Vinck A, Mullaart R, Rotteveel J, Maassen B. (2009) Neuropsychological assessment of attention in children with spina bifida. Cerebrospinal Fluid Research. 2009 May 28;6:6.
Department of Medical Psychology, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, the Netherlands. a.vinck@mps.umcn.nl.
ABSTRACT: BACKGROUND: Children with the severe form of spina bifida (SBM: spina bifida with myelomeningocele with accompanying hydrocephalus) may manifest attention deficits, and have a similar psychological profile to children with hydrocephalus due to other etiologies. It is unclear to what extent tests to assess attention in SBM are confounded by the accompanying cognitive or visual-motor impairments. The aim of this study was to analyse attention functions by administering two different types of attention tests, one with high and the other with low cognitive and motor requirements. This enabled the possible interaction between attention and cognitive and motor impairment to be assessed.
METHODS: The study group comprised 31 children with SBM with shunted hydrocephalus. Twenty children with SB-only formed a closely matched comparison group. Of these, 19 children with SBM and 18 with SB had a full-scale IQ (FSIQ) higher than 70. All had undergone spinal surgery and all children with SBM had been shunted within the first months of life. Between 6 and 15 years of age, the children were assessed on focused and sustained attention, encoding, and distractibility/impulsivity, using both traditional tests and computerized attention tests.
RESULTS: Compared to the SB group, attention scores of children with SBM were lower on the traditional tests, but when interfering cognitive and visual-motor requirements were eliminated using the computerised tasks, most differences disappeared. Furthermore, in contrast to traditional attention tasks, computerized tests showed no significant correlations with IQ-scores and visual-motor skills.
CONCLUSION: Assessment of attention functions in children with SBM by traditional tests may be misleading, because this paediatric population with complex cerebral malformations has difficulty with the cognitive and visual-motor requirements. To control for these interactions, the use of both traditional and computerized attention tests is recommended.
PMID: 19476646
Showing posts with label Myelomeningocele. Show all posts
Showing posts with label Myelomeningocele. Show all posts
Friday, July 3, 2009
Sunday, April 27, 2008
A unifying hypothesis of hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida
Williams H. A unifying hypothesis of hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida. Cerebrospinal Fluid Research. 2008 Apr 11;5(1):7
ABSTRACT: This review is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS) venous drainage. A new hypothesis regarding the pathogenesis, anencephaly and spina bifida is described. Any volume increase in the central nervous system can increase venous pressure. This occurs because veins are compressible and a CNS volume increase may result in reduced venous blood flow. This has the potential to cause progressive increase in cerebrospinal fluid (CSF) volume. Venous insufficiency may be caused by any disease that reduces space for venous volume. The flow of CSF has a beneficial effect on venous drainage. In health it moderates central nervous system pressure by moving between the head and spine. Conversely, obstruction to CSF flow causes localised pressure increases, which have an adverse effect on venous drainage. The Chiari malformation is associated with hindbrain herniation, which may be caused by low spinal pressure relative to cranial pressure. In these instances, there are hindbrain-related symptoms caused by cerebellar and brainstem compression. When spinal injury occurs as a result of a Chiari malformation, the primary pathology is posterior fossa hypoplasia, resulting in raised spinal pressure. The small posterior fossa prevents the flow of CSF from the spine to the head, as blood enters the central nervous system during movement. Consequently, intermittent increases in spinal pressure caused by movement, result in injury to the spinal cord. It is proposed that posterior fossa hypoplasia, which has origins in fetal life causes syringomyelia after birth and leads to damage to the spinal cord in spina bifida. It is proposed that hydrocephalus may occur as a result of posterior fossa hypoplasia, where raised pressure occurs as a result of obstruction to flow of CSF from the head to the spine, and cerebral injury with raised pressure occurs in anencephaly by this mechanism. The current view of dysraphism is that low central nervous system pressure and exposure to amniotic fluid, damage the central nervous system. The hypothesis proposed in this essay supports the view that spina bifida is a manifestation of progressive hydrocephalus in the fetus. It is proposed that mesodermal growth insufficiency influences both neural tube closure and central nervous system pressure, leading to dysraphism.
PMID: 18405364
ABSTRACT: This review is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS) venous drainage. A new hypothesis regarding the pathogenesis, anencephaly and spina bifida is described. Any volume increase in the central nervous system can increase venous pressure. This occurs because veins are compressible and a CNS volume increase may result in reduced venous blood flow. This has the potential to cause progressive increase in cerebrospinal fluid (CSF) volume. Venous insufficiency may be caused by any disease that reduces space for venous volume. The flow of CSF has a beneficial effect on venous drainage. In health it moderates central nervous system pressure by moving between the head and spine. Conversely, obstruction to CSF flow causes localised pressure increases, which have an adverse effect on venous drainage. The Chiari malformation is associated with hindbrain herniation, which may be caused by low spinal pressure relative to cranial pressure. In these instances, there are hindbrain-related symptoms caused by cerebellar and brainstem compression. When spinal injury occurs as a result of a Chiari malformation, the primary pathology is posterior fossa hypoplasia, resulting in raised spinal pressure. The small posterior fossa prevents the flow of CSF from the spine to the head, as blood enters the central nervous system during movement. Consequently, intermittent increases in spinal pressure caused by movement, result in injury to the spinal cord. It is proposed that posterior fossa hypoplasia, which has origins in fetal life causes syringomyelia after birth and leads to damage to the spinal cord in spina bifida. It is proposed that hydrocephalus may occur as a result of posterior fossa hypoplasia, where raised pressure occurs as a result of obstruction to flow of CSF from the head to the spine, and cerebral injury with raised pressure occurs in anencephaly by this mechanism. The current view of dysraphism is that low central nervous system pressure and exposure to amniotic fluid, damage the central nervous system. The hypothesis proposed in this essay supports the view that spina bifida is a manifestation of progressive hydrocephalus in the fetus. It is proposed that mesodermal growth insufficiency influences both neural tube closure and central nervous system pressure, leading to dysraphism.
