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Photoparoxysmal response 1(PPR1)

MedGen UID:
358382
Concept ID:
C1868677
Disease or Syndrome
Synonyms: EPILEPSY, PHOTOGENIC; PHOTOCONVULSIVE REACTION; PHOTOSENSITIVITY; PPR1
 
Cytogenetic location: 6p21.1
 
Monarch Initiative: MONDO:0007559
OMIM®: 132100

Definition

The photoparoxysmal response (PPR), also referred to as photosensitivity, is defined as the abnormal occurrence of cortical spikes or spike and wave discharges on electroencephalogram (EEG) in response to intermittent light stimulation (Doose and Waltz, 1993). Photosensitivity is a frequent finding in patients with idiopathic generalized epilepsy (see 600669), especially those with juvenile myoclonic epilepsy, suggesting a common epileptogenic pathway for both phenomena. The comorbidity of the 2 disorders suggests that presence of PPR may also increase the risk for epilepsy (Stephani et al., 2004; Tauer et al., 2005). Genetic Heterogeneity of Photoparoxysmal Response The PPR1 locus has been mapped to chromosome 6p21. See also PPR2 (609572), mapped to chromosome 13q31, and PPR3 (609573), mapped to chromosome 7q32. [from OMIM]

Clinical features

From HPO
EEG with photoparoxysmal response
MedGen UID:
765735
Concept ID:
C3552821
Finding
EEG abnormalities (epileptiform discharges) evoked by flashing lights or black and white striped patterns.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Etiology

Hiraiwa A, Kobayashi Y, Hojo M, Tohyama J
Epileptic Disord 2023 Feb;25(1):80-86. Epub 2023 Apr 17 doi: 10.1002/epd2.20052. PMID: 36946369
Beniczky S, Aurlien H, Franceschetti S, Martins da Silva A, Bisulli F, Bentes C, Canafoglia L, Ferri L, Krýsl D, Rita Peralta A, Rácz A, Cross JH, Arzimanoglou A
Epilepsia 2020 Sep;61(9):e124-e128. Epub 2020 Sep 19 doi: 10.1111/epi.16655. PMID: 32949474
Reyhani A, Özkara Ç
Epileptic Disord 2020 Jun 1;22(3):281-290. doi: 10.1684/epd.2020.1162. PMID: 32554361
Quirk JA, Fish DR, Smith SJ, Sander JW, Shorvon SD, Allen PJ
Electroencephalogr Clin Neurophysiol 1995 Oct;95(4):260-7. doi: 10.1016/0013-4694(95)00118-i. PMID: 8529557
Muszkat M, Guimarães RM, da Silva AB, de Campos CJ
Arq Neuropsiquiatr 1990 Sep;48(3):301-5. doi: 10.1590/s0004-282x1990000300006. PMID: 2264785

Diagnosis

Hiraiwa A, Kobayashi Y, Hojo M, Tohyama J
Epileptic Disord 2023 Feb;25(1):80-86. Epub 2023 Apr 17 doi: 10.1002/epd2.20052. PMID: 36946369
Beniczky S, Aurlien H, Franceschetti S, Martins da Silva A, Bisulli F, Bentes C, Canafoglia L, Ferri L, Krýsl D, Rita Peralta A, Rácz A, Cross JH, Arzimanoglou A
Epilepsia 2020 Sep;61(9):e124-e128. Epub 2020 Sep 19 doi: 10.1111/epi.16655. PMID: 32949474
Geithner J, Schneider F, Wang Z, Berneiser J, Herzer R, Kessler C, Runge U
Epilepsia 2012 Aug;53(8):1379-86. Epub 2012 Jun 12 doi: 10.1111/j.1528-1167.2012.03526.x. PMID: 22686598
Labate A, Ambrosio R, Gambardella A, Sturniolo M, Pucci F, Quattrone A
Epilepsy Res 2007 Oct;77(1):17-21. Epub 2007 Sep 11 doi: 10.1016/j.eplepsyres.2007.07.010. PMID: 17851038
Quirk JA, Fish DR, Smith SJ, Sander JW, Shorvon SD, Allen PJ
Electroencephalogr Clin Neurophysiol 1995 Oct;95(4):260-7. doi: 10.1016/0013-4694(95)00118-i. PMID: 8529557

