Neuroimmunology Reports最新文献

Pediatric multiple sclerosis (MS) is predominantly a relapsing-remitting demyelinating disorder. Toddler-onset MS (12–36 months) is rarely studied despite the likelihood of early-life adversity influencing its incidence. In this case, we present a 9-year-6-month-old girl who first exhibited symptoms at 30 months. Her disease course reflects the challenging mission of reaching a definitive MS diagnosis. Noteworthy observations include a complex perinatal history, initially normal diagnostic workup, and complete recovery after each relapse. It is crucial to recognize the hidden dynamics of MS, namely smoldering MS and the related progression independent of relapse activity, to commence a high-efficacy early treatment modality and establish a comprehensive multidisciplinary management strategy.

Myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is an idiopathic inflammatory demyelinating disease of the central nervous system commonly associated with optic neuritis, transverse myelitis, acute disseminated encephalomyelitis and cortical encephalitis. A somewhat rare and less recognized syndrome in MOGAD has been identified, and is characterized by cortical encephalitis and seizures with cortical FLAIR-hyperintense lesions on the MRI aptly termed FLAMES (FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures). We report a previously healthy middle-aged man who presented with focal seizures and status epilepticus refractory to initial interventions. He later developed headache, mood and behavioral changes and ultimately tested positive for anti-MOG antibody in the serum using a fixed cell-based assay. When his MRI was re-assessed in retrospect, it was determined to be in keeping with FLAMES.

Neuromyelitis Optica Spectrum Disorders (NMOSD) is an immune-mediated disease of the central nervous system that often leads to severe attacks of optic nerves and spinal cord. The discovery of Aquaporine 4 (AQP4) antibody as a potential biomarker of NMOSD has changed the global approach to this disease. There have been associations between NMOSD and systemic autoimmune diseases, both organ specific and non-organ specific. Here, we aimed to review the literature on co-incidence or possible association of kidney diseases with NMOSD.

Methods

we included All English relevant publications (reviews, original articles, case reports, case series and abstracts) from 1998 to 2023 using PubMed and google scholar database for this review.

Results

Nine case reports of co-existing NMOSD with renal disorders were evaluated. The patients were involved from 10–90 years old. The most common association was with nephrotic syndrome and in the context of systemic autoimmune disorders. However a case of NMOSD coincident with renal cell carcinoma without paraneoplastic etiology was reported.

Conclusion

Despite the expression of AQP4 in the collecting duct cells of kidney, whether AQP4-IgG, the pathogenic antibody in NMOSD, can damage to the kidney is still unknown.

Can structural kidney damages lead to AQP4 antigen being exposed to immune system and triggers the cascade of autoimmunity is a question that should be investigated in future studies.

Background

Azathioprine (AZA) remains a widely used treatment for neuromyelitis optica spectrum disorder (NMOSD). It heightens the risk of myelosuppression, a risk further intensified with the addition of allopurinol.

Case presentation

To the best of our knowledge, there have been no reported cases of this complication in NMOSD.

Case report

We present a 63-year-old female with NMOSD on AZA, who experienced severe myelosuppression upon initiating allopurinol. Following the discontinuation of these drugs, we prescribed granulocyte stimulants, recombinant erythropoietin, and supportive care, which resulted in hematologic improvement.

Conclusions

This case highlights a potential myelosuppression risk when combining AZA and allopurinol in NMOSD patients.

Background

Orbital apex syndrome as the presentation of neuromyelitis optica spectrum disorder has not been reported in literature.

Case presentation

We reported a case in which the patient initially presented with ptosis, diplopia, ophthalmoplegia and visual impairment of the right eye. Clinical examination revealed no light perception in the right eye with the presence of relative afferent pupillary defect and involvement of cranial nerves II, III, V, and VI. Magnetic resonance imaging of the cranial and orbital regions revealed thickening in the intraorbital segment of the right optic nerve with T2-weighted hyperintensity, and Gadolinium-enhancement of the right optic nerve and its myelin sheath. Longitudinally extensive transverse myelitis subsequently developed, with detection of serum anti-aquaporin-4 antibody. Intravenous methylprednisolone was initiated, leading to significant clinical improvement.

