U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Generalized non-convulsive status epilepticus without coma

MedGen UID:
124372
Concept ID:
C0270823
Disease or Syndrome
Synonyms: Absence Status; Petit Mal Status; Status, Absence; Status, Petit Mal
SNOMED CT: Petit mal status (7033004); Epileptic absence status (7033004); Absence status epilepticus (7033004); Non-convulsive status epilepticus with impaired consciousness (7033004); Prolonged epileptic twilight state (7033004); Epilepsia minoris continua (7033004); Petit-mal status (7033004); Status pyknolepticus (7033004); Spike wave stupor (7033004)
 
HPO: HP:0032860

Definition

Generalized non-convulsive status epilepticus without coma is a type of status epilepticus without prominent motor signs, which is electrographically generalized. It is a prolonged absence seizure. [from HPO]

Conditions with this feature

Progressive sclerosing poliodystrophy
MedGen UID:
60012
Concept ID:
C0205710
Disease or Syndrome
POLG-related disorders comprise a continuum of overlapping phenotypes that were clinically defined long before their molecular basis was known. Most affected individuals have some, but not all, of the features of a given phenotype; nonetheless, the following nomenclature can assist the clinician in diagnosis and management. Onset of the POLG-related disorders ranges from infancy to late adulthood. Alpers-Huttenlocher syndrome (AHS), one of the most severe phenotypes, is characterized by childhood-onset progressive and ultimately severe encephalopathy with intractable epilepsy and hepatic failure. Childhood myocerebrohepatopathy spectrum (MCHS) presents between the first few months of life and about age three years with developmental delay or dementia, lactic acidosis, and a myopathy with failure to thrive. Other findings can include liver failure, renal tubular acidosis, pancreatitis, cyclic vomiting, and hearing loss. Myoclonic epilepsy myopathy sensory ataxia (MEMSA) now describes the spectrum of disorders with epilepsy, myopathy, and ataxia without ophthalmoplegia. MEMSA now includes the disorders previously described as spinocerebellar ataxia with epilepsy (SCAE). The ataxia neuropathy spectrum (ANS) includes the phenotypes previously referred to as mitochondrial recessive ataxia syndrome (MIRAS) and sensory ataxia neuropathy dysarthria and ophthalmoplegia (SANDO). About 90% of persons in the ANS have ataxia and neuropathy as core features. Approximately two thirds develop seizures and almost one half develop ophthalmoplegia; clinical myopathy is rare. Autosomal recessive progressive external ophthalmoplegia (arPEO) is characterized by progressive weakness of the extraocular eye muscles resulting in ptosis and ophthalmoparesis (or paresis of the extraocular muscles) without associated systemic involvement; however, caution is advised because many individuals with apparently isolated arPEO at the onset develop other manifestations of POLG-related disorders over years or decades. Of note, in the ANS spectrum the neuropathy commonly precedes the onset of PEO by years to decades. Autosomal dominant progressive external ophthalmoplegia (adPEO) typically includes a generalized myopathy and often variable degrees of sensorineural hearing loss, axonal neuropathy, ataxia, depression, parkinsonism, hypogonadism, and cataracts (in what has been called "chronic progressive external ophthalmoplegia plus," or "CPEO+").

Professional guidelines

PubMed

Charalambous M, Muñana K, Patterson EE, Platt SR, Volk HA
J Vet Intern Med 2024 Jan-Feb;38(1):19-40. Epub 2023 Nov 3 doi: 10.1111/jvim.16928. PMID: 37921621Free PMC Article
Trinka E, Leitinger M
Continuum (Minneap Minn) 2022 Apr 1;28(2):559-602. doi: 10.1212/CON.0000000000001103. PMID: 35393970
Brophy GM, Bell R, Claassen J, Alldredge B, Bleck TP, Glauser T, Laroche SM, Riviello JJ Jr, Shutter L, Sperling MR, Treiman DM, Vespa PM; Neurocritical Care Society Status Epilepticus Guideline Writing Committee
Neurocrit Care 2012 Aug;17(1):3-23. doi: 10.1007/s12028-012-9695-z. PMID: 22528274

