Langerhans cell histiocytosis with refractory central nervous system involvement responsive to infliximab: Figure 1

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Abstract
A 48-year-old man presented with a 14-month history of gradually progressive disequilibrium, imbalance, oscillopsia, dysarthria and cognitive impairment. Sixteen months prior to presentation to our clinic, he developed a hard lump over the right frontal bone and biopsy confirmed the diagnosis of LCH on the basis of typical cellular morphology and strongly positive staining with CD1a and S100. Initial assessment suggested that the disease was limited to the skull, but progress CT imaging 2 months later demonstrated a separate, asymptomatic right intraconal orbital lesion. At that time he developed, but did not initially report, mild persistent disequilibrium and oscillopsia. Therapy (see online supplementary data for details) with prednisolone, etoposide and vinblastine resulted in regression of both lesions, but relapsed systemic disease with biopsy-confirmed bone marrow infiltration and abdominal masses 6 months later necessitated salvage therapy with monthly intravenous methotrexate and cytarabine, truncated at 2 months by an idiosyncratic febrile reaction and a possible seizure. Investigations including CT imaging of the brain and cerebrospinal fluid examination at that time were unremarkable. Intravenous cladribine therapy, initiated 3 months later for persistent visceral disease, ultimately resulted in complete systemic remission over 10 months, but had no effect on the neurological symptoms; progressive cerebellar dysarthria and imbalance prompted referral to our clinic.

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