Prognosis of patients with Guillain-Barré syndrome requiring mechanical ventilation.

INTRODUCTION: Severe Guillain-Barré syndrome (GBS) is associated with significant morbidity and also mortality. Identification of modifiable risk factors may help in reducing the morbidity and mortality. OBJECTIVE: To study the prognostic factors in a selected cohort of mechanically ventilated GBS patients. MATERIALS AND METHODS: Case records of GBS patients requiring mechanical ventilation admitted between 1997 and 2007 were analyzed. All patients satisfied the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria for GBS. Primary outcome parameters included mortality and GBS disability (Hughes) scale score at discharge. RESULTS: During the study period, 173 (118 men and 55 women; mean age of 33.5 ± 21 years) GBS patients were mechanically ventilated. A history of antecedent events was present in 83 (48%) patients. In addition to motor weakness, In all facial palsy was present in 106 (61%), bulbar palsy in 91 (53%), sensory involvement in 74 (43%), and symptomatic autonomic dysfunction in 27 (16%). The overall mortality was 10.4%. On univariate analysis the risk factors for mortality included elderly age (P = 0.014), autonomic dysfunction (P = 0.002), pulmonary complications (P = 0.011), hypokalemia (P = 0.011), and bleeding (P = 0.026). All these factors were significant in multivariate analysis except for bleeding from any site and hypokalemia. In univariate analysis factors associated with Hughes scale score ≤ 3 at discharge included younger age (P = 0.02), presence of bulbar symptoms (P = 0.03) and less severe weakness at admission (P = 0.02), slower evolution of disease over more than 3 days (P = 0.01), electrodiagnostic evidence of demyelinating neuropathy (P = 0.00), and absence of sepsis (P = 0.01), hyperkalemia (P = 0.0001), and anemia (P = 0.02). In multivariate analysis age was the only significant factor. CONCLUSIONS: Early identification of modifiable risk factors, such as pulmonary involvement, autonomic dysfunction, hypokalemia, sepsis, bleeding, and nutritional complications, may reduce the mortality and morbidity associated with GBS.

Survival after short- or long-term ventilation after acute spinal cord injury: a single-centre 25-year retrospective study.

STUDY DESIGN: A retrospective review of acute spinal cord injury patients having assisted ventilation on or after admission between 1981 and 2005. OBJECTIVE: To assess survival after acute ventilatory support. SETTING: Northwest Regional Spinal Injuries Centre, Southport, England. METHODS: Causes of death were ascertained from the Office of National Statistics. Kaplan-Meier analysis of survival was calculated according to ventilator-wean status at discharge. Risk factors were obtained by Cox regression analysis. RESULTS: Over 50% of deaths in weaned and ventilated patients were respiratory in origin. The mean survival of weaned patients in the age group 31-45 was 19.3 compared with 10.5 years for ventilated patients (P=0.047). Those under 30 survived a further 22.1 and 18.4 years (P=0.31), while those over 45 lived for 11.0 and 8.3 years (P=0.50), values for weaned and ventilated patients, respectively. The survival advantage for weaned patients in the middle age group was less evident when the 1-year survivors were compared. The mean survival time of younger patients with diaphragm pacing was 1.8 years longer than those on mechanical ventilation (P=0.142). The variables with significant hazard ratios were any comorbidity (3.07); mechanical ventilation on discharge (2.26); and older age at injury, (3.1). CONCLUSIONS: The survival time for patients with high tetraplegia on long-term ventilation compares with other datasets and older patients have a proportionately greater loss in life expectancy. Self-ventilating patients with tetraplegia remain at considerable risk from respiratory death and consideration needs to be given to more effective preventative measures.

Modern management of spinal muscular atrophy.

Spinal muscular atrophy is an incurable disease with a frequency of 8 per 100,000 live births. The disease gene, survival motor neuron 1 (SMN1), was identified with a disease modifying gene, SMN2. There is a high mortality rate in infancy and severe morbidity in childhood. Management depends on treating or preventing complications of weakness and maintaining quality of life. Weakness may affect several organ systems: respiratory, due to restrictive lung disease; gastrointestinal, in terms of dysphagia and constipation; and orthopedic, with progressive deformities. This review focuses on management of restrictive lung disease, the most common and most serious complication. Three areas of recent development are noninvasive ventilation using new technology, new awareness of the importance of identifying sleep-disordered breathing, and a new multidisciplinary approach to standard of care. Noninvasive ventilation and improved airway clearance are helpful for preoperative and postoperative management. Standard of care requires a multidisciplinary approach.

Pulmonary management of the ALS patient.

Amyotrophic lateral sclerosis (ALS) is a chronic progressive motor neuron disease with a poor prognosis which eventually weakens and paralyzes the respiratory muscles. ALS is characterized by progressive degeneration of both cortical and alpha motor neurons of the final common pathway. Early symptoms usually begin with alpha motor neuron involvement and then progress to include cortical motor neuron involvement. Degeneration of respiratory nerve centers in the anterior horn at the C3-C5 levels results in respiratory muscle fatigue, respiratory failure and eventually death. Treatment consists of preventing respiratory complications and supporting lung function for as long as possible. One case example of a critically ill patient with ALS highlights nursing concerns. With advanced directives and durable power of attorney, the patient now has better means available for making known the decision of whether to accept or reject mechanical ventilation.

Diaphragmatic pacing. An option for patients with quadriplegic respiratory paralysis.

Recent advances in design, increased reliability, and computerized pacing provide an exciting alternative to mechanical ventilation for the quadriplegic patient. Research into the development of totally implantable, demand-type respiratory pacemakers is ongoing. Careful patient selection, meticulous surgical technique, adequate training in the use of the device, and conscientious follow-up contribute to a successful outcome--increased mobility and independence for the ventilator-dependent quadriplegic patient.