LAUSR

Dear Editor-in-Chief, Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder usually observed during childhood and classically characterized by epilepsy, hemiparesia/hemiplegia, facial asymmetry, and mental retardation. It is radiologically characterized by partial or diffuse cerebral hemiatrophy [1, 2]. DDMS can be classified as congenital when it occurs before 2 years of age, typically resulting from intrauterine or neonatal vascular insults, whereas the acquired form can arise due to a variety of brain insults, including asphyxia, traumas, infections, prolonged febrile seizures, ischemia, and brain hemorrhages. The onset and presentation are variable [2]. It is our intention to expose an unusual case in which diffuse cerebral hemiatrophy occurred mid-life, accompanying a previous history of mild neurological symptoms. We explore the clinical features and radiological findings and propose an unusual evolution and late diagnosis of acquired DDMS. A 48-year-old male was admitted to our emergency room with convulsive status epilepticus that lasted less than 1 hour. Initial monitoring and supportive treatment were followed by anticonvulsive therapy with intravenous diazepam and phenytoin, successfully ending the seizure. Neurological evaluation after the event revealed left side hemiparesis and facial asymmetry. In addition, over the course of the following days, tonic-clonic seizures persisted, requiring multi-drug treatment with phenobarbital and phenytoin. The patient’s history revealed signs of mild psychomotor developmental delay, speech disorder, andminimal gait irregularity since childhood, and refractory tonic-clonic seizures had occurred since the patient was 13 years old. During hospitalization, an investigative panel revealed no changes in blood count and no metabolic or infectious changes. Cerebrospinal fluid analysis was normal, and a head computerized tomography (CT) revealed brain parenchyma with a slight mass difference between hemispheres. There were no signs of brain ischemia or hemorrhages and no ventricular changes (Fig. 1a). Ultrasonographic and angiographic examination of the carotid arteries exposed thickening of the artery walls, atheroma plaques, and stenosis of 60% on the right internal carotid artery. The patient was discharged after 7 days to the care of his family, with anticonvulsive therapy (phenytoin 100 mg prescribed twice daily and phenobarbital prescribed at 100 mg per day). Follow-ups were planned at our outpatient clinic for further investigation. The patient did not return until 3 years later, complaining of persistent seizures, left side hemiparesis, facial asymmetry, and logorrheic speech. In the intervening period, there was poor adherence to the long-term treatment due to chronic smoking, as well as alcohol and cannabis abuse for the past 20 years. A new laboratory investigation revealed no abnormalities; however, the head CT revealed severe atrophy of the right cerebral hemisphere accompanied by contralateral cerebellar atrophy (Fig. 1b). Moreover, his first documented electroencephalogram (EEG) revealed right anterior temporal epileptiform activity. We then proposed a diagnosis of DDMS, as the differential diagnosis investigation did not reveal any significant findings. The patient continued his treatment through a strict follow-up routine, cessation of drug, tobacco and alcohol abuse, and better adherence to antiepileptic drug therapy. He is currently being evaluated by our neurology department every 4 months, and seizures have been controlled, with no occurrences over the past year. Since its first description in 1933, only a few isolated reports of DDMS have emerged, mainly with childhood presentation and diagnosis. However, the pathophysiological mechanisms underlying DDMS remain uncertain, and its diagnosis is controversial. Recent reports have suggested that late diagnosis and mild presentation of symptoms are possible [1, 3, 4]. Biciçi et al. [3] described the diagnosis of a 50-year-old woman who exhibited mild and unspecific symptoms, although no specific etiology was disclosed, that case shows that a wide spectrum of * Renata P. Buainain [email protected]