Hormonal control of female sexual attractiveness proceptivity, and receptivity in rhesus monkeys

Microsurgical exploration of 15 adults with Arnold-Chiari malformation with and without hydromyelia using 3 to 20 X magnification has led to the following conclusions. Hydromyelia, associated with Arnold-Chiari malformation, is a progressive mechanical disorder that causes spinal cord deficits by pressure distention of the cord. Arnold-Chiari malformation causes slowly or suddenly progressive bulbar dysfunction by impaction of the malformation in the foramen magnum. Decompression of both can be achieved by a suboccipital carniectomy, upper cervical laminectomy, establishing an outlet from the fourth ventricle, and opening the distended cord in the thinnest exposed area, which is usually along the dorsal root entry zone. If Pantopaque myelography in patients in the supine position shows the Arnold-Chiari malformation, hydromyelia can be established as a cause of central cord deficit even if myelography shows the cord size to be normal. Syringomyelia, traditionally considered a degenerative disease, is a less common cause of a slowly progressive central cord deficit than either hydromyelia or intramedullary tumor.     

Hydrodynamics of syringomyelia

Abnormality of CSF hydrodynamics is an important factor of the pathogenesis of syringomyelia. Recent development of cine-MRI visualized real time CSF movement in the syrinx or subarachnoid space. In the present study, twenty cases of syringomyelia associated with Chiari I malformation, 10 cases of syringomyelia associated with spina bifida aperta, and 10 cases of syringomyelia associated with spina bifida occulta were studied with MRI and cine-MRI. The result demonstrated that hydrodynamics of these three groups were different to each other. Chiari I malformation showed CSF hydrodynamic abnormality at the foramen magnum and marked flow void in the syrinx. On the other hand, myelomeningocele showed hydrocephalus and less prominent flow void in the syrinx. Spinal lipoma which is the majority of spina bifida occulta showed no abnormality of CSF hydrodynamics. In conclusion, the selection of treatments should be individualized depending on the hydrodynamical abnormality of each patient.     

Effects of zinc supplementation on the immune system and on antibody response to multivalent influenza vaccine in hemodialysis patients

BACKGROUND: It has been theorized that fetal myelomeningocele repair may reduce ongoing intrauterine injury and perhaps allow healing and regeneration of dysplastic neural tissue. We report on the postnatal imaging studies of the first 4 patients to have undergone intrauterine myelomeningocele repair at our institution. METHODS: Each of the 4 patients underwent postnatal sonographic and MRI. In addition, the postnatal ultrasounds of these 4 were compared to a group of retrospective controls. RESULTS: MRI scans of the 4 experimental subjects revealed no evidence of hindbrain herniation while other stigmata of the Chiari-II malformation persisted. In comparison to the retrospective controls this absence of herniation was distinctly unusual. CONCLUSION: Intrauterine myelomeningocele repair may reduce the degree of hindbrain herniation normally seen in patients with myelomeningocele. This raises the possibility that intrauterine repair may decrease the morbidity associated with the Chiari type-II malformation including brainstem dysfunction, hydrocephalus and syringomyelia.     

Mucosal immunity in the human female reproductive tract: cytotoxic T lymphocyte function in the cervix and vagina of premenopausal and postmenopausal women

Hydromyelia is a dilation of the spinal cord central canal. In man this may be due to congenital malformations such as Dandy-Walker syndrome and Chiari malformations or may be acquired as result of infection, trauma or neoplasia. In dogs hydromyelia may be accidentally diagnosed during routine cisterna magna myelography. Hydromyelia, and its possible etiology, may be confirmed by means of computed tomography or magnetic resonance imaging. Three dogs with hydromyelia due to differing etiologies are described.     

Clinical features and MR imaging in children with repaired myelomeningocele

The aim of the study is to define the role of associated malformations in the clinical evolution of children affected by myelomeningocele. MRI investigation of the spinal cord was carried out on 25 patients between the age of 7.3 and 18.10 with MMC repaired and followed up for at least 7 years. The relation between associated malformations demonstrated by MRI and clinical trend was analysed. The results are the following: 1) presence of asymptomatic tethered cord in all cases; 2) high frequency (92%) of Chiari malformation; 3) presence of syringomyelia in 20% of patients, symptomatic in 1; 4) presence of ventricular enlargement in 72% of cases without increased intracranial pressure syndrome. For a better therapeutic approach prospective MRI studies are needed in order to follow up associated malformations.     

