Diabetic striatopathy and other acute onset de novo movement disorders in hyperglycemia.

BACKGROUND AND AIMS: Acute onset de novo movement disorder is an increasingly recognized, yet undereported complication of diabetes. Hyperglycemia can give rise to a range of different movement disorders, hemichorea-hemiballism being the commonest. This article delves into the current knowledge about this condition, its diverse presentations, ongoing debates regarding its underlying mechanisms, disparities between clinical and radiological findings, and challenges related to its management. METHODS: PubMed and Google Scholar were searched with the following key terms- "diabetes", "striatopathy", "hyperglycemia", "striatum", "basal ganglia", "movement disorder", "involuntary movement". Case reports, systematic reviews, meta-analysis, and narrative reviews published in English literature related to the topic of interest from January 1, 1950, to October 20, 2023, were retrieved. The references cited in the chosen articles were also examined, and those considered relevant were included in the review. RESULTS: Diabetic striatopathy is the prototype of movement disorders associated with hyperglycemia with its characteristic neuroimaging feature (contralateral striatal hyperdensitity on computed tomography or hyperintensity on T1-weighted magnetic resonance imaging). Risk factors for diabetic striatopathy includes Asian ethnicity, female gender, prolonged poor glycemic control, and concurrent retinopathy. Several hypotheses have been proposed to explain the pathophysiology of movement disorders induced by hyperglycemia. These hypotheses are not mutually exclusive; instead, they represent interconnected pathways contributing to the development of this unique condition. While the most prominent clinical feature of diabetic striatopathy is a movement disorder, its phenotypic expression has been found to extend to other manifestations, including stroke, seizures, and cognitive and behavioral symptoms. Fortunately, the prognosis for diabetic striatopathy is generally excellent, with complete resolution achievable through the use of anti-hyperglycemic therapy alone or in combination with neuroleptic medications. CONCLUSION: Hyperglycemia is the commonest cause of acute onset de novo movement disorders presenting to a range of medical specialists. So, it is of utmost importance that the physicians irrespective of their speciality remain aware of this clinical entity and check blood glucose at presentation before ordering any other investigations. Prompt clinical diagnosis of this condition and implementation of intensive glycemic control can yield significant benefits for patients.

Psychosocial Basis of Human Sufferings and Poverty in Patients with Neurological and Psychiatric Disorders.

Neurological disorders and psychiatric ailments often lead to cognitive disabilities and low attainment of education, pivoting misconceptions, myths, and misbeliefs. Poverty and low educational attainment are intriguingly associated with poor awareness and perception of these diseases that add to the suffering. Poverty goes parallel with a low level of education and is intricately associated with neuropsychiatric ailments, which have the potential to spread transgenerationally. Robust education policies, proper government rules and regulations against the spread of disease-related myths and misconceptions, uplifting medical education in its true sense, voices against consanguinity, and programs to raise scientific perception about diseases can help to throw light at the end of this dark tunnel. In this article, the authors intend to 1) decipher the potential psychosocial basis of human suffering and poverty in patients with neurological and psychiatric disorders, and 2) discuss the apropos way-outs that would potentially mitigate suffering, and alleviate the economic burden and cognitive disabilities of families with neuropsychiatric diseases.

Central Retinal Artery Occlusion in COVID-Associated Mucormycosis.

Introduction: Significant surge of mucormycosis was reported in the Indian Subcontinent during the second wave of the COVID-19 pandemic. COVID-associated mucormycosis (CAM) was defined as the development of features of mucormycosis with prior or current history of COVID-19 infection. Rapid angioinvasion is an important characteristic of mucormycosis. Authors intended to find out the prevalence of retinal arterial occlusion and its association with vascular embolic occlusion elsewhere in the body among CAM patients in this study. Methods: This was an observational study. All consecutive-confirmed cases of mucormycosis (n = 89) and age-/gender-/risk factor-matched controls (n = 324) admitted in the designated COVID center were included in the study. All cases and controls underwent comprehensive ophthalmological, otorhinological, and neurological examinations. All necessary investigations to support the clinical diagnosis were done. Qualitative data were analyzed using the Chi-square test. Quantitative data for comparison of means between the cases and controls were done using unpaired t-test. Results: Twenty-one (23.59%) patients manifested the defined outcome of central retinal artery occlusion (CRAO). Among age-matched control, with similar diabetic status, none had developed the final outcome as defined (P < 0.05). About 90.47% of subjects with CRAO presented with no perception of light vision. Thirteen subjects (61.9%) with the final outcome developed clinical manifestations of stroke during the course of their illness with radiological evidence of watershed infarction (P = 0.001). Orbital debridement was performed in 9 (42.85%) subjects while orbital exenteration was done in 8 (38.09%) subjects. Conclusions: CRAO in CAM patients was found to have aggressive nature turning the eye blind in a very short period of time. CRAO can serve as a harbinger for subsequent development of more debilitating and life-threatening conditions such as stroke among CAM patients.

