López VY, Durán RA, Arroyo MIB

Language: Spanish
References: 8
Page: 65-68
PDF size: 246.59 Kb.

ABSTRACT

Background: Dyke-Davidoff-Masson syndrome (DDMS) was first described by CG Dyke, LM Davidoff and CB Masson in 1933 with the help of advances in neuroimaging and electroencephalogram studies, it is characterized by cerebral hemiatrophy, contralateral hemiplegia or hemiparesis, cranial vault thickening, facial asymmetry, seizures, intellectual disability and behavioral changes. MRI is the key imaging modality that confirms clinical suspicion of DDMS. Two clinical cases are described.
Case No. 1: 9 years old male patient, with epileptic seizures since the age of 8 months, focal tonic seizures of the left arm with impaired awareness, difficult to control, with poor adherence to treatment, poor language, poor impulse control, hyperactive behavior and intellectual disability. Facial asymmetry and right hemiparesis.
Case No. 2: 8 years old female patient, with epileptic seizures since the age of 7 months, focal, tonic seizures in the left arm, with impaired awareness. She presents learning disorder and ADHD, facial asymmetry, left hemiparesis and left hemihypotrophy.
Conclusions: DDMS is a rare neurological disorder that leads to uncontrolled seizures and a spectrum of disabilities, due to its rarity, may easily be missed by the majority of treating clinicians.
Knowledge of its features on imaging enables timely and accurate diagnosis. The association with MRI features in order to optimally diagnose DDMS and offer appropriate management.

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