Dyke–Davidoff–Masson syndrome: a combination of clinical and radiological signs not to be missed

Síndrome de Dyke-Davidoff-Masson: uma combinação de sinais clínicos e radiológicos para não serem esquecidos

A 21-year-old man presented with complex partial status epilepticus . His past medical history was marked by epilepsy and his examination disclosed atrophy of the right limbs and hemiparesis in the same side of the body. A brain CT scan showed atrophy of the entire left hemisphere, with prominent sulci, enlargement of the lateral ventricle and overdevelopment of the frontal sinuses on the left side (see figure).

Dyke-Davidoff-Masson syndrome is an uncommon congenital or childhood-onset acquired neurologic syndrome, presenting mainly with complete hemiparesis, recurrent seizures and non-progressive chronic encephalopathy ^{1 , 2} . Neuroimaging features are characterized by unilateral cerebral atrophy with ipsilateral hypertrophy of paranasal sinuses ² .

Figure.
(A) examination discloses marked right-side atrophy, compared with left side; (B, C) brain computerized tomography shows atrophy of the entire left cerebral hemisphere. Note the prominent sulci, enlargement of the lateral ventricle and overdevelopment of the frontal sinuses on the left side.

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
  Chand G, Goel R, Kapur R. Dyke-Davidoff-Masson syndrome. Arch Neurol 2010;67:1026.

Publication Dates

• Publication in this collection
  Nov 2013

History

• Received
  27 May 2013
• rev-received
  26 June 2013
• Accepted
  03 July 2013