A CASE OF CEREBRAL HEMI ATROPHY - DYKE DAVIDOFF MASSON SYNDROME (DDMS) By Dr.K.PRASANNA RESIDENT RAJAH 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: Solomon GE, Hilal SK, Gold AP, Carter S. Natural history of acute hemiplegia of childhood Brain 1970:93: 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.