Case report
An 18-year-old male was admitted in our neurology clinic for recurring tonic clonic seizures that started three days prior to his admission. Medical history was consistent with a full-term infant, born from healthy and non-consanguineous parents after an uneventful pregnancy. However, delivery was done by caesarean section due to dystocia. Family history was unremarkable. He had mental and motor acquisition delay because he could not seat after one year of age and walked after the age of two. Parents reported that he presented focal tonic clonic seizures in the right half of his body at one year of age, at least once a day that worsened over the time to more than ten episodes daily. He was first treated with Valproic Acid, Phenobarbital and Carbamazepine at different times which could poorly control the seizures, despite having received the correct doses. He also presented frequent falls and injuries due to seizures. Later, they noticed that the right side of his body was becoming smaller and weaker than the left one. In addition, he presented a progressive intellectual disability that led him to drop out of school at the age of nine. On examination, the body temperature, blood pressure, heart and breathing rates, and the oxygen saturation were normal. Neurological examination found status epilepticus with right-sided focal tonic clonic seizures followed by secondary generalization. He had severe cognitive decline and couldn’t count from one to ten. Mini Mental State Examination (MMSE) was not possible. Body asymmetry and hemiparesis was confirmed in addition to ankylosis in the right wrist and elbow and a prominent forehead (Figure 1A&B&C). Abdominal, cardiovascular, and pulmonary examination were unremarkable. He did not present any cutaneous lesions other than scars caused by seizure-related injuries. Blood chemistries including total blood cell count, liver enzymes, serum creatinine and blood ions were normal. EEG performed two weeks after admission found a diffuse slow background with delta wave activities in hyperpnea and a continued focal to diffuse pseudo periodic activities (Figure 2A&B). Brain CT-scan revealed a pronounced atrophy of left cerebral hemisphere with ex vacuo of the ipsilateral ventricle. Furthermore, a bilateral abnormal thickening of the skull bone with an hyperpneumatization of the frontal sinuses were seen (Figure 2C&D). His clinical and imaging features were consistent with DDMS. He was initially put on intravenous Levetiracetam (1500mg/day), and continuous infusion of Clonazepam (2mg/day). The disease course remarkably changed to fewer seizures (down to 10 a day). Then, we added oral Valproic Acid (500mg twice a day) that drastically improved the seizures with a maximum of two seizures daily and the patient was discharged on this treatment. After the discharge, parents reported that he presented three episodes of seizures in one month.