition. Though diagnosis is usually straightforward, unusual presentations can occur, and it can be difficult to know when someone has transitioned from being an asymptomatic carrier into the disease state. This has become increasingly important recently, with several putative disease-modifying therapies entering trials. A growing number of conditions can mimic HD, including rare genetic causes, which must be considered in the event of a negative HD genetic test. Patients are best managed in specialist multidisciplinary clinics, including when considering genetic testing. Current treatments are symptomatic, and largely directed at the chorea and neurobehavioural problems, although supporting trial evidence for these is often limited.