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[3] Diagnostic challenge occurs when there are no detectable auto-antibodies in serum or CSF; these subgroup of patients are termed Seronegative (or antibody-negative) autoimmune encephalitis (SAE) with suspected immunological origin based on clinical and imaging features. [...]a large proportion of patients with suspected immune-mediated disorders do not have detectable auto-antibodies, resulting in diagnostic and therapeutic challenges. The field of autoimmune encephalitis is rapidly expanding with the advent of many newer auto-antibodies; the real life clinical experience of evaluation of AE without diagnostic biomarkers results in diagnostic and therapeutic challenges which has been addressed in this article.

Background: Rabies is transmitted to humans through the saliva of an infected animal. Case: We report a rare case of rabies encephalitis presenting with subarachnoid hemorrhage. An elderly gentleman presented with behavioral disturbances and rapid worsening in sensorium. Neuroimaging showed diffuse subarachnoid hemorrhage, hydrocephalus, and right vertebral dissecting aneurysm requiring extraventricular drainage. He had history of dog bite 4 months prior with incomplete immunization. Despite adequate management, his condition deteriorated further and succumbed to death. Autopsy confirmed the diagnosis of rabies. Conclusions: Rabies encephalitis can pose a diagnostic challenge requiring an extensive diagnostic evaluation because of its variable and uncommon clinical and radiological presentations.

Autoimmune encephalitis is a group of non-infectious immune-mediated inflammatory disorders manifesting with epilepsy and encephalitis syndromes that are associated with autoantibodies in the serum and/or cerebrospinal fluid (CSF). Pathogenic autoantibodies have been discovered against intracellular onconeural antigens, surface neuronal, or synaptic antigens with distinctive pathogenesis that underlie differences in response to immunotherapy. The onconeural antigens incite cytotoxic T-cell-mediated neuronal destruction, whereas surface antigens trigger direct damage by autoantibodies via complement mediated pathways, and hence respond well to immunomodulatory therapy, in contrast to poor response in the former. Neuroimaging, electroencephalogram, and CSF findings being non-specific, detection of autoantibodies is essential for a confirmatory diagnosis. Detection methods available include tissue-based assay, cell-based assays, immunoblot, cell culture, flow cytometry, and enzyme-linked immunosorbent assays. In this review, we discuss the various testing modalities available for onconeural and cell surface antibodies, their sensitivity and specificity and the emerging role of the pathologist in the diagnosis of autoimmune encephalitis. Early diagnosis is crucial for instituting treatment and preventing morbidity and mortality.

Nocardiosis has a predilection for affecting the lungs or occurs as a disseminated disease. Cerebral involvement resulting in multiple brain abscesses is rarely reported in 2% of cases. A 30-year-old woman diagnosed with immunocompromised state six months back presented with headache, multiple episodes of seizures, and progressive right hemiparesis for the past three months. She was initially treated with antibiotics, but in view of non-improvement, an early burr-hole aspiration of the abscess resulted in the correct diagnosis. She was then subsequently treated with antimicrobial therapy and surgical excision of the abscess, resulting in significant improvement. Follow-up at four-month duration showed significant improvement with complete resolution of all clinical features. Patients with multiple cerebral nocardial abscesses may require combined treatment with antimicrobial therapy and surgical excision to improve the mortality and prognosis. An early burr-hole-guided aspiration results in faster etiological diagnosis and prompt targeted approach.

Morvan's syndrome is a rare, complex autoimmune syndrome comprising peripheral nerve hyperexcitability, dysautonomia, insomnia, and encephalopathy. In this case report, we highlight the clinical and pathological findings of an elderly gentleman who presented to us with clinical features of Morvan's syndrome associated with anti-contactin-associated protein 2 (CASPR-2) antibodies. Histopathology [Figure 3] revealed cortical atrophy with gliosis and mild microglial proliferation. Microglial activation and gliosis were observed in the hippocampus, hypothalamus, and thalamus. Brainstem showed multifocal inflammation. Mild inflammation was observed in the leptomeninges. Morvan's syndrome is an autoimmune disease with antibodies targeted against CASPR within the voltage-gated potassium channel (VGKC) complex. Early diagnosis and treatment play a key role in the management of patients. Most patients show a good response when treated with plasmapheresis and steroids. This patient presented to us late into the illness and succumbed.

