Chemotherapyinduced autoimmunemediated encephalitis throughout germinoma treatment

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by persistent presence of positive antiphospholipid antibodies (aPLs). Historically, aPLs first attracted attention at the beginning of the 1970s, as a risk factor for the development of myelitis via cross-reaction with cephalin and sphingomyelin as neuronal tissue-enriched phospholipids. Primary APS manifestations include arteriovenous thrombosis and pregnancy complications; however, in rare cases, aPL-related neurological disorders including cognitive dysfunction, chorea, and transverse myelitis are observed." The pathogenesis and therapeutic strategies for thrombosis, including cerebral infarction in APS, have been established from basic and clinical research to date. However, pathological insights and clinical perspectives have not yet been well-defined for aPL-related cognitive dysfunction, chorea, and myelitis. Based on past experiences and findings of small observational studies, some patients with aPL-related neurological disorders recover following antiplatelet and anticoagulation therapy, but in some cases, multidisciplinary treatments with glucocorticoids and/or immunosuppressive agents, including cyclophosphamide, azathioprine, mycophenolate and rituximab, plasmapheresis, and psychoeducational support are required. A detailed research on pathophysiology and nationwide or international multicenter clinical trials for therapeutic strategies are vital for establishing sufficient basic and clinical understanding of aPL-related neurological manifestations.Damage of the central and peripheral nervous systems associated with systemic lupus erythematosus (SLE) is termed neuropsychiatric SLE (NPSLE). In this review, we have discussed SLE encephalopathy, which is associated with neurological symptoms in particular. At the time of diagnosis, disease severity should be evaluated based on clinical findings, imaging, laboratory tests, including cerebrospinal fluid tests and neurophysiological tests, of the patient. Subsequently, treatment involving both definitive therapy and symptomatic treatment is initiated. After introducing definitive therapy, it is further desirable to adopt an appropriate treatment approach by identifying the predominant type of pathogenesis (inflammatory or vascular). A collaborative approach involving specialists in collagen vascular disease, neurologists, and psychiatrists is important for appropriate management of NPSLE.Primary central nervous system (CNS) vasculitis is an uncommon disorder of unknown etiology that is restricted to the brain and spinal cord. Patients do not present with specific clinical features or a classical clinical course, and no blood or imaging investigations are available for diagnostic confirmation. Cerebral biopsy and angiography are the gold standards for diagnosis. No randomized clinical trials have described a therapeutic regimen effective for primary CNS vasculitis; therefore, treatment of primary CNS vasculitis is based on therapeutic strategies used for other types of vasculitis. Early diagnosis is important because corticosteroid-based treatment with or without concomitant cyclophosphamide administration can often prevent serious outcomes and may be followed by a favorable response. Several immunosuppressants such as mycophenolate mofetil, tumor necrosis factor-α blockers, and rituximab may be useful options for patients refractory to the aforementioned regimen. We describe some diagnostic and therapeutic approaches for the management of this condition, with a focus on the importance of obtaining tissue and angiographic evaluation (formal contrast-enhanced or magnetic resonance/computed tomography angiography).Granulomatosis with polyangiitis (GPA) is designated as an intractable disease by the Ministry of Health, Labor and Welfare, Japan, and is classified as an antineutrophil cytoplasmic antibody (ANCA)-related vasculitis syndrome. It is associated with upper respiratory tract symptoms (E; ear and nose), pulmonary symptoms (L; lung), renal symptoms (K; kidney), and systemic vasculitis symptoms, and often involves the central/peripheral nervous system. Patients with GPA can be easily diagnosed as they often show positive serum C (Proteinase 3)-ANCA findings. Remission can be induced using multiple immunosuppressants in combination, but caution is required as relapse and infection is common in patients with GPA.Giant cell arteritis (GCA), also referred to as temporal arteritis, is a variant of large-vessel vasculitis. GCA should be considered in the differential diagnosis in patients aged >50 years, who present with headache, abrupt onset of visual disturbances, unexplained fever, a high erythrocyte sedimentation rate, and high serum C-reactive protein levels. Diagnosis of GCA is based on accurate interpretation of laboratory data, temporal artery biopsy findings, and imaging study results. 1,2,3,4,6-O-Pentagalloylglucose clinical trial Imaging modalities used for GCA include positron emission tomography, computed tomography (CT), CT angiography, and conventional magnetic resonance angiography. Biopsy is the gold standard for diagnosis of GCA. Glucocorticoids (GCs) are used as standard treatment to induce remission and also for maintenance therapy. However, clinicians should be aware of the adverse effects of GC treatment, including hyperglycemia, an immunocompromised state, and delirium. If adverse effects outweigh the benefits of GC treatment, it is necessary to consider switching to or adding an immunosuppressant to the therapeutic regimen.Cerebral amyloid angiopathy (CAA) is a disorder characterized by the deposition of amyloid in the leptomeningeal and cortical blood vessels. Sporadic amyloid β (Aβ)-type CAA is the most common form of CAA. Although CAA is a well-known cause for recurrent cerebral lobar hemorrhage, inflammation, and vasculitis, CAA-related inflammation/vasculitis (CAA-ri/vasculitis) induced by Aβ deposition on vessel walls is emerging as a treatable condition. The estimated total number of cases of and prevalence of CAA-ri/vasculitis in Japan were 170 and 0.13 per 100 000 population, respectively. Patients with CAA-ri/vasculitis show acute or subacute-onset of cognitive impairment, behavioral changes, and headache. Brain magnetic resonance imaging, showing asymmetrical white matter abnormalities and occasional meningeal enhancement, is a useful tool for the diagnosis of CAA-ri/vasculitis. Moreover, elevation of anti-Aβ antibodies and inflammatory markers in the cerebrospinal fluid can help in clinical diagnosis. Although several clinical diagnostic criteria have been proposed, neuropathological examination of a brain biopsy remains the gold standard for detecting severe Aβ deposition and vasculopathic changes with lymphocytic infiltrations and/or granulomatous vasculitis.