DOI: 10.5958/j.2319-5886.2.2.

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International Journal of Medical Research & Health Sciences

www.ijmrhs.com
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Volume 2 Issue 2 April-June

Coden: IJMRHS
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Copyright @2013

ISSN: 2319-5886

Received: 28 Feb 2013 Case report

Revised: 20 Mar 2013

Accepted: 22nd Mar 2013

DYKE-DEVIDOFF-MASSON SYNDROME: A CASE REPORT *More Sumeet S1, Jadhavar Avinash L1, Garkal Shailendra M1, Tewari Suresh C2
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Senior Resident, 2 Professor and HOD, Department of Medicine, Pravara Rural Hospital & Medical College, Loni, Ahmednagar, Maharashtra, India. *Corresponding author email: sumeet_more2002@yahoo.com ABSTRACT

Dyke-Davidoff-Masson syndrome (DDMS) is characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation. The characteristic radiologic features are cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. We report a case of DDMS in an 18 years old girl who presented with a history of generalized tonic clonic seizures, hemiparesis and hemiatrophy of right side. Keywords: Dyke-Davidoff-Masson syndrome, Hemiatrophy, Seizure, Hemiplegia.
INTRODUCTION

Dyke-Davidoff and Masson described the plain skull radiographical features of Dyke-DavidoffMasson Syndrome (DDMS) in 19331. This was described in a series of 9 patients with hemiparesis, seizures, facial asymmetry and mental retardation. The radiographical features of the skull were asymmetry, ipsilateral osseous hypertrophy of the calvarium and hyperpneumatization of the sinuses2.
CASE REPORT

A 18 years old female with a history of epilepsy from her 2 years of life, presented to us with increasing frequency of generalized tonic-clonic seizures of about one to two in a month and not on any medication. The patient had normal developmental milestones upto 2 years of age when she had a seizure, followed by weakness in

right side of body with facial involvement. At present she had facial asymmetry, right sided hypertonia, brisk deep tendon reflexes, an extensor plantar reflex and hemiatrophy on right side. Her routine haematological and biochemical investigations were within normal limits. MRI of Brain revealed left cerebral hemiatrophy with areas of gliosis secondary to old ischemic or traumatic insult in frontotemporo-parieto-occipital region on left side, thickening of bony calvarium on left hemi cranium with hyperpneumatised ipsilateral frontal sinus and mastoid air cells, contralateral cerebellar atrophy. All these features are suggestive of DDMS. When the clinical features of cerebral hemiatrophy are associated with the radiological features of cerebral atrophy, osseous hypertrophy
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of the calvarium and hyper-pneumatisation of the sinuses dilatation, DDMS has to be considered3,4. The pathogenesis of DDMS is thought to originate from a childhood cerebral insult due to trauma, inflammation or vascular malformations/occlusions of the middle cerebral artery4, 5. In this case as described above, patient had history of a seizure episode at 2 years of age.

Such insult occurring early in life causes compensatory cranial changes like calvarial thickening and sinus enlargement due to a relative vacuum created by the hypoplastic brain5-9. A possible etiological relation between cerebral atrophy and seizures has been reported in two different studies from India 7,8.

Fig 1: Unilateral cerebral atrophy on left side with Fig 2: Ipsilateral osseous hypertrophy of the calvarium contralateral cerebellar atrophy. and hyper-pneumatization of the sinuses

Fig 3: Left cerebral hemiatrophy with thickening of bony calvarium on left hemi-caranium 298

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DISCUSSION

DDMS is a rare condition characterized clinically by variable degree of facial asymmetry, seizures, contralateral hemiparesis, mental retardation and learning disabilities in association with the classical radiological findings of asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral osseous hypertrophy and hyperpneumatization of sinuses1,10-12. Both sexes and any of the hemispheres may be affected but male gender and left hemisphere involvement are more frequent13. Age of presentation depends on time of neurologic insult and characteristic changes may be seen only in adolescence. The clinical findings may be of variable degree depending on the extent of the brain injury. Varying degree of

atrophy of one half of the body, sensory loss, speech and language disorder, mental retardation or learning disability and psychiatric manifestations like schizophrenia may also be present. In this case, the left hemisphere is smaller in size with prominence of sulcal space, sylvian fissure alongwith resultant mild dilatation and pulling of ipsilateral ventricle suggestive of areas of gliosis secondary to old ischemia or traumatic insult and chronic wallerian degeneration of axons in left cerebral peduncle. MR Angiography is suggestive of paucity of branches from M2 and M3 segments of left middle cerebral artery. A proper history, thorough clinical examination and radiologic findings provide the correct diagnosis.

Fig 4: MR Angiography suggestive of paucity of branches from M2 and M3 segments of Lt. middle cerebral artery.

Fig.5: Chronic wallerian degeneration of axons in

left cerebral peduncle.

CONCLUSION

Although Dyke- Davidoff-Masson syndrome is a rare condition but other differential diagnosis to be considered in a patient of cerebral hemiatrophy is Sturge-Weber Syndrome, some brain tumors, Silver Syndrome, as well as

Conditions, that are associated with unilateral magelancephaly as in the linear-nevus syndrome. A proper history, thorough clinical examination and its typical radiological findings can derive its correct diagnosis.
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REFERENCES

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10. Tasdemir HA, Incesu L, Yazicioglu AK, Belet U, Gungor L. Dyke Davidoff Masson syndrome. Clin Imaging 2002; 26: 13-17. 11. Sharma S, Goyal D, Negi A, Sood RG, Jhobta A, Surya M. Dyke-Davidoff Masson syndrome. Indian J Radiol Imaging 2006: 16: 165-66. 12. Dix JE, Coil WS. Cerebral hemiatrophy: Classification on the basis of MR Imaging findings of mesial temporal sclerosis and childhood febrile seizures. Radiology 1997; 203: 269-74. 13. Manas R Behera, Sibabrata Patnaik and Ashwini K Mohanty. Dyke-Devidoff-Masson Syndrome. J Neurosci Rural Pract. 2012 SepDec; 3(3): 41113.

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