Background Tuberculomas are prevalent in developing countries and demonstrate variable signals on MRI resulting in the overlap of the conventional imaging phenotype with other entities including glioma and brain metastasis. An accurate MRI diagnosis is important for the early institution of anti-tubercular therapy, decreased patient morbidity, mortality, and prevents unnecessary neurosurgical excision. This study aims to assess the potential of radiomics features of regular contrast images including T1W, T2W, T2W FLAIR, T1W post contrast images, and ADC maps, to differentiate between tuberculomas, high-grade-gliomas and metastasis, the commonest intra parenchymal mass lesions encountered in the clinical practice. Methods This retrospective study includes 185 subjects. Images were resampled, co-registered, skull-stripped, and zscore-normalized. Automated lesion segmentation was performed followed by radiomics feature extraction, train-test split, and features reduction. All machine learning algorithms that natively support multiclass classification were trained and assessed on features extracted from individual modalities as well as combined modalities. Model explainability of the best performing model was calculated using the summary plot obtained by SHAP values. Results Extra tree classifier trained on the features from ADC maps was the best classifier for the discrimination of tuberculoma from high-grade-glioma and metastasis with AUC-score of 0.96, accuracy-score of 0.923, Brier-score of 0.23. Conclusion This study demonstrates that radiomics features are effective in discriminating between tuberculoma, metastasis, and high-grade-glioma with notable accuracy and AUC scores. Features extracted from the ADC maps surfaced as the most robust predictors of the target variable.

A plethora of monoclonals have ushered up for NMOSD treatment. However, their limited availability and cost concerns poses a challenge for usage in developing nations. We compared relapse rates and disabilities among aquaporin-4 positive(AQP4+ve) patients on conventional immunosuppressants and rituximab in a tertiary referral center in southern India. This was a chart review of AQP4+ve patients registered under national demyelination registry maintained at institute. AQP4+ve patients were included if they were on azathioprine, MMF, methotrexate for six months; cyclophosphamide for three months and rituximab for one month. 207 records were screened, 154 fulfilled inclusion criteria. Drugs used were azathioprine (70), MMF (34) and rituximab (33). All three drugs were non-inferior to each other in terms of ARR reduction. Median EDSS at last follow-up was significantly lower for azathioprine(2;IQR:0–5) and rituximab(2;IQR:0.5–5) than MMF(3.5;IQR:2–5.6), however azathioprine was associated with highest switch rate(34.3%) and was the only drug which required change because of intolerance. Failure rate was least for rituximab(27.3%).Patients on azathioprine and MMF required higher mean duration of concurrent steroids(7.8±7.7 and 4.56±2.17 months respectively) when compared to rituximab(2.77±1.38) and had more relapses due to steroid withdrawal. Initial treatment with azathioprine, MMF and rituximab is comparable in terms of ARR reduction. Findings suggest that choice may be guided by adverse event profile of drug, rather than efficacy per se. Concurrent treatment duration with steroids should also guide clinical decision. Switch to second immunomodulation in event of initial failure adds to efficacy benefit, irrespective of the drug chosen. •Azathioprine and mycophenolate mofetil fare at par with rituximab in reducing pre-treatment ARR in AQP4+ve NMOSD patients.•Azathioprine was associated with highest switch rate (34.3%), majorly due to intolerance.•Failure rate was least for rituximab (27.3%).•Relapses owing to steroid withdrawal were least with rituximab.

Background: The term Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (\"SLIPPERS\") is an uncommon form of neuroinflammation. Case: We report a 57-year-old gentleman with rapidly progressive cognitive and behavioral changes. Magnetic resonance imaging (MRI) brain showed confluent T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities involving white matter of bilateral frontal, anterior temporal lobes, external capsule, right thalamus, and right corona radiata with peppering contrast enhancement. Stereotactic brain biopsy showed prominent perivascular lymphocytic inflammation. The patient responded well to immunotherapy. Conclusions: SLIPPERS syndrome is an exceedingly rare entity with varied clinical presentation, and diagnosis is dependent on neuropathological features. Prompt recognition is crucial for a timely response to immunomodulation.