Relapsing polychondritis (RPC) is a rare, immune-mediated condition affecting approximately 3.5 per million population per year. Neurological involvement in RPC is still rarer and is presumed to be the result of a vasculitic process, although this is seldom confirmed in the literature. We present two cases of RPC complicated by cognitive dysfunction with contrasting clinical trajectories. Our findings suggest that there are two clinical phenotypes of cognitive dysfunction in RPC. The first is a fulminant, multisystem presentation with sub-acute cognitive decline mimicking central nervous system vasculitis, and we provide histopathological evidence of this process occurring. The other is an insidious cognitive decline without associated constitutional or systemic symptoms.
Background Therapy related acute leukaemia (TRAL) is a concern for neurologists and patients when considering treatment with Mitoxantrone for Multiple Sclerosis (MS). In 2008 we performed a literature review to determine the risk of TRAL, the associated mortality and potential relationship to total dose exposure. 1 Four years on, there have inevitably been numerous further reports of TRAL associated with mitoxantrone therapy for MS, and we have therefore re-reviewed the literature to attempt to establish a definitive risk and stratify this according to dose. Methods A literature search was undertaken for relevant articles to identify all reported cases of TRAL in MS and data extracted from cases reporting individual exposure (dose or mg/m 2 ) and timing of TRAL. We also identified all case series of >50 patients, reporting follow-up and complications of treatment with Mitoxantrone in MS and combined this with the 250 cases from the Walton Centre, Liverpool treated since 1997. Results Case series including 12,511 patients were identified; mean dose of Mitoxantrone was 90.1 mg/m 2 (range 12-242 mg/m 2 ). TRAL was diagnosed in 0.72%; with a number needed to harm of 139.2 patients. In 142 TRAL cases sufficient data was available to inform analysis of exposure. Onset was a median of 22 months following treatment (range 1-64). APL was the most common leukaemia subtype (58.0%) and AML second most frequent (35.1%). Thirty-seven of 124 TRAL
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