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ABSTRACT: Diffuse midline glioma is a WHO grade IV tumor of the central nervous system (CNS). The most common sites from which diffuse midline glioma arises are the spinal cord, brain stem, thalamus, and rarely cerebellum. This is a highly aggressive and rare tumor of CNS, more common in the pediatric population. MRI brain lesions are T1-weighted (T1W) image hypointense and T2-weighted (T2W) image hyperintense and may show subtle contrast enhancement, with a more exophytic component and adjacent structure infiltration. Here, we report a case of 30 years old male who presented with symptoms of headache since 15 days, associated with one episode of transient blurring of vision and imbalance while walking. On examination, bilateral early papilledema and subtle bilateral cerebellar signs were present. MRI brain study showed multiple rounds to oval lesions in the corpus callosum, left thalamus, left periventricular region, vermis, and right cerebellar hemisphere, with low apparent diffusion coefficient and diffusion restriction and no blooming on gradient echo sequences images. Initially, no postcontrast enhancement was seen. Later on, the patient's condition deteriorated despite intravenous steroids and anti-epileptics. On repeat, MRI contrast showed subtle contrast enhancement with an increase in the size of lesions. Stereotactic biopsy from the corpus callosum lesion was done. Histopathology showed diffuse midline glioma WHO grade IV tumor. Even after receiving chemotherapy and radiotherapy, patient succumbed within 4 months of diagnosis. This is a case of adult-onset diffuse midline glioma with a very atypical lesion on the MRI brain as compared to prior studies where the midline structure is more commonly involved. [ABSTRACT FROM AUTHOR]

Introduction: Cavernous sinus syndrome (CSS) is a condition with varied etiology. It is characterized by ophthalmoplegia, facial sensory loss, and Horner’s syndrome. The objective of this study was to determine the clinical and radiological presentations and to identify the factors that could help in the identification of etiology. Methods: We prospectively observed all the patients presenting with CSS from October 2019 to September 2021 at a tertiary care hospital. Patients with coronavirus disease 2019 (COVID‑19)‑associated mucormycosis were excluded. All of these patients underwent detailed clinical examination, hematological, radiological, and specialized investigations based on clinical suspicion. Clinical presentations, imaging characteristics, etiology, and clinical outcomes were analyzed. 45 patients were included in our study. Results: The mean age was 44.6 ± 10.8 years with a female preponderance. The most common presentation was diplopia which was seen in all patients. The commonest cranial nerve involved was the abducens (75.9%). Infections (12 (26.6%)) constituted the commonest cause of CSS, followed by neoplastic involvement in 11 (24.4%), Tolosa–Hunt syndrome in 9 (20%) and vascular involvement in 8 (17.7%) patients. Other inflammatory causes were seen in 5 (11%) patients. Diabetes was positively associated with fungal infections. The presence of bruit was strongly associated with vascular etiology. The presence of sinusitis with bony erosions was strongly associated with fungal infections. Evidence of dilated superior ophthalmic vein with early filling in the angiographic phase was associated with carotid‑cavernous fistulas. Conclusions: CSS has variable etiology and radiological presentation. The presence of rapid progressive ophthalmoplegia with underlying diabetes with imaging evidence of sinusitis and bony erosions mandates a high degree of suspicion of fungal infections and empirical antifungal therapy needs to be considered for better outcomes. [ABSTRACT FROM AUTHOR]

Identifying early predictors of severe Covid-19 associated mucormycosis (CAM) can help improve management and treatment outcomes. Primary: To identify clinical and radiological predictors of disease severity in CAM. Secondary: To describe patterns of central nervous system (CNS) involvement in CAM. A total of 71 patients with CAM were included in the study. Based on the anatomical extent of involvement on MRI, patients were divided into three groups: Sinus (paranasal sinuses), Orbit (orbital spread), and CNS (CNS spread). Clinical parameters and radiological patterns of involvement of sinuses and extra sinus spaces were studied between the three groups. Patterns of CNS involvement were also described. A shorter time lag between COVID-19 infection and CAM, as well as high HbA1C levels, were found to be associated with severe disease. Involvement of the sphenoid, ethmoid and frontal sinuses, T1 hyperintense signal in the sphenoid, as well as bony involvement of the sphenoid sinus, were significantly associated with severe disease. Extra-sinus spread into pre/retroantral space, pterygopalatine fossa, and masticator spaces were also significantly associated with a severe disease course. The most common pattern of CNS spread was cavernous sinus involvement, followed by pachymeningeal spread and cranial nerve involvement. Early identification of the above-described predictors in patients presenting with CAM can help detect those at risk for developing severe disease. A longer duration of amphotericin, combined with a more aggressive surgical approach in selected cases, may lead to better long-term outcomes. [ABSTRACT FROM AUTHOR]

