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 |