IgG4-Related Neurological Disease: A Single Center Ambispective Study from South India.

IgG4-related disease (IgG4-RD) is an immune-mediated multi-system disorder. The nervous system (IgG4-RND) is rarely affected. We describe a short case series. We performed an ambispective analysis of IgG4-RND patients admitted at our centre between January 2016 and December 2022. Eight patients (M: F-2:6) were included with a mean age at presentation of 40.63 ± 17.88 years and disease duration of 5.16 ± 4.08 years. The common diseased sites were pachymeninges (7), orbits (4), paranasal sinuses (3), frontal lobe (1), hypophysis (1), leptomeninges (1), and middle ear (1). Common symptoms were headache and cranial neuropathy. The common nerves involved were the optic nerve, followed by the third, fifth, sixth, and seventh. Cerebrospinal fluid showed lymphocytic pleocytosis. Histopathology showed lymphoplasmacytic infiltrate (8), fibrosis (5), >10 IgG4 + cells (7), and IgG4/IgG >40% (6). Six had a relapsing course. The rituximab-based treatment regimen showed a favourable response.

First-Ever Ischemic Stroke In Covid-19: How Is It Different? - A Stroke Registry-Based Study From South India.

BACKGROUND AND AIMS: Stroke associated with COVID-19 has been characterised in several multicentre retrospective studies and meta-analyses. However, they did not distinguish first-ever ischemic strokes (F-AIS). Therefore, we aimed to study the incidence, clinical characteristics, and outcomes of a cohort of F-AIS associated with COVID-19 during the first wave of the pandemic and compare this cohort with those of F-AIS without COVID-19, COVID-19 without stroke. We also sought to compare the stroke admissions and mechanisms during the pandemic and immediate prepandemic periods.

Decoding "guitar pick sign" in COVID-19-associated mucormycosis: A case series.

"Guitar pick sign," also referred to as posterior globe tenting, is a radiological surrogate marker of tense orbit and profound vision loss. It is seen commonly in traumatic retrobulbar hemorrhage and carotico-cavernous fistula and less frequently in orbital cellulitis, subperiosteal abscess, and invasive fungal infections. We report a case series of Coronavirus disease-19-associated rhino-orbito-cerebral mucormycosis with guitar pick sign, of which none survived, and discuss the causative pathomechanisms, severity grade, and the clinical relevance of this unique radiological finding.

Pathogenetic factors fanning the flames of COVID-19 to cause rhino-orbito-cerebral mucormycosis: An observational study.

BACKGROUND AND AIMS: Published studies on coronavirus disease 19 (COVID-19) associated rhino-orbito-cerebral mucormycosis (CAROCM) were primarily descriptive. Therefore, we aimed to identify features of COVID-19 that could predispose to CAROCM and explore the pathogenic pathways. PATIENTS AND METHODS: This retrospective hospital-based study was done during the first (March 2020 - January 2021) and the second (February 2021 - June 2021) waves of the COVID-19 pandemic. Subjects were grouped into four categories: first-wave CAROCM (n-4); second-wave CAROCM (n-27); first-wave non-mucor COVID (n-75), and second-wave non-mucor COVID (n-50). Data elements included age, gender, comorbidities, COVID-19 severity, steroid therapy, peak values of interleukin-6 (IL-6), serum ferritin and D-dimer, nadir values of absolute lymphocyte count (ALC), absolute neutrophil count (ANC) and platelet count (Pl. C). RESULTS: Thirty-one patients of CAROCM were included. The mean (SD) age was 51.26 (11.48) years. 27 (87.1%) were aged ≥ 40 years and males. Severe COVID-19 was seen more often in the second wave than the first wave (P-0.001). CAROCM group was significantly younger (P-0.008) and showed a higher incidence of uncontrolled diabetes (P-0.001) and renal dysfunction (P-0.004) than non-mucor COVID. While IL-6, ferritin and D-dimer were significantly elevated in CAROCM than non-mucor COVID, clinical severity, ANC, ALC and Pl. C showed no significant difference. CONCLUSION: CAROCM is seen often in middle-aged diabetic males with uncontrolled hyperglycaemia, diabetic ketoacidosis, renal dysfunction and those infected by more transmissible delta variants and treated with steroids. IL-6, D-dimer, serum ferritin are more often elevated in CAROCM and might play a pathogenic role.

