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2016-.indb 4 2016/07/11 13:40:38

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2016-.indb 5 2016/07/11 13:40:38

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Introduction

1980

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2016-.indb 18 2016/07/11 13:40:39

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2016-.indb 19 2016/07/11 13:40:39

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ACTH adrenocorticotropic hormone

ADC apparent diffusion coefficient

ADEM acute disseminated encephalomyelitis

aEEG amplitude-integrated electroencephalography

AEFCSE acute encephalopathy with febrile convulsive status epilepticus

AESD

AESD acute encephalopathy with biphasic seizures and late reduced diffusion

AERRPS acute encephalitis with refractory, repetitive partial seizures

AIEF acute infantile encephalopathy predominantly affecting the frontal lobes

ALT alanine transaminase

GPT

ANE acute necrotizing encephalopathy

AR autosomal recessive

ARG arginase

ASL argininosuccinate lyase

ASS argininosuccinate synthetase

AST aspartate transaminase

GOT

BCAA branched chain amino acid

BCKS branched chain keto acid

BUN blood urea nitrogen

CACT carnitine acylcarnitine translocase

CHDF continuous hemodiafiltration

CK creatine kinase

Co coenzyme

CPS carbamoylphosphate synthetase

CPS complex partial seizure

CPT carnitine palmitoyl transferase

Cre creatinine

CRP C-reactive protein C

CT computed tomography

CQ clinical question

2016-.indb 20 2016/07/11 13:40:40

xxi

DIC disseminated intravascular coagulation

DWI diffusion-weighted imaging

EBP extracorporeal blood purification

EHEC enterohemorrhagic Escherichia coli

ETF electron transfer flavoprotein

ETFDH electron transfer flavoprotein dehydrogenase

FAD flavin adenine dinucleotide

FFP fresh frozen plasma

FIRDA frontal intermittent rhythmic delta activity

FIRES febrile infection-related epilepsy syndrome

FLAIR fluid attenuated inversion recovery

GABA gamma-aminobutyric acid

GCS Glasgow Coma Scale

GFAP glial fibrillary acidic protein

Gln glutamine

Glu glutamate

Hb hemoglobin

HH hemiconvuslsion-hemiplegia

HHE hemiconvuslsion-hemiplegia-epilepsy

HHH hyperornithinemia-hyperammonemia- homocitrullinuria

HHV human herpesvirus

HSES hemorrhagic shock and encephalopathy syndrome

Ht hematocrit

HUS hemolytic uremic syndrome

IL interleukin

JCS Japan Coma Scale

LDH lactate dehydrogenase

L / P lactate / pyruvate /

MCAD medium-chain acyl-CoA dehydrogenase CoA

MELAS mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes

MELAS

MERS mild encephalitis / encephalopathy with a reversible splenial lesion

MMP matrix metalloproteinase

2016-.indb 21 2016/07/11 13:40:40

xxii

mPSL methylprednisolone

MRI magnetic resonance imaging

MS multiple sclerosis

MSUD maple syrup urine disease

NAA N-acetyl aspartate N-

NAGS N-acetylglutamate synthase N-

NMDA N-methyl-D-aspartate N- -D-

NORSE new-onset refractory status epilepticus

NSE neuron specific enolase

OCTN organic cation transporter

OHdG hydroxydeoxyguanosine

OIRDA occipital intermittent rhythmic delta activity

ORNT ornithine / citrulline antiporter

OTC ornithine transcarbamylase

PDHC pyruvate dehydrogenase complex

PE plasma exchange

PICU pediatric intensive care unit

PLED periodic lateralized epileptic discharge

PMMA polymethyl methacrylate

PRES posterior reversible encephalopathy syndrome RPLS

PT prothrombin time

RESLES reversible splenial lesion syndrome

ROC Receiver Operating Characteristic

RPLS reversible posterior leukoencephalopathy syndrome

PRES

RRF ragged-red fiber

RSV respiratory syncytial virus RS

SCAD short chain acyl-CoA dehydrogenase CoA

SDH succinate dehydrogenase

SIDS sudden infant death syndrome

SIRS systemic inflammatory response syndrome

SNP single nucleotide polymorphism

SPECT single photon emission computed tomography

SSV strongly SDH-reactive blood vessel SDH

TCA tricarboxylic acid

TFP trifunctional protein

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xxiii

TIMP tissue inhibitor of metalloproteinase

TMA thrombotic microangiopathy

TNF tumor necrosis factor

TNFR tumor necrosis factor receptor

TTM targeted temperature management

TTW therapeutic time window

T2WI T2 weighted imaging T2

VLCAD very long chain acyl-CoA dehydrogenase CoA

VZV varicella-zoster virus

XLR X-linked recessive X

2016-.indb 23 2016/07/11 13:40:40

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2016-.indb 1 2016/07/12 9:25:14

