This paper is only available as a PDF. To read, Please Download here.
Abstract
P300 (P3) and other long latency auditory event-related potentials (ERPs) were recorded
in 33 adults with fragile X syndrome. All patients had an abnormal P3. It was longer
in latency and smaller in amplitude than in controls. In several cases, it was split
into two separate components, and in others, was generated in response to expected
as well as unexpected events. Abnormal P3 was not related to age, percentage cell
fragility, or intellectual ability, but complete splitting was associated with the
presence of physical dysmorphisms. Our results are interpreted as showing that in
fragile X syndrome there is dysgenesis of the hippocampus and related brain structures.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Biological PsychiatryAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Changes in auditory P3 event related potential in schizophrenia and depression.Br J Psychiatry. 1986; (in press).
- Basal ganglia and limbic system pathology in schizophrenia.Arch Gen Psychiatry. 1985; 42: 784-790
- Association of fragile X syndrome with autism.Lancet. 1982; i: 100
- Diagnoses of fragile X syndrome. Clinical findings in 22 males with fragile site at Xq28.J Ment Defect Res. 1983; 27: 211-226
- Autism: Processing of novel auditory information assessed by event-related brain potentials.Electroencephalogr Clin Neurophysiol. 1984; 59: 238-248
- Neurological model for childhood autism.Arch Neurol. 1978; 35: 777
- Peabody Picture Vocabulary Test.American Guidance Service, Circle Lines, MN1981
- The psychological profile of the fragile X syndrome.Clin Genet. 1984; 25: 131-134
- Association of fragile X syndrome with autism.Am J Psychiatry. 1985; 142: 108-110
- Long latency event related components of the auditory evoked potential in dementia.Brain. 1978; 101: 635-648
- Endogenous potentials generated in the human hippocampal formation and amygdala by infrequent events.Science. 1980; 210: 803-805
- Pneumographic findings in the infantile autism syndrome: A correlation with temporal lobe disease.Brain. 1975; 98: 667
- Developmental and behavioural disturbances in boys with fragile X syndrome.Eur J Paediatr. 1984; 141: 265
- A pedigree of mental defect showing sex linkage.J Neurol Neurosurg Psychiatry. 1943; 6: 154-157
- Evoked potentials (P300) during sensory conditioning in autistic children.in: Karrer R Cohen I Tueting P Sixth International Conference on Event Related Slow Potentials. Academy of Sciences, New York1983: 362-369
- Infantile autism associated with fragile X syndrome.J Aut Dev Dis. 1982; 12: 295-301
- An electrophysiologic indication of defective information storage in childhood autism.Psychiatry Res. 1979; 1: 101-108
- The hippocampal formation as a source of the slow endogenous potentials.Electroencephalogr Clin Neurophysiol. 1983; 55: 417-426
- Discovery of a connective tissue dysplasia in the Martin Bell syndrome.Am J Med Genet. 1984; 17: 359-366
- Bibliography on X-linked mental retardation and related subjects II.Am J Med Genet. 1985; 21: 719-729
- Bibliography on X linked mental retardation and related subjects III.Am J Med Genet. 1986; 21: 69-99
- Anatomical correlates of the distribution of the pathological changes in the neocortex in Alzheimer's disease.in: Proc Natl Acad Sci USA. 82. 1985: 4531-4534
- A clinical application of the P3 component of event related potentials in normal ageing.Electroencephalgr Clin Neurophysiol. 1984; 59: 104-124
- Prevalence of the fragile X syndrome in an institution for the mentally handicapped.Br J Psychiatry. 1986; 148: 655-657
- Neuropathological observations in adult fragile X syndrome.Am J Hum Genet. 1984; 36 (abstr).: 72S
- Premature senility in Down's.Lancet. 1985; ii: 34
- P3 and other long latency auditory evoked potentials in presenile dementia, Alzheimer type, and alcoholic Korsakoff syndrome.Br J Psychiatry. 1985; 147: 702-707
- Human temporal lobe potential evoked in memory and language tasks.Electroencephalogr Clin Neurophysiol. 1986; 63: 145-159
- Event related potential in assessment of sensory and cognitive defects in the mentally retarded.in: Lehman D Callaway E Evoked Potentials: Application and Problems. Plenum, New York1979: 397-413
- Human endogenous limbic potentials: Cross modality and comparisons in epileptic subjects.in: Gaillard AWK Ritter W Tutorials in ERP Research. Endogenous components. North Holland Publishing, Amsterdam1983: 217-232
- Cortical evoked potentials in monozygotic twins and unrelated subjects: Comparisons of exogenous and endogenous components.Behav Genet. 1980; 10: 201-209
- Fragile sites on human chromosomes: Demonstration of their dependence on the type of tissue culture medium.Science. 1977; 197: 265-266
- The hippocampus in Down's syndrome.J Ment Defect Res. 1983; 27: 227-236
- Alzheimer changes in nondemented patients younger than sixty-five: Possible early stages of Alzheimer's disease and senile dementia of Alzheimer type.Ann Neurol. 1985; 17: 273-277
Article info
Publication history
Received in revised form:
July 30,
1986
Received:
May 1,
1986
The authors are grateful to Dr. Primrose and his staff at the Royal Scottish National Hospital, Scotland, for providing clinical details of the patients. Cytogenetic information was courtesy of the Duncan Guthrie Institute, Glasgow, and the MRC Clinical and Population Cytogenetics Unit, Edinburgh; the clinical averager was lent by courtesy of the MRC Brain Metabolism Unit, Edinburgh (Professor George Fink).Identification
Copyright
© 1987 Published by Elsevier Inc.
