Myoclonic status in nonprogressive encephalopathies

An update

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Myoclonic status in nonprogressive encephalopathies (MSNE) is an epileptic syndrome in development, characterized by the early onset of continuous diffuse epileptiform abnormalities, accompanied by positive and/or negative phenomena correlated with transient and recurring motor, cognitive, or behavioral disturbances. Outcome of MSNE is poor and may determine progressive neurologic deterioration. MSNE is not easy to recognize and should be distinguished from progressive myoclonic epilepsies and other rarely reported infantile myoclonic epilepsies. The identification of MSNE may guide the diagnostic work-up, suggesting the presence of a genetic defect, such as Angelman syndrome, hypoxic-ischemic encephalopathy, or brain malformation.

Original languageEnglish
Pages (from-to)41-44
Number of pages4
JournalEpilepsia
Volume50
Issue numberSUPPL. 5
DOIs
Publication statusPublished - May 2009

Fingerprint

Brain Diseases
Progressive Myoclonic Epilepsy
Angelman Syndrome
Myoclonic Epilepsy
Brain Hypoxia-Ischemia
Nervous System
Brain

Keywords

  • Cognitive impairment
  • EEG
  • Myoclonus
  • Status epilepticus

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Myoclonic status in nonprogressive encephalopathies : An update. / Elia, Maurizio.

In: Epilepsia, Vol. 50, No. SUPPL. 5, 05.2009, p. 41-44.

Research output: Contribution to journalArticle

@article{bdbf9aa928944cfab66abe6322ed8b63,
title = "Myoclonic status in nonprogressive encephalopathies: An update",
abstract = "Myoclonic status in nonprogressive encephalopathies (MSNE) is an epileptic syndrome in development, characterized by the early onset of continuous diffuse epileptiform abnormalities, accompanied by positive and/or negative phenomena correlated with transient and recurring motor, cognitive, or behavioral disturbances. Outcome of MSNE is poor and may determine progressive neurologic deterioration. MSNE is not easy to recognize and should be distinguished from progressive myoclonic epilepsies and other rarely reported infantile myoclonic epilepsies. The identification of MSNE may guide the diagnostic work-up, suggesting the presence of a genetic defect, such as Angelman syndrome, hypoxic-ischemic encephalopathy, or brain malformation.",
keywords = "Cognitive impairment, EEG, Myoclonus, Status epilepticus",
author = "Maurizio Elia",
year = "2009",
month = "5",
doi = "10.1111/j.1528-1167.2009.02119.x",
language = "English",
volume = "50",
pages = "41--44",
journal = "Epilepsia",
issn = "0013-9580",
publisher = "Blackwell Publishing Inc.",
number = "SUPPL. 5",

}

TY - JOUR

T1 - Myoclonic status in nonprogressive encephalopathies

T2 - An update

AU - Elia, Maurizio

PY - 2009/5

Y1 - 2009/5

N2 - Myoclonic status in nonprogressive encephalopathies (MSNE) is an epileptic syndrome in development, characterized by the early onset of continuous diffuse epileptiform abnormalities, accompanied by positive and/or negative phenomena correlated with transient and recurring motor, cognitive, or behavioral disturbances. Outcome of MSNE is poor and may determine progressive neurologic deterioration. MSNE is not easy to recognize and should be distinguished from progressive myoclonic epilepsies and other rarely reported infantile myoclonic epilepsies. The identification of MSNE may guide the diagnostic work-up, suggesting the presence of a genetic defect, such as Angelman syndrome, hypoxic-ischemic encephalopathy, or brain malformation.

AB - Myoclonic status in nonprogressive encephalopathies (MSNE) is an epileptic syndrome in development, characterized by the early onset of continuous diffuse epileptiform abnormalities, accompanied by positive and/or negative phenomena correlated with transient and recurring motor, cognitive, or behavioral disturbances. Outcome of MSNE is poor and may determine progressive neurologic deterioration. MSNE is not easy to recognize and should be distinguished from progressive myoclonic epilepsies and other rarely reported infantile myoclonic epilepsies. The identification of MSNE may guide the diagnostic work-up, suggesting the presence of a genetic defect, such as Angelman syndrome, hypoxic-ischemic encephalopathy, or brain malformation.

KW - Cognitive impairment

KW - EEG

KW - Myoclonus

KW - Status epilepticus

UR - http://www.scopus.com/inward/record.url?scp=65549131047&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=65549131047&partnerID=8YFLogxK

U2 - 10.1111/j.1528-1167.2009.02119.x

DO - 10.1111/j.1528-1167.2009.02119.x

M3 - Article

VL - 50

SP - 41

EP - 44

JO - Epilepsia

JF - Epilepsia

SN - 0013-9580

IS - SUPPL. 5

ER -