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cationSubheading OR definitionAll FieldsAND FiltersEnglishJapaneseChildbirth-18 years92

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1 Mizuguchi M, Yamanouchi H, Ichiyama T, Shiomi M. Acute encephalopathy associated with influenza and other viral infec-

tions. Acta Neurol Scand 2007 ; 115 : 45-56.5 2 2009 ; 62 : 2483-2528.6

3 Tada H, Takanashi J, Barkovich AJ, et al. Clinically mild encephalitis/ encephalopathy with a reversible splenial lesion. Neu-

Glasgow Coma ScaleGlasgow Coma Scale

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1 HHV-67 VZV respiratory syncytial virusRSV 2 EHEC 3

1 Reyeclassical Reye syndrome 2 ANE hemorrhagic shock and encephalopathyHSES Reye-like syndrome3

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4

2016-.indb 5 2016/07/12 9:25:15

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rology 2004 ; 63 : 1854-1858.5 4 Sakuma H. Acute encephalitis with refractory, repetitive partial seizures. Brain Dev 2009 ; 31 : 510-514.5 5 RPLS / PRES No.26. 2I2013 : 283-288.5

6 ABC 2011 ; 74 : 937-944.5

a 122001

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2016-.indb 6 2016/07/12 9:25:15

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2016-.indb 7 2016/07/12 9:25:15

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ANE 59% 41%AESD 3.3 3.4 2 41%HHV-620%c

MERS 52% 45% 5.6 3.7 5AESD ANE 34%12%4% 3%c 58% 42% 6.3 3.4 6MERS 20%AESD10%ANE6%c

HHV-6 43% 57% 0 1 0.8 1.1 1 AESD 64%ANE5%MERS2%c

PubMed

PubMedacute encephalopathytiabORacute diseaseMeSH TermsAND brain diseasesMeSH TermsAND Virus

DiseasesmeshAND epidemiologyshFiltersEnglish, Japanese, Childbirth-18 years99

/TH or /ALand /TH or /ALand /TH or /

ALor /TH or /ALor /ALorSHandPT and CK12325612131816

1 Ishikawa T, Asano Y, Morishima T, et al. Epidemiology of acute childhood encephalitis. Aichi Prefecture, Japan, 1984-90.

Brain Dev 1993 ; 15 : 192-197.5 2 2011 ; 69 : 411-4165 3 Hoshino A, Saitoh M, Oka A, et al. Epidemiology of acute encephalopathy in Japan, with emphasis on the association of virus-

es and syndromes. Brain Dev 2012 ; 34 : 337-343.5

2016-.indb 8 2016/07/12 9:25:15

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2016-.indb 9 2016/07/12 9:25:15

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2016-.indb 10 2016/07/12 9:25:16

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1 Hoshino A, Saitoh M, Oka A, et al. Epidemiology of acute encephalopathy in Japan, with emphasis on the association of virus-

es and syndromes. Brain Dev 2012 ; 34 : 337-343.5 2 Mizuguchi M. Influenza encephalopathy and related neuropsychiatric syndromes. Influenza Other Resp Virus 2013 ; 7Suppl

3: 67-71.5

a 222011 : 3-10

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10% 5% 25% 55% 5%

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Hoshino A, Saitoh M, Oka A, et al. Epidemiology of acute encephalopathy in Japan, with emphasis on the association of viruses and syndromes. Brain Dev 2012 ; 34 : 337-343. 2220113-10

1

2016-.indb 11 2016/07/12 9:25:16

2016-.indb 12 2016/07/12 9:25:16

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2016-.indb 13 2016/07/12 9:25:16

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2016-.indb 15 2016/07/12 9:25:16

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electrical storm 24spindle wave

/ AST90150 IU/L LDH ALT2DIC /

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5 4

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2016-.indb 16 2016/07/12 9:25:16

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2016-.indb 17 2016/07/12 9:25:16

