Neuroimmunology Reports最新文献

Background

Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently identified and recurrent inflammatory demyelinating disease of the central nervous system (CNS) in both adults and children. Although MOGAD acute attacks usually appear to be very responsive to high dose steroids and plasma exchange, the long-term side effects of steroids should be highlighted especially in children. Attack-prevention treatments also lack of class-I evidence and prospective randomized placebo-controlled trials are needed to better guide prevention treatment in refractory cases. Long-term immunosuppressive maintenance with mycophenolate mofetil, azathioprine or rituximab may not always effective in patients experienced recurrent demyelinating attacks.

Case presentation

We reported a rare case of 17-year-old MOGAD patient who experienced eleven relapses with azathioprine, mycophenolate mofetil and rituximab in five years, and finally effectively treated with ofatumumab (OFA), a novel fully humanized anti-CD20 mAb.

Conclusions

Timely and efficient treatment is crucial for better prognosis in refractory MOGAD. The fully humanized OFA may be an optimal choice if multiple therapeutic drugs fail to achieve remission. Further research on comparing OFA and RTX in MOG patient is needed.

Diaphragmatic pacing (DP) has not been well-studied in adults with central respiratory failure secondary to inflammatory disorders of the brain and spinal cord. We present two novel cases of DP in the treatment of respiratory failure secondary to brainstem and high cervical spinal cord lesions in neuromyelitis optica spectrum disorder patients. In case 1, DP facilitated tracheostomy reversal and the patient was successfully weaned from DP. In case 2, DP helped improve chronic hypercapnia and the frequency of apneic events. Early initiation of DP should be considered in this patient population to treat central hypoventilation and central sleep apnea.

Background

VZV myelitis is a rare and typically monophasic complication of VZV reactivation. There is no solid evidence for a particular treatment regimen for VZV myelitis, especially in recurrent disease. No prior reports or studies have looked at using intravenous immunoglobulin for this condition, particularly for refractory cases.

Case report

A 75-year-old female presented with paresthesia on the right lateral leg, followed by a vesicular rash in a T2 dermatomal distribution. Over a period of 2 weeks, she experienced bilateral lower extremity weakness. MRI revealed a C3-C6 enhancing lesion. VZV positivity was confirmed by skin biopsy. She was treated with 5 days of Solumedrol 1gm IV and valacyclovir 1gm TID with improvement of weakness and rash. However, over the next two years, she continued to get occasional disseminated vesicular rash with each flare of her myelitis despite continued valacyclovir. She was started on monthly IV Ig (1gm/kg IV Ig over 2 days) for her recurrent myelitis. Since starting monthly IV Ig there have been no further zoster outbreaks or episodes of myelitis. Her balance and gait have improved significantly.

Conclusion

We present an unusual case of recurrent disseminated zoster with myelitis successfully treated with monthly IV Ig. The successful treatment of this patient should prompt consideration for its use in similar cases of recurrent VZV myelitis and may provide insight for future studies on how to treat VZV-related diseases.

Background

Anti-N-methyl-d-aspartate [anti-NMDA] receptor encephalitis is one of the most common types of immune-mediated encephalitis. It is characterized by a neuropsychiatric syndrome associated with immunoglobulin G [IgG]-type antibodies against the NMDA receptor's GluN1 subunit in cerebrospinal fluid [CSF]. To our Knowledge, only six cases of autoimmune encephalitis with positive CSF NMDA receptor IgG autoantibodies have been reported among patients on long-term immunosuppressive therapies. The etiologies of all six cases were related to solid organ transplantation. Therefore, this report is the first to present a case of immunosuppression associated with an etiology other than post-transplant immunosuppression.

Case presentation

We present the case of a 59-year-old female patient who was on a long-term continuous immunomodulatory therapy for rheumatoid arthritis, and she consulted the emergency department several times for headache, symptoms of neurologic deficit, and subsequent progression to neuropsychiatric symptoms. CSF examination of the patient confirmed the presence of antibodies against the NMDA receptor; hence, the diagnosis of anti-NMDA receptor encephalitis was made.

Conclusions

It is important to recognize the clinical features of autoimmune encephalitis and to consider this condition in the differential diagnosis when neuropsychiatric symptoms are manifested in such patients. Patients on long-term immunomodulatory therapy present with atypical manifestations of anti-NMDA receptor encephalitis. The consideration of these manifestations in the differential diagnosis can ensure efficient diagnosis, management, and reduction in the consequences affecting morbidity and mortality of these patients.

