Objective:To describe clinical, radiological and pathological features of patients with Baló’s concentric sclerosis (BCS) and assess overlap between BCS and other central nervous system inflammatory demyelinating diseases.Methods:Retrospective review of BCS cases from US and Australian tertiary care centers.Results:We identified 40 BCS cases with 38 available MRIs. Solitary MRI lesions were present in 26% (10/38). We saw >1 active concurrent BCS lesion in 45% (17/38). A third (13/38) had multiple sclerosis-suggestive lesions on the index MRI, of which 10 fulfilled Barkhof criteria. In patients with serial MRI performed within one month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially non-enhancing or centrally enhancing lesions subsequently developed single or multiple enhancing rings (41%; 9/22) and incomplete enhancing rings (14%; 3/22). Discordance between rings as they appear on ADC, DWI, and gadolinium-enhanced imaging was observed in 67% (22/33). AQP4-IgG (n=26) and MOG-IgG (n=21) were negative in all patients with serum available. Clinical response to steroid treatment was seen in 46% (13/28). A monophasic clinical course was present in 56% (18/32) at last follow-up (median 27.5 months; range 3-100 months). The initial attack was fatal in 10% (4/40). Median time from symptom onset to death was 23 days (range 19-49 days). All 17 patients with pathology available demonstrated typical findings of multiple sclerosis. Patients with active demyelinating lesions all demonstrated oligodendrocytopathy (pattern III).Conclusions:BCS may be a distinct subtype of multiple sclerosis characterized by pattern III immunopathology.
Autoimmune manifestations are a common occurrence with chronic lymphocytic leukaemia (CLL). We describe a case of CLL-associated immune thrombocytopenia (ITP) that had a loss of response to standard treatment for ITP. The thrombopoeitin receptor agonist, eltrombopag, was successfully used preoperatively to increase the platelet count to a safer level, in this instance to facilitate laparoscopic splenectomy.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.