Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Specialty Sites
    • COVID-19
    • Practice Current
    • Practice Buzz
    • Without Borders
    • Equity, Diversity and Inclusion
    • Innovations in Care Delivery
  • Collections
    • Topics A-Z
    • Residents & Fellows
    • Infographics
    • Patient Pages
    • Null Hypothesis
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Specialty Sites
    • COVID-19
    • Practice Current
    • Practice Buzz
    • Without Borders
    • Equity, Diversity and Inclusion
    • Innovations in Care Delivery
  • Collections
    • Topics A-Z
    • Residents & Fellows
    • Infographics
    • Patient Pages
    • Null Hypothesis
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Residents & Fellows

User menu

  • Subscribe
  • My Alerts
  • Log in

Search

  • Advanced search
Neurology
Home
The most widely read and highly cited peer-reviewed neurology journal
  • Subscribe
  • My Alerts
  • Log in
Site Logo
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Residents & Fellows

Share

October 17, 2017; 89 (16) Article

Variable course of Unverricht-Lundborg disease

Early prognostic factors

Laura Canafoglia, Edoardo Ferlazzo, Roberto Michelucci, Pasquale Striano, Adriana Magaudda, Antonio Gambardella, Elena Pasini, Vincenzo Belcastro, Patrizia Riguzzi, Martina Fanella, Tiziana Granata, Francesca Beccaria, Claudia Trentini, Amedeo Bianchi, Umberto Aguglia, Ferruccio Panzica, Silvana Franceschetti
First published September 20, 2017, DOI: https://doi.org/10.1212/WNL.0000000000004518
Laura Canafoglia
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Edoardo Ferlazzo
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Roberto Michelucci
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Pasquale Striano
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Adriana Magaudda
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Antonio Gambardella
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Elena Pasini
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Vincenzo Belcastro
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Patrizia Riguzzi
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Martina Fanella
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Tiziana Granata
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Francesca Beccaria
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Claudia Trentini
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Amedeo Bianchi
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Umberto Aguglia
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ferruccio Panzica
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Silvana Franceschetti
From the Department of Neurophysiopathology and Epilepsy Centre (L.C., C.T., F.P., S.F.) and Pediatric Neurology (T.G.), IRCCS Foundation C. Besta Neurological Institute, Milan; Department of Medical and Surgical Sciences (E.F., A.G., U.A.), Magna Graecia University, Catanzaro; Regional Epilepsy Centre (E.F., U.A.), Bianchi-Melacrino-Morelli Hospital, Reggio Calabria; Unit of Neurology (R.M., E.P., P.R.), IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna; Pediatric Neurology and Muscular Diseases Unit (P.S.), DINOGMI-Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, G. Gaslini Institute; Department of Neuroscience and Epilepsy Centre (A.M.), G. Martino Policlinico AOU, University of Messina; Institute of Neurological Sciences (A.G.), National Research Council, Mangone, Cosenza; Department of Neurology (V.B.), S. Anna Hospital, Como; Department of Neurology and Psychiatry (M.F.), Neurology Unit, Sapienza University, Rome; Epilepsy Center (F.B.), Department of Child Neuropsychiatry, C. Poma Hospital, Mantua; and Department of Neurology and Epilepsy Centre (A.B.), San Donato Hospital, Arezzo, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Full PDF
Citation
Variable course of Unverricht-Lundborg disease
Early prognostic factors
Laura Canafoglia, Edoardo Ferlazzo, Roberto Michelucci, Pasquale Striano, Adriana Magaudda, Antonio Gambardella, Elena Pasini, Vincenzo Belcastro, Patrizia Riguzzi, Martina Fanella, Tiziana Granata, Francesca Beccaria, Claudia Trentini, Amedeo Bianchi, Umberto Aguglia, Ferruccio Panzica, Silvana Franceschetti
Neurology Oct 2017, 89 (16) 1691-1697; DOI: 10.1212/WNL.0000000000004518

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Permissions

Make Comment

See Comments

Downloads
258

Share

  • Article
  • Figures & Data
  • Info & Disclosures
  • CME Course
Loading

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Abstract

Objective: To explore the course of Unverricht-Lundborg disease (EPM1) and identify the risk factors for severity, we investigated the time course of symptoms and prognostic factors already detectable near to disease onset.

