Forty-Five Years of Duchenne Muscular Dystrophy in The Netherlands.

BACKGROUND: Duchenne muscular dystrophy (DMD) is a progressive muscle disease. No curative therapy is currently available, but in recent decades standards of care have improved. These improvements include the use of corticosteroids and mechanical ventilation. OBJECTIVE: To present a detailed population based report of the DMD disease course in The Netherlands (1980-2006) and evaluate the effect of changes in care by comparing it with an historical Dutch DMD cohort (1961-1974). METHODS: Information about DMD patients was gathered through the Dutch Dystrophinopathy Database using a standardized questionnaire and information from treating physicians. RESULTS: The study population involved 336 DMD patients (70% of the estimated prevalence), of whom 285 were still alive. Mean age at disease milestones was: diagnosis 4.3 years, wheelchair dependence 9.7 years, scoliosis surgery 14 years, cardiomyopathy (fractional shortening <27%) 15 years, mechanical ventilation 17 years and death 19 years. Within our cohort, corticosteroid use was associated with an increased age of wheelchair dependence from 9.8 to 11.6 years (p < 0.001). When comparing the recent cohort to the historical cohort, mean survival improved from 17 to 27 years (p < 0.001). CONCLUSION: The current study gives detailed information about the disease course of DMD patients, provides evidence for the positive effect of steroid treatment and mechanical ventilation and supports the use of patient registries as a valuable resource for evaluating improvements in care.

An epidemiological description of spinal cord injuries in The Netherlands in 1994.

STUDY DESIGN: Retrospective descriptive study. OBJECTIVES: To ascertain the incidence of spinal cord injury in The Netherlands. METHODS: From all patients, discharged from a Dutch general hospital in 1994 with the ICD-9 diagnosis code 806 (fracture of the spine with injury of the spinal cord) and 952 (injury to the spinal cord without apparent spinal fracture), a copy of the anonymized medical correspondence was requested. The received correspondence was analyzed for the diagnosis of traumatic spinal cord injury with motor, sensory, bladder and bowel symptoms lasting longer than 2 weeks. RESULTS: 479 cases with the ICD-9 codes 806 or 952 were identified. On 329 cases we received information. According to our criteria 126 cases had a spinal cord injury with persisting symptoms. Of these 126 cases 18 died during hospital stay. Sex (77% male), level (57% tetra), completeness (48.7% complete), age distribution, cause of injury and incidence of stabilizing operation (44.2%) were assessed. Mean hospital stay was 31 days and of the subjects who survived the initial hospital phase, 70% were referred to a rehabilitation centre or a rehabilitation ward. CONCLUSION: The incidence of spinal cord injury surviving the acute phase in The Netherlands in 1994 was 10.4/million/annum.