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CASE REPORT |
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Year : 2014 | Volume
: 7
| Issue : 5 | Page : 672-674 |
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Dyke-Davidoff-Masson syndrome: A case report
Biswajyoti Rath, Maheswar Samant, Kali P Swain, Ashok K Mallick
Department of Neurology, Sriram Chandra Bhanja Medical College, Cuttack, Odisha, India
Date of Web Publication | 10-Sep-2014 |
Correspondence Address: Biswajyoti Rath Department of Neurology, Sriram Chandra Bhanja Medical College, Cuttack - 753 007, Odisha India
Source of Support: None, Conflict of Interest: None | Check |
DOI: 10.4103/0975-2870.140494
Dyke-Davidoff-Masson Syndrome (DDMS), also called as cerebral hemiatrophy, is a rare clinical condition characterized by seizures, facial asymmetry, contralateral spastic hemiplegia or hemiparesis, with learning difficulties. It is commonly diagnosed in presence of associated radiologic findings, which include cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. We present a 21-year female with increased frequency of convulsion and abnormal behavior for the last 2 months. She had a known seizure disorder for 15 years and had mental subnormality. On physical examination, she was disoriented with mild right hemiparesis. On CT scan the brain showed unilateral left cerebral atrophy. Keywords: Dyke-Davidoff-Masson syndrome, seizures, unilateral left cerebral atrophy
How to cite this article: Rath B, Samant M, Swain KP, Mallick AK. Dyke-Davidoff-Masson syndrome: A case report. Med J DY Patil Univ 2014;7:672-4 |
Introduction | | |
In 1933, C.G. Dyke, L.M. Davidoff and C.B. Masson described plain skull radiographic and pneumatoencephalographic changes in a series of nine patients. [1],[2],[3] The condition is characterized by cerebral hemiatrophy, facial asymmetry, thickening or thinning of cranial vault, contralateral hemiplegia or hemiparesis, seizures, mental retardation, and behavioral changes like schizophrenia. [4],[5],[6] The above features may be present in varying combinations and degrees of severity. Diagnosis is usually achieved by clinical examination and radiologic investigation. The condition is usually diagnosed in childhood but rare cases of the condition diagnosed in teenagers and young adults have been made. Here we describe a 21-year-old female patient who presented to us with seizures, mental subnormality and characteristic radiologic findings suggestive of DDMS.
Case Report | | |
A 21-year female of low socio-economic status presented to us with h/o recurrent episodes of seizure for last 15 years [Figure 1]. There was increased frequency of seizure and abnormal behavior for last 2 months. On enquiring about her past history she had normal uneventful delivery with h/o developmental delay, mental subnormality and early school drop-out. There was no history of any serious childhood infections like fever, neuroinfection or trauma. She was diagnosed of having seizure since 5 years of age and since then she was on irregular oral anti-epileptic therapy. At time of presentation she was on oral eptoin, 300 mg/day. On physical examination, she was moderately built, no facial asymmetry, no cafe-au-lait spots and CNS examination showing disorientation to time, place and person with abnormal behavior, mild right hemiparesis with right plantar was extensor. She had 3-4 episodes of GTCS during hospitalization for which a second antiepileptic was added. On CT scan, brain showed left cerebral hemiatrophy with prominent sinuses [Figure 2] and [Figure 3]. She was discharged with 2 anti-epileptics - eptoin and valproic acid. | Figure 1: A 21-year female presenting with recurrent seizure and abnormal behavior
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| Figure 2: CT scan brain showing left cerebral atrophy - axial view. Relative thickening of left calvaria than right
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| Figure 3: CT scan showing hyperpneumatization of sinuses – sagittal view
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Discussion | | |
Cerebral hemiatrophy or unilateral brain atrophy is the end-stage of various pathologies culminating in atrophy or hypoplasia of a single cerebral hemisphere. [7] DDMS is a rare condition characterized clinically by varying degrees of facial asymmetry, seizures, contralateral hemiparesis, mental retardation and learning disabilities with behavioral abnormalities. [6],[8] The radiological findings include cerebral hemiatrophy, ipsilateral osseous hypertrophy and hyper-pneumatization of sinuses. [8] Either sex may be affected with involvement of any cerebral hemisphere. Unal et al., in a retrospective study of 26 patients of cerebral hemiatrophy showed that it was more frequent in the males with left cerebral hemisphere involvement. [9] Clinical features vary depending on the extent of brain injury. A detailed history, meticulous clinical examination with radiologic findings provide clue to the diagnosis.