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PubMed

PubMedacute encephalopathytiabANDbiomarkerMeSH217

/TH or /ALand /TH or /ALandABY PT359

13 4 AESD visinin-like protein 1

visinin-like protein 1AESDAESD 1

14 4 AESD S-100NSEtau

S-100NSEtau AESD AESD 0 2 NSE S-100 tau 100 pg/mL 83.3%

15 4 AESD AESD 24

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17 4 AESD

IL-6 IL-6IL-10sTNFR1IL-6 LI-10 sTNFR1 2 2 IL-10 sTNFR1

18 5 AESD NSE AESD 7 NSE 13 6 NSE 85.4 40.6 ng/mL

19 4 HHV-6

HHV-6 IL-6IL-10sTNFR1 IL-6sTNFR1 IL-6sTNFR1 IL-6 IFN-IL-6IL-10sTNFR1

3

2016-.indb 18 2016/07/12 9:25:16

2 19

1 2009 ; 62 : 2483-252846

2 Nagase H, Nakagawa T, Aoki K, et al. Therapeutic indicators of acute encephalopathy in patients with complex febrileres. Pe-diatr Int 2013 ; 55 : 310-314.4

3 2010 ; 114 : 956-9604 4 c 2009 ; 61 : 245-2514

5 Tsukahara H, Fujii Y, Matsubara K, et al. Prognostic value of brain injury biomarkers in acute encephalitis/ encephalopathy. Pediatr Int 2013 ; 55 : 461-464.4

6 40 2011 ; 64 : 2215-22234 7

2012 ; 44 : 305-3094 8 2

2011 ; 10 : 9-134 9 2-microglobulin 2010 ; 42 : 233-2344

10 Morita H, Hosoya M, Kato A, Kawasaki Y, Suzuki H. Laboratory characteristics of acute encephalopathy with multiple organ dysfunctions. Brain Dev 2005 ; 27 : 477-482.4

11 Hosoya M, Nunoi H, Aoyama M, Kawasaki Y, Suzuki H. Cytochrome c and tumor necrosis factor-alpha values in serum and cerebrospinal fluid of patients with influenza-associated encephalopathy. Pediatr Infect Dis J 2005 ; 24 : 467-470.4

12 2006 ; 110 : 1550-15574

13 Hasegawa S, Matsushige T, Inoue H, et al. Serum and cerebrospinal fluid levels of visinin-like protein-1 in acute encephalopa-thy with biphasic seizures and late reduced diffusion. Brain Dev 2014 ; 36 : 608-612.4

14 Shiihara T, Miyake T, Izumi S, et al. Serum and cerebrospinal fluid S100B, neuron-specific enolase, and total tau protein in acute encephalopathy with biphasic seizures and late reduced diffusion : a diagnostic validity. Pediatr Int 2012 ; 54 : 52-55.

415 Hayashi N, Okumura A, Kubota T, et al. Prognostic factors in acute encephalopathy with reduced subcortical diffusion. Brain

Dev 2012 ; 34 : 632-639.416 Tanuma N, Miyata R, Kumada S, et al. The axonal damage marker tau protein in the cerebrospinal fluid is increased in patients

with acute encephalopathy with biphasic seizures and late reduced diffusion. Brain Dev 2010 ; 32 : 435-439.417 Ichiyama T, Suenaga N, Kajimoto M, et al. Serum and CSF levels of cytokines in acute encephalopathy following prolonged

febrile seizures. Brain Dev 2008 ; 30 : 47-52.418 neuron-specificenolase 2007 ; 60 : 1702-17065

19 Ichiyama T, Ito Y, Kubota M, Yamazaki T, Nakamura K, Furukawa S. Serum and cerebrospinal fluid levels of cytokines in acute encephalopathy associated with human herpesvirus-6 infection. Brain Dev 2009 ; 31 : 731-738.4

2016-.indb 19 2016/07/12 9:25:16

20

2

1.

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2016-.indb 20 2016/07/12 9:25:17

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2016-.indb 21 2016/07/12 9:25:17

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PubMedacute encephalopathytiab ORacute diseaseMeSH Terms AND brain diseasesMeSH TermsAND diagno-

sis, differentialMeSH TermsAND Sensitivity and SpecificityMesh9

/ALand /TH or /ALandPT and CK12325612131855

1 2009 ; 62 : 2483-2528.6

2 2000 ; 53 : 1739-1746.5 3 Takanashi J, Oba H, Barkovich AJ, et al. Diffusion MRI abnormalities after prolonged febrile seizures with encephalopathy.

