a case of cerebral hemi atrophy - dyke davidoff masson syndrome (ddms) by dr.k.prasanna resident...
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A CASE OF CEREBRAL HEMI ATROPHY - DYKE DAVIDOFF MASSON SYNDROME
(DDMS)
ByDr.K.PRASANNA
RESIDENTRAJAH MUTHIAH MEDICAL COLLEGE &
HOSPITAL, CHIDAMBARAM.
E-POSTER FOR IRIA 2014,CHENNAI
CLINICAL HISTORY• 7 year old female child presented with complaints of 7
episodes of seizures for the past 3 years for which she has
been treated with anti epileptics in nearby PHC.
• History of fever was associated with seizures in 2 episodes.
• On examination there is weakness in the right upper and
lower limbs. Routine laboratory evaluation, including a
complete blood count, urine analysis, and blood chemistry
testing, were all within normal limits.
MRI BRAIN• MRI brain shows evidence of
atrophy of unilateral
cerebral hemisphere on the
left side with associated
ipsilateral white matter
gliosis, minimal atrophy of
internal capsule & lateral
ventral dilatation.
• There is associated widened
sylvian fissure, thickened
calvarium, and dilated
mastoid air cells and frontal
sinuses
MRI BRAIN
• MRA shows normal
patency of ICA on both
sides
• But there is reduced
calibre of M2 and M3
segment of left MCA on
comparing with Right
MCA
MRI BRAIN
CEREBRAL HEMI ATROPHY• Cerebral hemi atrophy is an uncommon clinical entity.
• Its aetiologies can generally be grouped into congenital and acquired.
• The congenital type is intrauterine in origin while the acquired type
occurs early in life, usually before two year of life.
• CT and MRI play key diagnostic role in paediatric neurology. MRI is the
preferred modality in assessment of the aetiology and lesion extent of
cerebral parenchyma in atrophy, seizures, hemiparesis, and
craniofacial asymmetry.
DYKE-DAVIDOFF MASSON SYNDROME (DDMS)
• In 1933, Dyke, Davidoff, and Masson described the plain skull
radiographic and pneumatoencephalocele – graphic changes in their
series of 9 patients whose clinical characteristics included
hemiparesis, seizure s, facial asymmetry, and mental retardation.
• Dyke David of Masson Syndrome occur in intrauterine life when the
maturation of calvarium has not been completed, or due to brain
damage (usually traumatic) occurring in first 3 years of life.
DYKE-DAVIDOFF MASSON SYNDROME (DDMS)
• In congenital hemiatrophy, when the insult occurs in utero, there
is shift of midline structures towards the side of the disease and
the sulcal prominence replacing the gliotic tissue is absent.
• This feature differentiates it from cerebral hemiatrophy which
occurs in early life.
• The etiological factor for Dyke-Davidoff-Masson syndrome has been
postulated as trauma, inflammation or vascular malformations and
occlusions.
• When the insult occurs in-utero, it could be due to gestational
vascular occlusion, primarily involving the middle cerebral vascular
territory.
• A possible etiological relation of cerebral hemiatrophy and seizures
has been reported by different studies in India.
DYKE-DAVIDOFF MASSON SYNDROME (DDMS)
• The hemiatrophic cerebral parenchyma will have prominent sulci if the
vascular insult occurs after birth or after sulcation is complete.
• On the other hand, if the vascular ischaemia occurs during
embryogenesis, when the formation of gyri and sulci is incomplete, no
prominent sulci will be present.
• Encephalomalacia, gliosis, porencephaly, loss of white and gray matter
substance, hypoplastic cerebral peduncle, thalamus and internal
capsule, ventricular enlargement and midline shift toward the atrophic
side may also be present in the hemiatrophic brain.
DYKE-DAVIDOFF MASSON SYNDROME (DDMS)
• The compensatory skull changes reflect adaptations to the
unilateral decrease of brain substance and consist of
– ipsilateral calvarial thickening (diploic space and inner table) with loss of
convolutional markings of the inner table of the skull,
– overdevelopment of the paranasal sinuses (mainly frontal) and mastoid
air cells
– elevation of the petrous ridge, sphenoid wing and orbital roof,
– diminished size of the middle/anterior cranial fossae and displacement
of falx attachment.
DYKE-DAVIDOFF MASSON SYNDROME (DDMS)
• Dyke Davidoff Masson Syndrome should be differentiated from Basal
cell germinoma, Sturge Weber syndrome, Linear Nevus syndrome,
Fishman syndrome, Silver- Russell syndrome and Rasmussen
encephalitis.
• A proper clinical history and CT/MRI findings provide the correct
diagnosis
DYKE-DAVIDOFF MASSON SYNDROME (DDMS)
CONCLUSION• Dyke-Davidoff-Masson syndrome must be ruled out in differential
diagnosis of fetal unilateral ventriculomegaly.
• Early clinical assessment, differential diagnosis and cerebral
imaging including cerebral MRI angiography allow the clinicians to
diagnose also in early infancy this rare condition
References
• Dyke CG, Davidoff LM, Masson LB. Cerebral hemiatrotphy with homolateral hypertrophy of skull and sinus. Surg Gynecol Obstet.1933:57: 588 - 600
• Solomon GE, Hilal SK, Gold AP, Carter S. Natural history of acute hemiplegia of childhood Brain 1970:93:107-120
• Pressler RM, Binnie C, Cooper R, Robinson R: Neonatal and paediatric clinical neurophisiology. 1st edition. Amsterdam: Churchill Livingstone Elsevier; 2007:111–154.
• Unal O, Tombul T, Cýrak B, Anlar O, Incesu L, Kayan M: Left hemisphere and sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome). Clin. Imaging 2004, 28:163–165.