DISORDERS OF DIVERTICULATION AND CLEAVAGE, SULCATION AND CELLULAR MIGRATION

HOLOPROSENCEPHALY
COMPLETE OR PARTIAL FAILURE IN DIVISION OF DEVELOPING CEREBRUM INTO HEMISPHERES AND LOBES

HOLOPROSENCEPHALY

HOLOPROSENCEPHALY
ALOBAR SEMILOBAR LOBAR

ALOBAR HOLOPROSENCEPHALY
COMPLETE LACK OF VENTRICULAR AND HEMISPHERIC CLEAVAGE CENTRAL MONOVENTRICLE ABSENT CORPUS CALLOSUM -FALX CEREBRI INTERHEMISPHERIC FISSURRE THALAMI &BASAL GANGLIA -FUSED LARGE POSTERIOR MIDLINE CYST IS OFTEN PRESENT

 View of the brain demonstrating the holoprosencephaly

V i e w s

 ASSOSCIATED ABNORMALTIES SEVERE CRANIOFACIAL ANOMALIESCYCLOPS Cebocephaly (hypotelorism, single-nostril nose) ethmocephaly (hypotelorism, interorbital proboscis) OTHER ARE POLYDACTYLY RENAL DYSPLASIA ,OMPHALOCELE &HYDROPS

SEMILOBAR HOLOPROSENCEPHALY Intermediate in severity Partial interrupted attempt at brain diverticulation H shaped monoventricle with partially dev occipital and temporal horns Rudimentary falx cerebri

 Incompletely formed interhemisphric fissure Partial or complete fusion of basal ganglia Associated anomalies absent or are milder hypotelorism ,median and lateral cleft lip

LOBAR HOLOPROSENCEPHALY
NEARLY COMPLETE BRAIN CLEAVAGE ABSENT SEPTUM PELLUCIDUM-SQUARED OFF OR BOXLIKE CONFIGURATION TO FRONTAL HORNS NEARLY COMPLETE FORMED INTERHEMISPHERIC FISSURE AND FALX CEREBRI MOST ANTERO INFERIOR ASPECT MAY BE ABSENT AND FRONTAL LOBES FUSED ANTERIORLY ACROSS MIDLINE

 SEPARATION OF BASAL GANGLIA ABSENCE OF ANTERIOR OF CORPUS CALLOSUM AZYGOUS ACA MAY BE PRESENT

Associated anomalies;OPTIC

VESICLES AND OLFACTORY BULBS MAY BE HYPOPLASTIC

 PARTIAL FUSION OF FRONTAL LOBES INFERIORLY

SEPTOOPTIC DYSPLASIA( de MORSIER SYNDROME)

VERY MILD FORM OF OF LOBAR HPE ABSENCE OF SEPTUM PELLUCIDUM AND OPTIC NERVE HYPOPLASIA ARE BASIC COMPONENT SQUARED OFF FRONTAL HORNS

 TWO ANATOMIC SUBSETS-ONE; ASS WITH SCHIZENCEPHALY ,WHITE MATTER OF OPTIC RADIATIONS NORMAL SECOND; NO SCHIZENCEPHALY, DIFFUSE WHITE MATTER HYPOPLASIA WITH VENTRICULOMEGALY

ARHINENCEPHALY
OLFACTORY BULBS AND TRACTS ARE ABSENT CORONAL MRI SHOW OLFACTORY SULCI BUT NO OLFACTORY BULB OR TRACTS USUALLY PART OF COMPLX CEREBRAL AND SOMATIC MALFORMATIONS

LISSENCEPHALY
BRAIN WITH POOR OR ABSENT SULCATION CAN BE COMPLETE (AGYRIA) OR INCOMPLETE THREE TYPES I-COLPOCEPHALY, THICKENED CORTEX,STRAIGHT OBLIQUE OR SHALLOW SYLVIAN FISSURE (FIGURE EIGHT APPEARANCE)

Sulci are absent with smooth cortical surface. Sylvian fissures are straight oblique resulting a figure of eight appearance of brain. Colpocephaly +.

II-AGYRIA WITH SEVERLY DISORGANIZED UNLAYERED CORTEXWITH POOR CORTICOMEDULLARY DEMARCATION III-CEREBROCEREBELLAR TYPE MICROCEPHALY MODRATELY THICK CORTICES ENLARGED VENTRICLES AND HYPOPLASTIC CEREBELLUM AND BRAIN STEM

Axial and sagittal PD/T2WI show uniformly thickened cortex with only minimal sulcation of the frontal lobes. The sylvian fissures are widened. Parietooccipital sulcation is absent.

HETEROTOPIAS
COLLECTIONS OF OTHRWISE NORMAL NEURONS IN ABNORMAL LOCATIONS SECONDARY TO ARREST OF NEURONAL MIGRATION LAMINAR DIFFUSE ARREST ALAYER OF NEURONS IS INTERPOSED BETWEEN VENTRICLE AND CORTX ALTERNATING LAYERS OF GREY AND WHITE MATTER ARE SEEN

 NODULAR-CAN EITHER BE DIFFUSE OR FOCAL

SCHIZENCEPHALY
IT IS AGRAY MATTER LINED CSF FILLED CLEFT THAT EXTENTS FROM THE EPENDYMAL SURFACE OF BRAIN THROUGH THE WHITE MATTER TO THE PIA TYPE I-CLOSED LIP CLEFT WALLS ARE IN APPOSITION TYPE II-OPEN LIP WALLS ARE SEPARATED

UNILATERAL MEGALENCEPHALY
HAMARTOMATOUS OVERGROWTH OF PART OR ALL OF ONE CEREBRAL HEMISPHERE ASSC WITH LOCALIZED NEURONAL MIGRATIONAL ANAMOLIES HEMISPHERE ENLARGED ,IPSILATERAL VENTRICLE ENLARGED AND HETEROTOPIC GRAY MATTER WITH ABNORMAL SULCATION

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