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.