190 Nusinersen treatment in adults with spinal-muscular-atrophy with complex-spinal anatomy at St. George’s Hospital, London

We describe nusinersen, the only licensed treatment for adults with SMA in the UK, administra- tion with Single-Plane-Fluoroscopic-Guided(SPFG), Bi-Plane-Fluoroscopic-Guided(BPFG), or Computerised-Tomography-guided(CTG).We treated three patients with type-2-SMA (two with Severe-Scoliosis[SS], one with Posterior-Spinal- Fusion[PSF]) and four with type-3-SMA (two ambulant, one with Normal-Spine[NS] and one Mild-Lumbar- Spinal-Deformities[MLSD] and two non-ambulant; one each with PSF and MLSD) aged 17 to 57-years. All had homozygous exon-7 and 8 deletions. One had two copies, four had three copies, and the remainder had four copies of SMN-2-gene.At baseline, forced-vital-capacity was between 38% to 153%. Ambulant patients were assessed with HFMSE(score 37-60), RHS(scores 31-67) and, 6MTM(range 43-492m), and non-ambulant patients were assessed with CHOP-ATTEND(score 17-38), RUL(2-33), 9-hole-peg-test and dynamometer as appropriate.In type-2, patients with SS had a total of 6(20.7%) BPFG-injections. Only one had severe hypokalemia requiring ITU and treatment cessation. In one with PSF, BPFG-injection was unsuccessful.In type-3, SPFG-injections were successful in all 19 attempts with MLSD or NS. One each was complicated by transient low-pressure symptoms and radicular pain. Three CTG-injections on a patient with PSF were uncomplicated. All with successful LPs(96.5%) did not drop out.We found that nusinersen could be administered regardless of the spinal anatomy with interventional radiology.