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.

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.

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.

Behavioral and psychological symptoms in neurodegenerative dementias: harbinger, follower, or constant collateral?

Neurodegenerative dementias such as the behavioral variant of frontotemporal dementia, Alzheimer's disease, and Parkinson's disease dementia are linked to various behavioral and psychological abnormalities. Whether these abnormalities precede, coincide or follow the onset of cognitive symptoms is still controversial in existing literature, with trajectories available so far dependent on types of dementia. The authors aim to review the different kinds of premorbid behavioral symptoms/personality traits associated with an increased risk of developing specific types of neurodegenerative dementia. Neuroticism has been associated with an increased risk of Alzheimer's disease and late-onset behavioral abnormalities with the behavioral variant of frontotemporal dementia. The presence of obsessive-compulsive spectrum disorders in Parkinson's disease dementia is also not rare. Analyzing this evidence, we propose "behavioral biomarkers" as neuroticism in Alzheimer's disease, late-onset behavioral abnormalities in behavioral variant of frontotemporal dementia, and obsessive-compulsive traits in Parkinson's disease dementia. These noninvasive behavioral biomarkers will be of immense help, particularly in developing countries, and will prevent the need for costlier investigations and aid in therapeutic strategies.

Alien Limb Phenomenon After Diffuse Corpus Callosum Ischemic Stroke.

The alien limb is a phenomenon characterized by a cluster of clinical features wherein the limb behaves autonomously and as separated from a person's identity. We herein report a 36-year-old Indian female with multiple comorbidities who presented with recurrent episodes of limb-shaking transient ischemic attacks for 1 year, followed by left-sided hemiplegia. During recovering, the patient noticed a feeling that as if her left hand did not belong to herself and acted autonomously (alien limb phenomenon) along with visuospatial deficits. Magnetic resonance imaging of the brain revealed ischemic stroke diffusely involving corpus callosum. Magnetic resonance angiography was suggestive of compromised right-sided anterior circulation. This was corroborated by digital subtraction angiography that revealed reduced flow in right internal carotid artery. Diffuse infarction of the corpus callosum requires involvement of both the anterior and the posterior circulation. Due to the lack of clinical features suggestive of chronic internal carotid artery dissection, occlusive atherosclerotic disease of the anterior cerebral circulation associated with possible steal phenomenon from the posterior circulation was the most probable underlying mechanism for the callosal stroke. Steal phenomenon has been proposed as a compensatory mechanism in hemodynamically compromised ischemic parenchyma and it can explain the co-existence of anterior and posterior circulation strokes. This case also highlights how both anterior and posterior types of the alien limb phenomenon can co-exist in a background of vascular insult, resulting from intra-cranial atherosclerotic disease.

Higher order visual dysfunction and myoclonic-atonic seizure: an atypical presentation of CLN6 neuronal ceroid lipofuscinosis.

Neuronal ceroid lipofuscinosis is a rare childhood neurodegenerative disease, classified under the spectrum of progressive myoclonic epilepsy (PME). Cognitive decline, seizures including myoclonus, vision loss and ataxia are the commonly described manifestations of this illness. While visual failure in this disease is largely attributed to retinal, macular degeneration and optic atrophy, with this index case, we report an atypical presentation in the form of higher order visual dysfunction. The pattern of cognitive regression has further been explored here with higher order visual dysfunction and language regression being the predominant manifestations, stemming from an involvement of bilateral occipitoparietal/occipitotemporal networks. Yet another unique feature of this case also lies in the occurrence of myoclonic-atonic seizure, a semiology rarely reported before in PME.

Subacute measles encephalitis in a case of late presenting congenital HIV with epilepsia partialis continua as the first manifestation: A case report.

