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COMMENTARY |
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Year : 2016 | Volume
: 9
| Issue : 6 | Page : 733 |
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Dyke-Davidoff-Masson syndrome
Arzu Canan
Department of Radiology, Antalya Ataturk State Hospital, Clinic of Radiology, Antalya, Turkey
Date of Web Publication | 16-Nov-2016 |
Correspondence Address: Arzu Canan Clinic of Radiology, Antalya Ataturk State Hospital, Antalya Turkey
Source of Support: None, Conflict of Interest: None | Check |
DOI: 10.4103/0975-2870.194194
How to cite this article: Canan A. Dyke-Davidoff-Masson syndrome. Med J DY Patil Univ 2016;9:733 |
Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease which was first described in 1933. The plain skull radiographic and pneumatoencephalographic changes in nine patients with DDMS were reported by Dyke et al. [1] DDMS usually occurs in fetal life or early childhood due to brain damage. It is classified on basis of the etiological factors: congenital or acquired. In the fetal life, the reason usually is vascular and this type is called as congenital. In the acquired type (in the perinatal life or later), the reasons are trauma, ischemia, hemorrhage, infection, and vascular disorders. [2] Because of its etiological factors, DDMS is always diagnosed in the postnatal life or early childhood. Besides, there are a few adult case reports in the literature. [3] The current publication is one of them. [4] The patient is a young adult and the reason of the disease is thought to be trauma which is suggestive of acquired type.
The clinical symptoms are variable and depend on the degree of brain injury. They are seizures (focal or generalized), facial asymmetry, contralateral hemiplegia or hemiparesis, mental retardation, psychiatric problems, and speech or language disorders. [5] The diagnosis is made by clinical and radiological findings. Computed tomography (CT) and magnetic resonance imaging (MRI) findings are suggestive of the diagnosis. [6],[7] Unilateral cerebral hemiatrophy with ipsilateral thickness of adjacent bone and hyperpneumatization of sinuses due to vacuum effect are easily detected by CT. [6] MRI also demonstrates parenchymal signal changes due to gliosis and associated abnormalities such as porencephalic cysts. [7] In addition, differentiation from other diagnoses such as hemimegalencephaly, Sturge-Weber syndrome, and Rasmussen encephalitis is needed. [2] The treatment of this disease is symptomatic and consists of management of clinical symptoms, especially seizures.
In conclusion, DDMS is a rare disease that is usually diagnosed in the postnatal life or early childhood. Case reports as similar to the present publication [4] suggest that DDMS should be kept in mind in the differential diagnosis of seizures even in adult patients.
References | | |
1. | Dyke CG, Davidoff LM, Masson CB. Cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Surg Gynecol Obstet 1933;57:588-600. |
2. | Parker CE, Harris N, Mavalwala J. Dyke-Davidoff-Masson syndrome. Five case studies and deductions from dermatoglyphics. Clin Pediatr (Phila) 1972;11:288-92. |
3. | Canan A, Aksoy C, Özbilek Ö, Eray B, Koç K, Çýra K. Radiological findings of Dyke-Davidoff-Masson syndrome in an adult patient: Case report. J Case Rep Stud 2015;35:506. |
4. | Jilowa CS, Meena PS, Jain M, Sharma KK. Dyke-Davidoff-Masson syndrome. Med J DY Patil Univ 2017;10:1. [Page numbers to be given after pagination]. |
5. | Ono K, Komai K, Ikeda T. Dyke-Davidoff-Masson syndrome manifested by seizure in late childhood: A case report. J Clin Neurosci 2003;10:367-71. |
6. | Zilkha A. CT of cerebral hemiatrophy. AJR Am J Roentgenol 1980;135:259-62. |
7. | Shen WC, Chen CC, Lee SK, Ho YJ, Lee KR. Magnetic resonance imaging of cerebral hemiatrophy. J Formos Med Assoc 1993;92:995-1000. |
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