Revista Chilena de Epilepsia

Actualizaciones

Ao 15, N 2, Agosto 2015

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Kathleen Batalla1,3, Perla David1,2, Antonia Mena1,2
kbatalla@gmail.com

ABSTRACT
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Landau Kleffner and 1957, where six described
previously healthy patients develop loss of reception / expression language associated with seizures.
The International League Against Epilepsy (ILAE)
FODVVLHV DV DQ DJHGHSHQGHQW HSLOHSWLF HQFHSKDlopathy. It is characterized by global or mixed acquired verbal agnosia, aphasia variable evolution
and paroxysmal abnormalities EEGcon spike-wave
during non-REM sleep in auditory cortical areas,
sometimes with seizures and behavioral disorders.
Studies show that aphasia is closely associated
with EEG abnormalities, electrical status epilepticus during sleep (ESES) SLK favors a maladaptive
plasticity; the non-REM sleep disruption not only
prevents new learning but can alter corticalesya
developed structures. It has raised a possible role
of the thalamus and the reticular activating system (RAS) in developing the SLK and ESES, whose
study is crucial.
Recently it has been considered the genetic factor in
the continuous spectrum from BECTS, autoimmune
&6:67DPELpQ/.6DQGWKHRULHVEDVHGRQWKHGLVcovery of autoantibodies described.

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Key Words: Diagnosis, Pathogenesis and Treatment.

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LandauKleffner Syndrome (SLK) is a rare condition

but poses several challenges. Key is early diagnosis as an aggressive early treatment of the disease
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limited knowledge of the pathophysiology, at present there is not an effective treatment.



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Revista Chilena de Epilepsia

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Ao 15, N 2, Agosto 2015

El Sistema Reticular activador (SRA) como un

posible locus de patologa.
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Factores genticos
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XXxxxxx et al.

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Ao 15, N 2, Agosto 2015

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Electroencefalograma

EEG caso 4: Abundante actividad irritativa en forma de punta y punta onda, de gran amplitud en regiones
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Sndrome de Landau-Kleffner

XXxxxxx et al.

Control Monitoreo Video EEG caso 1. Sueo no REM, Normal.

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CNV type (inheritance)

Gene content.a

Relationship of CNV/gene with

(Reference)

70
109

Loss (de novo)

Loss (de novo)

MDGA2 (part)
GRINZA (part)

258

Loss (de novo)

CDHI3 (part),HSBPI

ASD (Bucan et al., 2009)

RE, EE, ESES-like (Endele et al,
2010; Reutlinger et al, 2010)
SLI(SLI Consortium, 2004)

Loss (absent in mother)

ASD, SPI (Durand et al., 2007)

Loss (ND)
Loss (ND)

SHANK3, HDACI0, PLXNB2

(+37 additional genes)b
CTNNA3 (Intronic)
DIAPH3 (part)

202
245
118
100
89
54

Loss (Pat)
Loss (Mat)
Loss (Mat)
Loss (Pat)
Gain (Mat)
Gain (Mat)

Between CDH9 and CDH6

CTNNA3 (part)
CTNNA3 (intronic)
MACROD2 (intronic)
KIF268 (intronic)
FGFI2 (part)

ASD (Ma et al., 2009; Wang et al, 2009)

AZD (Myashita et al., 2007)
AZD (Myashita et al., 2007)
ASD (Anney et al., 2010)
SPI, ESZ (Calebe et al., 2010)
ASD, SLI (Kwasnicka-Crawford
et al., 2005)

139
514

Gain (Pat)
Gain (Mat)

20p12.1,Chr20:14395797-14464507
20q13.3,Chr20:60015337-60078775

69
63

Gain (Pat)
Loss (Pat)

PGFI 2 (part)
HRASLS, ATPI3A5, ATP
13A4, OPAI (part)
MACROD2 (intronic)
CDH4 (intronic)

10q21.3,Chr10:68087319-68110043
7q35,Chr7:146226258-146254837

23
29

Loss (Mat)
Gain (Mat)

