HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Reiss, A. L. Articles by Freund, L. Search for Related Content PubMed PubMed Citation Articles by Reiss, A. L. Articles by Freund, L.

Neuroanatomy of fragile X syndrome

The temporal lobe

Kennedy Krieger Institute (Drs. Reiss and Freund and J. Lee) and the Departments of Psychiatry (Drs. Reiss and Freund) and Pediatrics (Dr. Reiss), The Johns Hopkins University School of Medicine, Baltimore, MD.

Fragile X syndrome, an X-linked genetic disorder caused by a mutation in the FMR-1 gene, is associated with a particular profile of abnormalities of behavior, learning, language, and memory, suggesting temporal lobe dysfunction. We undertook a quantitative neuroimaging study investigating the neuroanatomy of the temporal lobe in individuals with the fragile X mutation. The temporal lobe neuroanatomy of 15 young fragile X subjects was quantified and compared with that of 26 age- and IQ-matched control subjects. Analyses showed the right and left hippocampal volumes to be significantly larger in the fragile X group compared with the control group. Subjects with the fragile X mutation showed an age-related increase in volume of the hippocampus and an age-related decrease in volume of the superior temporal gyrus. Along with the findings of previous imaging studies of fragile X subjects, the results of the present investigation are consistent with studies showing a nonrandom distribution of expression of the FMR-1 gene in the developing brain, with increased expression in the cerebellum, hippocampus, and specific cortical regions. The results also suggest involvement of temporal lobe regions in the behavioral and cognitive abnormalities associated with fragile X syndrome.

Address correspondence and reprint requests to Dr. Allan L. Reiss, Director, Behavioral Neurogenetics and Neuroimaging, Kennedy Krieger Institute, 707 North Broadway, Baltimore, MD 21205.

Supported by grant MH00726 from the National Institute of Mental Health, grants HD24061 and HD25806 from the National Institutes of Health, and a grant from the John Merck Fund (Dr. Reiss).

Received November 16, 1993. Accepted in final form January 5, 1994.

This article has been cited by other articles:

				N. Kabakus, M. Aydin, H. Akin, T. A. Balci, A. Kurt, and E. Kekilli Fragile X Syndrome and Cerebral Perfusion Abnormalities: Single-Photon Emission Computed Tomographic Study J Child Neurol, December 1, 2006; 21(12): 1040 - 1046. [Abstract] [PDF]

				D. C. Rojas, J. A. Smith, T. L. Benkers, S. L. Camou, M. L. Reite, and S. J. Rogers Hippocampus and Amygdala Volumes in Parents of Children With Autistic Disorder Am J Psychiatry, November 1, 2004; 161(11): 2038 - 2044. [Abstract] [Full Text] [PDF]

				V. Menon, J. Leroux, C. D. White, and A. L. Reiss Frontostriatal deficits in fragile X syndrome: Relation to FMR1 gene expression PNAS, March 9, 2004; 101(10): 3615 - 3620. [Abstract] [Full Text] [PDF]

				A. S. Garrett, V. Menon, K. MacKenzie, and A. L. Reiss Here's Looking at You, Kid: Neural Systems Underlying Face and Gaze Processing in Fragile X Syndrome Arch Gen Psychiatry, March 1, 2004; 61(3): 281 - 288. [Abstract] [Full Text] [PDF]

				C. M. Greco, R. J. Hagerman, F. Tassone, A. E. Chudley, M. R. Del Bigio, S. Jacquemont, M. Leehey, and P. J. Hagerman Neuronal intranuclear inclusions in a new cerebellar tremor/ataxia syndrome among fragile X carriers Brain, August 1, 2002; 125(8): 1760 - 1771. [Abstract] [Full Text] [PDF]

				D. G. Munoz, R.J. Hagerman, C. Greco, S. Jacquemont, M. Leehy, and P. Hagerman Intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X Neurology, March 26, 2002; 58(6): 987 - 988. [Full Text]

				S. Eliez, C. M. Blasey, L. S. Freund, T. Hastie, and A. L. Reiss Brain anatomy, gender and IQ in children and adolescents with fragile X syndrome Brain, August 1, 2001; 124(8): 1610 - 1618. [Abstract] [Full Text] [PDF]

				W. T. Greenough, A. Y. Klintsova, S. A. Irwin, R. Galvez, K. E. Bates, and I. J. Weiler Synaptic regulation of protein synthesis and the fragile X protein PNAS, June 19, 2001; 98(13): 7101 - 7106. [Abstract] [Full Text] [PDF]

				K. M. Cornish, F. Munir, and G. Cross Differential Impact of the FMR-1 Full Mutation on Memory and Attention Functioning: A Neuropsychological Perspective J. Cogn. Neurosci., January 1, 2001; 13(1): 144 - 150. [Abstract] [Full Text]

				S. A. Irwin, R. Galvez, and W. T. Greenough Dendritic Spine Structural Anomalies in Fragile-X Mental Retardation Syndrome Cereb Cortex, October 1, 2000; 10(10): 1038 - 1044. [Abstract] [Full Text] [PDF]

				K. Braun and M. Segal FMRP Involvement in Formation of Synapses among Cultured Hippocampal Neurons Cereb Cortex, October 1, 2000; 10(10): 1045 - 1052. [Abstract] [Full Text] [PDF]

				J.C. Pruessner, L.M. Li, W. Serles, M. Pruessner, D.L. Collins, N. Kabani, S. Lupien, and A.C. Evans Volumetry of Hippocampus and Amygdala with High-resolution MRI and Three-dimensional Analysis Software: Minimizing the Discrepancies between Laboratories Cereb Cortex, April 1, 2000; 10(4): 433 - 442. [Abstract] [Full Text] [PDF]