Neurenteric cyst of the craniocervical junction--case report. (1/105)

A 60-year-old female presented with occipital headache and limitation of neck movement. Neurological examination showed weakness of the right sternocleidomastoid muscle. Magnetic resonance imaging revealed a cystic lesion at the craniocervical junction and posterior compression of the brain stem. The lesion was totally removed through the transcondylar approach. The histological diagnosis was neurenteric cyst. The transcondylar approach provides a direct operative view of the clivus and anterior craniovertebral junction.  (+info)

Split cord malformation (scm) in paediatric patients: outcome of 19 cases. (2/105)

There had been considerable debate regarding the surgical outcome of neuro-orthopaedic syndromes (NOS) and neurological syndromes in cases of split cord malformation (SCM). On retrospective analysis of 19 cases of SCM, thirteen were grouped under (Pang) type I and 6 in type II. Their age ranged from 1 month to 9 years (mean 3.5 years). 14 of these were male children. The NOS without neurological signs was detected in 6 cases where as pure neurological signs without NOS were seen in 8 patients. However, the rest 5 had mixed picture of NOS and neurological dysfunction. Nine of 19 cases presented with cutaneous stigmata, mainly in the form of hairy patch. 18 cases had other associated craniospinal anomalies i.e. hydrocephalus, meningomyelocoele, syrinx, dermoid, teratoma etc. Detethering of cord was done in all cases by removal of fibrous/bony septum. Associated anomalies were also treated accordingly. Follow up of these cases ranged from 6 months to 6 years. Six cases of NOS group neither showed deterioration nor improvement, and remained static on follow up. However, four of 8 children with neurological signs showed improvement in their motor weakness, and 1 in saddle hypoaesthesia as well as bladder/bowel function. In 5 cases of mixed group, two had improvement in their weakness and one in hypoaesthesia, but no change was noticed in NOS of this group as well. Hence surgery seemed to be effective, particularly in patients with neurological dysfunction.  (+info)

The effect of GABA receptor ligands in experimental spina bifida occulta. (3/105)

BACKGROUND: The pathophysiology behind spina bifida and other neural tube defects (NTDs) is unclear. Folic acid is one variable, but other factors remain. Studies suggest that substances active at the GABA receptor may produce NTDs. To test this hypothesis pregnant rats were exposed to either the GABA a agonist muscimol (1, 2 or 4 mg/kg), the GABA a antagonist bicuculline (.5, 1, or 2 mg/kg), the GABA b agonist baclofen (15, 30, 60 mg/kg), or the GABA b antagonist hydroxysaclofen (1, 3, or 5 mg/kg) during neural tube formation. Normal saline was used as a control and valproic acid (600 mg/kg) as a positive control. The embryos were analyzed for the presence of a spina bifida like NTD. RESULTS: After drug administration the pregnancies were allowed to proceed to the 21st day of gestation. Then embryos were removed and skeletons staining and cleared. Vertebral arch closure was measured. Results indicate that the GABAa receptor agonist muscimol, the GABAa receptor antagonist bicuculline, and the GABAb agonist baclofen produced NTDs characterized by widening of the vertebral arch. Oppositely the GABAb antagonist hydroxysaclofen produced narrowing of the vertebral arches. CONCLUSIONS: The findings indicate that GABA a or b ligands are capable of altering neural formation. GABA may play a greater than appreciated role in neural tube formation and may be important in NTDs. The narrowing of the vertebral arch produced by the GABA b antagonist hydroxysalcofen suggests that GABA b receptor may play an undefined role in neural tube closure that differs from the GABA a receptor.  (+info)

Spinal congenital dermal sinus: an experience of 23 cases over 7 years. (4/105)

Spinal congenital dermal sinus is a rare entity, which supposedly results from the failure of neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation. The present study was undertaken to know the clinical profile of these patients, to study associated anomalies and to assess the results of surgical intervention. We had 23 patients with male : female ratio of 9:16. Only 2 patients were below 2 years of age and most cases (16) were between 2-16 years (mean age =10.2 years). Lumbar region (17 cases) was most frequently involved, followed by lumbosacral and thoracic region in 3 patients each. Only three patients were asymptomatic at the time of presentation. Most of the cases presented with evidence of neural compression or tethered cord syndrome. Only one case presented with spinal abscess. The motor, sensory and autonomic deficits were seen in 20, 11 and 12 patients respectively. Scoliosis and CTEV (congenital talipus equino varus) were the common associated anomalies. MRI revealed associated dysraphic state of spinal cord in 21(>90%) cases. All patients underwent surgical exploration and repair of dysraphic state and excision of the sinus. None of the asymptomatic patients deteriorated. Overall 8 patients improved, 14 got their neurological status stabilized, including 3 asymptomatic cases. Only one patient deteriorated. Postoperative wound infection was seen in 2 cases. As age advances, the chance of developing neurological deficit increases. Associated dysraphic state should be looked for and treated simultaneously, using microsurgical technique, whenever possible. It is better to treat all these cases with aggressive surgical intervention before the neurological deficits appear.  (+info)

