INTRODUCTION

Known as Doose Syndrome (DS), myoclonic-astatic epilepsy is a childhood-onset encephalopathic epilepsy characterized by several types of seizures (mostly generalized, myoclonic and atonic) and producing cognitive retardation and ataxia in a previously normal child. Our aim is to describe several clinical aspects in children with DS and look for factors related to a worse evolution.

MATERIAL AND METHODS

We reviewed several clinical aspects of 20 children diagnosed of DS in our Department and studied their relationship with final seizure control and the motor and cognitive development achieved.

RESULTS

We found a statistically significative relationship between final cognitive development (CD) and types of seizure at onset (p<0.045), epileptic discharges in EEG (p<0.059) and types of seizures developed (p<0.063). The same was associated with motor development (p<0.049, p<0.007 and p<0.015, respectively). Epilepsy outcome was related to: types of seizures developed (p<0.041), epileptic discharges in EEG (p<0.002) and MRI (p<0.026).

A normal CD (45% of patients), absence of ataxia (60%) and seizure-free status (65%) were related to the absence of frontal discharges in EEG studies and not presenting absences nor focal frontal seizures as the disease develops. In addition, lack of myoclonic seizures at onset was associated with better cognitive and motor development, and a normal MRI (without atrophy) was related to a better seizure control.

CONCLUSIONS

Some DS patients have a bad evolution (mental retardation, ataxia, refractory epilepsy). We found in our patients some factors related to this that could help us identifying them early and improving their management.