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Table 2 Clinical manifestations of acute encephalopathy

From: Acute encephalopathy in children with tuberous sclerosis complex

Case

1

2

3

4

5

6

7

8

9

10

11

Fever to AE (h)

23

3

0

4

24

10

6

8

10

4

3

Duration of the initial seizure (min)

30

70

80

60

60

90

115

105

30

50

80

Pathogen (site of detection)

ND

ND

HHV-6 (blood)

HHV-7 (blood)

ND

ND

ND

HHV-6

(blood)

Influenza

(nasal swab)

ND

ND

Laboratory data on presentation

          

 AST (IU/L)

47

47

62

85

46

48

44

30

38

53

161

 ALT (IU/L)

13

28

20

25

16

19

12

28

11

20

33

 CK (IU/L)

109

135

184

315

78

148

57

85

70

109

227

 LD (IU/L)

463

262

319

435

309

299

409

352

296

377

812

 BUN (mg/dL)

13.3

14

11.5

8.2

14.4

17.2

18.9

13.6

12

14.3

16.3

 Creatinine (mg/dL)

0.32

0.44

0.39

0.33

0.4

0.3

0.44

0.21

0.45

0.51

0.28

 Glucose (mg/dL)

232

303

358

219

248

ND

286

329

ND

ND

ND

 Ammonia (μg/dL)

ND

256

343

434

320

147

243

103

152

ND

ND

 CSF cell count (/µL)

1

ND

ND

ND

2

ND

0

ND

3

ND

ND

 CSF protein (mg/dL)

18

ND

ND

ND

10

ND

19

ND

12

ND

ND

MRI-reduced water diffusion

Cx, SWM, and Th

Cx, SWM, BG, and Th

Cx and SWM

Cx and SWM

Cx and BG

Cx, SWM, Th, and CC

Cx, SWM, and CC

Cx and SWM

Cx, SWM, and BG

Cx and SWM

Cx and SWM

Treatment

           

 Artificial ventilation

Yes

Yes

Yes

Yes

Yes

Yes

Yes

Yes

Yes

Yes

Yes

 Steroid pulse therapy

Yes

No

No

Yes

Yes

Yes

Yes

Yes

No

Yes

Yes

 IVIG

No

No

No

Yes

No

Yes

No

No

No

No

Yes

 Hypothermia

No

No

No

Yes

No

No

No

No

Yes

Yes

No

 Use catecholamine

No

No

No

No

No

No

No

No

No

No

No

Outcome

Death

Severe sequelae

Severe sequelae

Severe sequelae

Severe sequelae

Severe sequelae

Mild sequelae

Severe sequelae

Mild sequelae

Severe sequelae

Mild sequelae

  1. AE acute encephalopathy, ND not determined, HHV human herpesvirus, AST Aspartate aminotransferase, ALT Alanine aminotransferase, CK creatine kinase, LD lactate dehydrogenase, BUN blood urea nitrogen, Cx cortex, SWM subcortical white matter, BG basal ganglia, Th thalamus, CC corpus callosum, IVIG intravenous immunoglobulin