PMID: 18405364
Wednesday, January 30, 2008
Assessment of brainstem function in Chiari II malformation utilizing brainstem auditory evoked potentials (BAEP), blink reflex and masseter reflex.
Koehler J, Schwarz M, Boor R, Hölker C, Hopf HC, Voth D, Urban PP, Ermert A. Assessment of brainstem function in Chiari II malformation utilizing brainstem auditory evoked potentials (BAEP), blink reflex and masseter reflex. Brain Development. 2000 Oct;22(7):417-20.
Brainstem dysfunction was evaluated in 67 patients with myelomeningocele and Chiari II malformation using brainstem auditory evoked potentials (BAEP), blink reflex (BR) and masseter reflex (MR). Signs and symptoms related to Chiari II malformation were observed in 18 patients while 49 patients had normal brainstem findings. BAEP and BR showed a higher sensitivity of brainstem involvement than MR (BAEP=1.0, BR=0.83, MR=0.50). BR, and in particular, MR were of higher accuracy (BR=0.52, MR=0.72) than BAEP (0.39) in separating patients with brainstem signs and symptoms related to Chiari II malformation. We feel that this is due to anatomic and physiologic peculiarities of the brainstem structures mediating BR and MR. Our results suggest that brainstem reflexes can support the decision of further treatment.
PMID: 11102725
Full Text
Brainstem dysfunction was evaluated in 67 patients with myelomeningocele and Chiari II malformation using brainstem auditory evoked potentials (BAEP), blink reflex (BR) and masseter reflex (MR). Signs and symptoms related to Chiari II malformation were observed in 18 patients while 49 patients had normal brainstem findings. BAEP and BR showed a higher sensitivity of brainstem involvement than MR (BAEP=1.0, BR=0.83, MR=0.50). BR, and in particular, MR were of higher accuracy (BR=0.52, MR=0.72) than BAEP (0.39) in separating patients with brainstem signs and symptoms related to Chiari II malformation. We feel that this is due to anatomic and physiologic peculiarities of the brainstem structures mediating BR and MR. Our results suggest that brainstem reflexes can support the decision of further treatment.
PMID: 11102725
Full Text
Electrophysiological studies on brainstem function in patients with myelomeningocele
Mori K, Nishimura T. Electrophysiological studies on brainstem function in patients with myelomeningocele. Pediatric Neurosurgery. 1995;22(3):120-31.
We investigated the brainstem auditory evoked potentials (BAEPs), somatosensory evoked potentials (SEPs), and electrically elicited blink reflexes (BRs) to evaluate the brainstem function in 31 patients with meyelomeningocele (MMC) including 22 with Chiari type-II malformation. The I-III interpeak latency (IPL) of the BAEPs and the N9-N13 IPL of the SEPs tended to become gradually prolonged from the normal range with increasing age. The III-V IPL of the BAEPs and the N13-N20 IPL of the SEPs were initially prolonged and decreased progressively to the normal range. These findings indicated a gradual latency shortening of the brainstem components and latency prolongation of the peripheral components. Thus, while primary brainstem dysfunction may improve with age, secondary dysfunction due to stretching and elongation of the lower cranial nerves and cervical nerve roots may intensify. The BRs showed an abnormal R2 in 90% of the cases, disclosing subclinical lesions in the medulla oblongata which were not detected by BAEPs alone. BAEPs, SEPs and BRs were combined to yield a functional evaluation of the brainstem and lower cranial nerves that could not be done by magnetic resonance imaging alone. No close relation was found between electrophysiological abnormalities and the degree of hindbrain anomaly by neuroimaging.
PMID: 7786804
We investigated the brainstem auditory evoked potentials (BAEPs), somatosensory evoked potentials (SEPs), and electrically elicited blink reflexes (BRs) to evaluate the brainstem function in 31 patients with meyelomeningocele (MMC) including 22 with Chiari type-II malformation. The I-III interpeak latency (IPL) of the BAEPs and the N9-N13 IPL of the SEPs tended to become gradually prolonged from the normal range with increasing age. The III-V IPL of the BAEPs and the N13-N20 IPL of the SEPs were initially prolonged and decreased progressively to the normal range. These findings indicated a gradual latency shortening of the brainstem components and latency prolongation of the peripheral components. Thus, while primary brainstem dysfunction may improve with age, secondary dysfunction due to stretching and elongation of the lower cranial nerves and cervical nerve roots may intensify. The BRs showed an abnormal R2 in 90% of the cases, disclosing subclinical lesions in the medulla oblongata which were not detected by BAEPs alone. BAEPs, SEPs and BRs were combined to yield a functional evaluation of the brainstem and lower cranial nerves that could not be done by magnetic resonance imaging alone. No close relation was found between electrophysiological abnormalities and the degree of hindbrain anomaly by neuroimaging.
