Background: Amyotrophic lateral sclerosis (ALS) is relatively rare, yet the economic and social burden is substantial. Having accurate incidence and prevalence estimates would facilitate efficient allocation of healthcare resources. Objective: To provide a comprehensive and critical review of the epidemiological literature on ALS. Methods: MEDLINE and EMBASE (1995-2011) databases of population-based studies on ALS incidence and prevalence reporting quantitative data were analyzed. Data extracted included study location and time, design and data sources, case ascertainment methods and incidence and/or prevalence rates. Medians and interquartile ranges (IQRs) were calculated, and ALS case estimates were derived using 2010 population estimates. Results: In all, 37 articles met the inclusion criteria. In Europe, the median incidence rate (/100,000 population) was 2.08 (IQR 1.47-2.43), corresponding to an estimated 15,355 (10,852-17,938) cases. Median prevalence (/100,000 population) was 5.40 (IQR 4.06-7.89), or 39,863 (29,971-58,244) prevalent cases. Conclusions: Disparity in rates among ALS incidence and prevalence studies may be due to differences in study design or true variations in population demographics such as age and geography, including environmental factors and genetic predisposition. Additional large-scale studies that use standardized case ascertainment methods are needed to more accurately assess the true global burden of ALS.

Thirty-four patients with ambulatory spastic diplegia (ages 10-19.8 years) who were part of a prospective study of selective dorsal rhizotomy (SDR) had standardized radiographs before and after SDR. Follow-up ranged from 5 to 11.6 years after surgery. Two different surgical approaches were used: laminectomy (14 patients) and laminoplasty (20 patients). Radiographs were measured for coronal and sagittal balance. Thirty patients had a spinal deformity at long-term follow-up compared with 10 patients before surgery. Seventeen patients (50%) developed lumbar hyperlordosis greater than 60 degrees. Six patients (18%) developed grade 1 spondylolisthesis, Scoliosis occurred de novo in eight patients (24%) and progressed by greater than 5 degrees in two patients with preoperative scoliosis. No significant differences were found between laminoplasty and laminectomy patients. None of the patients have undergone any surgical intervention for spinal deformity. There was a higher incidence of spinal deformity after SDR than in normals and an historical control population, which warrants clinical and radiographic long-term follow-up.

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