Therapy

Toyota T, Akamatsu N, Tanaka A, Tsuji S, Uozumi T
Clin Neurophysiol 2014 Feb;125(2):250-4. Epub 2013 Sep 4 doi: 10.1016/j.clinph.2013.08.002. PMID: 24011985
Geithner J, Schneider F, Wang Z, Berneiser J, Herzer R, Kessler C, Runge U
Epilepsia 2012 Aug;53(8):1379-86. Epub 2012 Jun 12 doi: 10.1111/j.1528-1167.2012.03526.x. PMID: 22686598
Jukkarwala A, Hassan H, Nair M, Iyer RS
Epileptic Disord 2011 Mar;13(1):103-6. doi: 10.1684/epd.2011.0416. PMID: 21393101
Yasuda K, Kobayashi K, Sugita K, Takanashi J, Kohno Y, Iwasa H, Mine S, Nakajima Y
Neuroreport 2000 Jan 17;11(1):203-6. doi: 10.1097/00001756-200001170-00040. PMID: 10683858
de Falco FA, Roberti R, Florio C, Franzese G
Acta Neurol (Napoli) 1992 Aug-Dec;14(4-6):290-6. PMID: 1293972

Prognosis

Hiraiwa A, Kobayashi Y, Hojo M, Tohyama J
Epileptic Disord 2023 Feb;25(1):80-86. Epub 2023 Apr 17 doi: 10.1002/epd2.20052. PMID: 36946369
Madaan P, Jauhari P, Luhar ZM, Chakrabarty B, Gulati S
Clin EEG Neurosci 2020 Nov;51(6):399-402. Epub 2020 Jan 6 doi: 10.1177/1550059419899327. PMID: 31903771
Geithner J, Schneider F, Wang Z, Berneiser J, Herzer R, Kessler C, Runge U
Epilepsia 2012 Aug;53(8):1379-86. Epub 2012 Jun 12 doi: 10.1111/j.1528-1167.2012.03526.x. PMID: 22686598
Ohtsuka Y, Hattori J, Ishida T, Ogino T, Oka E
Dev Med Child Neurol 1999 Mar;41(3):203-6. doi: 10.1017/s0012162299000390. PMID: 10210253
Quirk JA, Fish DR, Smith SJ, Sander JW, Shorvon SD, Allen PJ
Electroencephalogr Clin Neurophysiol 1995 Oct;95(4):260-7. doi: 10.1016/0013-4694(95)00118-i. PMID: 8529557

Clinical prediction guides

Hiraiwa A, Kobayashi Y, Hojo M, Tohyama J
Epileptic Disord 2023 Feb;25(1):80-86. Epub 2023 Apr 17 doi: 10.1002/epd2.20052. PMID: 36946369
Beniczky S, Aurlien H, Franceschetti S, Martins da Silva A, Bisulli F, Bentes C, Canafoglia L, Ferri L, Krýsl D, Rita Peralta A, Rácz A, Cross JH, Arzimanoglou A
Epilepsia 2020 Sep;61(9):e124-e128. Epub 2020 Sep 19 doi: 10.1111/epi.16655. PMID: 32949474
Geithner J, Schneider F, Wang Z, Berneiser J, Herzer R, Kessler C, Runge U
Epilepsia 2012 Aug;53(8):1379-86. Epub 2012 Jun 12 doi: 10.1111/j.1528-1167.2012.03526.x. PMID: 22686598
Trenité DG, French JA, Hirsch E, Macher JP, Meyer BU, Grosse PA, Abou-Khalil BW, Rosenfeld WE, van Gerven J, Novak GP, Parmeggiani L, Schmidt B, Gibson D, Guerrini R
Epilepsy Res 2007 May;74(2-3):193-200. Epub 2007 Apr 19 doi: 10.1016/j.eplepsyres.2007.03.012. PMID: 17448639
Muszkat M, Guimarães RM, da Silva AB, de Campos CJ
Arq Neuropsiquiatr 1990 Sep;48(3):301-5. doi: 10.1590/s0004-282x1990000300006. PMID: 2264785

Recent systematic reviews

Kasteleijn-Nolst Trenite D, Genton P, Brandt C, Reed RC
Epilepsy Res 2017 Jul;133:113-120. Epub 2016 Dec 2 doi: 10.1016/j.eplepsyres.2016.11.012. PMID: 28034485

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