New-onset severe eosinophilic granulomatosis with polyangiitis following the third dose of mRNA COVID-19 vaccine: A case report.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a complex multifactorial disease that results in multisystemic inflammation of the small- and medium-sized arteries. The exact pathogenesis of this syndrome is poorly understood, but it is postulated to result from a combination of eosinophilic dysfunction, genetic predisposition, and the development of autoantibodies after exposure to an unknown stimulus. We describe a case of new-onset EGPA following the third dose of the Pfizer-BioNTech mRNA vaccine in an infection-naive middle-aged man with a background history of allergic respiratory symptoms. The patient developed acute onset of mononeuritis multiplex, pauci-immune glomerulonephritis, and leucocytoclastic vasculitis 10 days after receiving the booster dose. His laboratory markers including eosinophil count, antineutrophil cytoplasmic antibodies, and renal function tests improved markedly after the initiation of pulse steroid therapy and rituximab infusion. However, his peripheral muscle weakness and neuropathic pain did not respond to the initial therapy but improved later with intravenous cyclophosphamide and intravenous immunoglobulin. To the best of our knowledge, this is the fourth case report of post-coronavirus disease 2019 vaccination precipitation of EGPA. All reported cases including our report were in patients with previous allergic manifestations who received mRNA-based coronavirus disease 2019 vaccines, and all the patients developed mononeuritis multiplex at presentation. Despite the few reported cases of post-vaccination autoimmune phenomena, the temporal association between vaccination administration and disease onset does not indicate causality, given the mass vaccination programmes employed. However, the novel use of the mRNA platform in vaccine delivery necessitates vigilant monitoring by the scientific committee.

The great imposter: A case report of IgG4-RD hypertrophic pachymeningitis with skull lytic lesion and pulmonary nodules.

Immunoglobulin G4-related disease (Ig4RD) is an inflammatory condition with unique clinical, serological, and pathological features. In this study, we report a challenging diagnostic clinical case of Ig4RD diagnosed based on histopathology. This unique imitating nature reinforces that it is crucial to consider the diagnosis of IgG4-RD in those presenting with pachymeningitis.

Corneal Confocal Microscopy detects a Reduction in Corneal Endothelial Cells and Nerve Fibres in Patients with Acute Ischemic Stroke.

Endothelial dysfunction and damage underlie cerebrovascular disease and ischemic stroke. We undertook corneal confocal microscopy (CCM) to quantify corneal endothelial cell and nerve morphology in 146 patients with an acute ischemic stroke and 18 age-matched healthy control participants. Corneal endothelial cell density was lower (P < 0.001) and endothelial cell area (P < 0.001) and perimeter (P < 0.001) were higher, whilst corneal nerve fibre density (P < 0.001), corneal nerve branch density (P < 0.001) and corneal nerve fibre length (P = 0.001) were lower in patients with acute ischemic stroke compared to controls. Corneal endothelial cell density, cell area and cell perimeter correlated with corneal nerve fiber density (P = 0.033, P = 0.014, P = 0.011) and length (P = 0.017, P = 0.013, P = 0.008), respectively. Multiple linear regression analysis showed a significant independent association between corneal endothelial cell density, area and perimeter with acute ischemic stroke and triglycerides. CCM is a rapid non-invasive ophthalmic imaging technique, which could be used to identify patients at risk of acute ischemic stroke.

Correction to: Pre-existing Small Vessel Disease in Patients with Acute Stroke from the Middle East, Southeast Asia, and Philippines.

The author names "Dr. Pablo Garcia Bermejo" and "Dr. Muhammad Faisal Wadiwala" needed to be added as the 6th and 7th authors, respectively. The authors regret this error.

Pre-existing Small Vessel Disease in Patients with Acute Stroke from the Middle East, Southeast Asia, and Philippines.

Asymptomatic small vessel disease (SVD), including white matter hyperintensities (WMHIs), periventricular hyperintensities (PVHIs), silent stroke (SS), and cerebral microbleeds (CMBs), increases the risk of stroke. There are limited studies of SVD in subjects from the Middle East and Southeast Asia (SA). All patients admitted to stroke service between 2014 and 2015 were reviewed for presence of "pre-existing" SVD. Stroke mimics with no previous history of stroke were used as controls. There were 1727 patients admitted with stroke. Analysis was done on 988 subjects (914 strokes and 74 controls) who had MRI scan done. Pre-existing SVD was seen in 642 (64.9%) patients (WMHIs 19.6%, PVHIs 33.2%, SS 51.4%, and CMBs 22%). Silent strokes were significantly more common with ischemic stroke (IS) compared to intracranial hemorrhage (ICH) (62.0 vs 34.3%, p < 0.001). CMBs were more in ICH compared to IS (42.9 vs 23.1%, p < 0.001). The risk of developing CMBs among Far Eastern (FE) patients was 1.58 times more (p = 0.07), while 1.48 times more in Arabs (AR) (p = 0.026) compared to SA after adjusting for age. The risk of developing PVHIs was significantly higher in Arab compared to SA (odds ratio (OR) 1.43; p value = 0.021). Similarly, the risk of developing WMHIs was also significantly higher in AR patients (OR 1.6; p value = 0.009) compared to SA. The majority of ethnic AR, SA, and FE populations show pre-existing SVD on MRI. The advanced changes at a young age may be related to high prevalence of untreated risk factors and possibly as yet defined genetic and environmental factors.

Decompressive surgery for malignant cerebral venous sinus thrombosis: a retrospective case series from Pakistan and comparative literature review.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke in the West; however, it is prevalent in Asia and the Middle East. CVST is treated with dose-adjusted heparin or heparinoid followed by warfarin to facilitate recanalization of venous sinuses. For those with progressive malignant cerebral edema, the role of decompressive surgery has been reported from developed countries. We present data on decompressive craniectomy from a tertiary care stroke center in a developing country and compare our results and population with that described in the international literature. METHODS: We retrospectively analyzed data of all patients who underwent a decompressive hemicraniectomy for CVST at the Aga Khan University Hospital, Karachi, Pakistan from 1999 till 2011. A record review of the Aga Khan University Hospital was performed as decompressive hemicraniectomy for malignant CVST is not being performed elsewhere in the country and the hospital is a major referral center. Using the International Classification of Diseases, Ninth Edition codes for CVST, we identified a total of 7 patients. Patients are presented along with descriptions of their presentation, neuroimaging, intraoperative findings, and long-term outcomes. Pearson chi-square test was done to identify features that predicted survival. A comparative literature review was also done through PubMed to identify all other reports of surgery for CVST. RESULTS: During a 12-year review, 134 patients were diagnosed with CVST. Of these, 7 received intervention. The age range of the patients was 15-60 years. Four of the 7 patients had an excellent outcome, 2 of 7 died, and 1 of 7 left against medical advice (in a comatose state) and was lost to follow-up. Patients presented alert but progressively deteriorated preoperatively. All those patients who had preoperative reactive pupils with low Glasgow Coma Scale scores made a complete neurologic recovery, and patients with fixed, dilated, and nonreactive pupils preoperatively died in the first postoperative week (P = .05). CONCLUSIONS: Patients who received decompressive hemicraniectomy in Pakistan for CVST had excellent outcomes in all cases when intervention was performed with intact preoperative pupillary reflexes. Of the data reviewed, most reported (two-third) patients show the same prognosticators; however, one third show that even with nonreactive pupils complete recovery is possible.