Multisystem neuronal degeneration, hepatosplenomegaly, and adrenocortical deficiency associated with reduced tissue arachidonic acid

Peter J Dyck, J. K. Yao, D. E. Knickerbocker, R. T. Holman, M. R. Gomez, A. B. Hayles, E. H. Lambert

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

The authors studied two brothers with a previously unrecognized syndrome characterized by failure to thrive, visual loss resulting from retinitis pigmentosa, sensorineural hearing loss, mental retardation, distal progressive muscular atrophy, hepatosplenomegaly, and adrenocortical deficiency. Biopsied tissue showed a consistent reduction of arachidonic acid and other polyunsaturated fatty acids, but not of linoleic acid. Deranged metabolism and specifically a deficiency of polyunsaturated fatty acids, including arachidonic acid, may be involved.

Original languageEnglish (US)
Pages (from-to)925-934
Number of pages10
JournalNeurology
Volume31
Issue number8
StatePublished - 1981

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Unsaturated Fatty Acids
Arachidonic Acid
Spinal Muscular Atrophy
Failure to Thrive
Retinitis Pigmentosa
Sensorineural Hearing Loss
Linoleic Acid
Intellectual Disability

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Dyck, P. J., Yao, J. K., Knickerbocker, D. E., Holman, R. T., Gomez, M. R., Hayles, A. B., & Lambert, E. H. (1981). Multisystem neuronal degeneration, hepatosplenomegaly, and adrenocortical deficiency associated with reduced tissue arachidonic acid. Neurology, 31(8), 925-934.

Multisystem neuronal degeneration, hepatosplenomegaly, and adrenocortical deficiency associated with reduced tissue arachidonic acid. / Dyck, Peter J; Yao, J. K.; Knickerbocker, D. E.; Holman, R. T.; Gomez, M. R.; Hayles, A. B.; Lambert, E. H.

In: Neurology, Vol. 31, No. 8, 1981, p. 925-934.

Research output: Contribution to journalArticle

Dyck, PJ, Yao, JK, Knickerbocker, DE, Holman, RT, Gomez, MR, Hayles, AB & Lambert, EH 1981, 'Multisystem neuronal degeneration, hepatosplenomegaly, and adrenocortical deficiency associated with reduced tissue arachidonic acid', Neurology, vol. 31, no. 8, pp. 925-934.
Dyck, Peter J ; Yao, J. K. ; Knickerbocker, D. E. ; Holman, R. T. ; Gomez, M. R. ; Hayles, A. B. ; Lambert, E. H. / Multisystem neuronal degeneration, hepatosplenomegaly, and adrenocortical deficiency associated with reduced tissue arachidonic acid. In: Neurology. 1981 ; Vol. 31, No. 8. pp. 925-934.
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