PMID: 7786804
Somatosensory evoked potentials to median nerve stimulation in meningomyelocele
Nishimura T, Mori K. Somatosensory evoked potentials to median nerve stimulation in meningomyelocele: what is occurring in the hindbrain and its connections during growth? Childs Nervous System. 1996 Jan;12(1):13-26. Review.
Twenty patients with meningomyelocele (MMC) and shunted hydrocephalus, ranging in age from 3 to 23 years old, underwent serial recording of short-latency somatosensory evoked potentials (SEPs) to median nerve stimulation, on the basis of which to assess the evolution of dysfunction in the brainstem and its connections (cervical spinal cord, cervical nerve roots, lower cranial nerves). Eighteen patients had Chiari type II malformations, none of which was symptomatic. In all 20 patients, serial SEP recordings were obtained repeatedly after periods ranging from 2 to 7 years. The results were as follows: (1) the ratio of EP-N13 interpeak latencies (IPLs) to body height (BH) tended to lengthen gradually after starting in the normal range with growth. (2) In contrast, the ratio N13-N20 IPLs/BH decreased continuously to the normal range with growth, after initial prolongation. These findings and our earlier studies of brainstem auditory evoked potentials suggest that compression and traction result in strangulation of the cervical spinal cord and kinked medulla as well as stretching of the cervical nerve roots and lower cranial nerves. However, primary intrinsic functional disorders of the brainstem appeared to improve gradually during growth. The natural history of Chiari type II malformations involves these opposing dynamic phenomena in the hindbrain and its connections.
PMID: 8869170
Twenty patients with meningomyelocele (MMC) and shunted hydrocephalus, ranging in age from 3 to 23 years old, underwent serial recording of short-latency somatosensory evoked potentials (SEPs) to median nerve stimulation, on the basis of which to assess the evolution of dysfunction in the brainstem and its connections (cervical spinal cord, cervical nerve roots, lower cranial nerves). Eighteen patients had Chiari type II malformations, none of which was symptomatic. In all 20 patients, serial SEP recordings were obtained repeatedly after periods ranging from 2 to 7 years. The results were as follows: (1) the ratio of EP-N13 interpeak latencies (IPLs) to body height (BH) tended to lengthen gradually after starting in the normal range with growth. (2) In contrast, the ratio N13-N20 IPLs/BH decreased continuously to the normal range with growth, after initial prolongation. These findings and our earlier studies of brainstem auditory evoked potentials suggest that compression and traction result in strangulation of the cervical spinal cord and kinked medulla as well as stretching of the cervical nerve roots and lower cranial nerves. However, primary intrinsic functional disorders of the brainstem appeared to improve gradually during growth. The natural history of Chiari type II malformations involves these opposing dynamic phenomena in the hindbrain and its connections.
PMID: 8869170
Anatomical progression of the Chiari II malformation.
Ruge JR, Masciopinto J, Storrs BB, McLone DG. Anatomical progression of the Chiari II malformation. Childs Nervous System. 1992 Mar;8(2):86-91.
To evaluate whether anatomic change of the relationship of the Chiari II malformation and the cranial base was occurring, 22 children with meningomyelocele had serial MRI scans reviewed. A ratio (B/A) was established between the distance from the foramen magnum to the caudalmost portion of herniated cerebellum (B) and the diameter of the foramen magnum (A) and this ratio was compared on serial MRI scans. Eighteen children had an increase in the B/A ratio, two children had a decrease, and two had no change. This indicates that continuous anatomic change of the Chiari II malformation and the skull base is occurring. Clinical deterioration in the older child may be explained by a combination of compressive and traction forces due to this change.
PMID: 1591752
To evaluate whether anatomic change of the relationship of the Chiari II malformation and the cranial base was occurring, 22 children with meningomyelocele had serial MRI scans reviewed. A ratio (B/A) was established between the distance from the foramen magnum to the caudalmost portion of herniated cerebellum (B) and the diameter of the foramen magnum (A) and this ratio was compared on serial MRI scans. Eighteen children had an increase in the B/A ratio, two children had a decrease, and two had no change. This indicates that continuous anatomic change of the Chiari II malformation and the skull base is occurring. Clinical deterioration in the older child may be explained by a combination of compressive and traction forces due to this change.
PMID: 1591752
Monday, October 29, 2007
Seven distinct coexistent cranial and spinal anomalies
Emmez H, Tokgoz N, Dogulu F, Yilmaz MB, Kale A, Baykaner MK. Seven distinct coexistent cranial and spinal anomalies. Pediatric Neurosurgery. 2006;42(5):316-9.
Existence of multiple cranial and spinal anomalies in spina bifida is well known; however, coexistence of seven different severe anomalies is extremely rare. The location of the anomalies, the patient's age and presentation are other interesting aspects of the presented case.
CASE REPORT: A 1-year-old girl with an enlarged head and big, infected lumbosacral myelomeningocele (MMC) was admitted to the emergency department in a comatose state. Further investigations revealed hydrocephalus, Chiari malformation, syringohydromyelia, split cord malformation, dermal sinus tract, lumbosacral MMC, and tethered cord. Unfortunately, the patient died 3 days after admission because of meningitis and sepsis.
