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Anatomic classification of HPE -Different levels of severity [1] [6] |
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| ALOBAR (complete) |
Small single forebrain ventricle No interhemispheric division Absence of olfactory bulbs and tracts Absence of corpus callosum Non separation of deep gray nuclei |
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| SEMI-LOBAR |
Rudimentary cerebral lobes Incomplete interhemispheric division Absence or hypoplasia of olfactory bulbs and tracts Absence of corpus callosum Varying non separation of deep gray nuclei |
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| LOBAR |
Fully-developed cerebral lobes Distinct interhemispheric division Midline continuous frontal neocortex Absent, hypoplasic or normal corpus callosum Separation of deep gray nuclei |
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| MIHF |
Failure of separation of the posterior frontal and parietal lobes Callosal genu and splenium normally formed Absence of corpus callosum Hypothalamus and lentiform nuclei normally separated Heterotopic gray matter |
Dubourg et al. Orphanet Journal of Rare Diseases 2007 2:8 doi:10.1186/1750-1172-2-8 |
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