Dyke-Davidoff-Masson Syndrome

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare clinical condition in which atrophy or hypoplasia of one cerebral hemisphere occurs secondary to brain insult during fetal or early childhood, which results in variable clinical manifestations like hemiparesis, seizures, expressive aphasia, and mental retardation. This rare entity mainly presents in childhood and is unusual in adults. DDMS is a rare cause of epilepsy and should be considered and excluded in cases of refractory seizures. Few cases have been reported from a developing nation like Nigeria but not from the north-eastern part of Nigeria to the best of our knowledge. Though fewer specialists exist in Adamawa State, efforts to train more specialists and education of medical officers to manage this rare case need to be strengthened. Herein is a case of an adolescent boy with recurrent generalized tonic-clonic convulsions complicated by left-sided hemiparesis, expressive aphasia, and mental age equivalent of a six-year-old by the Goodenough draw-a-person test. Found to be obese with Body Mass Index (BMI) of 29 kg/m 2 (Z-score >2 Standard deviation SD), microcephaly, Occipito-frontal Circumference (OFC) of 45 cm (Z-score > −3 SD), spastic left-sided hemiplegia and hemiplegic gait. Magnetic resonance imaging (MRI) of the brain showed hyper-intensity in the right cerebral hemisphere, extensive atrophy of the right cerebral hemisphere involving the ipsilateral fronto-temporoparietal lobes, cerebral peduncle, and a contralateral megalencephaly, ipsilateral lateral ventricular dilatation, hypertrophic calvarium, hyperpneumatization of sphenoidal sinuses and midline shift due to loss of volume on the right. A diagnosis of Dyke-Davidoff-Masson syndrome was made; the patient did well on carbamazepine and physiotherapy. Caregivers were counseled, and the patient was discharged home and is currently on a follow-up visit.