title: Death in Unverricht-Lundborg disease.
abstract: The objective of this study is to report cases of unexpected deaths in Unverricht-Lundborg disease (ULD) patients, a comparatively benign form of progressive myoclonus epilepsy. We performed a multicentric study of the circumstances of death in ULD patients seen in the last 16 years. We assessed age, sex, severity and duration of disease, antiepileptic drugs, circumstances and presumed cause of death. Nineteen observations (12 females, 7 males) were collected from four centers (Tunis, Marseille, Milan, Belgrade). The most common causes of death are (1) SUDEP (six cases, all female), with 4/6 on phenobarbital alone, and (2) complications of severe ULD (six cases). Two patients committed suicide. Only one death was clearly unrelated to ULD (car accident), while four patients died of stroke, drowning, complications of chronic alcoholism and Wernicke encephalopathy, respectively. In conclusion, although the prognosis of ULD has progressed, there are still spontaneously severe forms and high risk of early death, including SUDEP.
authors.affiliation: Neurological Department, Charles Nicolle Hospital, Boulevard 9 Avril, 1006, Tunis, Tunisia. helamrabet@yahoo.com
authors.forename: Hela Mrabet
authors.initials: HM
authors.lastname: Khiari
authors.forename: Silvana
authors.initials: S
authors.lastname: Franceschetti
authors.forename: Nebojsa
authors.initials: N
authors.lastname: Jovic
authors.forename: Amel
authors.initials: A
authors.lastname: Mrabet
authors.forename: Pierre
authors.initials: P
authors.lastname: Genton
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D002423:Cause of Death; D002648:Child; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012737:Sex Factors; D013226:Status Epilepticus / Q000209:etiology; D013405:Suicide; D011795:Surveys and Questionnaires; D020194:Unverricht-Lundborg Syndrome / Q000235:genetics / Q000401:mortality*; D055815:Young Adult
chemical_list: D000927:Anticonvulsants