Neurology 2006 ; 66 : 1304-1309.5 4 Mizuguchi M, Abe J, Mikkaichi K, et al. Acute necrotising encephalopathy of childhood : a new syndrome presenting with

multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry 1995 ; 58 : 555-561.5 5 Mizuguchi M, Yamanouchi H, Ichiyama T, Shiomi M. Acute encephalopathy associated with influenza and other viral infec-

tions. Acta Neurol Scand 2007 ; 1154 Suppl: 45-56.5

IICPT-II CoA II

I

-1,6-

Mizuguchi M, Yamanouchi H, Ichiyama T, Shiomi M. Acute encephalopathy associated with influen-za and other viral infections. Acta Neurol Scand 2007 ; 1154 Suppl: 45-56.

2

2016-.indb 22 2016/07/12 9:25:17

2 23

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2016-.indb 23 2016/07/12 9:25:17

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2016-.indb 24 2016/07/12 9:25:17

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2016-.indb 25 2016/07/12 9:25:18

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2016-.indb 26 2016/07/12 9:25:18

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2016-.indb 27 2016/07/12 9:25:18

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PubMed

PubMedacute encephalopathytiab ORacute diseaseMeSH Terms AND brain diseasesMeSH TermsANDneuroim-agingMeSH Terms OR neuroimagingAll FieldsANDultrasonographySubheading OR radionuclide imagingSubheadingOR radiographySubheadingFilters : English, Japanese, Child : birth-18 years84

/TH or /ALandPT and CK123256121318andSH93

1 35

1998 ; 30 : 244-249.5 2 2008 ; 40 : 117-121.6 3 2003 ; 35 : 1676-1680.6 4 Mizuguchi M, Yamanouchi H, Ichiyama T, Shiomi M. Acute encephalopathy associated with influenza and other viral infec-

tions. Acta Neurol Scand 2007 ; 115 : 45-56.6 5 Mizuguchi M. Acute necrotizing encephalopathy of childhood : a novel form of acute encephalopathy prevent in Japan and

Taiwan. Brain Dev 1997 ; 19 : 81-92.5

2016-.indb 28 2016/07/12 9:25:18

2 29

6 2004 ; 36 : 1129-1132.6 7 2007 : 310-319.6

8 Harada M, Hisaoka S, Mori K, Yoneda K, Noda S, Nishitani H. Differences in water diffusion and lactate production in two different types of postinfectious encephalopathy. J Magn Reson Imaging 2000 ; 11 : 559-563.5

9 Wong AM, Simon EM, Zimmerman RA, Wang HS, Toh CH, Ng SH. Acute nectrotizing encephalopathy of childhood : cor-relation of MR findings and clinical outcome. Am J Neuroradiol 2006 ; 27 : 1919-1923.5

10 Takanashi J, Oba H, Barkovich AJ, et al. Diffusion MRI abnormalities after prolonged febrile seizures with encephalopathy. Neurology 2006 ; 66 : 1304-1309.5

11 Takanashi J. Two newly proposed infectious encephalitis/encephalopathy syndromes. Brain Dev 2009 ; 31 : 521-528.6

12 Takanashi J, Tada H, Terada H, Barkovich AJ. Excitotoxicity in acute encephalopathy with biphasic seizures and late reduced diffusion. Report of 3 cases. AJNR Am J Neuroradiol 2009 ; 30 : 132-135.5

13 Yamanouchi H, Mizuguchi M. Acute infantile encephalopathy predominantly affecting the bilateral frontal lobesAIEF: a novel clinical category and its tentative diagnostic criteria. Epilepsy Res 2006 ; 70 : S263-268.5

14 Tada H, Takanashi J, Barkovich AJ, et al. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion. Neurol-ogy 2004 ; 63 : 1854-1858.5

15 Takanashi J, Imamura A, Hayakawa F, Terada H. Differences in the time course of splenial and white matter lesions in clinical-ly mild encephalitis/encephalopathy with a reversible splenial lesionMERSJ Neurol Sci 2010 ; 292 : 24-27.5

16 MERS 282011 : 184-187.6

a 2009 http://www.mhlw.go.jp/kinkyu/kenkou/influenza/hourei/2009/09/dl/info0925-01.pdf

2016-.indb 29 2016/07/12 9:25:18

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4

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subclinical seizures 215-1915, 16, 18, 191915, 19

electrical stormDay 1 Day 2Harden A, Boyd SG, Cole G, Levin M. EEG features and their evolution in the acute phase of haemorrhagic shock and encehalopathy syndrome. Neuropediatrics 1991 ; 22 : 194-197.

