Neuroimmunology Reports最新文献

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.

A 36-year-old male presented with 2 months of left-eye visual disturbance and was diagnosed with optic neuritis due to probable multiple sclerosis (MS). He was advised to undergo periodic ophthalmology follow-up without immunosuppressive treatment. Due to persistent symptoms, he expressed interest in very high-dose vitamin D₃ therapy of 54,000 IUs/day (1,000 IUs/kg/day) along with a zero-calcium diet. After starting the therapy, he experienced sustained symptomatic improvement of visual symptoms over 4 years, along with radiological stability of the optic neuritis lesion without developing hypercalcemia. This case supports the potential therapeutic efficacy of very high-dose vitamin D for MS.

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

Discussion

Varicella is a benign, self-limiting disease, but both primary VZV infection and VZV reactivation can be life-threatening in immunocompromised children, due to CNS and systemic dissemination of the virus. Reactivation of the vaccine-type VZV (vOka) has been reported sporadically, although SARS COV2 infection may have recently played a role in facilitating VZV reactivation. Here we report on the case of a young boy with Acute Lymphocytic Leukemia, in remission after induction chemotherapy, and recent SARS COV 2 infection. He developed VZV encephalitis due to massive reactivation of VZV virus, complicated by PRES, diagnosed with brain MRI, and Super Refractory Status Epilepticus, lasting until substantial suppression of VZV replication in the CNS. We also report and discuss the role of the remarkably augmented renal clearance persistently observed in our patient, complicating CNS involvement and making both antiviral and antiepileptic treatments more difficult to manage. Effective neuroprotection was completed by physical hypothermia and infusion of IVIG and steroids. The patient obtained complete functional recovery, with reversion of MRI signs of occipital involvement at presentation, at 6-month follow-up. Intense and daily interplay of intensivists, neurologists, hematologists and infectious disease experts likely made his uneventful recovery possible, combining all skills necessary to manage his unusual and complex clinical picture.

Introduction

Case Report

Conclusion

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.

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.