CTNNA3 (intronic)
CNTNAP2 (intronic)

Patient (gender)

Diagnosis

Chromosomal location
coordinates (nt, build37)

De novo CNVs
1=1813 (M)
EA85 (F)

i-CSWSS
LKS-woESES

14q21.3,Chr14:47592578-47662394
16p13.2,Chr16:10246239-10354862

i-CSWSS

16q23.3,Chr16:83599498-83857382

s-CSWSS

22q13.32-33,Chr22:49346697-51219009

EC08 (F)
DX94 (F)
Inherited CNVs.
unreported previously
DZ77 (M)
EB99 (F)
EA25 (M)
10A603 (M)
3-19 (F)
7-1514 (M)

i-CSWSS
s-CSWSS

10q21.3,Chr10:68438375-68506557
13q21.2,Chr 13:60419603-60647521

68
228

i-CSWSS
i-CSWSS
i-CSWSS
i-CSWSS
LKS-wiESES
i-CSWSS

Sp14.1,Chr5:28634980-28837425
10q21.3,Chr10:68251535-68496866
10q21.3,Chr 10:68550481-68668009
20p12.1,Chr20:14491297-14591133
1q44,Chr1:245320978-245410054
3q28-q29,Chr3:192067520-192122231

7-1514 (M)
7-1514 (M)

i-CSWSS
i-CSWSS

3q29,Chr3:192212953-192352465
3q29,Chr3:192870621-193385022

12-522 (M)
EA85 (F)
Inherited CNVs,
previously known
DZ29 (M)
12-522 (M)

LKS-woESES
LKS-woESES

LKS-wiESES
LKS-woESES

DZ52(F)
Possibly de novo CNVs
DY34 (M)b

Size (kb)

1,872

Revista Chilena de Epilepsia



Table I. List of CNVs detected in CSWSS and LKS patients and related with epileptic, autism spectrum, cognitive and/or speech and language disorders

AZD (Myashita et al., 2007)

ASD (Vorstman et al, 2011)

ASD (Anney et al., 2010)

CDH4: cadherin

AZD (Myashita et al., 2007)

ASD, ESZ, SLI, SCZ; FOXP2 target
(Vernes ert al., 2008; Peagarikano
et al., 2011)
Ao 15, N 2, Agosto 2015

ESES, electrical status epilepticus during low-wave sleep; CSWSS, continuous spike and waves during slow-wave sleep syndrome (i: idiopatic, s: symptomatic); LKS, Landau-Kleffner syndrome; LKS-wiESES, LKS with electrical
status epilepticus during slow-wave sleep; LKS-woESES, LKS without electrical status epilepticus during slow-wave sleep; EE, epileptic elcephalopathy; RE, rolandic epilepsy; ESZ, epileptic seizures; ASD, autism spectrum disorder;
(A+(SaOLPTLYZKPZLHZL":30ZWLJPJSHUN\HNLPTWHPYTLU[":70ZWLLJOPTWHPYTLU["(+/+H[[LU[PVUKLJP[O`WLYHJ[P]P[`KPZVYKLY":*AZJOPaVWOYLUPH"*5=JVW`U\TILY]HYPH[PVU"U[U\JSLV[PKL"RIRPSVIHZL"*OYJOYVTVZVTL"
part, partial deletion or duplication; ND, not determinated; Pat, paternally inherited; Mat, maternally inherited. All events including truncating deletions concerned coding sequences within the corresponding genes. F, female; M, male.
Chromosomal locations are given according to the February 2009 human reference sequence (build37) (http://genome.ucsc.edul).
a Genes are listed according to their chromosomal location from ptel (telomere of short arm) to qtel (telomare of long arm).
b Unaffected mother not carrier, unaffected father not tested; the very large size of the deletion and the large number of genes contained within strongly indicates de novo origin.

