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Rigid spine syndrome, also known as congenital muscular dystrophy with rigidity of the spine (CMARS), is a rare and often debilitating neuromuscular disorder.It is characterized by progressive muscle stiffness and rigidity, particularly in the spine, which can severely limit mobility and impact quality of life.
In adults the spinal cord stretches in the course of normal activity, usually leading to progressive spinal cord damage if untreated. [1] TCS is often associated with the closure of a spina bifida. It can be congenital, such as in tight filum terminale, or the result of injury later in life. [2] [3]
Neurologic signs result from severe angulation of the spine, narrowing of the spinal canal, instability of the spine, and luxation or fracture of the vertebrae. Signs include rear limb weakness or paralysis, urinary or fecal incontinence, and spinal pain. [5] Most cases of hemivertebrae have no or mild symptoms, so treatment is usually ...
Secondary scoliosis due to neuropathic and myopathic conditions can lead to a loss of muscular support for the spinal column so that the spinal column is pulled in abnormal directions. [ citation needed ] Some conditions which may cause secondary scoliosis include muscular dystrophy , spinal muscular atrophy , poliomyelitis , cerebral palsy ...
Bertolotti's syndrome is characterized by sacralization of the lowest lumbar vertebral body and lumbarization of the uppermost sacral segment. It involves a total or partial unilateral or bilateral fusion of the transverse process of the lowest lumbar vertebra to the sacrum, leading to the formation of a transitional 5th lumbar vertebra.
These attachments cause an abnormal stretching of the spinal cord. [citation needed] The course of the disorder is progressive. In children, symptoms may include the "stigmata" mentioned above and/or foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence. In adulthood, the signs and symptoms often include ...
[66] [67] Taking into account that signs and symptoms of spinal deformity cannot be changed by surgical intervention, surgery remains primarily a cosmetic indication [dubious – discuss], only especially in patients with adolescent idiopathic scoliosis, the most common form of scoliosis never exceeding 80°.
Changes in the spinal bones in the neck may also increase the risk of spinal cord damage. Other skeletal signs include flattened vertebrae (platyspondyly), a hip joint deformity in which the upper leg bones turn inward , and an inward- and downward-turning foot (called clubfoot). Decreased joint mobility and arthritis often develop early in life.