A 20-year-old woman presented with ataxia, right-sided hemiparesis, headache, and fever. Brain MRI showed a large non enhancing pontine T2 hyperintense lesion with slight diffusion restriction. Her clinical condition progressed to quadriparesis, dysarthria and ophthalmoplegia. The patient was diagnosed with tumefactive multiple sclerosis. She had a positive response to plasma exchange and cyclophosphamide, and ultimately returned to running at 1 year follow up. De novo tumefactive demyelination, diagnosis, and indications for aggressive treatment are discussed.

Susac syndrome is a rare autoimmune endotheliopathy characterized by occlusion of small arteries in brain, retina, and cochlea presenting with triad of encephalopathy, retinal vaso-occlusive disease and hearing loss. The disease can be monophasic or relapsing with variety of symptoms and the majority of the patients do not have the classic triad, especially at the initial clinical presentation. In CNS neuroinflammaory disorders, refractory hiccup is a recognized manifestation of NMOSD area postrema syndrome. To our knowledge, this is the first report of Susac syndrome presenting with encephalopathy and refractory hiccup.

Background

: Baló concentric sclerosis (BCS) is a rare demyelinating disorder that overlaps with other demyelinating diseases. BCS usually present in the context of multiple sclerosis (MS) or preceding typical MS. It rarely presents as an isolated lesion, . IV methyl prednisolone (IVMP) is the mainstay in the treatment with various outcomes. Maintenance therapy is still not clearly defined.

Case presentation/case report: We present a case of isolated BCS that responded clinically and radiologically to long-term rituximab therapy.

Conclusion

: A definite guideline for treating patient with BCS either with MS or as isolated entity is still controversial. Our case reflects a remarkable response to rituximab in achieving clinical and radiological long-term remission.

Background

VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic) is a recently described rare novel autoinflammatory syndrome characterized by systemic inflammatory and hematological symptoms. Based on reported cases thus far, central nervous system involvement is exceptionally infrequent.

Case report

A 77-year-old man had a five-year medical history of chronic fluctuating fever, weight loss, skin lesions, and dyshematopoiesis. He presented with acute headache, vomiting, and generalized weakness, and in the following days, he also developed dysarthria, diplopia, and rapidly decreasing consciousness. Investigations showed brainstem encephalitis with no evidence of concurrent infection. Despite treatment with high-dose Solu-Medrol, the patient died 20 days after hospitalization. Genetic screening confirmed the diagnosis of VEXAS syndrome.

Conclusion

This case presents a rare case of rhombencephalitis in a patient with VEXAS syndrome. There is a wide array of etiologies in rhombencephalitis, and clinicians may need to consider VEXAS syndrome in the diagnostic work-up if there is a history of systemic inflammatory symptoms.

Background

DPPX encephalitis, an ancillary, regulatory protein of the Kv4.2 potassium channel Dipeptidyl-peptidase-like protein-6, is caused by cell surface autoantigens to DPPX, and is considered a rare but treatable autoimmune disease. Patients generally start with gastrointestinal symptoms (diarrhea and abdominal pain) or weight loss, and then precede the central nervous system (CNS) hyperexcitability with or without cognitive dysfunction. Here we reported a DPPX-associated patient, whose titer of DPPX antibody was inconsistent with clinical symptoms.

Case presentation

This study presents a case exhibiting mild symptoms of DPPX encephalitis. A 30-year-old male suffered from mild memory loss after a fever, which could be easily misdiagnosed as influenza or other common diseases. Immunological examination based on cell-based assay (CBA) detected DPPX antibody in both cerebrospinal fluid and serum. All the symptoms recovered after the administration of immunotherapy within 3 weeks. The DPPX titer in serum, however, became higher at the same time. Six months later, the re-examination of the cerebrospinal fluid immunological test was negative for the DPPX antibody.

Conclusions

In view of the symptoms of DPPX-associated encephalitis and the fact that changes in antibody titers in CSF are more likely to reflect symptomatic changes, we suggest that clinicians actively perform lumbar puncture in suspected patients to avoid misdiagnosis or misjudgment of the disease.