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)

The protrusion of cervical intervertebral discs was divided into three pathological entities by Spurling; soft disc, hard disc and spondylosis. We applied these concept to the dorsal intervertebral disc disease and treated two cases of thoracic spondylosis. Case 1. A 41-year-old male entered the hospital because of the gradual progression of weakness of both legs of two months' duration. Since ten days before admission he had not had an errection and had not been to able to walk and micturate. He also complained of paresthesia radiating down the abdomen into both legs. There were no visceral complaints. Neurological examination revealed severe weakness of both legs with bilateral impairment of deep sensations and hypalgesia up to the level of T6. Reflexes in both legs were hyperactive with sustained clonus. Plantar responses were extensor bilaterally. Though plain X-rays showed no changes, tomography revealed a calcified intervertebral spur formation at the T5-6 interspace. A myelogram showed a complete block of the contrast medium at the level of the upper part of T6. The patient underwent a complete laminectomy from T3 through T6 and extradural anterior decompression with the removal of the calcified disc at the T5-6 interspace using an air drill. Postoperatively, he demonstrated an immediate improvement in sensation and a gradual recovery in motor power. At his follow-up examination 14 months after surgery he could walk without assistance. Case 2. A 47-year-old dwarfish woman (130 cm) with a low back pain and difficulty in walking for a few years duration was admitted. A few months before admission she felt pain at her left lateral abdomen. There was weakness of both legs, greater in the left. Reflexes in her left lower extremity were hyperactive with sustained clonus. Plantar responces were flexor bilaterally. Palin X-rays showed scoliosis of thoracic spine with the top at T7 level and calcified intervertebral masses at T10-11, T11-12 and T12-L1, extending into the canal that were confirmed more clearly by tomography. Myelography by a cisternal puncture disclosed a complete block at the level of T10. The patient underwent total laminectomy of T9 through L2 and extradural anterior decompression with the removal of calcified discs. At her follow-up examination 12 months after surgery she could walk for herself with some residual neurological signs, minimal weakness in the right leg and hypesthesia up to the level of T12 in the left. We have discussed the incidental, related diagnostic and operative problems of this disease.
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PMID:[The protrusion of thoracic intervertebral disc-thoracic spondylosis (author's transl)]. 123 40

The Chiba Spinal System was developed for three-dimensional correction of scoliosis. The system consists of a 7-mm diameter smooth solid rod, closed and open hooks, and specially designed conical sleeves. The rod does not have ratchets or threads, thus minimizing stress concentrations. The closed and open hooks are attached to the rod using the conical sleeves. The sleeves have longitudinal slits permitting compression during insertion. The outrigger system with a torque wrench is used to correct the deformity in the frontal plane. When correcting lordosis with vertebral rotation, a sagittal correction device may be used. Fifty-two patients with idiopathic scoliosis were operated on with this system from 1986 through 1989. The average correction of the scoliosis was 58%. For the 21 patients with thoracic lordosis less than 0 degrees, the mean preoperative lordosis was -8 degrees, which was corrected to +7 degrees. The mean correction of the vertebral rotation was 24%. Two patients complained of paresthesia of the thoracic region after operation, but this disappeared within 2 weeks. In 32 patients with more than 2 years' follow-up, the mean loss of correction was 3 degrees. One case with instrumentation failure was noted.
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PMID:Chiba Spinal System in the operative management of scoliosis. 144 5

Spinal cord monitoring with the spinal cord evoked potentials using direct spinal cord stimulation is a safe and reliable method. The purpose of this study was to determine the degree of the potential change that is permissible to avoid severe neurological complications. Three hundred and fifty-four patients with spinal disorders were monitored with this method. The peak amplitude of the potential used as an indicator of the spinal cord function was evaluated quantitatively. The amplitude changes of those with neurological complication were compared with those without complication. The results in 135 patients with scoliosis were analyzed. False negative results were obtained in two patients who had only slight paresthesia. In the patients with normal spinal cord function, up to 50% reduction of the amplitude was permissible. Spinal monitoring is also useful in the improvement of operative technique because it can immediately disclose injury to the cord resulting from surgical misconduct.
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PMID:[A clinical study on intra-operative spinal cord monitoring with spinal evoked potentials for scoliosis]. 317 Dec 80

A study of the efficacy of two techniques of spinal instrumentation on patients with adolescent idiopathic scoliosis was performed. Twenty consecutive patients treated with a single Harrington distraction rod and sublaminar wiring (H-SL) were compared with 20 consecutive patients treated with a single Harrington distraction and spinous process wiring (H-SP). The patients in both groups were similar in age, curve magnitude, curve flexibility, and curve type. The immediate postoperative correction (61% H-SL, 58% H-SP) and follow-up correction (50% H-SL, 49% H-SP) were equivalent. No pseudarthroses were recognized in either group. One patient with H-SL had transient postoperative paresthesias.
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PMID:A comparison between spinous process and sublaminar wiring combined with Harrington distraction instrumentation in the management of adolescent idiopathic scoliosis. 335 Sep 44