A case of true human tail accompanied with spinal lipoma

Human tails have been attributed to a disturbance in fetal tail regression which normally occurs at the gestation age of about 6 weeks. To date, more than 100 cases of human tails have been reported. However, reports of true human tails, which involve the coccygeal vertebrae, are rare. We recently encountered a patient with a true human tail which involved the coccygeal vertebrae and was accompanied by lumbar spinal lipoma and spina bifida. A four-year-old boy was brought to our clinic with complaints primarily of painful mass. The boy had no neurological abnormalities. Physically, a tail bone projected, slightly in the lumbar area, with a linear depression in the center. A hard tail bone was palpable subcutaneously. A soft mass was palpable in the lumbar region, which was accompanied by hemangioma on the superficial layer of the skin in this region. On X-ray films, the tail bone lacked the normal curvature and it projected linearly in the posterior direction. CT scans revealed spina bifida at the level below L2. MRI disclosed spina bifida (at the level below L2), spinal lipoma and a tethered cord. During surgery, the tail bone was first resected over a distance of one and a half vertebral bodies. The lumbar tumor, which continued into the spinal canal, was removed as completely as possible after incision of the dura mater. To free the tethered cord, the tensioned, hypertrophic filum terminale was dissected. Although the morphological diagnosis of this condition is easy, the high incidence of complication by other anomalies makes it essential to perform thorough preoperative examinations with CT and MRI.(ABSTRACT TRUNCATED AT 250 WORDS)     

Clinical consequences of spina bifida occulta

OBJECTIVE: To study data concerning the pathogenesis, frequency of occurrence, clinical manifestations and associated abnormalities of spina bifida occulta (SBO) and re-evaluate the clinical importance of the lesion. DATA SOURCES: International journal articles indexed through Medline, and specific related texts, the majority of which were published after 1989. Key indexing terms used were spina bifida occulta, tethered cord syndrome and spondylolysis. RESULTS: The reported frequency of occurrence of SBO varies widely, depending largely on the age groups included in a particular study. The most accurate estimate of occurrence rate is 17% of examined spines. There is a significant association of some cutaneous stigmata, most notably hypertrichosis, with midline posterior arch defects. An increasing amount of evidence links SBO with a number of specific anomalies and clinical syndromes, including intraspinal lipoma, tethered cord syndrome, genitourinary dysfunction, increased incidence of disc pathology, lumbar spondylolysis, foot deformities and syringomyelia. A questionable association exists with epilepsy. A supposed link between constipation and SBO is lacking sufficient data to support it. CONCLUSIONS: SBO may be associated with pathology and significant sequelae, although the majority of lesions pose no clinical threat. The predictive value for adverse sequelae in a particular lesion is difficult to assess; however, multilevel occurrence and more expansive involvement in a given segment seem to be associated with higher risk of sequela. The treatment for SBO with progressive neurologic deficit is surgical intervention; however, reversal of the deficit is unusual and a halting of neurologic deterioration is a more realistic goal. Early diagnosis of this lesion, before the age of 3 yr, is associated with better surgical outcomes.     

A case of lumbosacral lipoma-associated adult onset tethered cord syndrome with initial symptoms of sensory disturbance and intractable foot ulcers]

A 63-year-woman who complained of sensorimotor disturbance of the lower extremities and urinary disturbance was presented. She noted loss of superficial sensation in both feet and foot ulcers at the age of 20 years. Her illness was initially diagnosed as hereditary sensory neuropathy type 1 (HSN1). The foot ulcers were so intractable that she had to have her right leg amputated at the age of 48 years. She had a severely impaired superficial sensation in the lower extremities and buttock, distal weakness of the left leg, and dysuria at the age of 60 years. The neurological examination revealed that she had segmental sensorimotor disturbance below the levels of the 5th lumbar segment. MRI demonstrated tethered cord with a lumbosacral lipoma. Adult onset tethered cord syndrome (TCS) that presents with HSN 1-like symptoms as initial clinical features has not yet been reported. Foot ulcers are often seen in child onset TCS in which the degree of tethered cord is severer than adult onset cases. It is reported that release of the tethered cord promotes healing of the foot ulcers. We recommend MRI for the study of the lumbosacral cord of patients with HSN 1-like symptoms, because there is a possibility that such patients may have TCS and early surgical treatment is effective for TCS.     

