PMID- 16128150
OWN - NLM
STAT- MEDLINE
DCOM- 20050920
LR  - 20170214
IS  - 1550-0594 (Print)
IS  - 1550-0594 (Linking)
VI  - 36
IP  - 3
DP  - 2005 Jul
TI  - Ring chromosome 20 with nonconvulsive status epilepticus: electroclinical 
      correlation of a rare epileptic syndrome.
PG  - 151-60
AB  - The electroclinical features of two Thai women with ring chromosome 20 and 
      nonconvulsive status epilepticus (NCSE) were studied. Both have also had 
      generalized tonic-clonic seizures and complex partial seizures of varying 
      frequencies since adolescence. Their intellectual functions were normal. 
      Twenty-four-hour video/EEG telemetry recorded during the NCSE showed fluctuating 
      consciousness between overt unresponsiveness and normal awareness. The EEG 
      consisted of long-lasting generalized rhythmic 3-5 Hz sharp or slow waves with a 
      few spikes, lasting several days. Despite the continuous discharges, the patients 
      had relatively subtle clinical episodes of seizures, during which they were 
      sometimes responsive to verbal stimuli. Intravenous antiepileptic drugs (AED) had 
      little effect on the rhythmic EEG. No lesion in their MRIs contributed to NCSE. 
      Ring chromosome 20 was found in 20% of female karyotype in both patients 
      [46,XX,r(20) (p13 q13)/46,XX] but were negative in four healthy siblings. Oral 
      AEDs decreased more than 75% of the overt CPS episodes in both patients at 22 and 
      26 months of follow-up but had no effect on the natural history of electrical 
      NCSE. The patients' daily activities were minimally affected by the ongoing 
      electrical discharges. These are the first two cases reported of ring chromosome 
      20 with NCSE in Thailand. Our patients present a rather benign and 
      pharmacologically responsive course probably because of the low percentage of 
      r(20) mosaicism. The electroclinical correlations in our cases raise the 
      possibility that the mechanism of continuous rhythmic waves in this syndrome may 
      be unrelated to epilepsy. Assessing the severity of this syndrome using both 
      clinical seizures and EEG is crucial.
FAU - Locharernkul, Chaichon
AU  - Locharernkul C
AD  - Chulalongkorn Comprehensive Epilepsy Program, King Chulalongkorn Memorial 
      Hospital, Bangkok, Thailand. chaichon@yahoo.com
FAU - Ebner, Alois
AU  - Ebner A
FAU - Promchainant, Chinvorn
AU  - Promchainant C
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Clin EEG Neurosci
JT  - Clinical EEG and neuroscience
JID - 101213033
SB  - IM
MH  - Adult
MH  - Chromosomes, Human, Pair 20/*genetics
MH  - Electroencephalography
MH  - Female
MH  - Humans
MH  - *Ring Chromosomes
MH  - Status Epilepticus/*diagnosis/genetics
MH  - Syndrome
EDAT- 2005/09/01 09:00
MHDA- 2005/09/21 09:00
CRDT- 2005/09/01 09:00
PHST- 2005/09/01 09:00 [pubmed]
PHST- 2005/09/21 09:00 [medline]
PHST- 2005/09/01 09:00 [entrez]
AID - 10.1177/155005940503600305 [doi]
PST - ppublish
SO  - Clin EEG Neurosci. 2005 Jul;36(3):151-60. doi: 10.1177/155005940503600305.