Spinal Decompensation in Neuromuscular Disease

Jw, Kouwenhoven; Pm, Van Ommeren; He, Pruijs; Rm, Castelein

doi:10.1097/01.brs.0000208131.42824.c3

Cited by 16 publications

(10 citation statements)

References 38 publications

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“…However, Goldberg at al states that the lateralization of scoliotic curve is not a reliable indicator of underlying disease, because right thoracic curve patterns are always more common in scoliosis developing after infancy (34). In contrast to our findings, Kouwenhoven at al reported that a cohort of neuromuscular 198 patients with Duchenne muscular dystrophy, cerebral palsy, spinal muscular atrophy, or spina bifida demonstrated curve patterns similar to what is seen in the most prevalent types of adolescent idiopathic scoliosis (35).…”

Section: Discussioncontrasting

confidence: 55%

Spinal Deformities in Hereditary Motor and Sensory Neuropathy

Horáček¹,

Mazanec²,

Morris³

et al. 2007

Spine

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Study Design. Retrospective study of 175 patients with hereditary motor and sensory neuropathy (HMSN), i.e. Charcot-Marie-Tooth (CMT) Disease Objective: To investigate the frequency, age of onset, character, familial and genotypical incidence of spinal deformities among HMSN patients. Summary of Background Data: Prior studies addressing HMSN discuss the associated spinal deformities. However these data vary significantly, while inconsistently including genotypes within the classification framework. Methods: Plain-film radiographic spine studies of 175 HMSN patients were performed to determine the incidence, character, and severity of spinal deformity. The degree of the spinal deformity was evaluated measuring Cobb's angle of the main curve. The results of the entire cohort were initially assessed before being classified by genotype. Results: The incidence of spinal deformity for the entire group was 26%. Of these, 58% demonstrated scoliosis, 31% had kyphoscoliosis and 11% had thoracic hyperkyphosis. 73% of patients with spinal deformity were classified as HMSN type I with confirmed duplication of the PMP 22 (peripheral myelin protein) gene on chromosome 17. The incidence of spinal deformity by genotype was: duplication of the PMP 22 gene: 29%; deletion of the PMP 22 gene: 0%; Cx32 (connexin 32) gene mutation: 24%; and MPZ (myelin protein zero) gene mutation: 100%. Familial incidence of spinal deformity was found in "MPZ gene mutation" and "duplication of PMP 22 gene" subgroups. Conclusions:The study demonstrates as 26 % incidence of spinal deformity among HMSN patients. Spinal deformity was most frequently observed in patients with the MPZ gene mutation, where the most common familial incidence was also found. * Structured Abstract (300 words)Spinal deformities in HMSN Key Points:  A group of 175 HMSN patients was analyzed.  Spinal radiographs of the entire cohort demonstrated spinal deformity in 45 (26%) patients.  Four genotypes were found (duplication or deletion of the PMP 22 gene on chromosome 17, mutation of the Cx 32 gene and mutation of the MPZ gene).  Spinal deformity rates varied significantly by genotype. The highest percentage incidence of spinal deformity was found in patients demonstrating the MPZ gene mutation (100%).  Familial incidence was observed in 4 families. The mutation MPZ gene mutation was confirmed in three of these. Key Points (3-5 main points of the article)Spinal deformities in HMSN Mini Abstract/PrécisWe performed radiographic analyses on 175 HMSM patients. Spinal deformity was present in 45 patients (26%), with incidence rates varying by genotype. Spinal deformity prevalence by genotype was greatest with the MPZ mutation (100%). Familial incidence was found in four families, with the MPZ mutation noted in three.

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Section: Discussioncontrasting

confidence: 55%

Spinal Deformities in Hereditary Motor and Sensory Neuropathy

Horáček¹,

Mazanec²,

Morris³

et al. 2007

Spine

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“…Idiopathic adolescent curves are more likely to be right-sided and thoracic in location. Experts have believed that neuromuscular curves have a higher incidence of left-sided convexity [11], although a recent retrospective study suggests that the curve patterns and apical levels in neuromuscular scoliosis are similar to those reported for idiopathic adolescent scoliosis [38]. Before surgery, curve flexibility may be assessed using supine lateral bending, fulcrum, or traction radiographs [39].…”

Section: Discussionmentioning

confidence: 96%

Musculoskeletal Complications of Neuromuscular Disease in Children

Driscoll

Skinner

2008

Physical Medicine and Rehabilitation Clinics of North America

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“…It was previously demonstrated that in the normal non-scoliotic spine a predominant left-sided rotation exists in the high thoracic vertebrae, whereas the mid- and lower thoracic vertebrae are predominantly rotated to the right [20]. This prevalent rotational pattern in the normal spine corresponds with the predominance of right-sided thoracic and thoracolumbar curves in idiopathic and neuromuscular scoliosis [19, 38]. The lumbar vertebrae of the normal spine do not show a predominant rotation.…”

Section: Discussionmentioning

confidence: 98%

“…Most prevalent curve types of adolescent idiopathic scoliosis were found to demonstrate comparable patterns [8, 11, 12, 14, 21, 23, 32, 35, 40], which correspond to the predominant rotational pattern in the normal spine [19]. This implies that once the spine starts to decompensate, due to a still unknown cause, it follows this already built-in rotational tendency [19]. In degenerative scoliosis factors causing asymmetrical degeneration and the eventual disruption of the spinal equilibrium are still unidentified.…”

Section: Introductionmentioning

confidence: 99%

Spinal decompensation in degenerative lumbar scoliosis

et al. 2010

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Due to the aging population, degenerative scoliosis is a growing clinical problem. It is associated with back pain and radicular symptoms. The pathogenesis of degenerative scoliosis lies in degenerative changes of the spinal structures, such as the intervertebral disc, the facet joints and the vertebrae itself. Possibly muscle weakness also plays a role. However, it is not clear what exactly causes the decompensation to occur and what determines the direction of the curve. It is known that in the normal spine a pre-existing rotation exists at the thoracic level, but not at the lumbar level. In this retrospective study we have investigated if a predominant curve pattern can be found in degenerative scoliosis and whether symptoms are predominantly present at one side relative to the curve direction. The lumbar curves of 88 patients with degenerative scoliosis were analyzed and symptoms were recorded. It was found that curve direction depended significantly on the apical level of the curve. The majority of curves with an apex above L2 were convex to the right, whereas curves with an apex below L2 were more frequently convex to the left. This would indicate that also in degenerative scoliosis the innate curvature and rotational pattern of the spine plays a role in the direction of the curve. Unilateral symptoms were not coupled to the curve direction. It is believed that the symptoms are related to local and more specific degenerative changes besides the scoliotic curve itself.

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Spinal Decompensation in Neuromuscular Disease

Abstract: In neuromuscular scoliosis, curve patterns and apical levels are similar to what is seen in the most prevalent types of adolescent idiopathic scoliosis.

Cited by 16 publications

References 38 publications

Spinal Deformities in Hereditary Motor and Sensory Neuropathy

Spinal Deformities in Hereditary Motor and Sensory Neuropathy

Musculoskeletal Complications of Neuromuscular Disease in Children

Spinal decompensation in degenerative lumbar scoliosis

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