The Chiari-syndrome Type I being a malformation of the posterior cranial fossa often leads to syringomyelia. The disease becomes apparent in adolescence with kyphoscoliosis, headache, vertigo, ataxy, hearing loss, partial paralysis and other neurological disorders. The onset is typically characterized by dissociated anesthesia, due to the frequent localization of the syrinx in the neighborhood of the posterior column of the spinal cord. It is reported a case of an 15-years-old-girl suffering from intensive headache, recurrent left-sided paresthesias and progredient scoliosis. Somatosensory evoked potentials of left ulnar and tibial nerves revealed a complete loss of cortical response. Diagnostic imaging showed an Chiari-malformation I with herniation of cerebellar tonsils and secondary syringomyelia of the cervical spinal cord. After surgical treatment with posterior fossa decompression, C1 laminectomy and partial excision of cerebellar tonsils the patient had a clear improvement in symptoms. Postsurgical the width of cervical syrinx decreases. Now somatosensory evoked potentials of the left ulnar and tibial nerves show amplitude-reduced cortical responses with a normal latency.
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PMID:[Evoked potentials in Chiari-malformation type I with syringomyelia--a case history]. 1292 16

The most frequent type of Charcot-Marie-Tooth (CMT) neuropathy is that associated with the 17p11.2-p12 chromosome duplication, whose characteristics have been well described in European and North American populations. In this study, we analyzed a Brazilian population exhibiting the mutation, found in 57 patients from 42 families (79%) of a cohort of 53 families with demyelinating CMT. Almost 20% of the duplicated cases were sporadic. In 77% of the duplicated families the mutation event occurred in the hot spot area of the CMT1A-Rep region. Forty-five percent of patients were females, 84% were Caucasians and 13% of African descent. Distal limb weakness was the most frequent abnormality, appearing in 84% of patients, although uncommon manifestations such as severe proximal weakness, floppy baby syndrome, diaphragmatic weakness and severe scoliosis were also observed. One patient was wheelchair-bound, and three suffered severe hand weakness. Sensory abnormalities were detected in 84% of the cases, but 80% were unaware of this impairment. Twelve patients complained of positive sensory manifestations such as pain and paresthesias. Progression was reported by 40%. Motor conduction velocities in the upper limbs were always less than 35 m/s, and less than 30.4 m/s in the peroneal nerve. The findings of this study expand the clinical spectrum of the disease.
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PMID:17p duplicated Charcot-Marie-Tooth 1A: characteristics of a new population. 1576 65

Autosomal dominant brachyolmia (Type 3, OMIM #113500) belongs to a group of skeletal dysplasias caused by mutations in the transient receptor potential cation channel, subfamily V, member 4 (TRPV4) gene, encoding a Ca++-permeable, non-selective cation channel. The disorder is characterized by disproportionate short stature with short trunk, scoliosis and platyspondyly. The phenotypic variability and long-term natural course remain inadequately characterized. The purpose of this study was to describe a large Swedish family with brachyolmia type 3 due to a heterozygous TRPV4 mutation c.1847G>A (p.R616Q) in 11 individuals. The mutation has previously been detected in another family with autosomal dominant brachyolmia [Rock et al., 2008]. Review of hospital records and patient assessments indicated that clinical symptoms of brachyolmia became evident by school age with chronic pain in the spine and hips; radiographic changes were evident earlier. Growth was not affected during early childhood but deteriorated with age in some patients due to increasing spinal involvement. Affected individuals had a wide range of subjective symptoms with chronic pain in the extremities and the spine, and paresthesias. Our findings indicate that autosomal dominant brachyolmia may be associated with significant long-term morbidity, as seen in this family.
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PMID:Autosomal dominant brachyolmia in a large Swedish family: phenotypic spectrum and natural course. 2467 93

An 11-year-old girl underwent T4 to L1 posterior instrumented scoliosis correction for adolescent idiopathic scoliosis. Postoperative clinical examination revealed left-sided Horner's syndrome which was preceded by left-sided C8 paraesthesia. The Horner's syndrome resolved after 14 hours following weaning and removal of the epidural catheter. Horner's syndrome following posterior instrumented scoliosis correction associated to epidural use is extremely rare. Surgeons must be aware of the risks of epidural placement and the need for close monitoring of associated complications. Alternative aetiology producing a Horner's syndrome must always be considered because of its devastating long term sequela if missed.
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PMID:Horner's Syndrome Secondary to Epidural Anaesthesia Following Posterior Instrumented Scoliosis Correction. 2570 45