2017 Volume 35 Issue 1 Pages 66-73
Myoclonic encephalopathy in non-progressive disorders (MEND) is characterized by recurrent myoclonic status (MS). Other initial seizure types, except for myoclonus, might also occur at onset. Prognosis is typically unfavorable with severe psychomotor developmental delay. Case 1: A 31-year-old man presented with severe psychomotor developmental delay. His seizures began at 1 year of age. Since the age of 8 years, he had recurrent MS. Case 2: A 39-year-old man presented with severe psychomotor developmental delay. At the age of 1 year, he experienced epileptic seizures. At 6 years, he had recurrent MS. Ictal electroencephalography (I-EEG) of cases 1 and 2 revealed rhythmic alpha activities. Case 3: A 5-year-old boy experienced epileptic seizures and deterioration since 4 years of age. At 5 years, he had MS. I-EEG revealed diffuse bilateral slow spike-wave discharge. Cerebral spinal fluid examination was positive for N-methyl-D-aspartate receptor antibody. Steroid pulse therapy was effective in this case. All the cases had common clinical features of MEND and one case might have a pathology associated with autoimmunity.