Corneal Confocal Microscopy Abnormalities in Children and Adolescents With Type 1 Diabetes.

OBJECTIVE: Utility of corneal confocal microscopy (CCM) in children and adolescents with type 1 diabetes mellitus (T1DM) without neuropathic symptoms or signs and minimal abnormality in large and small nerve fiber function tests remains largely undetermined. This study aimed to evaluate the performance of CCM in comparison to thermal detection thresholds (TDT) testing and nerve conduction studies (NCS) for detecting neuropathy in children with T1DM. METHODS: A cohort of children and adolescents with T1DM (n = 51) and healthy controls (n = 50) underwent evaluation for symptoms and signs of neurological deficits, including warm detection threshold, cold detection threshold, vibration perception threshold, NCS, and CCM. RESULTS: Children with T1DM had no or very minimal neuropathic symptoms and deficits based on the Toronto Clinical Neuropathy Score, yet NCS abnormalities were present in 18 (35%), small fiber dysfunction defined by an abnormal TDT was found in 13 (25.5%) and CCM abnormalities were present in 25 (49%). CCM was abnormal in a majority of T1DM children with abnormal TDT (12/13, 92%) and abnormal NCS (16/18, 88%). CCM additionally was able to detect small fiber abnormalities in 13/38 (34%) in T1DM with a normal TDT and in 9/33 (27%) with normal NCS. CONCLUSION: CCM was able to detect corneal nerve loss in children with and without abnormalities in TDT and NCS.

High-resolution vessel wall magnetic resonance imaging in intracranial vasculopathies: an experience from eastern India.

OBJECTIVE: To evaluate the role of high-resolution intracranial vessel wall imaging (HR-IVWI) in differentiation of various intracranial vasculopathies in addition to luminal and clinical imaging in the largest cohort of Indian stroke patients. METHODS: A single-center, cross-sectional study was undertaken recruiting consecutive stroke or TIA patients presenting within a month of onset, with luminal irregularity/narrowing upstream from the stroke territory. The patients were initially classified into TOAST and Chinese ischemic stroke sub-classification (CISS) on the basis of clinical and luminal characteristics and reclassified again following incorporation of HR-IVWI findings. RESULTS: In our cohort of 150 patients, additional use of HR-IVWI led to a 10.7 and 14% change in initial TOAST and CISS classification respectively (p < 0.001). In TOAST classification, 12 "undetermined aetiology" were reclassified into intracranial atherosclerotic disease (ICAD), 1 "undetermined aetiology" into CNS angiitis and 1 "undetermined aetiology" into arterial dissection. Similarly, in CISS 19 "undetermined aetiology" was reclassified into 16 large artery atherosclerosis (LAA) and 3 "other aetiology" consisting of one CNS angiitis, Moyamoya disease (MMD) and arterial dissection each. Two initial classification of MMD by CISS and TOAST were changed into ICAD. The observed change in diagnosis following incorporation of HR-IVWI was proportionately highest in ICAD (LAA) subgroup (TOAST-9.3%, CISS-12%). CONCLUSION: Adjunctive use of HR-IVWI, to clinical and luminal assessment, can significantly improve diagnostic accuracy during evaluation of intracranial vasculopathies, with its greatest utility in diagnosing in ICAD, CNS angiitis and dissection. ADVANCES IN KNOWLEDGE: HR-IVWI allows clearer etiological distinction of intracranial vasculopathies having therapeutic and prognostic implications.

Mitochondrial Calcium Uptake 1 (MICU1) Gene-Related Myopathy with Extrapyramidal Signs: A Clinico-Radiological Case Report from India.

Myopathy with extrapyramidal signs (MPXPS) is a rarely reported entity worldwide, manifesting as a muscular dystrophy with movement disorders. It results from mutations in the mitochondrial calcium uptake 1 (MICU1) gene. We hereby describe a 17-year-old boy who presented with proximal myopathy, calf muscle hypertrophy, and skeletal deformities along with choreiform movements of his upper extremities. Muscle MRI revealed a distinctively early involvement of adductors with sparing of antero-lateral compartment of thigh. This report expands the clinico-radiological presentation and to the best of our knowledge, is the first report of MICU-related MPXPS from India.

