Code | Age of death (GW) | Corrected age of death | Gender | PMI | Seizure (Y/N) | Seizure interval | GCD (Y/N) | Diagnoses | |||
---|---|---|---|---|---|---|---|---|---|---|---|
TT | DA | CNS malformation/anomaly | Other (CNS) | Non-CNS conditions | |||||||
SZ-1a | 7 days | 7 days | M | 12 h | Y | < 24 h | Y | Y | encephalopathy, seizures | – | Proteus mirabilis septic shock, coagulopathy and acidosis, hypoxic respiratory failure |
SZ-2a | 6 years | 6 years | F | 10 h | Y | 6 years; intractable | N | N | global developmental delay, chronic seizure disorder with static encephalopathy | hypoplastic cerebellum | recurrent pulmonary infections |
SZ-3a | 16 years | 16 years | F | 22 h | Y | 13 years | N | Y | severe congenital neuromuscular disorder, complex status epilepticus | severe gliosis and neuronal loss | acute bronchopneumonia, cardiac arrest |
SZ-4b | 6 years | 6 years | M | not known | Y | > 5 years; multiple episodes till death, intractable | Y | Y | brain overgrowth with polymicrogyria, diffused cortical dysplasia, AKT3 R465W mutation, possibly SUDEP | gliosis, mild ventriculomegaly | congenital diaphragmatic hernia, coagulopathy, defects in liver, spleen and kidney |
SZ-5a | 3 years | 3 years | M | 2 h | Y | 7 weeks; multiple episodes till death, intractable | Y | N | subclinical status epilepticus | cerebral edema, HIE, anoxic brain injury secondary to pulmonary arrest | pulmonary arrest |
SZ-6a | 2 days | 2 days | F | 21.5 h | Y | 2 days (possible seizures) | Y | Y | – | cerebral edema, HIE | acute chorioamnionitis with funisitis and three vessel umbilical vasculitis |
SZ-7a | 4 days | 4 days | M | 38 h | Y | < 24 h | N | Y | – | HIE | severe acidosis, cardiorespiratory failure, visceral anomalies |
SZ-8a | 18mo | 18mo | M | 26 h | Y | 1–3 months of age; no further seizures post-treatment of phenobarbital | Y | Y | developmental delay and seizure disorder (2q21.1 duplication) | focal neuronal loss and gliosis in hippocampus | abdominal distension, brady-cardiac arrest, acute pan-lobar pneumonia |
SZ-9a | 2 years | 2 years | M | 72 h | Y | 4 months; multiple episodes between first seizure and death | N | N | retinoblastoma with diffuse leptomeningeal spread and direct infiltration of brain and spinal cord | – | pulmonary edema, congestive hepatomegaly |
SZ-10a | 5 weeks (28GW) | 33GW | F | 17.5 h | Y | < 24 h | Y | Y | multiple seizure events | hemorrhage and necrosis secondary to dural venous thrombosis. | necrotizing enterocolitis |
SZ-11a | 12 years | 12 years | F | 96 h | Y | 16 months; intractable | N | N | – | cerebral edema, HIE and brain death | appendicitis |
SZ-12a | 19 years | 19 years | M | 216 h | Y | 14 years; intractable | N | N | seizure disorder | – | Trisomy 16p/monosomy 9p, respiratory distress syndrome, Pseudomonas pneumonia, cardiomegaly |
SZ-13a | 7 weeks (32 4/7 GW) | 39 4/7 GW | F | 14 h | Y | 6 weeks (possible seizures) | N | Y | epilepsy (focal apoptotic neurons within DG) | – | severe pulmonary hypertension, veno-occlusive disease |
SZ-14a | 8 years | 8 years | F | 65 h | Y | 2 years (sleep EEG done) | N | N | encephalomalacia, hydrocephalus, seizures, developmental delay | – | unbalanced chromosomal translocation, congenital mitral valve stenosis, heart failure |
SZ-15a | 7 weeks | 7 weeks | F | 3 h | Y | 6 weeks | N | Y | hypotonia and episodic breathing progressing to seizures | elevated CSF and plasma glycine levels | – |
SZ-16a | 17 years | 17 years | F | 96 h | Y | 5 days | N | N | generalized tonic-clonic seizure, brain herniation | acute hemorrhage, edema | Type 1 diabetes, oligoarticular juvenile arthritis, celiac disease |
SZ-17a | 17 years | 17 years | M | 64 h | Y | ~ 17 years; intractable | N | Y | spastic quadriplegia, epilepsy, static leukoencephalopathy, ventriculomegaly | white matter gliosis | acute kidney injury, obstructive apnea, hypotonia |
SZ-18a | 8 days | 8 days | M | 42 h | Y | 8 days (onset at birth) | N | N | seizures (treated with antiepileptics) | brain herniation, diffuse cerebral edema | ornithine transcarbamylase deficiency, hyperammonemia, hepatosplenomegaly |
SZ-19a | 3 years | 3 years | F | 15.6 h | Y | 2.5 years | N | N | severe craniofacial malformations, seizure history | neurological injury, meningitis | – |
SZ-20a | 4 years | 4 years | M | 15.5 h | Y | 16 months | N | N | seizures (no recurrence post-treatment with medications), global developmental delay | subacute diffuse CNS hemorrhagic necrosis with massive intraventricular blood clot | multiple chromosomal abnormalities and associated chronic health problems, atypical lymphoid hyperplasia, concerning primary or secondary immunodeficiency |
SZ-21a | 9 years | 9 years | M | 2 h | Y | 2 months | N | N | refractory status epilepticus secondary to febrile infection-related status epilepticus, multiple events | diffuse severe gliosis, patchy neuronal loss, dramatic loss of CA1 neurons | – |