Methods: We retrospectively evaluated the features of 59 Italian patients carrying the CSTB expansion mutation, and coded the information every 5 years after the disease onset in order to describe the cumulative time-dependent probability of reaching disabling myoclonus, relevant cognitive impairment, and inability to work, and evaluated the influence of early factors using the log-rank test. The risk factors were included in a Cox multivariate proportional hazards regression model.

Results: Disabling myoclonus occurred an average of 32 years after disease onset, whereas cognitive impairment occurred a little later. An age at onset of less than 12 years, the severity of myoclonus at the time of first assessment, and seizure persistence more than 10 years after onset affected the timing of disabling myoclonus and cognitive decline. Most patients became unable to work years before the appearance of disabling myoclonus or cognitive decline.

Conclusions: A younger age at onset, early severe myoclonus, and seizure persistence are predictors of a more severe outcome. All of these factors may be genetically determined, but the greater hyperexcitability underlying more severe seizures and myoclonus at onset may also play a role by increasing cell damage due to reduced cystatin B activity.

GLOSSARY

ANOVA=
analysis of variance;
KM=
Kaplan-Meier;
PHT=
phenytoin;
PME=
progressive myoclonus epilepsy;
PPR=
photoparoxysmal responses;
PSW=
polyspikes and waves;
SSEP=
somatosensory evoked potential;
VPA=
valproate

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Received March 8, 2017.
  • Accepted in final form July 23, 2017.
  • © 2017 American Academy of Neurology
View Full Text

AAN Members

We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.

Google Safari Microsoft Edge Firefox

Click here to login

AAN Non-Member Subscribers

Click here to login

Purchase access

For assistance, please contact:
AAN Members (800) 879-1960 or (612) 928-6000 (International)
Non-AAN Member subscribers (800) 638-3030 or (301) 223-2300 option 3, select 1 (international)

Sign Up
Information on how to subscribe to Neurology and Neurology: Clinical Practice can be found here 

Purchase
Individual access to articles is available through the Add to Cart option on the article page.  Access for 1 day (from the computer you are currently using) is US$ 39.00.  Pay-per-view content is for the use of the payee only, and content may not be further distributed by print or electronic means.  The payee may view, download, and/or print the article for his/her personal, scholarly, research, and educational use.  Distributing copies (electronic or otherwise) of the article is not allowed.

Disputes & Debates: Rapid online correspondence

No comments have been published for this article.
Comment

NOTE: All authors' disclosures must be entered and current in our database before comments can be posted. Enter and update disclosures at http://submit.neurology.org. Exception: replies to comments concerning an article you originally authored do not require updated disclosures.

  • Stay timely. Submit only on articles published within the last 8 weeks.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • 200 words maximum.
  • 5 references maximum. Reference 1 must be the article on which you are commenting.
  • 5 authors maximum. Exception: replies can include all original authors of the article.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Disputes & Debates Submission Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
  • Article
    • Abstract
    • GLOSSARY
    • METHODS
    • RESULTS
    • DISCUSSION
    • AUTHOR CONTRIBUTIONS
    • STUDY FUNDING
    • DISCLOSURE
    • Footnotes
    • REFERENCES
  • Figures & Data
  • Info & Disclosures
  • CME Course

More Online

CME Course

Topics Discussed

  • EEG
  • Natural history studies (prognosis)
  • Generalized seizures

Alert Me

  • Alert me when eletters are published
Neurology: 96 (9)

Articles

  • Ahead of Print
  • Current Issue
  • Past Issues
  • Popular Articles
  • Translations

About

  • About the Journals
  • Ethics Policies
  • Editors & Editorial Board
  • Contact Us
  • Advertise

Submit

  • Author Center
  • Submit a Manuscript
  • Information for Reviewers
  • AAN Guidelines
  • Permissions

Subscribers

  • Subscribe
  • Activate a Subscription
  • Sign up for eAlerts
  • RSS Feed
Site Logo
  • Visit neurology Template on Facebook
  • Follow neurology Template on Twitter
  • Visit Neurology on YouTube
  • Neurology
  • Neurology: Clinical Practice
  • Neurology: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology | Print ISSN:0028-3878
Online ISSN:1526-632X

© 2021 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise