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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three cases of tethered cord syndrome are presented with reference of reported literatures. This syndrome is usually associated with spina bifida occulta and cutaneous manifestation such as subcutaneous tumor, skin dimple, hairy patch, and neavus. Presented symptoms are various progressive neurological deficits involving weakness and/or sensory disturbance of lower limbs, bowel and/or bladder dysfunction, leg and/or low back pain, and orthopedic pathological conditions such as scoliosis and/or foot deformities. Properly performed myelography is the only method of preoperative confirmation of this syndrome. As the surgical release of the tethered spinal cord is effective for these various symptoms except for foot deformity, early investigation and early surgery are recommended.
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PMID:[Tethered cord syndrome--our three cases and the review of the literature (author's transl)]. 66 74

Homogenised tissues or tissue culture supernatant fluid containing a noncytopathic pestivirus obtained from a lamb with a neurologic form of border disease, were inoculated into ewes at different stages of pregnancy. Foetal death occurred in 9 ewes of those inoculated between 19 and 47 days of pregnancy while 3 ewes did not lamb. Eight of the foetuses were aborted between 77 and 132 days of pregnancy; of these 6 were autolysed or mummified and one had arthrogryposis. The one full-term dead lamb had a hairy birth coat and lissencephalic micrencephaly. Foetal death occurred in only 7 of 14 ewes inoculated between 57 and 72 days of pregnancy. Four of these ewes aborted between 77 and 108 days of pregnancy and 3 gave birth to full-term, dead, hairy lambs. The remaining 7 ewes gave birth to live hairy lambs with severe inco-ordination. All lambs carried to term and aborted foetuses or lambs that could be examined had a range of intracranial malformations including focal leucomalacia, micrencephaly, hydranencephaly, porencephaly, lissencephaly and cerebellar hypoplasia. Some lambs also had skeletal abnormalities including arthrogryposis, scoliosis and brachygnathia inferior. The pestivirus isolate used in these trials produced more severe effects on the ovine foetus than previously observed in similar inoculation trials using pestivirus isolates from border disease lambs without nervous signs.
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PMID:Pathology in the ovine foetus caused by an ovine pestivirus. 631 Nov 54

A clinical entity called "the epidermal nevus syndrome" does not exist. Rather, there are various epidermal nevus syndromes that can be distinguished by clinical, histopathological, and genetic criteria. In this review, five distinct epidermal nevus syndromes, recognizable by different types of associated epithelial nevi, are described. The Schimmelpenning syndrome is characterized by a sebaceous nevus associated with cerebral anomalies, coloboma, and lipodermoid of the conjunctiva. By contrast, cataracts are a prominent feature of the nevus comedonicus syndrome. The pigmented hairy epidermal nevus syndrome includes Becker nevus, ipsilateral hypoplasia of the breast, and skeletal defects such as scoliosis. In the Proteus syndrome, the associated epidermal nevus is of a flat, velvety, nonorganoid type. The CHILD syndrome occurs almost exclusively in girls. The associated CHILD nevus shows unique features such as a diffuse form of lateralization, ptychotropism, and microscopic changes of verruciform xanthoma. The five epidermal nevus syndromes differ in their genetic basis. The Schimmelpenning and nevus comedonicus syndromes are most likely nonhereditary traits. By contrast, the pigmented hairy epidermal nevus syndrome and the Proteus syndrome may be explained by paradominant inheritance. The CHILD syndrome is caused by an X-linked dominant mutation exerting a lethal effect on male embryos. A correct diagnosis of these phenotypes is important for both recognition and treatment of associated anomalies as well as for genetic counseling.
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PMID:Epidermal nevus syndromes. 764 Jan 90

We reviewed the results for forty-three patients who had a diastematomyelia. All of the patients had been skeletally immature when the diagnosis was made, the mean age being six years (range, birth to thirteen years), and were skeletally mature by the time that they were evaluated by us. When they were first seen at our institution, twenty-four patients (56 per cent) had a cutaneous lesion, such as hairy patch, dimple, hemangioma, subcutaneous mass, or teratoma at or near the level of the diastematomyelia; thirty-four patients (79 per cent) had congenital scoliosis; and forty-two patients (98 per cent) had at least one associated musculoskeletal anomaly, such as spinal dysraphism, asymmetry of the lower extremities, club foot, or a cavus foot. In twenty-seven patients (63 per cent), the diastematomyelia was located in the lumbar spine. Thirty-six patients had eighty-four neurological manifestations. Resection of the spur was performed in thirty-three patients at a mean age of seven years (range, three months to seventeen years). Twenty-two patients who had a resection had no change in neurological condition, nine patients had improvement, and one patient had one symptom improve and another symptom worsen after the operation. We believe that resection of the spur should be performed in patients who have progressive neurological manifestations. Patients who do not have progressive neurological manifestations should be observed; if progression is noted, a resection should then be performed.
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PMID:Evaluation and treatment of diastematomyelia. 840 52

A 15 year-old girl presented with numerous congenital melanocytic nevi, occasionally hairy, with a segmental distribution at the left pre-auricular region. On the left side of the back of the neck there were multiple melanocytic nevi with a warty appearance, which had started to appear when she was 5 and which had remained stable from the time she was 10. These lesions had a distribution reminiscent of an epidermal nevus with a pattern similar to Blaschko's lines. On general physical examination there was a moderate degree of idiopathic scoliosis, with a left lumbar curvature. Biopsies were taken from both types of nevus which were diagnosed as congenital compound melanocytic nevus and acquired compound melanocytic nevus respectively. We consider that the segmental distribution of the melanocytic nevi of this patient supports the theory that a genetic defect determined the appearance of both these congenital and acquired lesions.
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PMID:Segmental arrangement of multiple, partly congenital and partly acquired melanocytic nevi. 964 18

Becker nevus is a hyperpigmented hamartoma with an irregular outline and often hairy. It is normally found on the shoulders and chest, although it can appear in other areas. Becker nevus is sometimes associated with other muscular, skeletal, or cutaneous abnormalities such as ipsilateral breast hypoplasia or scoliosis. This characteristic phenotype of Becker nevus associated with unilateral breast hypoplasia or other abnormalities is referred to as Becker nevus syndrome. Although the lesions usually become apparent during adolescence, they are present from birth and represent part of the spectrum of so-called epidermal nevus syndromes. We present 4 cases of Becker nevus syndrome in which Becker nevus was associated with ipsilateral breast hypoplasia and, less consistently, other abnormalities.
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PMID:[Becker nevus syndrome]. 1796 53

Concurrent infection with peste des petits ruminants virus (PPRV) and pestivirus was diagnosed in stillborn twin lambs. With the flock history, the findings of epidermal syncytial cells and necrotizing bronchitis/bronchiolitis prompted testing for PPRV infection, and PPRV antigen was detected by immunohistochemistry (IHC) in the skin, lungs, kidneys, rumen, and thymus. Macroscopic anomalies that were typical of border disease included scoliosis, brachygnathism, prognathism, arthrogryposis, hydranencephaly, cerebellar hypoplasia, and hairy fleece; pestiviral antigen was detected by IHC in the brain, liver, lungs, and kidneys. Tissues from both lambs were positive by reverse transcriptase-polymerase chain reaction (RT-PCR) for PPRV and pestivirus. To the authors' knowledge, PPR has not been reported previously as a congenital infection or in combination with pestiviral infection.
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PMID:Concurrent peste des petits ruminants virus and pestivirus infection in stillborn twin lambs. 1842 32