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D the diagnostic criteria for AAV [8] with central nervous system (CNS) involvement. Indeed, he had positivity for p-ANCA along with systemic signs of disease (glomerulonephritis and pulmonary interstitiopathy), histological proof of vasculitis and no other diagnosis accounting for the clinical picture. MRI picture could suggest a "posterior reversible encephalopathy syndrome or PRES [9]. However, contrast enhancement at MRI, normality of blood pressure, and absence of worsening of kidney function during SE, does not support this hypothesis. Patient 2 also had positivity Staurosporine PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/17139194 of pANCA, in the presence of non-specific signs of vasculitis (fever, lymphadenopathy and haemolytic anaemia) along with absence of other diagnosis accounting for the clinical picture, thus fulfilling only 2 out of 3 necessary criteria. However, the positivity of p-ANCA prompted us to start treatment with rituximab with good outcome. Patient 3 also fulfilled 2 out of 3 proposed criteria for AAV (positivity for p-ANCA and exclusion for other conditions accounting for her symptoms, including the above-mentioned diseases). An atypical form of Rasmussen encephalitis could besuggested in this patient [10]. However, the clinical findings as well as the extensive laboratory investigation, including the MRI features (with no characteristic evolutional changes of MRI occurring at long-term follow-up, including the atrophy of the head of the caudate nucleus) made this diagnosis very unlikely. Therefore, all these data, along with the prompt response to azathioprine treatment, strongly suggested the diagnosis of an autoimmune disorder with p-ANCA positivity limited to CNS in this young patient. Of interest, in the same patient, we also excluded genetic conditions potentially leading to focal brain lesions and seizure disorder, including POLG mutations. Indeed, it is known that POLG mutations may cause a progressive neurological disorder usually starting in teens with occipital lobe epilepsy and frequent SE [11]. This observation suggests that AAV should be considered in the differential diagnosis with POLG mutations in patients presenting with occipital seizures or SE. A study [12] on natural history of AAV found that average patient survival is about five months and more than 90 of patients die within two years. Standard treatment of AAV usually consists of inducing remissions with high dose steroids or cyclophosphamide, and maintaining remission with immunosuppressants. New therapeutic agents include rituximab, a monoclonal antibody depleting B cell, approved by FDA in US for use in AAV. The striking and long-lasting response in patient 2 confirms that rituximab may represent a therapeutic alternative to classic immunosuppressant for AAV in some patients. Patient 3 too had a good outcome and remained seizure-free at follow-up with immunosuppressant and antiepileptic treatment. On the contrary, patient 1 had extremely rapid and fatal evolution due to small vessel necrotizing vasculitis and multiple organs bleeding despite treatment with steroids. The exact nature of cortico-subcortical hyperintensities associated with SE remains elusive, even if such changes may be at least in part related to cytotoxic edema [13]. Regardless the pathophysiological mechanism, we believe that the present series strengthens the view that both SE and brain hyperintensities should be targets of rational treatment, as there is now agreement that SE and its combination with sterile brain inflammation a.