One-step fluorescent probe product-enhanced reverse transcriptase assay

The Chiari type II malformation is the leading cause of death in infants with myelomeningocele. The authors report 17 cases of symptomatic Chiari type II malformation occurring in two distinct age dependent population. In Group I, 13 neonates and infants in the first year of life presented with cranial nerve and brain stem dysfunction characterized by vocal cord paralysis, apnea, dysphagia and laryngeal stridor. In Group II, 4 patients developed signs and symptoms after the first year of life. In this group, the presentation was more insidious and included neck pain and cerebellar manifestations. The surgical treatment consisted initially in shunt implantation or revision and when there was no improvement, posterior fossa decompression was performed. The response to the surgical treatment differed considerably between the two groups: older patients improved promptly after surgery and there was no casualties; in newborn and infants, especially those under 6 months of age, the mortality rate was 46.1%. The authors stress that prompt diagnosis and surgical intervention should be performed in order to produce a favorable outcome.     

Hyperhidrosis as the presenting symptom in post-traumatic syringomyelia

Post-traumatic syringomyelia is now a well known entity and occurs months or years after a spinal cord injury. The presenting symptoms are usually pain, progressive motor weakness, sensory changes, and increased spasticity. Profuse sweating or hyperhidrosis can be a symptom of the post-traumatic syrinx or can occur in autonomic dysreflexia provoked by peripheral stimuli. We present two patients with cervical spine fractures whose presenting symptom of post-traumatic syringomyelia was hyperhidrosis affected by posture. The pathophysiology involved and the management of these patients is discussed.     

Acute effects of nasal continuous positive airway pressure on 24-hour blood pressure and catecholamines in patients with obstructive sleep apnea

OBJECTIVE: To estimate the risk for spina bifida associated with the common mutation C677T of the MTHFR gene in a country with a relatively low prevalence of NTDs. DESIGN: Case-control study. SUBJECTS: Cases: 203 living patients affected with spina bifida (173 myelomeningocele and 30 lipomeningocele); controls: 583 subjects (306 young adults and 277 unselected newborns) from northern and central-southern Italy. SETTING: Cases: three spina bifida centres; young adult controls: DNA banks; newborn controls: regional neonatal screening centres. MAIN OUTCOME MEASURES: Prevalence of the C677T genotypes in cases and controls by place of birth; odds ratios for spina bifida and estimated attributable fraction. RESULTS: The prevalence of T/T, T/C, and C/C genotype was 16.6%, 53.7%, and 29.7% in controls and 25.6%, 43.8%, and 30.6% in cases, respectively. We found no differences between type of defect or place of birth. The odds ratio for spina bifida associated with the T/T genotype v C/C plus T/C was 1.73 (95% CI 1.15, 2.59) and the corresponding attributable fraction was 10.8%. No increased risk was found for heterozygous patients (OR=0.79, 95% CI 0.53-1.18). CONCLUSION: This study, as well as the meta-analysis we updated, shows that homozygosity for the MTHFR C677T mutation is a moderate risk factor in Europe, and even in Italy where there is a relatively low prevalence of spina bifida. The estimated attributable fraction associated with this risk factor explains only a small proportion of cases preventable by periconceptional folic acid supplementation. Thus, other genes involved in folate-homocysteine metabolism, their interaction, and the interaction between genetic and environmental factors should be investigated further.     

Congenital tethered spinal cord syndrome in adults

OBJECT: The management of tethered spinal cord syndrome with onset of symptomatology occurring in adulthood remains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. To ascertain the results of surgery in adult patients with this anomaly, the authors undertook a retrospective review of 34 cases. METHODS: The authors studied the hospital records of 34 consecutive patients who presented in adulthood with tethered cord syndrome and conducted follow-up phone interviews with 28 of them. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The most common presenting feature was pain, followed by weakness and incontinence. All patients underwent surgery. The most common operative findings were tight filum terminale, split cord malformation, and lipomyelomeningocele, paralleling those observed in pediatric studies. Long-term surgical results and patient outcome ratings were encouraging. After a mean clinical follow-up period of 4 years, significant improvement occurred in 22 of 27 patients presenting with pain, 13 of 27 patients with motor or sensory dysfunction, and 11 of 18 patients with bowel and bladder disturbance. In addition, telephone interviews were obtained after a period of 8.6 years. Twenty-two (79%) of 28 patients called the operation a long-term success; 21 (75%) of 28 patients believed that they had significant postoperative improvement (and not just stabilization) in pain and/or neurological function. Surgical complications were generally minor. Nineteen (86%) of 22 employed patients returned to work after surgery. Two (33%) of six patients who were not employed before surgery worked full time postoperatively. Only two of the 28 patients interviewed had received Workers' Compensation benefits; both of these had good outcomes and returned to work. CONCLUSIONS: Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Because neurological deficits are generally irreversible, early surgery is recommended.     

