Dyke–Davidoff–Masson syndrome: A rare case report

We hereby report a 30-year-old young male who had a history of generalized tonic-clonic seizures since age of 5 years. Patient took antiepileptic drug treatment, including phenytoin and phenobarbitone and some indigenous medications. However, continued to have a seizure on and off and presented in an emergency department with intractable seizures. Patient had history of weakness left half of the body for last 8 years, non-progressive in nature. His mother revealed history of developmental delay including gross motor, fine motor, language, perceptive cognitive, social and personal delay. There was no history of similar illness in any other sibling or family member. On examination, patient was conscious with an intelligence quotient of 60 indicating mild mental retardation. Neurological examination revealed 4/5 power in left upper and lower limb with brisk reflexes. Left planter was extensor. There was no neck rigidity, any sensory deficit, cranial nerve or bowel bladder involvement. All routine biochemical investigations including liver and renal function were within normal limits. Random blood sugar, serum electrolytes including calcium levels were within normal range. Computed tomography scan showed hyperpneumatization of frontal sinus, thickened calvarial bone with hyperpneumatization of mastoid air cells (Figure 1). Magnetic resonance imaging (MRI) brain showed loss of volume of right cerebral hemisphere with gliosis and prominent right lateral ventricle and cortical sulci associated with ipsilateral calvarial thickening. Diffuse cerebellar atrophy was also noted. There was no evidence of any focus of restricted diffusion to suggest acute infarct (Figure 2). Twenty-three channel electroencephalography recordings under sedation showed a poorly organized background activity of bursts of generalized spike and wave discharged admixed with low and high voltage slow waveforms. Based on characteristic radiological finding patient was diagnosed as a case of Dyke–Davidoff–Masson syndrome (DDMS) and was treated with valproic acid and levetiracetam. Patient was controlled on medication for next 4 months but then, unfortunately, lost to follow-up. The DDMS first reported in 1933, is a rare condition, and refers to atrophy or hypoplasia of one cerebral hemisphere (hemiatrophy).1 It is characterized clinically by variable degrees of facial asymmetry, recurrent seizures, contralateral hemiparesis, mental retardation, speech and language disorders along with various learning disabilities. Seizures can be focal or generalized. Occasionally, psychiatric manifestations have also been reported. Both sexes and hemispheres may be affected with a slight preponderance for male sex and left hemisphere.2 The cause is cerebral insult, which may occur in the prenatal, perinatal or postnatal period. Prenatal causes are congenital malformation, infection and vascular insult while, in the perinatal period birth trauma, anoxia, hypoxia and intracranial hemorrhage are most common causes. Trauma, tumor, infection and prolonged febrile seizures account for postnatal causes.3 Iranian Journal of Neurology