DISCUSSION: Coexistence of seven different craniospinal anomalies is extremely unusual. Further investigations with magnetic resonance imaging for associated anomalies, early shunt placement and MMC repair are required to prevent this life-threatening condition in spina bifida.
PMID: 16902346
Existence of multiple cranial and spinal anomalies in spina bifida is well known; however, coexistence of seven different severe anomalies is extremely rare. The location of the anomalies, the patient's age and presentation are other interesting aspects of the presented case.
CASE REPORT: A 1-year-old girl with an enlarged head and big, infected lumbosacral myelomeningocele (MMC) was admitted to the emergency department in a comatose state. Further investigations revealed hydrocephalus, Chiari malformation, syringohydromyelia, split cord malformation, dermal sinus tract, lumbosacral MMC, and tethered cord. Unfortunately, the patient died 3 days after admission because of meningitis and sepsis.
DISCUSSION: Coexistence of seven different craniospinal anomalies is extremely unusual. Further investigations with magnetic resonance imaging for associated anomalies, early shunt placement and MMC repair are required to prevent this life-threatening condition in spina bifida.
PMID: 16902346
Friday, October 12, 2007
Cervical meningomyelocele-an institutional experience
Kasliwal MK, Dwarakanath S, Mahapatra AK. Cervical meningomyelocele-an institutional experience. Child's Nervous System. 2007 Nov;23(11):1291-3. Epub 2007 Jul 13.
OBJECTIVE: Cervical myelomeningoceles (CMMC) are a less common but distinct subgroup of myelomeningoceles. Their embryology and clinical characteristics vary from the more common thoracolumbar variant. Only a few small series have been published addressing this lesion in the literature with the largest one of them addressing nine patients. The authors present one of the largest series of cervical myelomeningoceles, review their embryology, clinical features, and their management strategies.
METHODS: This study included all the children who were managed for cervical myelomeningocele between Jan 2001 to July 2006 at our center.
RESULTS: There were a total of ten children (five boys and five girls) operated on for cervical myelomeningoceles. The ages ranged between 2 months to 14 months. Neurological examination was normal in majority of the children with absence of gross orthopedic deformity in all the children. Three patients had associated hydrocephalus, two had Chiari malformation, and four of them had a syrinx. Surgical excision of the sac was performed for all.
CONCLUSION: Cystic dysraphisms of the cervical differ embryologically, clinically, and structurally from thoracolumbar meningomyelocele and have a more favorable outcome. A good pre operative evaluation is recommended to assess any associated anomalies and identify the internal structures. Surgery excision of these lesions with intradural exploration of the sac to release any potential adhesion bands as well as other associated anomalies is recommended.
PMID: 17628809
OBJECTIVE: Cervical myelomeningoceles (CMMC) are a less common but distinct subgroup of myelomeningoceles. Their embryology and clinical characteristics vary from the more common thoracolumbar variant. Only a few small series have been published addressing this lesion in the literature with the largest one of them addressing nine patients. The authors present one of the largest series of cervical myelomeningoceles, review their embryology, clinical features, and their management strategies.
METHODS: This study included all the children who were managed for cervical myelomeningocele between Jan 2001 to July 2006 at our center.
RESULTS: There were a total of ten children (five boys and five girls) operated on for cervical myelomeningoceles. The ages ranged between 2 months to 14 months. Neurological examination was normal in majority of the children with absence of gross orthopedic deformity in all the children. Three patients had associated hydrocephalus, two had Chiari malformation, and four of them had a syrinx. Surgical excision of the sac was performed for all.
CONCLUSION: Cystic dysraphisms of the cervical differ embryologically, clinically, and structurally from thoracolumbar meningomyelocele and have a more favorable outcome. A good pre operative evaluation is recommended to assess any associated anomalies and identify the internal structures. Surgery excision of these lesions with intradural exploration of the sac to release any potential adhesion bands as well as other associated anomalies is recommended.
PMID: 17628809
Tuesday, July 31, 2007
Interhemispheric transfer of visual, auditory, tactile, and visuomotor information in children with hydrocephalus and partial agenesis of the corpus c
Klaas PA, Hannay JH, Caroselli JS, Fletcher JM. Interhemispheric transfer of visual, auditory, tactile, and visuomotor information in children with hydrocephalus and partial agenesis of the corpus callosum. Journal of Clinical and Experimental Neuropsychology. 1999 Dec;21(6):837-50.
Interhemispheric transfer was examined in 13 normal controls and in 13 children with hydrocephalus and partial agenesis of the corpus callosum involving a missing splenium associated with spina bifida meningomyelocele and aqueductal stenosis. Children with hydrocephalus demonstrated greater left visual-field superiority for forms indicative of difficulty in interhemispheric transfer of visual information. Interhemispheric transfer time (ITT) was somewhat longer in the children with hydrocephalus, but neither the ITT task nor the dichotic listening and tactile naming tasks provided clear evidence of difficulties in callosal transfer. Sensory and perceptual control tasks revealed similar visual acuity and monotic word perception but larger 2-point discrimination thresholds and poorer tactile matching.