Unusual malformations in occult spinal dysraphism

Two cases of occult spinal dysraphism with different clinical symptoms, signs and congenital pathologies are presented. One had malformations including scoliosis, dermoid tumor, hydromyelia, diastematomyelia, dermal sinus, low conus, vertebrae anomalies and dextrocardia. The occurrence of dextrocardia in association with occult spinal dysraphism was found to be extremely unusual. The second case is presented in relation to the rarity of teratoma with dermal sinus and tethered cord in the lumbar area. Myelography, computed tomography, (CT), Myelo CT and magnetic resonance were used in making a diagnosis.     

Posterior fossa decompression without duraplasty in infants and young children for treatment of Chiari malformation and achondroplasia

Some children with Chiari malformation and achondroplasia require posterior fossa decompression that typically includes expansion of the dural tube with duraplasty. Infants and young children, however, may have a more distensible dura mater than do older patients. Furthermore, the structures that compress the hindbrain of young patients may be the bone and abnormally thickened atlantooccipital membrane, i.e., dural band, rather than the dura mater. We have treated 7 children who had Chiari malformation or achondroplasia with posterior fossa decompression without duraplasty. All children were symptomatic; 3 had Chiari-I malformations, 2 Chiari-II malformations, and 2 achondroplasia. The age range was 3 months to 2.5 years (mean 15.1 months). The exent of tonsillar herniation and other hindbrain anomalies was assessed on preoperative magnetic resonance imaging. The infants with Chiari-II malformations underwent cervical laminectomies, whereas the other young children with Chiari-I malformations or achondroplasia underwent suboccipital craniectomy as well as cervical laminectomy. In Chiari malformation, the dural band was divided; in achondroplasia, there was no identifiable dural band. Following bony decompression and division of the identifiable dural band, immediate expansion of the stenotic region with visible cerebrospinal fluid space posterior to the neural elements could be ascertained by intraoperative ultrasonography. During a follow-up period ranging from 4.5 months to 4 years (mean 22 months), all patients made improvements in their symptoms, 3 having complete resolution of their symptoms. This preliminary experience indicates that in children 2 years of age or younger, posterior fossa bony decompression without duraplasty can be effective treatment for Chiari malformations or achondroplasia.     

Magnetic resonance detection of myelodysplasia in children with Currarino triad

PURPOSE: To evaluate the role of MRI in the detection of myelodysplasia in children with Currarino triad. MATERIALS AND METHODS: Six patients (two girls, four boys, aged 7 months-14 years, mean age 6 years) were studied with MRI, voiding cystourethrogram and barium enema or fistulography. CT and ultrasonography were also performed in two patients. RESULTS: All patients presented with partial agenesis of the sacrum. Three patients suffered from an intermediate form of anorectal malformation (ARM) and three had a high form of ARM. The presacral masses consistent with Currarino triad included anterior meningocoele in three patients, lipoma in two patients and anterior lipomeningocoele in one patient. MRI diagnosed tethering of the spinal cord in four of six patients. The tethering of the spinal cord was due to a lipomeningocoele in one patient, an intradural lipoma in one patient and a lipoma of the filum in two patients. CONCLUSION: The association of Currarino triad with tethered spinal cord seems more common than generally reported in the literature. Preoperative MRI of the lumbosacral spine is essential to detect significant myelodysplasia in all patients with Currarino triad.     