Recent clinical studies

Etiology

Wang X, Yang F, Chen B, Jiang W
Acta Neurol Scand 2022 Dec;146(6):752-760. Epub 2022 Oct 26 doi: 10.1111/ane.13718. PMID: 36285875
Samanta D, Garrity L, Arya R
Indian Pediatr 2020 Mar 15;57(3):239-253. doi: 10.1007/s13312-020-1759-0. PMID: 32198865Free PMC Article
Dupont S
Geriatr Psychol Neuropsychiatr Vieil 2019 Mar 1;17(S1):25-30. doi: 10.1684/pnv.2019.0782. PMID: 30916648
Trinka E, Cock H, Hesdorffer D, Rossetti AO, Scheffer IE, Shinnar S, Shorvon S, Lowenstein DH
Epilepsia 2015 Oct;56(10):1515-23. Epub 2015 Sep 4 doi: 10.1111/epi.13121. PMID: 26336950
Leitinger M, Beniczky S, Rohracher A, Gardella E, Kalss G, Qerama E, Höfler J, Hess Lindberg-Larsen A, Kuchukhidze G, Dobesberger J, Langthaler PB, Trinka E
Epilepsy Behav 2015 Aug;49:158-63. Epub 2015 Jun 17 doi: 10.1016/j.yebeh.2015.05.007. PMID: 26092326

Diagnosis

Coppler PJ, Elmer J
Crit Care Clin 2023 Jan;39(1):87-102. Epub 2022 Oct 7 doi: 10.1016/j.ccc.2022.07.006. PMID: 36333039
Trinka E, Leitinger M
Continuum (Minneap Minn) 2022 Apr 1;28(2):559-602. doi: 10.1212/CON.0000000000001103. PMID: 35393970
Trinka E, Cock H, Hesdorffer D, Rossetti AO, Scheffer IE, Shinnar S, Shorvon S, Lowenstein DH
Epilepsia 2015 Oct;56(10):1515-23. Epub 2015 Sep 4 doi: 10.1111/epi.13121. PMID: 26336950
Betjemann JP, Lowenstein DH
Lancet Neurol 2015 Jun;14(6):615-24. Epub 2015 Apr 20 doi: 10.1016/S1474-4422(15)00042-3. PMID: 25908090
Brophy GM, Bell R, Claassen J, Alldredge B, Bleck TP, Glauser T, Laroche SM, Riviello JJ Jr, Shutter L, Sperling MR, Treiman DM, Vespa PM; Neurocritical Care Society Status Epilepticus Guideline Writing Committee
Neurocrit Care 2012 Aug;17(1):3-23. doi: 10.1007/s12028-012-9695-z. PMID: 22528274

Therapy

Rossetti AO, Claassen J, Gaspard N
Intensive Care Med 2024 Jan;50(1):1-16. Epub 2023 Dec 20 doi: 10.1007/s00134-023-07263-w. PMID: 38117319
Coppler PJ, Elmer J
Crit Care Clin 2023 Jan;39(1):87-102. Epub 2022 Oct 7 doi: 10.1016/j.ccc.2022.07.006. PMID: 36333039
Sánchez Fernández I, Goodkin HP, Scott RC
Seizure 2019 May;68:16-21. Epub 2018 Aug 6 doi: 10.1016/j.seizure.2018.08.002. PMID: 30170734Free PMC Article
Betjemann JP, Lowenstein DH
Lancet Neurol 2015 Jun;14(6):615-24. Epub 2015 Apr 20 doi: 10.1016/S1474-4422(15)00042-3. PMID: 25908090
Brophy GM, Bell R, Claassen J, Alldredge B, Bleck TP, Glauser T, Laroche SM, Riviello JJ Jr, Shutter L, Sperling MR, Treiman DM, Vespa PM; Neurocritical Care Society Status Epilepticus Guideline Writing Committee
Neurocrit Care 2012 Aug;17(1):3-23. doi: 10.1007/s12028-012-9695-z. PMID: 22528274

