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1
1
2

- : , (IPS),
(PPR), , .
: , 6947400074, 72
.. 15561

1.

(PPR, photoparoxysmal response)
(IPS, intermittent photic stimulation)
. ,
.1
,
EEG, , . , ,
,
,
(JME),
30 % - 40%. reflex
.2-4
, .
(
84% ), (6%), --
. ,


.

, ,
(pure photosensitive epilepsy).4
2.
.


. H
10 20% 5 % .
40% 3, 5
1.1 100,000 ,
. ( 2% )
7-19 7 100,000
10% .
,

6.

:
PPR ,
( ).

group 11-25
20 . 8-18 .

( 3 ),
Dravet.3
58
,
. (, /

1-4 ).7
:
,
1,7/1 (/). 3

.8
/ :
PPR. ,
. Familusi et al. (1998)

.3 De Graaf (1992)
,
PPR
.
De Graaf
.3
:
PPR
. (Quirk et al. (1995).3

3.

. ,
,
- .
.
Pokemon , 97,
, ( ) ,
. 9

3-30 Hz 60 Hz.
,
, 700
nm.3,10
, 3
, , 3,11


,
(eye closure sensitivity),
- . 12,13
4.
,

. .

. ,
, . 14
Nedd4-2 (18q21) , ,
.
. 15. PPR . 7q32 16p13

. 16p13

PPR. 16 60
PPR 6p21.2 13q31.3
6p21.2 PPR, 13q31.3
PPR
. 17 , PPR
. 18
.
Papio Papio Fayoumi chicken.
, Fepi strain of the Fayoumi
chicken


. (SV2A)
( 2 ). ,

mRNA .
SV2A
, .
. (
HSA1q21.1-21.2 ).19 ,
PPR Papio Papio
.18

5.
EEG
PPR, .


.18 PPR
.
, - .



100ms. PPR
. M
90 - 95%.
30 cm
.
.
, ppr (eye
closure test). 16 Hz. PPR
. ,
1 Hz ppr. 2
PPR,
60 Hz .
flash rate
PPR. 20
, CT, MRI
fMRI

6.


( Brodmann 17 , 18 19- ).
,
.
.

,

.
.21
,
( ).
,
.
( )
PPR
.
.
.
( magnocells)

,
.


. 4 .

, ,

PPR.
( Papio
Papio).
.


.
PPR IPS. :
.
L- ( )
, ,
,

.

( ).3,22
SPECT

,
. , ,
.23
7.
7.1

.


,

.

(), , ,
. (~12 )
100 Hz,
,
2m
. 24
, . ,
,
.
..

, , . 25
,

(
) ,
.
.
, .
7.2

, ,
.

.26,27


(PPR)
,
.

(sodium valproate,VPA). 27
VPA GABA

GABA .

53-76% .27

Covanis et al. (1982) .
Harding et al (1997)
84%
.27
(~14%-37%
) ,
.
VPA .

(lamotrigine),
(topiramate), (levetiracetam)
(piracetam) ( clobazam, clonazepam ethosuximide).
GABA

.
.

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Aug;70 Suppl 1:S269-79. Epub 2006 Jun 30.
2. Panayiotopoulos CP: The Epilepsies: Seizures, Syndromes and Management. Oxfordshire
(UK): Bladon Medical Publishing; 2005.
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9:67-72.
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revisited: updated European algorithm for visual stimulation in the EEG laboratory.
Epilepsia. 2012 Jan;53(1):16-24. doi: 10.1111/j.1528-1167.2011.03319.x. Epub 2011 Nov 16.

7 Anyanwu EC, Ehiri JE, Jones J: Photosensitive epilepsy beyond adolescence: is freedom
from photosensitivity age-dependent? (2003). Int J Adolesc Med Health. 2003 AprJun;15(2):125-32.
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Epilepsy Foundation of America Working Group. Epilepsy Foundation of America Working
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consensus of the Epilepsy Foundation of America Working Group. Epilepsia. 2005
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Jul;71(7):649-51.
13Singhi PD, Bansal D: Photosensitive epilepsies of children. Rev Neurol. 2001 Apr 1630;32(8):768-72.
14. Rudolf G, Valenti MP, Hirsch E: Genetic reflex epilepsies orphanet encyclopedia. march
2004
15. Dibbens LM, Ekberg J, Taylor I,et al: NEDD4-2 as a potential candidate susceptibility
gene for epileptic photosensitivity. Genes Brain Behav. 2007 Nov;6(8):750-5
16. Pinto D, Westland B, de Haan GJ et al: Genome-wide linkage scan of epilepsy-related
photoparoxysmal electroencephalographic response: evidence for linkage on
chromosomes 7q32 and 16p13. Hum Mol Genet. 2005 Jan 1;14(1):171-8
17. Tauer U, Lorenz S, Lenzen KP et al: Genetic dissection of photosensitivity and its
relation to idiopathic generalized epilepsy. Ann Neurol. 2005 Jun;57(6):866-73
18. Ulrich Stephani, Ulrike Tauer, Bobby Koeleman et al: Genetics of Photosensitivity
(Photoparoxysmal Response): A Review. Epilepsia, 45(Suppl. 1):1923, 2004)
19. Douaud M, Feve K, Pituello F, et al: Epilepsy caused by an abnormal alternative splicing
with dosage effect of the SV2A gene in a chicken model. PLoS One. 2011;6(10):e26932
20.Rubboli G, Parra J, Seri S et al: EEG diagnostic procedures and special investigations in
the assessment of photosensitivity. Epilepsia. 2004;45 Suppl 1:35-9.
21. Wilkins AJ, Bonanni P, Porciatti V et al: Physiology of human photosensitivity. Epilepsia.
2004;45 Suppl 1:7-13.
22. Parra J, Kalitzin SN, Lopes da Silva FH: Photosensitivity and visually induced seizures.
Curr Opin Neurol. 2005 Apr;18(2):155-9.
23. Kapucu LO, Gcyener K, Vural G et al: Brain SPECT evaluation of patients with pure
photosensitive epilepsy. J Nucl Med. 1996 Nov;37(11)
24. Takahashi Y, Fujiwara T: Effectiveness of broadcasting guidelines for photosensitive
seizure prevention. Neurology. 2004 Mar 23;62(6):990-3.
25. Wilkins AJ, Baker A, Amin D et al :Treatment of photosensitive epilepsy using coloured
glasses. Seizure. 1999 Dec;8(8):444-9.
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Aug;19(8):571-8.

27. Covanis A, Stodieck SR, Wilkins AJ. : Treatment of photosensitivity. Epilepsia. 2004;45
Suppl 1:40-5

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