We report a case of a 14-year-old non-immunised girl with a prior history of measles infection presenting with afebrile seizures progressing to epilepsia partialis continua (EPC), quadriparesis and headache. Further evaluation revealed Human immunodeficiency virus (HIV) seropositivity with elevated anti-measles antibody titres in Cerebrospinal fluid (CSF). Electroencephalography showed focal epileptiform activity and Magnetic resonance imaging (MRI) of the brain revealed bilateral, asymmetrical long repetition time MRI (TR) hyperintensities involving juxtacortical white matter in both parietal lobes, left temporal and also in the left basal ganglia without any contrast enhancement or Diffusion weighted imaging (DWI) restriction. We describe the intriguing association of EPC with subacute measles encephalitis/measles inclusion body encephalitis (SME/MIBE) in the backdrop of immunocompromised state (HIV seropositivity), thought to have been acquired by vertical transmission. Also, prolonged asymptomatic HIV infection, first unmasked by measles infection, followed by rapidly deteriorating neurological illness makes this index case worthy to be reported.

COVID-19 associated rhino-orbital-cerebral mucormycosis: An observational study from Eastern India, with special emphasis on neurological spectrum.

AIMS: 1: Describe the epidemiology and determine risk factors for COVID-19 associated mucormycosis. 2: Elaborate the clinical spectrum of Rhino-Orbital-Cerebral Mucormycosis (ROCM), pattern of neuroaxis involvement and it's radiological correlates. METHODS: Observational study. Consecutive, confirmed cases of mucormycosis (N = 55) were included. A case of mucormycosis was defined as one who had clinical and radiological features consistent with mucormycosis along with demonstration of the fungus in tissue via KOH mount/culture/histopathological examination (HPE). Data pertaining to epidemiology, risk factors, clinico-radiological features were analysed using percentage of total cases. RESULTS: Middle aged, diabetic males with recent COVID-19 infection were most affected. New onset upper jaw toothache was a striking observation in several cases. Among neurological manifestations headache, proptosis, vision loss, extraocular movement restriction; cavernous sinus, meningeal and parenchymal involvement were common. Stroke in ROCM followed a definitive pattern with watershed infarction. CONCLUSIONS: New onset upper jaw toothache and loosening of teeth should prompt an immediate search for mucormycosis in backdrop of diabetic patients with recent COVID-19 disease, aiding earlier diagnosis and treatment initiation. Neuroaxis involvement was characterized by a multitude of features pertaining to involvement of optic nerve, extraocular muscles, meninges, brain parenchyma and internal carotid artery.

Claustrum hyperintensity: a rare radiological correlate in Niemann-Pick disease.

A 5-year-old male child of consanguineous parentage, without any adverse perinatal history, presented with progressive cognitive regression predominantly in the language and attention domains, for 2 years. He had simultaneous pyramidal and extrapyramidal involvement, frequent generalised tonic-clonic seizures and recurrent respiratory tract infections. Examination was significant for vertical supranuclear gaze palsy, coarse facial features and splenomegaly. Given the clinical features, in the background of consanguinity and mother's history of spontaneous pregnancy losses, inborn errors of metabolism were suspected. Following relevant investigations including tailored genetic study, Niemann-Pick disease type C (NPC) was diagnosed. Interestingly, MRI brain showed bilateral T2/fluid-attenuated inversion recovery claustrum hyperintensities, which are more commonly associated with autoimmune encephalitis and febrile infection-related epilepsy syndrome and not reported previously in NPC. Additionally, language regression as a presenting manifestation in NPC as opposed to classical dysarthria makes this case truly unique.

"Dancing belly" in an old diabetic lady.

Movement disorder, although rare, is increasingly being recognized as the initial presenting sign of the hyperglycemic state. Although chorea-ballism has frequently been reported among diabetics, monoballism is a very rare phenomenon. While myoclonus is common, diaphragmatic myoclonus is extremely rare. Moreover, diaphragmatic myoclonus as the initial presenting manifestation has never been reported before. Herein, we report an index case of a 62-year-old previously undiagnosed diabetic lady presented with acute onset constellation of multiple abnormal movements viz. monoballism, focal myoclonus, action myoclonus, and diaphragmatic myoclonus. All of them disappeared with achieving normoglycemia. This case underscores the importance of rapid capillary blood glucose testing in any patient presenting with acute onset abnormal movements. This approach can especially be rewarding as it helps in the rapid diagnosis of a reversible catastrophe and avoiding unnecessary costly investigations.