Prognosis

Trinka E, Leitinger M
Continuum (Minneap Minn) 2022 Apr 1;28(2):559-602. doi: 10.1212/CON.0000000000001103. PMID: 35393970
Specchio N, Pietrafusa N
Dev Med Child Neurol 2020 Aug;62(8):897-905. Epub 2020 May 5 doi: 10.1111/dmcn.14553. PMID: 32372459
Dupont S
Geriatr Psychol Neuropsychiatr Vieil 2019 Mar 1;17(S1):25-30. doi: 10.1684/pnv.2019.0782. PMID: 30916648
Sculier C, Gaspard N
Seizure 2019 May;68:72-78. Epub 2018 Sep 29 doi: 10.1016/j.seizure.2018.09.018. PMID: 30482654
Patterson JL, Carapetian SA, Hageman JR, Kelley KR
Pediatr Ann 2013 Dec;42(12):249-54. doi: 10.3928/00904481-20131122-09. PMID: 24295158

Clinical prediction guides

Benghanem S, Robieux EP, Neligan A, Walker MC
Curr Opin Crit Care 2024 Apr 1;30(2):131-141. Epub 2024 Feb 13 doi: 10.1097/MCC.0000000000001137. PMID: 38441162
Trinka E, Leitinger M
Continuum (Minneap Minn) 2022 Apr 1;28(2):559-602. doi: 10.1212/CON.0000000000001103. PMID: 35393970
Rosenthal ES
Continuum (Minneap Minn) 2021 Oct 1;27(5):1321-1343. doi: 10.1212/CON.0000000000001012. PMID: 34618762
Nelson SE, Varelas PN
Continuum (Minneap Minn) 2018 Dec;24(6):1683-1707. doi: 10.1212/CON.0000000000000668. PMID: 30516601
Leitinger M, Beniczky S, Rohracher A, Gardella E, Kalss G, Qerama E, Höfler J, Hess Lindberg-Larsen A, Kuchukhidze G, Dobesberger J, Langthaler PB, Trinka E
Epilepsy Behav 2015 Aug;49:158-63. Epub 2015 Jun 17 doi: 10.1016/j.yebeh.2015.05.007. PMID: 26092326

Recent systematic reviews

Jain P, Aneja S, Cunningham J, Arya R, Sharma S
Seizure 2022 Nov;102:74-82. Epub 2022 Sep 26 doi: 10.1016/j.seizure.2022.09.017. PMID: 36209676
Maan G, Keitoku K, Kimura N, Sawada H, Pham A, Yeo J, Hagiya H, Nishimura Y
J Antimicrob Chemother 2022 Oct 28;77(11):2908-2921. doi: 10.1093/jac/dkac271. PMID: 35971666
Jafarpour S, Hirsch LJ, Gaínza-Lein M, Kellinghaus C, Detyniecki K
Seizure 2019 May;68:9-15. Epub 2018 May 21 doi: 10.1016/j.seizure.2018.05.013. PMID: 29871784
Payne LE, Gagnon DJ, Riker RR, Seder DB, Glisic EK, Morris JG, Fraser GL
Crit Care 2017 Nov 14;21(1):276. doi: 10.1186/s13054-017-1856-1. PMID: 29137682Free PMC Article
Strzelczyk A, Zöllner JP, Willems LM, Jost J, Paule E, Schubert-Bast S, Rosenow F, Bauer S
Epilepsia 2017 Jun;58(6):933-950. Epub 2017 Mar 11 doi: 10.1111/epi.13716. PMID: 28295226

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...