The disease is generally classified into Infantile (congenital) and Acquired variety. [5] Congenital variety is mainly caused due to vascular occlusions or malformations in-utero or in the neonatal period. Neonatal or gestational vascular occlusion involving the middle cerebral vascular territory, unilateral cerebral arterial circulation anomalies, coarctation of the mid-aortic arch, mesencephalon hypoplasia and Wallerian degeneration have been propounded as some of the etiologies for the congenital variety. [4],[5],[10] Hageman et al. proposed the terms cerebral hemi-hypoplasia or unilateral cerebral hypoplasia for the primary (congenital) cerebral atrophy owing to the fact that there is lack of cerebral development rather than atrophy. [11] When the cerebral hemiatrophy develops in-utero or during first two years of life, it is associated with certain cranial changes like ipsilateral hypertrophy of the skull and sinuses as a compensatory change to take up the relative vacuum created by the hypoplastic cerebrum. [10] The brain reaches half the adult size during first year of life and three-fourths by the end of third year. As it enlarges, the brain presses outward on the bony tables, which gradually results in general shape of the adult head. But, failure of the cerebrum to grow causes other structures to direct their growth inward, accounting for ipsilateral hyper-pneumatization of the sinuses, increased width of the diploic space and elevations of the greater wing of the sphenoid and petrous ridge. [4] The other change in infantile type is a shift of midline structures towards the side of the disease and the sulcal prominence replacing the gliotic tissue is absent. [10] This feature differentiates it from cerebral hemiatrophy that occurs in later life.
Acquired variety may be due to infections, trauma, ischemia and hemorrhage. Age of presentation depends on the time of occurrence of the brain insult and often clinical features may not be evident till adolescence. The characteristic calvarial changes may or may not be present depending upon the time of injury. Further, these insults occur after birth, after completion of sulci formation. [12] Our case report with above clinical features suggest acquired variety.
The exact mechanism of cerebral atrophy is still unclear in either type. It is hypothesized that ischemic episodes from a variety of different causes reduce the production of brain derived neurotrophic factors, which in turn lead to cerebral atrophy. [12] Atalar et al., in their clinico-radiologic analysis of 19 patients concluded that, computed tomography and, in particular, magnetic resonance imaging is the procedures of choice with respect to assessment of the etiology and extent of cerebral parenchymal involvement in cerebral hemiatrophy. [13]
The condition needs to be differentiated from Basal ganglia germinoma, Sturge Weber syndrome, linear sebaceous nevus syndrome, neurofibromatosis, Parry Romberg Syndrome, Silver-Russell syndrome and Rasmussen encephalitis. [8]
The treatment is symptomatic, and includes management of convulsion, hemiplegia, hemiparesis and learning difficulties. Prognosis is better if hemiparesis occurs after the age of 2 years and in absence of prolonged or recurrent seizures. Children with intractable disabling and hemiplegia are the potential candidates for hemispherectomy with a success rate of 85% in carefully selected cases. [8],[10]
References | | |
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7. | Jacoby, CG, Go RT, Hahn FJ. Computed tomography in cerebral hemiatrophy. Am J Roentgenol 1977;129:5-9. |
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9. | Unal O, Tombul T, Cirak B, Anlar O, Incesu L, Kayan M. Left hemisphere andmale sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome). Clin Imaging 2004;28:163-5. |
10. | Goyal J, Shah V, Rao S, Jindal N. Dyke Davidoff Masson syndrome in Children. Internet J Pediatr Neonatol 2009;10:101-7. |
11. | Hageman G, Gooskens RH, Willemse J. A cerebral cause ofarthrogryposis: Unilateral cerebral hypoplasia. Clin Neurol Neurosurg 1985;87:119-22. |
12. | Lee JH, Lee ZI, Kim HK, Kwon SH. A case of Dyke-Davidoff-Masson syndrome in Korea. Korean J Pediatr 2006;49:208-11. |
13. | Atalar MH, Icagasioglu D, Tas F. Cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome) in childhood: Clinicoradiological analysis of 19 cases. Pediatr Int 2007;49:70-5. |
[Figure 1], [Figure 2], [Figure 3]
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