PMID: 10649538
Interhemispheric transfer was examined in 13 normal controls and in 13 children with hydrocephalus and partial agenesis of the corpus callosum involving a missing splenium associated with spina bifida meningomyelocele and aqueductal stenosis. Children with hydrocephalus demonstrated greater left visual-field superiority for forms indicative of difficulty in interhemispheric transfer of visual information. Interhemispheric transfer time (ITT) was somewhat longer in the children with hydrocephalus, but neither the ITT task nor the dichotic listening and tactile naming tasks provided clear evidence of difficulties in callosal transfer. Sensory and perceptual control tasks revealed similar visual acuity and monotic word perception but larger 2-point discrimination thresholds and poorer tactile matching.
PMID: 10649538
Functioning of the corpus callosum in children with early hydrocephalus
Hannay HJ. Functioning of the corpus callosum in children with early hydrocephalus. Journal of the International Neuropsychological Society. 2000 Mar;6(3):351-61. Review.
The development and organization of the corpus callosum is described as well as the relationship between the timing of insults and the type of partial agenesis of the corpus callosum are discussed. Neuropathology and callosal damage associated with spina bifida meningomyelocele, aqueductal stenosis, and prematurity-IVH are outlined. Relationships between corpus callosum/whole brain ratios and cognitive functioning as well as interhemispheric transfer in children with these disorders are outlined. Shortcomings of current research and future directions are suggested.
PMID: 10824507
The development and organization of the corpus callosum is described as well as the relationship between the timing of insults and the type of partial agenesis of the corpus callosum are discussed. Neuropathology and callosal damage associated with spina bifida meningomyelocele, aqueductal stenosis, and prematurity-IVH are outlined. Relationships between corpus callosum/whole brain ratios and cognitive functioning as well as interhemispheric transfer in children with these disorders are outlined. Shortcomings of current research and future directions are suggested.
PMID: 10824507
Thursday, July 26, 2007
Hand function in subjects with spina bifida
Muen WJ, Bannister CM. Hand function in subjects with spina bifida.
European Journal of Pediatric Surgery. 1997 Dec;7 Suppl 1:18-22.
The aim of our study was to assess the influence of the Arnold-Chiari malformation, cervical cord dysraphism and hydrocephalus on hand function in subjects with meningomyelocele, but without many of the other confounding factors commonly found in this group e.g low IQ and cerebral palsy, which in themselves cause poor hand function. Three groups of subjects who attended main stream schools underwent a basic neurological examination of the upper limbs and a battery of hand function tests. Group 1 consisted of subjects with meningomyelocele and shunted hydrocephalus (n = 21), Group 2 subjects had isolated shunted hydrocephalus (n = 13) and Group 3 were normal controls (n = 18). The results show that subjects with meningomyelocele had weaker power in the small muscles of the hand as compared to the controls and those with isolated hydrocephalus (p = 0.01) and poorer fine motor control and co-ordination. The meningomyelocele group scored significantly poorer in all of the tests as compared to the control group using the dominant hand (overall, p = 0.0003), and all of the tests but one using the non-dominant hand (overall, p = 0.0005). Our study has demonstrated that children with meningomyelocele have poorer hand function than healthy controls, which is due to causes other than hydrocephalus, poor IQ or cerebral hemisphere pathology. Instead, cerebellar and cervical cord abnormalities appear to be exerting a significant deleterious influence on the function of the upper limbs.
PMID: 9497111
European Journal of Pediatric Surgery. 1997 Dec;7 Suppl 1:18-22.
The aim of our study was to assess the influence of the Arnold-Chiari malformation, cervical cord dysraphism and hydrocephalus on hand function in subjects with meningomyelocele, but without many of the other confounding factors commonly found in this group e.g low IQ and cerebral palsy, which in themselves cause poor hand function. Three groups of subjects who attended main stream schools underwent a basic neurological examination of the upper limbs and a battery of hand function tests. Group 1 consisted of subjects with meningomyelocele and shunted hydrocephalus (n = 21), Group 2 subjects had isolated shunted hydrocephalus (n = 13) and Group 3 were normal controls (n = 18). The results show that subjects with meningomyelocele had weaker power in the small muscles of the hand as compared to the controls and those with isolated hydrocephalus (p = 0.01) and poorer fine motor control and co-ordination. The meningomyelocele group scored significantly poorer in all of the tests as compared to the control group using the dominant hand (overall, p = 0.0003), and all of the tests but one using the non-dominant hand (overall, p = 0.0005). Our study has demonstrated that children with meningomyelocele have poorer hand function than healthy controls, which is due to causes other than hydrocephalus, poor IQ or cerebral hemisphere pathology. Instead, cerebellar and cervical cord abnormalities appear to be exerting a significant deleterious influence on the function of the upper limbs.
PMID: 9497111
Wednesday, July 25, 2007
Taxonicity of nonverbal learning disabilities in spina bifida.
Ris MD, Ammerman RT, Waller N, Walz N, Oppenheimer S, Brown TM, Enrile BG, Yeates KO. Taxonicity of nonverbal learning disabilities in spina bifida. Journal of the International Neuropsychological Society. 2007 Jan;13(1):50-8.
