Brain mechanisms for reading and language processing in spina bifida meningomyelocele: a combined magnetic source- and structural magnetic resonance imaging study.

Simos PG, Papanicolaou AC, Castillo EM, Juranek J, Cirino PT, Rezaie R, Fletcher JM. (2011) Brain mechanisms for reading and language processing in spina bifida meningomyelocele: a combined magnetic source- and structural magnetic resonance imaging study. Neuropsychology. 2011 Sep;25(5):590-601.


OBJECTIVE:
The development of the ability to process spoken and written language depends upon a network of left hemisphere temporal, parietal, and frontal regions. The present study explored features of brain organization in children with spina bifida meningomyelocele (SBM) and shunted hydrocephalus, who commonly present with precocious development of word reading skills and preservation of vocabulary and grammar skills.

METHOD:
Eight children with SBM were compared with 15 IQ and reading-level matched, typically developing controls on MRI-based morphometric and Magnetic Source Imaging-derived neurophysiological profiles.

RESULTS:
Children with SBM showed reduced magnetic activity in left inferior parietal regions during spoken word recognition and pseudoword reading tasks. We also noted reduced surface area/volume in inferior parietal and posterior temporal regions in SBM and increased gray matter volumes in left middle frontal regions and gyral complexity in left posterior temporal and inferior parietal regions.

CONCLUSIONS:
A complex pattern of changes in cortical morphology and activation may serve as evidence for structural and functional brain reorganization ensuring preservation of language and decoding abilities in children with SBM.

PMID: 21574714

Posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study

Salman MS. (2011) Posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study. Childs Nerv Syst. 2011 Mar;27(3):457-62.

OBJECTIVES:
Chiari type II malformation (CII) is a congenital deformity of the hindbrain. The posterior fossa and cerebellum are small in CII. The cerebellar atrophy is associated with cognitive and motor deficits. Brainstem compression occurs in some patients with CII for whom posterior fossa decompression may be life saving. The aim was to determine whether posterior fossa decompression can prevent or reduce the cerebellar atrophy in CII.

METHODS:
Cerebellar volumes and their tissue types (gray matter, white matter, and CSF volumes) from brain MRI were compared among four CII patients, aged 9.5 to 16.5 years, who had had posterior fossa decompression in infancy, 28 CII patients who had not had posterior fossa decompression, and ten age-matched normal controls. Parametric and non-parametric tests investigated group differences.

RESULTS:
Compared to controls, mean cerebellar volume was significantly smaller in CII patients (p<0.0001). Mean CSF volume within the cerebellar fissures and fourth ventricle was significantly smaller in patients without posterior fossa decompression compared to the CII patients who had the decompression, p=0.043. Mean CSF volume of the latter group was similar to the controls. Other cerebellar volumetric measurements did not differ between the CII groups. CONCLUSIONS: Posterior fossa decompression normalizes CSF spaces within the posterior fossa in CII but does not prevent the cerebellar atrophy. The author proposes that surgical expansion of the posterior fossa should be considered in infants with CII who have a significantly small posterior fossa, to prevent or reduce the deficits associated with the cerebellar atrophy.

PMID: 21221976

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

Proof of the patent subcutaneous fibrous tract in children with V-P shunt malfunction.

Kazan S, Açikbaş C, Rahat O, Tuncer R. Proof of the patent subcutaneous fibrous tract in children with V-P shunt malfunction. Child's Nervous System. 2000 Jun;16(6):351-6.

A connective tissue sheath that forms around the peritoneal catheter of silicone ventriculo-peritoneal (V-P) shunt tubing is quite often observed in children with V-P shunts. However, proof of the passage of cerebrospinal fluid (CSF) through these sheaths has been reported in only one published study to date. We present four cases associated with chronic malfunction of the V-P shunt peritoneal catheter. In these cases, CSF passage through the subcutaneous fibrous tract, which had a pericatheter connective tissue sheath, was demonstrated around the V-P shunt peritoneal catheter. In the first case the patient suffered intermittent headache attacks over a long period of time; abdominal migration of the peritoneal catheter was detected. The second patient, who had been asymptomatic in the follow-up period with an outgrown peritoneal catheter, was admitted with acute hydrocephalus symptoms. A peritoneal catheter disconnection was detected in another patient, who had had multiple shunt revisions previously. In the last case, an obstruction of the peritoneal catheter was detected. The existence of the subcutaneous fibrous tract and its function were demonstrated by radio-opaque shuntogram in two cases and radionuclide shuntogram in the other two cases. In all four cases V-P shunt revision was performed. Within this study, the possible passage of CSF through a fibrous tract in cases of migration, outgrowth, disconnection or obstruction of the peritoneal shunt catheter was demonstrated. In conclusion, patients with shunt malfunction with a well-grown pericatheter fibrous sheath who are either asymptomatic or minimally symptomatic and show no evidence of active ventricular dilatation on their cranial CT scan should not be regarded as having arrested hydrocephalus until radio-opaque or radionuclide shuntogram studies have been done.

