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 | |||||||||||||