Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating central nervous system illness encountered in the setting of immunosuppressive conditions like human immunodeficiency virus / acquired immunodeficiency syndrome, autoimmune diseases and hematologic malignancies. We had a 54-year-old woman with systemic lupus erythematosus and coexisting autoimmune hepatitis who presented with progressive cognitive decline, right hemiparesis and ataxia who was found to have PML. She had severe CD4 lymphopenia. She was managed with low-dose prednisolone and plasma exchange after which she showed significant clinical improvement. This case highlights the diagnostic and therapeutic challenges encountered in managing a case of PML in the setting of autoimmune conditions with profound lymphopenia.

Wunderlich syndrome is a rare condition characterised by acute spontaneous non-traumatic renal haemorrhage into the subcapsular and perirenal spaces. Our case of anti-GAD65-associated autoimmune encephalitis (AE), aged 30 years, developed this complication following use of enoxaparin and was managed by selective glue embolisation of subsegmental branches of right renal cortical arteries. Our case had opsoclonus as one of the clinical manifestations, which has till now been described in only two patients of this AE. This patient received all forms of induction therapies (steroids, plasmapheresis, intravenous immunoglobulin and rituximab) following which she had good improvement in her clinical condition. The good response to immunotherapy is also a point of discussion as this has been rarely associated with anti-GAD65 AE.

Background and objective Amongst the varied neurological manifestations of COVID-19 infection, stroke is one of the common complications. A large portion of patients had large vessel occlusion (LVO) which increases the risk of malignant cerebral infarction requiring decompressive craniectomy. The impact of COVID-19, however, on decompressive craniectomy is not well described. Through this study, we aim to study the impact of decompressive craniectomy in COVID-19 patients presented to our tertiary care hospital. Material and methods Data of all acute ischemic stroke patients who tested COVID positive during the study period was collected. From among them, patients requiring decompressive craniectomy were included. The demographic, clinico-radiological parameters related to stroke, treatment received, outcome and complications were noted. In addition, data from all case reports and case series published on patients with COVID-19, who had developed ischemic stroke and underwent decompression craniectomy was collected and systematically reviewed. Results Twenty-seven stroke patients tested positive for COVID-19 infection, out of whom five patients underwent decompression hemicraniectomy in view of neurologic deterioration. The review of literature yielded 453 abstracts. After reading the full text of 69 articles, 12 studies on 15 patients finally met our inclusion criteria and were included in the systematic review. The mortality rate was 40% among stroke patients requiring decompressive craniectomy in COVID-19 patients. The mortality rate and functional outcomes of this cohort are comparable to the pre-pandemic period. Conclusion Decompressive craniectomy is a life-saving procedure in COVID-19 patients with malignant infarctions similar to patients in the pre-COVID-19 era.

Multiple cranial nerve palsies have many possible causes, including infective, inflammatory, neoplastic and infiltrative diseases of the meninges or skull base. We present the clinicopathological conference of a 27-year-old man with a smouldering course of sequential cranial nerve palsies. His imaging showed dural thickening and osteosclerosis of the skull base with otomastoiditis. Cerebrospinal fluid showed lymphocytic pleocytosis with reduced glucose and normal protein concentrations. There was a possible response to corticosteroids and anti-tubercular treatment. Initial biopsy from the thickened and enhancing dura was unremarkable. His condition deteriorated after the steroids were tapered; MR imaging showed progression of lesions and positron emission tomography showed intense hypermetabolism. Subsequently, a diagnostic test revealed the final diagnosis. This case was presented at the All India Institute of Medical Sciences’ monthly clinicopathological conference series in February 2021.

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Myasthenia gravis (MG) is an autoimmune disorder caused by antibodies that act against the myoneural junction. Conventional immunosuppressants such as corticosteroids, azathioprine and mycophenolate are associated with long-term side effects and many patients do not achieve remission and may become refractory. Thus, there is an unmet need for target-specific therapies that act faster, have fewer side effects and lead to stable disease remission. However, many of the novel therapeutic agents being described are not meeting their primary endpoints. We reviewed the current status of novel immunotherapies for MG, their mechanisms of action, along with the side effect profiles. Fast onset of action, sustained disease remission and relatively low frequency of side effects of the new agents are attractive. However, the unknown long-term safety and high cost are precluding factors. Better preclinical studies and more randomized trials are needed before novel agents are routinely employed.

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