Blepharospasm and Primary Generalized Epilepsy with Photosensitivity: An Interesting but Confusing Association
Abstract number :
2.095
Submission category :
Year :
2000
Submission ID :
1554
Source :
www.aesnet.org
Presentation date :
12/2/2000 12:00:00 AM
Published date :
Dec 1, 2000, 06:00 AM
Authors :
Carol S Camfield, Peter R Camfield, Mark R Sadler, IWK Grace Health Ctr, Dalhousie Univ, Halifax, NS, Canada; QE II Health Science Ctr, Dalhousie Univ, Halifax, NS, Canada.
RATIONALE: Blepharospasm is an involuntary dystonic closure of eyelids. We observed this disorder in 10 children and adults with primary generalized epilepsy. METHODS: Patients were identified by physician recall with chart and EEG review and direct patient contact for confirmation of details. RESULTS: All 10 patients are female and currently have active idiopathic epilepsy requiring AED's - 4 with childhood absence epilepsy (CAE) and 6 with juvenile myoclonic epilepsy (JME). The age of onset of epilepsy was 4.5 years in CAE and 13 years in JME. In 2 children with JME, blepharospasm began 2-4 years before their epilepsy was noted - in the remainder it was noted when epilepsy was first diagnosed. Age at last follow up was 8-33 years (ave: 21 years) with average follow-up of 9 years. All patients showed photosensitive generalized spike wave discharges on EEG. Blepharospasm was a source of diagnostic confusion in those with absence but direct examination during EEG recording provided a definite diagnosis. Video of the blepharospasm showed differences from absence seizures - the eyelid flutter was more rapid, eyelid closure was more complete, the eyes deviated strongly upward, upper facial muscles contracted often raising the eyebrow, the lower face did not slack and there were no automatisms. Of course, during blepharospasm the patient was fully conscious. The length of blepharospasm and absence attacks was similar, although we thought that blepharospasm was somewhat shorter. In all cases, the epilepsy has come under good to excellent control with AED's but there has been no resolution of the blepharospasm with age. Mothers of 3 cases also had blepharospasm but not epilepsy. We have not noted blepharospasm in patients with other epilepsy types. CONCLUSIONS: The association between blepharospasm and photosensitive primary generalized epilepsy appears to be too frequent to be by chance alone. A genetic link is postulated. Blepharospasm must be carefully assessed in patients with absence seizures to avoid treatment errors.