Long-term sequelae of herpes simplex virus encephalitis-related white matter injury: correlation of neuropsychological outcome and diffusion tensor imaging.

The pathophysiology of the memory impairment following Herpes Simplex virus encephalitis is not yet established and understood. This study attempts to elucidate the role of white matter injury and its impact on neuropsychological outcome in patients with history of Herpes Simplex virus encephalitis. This is a single-institution prospective study assessing 9 patients and 15 matched controls utilizing a combination of MRI with diffusion tensor imaging and neuropsychological testing. Tract-based spatial statistics analysis was performed and correlated with neuropsychological outcomes. Significantly decreased fractional anisotropy (FA) values were noted in corpus callosum, corona radiata, left posterior thalamic radiation, cingulum, superior longitudinal fasciculus, fornix, inferior longitudinal fasciculus, inferior fronto-occipital fasciculus, and uncinated fasciculus. Impaired facial recognition significantly correlated with reduction in FA of right uncinate fasciculus, right inferior longitudinal fasciculus, and splenium genu of corpus callosum. FA value of left cingulum significantly correlated with logical memory, auditory verbal learning. FA value of fornix correlated with visual recognition; FA value of left uncinate fasciculus with auditory verbal learning and delayed recall. In conclusion, this study demonstrates microstructural abnormalities involving several white matter tracts corresponding to neuropsychological deficits.

Apical pleural aspergillosis with pancoast's syndrome and posterior circulation stroke: a case report.

Pancoast's syndrome and subclavian arteritis are rarely caused by Aspergillus sp. . Here we report a case of a 22-yr-old, immunocompetent male who presented with fever, weight loss, right-sided facial anhidrosis and hand weakness of six months duration. Neurological examination confirmed right Horner's syndrome and weakness of small muscles of right hand. Contrast MRI of neck and chest revealed a pleural-based right apical mass abutting subclavian artery and C8-T1 root and multiple enlarged lymph nodes. He developed right hemiataxia due to cerebellar infarct before the planned excision of mass. Surgical exploration showed abscess encasing subclavian artery. Biopsy of the mass resulted in accidental injury of subclavian artery which was repaired. He developed bleeding from suture site postoperatively due pseudo-aneurysm of the subclavian artery which was stented. Histopathology of mass was suggestive of Aspergillus sp. . He was successfully treated with voriconazole. This is probably the first report of Pancoast's syndrome and large vessel angiitis caused by Aspergillus fumigatus which has been successfully managed.

Primary Angiitis of Central Nervous System-Tumor-Like Mass Lesions: Report of Six Biopsy Confirmed Cases.

Tumor-like mass lesion is a rare subtype of primary angiitis of the central nervous system (ML-PACNS). This report describes six patients of histologically verified ML-PACNS. The mean age was 44.5 years (range 25-68) and four were males. Presenting symptoms headache (5), focal neurologic deficits (5), and seizures (4). On magnetic resonance imaging (MRI) the lesion was unifocal in two and multifocal in four patients. Consistent radiological findings were mass lesions with heterointense internal morphology with areas of diffusion restriction (DWI), and variable post-contrast enhancement. Pathologically vasculitis was classified as: Granulomatous in one, necrotizing in two and lymphocytic in three. There were two deaths. In conclusion patients with ML-PACNS are likely to be younger and more likely to present with seizures. MRI lesions with hetrointense internal morphology with areas of DWI was a consistent finding and may be a clue for the diagnosis of ML-PACNS.

Pial arteriovenous fistula: A clinical and neuro-interventional experience of outcomes in a rare entity.

