Immunoglobulin G4-related disease presenting with peripheral neuropathy: a case report.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition characterized by high serum IgG4 concentrations and tissue infiltration by IgG4-positive plasma cells. Reports have demonstrated that IgG4-RD affects various organs, including the pancreas, kidney, lung, thyroid, and lacrimal and salivary glands. In the nervous system, hypertrophic pachymeningitis and hypophysitis are mainly related to IgG4-RD; however, the peripheral neuropathy involvement is unusual. CASE PRESENTATION: We report on a 69-year-old woman with multiple mononeuropathy, weight loss and kidney mass in the setting of IgG4-RD. Biopsies of the kidney mass showed lymphoplasmacytic sclerosing inflammation with numerous IgG4-positive plasma cells. IgG4 and IgG4/IgG ratios in the blood were elevated. The patient was treated with high dose methylprednisolone with improvement in her neuropathy. CONCLUSIONS: IgG4-RD is a relatively recently reported systemic fibrous inflammatory disease caused by the infiltration of IgG4-positive plasma cells in various organs. In the nervous system, symptomatic peripheral nerve invasion is very rare. However, as demonstrated in our case, IgG4-RD may present with primarily peripheral nerve disease.

Rescue Endovascular Treatment to Prevent Neurological Deterioration in Acute Symptomatic Bilateral Vertebral Artery Occlusion.

Bilateral vertebral artery occlusive disease has been considered as a favorable condition with good collaterals. However, the prognosis of acute ischemic stroke secondary to symptomatic bilateral vertebral artery occlusion (BVAO) and endovascular treatment (EVT) has rarely been reported. We retrospectively selected patients with acute ischemic stroke admitted for symptomatic BVAO between January 2020 and February 2023. All patients with ischemic stroke were evaluated for ischemic lesion and arterial status using brain imaging and angiography. The prognosis of acute stroke with symptomatic BVAO was compared between EVT and conventional treatment. Outcomes were evaluated using modified Rankin Scale (mRS) score at 3 months follow-up. Within the study period, 17 of 2,655 acute ischemic stroke patients were diagnosed with ischemic stroke with symptomatic BVAO. The median age of these patients was 70 (interquartile range 44-89) years, and 13 (76%) were male. Seven patients received emergent EVT with stenting and 10 patients received conventional medical treatment only. Nine of 10 patients with conventional treatment had in-hospital stroke progression and developed new ischemic lesions in the pons and midbrain. Five patients with fetal and hypoplastic posterior communicating artery presented bilateral cerebral peduncular lesions. At 3 months follow-up, 6 patients (35%) had favorable outcomes (mRS 0-2), of which 5 were treated with vertebral artery stenting and 1 received conventional treatment. Ischemic stroke in patients with acute symptomatic BVAO is uncommon. However, stroke progression is common, and the prognosis of most patients is poor. Rescue management such as EVT might be considered for symptomatic BVAO.

Endovascular Reperfusion Therapy in Minor Stroke with Neurologic Deterioration beyond 24 Hours from Onset.

PURPOSE: Patients with minor stroke (National Institutes of Health Stroke Scale score ≤5) and large vessel occlusion (LVO) often experience neurological deterioration >24 hours after onset. However, the efficacy of endovascular reperfusion therapy in these patients remains unclear. The aim of this study was to determine the efficacy and safety of reperfusion therapy in patients with minor stroke and neurological deterioration >24 hours after onset. MATERIALS AND METHODS: Data were retrospectively reviewed from patients between January 2019 and April 2022 who met the following criteria: (1) minor stroke and small definitive ischemic lesions at initial visit, (2) onset to neurological deterioration >24 hours, (3) cortical signs, Alberta Stroke Program Early computed tomography (CT) Score >6 points, and large artery occlusion confirmed by CT angiography at neurological deterioration. Efficacy and safety outcomes were based on final thrombolysis in cerebral infarction (TICI), incidence of symptomatic intracranial hemorrhage (ICH), and mortality. Outcomes were assessed using the modified Rankin Scale (mRS) at 3 months. Good outcome was defined as a mRS of 0, 1, or 2. RESULTS: Data from 26 patients (38.4% female, mean age 75.8 years) were analyzed; 18 (69.2%) had a good outcome. A final TICI of 2b or 3 was observed in 24 (92.3%) patients. No other adverse events, including dissection, vasospasm or distal embolization, were observed during the procedures. Hemorrhagic events occurred in 8 patients after the procedure; however, there were no symptomatic ICHs. Good prognostic factors were younger age (P=0.062) and carotid stenting (P=0.025). CONCLUSION: Endovascular reperfusion therapy performed in selected patients with minor stroke, LVO, and neurological deterioration >24 hours after stroke onset demonstrated favorable outcomes and safety.

Two possible etiologies of Guillain-Barré syndrome: mRNA-1273 (Moderna) vaccination and scrub typhus: A case report.

RATIONALE: Guillain-Barré syndrome (GBS) is an acute inflammatory polyneuropathy related to infection with bacteria or virus and vaccination. Cases of GBS after coronavirus infection-19 (COVID-19) vaccination have been reported. However, cases of GBS after inoculation with mRNA-based COVID-19 vaccines, especially mRNA-1273, have rarely been reported compared to after inoculation with adenovirus vector-based COVID-19 vaccines. On 1 hand, GBS occurring after scrub typhus is often reported, but the exact pathological mechanism has not been elucidated. We report the case of a patient with GBS after inoculation with mRNA-1273 COVID-19 vaccine and scrub typhus. PATIENT CONCERNS: A 47-year-old man received COVID-19 vaccination 4 weeks before admission. He had a fever, rash and general weakness 1 day after vaccination. After 3 weeks, the muscle strength of the extremities deteriorated to the extent that walking was impossible. DIAGNOSIS, INTERVENTIONS, AND OUTCOMES: The patient developed quadriplegia with areflexia, axonal-type sensorimotor polyneuropathy was confirmed by nerve conduction study. The patient was diagnosed as GBS. Scrub typhus was also diagnosed as eschar was observed in the chest area and the serologic test of anti-R-tsutsugamushi antibody showed a strongly positive result. The patient received treatment with intravenous immunoglobulin at 0.4 g/kg daily for 5 days. Mechanical ventilation was applied during the intensive care unit. He was treated for scrub typhus simultaneously. Six months after the onset of the disease, the patient showed improvement to the point where he could work and exercise alone. LESSONS: When GBS is suspected, early evaluation and treatment can lead to favorable outcomes. Considering that cases of GBS after COVID-19 vaccination have been reported, it is important to conduct early evaluation and management of patients with muscle weakness after COVID-19 vaccination to ensure early detection of GBS. And even if fever and rash are side effects that can occur frequently after vaccination, it is necessary to consider other diseases in addition to the side effects of the vaccine. This is to prevent delay in diagnosis and treatment of other diseases.