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Table 1 Main characteristics of the reported cases of hemihydranencephaly

From: Hemihydranencephaly: living with half brain dysfunction

Authors Sex Familiarity Perinatal period Age at diagnosis Age of onset Clinical signs Radiological Findings Affected Side Annotations
Motor Impairment Mental Retardat. Seizures Language C.C. Ocular Signs
Warkany, 1971 F NR NR 3½YY NR NR NR NR NR NR NR NO L Reported by Warkany
Moser 1981 NR N N NR 7 MM Right mild NO NR NR NR NR Absence left hemisphere L Shift towards middle; EEG: absence of left electrocerebral activity
Van Doormik, 1992 F NO N 5 YY 4 MM Left Facial paralysis IQ 69 NO Delayed NR Strabismus,abducent paresis Right side HHE R EEG: absence of electrocerebral activity on the right side. Reduced capacity of non verbal IQ and expressive language
No middle right cerebral artery
Greco et al., 2001 M NO Prematurity respiratory distress 4MM Neonatal Right IQ 45 NO Delayed M NR Left side HHE L Hydrocephaly. EEG: absence of left electrocerebral activity
IQ 45, low cognitive potential
Ulmer et al., 2005 M NO N 36 YY Early Childhood Right hemiparesis N One seizure 28 Years N NR NR Left side HHE L Mirror movements
Left internal carotid artery   Motor acuity test : fine motor impairment
Aachenen aphasia test: normal
Altshuler et al., 2005 M NO N NR 3½ MM Generalized hypertonia, more on the right NR NR NR NR NR Absence left hemisphere L -
Balpande M et al., 2009 M NO N NR 13 YY Left N NO N NR - Right side HHE R Diffuse atrophy, facial weakness
Absence of internal right cerebral artery
Hassanein et al., 2011 F NR NR 27 MM NR Left Delayed mental milestones NO Delayed ≥ 3 C Optic atrophy Nystagmus, bilateral blindness Right side HHE R Diabetes insipidus.
Right middle and anterior cerebral artery occlusion   Protein S deficiency
Diffuse tensor MR: reduced
Dias LS et al., 2011 M NO N 21 YY 3 MM Left Severe GTCS Delayed 1,5 y NR Left asymmetric pupil Right side HHE R -
         Status epilepticus     Internal carotid occlusion   
  1. Legend: HHE: hemihydranencephaly; NR: not reported; NP: not performed; N: normal; YY: years; MM: months; M: macrocephaly.