Midline interhemispheric variant of holoprosencephaly

Orpha code: 93926OMIM code: 609637

Definition

Midline interhemispheric variant of holoprosencephaly (MIH) or syntelencephaly is a form of holoprosencephaly (HPE; see this term) characterized by non-separation of the posterior frontal and parietal lobes, normally-formed callosal genu and splenium, absence of the callosal body, normally-separated hypothalamus and lentiform nucleus, and frequent heterotopic gray matter.

Disease data
Classification

Clinical subtype

Synonyms
MIH
MIH
MIH typu HPE
MIHF
MIHV
Pośrodkowy międzypółkulowy wariant holoprozencefalii
Syntelencefalia
MIH type HPE
MIHF
MIHV
Middle interhemispheric fusion variant
Middle interhemispheric variant of holoprosencephaly
Syntelencephaly
ORPHA code
93926
OMIM code
609637
ICD10 code
Q04.2
ICD11 code
-

No additional description.

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