PURPOSE: Pial arteriovenous fistulae (PAVF) are rare intracranial vascular malformations, predominantly seen in children and distinct from arteriovenous malformations and dural arteriovenous fistulae. PAVF often leads to high morbidity and mortality. The aim of our study was to describe the clinical features and endovascular management of PAVF at various intracranial locations; to analyze the use of liquid embolic agents and coils alone or in combination in the treatment of PAVF and to analyze the outcome of embolization. MATERIALS AND METHODS: Retrospective review of diagnostic angiography and neurointerventional database of our institution identified a cohort of 15 patients with non-galenic PAVF from 2008 to 2014 out of 6750 patients. Fourteen patients were treated endovascularly with coils and liquid embolic materials in combination or alone. Patients were followed up for evaluation of prognosis. RESULTS: Age of the patients ranged from 3 to 37 years. Most patients were male and most common presentation was headache followed by seizure. Most common location of fistula was frontal lobe. The most common type was single artery single hole fistula with venous varix. Satisfactory obliteration was seen in all cases. One patient developed intraparenchymal hematoma on the first post procedural day and outcome was poor. CONCLUSIONS: PAVF are rare intracranial vascular malformations which can effectively be managed endovascularly with liquid embolic, coils alone, or in combination. Complete occlusion of the fistula can be achieved in most cases in a single sitting with a reasonable morbidity related to the procedure, compared with the natural history of this disease.

Mechanical Thrombectomy for Cerebral Venous Sinus Thrombosis: A Case Series and Technical Note.

BACKGROUND: Cerebral venous sinus thrombosis is a rare cause of acute stroke. It may lead to hemorrhagic venous infarctions, brain swelling, or raised intracranial pressure and can be associated with significant mortality and morbidity. Low-molecular-weight heparin is the mainstay of treatment. Endovascular treatment is reserved for patients who deteriorate despite medical management. METHODS: Retrospective evaluation of our institutional databases from 2018-2019 revealed 7 patients who underwent aspiration thrombectomy using large-bore aspiration catheters for recanalization of the dural sinuses with or without intrasinus thrombolysis during the procedure. Their clinical, imaging findings, endovascular technique, and outcome are discussed. RESULTS: We treated 7 patients who did not respond to best medical management. Aspiration thrombectomy was the primary mode of endovascular treatment. Adjuvant low-dose (10 mg recombinant tissue plasminogen activator), short-duration (20-minute) intrasinus thrombolysis was used in 4 patients only during the procedure. Headache was the most common symptom, followed by seizures, focal neurologic deficits, and vomiting. There was improvement in clinical condition within 24 hours of procedure in all patients. The 30-day modified Rankin Scale score was 0 in 6 patients. One patient had residual hemiparesis and aphasia. There were no procedure-related complications. CONCLUSIONS: The described technique appears to be simple, safe, and effective and results in a relatively short procedure time in achieving complete or partial recanalization of the dural sinuses in patients who deteriorate despite clinical management. Endovascular thrombectomy along with conventional medical management restores the final drainage pathway of the brain with good clinical outcome.

Nocardiosis-an uncommon infection in patients with myasthenia gravis: report of three cases and review of literature.

Nocardiosis is a rare infection in patients with myasthenia gravis (MG). We identified three cases of MG admitted with nocardiosis in our unit. We performed systematic literature search of previous publications and identified 18 patients. This paper presents three patients and reviews the clinical characteristics of 21 patients. The first case was a 69-year-old woman with thymomatous MG who presented with pustules and left lower limb pain. Evaluation showed osteomyelitis of the pubic ramus and ileopsoas abscess. The second case was a 54-year-old man who presented in myasthenic crisis due to pulmonary nocardiosis. The third case was a 48-year-old man with thymomatous MG who presented with lung abscess. All of them recovered completely after treatment with co-trimoxazole. Analysis of the 21 patients identified four risk factors for nocardiosis in MG: elderly men; thymoma; immunosuppressant medication, mainly steroid therapy; and pre-existing lung disease. Lungs was the most common site of infection. Suppurative disease was common manifestation regardless of organ involved. Clinical course is not unfavourable.