As currently defined, it is not clear whether Nonverbal Learning Disabilities (NLD) should be considered a matter of kind or magnitude (Meehl, 1995). The taxonicity of NLD, or the degree to which it is best construed as discrete versus continuous, has not been investigated using methods devised for this purpose. Latent Class Analysis (LCA) is a method for finding subtypes of latent classes from multivariate categorical data. This study represents an application of LCA on a sample of children and adolescents with spina bifida myelomeningocele (SBM) (N = 44), those presenting with features of NLD (N = 28) but no medical condition, and control volunteers (N = 44). The two-class solution provided evidence for the presence of a taxon with an estimated base-rate in the SBM group of .57. Indicator validities (the conditional probabilities of indicator endorsement in each latent class) suggest a somewhat different priority for defining NLD than is typically used by researchers investigating this disorder. A high degree of correspondence between LCA classifications and those based on a more conventional algorithm provided evidence for the validity of this approach.
PMID: 17166303
As currently defined, it is not clear whether Nonverbal Learning Disabilities (NLD) should be considered a matter of kind or magnitude (Meehl, 1995). The taxonicity of NLD, or the degree to which it is best construed as discrete versus continuous, has not been investigated using methods devised for this purpose. Latent Class Analysis (LCA) is a method for finding subtypes of latent classes from multivariate categorical data. This study represents an application of LCA on a sample of children and adolescents with spina bifida myelomeningocele (SBM) (N = 44), those presenting with features of NLD (N = 28) but no medical condition, and control volunteers (N = 44). The two-class solution provided evidence for the presence of a taxon with an estimated base-rate in the SBM group of .57. Indicator validities (the conditional probabilities of indicator endorsement in each latent class) suggest a somewhat different priority for defining NLD than is typically used by researchers investigating this disorder. A high degree of correspondence between LCA classifications and those based on a more conventional algorithm provided evidence for the validity of this approach.
PMID: 17166303
Tuesday, July 24, 2007
Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study
George TM, Fagan LH. Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study. Journal of Neurosurgery. 2005 Mar;102(2 Suppl):150-6. Review.
OBJECT: As patients with myelomeningocele age, tethered cord syndrome (TCS) in adults with postrepair myelomeningocele has become more common. The authors have structured an evidence-based review of the literature for the purpose of addressing the following questions. (1) How is the diagnosis made? (2) What is the natural history that occurs in adults with postrepair myelomeningocele and TCS? (3) What are the criteria for operative intervention? (4) What is the functional outcome for patients with postrepair myelomeningocele?
METHODS: A computerized search of the National Library of Medicine of the English-language literature published from 1966 to 2003 was performed. Articles pertaining to the clinical aspects and management of postrepair myelomeningocele in adults, TCS in adults resulting from closed defects, and pediatric patients with postrepair myelomeningocele were reviewed. No Level 1 or 2 data were located; however, the search yielded Level 3 and 4 evidence in the literature in which the clinical syndrome, underlying pathology, and the imaging and electrophysiological evaluation for TCS in adults are discussed.
CONCLUSIONS: Analysis of the available data indicates the following. (1) A lower lesion level predisposes patients to symptomatic tethering; moreover, orthopedic and urological deterioration will occur in the majority of these patients. (2) Tethered cord release should be considered for adult patients with postrepair myelomeningocele when clinical symptoms, imaging studies, urodynamics, and somatosensory evoked potentials are consistent with TCS. (3) Prompt, aggressive untethering surgery within 5 years of symptom onset, along with long-term follow up to check for delayed retethering, is recommended. The overall outcome for patients with postrepair myelomeningocele may not be as good as the outcome for adults with closed dysraphism.
PMID: 16156223
OBJECT: As patients with myelomeningocele age, tethered cord syndrome (TCS) in adults with postrepair myelomeningocele has become more common. The authors have structured an evidence-based review of the literature for the purpose of addressing the following questions. (1) How is the diagnosis made? (2) What is the natural history that occurs in adults with postrepair myelomeningocele and TCS? (3) What are the criteria for operative intervention? (4) What is the functional outcome for patients with postrepair myelomeningocele?
METHODS: A computerized search of the National Library of Medicine of the English-language literature published from 1966 to 2003 was performed. Articles pertaining to the clinical aspects and management of postrepair myelomeningocele in adults, TCS in adults resulting from closed defects, and pediatric patients with postrepair myelomeningocele were reviewed. No Level 1 or 2 data were located; however, the search yielded Level 3 and 4 evidence in the literature in which the clinical syndrome, underlying pathology, and the imaging and electrophysiological evaluation for TCS in adults are discussed.
CONCLUSIONS: Analysis of the available data indicates the following. (1) A lower lesion level predisposes patients to symptomatic tethering; moreover, orthopedic and urological deterioration will occur in the majority of these patients. (2) Tethered cord release should be considered for adult patients with postrepair myelomeningocele when clinical symptoms, imaging studies, urodynamics, and somatosensory evoked potentials are consistent with TCS. (3) Prompt, aggressive untethering surgery within 5 years of symptom onset, along with long-term follow up to check for delayed retethering, is recommended. The overall outcome for patients with postrepair myelomeningocele may not be as good as the outcome for adults with closed dysraphism.
PMID: 16156223
Tissue expansion for the delayed closure of large myelomeningoceles.
Mowatt DJ, Thomson DN, Dunaway DJ. Tissue expansion for the delayed closure of large myelomeningoceles. Journal of Neurosurgery. 2005 Dec;103(6 Suppl):544-8.
