PMID: 9187480Jun 1, 1997Paper

Two different pathological conditions of photoparoxysmal responses in hereditary dentatorubral-pallidoluysian atrophy

Brain & Development
Y TakahashiM Seino

Abstract

In order to reveal the pathophysiology of photoparoxysmal responses (PPRs) in photosensitive patients with hereditary dentatorubral-pallidoluysian atrophy (DRPLA) who had expansion of the CAG repeat in the DRPLA gene, we studied the characteristics of PPRs using optical filters with specific wavelength transmission. In two patients, the wavelength spectrum around 700 nm (670-720 nm) was apparently the only visible range essential for eliciting PPRs, and flash lights containing the essential wavelength elicited PPRs. In another patient, PPRs were elicited by flash lights above certain quantity of light and independent of the wavelength composition of the lights. These data suggest that two different pathological conditions contribute to PPRs in DRPLA patients; one condition depends on the essential wavelength spectrum around 700 nm, and the other not on the wavelength, but on the quantity of light. The condition contributing to PPRs in all three patients was not determined directly by the level of the CAG repeat expansion in the DRPLA gene.

References

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Citations

Jan 20, 1999·Acta Paediatrica Japonica; Overseas Edition·J FurushoK Kumagai
Mar 3, 2001·Acta Neurologica Scandinavica·Y TakahashiN Kondo
Feb 1, 1997·The Tohoku Journal of Experimental Medicine·Y TakahashiM Seino
Dec 21, 2004·Journal of Child Neurology·Alberto VerrottiFrancesco Chiarelli
Mar 6, 2008·Clinical EEG and Neuroscience·John R Hughes

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