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

SexOnsetInitial symptomsPorencephalic locationGenetic risk factorAge at final diagnosisOutcomeFamily history
M1 dayFocal seizuresL middle cerebral arteryF V Leiden2 yearsLennox+
/ Lp (a): 120 mg/dl
M6 weeksSeizuresL middle cerebral arteryF V Leiden4 yearsLennox
MBirthFocal seizuresR middle cerebral arteryProtein S type I4 weeksBlindness death+
free ag: 8%; total: 10%
FBirthHypotonia seizuresL middle cerebral arteryProtein C type IS yearsLennox
ac: 25%; ag 20%
M3 yearsMacrocephalyR middle cerebral arteryLp (a): 60 mg/dl16 yearsL hemiparesis
L hemiparesisR posterior artery
M8 weeksHypotonia seizuresR/L middle cerebral arteryLp (a): 70 mg/dl7 yearsTetraparesis/ retardation
M8 weeksSeizuresR middle cerebral arteryProtein C type I15 yearsL hemiparesis
ac: 40%; ag 45%
M8 weeksFocal seizuresL middle cerebral artery14 yearsL hemiparesis
M5 monthsSeizuresR middle cerebral arteryF V Leiden18 yearsL hemiparesis+
L hemiparesisLp (a): 145 mg/dl
MBirthSeizuresL middle cerebral arteryF V Leiden3 yearsR hemiparesis+
L posterior arteryProtein S type ISevere retardation
free ag: 15%, total: 20%
F2 yearsSeizuresL middle cerebral arteryProtein C type I4 yearsNo handicap
ac: 22%; ag: 30%
M4 weeksFocal seizuresL middle cerebral arteryF V Leiden8 yearsR hemiparesis
M6 weeksFocal seizuresL middle cerebral artery10 yearsR hemiparesis: mild
F3 monthsFocal seizuresL middle cerebral arteryLp (a): 90 mg/dl16 yearsR hemiparesis: mild
M4 monthsL hemiparesisR middle cerebral artery6 yearsL hemiparesis
Seizures
M4 monthsR hemiparesisL middle cerebral artery10 yearsR hemiparesis: mild
L posterior artery
F5 monthsTetraparesisR/L middle cerebral arteryF V Leiden5 yearsTetraparesis
SeizuresBlindness/retardation
F3 monthsFocal seizuresL middle cerebral arteryProtein C type I2 yearsR hemiparesis+
L anterior arteryac: 15%; ag: 22%Retardation
F8 weeksMacrocephaly L posterior artery13 yearsNo handicap
Seizures
FBirthFocal seizuresL middle cerebral arteryProtein C type I12 yearsR hemiparesis
ac: 51%; ag: 50%Retardation
M4 monthsR hemiparesisL middle cerebral artery4 yearsR hemiparesis
SeizuresRetardation
M3 monthsFocal seizuresL middle cerebral artery18 yearsR hemiparesis
Blindness
Retardation
FBirthFocal seizuresL middle cerebral arteryProtein C type I4 yearsR hemiparesis
ac: 22%; ag 20%
F4 monthsSeizuresL middle cerebral artery14 yearsR hemiparesis
R hemiparesisRetardation
  • M=male; F=female; L=left; R=right; Lp (a)=lipoprotein (a); ac=activity; ag: antigen.