The discrepancies in the practitioners’ knowledge, family resources, and differences in patients culture and/or residency have played a part in the outcome of the patient. Prognosis
DSMA1 is usually fatal in early childhood. The patient, normally a child, suffers a progressive degradation of the respiratory system until respiratory failure. There is no consensus on the life expectancy in DSMA1 despite a number of studies being conducted. A small number of patients survive past two years of age but they lack signs of diaphragmatic paralysis or their breathing is dependent on a ventilation system. Research directions
The disease has only been identified as distinct from SMA recently, so research is still experimental, taking place mostly in animal models. Several therapy pathways have been devised which include gene therapy, whereby an IGHMBP2 transgene is delivered to the cell using a viral vector, and small-molecule drugs like growth factors (e.g., IGF-1 and VEGF) or olesoxime. Studies in amyotrophic lateral sclerosis are also considered helpful because the condition is relatively similar to DSMA1. See also
Distal hereditary motor neuropathies
Spinal muscular atrophies
Spinal muscular atrophy

References
Further reading


== External links ==
An intervertebral disc (or intervertebral fibrocartilage) lies between adjacent vertebrae in the vertebral column. Each disc forms a fibrocartilaginous joint (a symphysis), to allow slight movement of the vertebrae, to act as a ligament to hold the vertebrae together, and to function as a shock absorber for the spine. Structure
Intervertebral discs consist of an outer fibrous ring, the anulus fibrosus disci intervertebralis, which surrounds an inner gel-like center, the nucleus pulposus. The anulus fibrosus consists of several layers (laminae) of fibrocartilage made up of both type I and type II collagen. Type I is concentrated toward the edge of the ring, where it provides greater strength. The stiff laminae can withstand compressive forces. The fibrous intervertebral disc contains the nucleus pulposus and this helps to distribute pressure evenly across the disc. This prevents the development of stress concentrations which could cause damage to the underlying vertebrae or to their endplates. The nucleus pulposus contains loose fibers suspended in a mucoprotein gel. The nucleus of the disc acts as a shock absorber, absorbing the impact of the bodys activities and keeping the two vertebrae separated. It is the remnant of the notochord.There is one disc between each pair of vertebrae, except for the first cervical segment, the atlas. The atlas is a ring around the roughly cone-shaped extension of the axis (second cervical segment). The axis acts as a post around which the atlas can rotate, allowing the neck to swivel.