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.

A Novel Adult Case of Recurrent Acute Pancreatitis Caused by Hypercalcemia With Concurrent Manifestation of Posterior Reversible Encephalopathy Syndrome Revealing Undiagnosed Primary Hyperparathyroidism due to an Underlying Parathyroid Adenoma.

Background: Hypercalcemia-induced posterior reversible encephalopathy syndrome (PRES) is a rare entity primarily associated with iatrogenic vitamin D/calcium overdose, malignancy, or, infrequently, primary hyperparathyroidism. Case Report: We present a novel case of an adult male from rural India who experienced recurrent acute pancreatitis caused by hypercalcemia with concurrent manifestation of PRES. Diagnostic evaluation revealed markedly elevated serum calcium levels and parathyroid hormone concentrations, consistent with primary hyperparathyroidism. Imaging studies identified a parathyroid adenoma near the right thyroid lobe, subsequently surgically excised. Discussion: This case underscores the importance of considering primary hyperparathyroidism as an underlying cause of PRES, especially in the absence of acute arterial hypertension or autonomic dysfunction. Early recognition and intervention are essential in mitigating the morbidity and mortality of PRES.

Reported head trauma and odds of acute ischemic stroke or transient ischemic attack: A population-based study (NEDICES).

BACKGROUND: Six previous observational studies have found a higher risk for stroke among traumatic head injury survivors. However, these studies have used record linkage systems, which could lead to underestimating stroke numbers. This study aims to reexamine the relationship between head trauma and the risk of ischemic stroke/transient ischemic attack (TIA) in an older population using data from the Neurological Disorders in Central Spain (NEDICES) study, a population-based study that includes rigorous clinical examinations for patients with suspected neurological diseases. METHODS: We asked participants if they had ever experienced head trauma that was severe enough to warrant a consult with a physician, leading, among others, to visiting the emergency room, hospitalization, or resulting in loss of consciousness. The history of head trauma was evaluated in 196 (5.7%) acute ischemic stroke/TIA cases and 3256 controls in the NEDICES study. RESULTS: Among the final sample of 3452 participants, 354 (10.3%) subjects had a history of head trauma. Twenty-nine (14.8%) of 196 acute ischemic stroke/TIA cases vs. 325 (10.0%) of 3256 controls reported a history of head trauma (p = 0.039). In a regression analysis that adjusted for several variables (age in years, sex, educational level, ever smoker, ever drinker, diabetes mellitus, arterial hypertension, and heart disease), the odds ratio was 1.54 (95% CI = 1.02-2.35, p = 0.042). CONCLUSIONS: The reported head injury was associated with a 54% higher probability of acute ischemic stroke/TIA. More research is needed to confirm these findings, especially using population-based longitudinal studies.

Familial cerebral cavernous malformation presenting with cerebellopontine angle syndrome in a patient with autosomal dominant polycystic kidney disease.

Malformación cavernosa cerebral familiar presentándose como un síndrome del ángulo pontocerebeloso en un paciente con enfermedad renal poliquística autosómica dominante.

Headache as the presenting manifestation of Gorlin-Goltz syndrome with diastematomyelia: A case report.

Gorlin-Goltz syndrome (GGS) is an autosomal dominant multisystemic disease with high penetrance. Headache heralding GGS has been previously reported but without discussing potential sources. We report a patient with headache and a novel association (diastematomyelia), which helped with the diagnosis. A 46-year-old woman presented with persistent holocranial headache. On examination, countless hyperpigmented basal cell nevi over the face, pits over the palmar/plantar surface, and palmar and plantar keratosis were observed. A magnetic resonance imaging (MRI) of the spinal cord revealed diastematomyelia. Diagnosis of GGS was finally made. Headache and diastematomyelia should be included in the clinical picture of GGS.

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.

Downbeat Nystagmus as a Presenting Manifestation of Neurolisteriosis in a Pregnant Woman.

Listeria monocytogenes has tropism towards two immunologically "privileged" sites, the fetoplacental unit in pregnant women and the central nervous system (neurolisteriosis) in immunocompromised individuals. We report a case of neurolisteriosis in a previously asymptomatic pregnant woman from rural West Bengal, India, who presented with a subacute onset febrile illness with features of rhombencephalitis and a predominantly midline-cerebellopathy (slow and dysmetric saccades, florid downbeat nystagmus, horizontal nystagmus, and ataxia). With timely detection and the institution of prolonged intravenous antibiotic therapy, both the mother and the fetus were saved uneventfully.

Effective treatment of choreaballism due to an MT-CYB variant with haloperidol, tetrabenazine, and antioxidants.

