Complaint :
45 days female baby suffering with fever & intractable
convulsions from last 4 days.
Clinical
futures & lab :
systemic examination is normal.CSF analysis is normal.
Findings :
Sulci are absent with smooth cortical
surface. Sylvian fissures are straight oblique resulting
a figure of eight appearance of brain.
Colpocephaly +.
Diagnosis :
Lissencephaly.
Discussion
:
Lissencephaly or smooth brain refer to brain with absent or poor
sulcation. It is physiological at 16-17 weeks of intrauterine life.
Intrauterine infections can also results in asmooth, Lissencephalic
-appearing brain.
Lissencephaly is 3 types.
Type 1
shows colpocephaly
and a thickened cortex with broad, flat gyri, smooth white- gray
matter interface, and straight oblique or shallow sylvian
fissures,giving a figure of eight appearance. Parenchymal calcifications
can be present if Lissencephaly is due
intrauterine infections.
Lissencephaly
type 11 shows
agyric, severely disorganized unlayered thick cortex with poor cortico -
medullary demarcation.
On MRI ,cortex is thick with poly microgyric and hypo myelination.
Lissencephaly
type 111 shows
microcephaly ,thick cortex, enlarged ventricles and hypoplastic
cerebellum
& brain stem.
Reference:
Diagnostic neuro-radiology by ANNE G. OSBORN.
