Table 4. Analysis of General Characteristics by Subgroup and Clinical Status of Mitochondrial Disease at the Last Visit.
Characteristics | History of West syndrome (n=13) | No history of West syndrome (n=27) | p value |
---|---|---|---|
Sex (male:female) | 6 (46.2):7 (53.8) | 11 (40.7):16 (59.3) | 0.746 |
Birth history | |||
Prematurity (<37 wks) | 0 (0) | 1 (3.7) | 0.675 |
SGA | 1 (7.7) | 5 (18.5) | 0.351 |
Prenatal asphyxia | 0 (0) | 3 (11.1) | 0.296 |
HIE | 1 (7.7) | 1 (3.7) | 0.550 |
Neonatal seizure | 3 (23.1) | 0 (0) | 0.029 |
Family history | |||
None | 12 (92.3) | 23 (85.2) | 0.469 |
Epilepsy | 1 (7.7) | 2 (7.4) | 0.704 |
Mitochondrial disease | 0 (0) | 1 (3.7) | 0.675 |
Neurologic disorder | 0 (0) | 1 (3.7) | 0.675 |
First presenting symptoms | |||
Seizure | 10 (76.9) | 10 (37) | 0.018 |
Delayed development | 3 (23.1) | 13 (48.1) | 0.130 |
Ataxia | 0 (0) | 1 (3.7) | 0.675 |
Hemiparesis | 0 (0) | 1 (3.7) | 0.675 |
Perinatal asphyxia | 0 (0) | 1 (3.7) | 0.675 |
LOC | 0 (0) | 1 (3.7) | 0.675 |
Age of first symptoms (yr) | 0.6±0.4 | 1.6±0.9 | 0.003 |
Age of first seizure (yr) | 0.9±0.7 | 3.9±3.1 | <0.001 |
Follow-up duration (yr) | |||
From first symptom to last visit | 9.5±3.7 | 11.7±5.9 | 0.224 |
From first seizure to last visit | 9.2±3.9 | 9.6±4.8 | 0.786 |
From LGS diagnosis to last visit | 7.5±3.4 | 2.9±4.6 | 0.806 |
Organ involvement | |||
Neuromuscular system | 13 (100) | 27 (100) | - |
Gastrointestinal system | 5 (38.5) | 5 (18.5) | 0.165 |
Respiratory system | 2 (15.4) | 2 (7.4) | 0.392 |
Cardiologic system | 1 (7.7) | 1 (3.7) | 0.550 |
Eye | 2 (15.4) | 0 (0) | 0.100 |
Renal system | 1 (7.7) | 0 (0) | 0.325 |
Endocrinologic system | 1 (7.7) | 0 (0) | 0.325 |
Hematologic system | 1 (7.7) | 0 (0) | 0.325 |
Psy | 0 (0) | 1 (3.7) | 0.325 |
Clinical severity | |||
Functional state | |||
Mild | 0 (0) | 5 (18.5) | 0.123 |
Moderate | 3 (23.1) | 7 (25.9) | 0.586 |
Severe | 8 (61.5) | 12 (44.4) | 0.311 |
Expire | 2 (15.4) | 3 (11.1) | 0.531 |
Respiration | |||
Oxygen dependency | 0 (0) | 1 (3.7) | 0.675 |
Tracheostomy | 1 (7.7) | 3 (11.1) | 0.608 |
Home ventilator | 0 (0) | 1 (3.7) | 0.675 |
Enteral feeding | |||
Yes | 7 (53.8) | 8 (29.6) | 0.129 |
No | 6 (46.2) | 19 (70.4) | 0.129 |
SGA, small for gestational age (<2500 gm); HIE, hypoxic ischemic encephalopathy; LOC, loss of consciousness; LGS, Lennox-Gastaut syndrome.
Data are given as mean±standard deviation or number (percentage).