We report our experience of the monitoring of spinal somatosensory evoked potentials in 60 patients with neuromuscular scoliosis. In 15 cases a significant change occurred in the trace when a sublaminar wire was tightened. There were no postoperative neurological deficits attributable to the surgery.

Of 55 patients with Duchenne muscular dystrophy offered surgical stabilisation of the spine, 32 accepted and 23 refused. We compared both groups pre-operatively and at six-month intervals in respect of survival, forced vital capacity, peak expiratory flow rate and severity of scoliosis. In the nonoperated patients, the forced vital capacity deteriorated by a mean of 8% per annum; in the operated group it remained static for 36 months and diminished slightly thereafter. Spinal stabilisation resulted in an improvement in the peak expiratory flow rate which was maintained for up to five years. In the nonoperated patients the scoliosis progressed from a mean of 37 degrees to a mean of 89 degrees at five years; in the stabilised spines it was improved from a mean of 47 degrees to a mean 34 degrees at five years. There was significantly improved survival in the patients who had undergone spinal stabilisation.

Fifty-five patients with severe pain from spinal instability secondary to metastatic cancer were referred to Hope Hospital, none being judged to be in a terminal condition. One patient had too extensive disease for surgery so 54 were treated by 55 spinal stabilisations; 49 obtained complete relief of pain and two had partial relief. There were three failures. Twenty-eight of the patients had clinical evidence of spinal cord or cauda equina compression and were decompressed at the time of stabilisation. Of these, 20 had major recovery of neurological function. Patients with pre-operative evidence of extradural tumour had 'prophylactic' decompression at the time of stabilisation; none of these patients later developed signs of cord or cauda equina compression. The results suggest that alleviation of pain and restoration of mobility are best achieved by segmental spinal stabilisation; a few patients require a combined anterior and posterior stabilisation. Postoperative radiotherapy should be given whenever possible, and the causative tumour should be treated by endocrine or chemotherapy, as indicated.

The Matrix seating system is an adaptable orthosis made of interlocking plastic components which can be shaped to fit the needs of the disabled. Twenty-five patients who had used this system for a minimum of 12 months have been assessed clinically. It was found to have several advantages over its rivals particularly in patient and guardian acceptance, versatility and on economic grounds. However, it was found not to prevent deterioration in spinal deformity nor to prevent hip dislocation.

Unlike most other tumours, myeloma causes bone destruction without an osteoblastic reaction; we tried to assess whether myeloma secretes a humoral factor that inhibits osteoblasts. Human bone-derived cells were either co-cultured with myeloma cells, or cultured in medium conditioned by myeloma cells. Bone-derived cell growth was measured by cell counts and by uptake of tritiated thymidine (3H-Tdr); growth was inhibited when cultured in medium conditioned by myeloma cells and some inhibition was seen when the bone-derived cells were co-cultured with myeloma cells. The inhibiting effect was dose-dependent and also dependent upon the density of the myeloma cells conditioning the medium. The results of our study suggest that myeloma secretes an osteoblast inhibiting factor of less than 50,000 Dalton molecular weight.

For clinical, psychological and social reasons the diagnosis of Duchenne muscular dystrophy should be established as early as possible. In a survey of 83 families with 93 affected boys, the diagnosis was missed in every case referred to an orthopaedic surgeon (37 patients). In the whole group there was a mean delay of 2.0 years (0 to 6 years) during which time inappropriate treatment, difficulties in communication with parents, much parental anxiety and further pregnancies occurred. A serum creatine kinase estimation is a simple outpatient test which should be carried out on any boy with clumsy or abnormal gait, with flat feet or with an unexplained equinus deformity.

Equinus deformity of the ankle is one of the serious orthopaedic problems associated with Duchenne muscular dystrophy. Sixty-nine patients (age range 4 to 17 years) were treated, 43 conservatively and 26 operatively. They were followed up at six-monthly intervals for a minimum of two years and a maximum of six years. The patients were divided into three groups: independently mobile, mobile in calipers, and wheelchair-bound. It was found that conservative treatment could at best only minimise progression of the deformity. The indications for surgery, the operative procedure and the postoperative management are described; all varied according to the stage of the disease. The postoperative follow-up suggests that, though the deformity recurs, the patients have several years of benefit from the procedure.