Profile of precipitating factors and its implication in 160 Indian patients with Moyamoya angiopathy.

INTRODUCTION: Moyamoya angiopathy (MMA) has been known to manifest with myriad of neurological manifestations, often in association with various precipitating factors. This is the first study to systematically analyze the precipitating triggers to neurological symptoms done on the largest cohort of MMA in India. METHODS: A single-centered, cross-sectional observational study, recruiting 160 patients with consecutive angiographically proven MMA over a period of 5 years (2016-2021), was undertaken to evaluate the profile of immediate precipitating factors in temporal association to the neurological symptoms, along with their clinical and radiological characteristics. SPSS 25 was used for statistical analysis. RESULTS: Among the 160 patients (Adult-85, children-75), precipitating factors were seen in 41.3%, significantly higher in children (52%) than adults (31.8%) (p value: 0.011). The commonest triggers included fever (18.8%), emotional stress (8.1%), heavy exercise and diarrhea (6.3% each). Cold bath triggered MMA symptoms in 1.3%. Fever (p value: 0.008) and persistent crying (p value: 0.010) triggered neurological symptoms more commonly in children than in adults. Amongst MMA patients with precipitating factors, the commonest MMA presentation included cerebral infarction type (37.9%) and TIA (31.8%). The majority of precipitating factors that preceded an ischemic event were BP-lowing ones (54.7%). CONCLUSION: Neurological symptoms of MMA are commonly associated with several precipitating factors, including the lesser known triggers like cold bath. The frequency and profile precipitating factors varies with the age of presentation and type of MMA. It can serve as an early clue to the diagnosis of MMA and its careful avoidance can be largely beneficial in limiting the distressing transient neurological symptoms.

Expanding the Spectrum of Diabetic Striatopathy: Insights from a Case of Hyperglycemia-Induced Propriospinal Myoclonus.

This video abstract delves into the expanded definition of diabetic striatopathy, linked initially to hyperglycemia-induced choreoballism and striatal hyperintensity on magnetic resonance imaging, but now recognized to encompass a broader range of acute onset, non-choreoballistic movement disorders in diabetes mellitus, including tremors, hemifacial spasm, parkinsonism, different types of myoclonus, dystonia, restless leg syndrome, ataxia, and dyskinesias. We report the case of a 45-year-old female patient with type-2 diabetes mellitus who developed propriospinal myoclonus, characterized by painless, involuntary jerky movements of the bilateral lower limbs in a supine position after admission for suspected rhino-orbital mucormycosis. The abnormal movements resolved entirely following the control of her blood glucose levels, suggesting a direct correlation between hyperglycemia and the clinical picture. This case highlights the importance of considering a wide range of differential diagnoses for abnormal lower limb movements in diabetic patients, emphasizing the need for accurate identification of movement semiology, routine bedside capillary blood glucose checks, and prompt hyperglycemia management to resolve such movement disorders effectively.

Cerebral Venous Thrombosis as The Sole Presenting Manifestation of Human Immunodeficiency Virus and Hepatitis B Virus Co-Infection.

Background: Cerebral venous thrombosis (CVT) following either human immunodeficiency virus (HIV) infection or hepatitis B virus (HBV) infection is a very rare condition. Moreover, it has never been reported as the presenting manifestation of HIV and HBV co-infection, even more so when the patient had a normal CD4 count and no demonstrable opportunistic infections. We aimed to report the first case of an adult Indian male, an intravenous drug abuser who developed CVT as the presenting manifestation of HIV-HBV co-infection. Methods: Patient data were obtained from medical records from the Bangur Institute of Neurosciences, Institute of Post Graduate Medical Education and Research & SSKM Hospital, Kolkata, West Bengal, India. Results: A 25-year-old male with a history of intravenous drug abuse and a normal CD4 count developed CVT as the presenting manifestation of HIV-HBV co-infection. His CD4 count was normal, and he had no demonstrable opportunistic infections. He had an uneventful recovery of the condition (CVT) following the institution of conventional anticoagulation therapy alongside anti-retroviral therapy. Conclusion: Whether illicit drug abuse or HIV/HBV infection itself or all in combination led to this thrombotic event cannot be precisely established. Notwithstanding, we recommend serologic testing for HIV and HBV in patients suffering from CVT with high-risk behavior.

Thalassemia and Moyamoya Syndrome: Epidemiology and long-term outcome.

