Academic literature on the topic 'Psychological aspects of Intifada, 1987-'

The dystrophinopathies, including Duchenne and Becker muscular dystrophies, are X-linked, developmental neuromuscular disorders. The dystrophinopathies are so named because of their effect on the production of the protein dystrophin, and are known primarily as muscle diseases in males that present with progressive weakness that is eventually fatal. To date, there is no cure for the dystrophinopathies, and treatment focuses on slowing the disease progression. Medical management is complex and multifaceted. It includes care for neuromuscular, orthopedic, rehabilitative, nutritional, respiratory, cardiac, gastrointestinal, psychological, and palliative aspects of the disease. The devastating physical toll of the diseases is well known. In contrast, the associated cognitive and behavioral abnormalities are less familiar to most clinicians. Nonetheless, the cognitive and behavioral attributes of the dystrophinopathies impact on the affected individual’s and his family’s functioning in far-ranging ways. More importantly, identifying any associated cognitive and behavioral abnormalities early, and providing appropriate interventions, can contribute substantially to an affected individual’s quality of life. The dystrophinopathies are the most common neuromuscular diseases of childhood, affect all ethnic groups, and have an estimated overall prevalence of 63 per million (Emery 1991; Emery 1992). Positive diagnosis for the Duchenne muscular dystrophy (DMD, the more severe form) is based on the following criteria: (a) male; (b) onset of weakness before age 5; (c) initial proximal muscle weakness; (d) muscle hypertrophy, most prominent in the calves; (e) elevated creatine kinase activity of at least 10 times above the upper limit of normal; and either (f) positive histopathological confirmation by muscle biopsy or (g) molecular characterization of a mutation within the gene for dystrophin. Approximately 1 in 3,500 live male births meet these criteria (Emery 1992). The diagnosis of Becker muscular dystrophy (BMD, the less severe form) is clinically determined by those children who remain walking at age 12. Individuals with BMD have a slower course and considerably longer lifespan than those with DMD. Natural history studies have been conducted to characterize the course of the disease. The Clinical Investigation of Duchenne Dystrophy group (CIDD) followed more than 200 individuals affected with DMD for more than 10 years (Brooke et al. 1983; Hyser et al. 1987; Mendell et al. 1987).