Leukocytoclastic vasculitis (LCV) is a histopathologic descriptor for a prevalent type of small-vessel vasculitis (SVV) that affects arterioles, capillaries, and postcapillary venules. Although the association between vasculitis and malignancy only accounts for <5% of vasculitis cases, it has been recognized as a true paraneoplastic syndrome in several studies. A 57-year-old Filipino male presented with erythematous, nonblanching macules on his lower extremities, which rapidly progressed to violaceous lesions on his trunk, buttocks, and lower extremities. He also reported significant weight loss, decreased appetite, and vomiting. A skin biopsy confirmed LCV. Initially treated for meningococcemia, his condition did not improve. Abdominal imaging revealed an enlarged heterogeneous liver with retroperitoneal lymphadenopathy and a parenchymal nodule. He was eventually diagnosed with vasculitis secondary to an underlying hepatic malignancy and expired later from multiorgan failure.

INTRODUCTION

Demyelinating central nervous system (CNS) disorders such as transverse myelitis (TM) and multiple sclerosis (MS) have been reported with mRNA Covid-19 vaccine. Some cases were relapses of a pre-existing condition but de novo and initial presentation of MS after BNT162b2 COVID-19 mRNA vaccine has very rarely been documented.

We report a 72-year-old female, right-handed, Filipina, with a one-month history of bilateral lower extremity weakness which occurred 7 days after she received her first booster dose of BNT162b2 mRNA vaccine. This was later accompanied by fecal and urinary incontinence. On examination, she had motor deficit below L1 myotome manifesting with loss of hip flexion, knee extension, dorsiflexion, and plantar flexion. There was also sensory deficit below T10 level with relative 80% sensation of vibratio, proprioception, light touch and complete loss of pain and temperature sensation. The initial impression was Transverse Myelitis which may be related to a post-vaccination state. Spinal magnetic resonance imaging (MRI) revealed long segment enhancing T2W hyperintense lesion at T2 to T7. Cranial MRI revealed ovoid areas of heterogeneous, predominantly T2/FLAIR hyperintense signals exhibiting restricted diffusion in the periventricular white matter of the fronto-parietal lobes. Cerebrospinal fluid (CSF) analysis was negative for infectious causes such as tuberculosis but with high levels of CSF immunoglobulin G. She was then diagnosed to have Multiple Sclerosis (MS) and was treated with high dose oral prednisone. However, there was no improvement in neurological deficits on follow-up.

This case adds to the reported rare cases of initial presentation of MS occurring after vaccination for COVID-19 and the first reported case in the Philippines. Early recognition and prompt treatment is important to improve outcomes.

Dermatomyositis (DM) is a rare autoimmune myopathy characterized by progressive muscle weakness that typically affects the shoulder and hip girdle first. It is a multisystem disorder characterized by symmetric proximal, extensor, inflammatory myopathy, vascular involvement and a constellation of pathognomonic (Gottron papules, Gottron Sign, Heliotrope rash) and characteristic cutaneous eruptions, which frequently presents in the fifth and sixth decades. As adult DM presents as a paraneoplastic syndrome in up to 30% of cases, metastatic workups appropriate for age are warranted.

Humans ; Herpesvirus 3, Human ; Myelitis, Transverse/complications* ; Guillain-Barre Syndrome/complications* ; Herpes Zoster/complications* ; Varicella Zoster Virus Infection/complications*

OBJECTIVES: To study the clinical features of children with autoimmune encephalitis (AE) secondary to epidemic encephalitis B (EEB). METHODS: A retrospective analysis was performed on the medical data of five children with EEB with "bipolar course" who were treated in Children's Hospital Affiliated to Zhengzhou University from January 2020 to June 2022. RESULTS: Among the five children, there were three boys and two girls, with a median age of onset of 7 years (range 3 years 9 months to 12 years) and a median time of 32 (range 25-37) days from the onset of EEB to the appearance of AE symptoms. The main symptoms in the AE stage included dyskinesia (5/5), low-grade fever (4/5), mental and behavioral disorders (4/5), convulsion (2/5), severe disturbance of consciousness (2/5), and limb weakness (1/5). Compared with the results of cranial MRI in the acute phase of EEB, the lesions were enlarged in 3 children and unchanged in 2 children showed on cranial MRI in the AE stage. In the AE stage, four children were positive for anti-N-methyl-D-aspartate receptor antibody (one was also positive for anti-γ-aminobutyric acid type B receptor antibody), and one was negative for all AE antibodies. All five children in the AE stage responded to immunotherapy and were followed up for 3 months, among whom one almost recovered and four still had neurological dysfunction. CONCLUSIONS EEB can induce AE, with anti-N-methyl-D-aspartate receptor encephalitis as the most common disease. The symptoms in the AE stage are similar to those of classical anti-N-methyl-D-aspartate receptor encephalitis. Immunotherapy is effective for children with AE secondary to EEB, and the prognosis might be related to neurological dysfunction in the acute phase of EEB.
Male ; Female ; Humans ; Child ; Infant, Newborn ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; Retrospective Studies ; Hashimoto Disease/therapy* ; Encephalitis, Arbovirus