OBJECTIVES: Moyamoya Angiopathy (MMA) is a chronic, progressive intracranial vasculopathy. Unlike Sickle-cell-disease, thalassemia-syndrome has rarely been described in association with MMA. This study was aimed to analyze the demographic, clinical, radiological features and long-term outcome (and possible factors influencing prognosis) of Moyamoya Syndrome (MMS) in the largest cohort of thalassemia-related-to-MMS. MATERIALS & METHODS: A single-centered, observational study with longitudinal follow-up was undertaken for 12 cases of MMS-related-to-thalassemia-syndrome amongst 160 consecutive MMA patients. The baseline demographic, clinical and radiological characteristics were noted; and were longitudinally followed-up to assess disease progression (clinical or radiological). Fifteen previously reported cases of thalassemia and MMA were retrieved by literature search in PubMed and Google-Scholar using keywords "Moyamoya" AND "thalassemia". RESULTS: The mean age at diagnosis of thalassemia-syndrome and diagnosis of MMS were 6.4 ± 6.55 years (mean ± SD) and 10.4 ± 8.68 years respectively in our 12 cases; 3.2 ± 2.25 years and 10.6 ± 6.1 years respectively in the previously reported 15 cases. Cerebral ischemic insult was the predominant brain lesion at base-line, noted in 91.7% of our cases and 80% of the previous cases. The mean hemoglobin, transfusion-dependency and previous splenectomy were seen in 8.7 ± 3.02 gm%, 33.3% and 8.3% of our cases respectively; 7.0 ± 3.04 gm%, 53.9% and 18.2% of previous cases. All our cases were medically managed for mean follow-up of 28.3 ± 13.9 months, none had evidence of angiographic progression, 1 of our 12 cases (8.3%) had new onset neuro-deficit and subsequent mortality, rest 11 of the 12 cases (91.7%) didn't have any appearance of silent cerebral infarction or evidence of progression of brain atrophy. Among the 15 previous cases, 5 out of 9 medically managed cases and 1 revascularized case described no further clinical recurrence. CONCLUSION: Thalassemia-related-MMS may not be so rare. Future development of consensus guidelines in diagnosing and managing cases of MMS-associated-with-thalassemia is of essence.

Persistent "MRI-negative" lupus myelitis-disease presentation, immunological profile and outcome.

Introduction: Myelitis is the least common neuropsychiatric manifestation in systemic lupus erythematosus (SLE). Magnetic resonance imaging (MRI)-negative myelitis is even rarer. Here, we present the largest cohort of MRI-negative lupus myelitis cases to assess their clinical and immunological profiles and outcome. Method: A single-center, observational study conducted over a period of 5 years (2017-2021) was undertaken to evaluate patients with MRI-negative lupus myelitis for the epidemiological, clinical, immunological, and radiological features at baseline and followed up at monthly intervals for a year, and the outcomes were documented. Among the 22 patients that presented with MRI-negative myelopathy (clinical features suggestive of myelopathy without signal changes on spinal-cord MRI [3Tesla], performed serially at the time of presentation and 7 days, 6 weeks, and 3 months after the onset of symptoms), 8 patients had SLE and were included as the study population. Results: In 8 of 22 patients presenting with MRI-negative myelopathy, the etiology was SLE. MRI-negative lupus myelitis had a female preponderance (male: female ratio, 1:7). Mean age at onset of myelopathy was 30.0 ± 8.93 years, reaching nadir at 4.9 ± 4.39 weeks (Median, 3.0; range, 1.25-9.75). Clinically, cervical cord involvement was observed in 75% of patients, and 62.5% had selective tract involvement. The mean double stranded deoxyribonucleic acid, C3, and C4 titers at onset of myelopathy were 376.0 ± 342.88 IU/ml (median, 247.0), 46.1 ± 17.98 mg/dL (median, 47.5), and 7.3 ± 3.55 mg/dL (median, 9.0), respectively, with high SLE disease activity index 2,000 score of 20.6 ± 5.9. Anti-ribosomal P protein, anti-Smith antibody, and anti-ribonuclear protein positivity was observed in 87.5, 75, and 75% of the patients, respectively. On follow-up, improvement of myelopathic features with no or minimal deficit was observed in 5 of the 8 patients (62.5%). None of the patients had recurrence or new neurological deficit over 1-year follow-up. Conclusion: Persistently "MRI-negative" lupus myelitis presents with white matter dysfunction, often with selective tract involvement, in light of high disease activity, which follows a monophasic course with good responsiveness to immunosuppressive therapy. A meticulous clinical evaluation and a low index of suspicion can greatly aid in the diagnosis of this rare clinical condition in lupus.