Conclusion

This case report highlights the diverse manifestations of neuromyelitis optica spectrum disorder, including the orbital apex syndrome.

Background

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disease of the central nervous system. Relapse may be moderate to severe with an Expanded Disability Status Scale (EDSS) above 4.0 in half of patients, albeit most experience good to excellent motor recovery.

Results

Herein, we present an atypically severe case of MOGAD with an unusual clinical course. Patient initially presented with diplopia, lower limb motor deficit and hypoesthesia which rapidly deteriorated into quadriplegia. Corticosteroid regimen did not initially translate to significant clinical improvement (EDSS=9.0). At the 1 year follow up, patient had regained some mobility although with marked sequela (EDSS=6.5).

Conclusion

Although MOGAD is generally thought to be benign, it could also present with severe episodes.

Introduction

Susac syndrome (SuS) is a rare autoimmune disease characterized by the clinical triad of brain dysfunction, branch retinal artery occlusion, and hearing loss. Many cases have been reported with irreversible sequelae due to misdiagnosis and late treatment of the disease.

Case

In this article, we describe the clinical course and diagnosis of SuS in a young woman with a history of type 1 diabetes mellitus and kidney transplantation who presented with the complete triad. The complex medical history required a tailored treatment approach, including plasmapheresis and rituximab, which significantly improved her condition.

Conclusion

This report highlights the diagnostic complexity of SuS and the value of personalized treatment strategies, contributing to the understanding of this rare disorder, and briefly reviews the current knowledge of the disease and discusses the probable pathophysiological relationship between SuS and kidney failure.

A 52-year-old lady developed apathy, reduced verbal output, decreased visuospatial orientation, cognitive decline, visual impairment and left hemiparesis for 4 months. On MRI head, she displayed T2/FLAIR asymmetric white matter hyperintensities in parietal and occipital lobes and cerebellar peduncle and her CSF PCR was positive for John Cunningham (JC) virus and HIV ELISA and other bacterial, mycobacterial and viral markers were negative, confirming Progressive multifocal leukoencephalopathy (PML). Thereafter, we administered monthly intravenous immunoglobulin (IVIg) and patient's condition and neuroimaging has been improving for past 8 months. Hence, IVIg improving the condition of a potentially fatal disease patient, offers some hope.

Background

Multiple sclerosis (MS) is a chronic autoimmune disease of the central nervous system characterized by inflammation, demyelination, gliosis, and neuronal loss. The risk of relapse may increase in the perioperative period in surgical interventions, especially in cardiac surgeries performed using cardiopulmonary bypass (CPB) and the use of CPB with MS patients is controversial.

Case presentation

In this case report, the management of a patient with MS to reduce the risk of relapse in the perioperative period is described and a case of coronary artery bypass graft (CABG) performed with CPB without any problems is presented.

Conclusions

CABG with CPB can be performed successfully in MS patients. Adjusting the operation time and neurological medications, closely monitoring body temperature and electrolytes in the intraoperative and postoperative period, and ensuring hemodynamic stability can reduce the risk of relapse and infection.

Background

Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease that may coexist in the multiple sclerosis population and remain undiagnosed. AIH has previously been reported following treatment with interferon beta, glatiramer acetate, natalizumab and high dose corticosteroids in people with multiple sclerosis (MS).

Case presentation

We present a rare case of autoimmune hepatitis onset after B cell depletion with ocrelizumab in a person with multiple sclerosis (MS).

Case report

Two weeks after the second dose of ocrelizumab, patient presented with jaundice and acute liver injury. Liver biopsy revealed pathological features of autoimmune hepatitis with negative workup for viral etiologies. A six month steroid taper and azathioprine resulted in normalization of liver function tests and clinical and radiological stability of multiple sclerosis and AIH at two years of follow-up.

Conclusions

Whether B cell depletion with ocrelizumab directly resulted in emergence of AIH is unclear. Regulatory B cells have been proposed to play a protective role in AIH pathogenesis and B cell depletion may provoke the emergence of AIH through loss of regulatory B cells.