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2016-.indb 34 2016/07/12 9:25:19

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PubMedacute encephalopathytiab ORacute diseaseMeSH Terms AND brain diseasesMeSH TermsANDelectro-

encephalographyMeSH Terms OR Filters activatedEnglish, Japanese, Childbirth-18 years224acute encephalopathytiab ORacute diseaseMeSH Terms AND brain diseasesMeSH TermsANDaEEGAll

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/TH or /ALand /TH or /ALandPT and CK123256121318129 /TH or /ALandamplitude-integrated/ALand /TH or electroencephalography/AL5

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2016-.indb 47 2016/07/12 9:25:20

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2016-.indb 66 2016/07/12 9:25:22

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2016-.indb 92 2016/07/12 9:25:25

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2016-.indb 93 2016/07/12 9:25:26

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2016-.indb 98 2016/07/12 9:25:26

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PubMed

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a 2015 http://www.nanbyou.or.jp/entry/4515

b 2009 http://www.mhlw.go.jp/kinkyu/kenkou/influenza/hourei/2009/09/dl/info0925-01.pdf

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Saito Y, Maegaki Y, Okamoto R, et al. Acute encephalitis with refractory, repeti-

tive partial seizures : Case reports of this

unusual post-encephalitic epilepsy. Brain

Dev 2007 ; 29 : 147-156.

2

2016-.indb 103 2016/07/12 9:25:27

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2016-.indb 104 2016/07/12 9:25:27

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PubMed

PubMedacute encephalopathytiabORacute diseaseMeSH TermsAND brain diseasesMeSH TermsANDaerrps OR refractory OR repetitive OR partialAND seizureFiltersEnglishJapaneseChildbirth-18 years91

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a 2324 2013

2016-.indb 105 2016/07/12 9:25:27

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Dravet Sakauchi M, Oguni H, Kato I, et al. Retrospective multiinstitutional study of the preva-lence of early death in Dravet syndrome. Epilepsia 2011 ; 52 : 1144-1149.

1

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defOkumura A, Uematsu M, Imataka G, et al. Acute encephalopathy in children with Dravet syndrome. Epilepsia 2012 ; 53 : 79-86.

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2016-.indb 111 2016/07/12 9:25:28

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PubMed

PubMedacute encephalopathytiabORacute diseaseMeSH TermsAND brain diseasesMeSH TermsAND dravet Fil-

tersEnglishJapaneseChildbirth-18 years9

/ALand - /TH or - /ALor Dravet/ALandPT and CK1232561213184

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7 113

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PubMedacute encephalopathytiabORacute diseaseMeSH TermsAND brain diseasesMeSH TermsAND Adrenal In-sufficiency FiltersEnglishJapaneseChildbirth-18 years11

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1 2002 ; 50 : 1165-1169.4

2 2013 ; 45 : 390-393.6 3 2012 ; 2 : 918-924.6 4 Lee S, Sanefuji M, Hara T, et al. Clinical and MRI characteristic of acute encephalopathy in congenital adrenal hyperplasia. J

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a 2014

b 21-2014

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2016-.indb 115 2016/07/12 9:25:28

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PubMedacute encephalopathytiab OR acute diseaseMeSH Terms AND brain diseasesMeSH Terms AND reversible

splenial lesion OR MERS Filters : English ; Japanese ; Child : birth-18 years5

/AL and /AL or MERS/AL and PT and CK12325612131817

1 Garcia-Monco JC, Cortina IE, et al. Reversible splenial lesion syndrome RESLES: whats in a name? J Neuroimaging

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ly mild encephalitis/ encephalopathy with a reversible splenial lesion MERS. J Neurol Sci 2010 ; 292 : 24-27.5 8 MERS 282011 : 184-187.6

9 2013 ; 45 : 366-370.610 Hoshino A, Saitoh M, Oka A, et al. Epidemiology of acute encephalopathy in Japan, with emphasis on the association of virus-

es and syndrome. Brain Dev 2012 ; 3