Death in Unverricht - Lundborg disease

Hela Mrabet Khiari, Silvana Franceschetti, Nebojsa Jovic, Amel Mrabet, Pierre Genton

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)315-318
Number of pages4
JournalNeurological Sciences
Issue number4
Publication statusPublished - 2009


  • Death
  • Epilepsy
  • Multicentric study
  • Unverricht Lundborg disease

ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Dermatology


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