Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome

Thomas M. Kelley, Laura A. Hatfield, Doris Lin, Anne Marie Spalding Comi

Research output: Contribution to journalArticle

Abstract

Sturge-Weber syndrome is a neurocutaneous disorder with vascular malformations of the skin, brain, and eye. The objective of this study was to determine whether cortical atrophy in patients with Sturge-Weber syndrome correlates with clinical severity. Eighteen subjects (age 4 months-35 years) with unilateral Sturge-Weber syndrome received a neurologic examination and submitted previous magnetic resonance imaging (MRI) films. A blinded investigator assigned clinical severity scores based on seizures, hemiparesis, visual field cut, and cognitive impairments. Computer-aided analysis of MRIs produced laterality scores for cortical volume asymmetry. A significant relationship existed between clinical severity and laterality scores (Spearman's rho = - 0.804; P <.001). Laterality scores also correlated well with hemiparesis subscores and weakly with cognitive impairment subscores (Kendall's tau b; P <.05). Using this simple, computer-aided analysis, cortical volume asymmetry correlated with clinical status. This method offers the advantages of relative simplicity, objectivity, and wide applicability to films from outside institutions, as would be encountered in clinical practice.

Original languageEnglish (US)
Pages (from-to)867-870
Number of pages4
JournalJournal of Child Neurology
Volume20
Issue number11
DOIs
StatePublished - Nov 2005

Fingerprint

Sturge-Weber Syndrome
Atrophy
Paresis
Neurocutaneous Syndromes
Central Nervous System Vascular Malformations
Neurologic Examination
Visual Fields
Seizures
Research Personnel
Magnetic Resonance Imaging
Skin
Cognitive Dysfunction

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

Cite this

Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome. / Kelley, Thomas M.; Hatfield, Laura A.; Lin, Doris; Comi, Anne Marie Spalding.

In: Journal of Child Neurology, Vol. 20, No. 11, 11.2005, p. 867-870.

Research output: Contribution to journalArticle

@article{56b6ee021e634107b7512cdfbe5c6680,
title = "Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome",
abstract = "Sturge-Weber syndrome is a neurocutaneous disorder with vascular malformations of the skin, brain, and eye. The objective of this study was to determine whether cortical atrophy in patients with Sturge-Weber syndrome correlates with clinical severity. Eighteen subjects (age 4 months-35 years) with unilateral Sturge-Weber syndrome received a neurologic examination and submitted previous magnetic resonance imaging (MRI) films. A blinded investigator assigned clinical severity scores based on seizures, hemiparesis, visual field cut, and cognitive impairments. Computer-aided analysis of MRIs produced laterality scores for cortical volume asymmetry. A significant relationship existed between clinical severity and laterality scores (Spearman's rho = - 0.804; P <.001). Laterality scores also correlated well with hemiparesis subscores and weakly with cognitive impairment subscores (Kendall's tau b; P <.05). Using this simple, computer-aided analysis, cortical volume asymmetry correlated with clinical status. This method offers the advantages of relative simplicity, objectivity, and wide applicability to films from outside institutions, as would be encountered in clinical practice.",
author = "Kelley, {Thomas M.} and Hatfield, {Laura A.} and Doris Lin and Comi, {Anne Marie Spalding}",
year = "2005",
month = "11",
doi = "10.1177/08830738050200110201",
language = "English (US)",
volume = "20",
pages = "867--870",
journal = "Journal of Child Neurology",
issn = "0883-0738",
publisher = "SAGE Publications Inc.",
number = "11",

}

TY - JOUR

T1 - Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome

AU - Kelley, Thomas M.

AU - Hatfield, Laura A.

AU - Lin, Doris

AU - Comi, Anne Marie Spalding

PY - 2005/11

Y1 - 2005/11

N2 - Sturge-Weber syndrome is a neurocutaneous disorder with vascular malformations of the skin, brain, and eye. The objective of this study was to determine whether cortical atrophy in patients with Sturge-Weber syndrome correlates with clinical severity. Eighteen subjects (age 4 months-35 years) with unilateral Sturge-Weber syndrome received a neurologic examination and submitted previous magnetic resonance imaging (MRI) films. A blinded investigator assigned clinical severity scores based on seizures, hemiparesis, visual field cut, and cognitive impairments. Computer-aided analysis of MRIs produced laterality scores for cortical volume asymmetry. A significant relationship existed between clinical severity and laterality scores (Spearman's rho = - 0.804; P <.001). Laterality scores also correlated well with hemiparesis subscores and weakly with cognitive impairment subscores (Kendall's tau b; P <.05). Using this simple, computer-aided analysis, cortical volume asymmetry correlated with clinical status. This method offers the advantages of relative simplicity, objectivity, and wide applicability to films from outside institutions, as would be encountered in clinical practice.

AB - Sturge-Weber syndrome is a neurocutaneous disorder with vascular malformations of the skin, brain, and eye. The objective of this study was to determine whether cortical atrophy in patients with Sturge-Weber syndrome correlates with clinical severity. Eighteen subjects (age 4 months-35 years) with unilateral Sturge-Weber syndrome received a neurologic examination and submitted previous magnetic resonance imaging (MRI) films. A blinded investigator assigned clinical severity scores based on seizures, hemiparesis, visual field cut, and cognitive impairments. Computer-aided analysis of MRIs produced laterality scores for cortical volume asymmetry. A significant relationship existed between clinical severity and laterality scores (Spearman's rho = - 0.804; P <.001). Laterality scores also correlated well with hemiparesis subscores and weakly with cognitive impairment subscores (Kendall's tau b; P <.05). Using this simple, computer-aided analysis, cortical volume asymmetry correlated with clinical status. This method offers the advantages of relative simplicity, objectivity, and wide applicability to films from outside institutions, as would be encountered in clinical practice.

UR - http://www.scopus.com/inward/record.url?scp=31144477762&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=31144477762&partnerID=8YFLogxK

U2 - 10.1177/08830738050200110201

DO - 10.1177/08830738050200110201

M3 - Article

C2 - 16417855

AN - SCOPUS:31144477762

VL - 20

SP - 867

EP - 870

JO - Journal of Child Neurology

JF - Journal of Child Neurology

SN - 0883-0738

IS - 11

ER -