Neuromuscular Scoliosis

Neuromuscular scoliosis is the second most common type of scoliosis. These patients have pathology in the brain, spinal cord, peripheral nerves, neuromuscular junction or muscles. The main problem is torso imbalance due to muscle weakness. The main problem of the patients is the inability to sit and problems related to this situation. The simple sitting activity becomes very difficult. The main difference of these patients from idiopathic scoliosis is that they are painful. In addition, neuromuscular scoliosis can continue to progress even in adulthood. The physical and functional difficulties of these patients are accompanied by psychological problems.

Head-neck and torso control is impaired, sometimes absent 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. In addition to these, mental disorders, communication disorders, and malnutrition may be seen in these patients. Bone structures may be osteopenic due to non-use or osteomalasic due to use of antiepileptic drugs. Multiple organ involvement such as lung dysfunction and cardiomyopathy may be seen.

Natural course

It is the underlying disease that determines the natural course of neuromuscular scoliosis. In general, the normal population has less life span than the average life expectancy. The degree of curvature is directly proportional to the growth curve and can continue to progress even in 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 followed-up. Radiological examination is performed after the medical examination. Due to general muscle weakness, kyphoscoliosis is often seen as “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 done with Computed Tomography (CT).

Treatment

The aim of the treatment is to prevent the progression of the curvature as well as to increase the functions of the patient. The goal is to obtain 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 correcting the torso balance. For corset use, the curvature must be moving and the patient should be thin. It is used when the patient is awake and in a sitting position. Usually used up to 10-11 years.

Surgical treatment: The aim is to provide a balanced spine by correcting scoliosis and kyphosis as much as possible. Patients with advanced curvatures, progressive curvatures of Duchenne Muscular Dystrophy, patients with sitting problems and heart-lung problems may require surgery. Generally, fusion surgery is performed from behind and with long level spine fixations.