OBJECT: Numerous techniques for the soft-tissue closure of large myelomeningoceles have been described. When patients present for delayed repair beyond the neonatal period, the indications for surgery are both different and less immediate.
METHODS: Six patients between 6 weeks and 3.5 years of age with defects ranging from 27 to 49 cm2 (mean 37 cm2) were referred to the authors' institution and were successfully treated by direct skin closure after tissue expansion.
CONCLUSIONS: In this paper the authors describe the technique and advantages of expansion compared with those of locoregional flaps in this subgroup of patients with myelomeningoceles.
PMID: 16383254
OBJECT: Numerous techniques for the soft-tissue closure of large myelomeningoceles have been described. When patients present for delayed repair beyond the neonatal period, the indications for surgery are both different and less immediate.
METHODS: Six patients between 6 weeks and 3.5 years of age with defects ranging from 27 to 49 cm2 (mean 37 cm2) were referred to the authors' institution and were successfully treated by direct skin closure after tissue expansion.
CONCLUSIONS: In this paper the authors describe the technique and advantages of expansion compared with those of locoregional flaps in this subgroup of patients with myelomeningoceles.
PMID: 16383254
Intelligence quotient in children with meningomyeloceles: a case-control study.
Nejat F, Kazmi SS, Habibi Z, Tajik P, Shahrivar Z. Intelligence quotient in children with meningomyeloceles: a case-control study. Journal of Neurosurgery. 2007 Feb;106(2 Suppl):106-10.
OBJECT: Meningomyelocele (MMC) is a common central nervous system birth defect. As one of many problems facing patients with MMC, learning disabilities are often overlooked. The aim of this study was to evaluate IQs in a group of children with MMCs and determine if a correlation exists between intelligence level and the presence of an MMC and/or its complications.
METHODS: A case-control study was conducted at the Children's Hospital Medical Center in Tehran, Iran, from December 2004 through December 2005. The patient group included 50 children with MMC from 5 to 12 years of age who were referred to the authors' institution for treatment of complications or for follow up after surgery for MMC closure. The patient group was individually matched for age and sex with a control group of 50 children referred to the hospital for other reasons and who did not have MMC or other neurological abnormalities. The IQs in all children in this study were evaluated using the Ravens Progressive Matrices test. The children in both groups were similar in the socioeconomic status of the family (p = 0.347) and educational status of the father (p = 0.117) and mother (p = 0.439). Patient age at the time of surgery for MMC closure varied from 1 day to 96 months (mean 4.1 months). Only 20% of the patients with MMC could walk with a normal gait. Forty-six percent of the patients had undergone placement of a ventriculoperitoneal shunt, and half of these patients experienced shunt-related complications; 72% of the children in the patient group were completely incontinent for both urine and feces. The IQ results obtained in the patient group ranged from 73 to 134 with a mean (+/- standard deviation) of 96.62 +/- 13.01. In the control group the IQ range was 70 to 128, and the mean was 104.82 +/- 12.30. Compared with the control group there was a statistically significant correlation between having an MMC and having a lower IQ (p < 0.001, paired t-test).
CONCLUSIONS: Although the average IQ in the patient group was significantly lower than that in the control group, it is important to note that all children in the patient group had an average or above-average IQ. In contrast with the results reported in other studies, earlier repair of the MMC, the presence of a shunt or shunt-related complications, walking difficulty, and the spinal level of the lesion did not correlate significantly with IQs. Therefore, the lower IQ and reduced cognitive levels noted in these patients result from the disease process itself and not from the associated complications.
PMID: 17330535
OBJECT: Meningomyelocele (MMC) is a common central nervous system birth defect. As one of many problems facing patients with MMC, learning disabilities are often overlooked. The aim of this study was to evaluate IQs in a group of children with MMCs and determine if a correlation exists between intelligence level and the presence of an MMC and/or its complications.
METHODS: A case-control study was conducted at the Children's Hospital Medical Center in Tehran, Iran, from December 2004 through December 2005. The patient group included 50 children with MMC from 5 to 12 years of age who were referred to the authors' institution for treatment of complications or for follow up after surgery for MMC closure. The patient group was individually matched for age and sex with a control group of 50 children referred to the hospital for other reasons and who did not have MMC or other neurological abnormalities. The IQs in all children in this study were evaluated using the Ravens Progressive Matrices test. The children in both groups were similar in the socioeconomic status of the family (p = 0.347) and educational status of the father (p = 0.117) and mother (p = 0.439). Patient age at the time of surgery for MMC closure varied from 1 day to 96 months (mean 4.1 months). Only 20% of the patients with MMC could walk with a normal gait. Forty-six percent of the patients had undergone placement of a ventriculoperitoneal shunt, and half of these patients experienced shunt-related complications; 72% of the children in the patient group were completely incontinent for both urine and feces. The IQ results obtained in the patient group ranged from 73 to 134 with a mean (+/- standard deviation) of 96.62 +/- 13.01. In the control group the IQ range was 70 to 128, and the mean was 104.82 +/- 12.30. Compared with the control group there was a statistically significant correlation between having an MMC and having a lower IQ (p < 0.001, paired t-test).
CONCLUSIONS: Although the average IQ in the patient group was significantly lower than that in the control group, it is important to note that all children in the patient group had an average or above-average IQ. In contrast with the results reported in other studies, earlier repair of the MMC, the presence of a shunt or shunt-related complications, walking difficulty, and the spinal level of the lesion did not correlate significantly with IQs. Therefore, the lower IQ and reduced cognitive levels noted in these patients result from the disease process itself and not from the associated complications.
