Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Scoliosis is an important cause of disability in childhood, due to its incidence and limitation on ability. In neurodevelopmental disabilities, scoliosis worsens the already limited functional capacities of the child and can thwart abilities partially recovered through rehabilitation. In celebral palsied children (CP), scoliosis is considered a consequence of damage to the central nervous system or a complication of a peripheral impairment, in particular, through obliquity of pelvis, of the unilateral dislocation of hip. In order to explain the scoliosis of central origin, primitive and pathological reflexes, reactions or automatisms must be taken into account, especially the asymmetrical ones. This contradicts the absence of scoliosis in hemiplegia. On the contrary, symmetrical patterns should act as protective factors. However, the greater incidence of scoliosis in tetraplegia than in dipegia does not confirm this idea. Scoliosis is secondary when it is linked to an unilateral hip dislocation for side of convexity, proximity, measure and timing sequence. In childhood neuromuscular diseases (NMD), scoliosis is the unavoidable consequence of muscle weakness. The only protective factor may be muscle stiffness in case of fibrotic degeneration. The main curve is reducible for longer periods, while the less reducible secondary one at cervical level can limit the correction possibilities, due to the need to keep the head aliened. In spina bifida (SB) children, lesional and supralesional scoliosis can be present. In the former, the malformative ones directly derive from the vertebrae involved in the myelomeningocele and the secondary ones develop from the unilateral hip dislocation similar to CP, or from muscular imbalance (a typical feature of SB, especially for lower lumbar levels) or from primitive skeletal malformations of pelvic girdle or lower limbs. The acquired ones are caused by muscle weakness as in NMD. In the supralesional scoliosis, the curve is the consequence of an impairment in neurological structures especially of ponto-cerebellar carrefour or of an acquired tethered cord and may affect any segment of spinal column, also above the primitive lesion level. The scoliosis in neurodevelopmental disabilities can be treated conservatively with corsets and postural systems, with limited results. or through a surgical approach, often made difficult due to the multiple impairments present in the same patient.
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PMID:Severe scoliosis in neurodevelopmental disabilities: clinical signs and therapeutic proposals. 2122 89

Stiff person syndrome is a neuroimmunological disorder characterized by progressive muscular rigidity and spasms that affect axial/limb muscles, resulting in severe pain and functional limitations. When refractory to conservative treatments, intrathecal baclofen is a viable option to treat the increased tone. Intrathecal baclofen has been shown to accelerate underlying neuromuscular scoliosis in the pediatric population with cerebral palsy. This adverse effect has never been reported in adults with stiff person syndrome. We report a case of an adult with stiff person syndrome and underlying scoliosis who experienced accelerated progression of scoliosis after initiation of intrathecal baclofen, subsequently requiring neurosurgical intervention.
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PMID:Advanced Progression of Scoliosis After Intrathecal Baclofen in an Adult With Stiff Person Syndrome: A Case Report. 3275 18