Prof. Dr. Erkan Kaptanoğlu

Diseases

Neuromuscular Scoliosis: Scoliosis Due to Neurological-Muscular Diseases

Neuromuscular scoliosis is the second most common type of scoliosis. A pathology in the brain, spinal cord, peripheral nerves, neuromuscular junction or muscles is present in these patients. The main problem is torso imbalance due to muscle weakness. The main problem of the patients is the inability to sit and issues arising from this situation. The simple act of sitting becomes extremely difficult. The main difference of these patients from idiopathic scoliosis patients is that they experience more pain. In addition, neuromuscular scoliosis can continue to progress even during adulthood. The physical and functional difficulties of these patients are accompanied by psychological problems.

Head-neck and torso control is impaired or sometimes non-existent in these patients. Contractures may be seen in the joints of patients. These patients may have convulsive seizures, their hygienes are poor and may have pressure ulcers. On top of these, mental disorders, communication disorders, and malnutrition may be observed in these patients. Bone structures may be osteopenic due to atrophy or osteomalasic due to the use of antiepileptic drugs. Multiple organ involvement such as lung dysfunctions and cardiomyopathy may be seen.

Natural Course

It is the underlying disease that determines the natural course of neuromuscular scoliosis. In general, they have a shorter lifespan than the average life expectancy of the normal population. The degree of curvature is directly proportional to the growth curve and can continue to progress even during adulthood.

The Underlying Diseases For Neuromuscular Scoliosis
Neuropathic Causes
  • Upper motor neuron
  • Cerebral palsy
  • Spinocerebellar degeneration
  • Friedreich’s ataxia
  • Charcot-Marie-Tooth
  • Roussy-Levy
  • Syringomyelia
  • Spinal cord tumor
  • Spinal cord trauma
Lower Motor Neuron
  • Poliomyelitis
  • Other viral myelitis
  • Traumatic
  • Spinal muscular atrophy
  • Werdnig-Hoffmann
  • Kugelberg-Welander
  • Dysautonomia (Riley-Day syndrome)
Myopathic causes
  • Arthrogryposis
  • Muscular dystrophy
  • Duchenne
  • Limb-girdle
  • Facioscapulohumeral
  • Fiber-type disproportion
  • Congenital hypotony
Diagnosis

It is usually noticed by the family and the doctor while the underlying disease is treated and monitored. Radiological examination is performed after the medical examination. Due to general muscle weakness, kyphoscoliosis is seen often in “C” shape. Spinal cord and soft tissues are examined by Magnetic Resonance Imaging (MRI) after direct scoliosis radiographs taken at various positions. If necessary, a detailed examination of the bones may be performed with Computed Tomography (CT).

Treatment

The objective of the treatment is to prevent the progression of the curvature as well as to increase the functionality of the patient. The goal is to ensure a balanced spine that will allow the patient to sit comfortably.

Conservative Treatment

It can be used to allow the patient’s spine to grow until surgical treatment. In the corsets to be used, the aim is not to stop the progression of the curvature, but to ensure that the patient can sit by way of correcting the torso balance. For a corset to be used, the curvature must be mobile and the patient should be lean. It is used when the patient is awake and in a sitting position. It can be typically used for up to 10-11 years of age.

Surgical Treatment

The aim is to ensure a balanced spine by correcting the scoliosis and kyphosis as much as possible. Patients with advanced curvatures, progressive curvatures of Duchenne Muscular Dystrophy, patients with sitting problems and cardiac-pulmonary problems may require surgery. Usually, fusion surgery is performed from behind and with long level spine fixations.

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