M. AMOR, S. MAJDOUB, B. BEN SALAH, M. DHIFALLAH, H. ZAGHOUANI, T. RZIGA, H. AMARA, D. - PowerPoint Presentation

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M. AMOR, S. MAJDOUB, B. BEN SALAH, M. DHIFALLAH, H. ZAGHOUANI, T. RZIGA, H. AMARA, D. BAKIR, C. KRAIEM Radiology service, University Hospital Farhat Hached Sousse, Tunisia

MR IMAGING OF PACHYGYRIA: A CASE REPORT AND LITERATURE REVIEW

PED11

OBJECTIVESMRI manifestations of pachygyria

are presented with a review of the pathological features of the disorder and an evaluation of the diagnostic value of imaging modalities.

Materials and methodsWe reported a case of

pachygyria brain in a 1 month-old girl with recurrent convulsive

seizures and axial

hypotonia

.

T

ransfontanellar

sound and

brain MRI using

axial FLAIR, diffusion,T2-weighted and coronal, sagittal T1-weighted, and

MPR 3D sequences with

multiplanar

reformations was performed

.

RESULTS

Transfontanellar ultrasound showed signs of leukodystrophy.MR

imaging demonstrate patterns of

pachygyria

, including:

Smooth and markedly thickened cortex Broad, widespread flat

gyri

with shallow sulci Vertically-oriented and shallow

Sylvian

fissures and the white matter is reduced in

proportion (figure 1, 2, 3 and 4)

Figura 1

: axial FLAIR

sequences

Figure 2:

axial T2-weighed

sequences

RESULTSAbsence of leukodytrophy

, areas of myelination in the cerebellum, pons

,

posterior

limb

of the

internal

capsule and thalamus

with

high

signal on FLAIR-

weighted

MR images (figure 1)

Mega

cisterna

magna (figure 3)

Figure 3

: sagittal T1-weighned

Figure 4

: coronal T1-weighned

DISCUSSIONAdvances in neuroimaging techniques have resulted in an increasing recognition of the cortical lesions as the etiology of a number of epilepsies that were previously defined as

cryptogenic.Agyria-pachygyria is a disease of neuronal migration characterized by the total or partial absence of sulci and a thickened cortical mantle.

DISCUSSIONIn many cases,

agyria and pachygyria coexist in the same patient. They represent degrees of the same fundamental disorders in cell migration and cortical organization.

More

and more patients have been documented as

agyria-pachygyria

since the advent

of CT scan

and

MRI.

Although some of these disorders have a genetic basis, the cause of the

migrational

arrest is still unknown.

DISCUSSION Pachygyria is considered the less severe form and exhibits a broader spectrum of clinical features than

agyria.It is a rare developmental disorder resulting from the abnormal migration of neurons in the developing brain and nervous system.

the

gyri

are relatively few are are usually broad and flat. The condition is also known as "incomplete

lissencephaly

".

It is a milder variant of

lissencephaly

, and part of the

lissencephaly

spectrum of

disorders.

DISCUSSIONMRI is commonly used for diagnosis. It provides a high contrast image for better delineation of white and gray matter

. Pachygyria shows on an MRI as thickened cerebral cortices with few and large gyri

and incomplete development of

Sylvian

fissures

.

The prognosis for children with

pachygyria

varies depending on the degree of brain abnormality and subsequent neurological signs and symptoms.

CONCLUSIONPachygyria is

a neuronal migration disorder characterized by reduced and

broad

cerebral

gyri

. The

regions

of the

brain

with

pachygyria

have an

abnormally

thick

cortex

that

lacks

normal

folding

and has

deficient

layering

the onset and severity depending on the severity of the cortical malformation

.

Infantile spasms are common in affected children, as is intractable epilepsy.

Neuroblast

migration disorders are better known, mainly due to high resolution MRI techniques that by the quality of

multiplanar

study and a better differentiation between gray and white matter, allow a more precise diagnosis.