Trinity Medical Center I Dr. Marin Guentchev, MD, PhD Neuromuscular scoliosis

Neuromuscular scoliosis

ANATOMY OF THE SPINE

The human spine is formed by individual vertebrae and connective tissue discs in between. The vertebrae form the spinal canal. There are seven cervical, twelve thoracic, and five lumbar vertebrae. The intervertebral discs are the link between the individual vertebral bodies.
Normal anatomy of the spine. Longitudinal section and cross sections through the cervical, thoracic and lumbar spine.
The little joints that link the vertebrae together are known as facet joints. They help to stabilize the spine and, together with the intervertebral discs, allow a certain degree of mobility of the spinal cord. The spinal canal should be wide enough to allow nerve roots to float freely in cerebrospinal fluid. The front border of the spinal canal is built by the vertebral bodies and intervertebral discs, the side by the intervertebral joints (facets) and back by the ligamentum flavum (yellow band) and vertebral arches. Discs consist of an outer fibrous ring (annulus fibrosus), which surrounds an inner gel-like center (nucleus pulposus). The spinal cord and nerve roots lie within the spinal canal. The spinal cord extends downwards approx. to the 1st lumbar vertebra. Below, only nerve roots are present in the spinal canal. At the level of the intervertebral disc the nerve roots pass through the neural root foramina to exit the spinal canal. The spinal cord and nerve roots conduct electric-like signals from the skin and joints to the brain, and process of movement is initiated from the brain to the muscles.

DESCRIPTION

Diseases of the brain, spinal cord, and muscular system including cerebral palsy, muscular dystrophy, spinal muscular atrophy, spina bifida and others, may cause a spinal deformity known as neuromuscular scoliosis. Neuromusccular scoliosis progresses more rapidly even after maturity than the idiopatic one. It is commonly associated with pelvic obliquity and has a high rate of perioperative pulmonary complications.

CAUSE OF SYMPTOMS

Gradually progressing compression of nerve roots
Gradually progressing deformation of the spine
Gradually progressing inflammation and degeneration in the intervertebral joints

SYMPTOMS AND SIGNS

Total/partial paralysis and numbness from the site of the spinal cord damage downwoads
Visible spine deformity Chest deformity (protrusion on one side and concavity on the other) Waist asymmetry and pelvic tilt Shoulder height differences Inclined head position
Pain and stiffness along the spine

THE DIAGNOSIS IS BASED ON

Medical history Clinical exam
and at least one of the following tests:
Magnetic resonance imaging (MRI) Computer tomography
additionally you may have to do:
Spirometry Radiographs Genetic tests
Study of creatine kinase (CK/KK) levels
Muscle biopsy
Electromyography

TREATMENT

Treatment may consist of surgical and non-surgical methods. Non-surgical treatment rarely can significantly improve the condition of the patient, but aims to prevent secondary vertebral complications and to delay the need for surgical stabilization. It includes:
Non-surgical treatment may include
Immobilization with brace
Physiotherapy Electrotherapy Ultrasound therapy Ergotherapy

WHEN SHOULD AN OPERATION BE PERFORMED?

The scoliosis angle increases
loss of ability to sit

WHAT IS THE GOAL OF SURGERY?

Prevent the progression of the scoliosis curve
Improve balance while sitting
Reduce pain

HOW IS SURGERY PERFORMED?

Non-fusion procedures (e.g. growing rods) in small children, allow for continued spinal growth.
Anterior/posterior spinal fusion in younger patients, prevents crankshaft phenomenon.

WHICH OTHER DISEASES SHOULD BE EXCLUDED (DIFFERENTIAL DIAGNOSIS)?

Cerebral Palsy Myelomeningocele Spinal muscular atrophy Muscular dystrophy (Duchenne and Becker) Syringomyelia Polyomyelitis Spinocerebellar ataxia (SCA) Arthrogryposis multiplex congenita (АМС) learn more ...
Adress
Trinity Medical Center 117 Zaichar St /Ground floor/ /Konstantin Velichkov Metro Station/ BG-1309 Sofia, Bulgaria
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