Spina bifida occulta in isthmic spondylolisthesis: a surgical trap. (5/105)

An 11-year-old girl presented with symptomatic grade IIB isthmic type spondylolisthesis, with an elongated pars, confirmed on magnetic resonance imaging (MRI). Posterolateral in situ fusion of L5/S1 was performed. At surgery, a significant bony defect in the posterior aspect of S1 was noted. Awareness of this possible co-existence is paramount if iatrogenic damage to neural elements is to be avoided during surgery.  (+info)

Investigation of daytime wetting: when is spinal cord imaging indicated? (6/105)

BACKGROUND: Most children with daytime wetting have detrusor instability. A minority have neuropathic vesicourethral dysfunction. The commonest cause is spina bifida, which may be closed. Clinical features suggestive of closed spina bifida include cutaneous, neuro-orthopaedic or lumbosacral spine x ray abnormalities, impaired bladder sensation, and incomplete bladder emptying. MRI is the ideal method for detecting spinal cord abnormality. It has been suggested that MRI spine is an unnecessary investigation in children with daytime wetting in the absence of cutaneous, neuro-orthopaedic, or lumbosacral spine x ray abnormalities. AIM: To clarify indications for magnetic resonance imaging (MRI) of the spine in children with voiding dysfunction. METHODS: Retrospective study of children with voiding dysfunction referred from the Guy's Hospital neurourology clinic for MRI spine between April 1998 and April 2000. Clinical notes and results of investigations, including urodynamic studies and MRI spine were reviewed. RESULTS: There were 48 children (median age 9.1 years). Closed spina bifida was detected in five, of whom four had neuropathic vesicourethral dysfunction confirmed by urodynamic studies. Impaired bladder sensation and incomplete bladder emptying were more frequent in these children than in those with normal MRI spine. One child with spinal cord abnormality had no cutaneous, neuro-orthopaedic, or lumbosacral spine x ray abnormalities. CONCLUSION: Spinal cord imaging should be considered in children with daytime wetting when this is associated with impaired bladder sensation or poor bladder emptying, even in the absence of neuro-orthopaedic, cutaneous, or lumbosacral spine x ray abnormalities.  (+info)

Sonography for detection of spinal dermal sinus tracts. (7/105)

OBJECTIVE: It is well known that spinal dysraphism may be complicated by meningitis as a result of infection traveling from the skin along a patent dermal sinus tract. The only clue is the lower back cutaneous lesions. Our aim was to investigate the correlation between cutaneous lesions, patent dermal sinus tracts, and spinal dysraphism and their complications. METHODS: Five patients (3 female and 2 male) with spinal patent dermal sinus tracts were studied. We used a 7-MHz linear transducer with a two-dimensional real-time sonographic system to insonate and obtain transverse and longitudinal views of the spinal cord and subcutaneous area, extending from the cervical cord to the sacral areas. Subsequently, we performed spinal magnetic resonance imaging in every patient to confirm the diagnosis and to evaluate the intraspinal conditions. RESULTS: The associated central nervous system anomalies and complications were tethered cords (n = 5), dermoid cysts (n = 3), lipoma (n = 2), central nervous system infections (n = 2), and syringomyelia (n = 1). The outcomes were better in those who received surgical intervention before they were infected. CONCLUSIONS: Early detection of spinal patent dermal sinus tracts and related anomalies was accomplished with spinal sonography and allowed for prophylactic treatment (e.g., early surgical intervention) before the onset of neurologic deficits.  (+info)

Occult spinal dysraphism and its association with hip dysplasia in females. (8/105)

We examined the pelvic radiographs of two groups of patients (more than 12 years of age) from six medical centres. Hip dysplasia was considered to be present if Shenton's line was broken and more than one third of the femoral head was revealed to be uncovered in an antero-posterior radiograph of the pelvis. Patients with hip dysplasia due to teratological or neurological causes were excluded. There were 291 patients with treated or untreated hip dysplasia in the dysplastic group. The control group of 415 individuals was collected from consecutive outpatients (with a pre-set standardised female/male ratio) for whom an antero-posterior radiograph of the pelvis had been made in one of two medical centres and which did not disclose any abnormality of the hip joints. The aim of the study was to assess the co-existence of hip dysplasia and occult spinal dysraphism. Radiographs of all patients were examined, and any partial or complete defect of the posterior vertebral arch was recorded. In the dysplastic group, a defect was recorded in 23% (67/291) radiographs and in the control group in 12% (48/415). In both groups, L5 and S1 were the most commonly recorded sites with a defect. In the dysplastic group, a defect was recorded in 56/190 females and in the control group in 30/302 females. In males, there was no significant difference between the recorded findings in the two groups. In females with hip dysplasia, occult spinal dysraphism seems to be fairly common.  (+info)

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