Intracranial arterial dolichoectasia is a disorder of cerebral vasculature characterized by abnormal elongation, tortuosity, and dilatation of the cerebral arteries. The posterior circulation is more frequently affected than the anterior circulation. Dolichoectasia of internal carotid arteries (ICAs) presenting with binasal hemianopia is rare. We report a case report of a 48-year-old male who presented with a history of binasal hemianopia. Magnetic resonance (MR) imaging of the brain with MR angiography showed bilateral dolichoectasia of ICAs compressing bilateral optic nerve and optic chiasm. Diagnosis of bilateral optic neuropathy due to dolichoectasia of ICAs was made after thorough investigation and ruling out of other possible etiologies of bilateral optic neuropathy. Dolichoectasia is a rare cause of bilateral optic neuropathy and should be suspected in patients who present with gradually progressive visual field loss with onset in the nasal field. [ABSTRACT FROM AUTHOR]

Familial amyloid polyneuropathy (PN), also known as amyloid transthyretin (TTR)-PN is an autosomal dominant adult-onset fatal disease, if not treated. It occurs due to mutations in (TTR) gene which leads to a faulty TTR protein which folds up to form amyloid and gets deposited mainly on nerves and causes length-dependent PN and autonomic dysfunction. We report a case of a 45-year-old female who presented with symptoms of painful peripheral neuropathy for 5 months, a history of deafness for 5 years, and cardiac pacemaker implantation 2 years ago for complete heart block. She denied any symptoms of autonomic dysfunction. Her brother with similar symptoms died of cardiac arrest at the age of 50 years. Clinical examination was suggestive of symmetrical sensorimotor PN. The nerve conduction study was suggestive of axonal sensorimotor PN. Abdominal fat biopsy was negative for amyloid. Sural nerve biopsy was suggestive of amyloid neuropathy. Genetic analysis showed c. 165G > T mutation encoding amino acid p. Lys55Asn on exon-4 of TTR gene. This mutation has not been reported from India. [ABSTRACT FROM AUTHOR]

Chikungunya is a common tropical viral infection in India. The majority of patients have limited systemic manifestations. Neurological manifestations of chikungunya may be due to direct viral infection or immune mediated. We present a case of a 45-year-old male who presented with acute onset paraplegia with diminution of vision in the right eye. A detailed evaluation revealed a diagnosis of chikungunya myeloradiculitis with viral keratitis. The patient was treated with steroids followed by intravenous immunoglobulin and had a good recovery. [ABSTRACT FROM AUTHOR]

Background: Nowadays non-organophosphorous compound poisonings are increasing, thus study of their clinical manifestations and outcome is the need of time. In this study, 50 patients who had history of consumption of nonorganophosphorous compound were studied and outcome was studied, it was a prospective observational study. The commonest cause of poisoning in India and other developing countries is organophosphorus compounds. along with organophosphorous compounds non-organophosphorous compound poisoning and their outcome needs to be studied. Materials and methods: For this prospective observational study, 50 patients who had consumed non organophosphate poison and having symptoms were included in the study after meeting inclusion criteria. It was conducted between September 2016 to May 2018 in PRH Loni, each patient was subjected to detailed clinical history and clinical examination, well written and informed consent was taken from each relative before including the patient for this study. All the calculations were done by standard formula for calculating mean/average of each variable. Results: Majority of the patients n=29(58 %) were males rest n=21 (42%) were females, the mode of exposure was oral in all patients, most patients survived, overall mortality was 4 %, mean duration of hospital stay was 3 days, most common poisoning was due to amitraz followed by phenyl compounds. Conclusion: Most common cause for poisoning was amitraz compound followed by phenyl compounds, overall mortality was only 4%. [ABSTRACT FROM AUTHOR]

Spinocerebellar ataxia (SCA)-1, 2, 3, 6, and 31 are associated with sleep behavior disorder. We had a 66-year-old male who presented with late-onset cerebellar ataxia. Before 2 years, the onset of cerebellar symptoms, he had a history of sleep behavior disorder. He had a strong family history of similar symptoms in his younger sister. Whole exome sequencing of the patient and Sanger sequencing of his sister confirmed the diagnosis of SCA-6., (© Journal of The Association of Physicians of India 2024.)

Migraine prevalence has risen over the last few decades, which may be attributed to lifestyle changes. Epistaxis is unusual in migraine. Here we present a case series of four patients, who are presented with headaches associated with epistaxis. A detailed history revealed cardinal symptoms of migraine according to the International Headache Society, including hemicranial throbbing headache of moderate to severe intensity lasting for a duration of four to 72 hours, along with associated features of nausea, vomiting, photophobia, and phonophobia. Investigations, including ENT (ear, nose, and throat) examination, nasal endoscopy, gastroscopy, bronchoscopy, hematological, and coagulation parameters, were negative. All patients were started on prophylactic treatment for migraine, and they responded well. Epistaxis occurs at the peak of headache following which symptoms tend to resolve. The pathophysiology behind this is stimulation of the trigeminovascular system leading to dilatation of external and internal carotid arteries., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Rohatgi et al.)

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., (© Journal of the Association of Physicians of India 2024.)