Surgery for equinovarus deformity in Friedreich's ataxia improves mobility and independence.

UNLABELLED: Friedreich's ataxia results in morbidity because of many factors; progressive equinovarus deformity is one of these. We studied the risk factors and incidence of this deformity. We sought to assess whether surgical management of fixed equinovarus deformity leads to functional improvement. Thirty-six patients with Friedrich's ataxia were assessed for this deformity. These patients were treated by splinting, botulinum toxin Type A injection, and surgery, as indicated by the severity, followed by an ongoing rehabilitation program. The effect of surgery was assessed using subscales of the Barthel index and functional independence measure. Severe foot deformities in which either surgery or botulinum toxin injection was recommended correlated with current age, years since disease onset, and years that the patient required a wheelchair for mobility, but not with the GAA repeat size or age at disease onset. Function and mobility were improved after surgery compared with a similar period before surgery. Three of seven patients who had surgery had significant complications. Aggressive management of foot deformities should be considered, and active measures to prevent permanent foot deformities should be pursued to maximize quality of life and independence of patients with Friedreich's ataxia. LEVEL OF EVIDENCE: Therapeutic study, Level IV (case series-no, or historical control group).

Nontraumatic spinal cord injury rehabilitation: pressure ulcer patterns, prediction, and impact.

OBJECTIVE: To investigate the characteristics, predictors, and consequences of pressure ulcers in patients with nontraumatic spinal cord injury (SCI). DESIGN: Retrospective, 3-year, case series. SETTING: Tertiary medical unit specializing in SCI rehabilitation. PARTICIPANTS: Consecutive sample of 134 adult inpatient referrals with nontraumatic SCI. Patients requiring initial rehabilitation or readmission were included. INTERVENTION: Chart review. MAIN OUTCOME MEASURES: Primary outcome measures were presence of pressure ulcers on admission to rehabilitation, incidence of new pressure ulcers developing during hospitalization, and any complications attributable to pressure ulcers during inpatient rehabilitation. Secondary objectives were to examine the predictability of risk factors for pressure ulcers, to assess the usefulness of a model previously developed for predicting pressure ulcers in patients with chronic SCI, and to estimate the effect of pressure ulcers on rehabilitation of nontraumatic SCI. RESULTS: Prevalence of pressure ulcers among admissions was 31.3% (n=42). Only 2.2% (n=3) of patients developed a new pressure ulcer after admission. The length of stay (LOS) of patients admitted with a pressure ulcer was significantly longer than that of those without a pressure ulcer (geometric mean, 62.3 d for pressure ulcer vs 28.2 d for no pressure ulcer, P=.0001). Many previously identified risk factors for pressure ulcers in SCI patients did not apply to our nontraumatic SCI patients. It is estimated that the inpatient LOS for those patients with a significant pressure ulcer was increased by 42 days. CONCLUSIONS: Pressure ulcers are a common complication for people with nontraumatic SCI who are admitted for rehabilitation, and they have a significant impact on LOS.

Nontraumatic spinal cord injury: demographic characteristics and complications.

OBJECTIVES: To investigate the demographic characteristics and complications of nontraumatic spinal cord injury (NT/SCI), to compare patients who were admitted for initial rehabilitation with readmission rehabilitation patients, to compare our findings with those of other studies, and to develop a model to predict the length of stay (LOS). DESIGN: Retrospective, 3-year, case series. SETTING: Tertiary medical unit specializing in SCI rehabilitation in Australia. PARTICIPANTS: Consecutive sample of 134 adult referred inpatients with NT/SCI (58% women; median age, 61y). Patients requiring initial rehabilitation or readmission were included. INTERVENTION: Chart review. MAIN OUTCOMES MEASURES: Demographic characteristics, neurologic injury, etiology, comorbidities, and complications of NT/SCI. RESULTS: The most common cause of NT/SCI was tumor (20.1%), but there were many different etiologies. Tetraplegia occurred in 32.8% of patients, and 56% had motor incomplete injuries. Most patients (63%) had at least 1 complication, including urinary tract infection (32.8%), pressure ulcer (31.5%), and pain (18.7%). Initial rehabilitation patients were significantly older (initial median, 69y vs readmission median, 54y; P=.0001). A multivariate model for LOS was able to predict 52% of the variance. CONCLUSIONS: NT/SCI rehabilitation patients have a different demographic profile compared with traumatic SCI (T/SCI) patients and a lower prevalence of many of the complications that affect T/SCI patients. There are differences between initial and readmission patients.

Semen retrieval in spinal cord injured men.

A fertility programme for spinal cord injured men has been in progress for nearly 5 years. Thirty eight men have been treated in this programme. Electroejaculation, vibration ejaculation and subcutaneous physostigmine have all been used successfully to obtain semen. Semen has been obtained from 21 of 24 men with a lesion at T8 or above, and from 4 of 11 men with lesions below T10. There have been 8 pregnancies from 6 couples.

Assisted fertility in complete paraplegia: case report.

A 34-year-old paraplegic man with a spinal cord injury complete below the 6th thoracic segment fathered a child by artificial insemination using semen obtained by electro-ejaculation. A long fertility programme culminated in the delivery of a healthy male child weighing 3665 g in April 1987. Guidelines for a comprehensive fertility programme are discussed briefly.