Sndrome de Landau-Kleffner

Table 2. List of other rare CNVs of interest detected in CSWSS and LKS patients
Patient (gender)

Diagnosis

Chromosomal location coordinates

(nt, build37)

Size (kb)

De novo CNVs
4-914 (M)

i-CSWSS

8p23.1,Chr8:9598226-10787792

1,189

CNV type
(inheritance)

Gene content.a

Comment (Reference)

Loss (de novo)

TNKS (part), MR124-1,MSRA,PRSS55,
RPILI, SOX7, PINOCI, XKR6 (part)
Loss (de novo)
MMP16 (part)

MIR124-1: micro RNA involved in neuronal

differentiation (Yoo et al., 2011)
MMP16: matrix metalloproteinase

Gain (absent in
the mother)
Loss (absent in
the mother)
Gain (absent in
the mother)
Loss (Parents
not available)

ARPCS, APOBEC4, RGLI (part)

RSRCI (part), MLFI (part)

KONMB3 (part)
ZNF639, MFNI, GNB4 (part)
CNKSR2 (part)

KCNMB3: potassium channel subunit

i-CSWSS

8q21,Chr8:89102084-89398298

296

i-CSWSS

1q25.3,Chr1:183594532-183820790

226

029a (M)

i-CSWSS

3q25,Chr3:158183313-158296641

113

DX94 (F)

s-CSWSS

3q26.32-33,Chr3:178969064-179150965

182

DV95 (F)

i-CSWSS

Xp22.12,ChrX:21523673-21558329

35

Inherited CNVs,
unreported
previously
09A1613 (M)
1-1813 (M)
DY14 (M)

i-CSWSS
i-CSWSS
LKS-wiESES

3q25,Chr3:154395454-154788305
5q11.2,Chr5:58571292-58745139
6q27,Chr6:167355901-167373534

393
174
18

Loss (Pat)
Loss (Pat)
Loss (Mat)

MME (part)
PDE4D (intronic)
RNASET2 (part)

13-13 (M)
13-19 (M)
ED31 (M)

i-CSWSS
i-CSWSS
s-CSWSS

7q22,Chr7:107214193-107262539
8p22,Chr8:14553553-14572370
8q22.3,Chr8:102849359-102868211

48
19
19

Loss (Mat)
Loss (Mat)
Loss (Pat)

DUS4L (part),BCAP29 (part)

SGCZ (intronic)
NCALD (intronic)

4-52 (M)
003a (M)
016a (M)

i-CSWSS
i-CSWSS
s-CSWSS

1p21.2-21.1,Chr1:102123099-103099662
3p11.2,Chr3:87917810-8877883
3q29,Chr3:194088557-194130145

977
29
217

Gain (Mat)
Gain (Mat)
Gain (Mat)

3-81 (F)
EA85 (F)

LKS-wiESES
LKS-woESES

8q11.23,Chr8:53397126-53808953
9p13.2,Chr9:37299058-37451697

412
153

Gain (Pat)
Gain (Mat)

DY64 (M)
DY61 (P)

i-CSWSS
s-CSWSS

10q21.1,Chr10:56034426-56089442
14q21.3,Chr14:46524008-47161263

55
637

Gain (Pat)
Gain (Mat)

OLMF3
HTRIF, CGGBPI,ZNF654,C3orf38
LRRCI5 (part),GPS,ATPI3A3,
LOC100131551
FAM150A,RBICCI
ZCCHC7 (part), GRHPR,
ZBTB5 (part)
PCDH15 (part)
RPL10L

MME: enkephalinase
RNASET2: autosomal recessive
leucoencephalography (Henncke et al., 2009)
SGCZ: sarcoglycan, zeta
NCALD: reuronal calcium sensor neurocalcin
delta: EF-hand calcium binding domain
OLMF3: downstream target of PAX6
HTRIF: serotonin receptor
Neighboring ATPI3A4: ASD, SLI
(Kwasnicka-Crawford et al., 2005)
Protocadherin
RPL10 mutations reported in ASD
patients (Klauck et al., 2006)



Continued

XXxxxxx et al.

025a (M)
CNVs, undetermined
whether de novo
or inherited
029a (M)

Revista Chilena de Epilepsia

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