Juvenile myoclonic epilepsy is a comparatively benign form of idiopathic ge
neralised epilepsy. Little is known about the prevalence of difficult to tr
eat or drug resistant patients. Among 155 consecutive patients with newly d
iagnosed juvenile myoclonic epilepsy evaluated between 1981 and 1998 and fo
llowed up for at least 1 year (61 men, 94 women; aged 15-70 years, mean 33
(SD 10.3); onset of juvenile myoclonic epilepsy at the age of 14.5 (SD 3.7)
, range 6-26; follow up 1-52 years, mean 13.5 (SD 9.9)), there were 15 pseu
doresistant patients (9.7%: lack of compliance (eight), insufficient treatm
ent (three), abnormal lifestyle (four)) and 24 patients (15.5%) who had per
sisting seizures despite adequate therapy and lifestyle. Clinical features
associated with drug resistance were (1) the presence of psychiatric proble
ms (58.3% v 19%; chi (2) p<0.001) and (2) independently, the combination of
seizure types (Fischer's exact 2 by 4, p=0.0026). Three types were present
in 62.5% of resistant patients versus 23.3% in non-resistant patients (<ch
i>(2), p=0.0001). None of the resistant patients had myoclonic jerks as the
only seizure type or a combination of absences and myoclonic jerks. Family
history of epilepsy, age at onset of seizures, sex, presence of photoparox
ysmal response, results of conventional neuroimagings (CT and MRI), and del
ayed diagnosis were not significantly associated with drug resistance. Ther
e is thus a significant subgroup of patients with juvenile myoclonic epilep
sy who pose difficult therapeutic problems, and the prevalence of resistant
cases may be increased in the experience of a referral epilepsy centre.