Hypokinetic and hyperkinetic movement disorders are a common phenotypic feature of mitochondrial disorders. Choreaballism has been reported particularly in patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome and in maternally inherited diabetes and deafness syndrome. The pathophysiological basis of movement disorders in mitochondrial disorders is the involvement of the basal ganglia or the midbrain. Haloperidol and mitochondrial cocktails have proven beneficial in some of these cases. Here we present another patient with mitochondrial choreaballism who benefited significantly from symptomatic therapy. The patient is a 14-year-old male with a history of hypoacusis, ptosis, and focal tonic-clonic seizures of the upper/lower limbs on either side since childhood. Since this time he has also developed occasional, abnormal involuntary limb movements, choreaballism, facial grimacing, carpopedal spasms, and abnormal lip sensations. He was diagnosed with a non-syndromic mitochondrial disorder after detection of the variant m.15043G > A in MT-CYB. Seizures have been successfully treated with lamotrigine. Hypocalcemia was treated with intravenous calcium. For hypoparathyroidism calcitriol was given. Choreaballism was treated with haloperidol and tetrabenazine. In addition, he received coenzyme Q10, L-carnitine, thiamine, riboflavin, alpha-lipoic acid, biotin, vitamin-C, vitamin-E, and creatine-monohydrate. With this therapy, the choreaballism disappeared completely. This case shows that mitochondrial disorders can manifest with cognitive impairment, seizures, movement disorder, hypoacusis, endocrinopathy, cardiomyopathy, neuropathy, and myopathy, that choreaballism can be a phenotypic feature of multisystem mitochondrial disorders, and that choreaballism favorably responds to haloperidol, tetrabenazine, and possibly to a cocktail of antioxidants, cofactors, and vitamins.

Association between cumulative smoking exposure and cognitive decline in non-demented older adults: NEDICES study.

Whether cumulative smoking exposure is associated with cognitive decline among older adults remains unresolved. To address this question, we used data from the Neurological Disorders in Central Spain (NEDICES) cohort study, in which 2624 older adults were evaluated at two-time points separated by three years. A 37-item version of the Mini-Mental State Examination (MMSE-37) was administered at two visits to assess cognitive change. Regarding smoking exposure, we calculated an individual baseline score based on pack-years (i.e., packs of cigarettes smoked per day multiplied by years of smoking) in current and former smokers. Thus, smoking exposure was categorized into tertiles (low: < 19.0, medium: 19.0-47.0, and high: > 47.0). We used multivariable generalized estimating equation models to assess associations between pack-years and smoking status with 37-MMSE total score change from baseline to follow-up. The MMSE-37 total score had a decline of 1.05 points (confidence interval [CI] 95% 0.62 to 1.48) in the lower tertile of pack-years, 1.16 (CI 95% 0.70 to 1.62) in the middle tertile and 1.17 (CI 95% 0.70 to 1.65) in the higher tertile compared to never smokers, after adjusting for several demographic and clinical variables. The same occurred with smoking status, i.e., a decline of 1.33 (CI 95% 0.87 to 1.79) in current smokers and 1.01 (CI 95% 0.63 to 1.40) in former smokers. Our study provides evidence of the cumulative effect of smoking on cognition in older adults. Using a prospective population-based design, we demonstrated that cumulative smoking exposure was associated with cognitive decline in non-demented older adults. More population-based evidence is required to elucidate this association in older adults without dementia.

The Effects of SARS-CoV-2 Infection on the Cognitive Functioning of Patients with Pre-Existing Dementia.

Background: Cognitive postscripts of COVID-19, codenamed as 'cognitive COVID' or 'brain fog,' characterized by multidomain cognitive impairments, are now being reckoned as the most devastating sequelae of COVID-19. However, the impact on the already demented brain has not been studied. Objective: We aimed to assess the cognitive functioning and neuroimaging following SARS-CoV-2 infection in patients with pre-existing dementia. Methods: Fourteen COVID-19 survivors with pre-existing dementia (four with Alzheimer's disease, five with vascular dementia, three with Parkinson's disease dementia, and two with the behavioral variant of frontotemporal dementia) were recruited. All these patients had detailed cognitive and neuroimaging evaluations within three months before suffering from COVID-19 and one year later. Results: Of the 14 patients, ten required hospitalization. All developed or increased white matter hyperintensities that mimicked multiple sclerosis and small vessel disease. There was a significant increase in fatigue (p = 0.001) and depression (p = 0.016) scores following COVID-19. The mean Frontal Assessment Battery (p < 0.001) and Addenbrooke's Cognitive Examination (p = 0.001) scores also significantly worsened. Conclusion: The rapid progression of dementia, the addition of further impairments/deterioration of cognitive abilities, and the increase or new appearance of white matter lesion burden suggest that previously compromised brains have little defense to withstand a new insult (i.e., 'second hit' like infection/dysregulated immune response, and inflammation). 'Brain fog' is an ambiguous terminology without specific attribution to the spectrum of post-COVID-19 cognitive sequelae. We propose a new codename, i.e. 'FADE-IN MEMORY' (i.e., Fatigue, decreased Fluency, Attention deficit, Depression, Executive dysfunction, slowed INformation processing speed, and subcortical MEMORY impairment).