PMID: 10933230

Spinal lesion level in spina bifida: a source of neural and cognitive heterogeneity.

Fletcher JM, Copeland K, Frederick JA, Blaser SE, Kramer LA, Northrup H, Hannay HJ, Brandt ME, Francis DJ, Villarreal G, Drake JM, Laurent JP, Townsend I, Inwood S, Boudousquie A, Dennis M. Spinal lesion level in spina bifida: a source of neural and cognitive heterogeneity. J Neurosurg. 2005 Apr;102(3 Suppl):268-79.

OBJECT: The aim of this study was to evaluate whether the level of a spinal lesion is associated with variations in anomalous brain development and neurobehavioral outcomes in children suffering from the meningomyelocele form of spina bifida and hydrocephalus (SBM-H).

METHODS: Two hundred sixty-eight children with SBM-H were divided into upper (T-12 and above; 82 patients) and lower (L-1 and below; 186 patients) lesion-level groups. Magnetic resonance images were qualitatively coded by radiologists and quantitatively segmented for cerebrum and cerebellum volumes. Psychometric assessments of handedness, intelligence, academic skills, and adaptive behavior were compared between lesion-level groups and also used to determine the number of children who met research-based criteria for mental retardation, attention deficit hyperactivity disorder, and learning disabilities. The magnetic resonance images obtained in children with upper-level spinal lesions demonstrated more qualitative abnormalities in the midbrain and tectum, pons, and splenium, although not in the cerebellum, compared with images obtained in children with lower-level spinal lesions. Upper-level lesions were also associated with reductions in cerebrum and cerebellum volumes, lower scores on measures of intelligence, academic skills, and adaptive behavior, and with a higher frequency of individuals meeting the criteria for mental retardation. Hispanic children (who were also more economically disadvantaged) were more likely to have upper-level lesions and poorer neurobehavioral outcomes, but lesion-level effects were generally independent of ethnicity.

CONCLUSIONS: A higher level of spinal lesion in SBM-H is a marker for more severe anomalous brain development, which is in turn associated with poorer neurobehavioral outcomes in a wide variety of domains that determine levels of independent functioning for these children at home and school.

PMID: 15881750

Morphometric evaluation of the hydrocephalic brain: relationships with cognitive development.

Fletcher JM, Bohan TP, Brandt ME, Kramer LA, Brookshire BL, Thorstad K, Davidson KC, Francis DJ, McCauley SR, Baumgartner JE. Morphometric evaluation of the hydrocephalic brain: relationships with cognitive development. Childs Nervous System. 1996 Apr;12(4):192-9.

The effects of early hydrocephalus and related brain anomalies on cognitive skills are not well understood. In this study, magnetic resonance scans were obtained from 99 children aged from 6 to 13 years with either shunted hydrocephalus (n = 42) or arrested (unshunted) hydrocephalus (n = 19), from patient controls with no hydrocephalus (n = 23), and from normal, nonpatient controls (n = 15). Lateral ventricle volumes and area measurements of the internal capsules and centra semiovale in both hemispheres were obtained from these scans, along with area measurements of the corpus callosum. Results revealed reductions in the size of the corpus callosum in the shunted hydrocephalus group. In addition, lateral ventricle volumes were larger and internal capsule areas were smaller in both hemispheres in children with shunted and arrested hydrocephalus. The centra semiovale measurements did not differentiate the groups. Correlating these measurements with concurrent assessments of verbal and nonverbal cognitive skills, motor abilities, and executive functions revealed robust relationships only between the area of the corpus callosum and nonverbal cognitive skills and motor abilities. These results support the theory of a prominent role for the corpus callosum defects characteristic of many children with shunted hydrocephalus in the spatial cognition deficits commonly observed in these children.

MRI morphometric study and correlation with cognitive functions in young adults shunted for congenital hydrocephalus related to spina bifida.

Hommet C, Cottier JP, Billard C, Perrier D, Gillet P, De Toffol B, Sirinelli D, Bertrand P, Autret A. MRI morphometric study and correlation with cognitive functions in young adults shunted for congenital hydrocephalus related to spina bifida. European Neurology. 2002;47(3):169-74. Links

We studied the morphometric MRI findings and their correlation with cognitive functions in a population of 10 young adults shunted for congenital hydrocephalus related to spina bifida. Morphometric MRI analysis included measurement of the ventricular dilatation index, frontal and occipital parenchymal thickness and the size of the corpus callosum. The neuropsychological status was evaluated, notably to look for a discrepancy between verbal and performance skills, a finding which has previously been described in hydrocephalic children. We also investigated whether there was a correlation between cognitive function and cerebral morphometric indexes. In each case, MRI demonstrated the structural changes associated with the Chiari II malformation. The size of the lateral ventricles varied, ranging from important dilatation to small ventricles. Six patients had only partial development of the corpus callosum. All patients had a normal global IQ. In our population of young adults, we did not observe any discrepancy between verbal and visuospatial performances as has been described in children with hydrocephalus. We found no relationship between cognitive function and ventricle dilatation or parenchymal thickness or between the size of the corpus callosum and callosal transfer.