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.

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.

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.

Monomorphic ventricular tachycardia and mediastinal adenopathy due to granulomatous infiltration in patients with preserved ventricular function.

OBJECTIVES: This report characterizes a syndrome of granulomatous infiltration presenting as sustained monomorphic ventricular tachycardia (SMVT) with mediastinal adenopathy in patients with preserved ventricular function. BACKGROUND: Unlike truly idiopathic ventricular tachycardia, SMVT due to granulomatous infiltration responds poorly to radiofrequency ablation and has a poor prognosis. METHODS: Patients without obstructive coronary artery disease and with normal ventricular function presenting with SMVT other than posterior fascicular morphology were evaluated. Computed chest tomograms, cardiac magnetic resonance imaging, and 18-fluorodeoxyglucose positron emission tomographic scans ((18)FDG PET-CT) were performed. Significant lymph nodes were evaluated for tuberculosis and sarcoidosis. Initial treatment included antiarrhythmic drugs ± radiofrequency ablation. Additionally, patients with evidence of tuberculosis received anti-tuberculosis therapy; the rest were treated as sarcoidosis. RESULTS: Mediastinal adenopathy with mid-myocardial scar and/or focal myocardial inflammation was observed in 14 patients; lymph nodes revealed noncaseating granulomas in all. Evidence of tuberculosis was present in 79%. During follow-up (median duration 25 months), SMVT recurred despite initial treatment in 92%. Addition of disease-specific therapy abolished further recurrences in 64% of them. Decrease in SMVT correlated with resolution of myocardial inflammation on serial (18)FDG PET-CTs. Appropriate therapies occurred in 67% of patients receiving implantable cardioverter-defibrillators. CONCLUSIONS: A subset of patients with SMVT with preserved ventricular function has a syndrome of arrhythmogenic myocarditis with granulomatous mediastinal adenopathy due to myocardial tuberculosis or cardiac sarcoidosis. This entity is optimally managed with a combination of disease-specific therapy and antiarrhythmic measures.