Table 1
Sex, age at onset, initial symptoms, cystic location, genetic risk factors, age at final diagnosis, outcome and family history in children with porencephaly
| Sex | Onset | Initial symptoms | Porencephalic location | Genetic risk factor | Age at final diagnosis | Outcome | Family history |
|---|---|---|---|---|---|---|---|
| M | 1 day | Focal seizures | L middle cerebral artery | F V Leiden | 2 years | Lennox | + |
| / Lp (a): 120 mg/dl | |||||||
| M | 6 weeks | Seizures | L middle cerebral artery | F V Leiden | 4 years | Lennox | − |
| M | Birth | Focal seizures | R middle cerebral artery | Protein S type I | 4 weeks | Blindness death | + |
| free ag: 8%; total: 10% | |||||||
| F | Birth | Hypotonia seizures | L middle cerebral artery | Protein C type I | S years | Lennox | − |
| ac: 25%; ag 20% | |||||||
| M | 3 years | Macrocephaly | R middle cerebral artery | Lp (a): 60 mg/dl | 16 years | L hemiparesis | − |
| L hemiparesis | R posterior artery | ||||||
| M | 8 weeks | Hypotonia seizures | R/L middle cerebral artery | Lp (a): 70 mg/dl | 7 years | Tetraparesis/ retardation | − |
| M | 8 weeks | Seizures | R middle cerebral artery | Protein C type I | 15 years | L hemiparesis | − |
| ac: 40%; ag 45% | |||||||
| M | 8 weeks | Focal seizures | L middle cerebral artery | 14 years | L hemiparesis | − | |
| M | 5 months | Seizures | R middle cerebral artery | F V Leiden | 18 years | L hemiparesis | + |
| L hemiparesis | Lp (a): 145 mg/dl | ||||||
| M | Birth | Seizures | L middle cerebral artery | F V Leiden | 3 years | R hemiparesis | + |
| L posterior artery | Protein S type I | Severe retardation | |||||
| free ag: 15%, total: 20% | |||||||
| F | 2 years | Seizures | L middle cerebral artery | Protein C type I | 4 years | No handicap | − |
| ac: 22%; ag: 30% | |||||||
| M | 4 weeks | Focal seizures | L middle cerebral artery | F V Leiden | 8 years | R hemiparesis | − |
| M | 6 weeks | Focal seizures | L middle cerebral artery | 10 years | R hemiparesis: mild | − | |
| F | 3 months | Focal seizures | L middle cerebral artery | Lp (a): 90 mg/dl | 16 years | R hemiparesis: mild | − |
| M | 4 months | L hemiparesis | R middle cerebral artery | 6 years | L hemiparesis | − | |
| Seizures | |||||||
| M | 4 months | R hemiparesis | L middle cerebral artery | 10 years | R hemiparesis: mild | − | |
| L posterior artery | |||||||
| F | 5 months | Tetraparesis | R/L middle cerebral artery | F V Leiden | 5 years | Tetraparesis | − |
| Seizures | Blindness/retardation | ||||||
| F | 3 months | Focal seizures | L middle cerebral artery | Protein C type I | 2 years | R hemiparesis | + |
| L anterior artery | ac: 15%; ag: 22% | Retardation | |||||
| F | 8 weeks | Macrocephaly | L posterior artery | 13 years | No handicap | − | |
| Seizures | |||||||
| F | Birth | Focal seizures | L middle cerebral artery | Protein C type I | 12 years | R hemiparesis | − |
| ac: 51%; ag: 50% | Retardation | ||||||
| M | 4 months | R hemiparesis | L middle cerebral artery | 4 years | R hemiparesis | − | |
| Seizures | Retardation | ||||||
| M | 3 months | Focal seizures | L middle cerebral artery | 18 years | R hemiparesis | − | |
| Blindness | |||||||
| Retardation | |||||||
| F | Birth | Focal seizures | L middle cerebral artery | Protein C type I | 4 years | R hemiparesis | − |
| ac: 22%; ag 20% | |||||||
| F | 4 months | Seizures | L middle cerebral artery | 14 years | R hemiparesis | − | |
| R hemiparesis | Retardation |
-
M=male; F=female; L=left; R=right; Lp (a)=lipoprotein (a); ac=activity; ag: antigen.
