Dyke-Davidoff-Masson Syndrome: A Case Report

Authors

DOI:

https://doi.org/10.5195/ijms.2022.1217

Keywords:

Seizures, Neuroimaging, Anticonvulsants, Cerebral atrophy, Hemiplegia, Dyke-Davidoff-Masson syndrome

Abstract

Background: Dyke-Davidoff-Masson syndrome (DDMS) is a neurological syndrome characterized by the presence of convulsions, facial asymmetry due to palsy of the facial nerve (CN VII), contralateral hemiplegia, and reduced intellectual capacity.

The Case: We report a case of DDMS in a 20-year-old male who is a previously known case of generalized epilepsy on medication presenting with status epilepticus and initially managed by anticonvulsants. On admission, the seizures manifested again which required the patient to be sedated with injectable anesthetics and intubated. Clinical examination showed no focal neurological deficits or neurocutaneous markers. Imaging studies showed characteristic features of DDMS which were hemiatrophy of the right cerebrum with calvarial thickening, and sinuses showing hyperpneumatization on the same side as hemiatrophy. Previous history of such episodes had been recorded and the patient was kept on strict pharmacotherapy. Failure of adherence to these led to the current presentation. The diagnosis of DDMS was kept and the patient was treated conservatively with anticonvulsants and referred to a higher center for further management.

Conclusion: DDMS, being a rare but important cause of refractory epilepsy, is easily missed on initial assessment and failure of adequate management leads to higher rates of morbidity and mortality associated with this syndrome. In cases with an atypical presentation, such as this one, a good background in radio-imaging and knowledge of the physical manifestations are required for final diagnosis.

References

Dyke CG, Davidoff LM, Masson LB. Cerebral hemiatrophy with homolateral hypertrophy of the skull and sinus. Surg Gynecol Obstet. 1933;57:588–600.

Shetty DS, Lakhkar BN, John JR. Dyke-Davidoff-Masson syndrome. Neurol India. 2003 Mar;51(1):136.

Roy U, Panwar A, Mukherjee A, Biswas D. Adult Presentation of Dyke-Davidoff-Masson Syndrome: A Case Report. Case Rep Neurol. 2016 Jan 16;8(1):20-6.

Unal O, Tombul T, Cirak B, Anlar O, Incesu L, Kayan M. Left hemisphere and male sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome). Clin Imaging. 2004 May-Jun;28(3):163-5.

Afifi AK, Godersky JC, Menezes A, Smoker WR, Bell WE, Jacoby CG. Cerebral hemiatrophy, hypoplasia of internal carotid artery, and intracranial aneurysm. A rare association occurring in an infant. Arch Neurol. 1987 Feb;44(2):232-5.

Engel J Jr. Approaches to refractory epilepsy. Ann Indian Acad Neurol. 2014 Mar;17(Suppl 1):S12-7.

Sener RN, Jinkins JR. MR of craniocerebral hemiatrophy. Clin Imaging. 1992 Apr-Jun; 16:93–97.

Stred SE, Byrum CJ, Bove EL, Oliphant M. Coarctation of the midaortic arch presenting with monoparesis. Ann Thorac Surg. 1986 Aug;42(2):210-2.

Solomon GE, Hilal SK, Gold AP, Carter S. Natural history of acute hemiplegia of childhood. Brain. 1970 Jan 1;93(1):107-20.

Rao KC: Degenerative diseases and hydrocephalus; in Lee SH, Rao KC, Zimmerman RA (eds): Cranial MRI and CT. New York, McGraw-Hill, 1999, pp 212-214.

Zilkha A: CT of cerebral hemiatrophy. Am J Roentgenol 1980 Aug;135:259-262.

Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: a review. Pediatr Neurol. 2004 May;30(5):303-10.

Sheybani L, Schaller K, Seeck M: Rasmussen encephalitis: an update. Schweiz Arch Neurol Psychiatr 2011 Sep 7;162:225-231.

Qiu BP, Shi CH: Silver-Russel syndrome: a case report. World J Pediatr 2007;3:68-70.

Amor DJ, Kornberg AJ, Smith LJ. Encephalocraniocutaneous lipomatosis (Fishman syndrome): a rare neurocutaneous syndrome. J Paediatr Child Health. 2000 Dec;36(6):603-5.

Jacoby CG, Go RT, Hahn FJ. Computed tomography in cerebral hemiatrophy. AJR Am J Roentgenol. 1977 Jul;129(1):5-9.

Ono K, Komai K, Ikeda T. Dyke-Davidoff-Masson syndrome manifested by seizure in late childhood: a case report. J Clin Neurosci. 2003 May;10(3):367-71.

Aguiar PH, Liu CW, Leitão H, Issa F, Lepski G, Figueiredo EG, et.al. MR and CT imaging in the Dyke-Davidoff-Masson syndrome. Report of three cases and contribution to pathogenesis and differential diagnosis. Arq Neuropsiquiatr. 1998 Dec;56(4):803-7.

Abdul Rashid AM, Md Noh MSF. Dyke-Davidoff-Masson syndrome: a case report. BMC Neurol. 2018 May 29;18(1):76.

George P, Shenoy BR. Dyke-Davidoff-Masson Syndrome: an uncommon cause of refractory epilepsy identified by neuroimaging. Journal of Clinical and Diagnostic Research 2011;5:833-834.

Narain NP, Kumar R, Narain B. Dyke-Davidoff-Masson syndrome. Indian Pediatr. 2008 Nov;45(11):927-8.?

Published

2022-01-25 — Updated on 2022-04-13

Versions

How to Cite

Urs, G. M., & R. Doddabele, H. (2022). Dyke-Davidoff-Masson Syndrome: A Case Report. International Journal of Medical Students, 10(1), 82–85. https://doi.org/10.5195/ijms.2022.1217 (Original work published April 5, 2022)

Issue

Section

Case Report

Similar Articles

You may also start an advanced similarity search for this article.