Hydromyelia associated with spinal lipoma

We studied morphological changes of hydromyelia complicated with spinal lipoma of infants who were untethered. Since the MRI was introduced, early detection of spinal lipoma with lumbosacral skin abnormalities has become possible. We have experienced 44 surgical cases of spinal lipoma. Out of 36 such cases, 25 (69.4%) had hydromyelia, and hydromyelia of 9 patients was found to be of the terminal ventricle type. As many as 36% of spinal lipoma patients with hydromyelia concurrently had terminal-type hydromyelia, which was considered to fall under a specific category of congenital hydromyelia. At the stage of embryogenesis of the spinal cord, the caudal cell mass undergoes vacuolization, canalization and retrogressive differentiation, and during this process, the terminal ventricle with ependymallined cells becomes morbid. Though the morphological changes of hydromyelia after surgery as untethering were varied, hydromyelia of 9 patients became smaller after untethering, and hydromyelia of 8 expanded but attenuated afterwards, totaling 17 (68.0%). However, 5 had an expanding tendency and 3 did not show any morphological changes during the follow-up period (20.6-26.9 months) by MRI.     

Peripheral neurological involvement as the first manifestation of spina bifida occulta

A 42-year-old male was admitted for right-sided sciatica with asymptomatic septic arthritis of the fifth toe of the right foot. He had a history of active chronic hepatitis C and septic arthritis of the fifth toe of the left foot. His symptoms included low back pain, poorly systematized right-sided sciatica, impairment of all forms of sensation in both lower limbs, absent ankle jerks, episodes of urinary retention, urgency, and painless septic arthritis of the fifth toe of the right foot. Roentgenograms showed a spina bifida occulta of L5 and a bony erosin in the distal interphalangeal joint of the right fifth toe. Distal denervation in the territory of L5 was demonstrated by the electromyographic study. Magnetic resonance imaging disclosed an area of high signal on T1 and T2 images, located within the spinal canal opposite L4 and suggestive of an intraspinal lipoma, as well as tethering of the spinal cord in an abnormally distal position. Antimicrobial therapy was effective in ensuring resolution of the infectious arthritis. The low back pain and sciatica responded to nonsteroidal antiinflammatory drug therapy and did not recur subsequently. Many patients who have roentgenograms taken to evaluate low back pain and sciatica are found to have a spina bifida occulta. This complex birth defect involving the spinal canal, meninges and spinal cord or cauda equina can cause neurologic and/or urinary symptoms in adulthood. Magnetic resonance imaging is essential in this situation to evaluate the spinal cord and to look for an intraspinal lipoma.     

Granulocytic sarcoma as isolated extramedullary relapse after donor lymphocyte infusion in a patient with CML who relapsed after allogeneic bone marrow transplantation: a case report

Nineteen patients with spina bifida and bilateral dislocation of the hips were studied with a minimal follow-up of 10 years. The average age at review was 21 years (range, 10-31). Ten patients had an upper neurologic level (thoracic to L3), and nine had a low lesion (L4 to sacrum). Three patients had no hip surgery. A closed or open reduction was performed in 12 hips, supplemented by one or more surgical procedures. Of these, 10 remained enlocated, and two had redislocated. In all other hips, several surgical procedures were performed, aimed at improvement of hip-flexion deformity or stability. At follow-up, one patient had occasional pain in one hip, which was dysplastic. Of the 10 patients with a high lesion, only two were walkers, but both had bilateral dislocation of the hips at follow-up. In contrast, all nine patients were walkers, but four of these had bilateral dislocation at follow-up. We found that a level pelvis and good range of motion of the hips are more important for ambulation than is reduction of bilateral hip dislocation.     

The impact of family, peer, and school contexts on depressive symptoms in adolescents with spina bifida.

Objective: Based on social ecological theory, this study examined the joint relations among adolescents' family, peer, and school contexts and depressive symptoms in youth with spina bifida using cumulative, protective, and specific effects models. Method: Sixty families of adolescents with spina bifida and 65 comparison families reported on adolescents' positive experiences within these contexts and on depressive symptoms when youth were 14–15 and 16–17 years old. Results: Adolescents with spina bifida had fewer total positive contexts and less positive experience within peer and school contexts, as compared to typically developing adolescents. Greater total number of positive contexts and higher levels of positive experiences within family and school contexts were associated with fewer depressive symptoms for both groups; peer positive experiences were related to lower depressive symptoms for typically developing adolescents only. Conclusion: Adolescents with spina bifida have fewer positive contexts, which may place them at risk for higher levels of depressive symptoms. (PsycINFO Database Record (c) 2010 APA, all rights reserved)