PMID: 17330535
Double spinal dysraphism. Report of three cases
Keshavarzi S, Nejat F, Kazemi H. Double spinal dysraphism. Report of three cases. Journal of Neurosurgery. 2007 Apr;106(4 Suppl):316-8.
The simultaneous presence of multiple spinal neural tube defects is unusual. There have been only a few of these cases reported in the literature. The authors report on three cases of double spina bifida cystica. One patient had two myelomeningoceles (MMCs) at the cervical and lumbosacral regions, one was noted to have both thoracolumbar and sacral defects, and the third presented with double MMCs at lumbar and lumbosacral levels. All three neonates in these cases underwent surgical treatment and ventriculoperitoneal (VP) shunt insertion for associated hydrocephalus. One child died at the age of 2 months despite a well-functioning VP shunt. The other two patients had no complications. Current models of neural tube closure do not thoroughly explain the mechanisms of multiple spinal dysraphism, but the multisite closure model provides a better understanding of caudal neural tube closure than other closure-site models.
PMID: 17465369
The simultaneous presence of multiple spinal neural tube defects is unusual. There have been only a few of these cases reported in the literature. The authors report on three cases of double spina bifida cystica. One patient had two myelomeningoceles (MMCs) at the cervical and lumbosacral regions, one was noted to have both thoracolumbar and sacral defects, and the third presented with double MMCs at lumbar and lumbosacral levels. All three neonates in these cases underwent surgical treatment and ventriculoperitoneal (VP) shunt insertion for associated hydrocephalus. One child died at the age of 2 months despite a well-functioning VP shunt. The other two patients had no complications. Current models of neural tube closure do not thoroughly explain the mechanisms of multiple spinal dysraphism, but the multisite closure model provides a better understanding of caudal neural tube closure than other closure-site models.
PMID: 17465369
Thursday, July 19, 2007
Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study.
George TM, Fagan LH. Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study. Journal of Neurosurgery. 2005 Mar;102(2 Suppl):150-6.
OBJECT: As patients with myelomeningocele age, tethered cord syndrome (TCS) in adults with postrepair myelomeningocele has become more common. The authors have structured an evidence-based review of the literature for the purpose of addressing the following questions. (1) How is the diagnosis made? (2) What is the natural history that occurs in adults with postrepair myelomeningocele and TCS? (3) What are the criteria for operative intervention? (4) What is the functional outcome for patients with postrepair myelomeningocele?
METHODS: A computerized search of the National Library of Medicine of the English-language literature published from 1966 to 2003 was performed. Articles pertaining to the clinical aspects and management of postrepair myelomeningocele in adults, TCS in adults resulting from closed defects, and pediatric patients with postrepair myelomeningocele were reviewed. No Level 1 or 2 data were located; however, the search yielded Level 3 and 4 evidence in the literature in which the clinical syndrome, underlying pathology, and the imaging and electrophysiological evaluation for TCS in adults are discussed.
CONCLUSIONS: Analysis of the available data indicates the following. (1) A lower lesion level predisposes patients to symptomatic tethering; moreover, orthopedic and urological deterioration will occur in the majority of these patients. (2) Tethered cord release should be considered for adult patients with postrepair myelomeningocele when clinical symptoms, imaging studies, urodynamics, and somatosensory evoked potentials are consistent with TCS. (3) Prompt, aggressive untethering surgery within 5 years of symptom onset, along with long-term follow up to check for delayed retethering, is recommended. The overall outcome for patients with postrepair myelomeningocele may not be as good as the outcome for adults with closed dysraphism.
PMID: 16156223
OBJECT: As patients with myelomeningocele age, tethered cord syndrome (TCS) in adults with postrepair myelomeningocele has become more common. The authors have structured an evidence-based review of the literature for the purpose of addressing the following questions. (1) How is the diagnosis made? (2) What is the natural history that occurs in adults with postrepair myelomeningocele and TCS? (3) What are the criteria for operative intervention? (4) What is the functional outcome for patients with postrepair myelomeningocele?
METHODS: A computerized search of the National Library of Medicine of the English-language literature published from 1966 to 2003 was performed. Articles pertaining to the clinical aspects and management of postrepair myelomeningocele in adults, TCS in adults resulting from closed defects, and pediatric patients with postrepair myelomeningocele were reviewed. No Level 1 or 2 data were located; however, the search yielded Level 3 and 4 evidence in the literature in which the clinical syndrome, underlying pathology, and the imaging and electrophysiological evaluation for TCS in adults are discussed.
CONCLUSIONS: Analysis of the available data indicates the following. (1) A lower lesion level predisposes patients to symptomatic tethering; moreover, orthopedic and urological deterioration will occur in the majority of these patients. (2) Tethered cord release should be considered for adult patients with postrepair myelomeningocele when clinical symptoms, imaging studies, urodynamics, and somatosensory evoked potentials are consistent with TCS. (3) Prompt, aggressive untethering surgery within 5 years of symptom onset, along with long-term follow up to check for delayed retethering, is recommended. The overall outcome for patients with postrepair myelomeningocele may not be as good as the outcome for adults with closed dysraphism.
PMID: 16156223
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