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune inflammatory disease of the central nervous system, and little is known about its immune mechanism at present. There is a lack of disease-related biomarkers in cerebrospinal fluid except anti-NMDAR antibody, which leads to delayed diagnosis and treatment in some patients. Therefore, there has been an increasing number of studies on related cytokines in recent years to assess whether they can be used as new biomarkers for evaluating disease conditions and assisting diagnosis and treatment. Current studies have shown that some cytokines may be associated with the progression of anti-NMDAR encephalitis, and this article reviews the research advances in such cytokines associated with anti-NMDAR encephalitis.
Humans ; Cytokines ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/therapy* ; Biomarkers

To investigate the risk factors of poor prognosis and recurrence in patients with anti-NMDAR encephalitis. A single center, observational cohort study was used to retrospectively analyze 44 patients with anti NMDAR encephalitis hospitalized in the Department of Neurology of Beijing Tong Ren Hospital from January 2014 to October 2020. The results showed that the interval from onset to immunotherapy in the poor prognosis group was significantly longer than that in the good prognosis group (t=2.045,P=0.047), and the course of disease in the poor prognosis group was significantly longer than that in the good prognosis group (t=4.127,P=0.000 2). The number of patients with clinical manifestations of dyskinesia was significantly increased (Fisher exact test: P=0.014). The patients with abnormal brain MRI in the poor prognosis group were significantly more than those in the good prognosis group (Fisher exact test: P=0.017), and the patients with slow wave>50% in the poor prognosis group were significantly more than those with slow wave <50% (Fisher exact test: P<0.001). Patients with the first onset of immunotherapy time <3 months, long course of disease, high intracranial pressure, and high cerebrospinal fluid protein are prone to relapse. Bivariate logistic regression analysis showed that patients with dyskinesia, abnormal brain MRI, and slow wave EEG more than 50% were risk factors for poor prognosis (OR values were 4.687, 4.978, and 24.500, respectively; P values were 0.018, 0.016, and 0.000, respectively). The time of first-line immunotherapy for the first onset<3 months was the risk factor for recurrence (OR 17.231, P=0.010). In conclusion, dyskinesia, abnormal brain MRI and slow wave of EEG more than 50% may be the risk factors for poor prognosis of patients. The duration of immunotherapy less than 3 months after the first onset might be the risk factor for recurrence.
Humans ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/cerebrospinal fluid* ; Retrospective Studies ; Neoplasm Recurrence, Local ; Risk Factors ; Dyskinesias

Humans ; Soft Tissue Neoplasms ; Mesenchymoma/diagnostic imaging* ; Paraneoplastic Syndromes/pathology*

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a central nervous system disease characterized by neurological and psychiatric symptoms. Immunotherapy is the basic treatment for this disease, including first- and second-line therapies for the acute stage and the long-course therapy for the chronic stage. Anti-NMDAR encephalitis often has a good prognosis, but some patients may still have neurological dysfunction due to poor response to current immunotherapy. In addition, the adverse reactions and economic burden of drugs are practical problems in clinical practice. To solve the above problems, continuous improvements have been made in immunotherapy regimens in terms of dose, route of administration, and course of treatment, and some new immunotherapy drugs have emerged. This article reviews the recent research on immunotherapy for anti-NMDAR encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; Humans ; Immunotherapy ; Receptors, N-Methyl-D-Aspartate ; Retrospective Studies

Objective: To evaluate the clinical characteristics, treatment, and prognosis of patients with paraneoplastic neurological syndrome (PNS) associated with lymphoma. Methods: Between January 2012 and May 2021, the clinical data of 11 patients with lymphoma complicated with PNS treated at Peking Union Medical College Hospital were retrospectively reviewed. Results: Among the 11 patients (8 male and 3 female) , the median onset age was 61 (range, 33-78) years. The symptoms of PNS preceded lymphoma in 10 patients. The median time from the onset of PNS to the diagnosis of lymphoma was 4 months. Of the 11 patients, one had Hodgkin's lymphoma, 8 had B-cell non-Hodgkin's lymphoma, and 2 had peripheral T-cell lymphoma. Seven patients were evaluated for onconeural antibody, of whom 2 were positive (1 for anti-Ma2 antibody and 1 for anti-Yo antibody) . Of the 11 patients, the PNS symptoms of 3 patients were located in the central nervous system, 4 were located in the peripheral nervous system, and 3 were located in the muscle. Eight of the 11 patients were treated with glucocorticoid-based immunosuppressive therapy before the diagnosis of lymphoma. Patients with central nervous system involvement and dermatomyositis responded well to glucocorticoid, whereas patients with peripheral neuropathy did not significantly benefit. All 11 patients were treated with chemotherapy after the diagnosis of lymphoma. The efficacy of chemotherapy was assessed in 9 patients, 7 cases achieved complete remission, 1 case was evaluated as stable disease, and 1 case was evaluated as disease progression. The PNS symptoms of the patients who achieved complete response were almost completely recovered. The median follow-up time was 42 (range, 4-95) months. At the end of the follow-up period, 6 of the 11 patients survived, 3 were lost to follow-up, and 2 died. The median overall survival of the whole group was not reached. Conclusions: PNS can involve various parts of the nervous system and can be associated with different types of lymphoma. Through early diagnosis and treatment, the PNS symptoms could improve in most patients who achieve complete remission of lymphoma.
Adult ; Aged ; Antibodies, Neoplasm ; Autoantibodies ; Female ; Glucocorticoids ; Humans ; Lymphoma/diagnosis* ; Male ; Middle Aged ; Paraneoplastic Syndromes, Nervous System/complications* ; Retrospective Studies