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.