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At least 1,243 records · Page 69Linked to original sources
PubMed1975

Hereditary defect of the sacrum.

A family in which both the mother and two of her daughters had partial agenesis of the sacrum and coccygeal bones is presented. Further studies demonstrate a neurogenic bladder and a presacral mass in the 4-year-old girl who had severe urinary incontinence. The mode of inheritance in this entity may be x-linked dominant as has been suggested. Previously by Cohn and Bay-Nielsen (1969). The gene may act as a lethal factors in hemizygous males. A brief review of the literature showed a remarkable paucity of reports on the hereditary aspects of sacrococcygeal defects in man. It is concluded that this may be misleading, since the true incidence of hereditary sacroccygeal deformities can not possibly be established until family members of the probands are also examined radiologically.

Adult↗
PubMed1990

Occult spinal dysraphism: neuroradiological study.

We present a retrospective study of occult spinal dysraphism in 47 children aged 0 to 14 years, all studied with plain X-rays, 60% with CT and myelo-CT, and 40% with MR. We consider the classification and grading of these malformations, clinical, neuroradiological patterns, and indications for surgery. In the light of our findings and of the published data MR emerges as the key investigation. Only in a few cases of great anatomical complexity is it now necessary to perform CT and myelo-CT as well. A case in point is when the conus and thickened filum terminale are inextricably bound together and can no longer be considered separate structures. We propose the term "neurofibrous structure" to define the conus-thickened-filum-terminale unit when these structure are no longer distinguishable.

Adolescent↗
PubMed1985

Intrasacral meningeal cyst demonstrated by sacral epidurography.

A case of intrasacral meningeal cyst is reported in which radiculography and computed tomography were not conclusive in diagnosing the lesion. Sacral epidurography delineated the cyst very clearly and is proposed as a complementary imaging technique in lesions of the sacral canal.

Adult↗
PubMed1982

Investigation of brachial plexus injuries by intraoperative cortical somatosensory evoked potentials.

With the intention of estimating the extent and site of damage in brachial plexus injuries which involve close to the root outlet and also the distal portion, cortical somatosensory evoked potentials (SEP) were recorded in 21 patients by directly stimulating the exposed brachial plexus. In 38 avulsed nerve roots which showed positive sensory action potentials (SNAP)/nerve action potentials (NAP) in their peripheral part, 15 roots (nine patients) were apparently in continuity and confirmed as root avulsion injury by the absence of cortical SEP. However, 11 roots (ten patients) which showed neither SNAP nor SEP would suggest either extensive lesions involving root and more distal segment or a combination of root avulsion and postganglionic injury. Fourteen patients who showed a positive Tinel's sign had at least one root with a postganglionic type lesion, but cortical SEP evoked by stimulation of the most proximal root zone sometimes revealed a reduced amplitude and prolonged latency. This would indicate the retrograde extension of damage. To confirm the extent and degree of the nerve lesions in brachial plexus injuries, an intraoperative SEP and NAP recording is very useful, practical, and also indispensable.

Adolescent↗
PubMed1995

Intranasal (transethmoidal) encephalomeningocele. Case report.

Intranasal encephaloceles are rarely encountered in pediatric neurosurgery. The symptoms and clinical features may mimic those of nasal polyp. It is important to know the type of basal encephalomeningocele for appropriate surgical intervention. Computed tomographic examination is helpful for differential diagnosis of the encephalocele sac and localization of the cranial bone defect.

Craniotomy↗
PubMed1983

Occult cranium bifidum. Radiological and surgical findings.

Six cases of congenital subscalp nodule associated with underlying cranium bifidum are reported. A plain skull roentgenogram showed a midline bone defect in the parieto-occipital region near the lambda. CT scan demonstrated neither brain malformation nor ventricular deformity except for the high position of the straight sinus. Cerebral angiography revealed an elongation of the vein of Galen and anomalous upward course of the straight sinus. At surgery, the tumor was solid and connected to a cord which extended intracranially via the cranium bifidum and blended with thickened arachnoid membrane either on the dorsal aspect of the midbrain or at the surface of the anterior vermis. Histologically, the tumor consisted in all cases of arachnoid cells and fibrous tissue with immature glial cells in one case. Possible pathogenesis of these tumors could be a result of the fetal nuchal bleb.

Cerebral Angiography↗
PubMed1992

Neurofibromatosis with dural ectasia and bilateral symmetrical pedicular clefts: report of two cases.

We present two cases of bilateral, symmetrical pedicular clefts associated with dural ectasia in von Recklinghausen's disease (neurofibromatosis). In one case the pedicular cleft was at the T12 level, while in the other it was at L4, and was responsible for spondylolisthesis. Two hypotheses are advanced to explain the cleft: (1) a congenital, dysplastic osseous defect and (2) bilateral stress fractures related to thinning of the pedicle caused by dural ectasia.

Adult↗
PubMed1996

Idiopathic dural herniation of the thoracic spinal cord.

Symptomatic anterior or anterolateral dural herniation of the spinal cord is rare, and not uncommonly misdiagnosed, both clinically and radiologically. We present four patients with a radiological diagnosis of herniation of the thoracic spinal cord, and review the current literature. All affected patients have been adults, typically presenting with long-standing, unexplained sensory symptoms and eventually developing a Brown-Séquard syndrome, with or without motor changes. Herniation occurs in the upper or midthoracic region, between the T2 and T8 levels.

Adult↗
PubMed1985

Congenital absence of pyramids and its significance in genetic diseases.

Bilateral absence of the pyramids, best assessed in transverse sections of the medulla oblongata, was found in 0.7% of 2,850 autopsies performed at the Royal Children's Hospital, Melbourne (Australia). A strong association with some forms of congenital aqueductal stenosis was observed and evaluated in further cases ascertained through other hospitals. The very strong association observed with X-linked congenital aqueduct stenosis may be useful diagnostically but must be evaluated in other centres. At least, the presence of pyramids seems strong evidence against this diagnosis. Absence of the pyramids was also observed in several known or new genetic syndromes - Meckel's syndrome, the HARD syndrome, two sisters with congenital aqueductal stenosis and polydactyly and two families with microcephaly and arthrogryposis.

Abnormalities, Multiple↗
PubMed1977

[Enterogenous cyst of the spinal cord associated with mucomyelia (author's transl)].

A woman of 22 years developed heavy pain in the neck and arms with weakness and paresthesia. She died two days after a myelography which was normal. At autopsy an enterogenous extra- and intra-medullary cyst of the cervical cord (C3, C4) was found. In addition the cervical cord apart from the cyst and the uppermost thoracic cord presented central clefts which were filled with mucilage. These findings are named mucomyelia and have not been reported previously.

Cysts↗
PubMed1985

Surgically confirmed myelographic classification of congenital intraspinal lipoma in the lumbosacral region.

Twenty-four cases of histologically confirmed congenital intraspinal lipoma of the lumbosacral region were studied by means of myelography with metrizamide. The findings were compared with intraoperative observations. Myelography with metrizamide clearly revealed the detailed intrathecal structures and allowed a classification of intraspinal lipomas into four types, in terms of their insertion into the conus medullaris: dorsal type, either with direct or indirect (via an intrathecal stalk) insertion of the extrathecal lipoma into the dorsal aspect of the conus medullaris; caudal type; combined type; and filar lipoma. Based on our surgical experience in untethering and decompression of the lesions, the classification was found to be useful in designing a safe and effective surgical procedure which minimized all possible trauma to the intrathecal neural structures.

Adolescent↗