3. Treatment
Prior to diagnosis, IV Methylprednisolone 1g was given for three days with a partial subjective response to limb paralysis but no response as regards her abducens nerve palsy. Following CSF analysis, a combination of chemo- and immunotherapy with intrathecal (IT) Methotrexate (MTX), subcutaneous Rituximab and pulsed oral dexamethasone were urgently commenced. Twenty-four hours post IT-MTX, Ibrutinib 420mg PO OD was introduced. Due to the patient’s neurological deficits and difficulty performing LPs, as well as reluctance to interrupt ibrutinib for LPs, a decision was made to switch from IT-MTX to high dose IV MTX (3.5g/m2) for second and third cycles. In total the patient received the following induction therapy: one IT MTX, two cycles of high dose IV MTX, weekly rituximab for four weeks, four pulses of oral dexamethasone (40mg fortnightly) and oral ibrutinib. This treatment was well-tolerated. A repeat CSF sample was sent for flow cytometry post the above treatment with no clonal population of B cells detected. In addition, the patient’s neurological symptoms responded over weeks from 1/5 power to 4/5 power in her lower limbs. As per the 2014 task force guidelines on BNS these CSF findings in conjunction with the resolution of symptoms suggests a clinical response in keeping with complete remission [1]. The patient received two further doses of subcutaneous rituximab monthly. She currently remains on oral ibrutinib, with a plan to continue this for at least two years.