# Dyke-Davidoff-Masson syndrome: Adult female patient with refractory epilepsy and global cognitive decline

## TL;DR

A 19-year-old woman with severe epilepsy and cognitive decline was diagnosed with the rare Dyke-Davidoff-Masson syndrome, highlighting the importance of recognizing this condition in adults.

## Contribution

This case report adds to the limited literature on Dyke-Davidoff-Masson syndrome diagnosed in an adult female patient.

## Key findings

- The patient exhibited cerebral hemiatrophy, cranial vault thickening, and epileptic seizures consistent with Dyke-Davidoff-Masson syndrome.
- Neuropsychological tests confirmed global cognitive impairment in the patient.
- The case emphasizes the need for clinicians to consider this rare syndrome in differential diagnoses of refractory epilepsy.

## Abstract

The Dyke-Davidoff-Masson syndrome is characterized by the presence of cerebral hemiatrophy, craneal vault thickening, epileptic seizures, hemiparesis, and cognitive impairment. It is typically diagnosed in childhood and requires specific diagnostic tools for accurate assessment.

This report describes the case of a 19-year-old woman who presented with epileptic seizures and regression in neurodevelopment. She was admitted to the emergency department due to high ictal frequency. During her hospitalization, imaging and electroencephalographic findings were consistent with Dyke-Davidoff-Masson syndrome. Additionally, neuropsychological tests revealed global cognitive impairment. After ten days of hospitalization and five days without epileptic seizures, the patient was discharged. Dyke-Davidoff-Masson syndrome is a rare and often unrecognized condition with high morbidity. Clinicians has the responsibility to identify the key characteristics of the syndrome and perform an adequate differential diagnosis.