Title: A Case Study of Dyke Davidoff Masson Syndrome
Authors: Sadaf Sultana, Gotte Priyanka, N. Sai Kumar, Dr Thatikonda Anil Kumar, Dr K.Rohith Kumar
DOI: https://dx.doi.org/10.18535/jmscr/v7i12.95
Abstract
A 19 years old female presented with recurrent generalized seizures, right sided hemiplegia, and had developmental delay in motor and speech domains. CT of the brain revealed characteristic features diagnostic of infantile type of cerebral hemi atrophy or Dyke-Davidoff-Masson syndrome. It is an important cause of intractable and drug-resistant seizures. It has varied clinical presentation and history with distinct neuroimaging features. Here, we describe a female patient presented with recurrent intractable convulsion, mental retardation, hemiparesis, dystonia and characteristic neuroimaging features of cerebral hemiatrophy, calvarial thickening, and ipsilateral hyperpneumatization of the frontal sinuses which is suggestive of DDMS. Early institution of neuroimaging in patients with intractable epilepsy will make early diagnosis and better outcome. DDMS also occur due to atrophy or hypoplasia of one cerebral hemisphere (hemiatrophy), which is usually due to any trauma or injury to the growing brain in early childhood. The manifestations are variable and depend on the extent of brain injury. More common symptoms are recurrent seizures, facial asymmetry, hemiplegia, mental retardation, mutism. The CT of brain mostly give impression of cerebral hemiatrophy with ipsilateral hypertrophy of the skull and sinuses.
Keywords: Dyke-Davidoff-Massion Syndrome, hemiparesis, mental retardation, hemiatrophy, hyperpneumatization.
References
- Sharma S, Goyal l, Negi A, Sood RG, Jhobta A, Surya M. Dyke-Davidoff-Masson Syndrome. Ind J Radiol Imag. 2006; 16:165–6.
- 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.
- Amann B, Garcia de la Iglesia C, Mckenna P, Pomarol-Clotel E, Sanchez-Guerra M, Orth M. Treatment- refractory Schizoaffective disorder in a patient with Dyke-Davidoff Masson Syndrome. CNS Spectra. 2009; 14:36–9.
- Singh P, Saggar K, Ahluwalia A. Dyke-Davidoff-Masson Syndrome: Classical imaging findings. J Pediatr Neurosci. 2010;5:124–5.
- Koshy B, Surendrababu NR. Dyke-Davidoff-Masson Syndrome. Ann Acad Med Singapore. 2010; 39:501–2.
- Shetty DS, Lakhkar BN, John JR. Dyke-Davidoff-Masson Syndrome. Neurol india. 2003;51:136.
- Narain NP, Kumar R, Narain B. Dyke-Davidoff-Masson Syndrome. Indian Pediatr. 2008;45:927–8.
- Graham A, Molnar Z. Development of the Nervous system. In: Standring S, editor. Gray's Anatomy. 40th ed. London, Churchill Livingstone: Elsevier; 2008. p. 385.
- Parker CE, Harris N, Mavalwala J. Dyke-Davidoff-Masson Syndrome: Five case studies and deductions from dermatoglyphics. Clin Pediatr. 1972;11: 288–92.
- Solomon GE, Hilal SK, Gold AP, Carter S. Natural history of acute hemiplegia of childhood. Brain. 1970;93:107–20.
- Sener RN, Jinkins JR. MR of craniocerebral hemiatrophy. Clin Imaging. 1992; 16:93–7.
- Stred SE, Byrum CJ, Bove EL, Oliphant M. Coarctation of midaortic arch presenting with monoparesis. Ann Thorac Surg. 1986;42:210–12.
- Dyke CG, Davidoff LM, Masson CB: Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. Surg Gynecol Obstet 1933;57:588–600.
- Unal O, Tombul T, Cirak B, Anlar O, Incesu L, Kayan M: Left hemisphere and male sex dominance of cerebral hemiatrophy (DDMS). Clin Imaging 2004;28:163–165.
- Afifi AK, Godersky JC, Menezes A, et al: Cerebral hemiatrophy, hypoplasia of internal carotid artery and intracranial aneurysm. Arch Neurol 1987;44:232–235.
- Pendse NA, Bapna P, Menghani V, Diwan A. Dyke-Davidoff-Masson Syndrome. Indian JPediatr.2004;71:943.