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Epilepsia partialis continua

MedGen UID:
39303
Concept ID:
C0085543
Disease or Syndrome
Synonyms: Epilepsia Partialis Continua; Epilepsies, Kojevnikov's; Epilepsy, Kojevnikov's; Epilepsy, Kojewnikov's; Kojevnikov Epilepsy; Kojevnikov's Epilepsies; Kojevnikov's Epilepsy; Kojewnikov Epilepsy; Kojewnikov's Epilepsy; Kojewnikow Syndrome; Kojewnikow's Syndrome; Kozhevnikov Syndrome; Kozhevnikov's Syndrome
SNOMED CT: Motor simple partial status (241006); Epilepsia partialis continua (241006); Kojewnikov's epilepsy (241006); Focal status epilepticus (241006); Kojevnikov epilepsy (241006)
 
HPO: HP:0012847
Monarch Initiative: MONDO:0006748

Definition

Epilepsia partialis continua (also called Kojevnikov's or Kozhevnikov's epilepsia) is a type of focal motor status epilepticus characterized by repeated stereotyped simple motor manifestations such as jerks, typically of a limb or the face, recurring every few seconds or minutes for extended periods (days or years). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpilepsia partialis continua

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+").
Infantile onset spinocerebellar ataxia
MedGen UID:
338613
Concept ID:
C1849096
Disease or Syndrome
Infantile-onset spinocerebellar ataxia (IOSCA) is a severe, progressive neurodegenerative disorder characterized by normal development until age one year, followed by onset of ataxia, muscle hypotonia, loss of deep-tendon reflexes, and athetosis. Ophthalmoplegia and sensorineural deafness develop by age seven years. By adolescence, affected individuals are profoundly deaf and no longer ambulatory; sensory axonal neuropathy, optic atrophy, autonomic nervous system dysfunction, and hypergonadotropic hypogonadism in females become evident. Epilepsy can develop into a serious and often fatal encephalopathy: myoclonic jerks or focal clonic seizures that progress to epilepsia partialis continua followed by status epilepticus with loss of consciousness.
Autosomal recessive ataxia due to ubiquinone deficiency
MedGen UID:
436985
Concept ID:
C2677589
Disease or Syndrome
Primary coenzyme Q10 (CoQ10) deficiency is usually associated with multisystem involvement, including neurologic manifestations such as fatal neonatal encephalopathy with hypotonia; a late-onset slowly progressive multiple-system atrophy-like phenotype (neurodegeneration with autonomic failure and various combinations of parkinsonism and cerebellar ataxia, and pyramidal dysfunction); and dystonia, spasticity, seizures, and intellectual disability. Steroid-resistant nephrotic syndrome (SRNS), the hallmark renal manifestation, is often the initial manifestation either as isolated renal involvement that progresses to end-stage renal disease (ESRD), or associated with encephalopathy (seizures, stroke-like episodes, severe neurologic impairment) resulting in early death. Hypertrophic cardiomyopathy (HCM), retinopathy or optic atrophy, and sensorineural hearing loss can also be seen.
Mitochondrial DNA depletion syndrome 17
MedGen UID:
1684823
Concept ID:
C5231412
Disease or Syndrome
Mitochondrial depletion syndrome-17 (MTDPS17) is an autosomal recessive dystonic or movement disorder (summary by Shafique et al., 2023). For a discussion of genetic heterogeneity of autosomal recessive mtDNA depletion syndromes, see MTDPS1 (603041).
Combined oxidative phosphorylation deficiency 58
MedGen UID:
1841277
Concept ID:
C5830641
Disease or Syndrome
Combined oxidative phosphorylation deficiency-58 (COXPD58) is an autosomal recessive disorder characterized by a wide range of clinical presentations including neonatal lactic acidosis, epileptic encephalopathy, developmental delay and impaired intellectual development with nonspecific changes on brain MRI, or mitochondrial myopathy with a treatable neuromuscular transmission defect (Van Haute et al., 2023). For a discussion of genetic heterogeneity of combined oxidative phosphorylation deficiency, see COXPD1 (609060).

Professional guidelines

PubMed

Carrette S, Boon P, Dekeyser C, Klooster DC, Carrette E, Meurs A, Raedt R, Baeken C, Vanhove C, Aldenkamp AP, Vonck K
Expert Rev Neurother 2016 Sep;16(9):1093-110. Epub 2016 Jun 30 doi: 10.1080/14737175.2016.1197119. PMID: 27254399
Bien CG, Elger CE
Epileptic Disord 2008 Mar;10(1):3-7. doi: 10.1684/epd.2008.0161. PMID: 18367424
Graus F, Dalmau J
Curr Opin Neurol 2007 Dec;20(6):732-7. doi: 10.1097/WCO.0b013e3282f189dc. PMID: 17992098

Recent clinical studies

Etiology

Jagtap SA, Deshmukh Y, Joshi A, Patil S, Kurwale N, Nilegaonkar S
Epileptic Disord 2023 Jun;25(3):321-330. Epub 2023 Apr 20 doi: 10.1002/epd2.20033. PMID: 36939721
Gutiérrez-Viedma Á, Romeral-Jiménez M, Serrano-García I, Parejo-Carbonell B, Cuadrado-Pérez ML, Sanz-Graciani I, García-Morales I
Neurologia (Engl Ed) 2022 May;37(4):263-270. Epub 2021 Apr 6 doi: 10.1016/j.nrleng.2019.03.020. PMID: 35595402
Atmaca MM, Bebek N, Kocasoy-Orhan E, Gürses C
Clin Neurol Neurosurg 2018 Aug;171:143-150. Epub 2018 Jun 9 doi: 10.1016/j.clineuro.2018.06.004. PMID: 29909187
Bentes C, Franco AC, Peralta AR, Viana P, Martins H, Morgado C, Casimiro C, Fonseca C, Geraldes R, Canhão P, Pinho E Melo T, Paiva T, Ferro JM
Eur J Neurol 2017 Jul;24(7):929-934. Epub 2017 May 12 doi: 10.1111/ene.13310. PMID: 28497610
Li H, Xue J, Qian P, Zhang Y, Bao X, Liu X, Yang Z
Brain Dev 2017 Jun;39(6):506-514. Epub 2017 Feb 21 doi: 10.1016/j.braindev.2017.01.011. PMID: 28238390

Diagnosis

Muthaffar OY, Alyazidi AS
Neurosciences (Riyadh) 2024 May;29(2):71-76. doi: 10.17712/nsj.2024.2.20230074. PMID: 38740401
Cruz-Flores S
Curr Neurol Neurosci Rep 2021 Mar 11;21(5):21. doi: 10.1007/s11910-021-01105-2. PMID: 33709163
Atmaca MM, Bebek N, Kocasoy-Orhan E, Gürses C
Clin Neurol Neurosurg 2018 Aug;171:143-150. Epub 2018 Jun 9 doi: 10.1016/j.clineuro.2018.06.004. PMID: 29909187
Granata T, Andermann F
Handb Clin Neurol 2013;111:511-9. doi: 10.1016/B978-0-444-52891-9.00054-3. PMID: 23622199
Saneto RP, Cohen BH, Copeland WC, Naviaux RK
Pediatr Neurol 2013 Mar;48(3):167-78. doi: 10.1016/j.pediatrneurol.2012.09.014. PMID: 23419467Free PMC Article

Therapy

Muthaffar OY, Alyazidi AS
Neurosciences (Riyadh) 2024 May;29(2):71-76. doi: 10.17712/nsj.2024.2.20230074. PMID: 38740401
Li H, Xue J, Qian P, Zhang Y, Bao X, Liu X, Yang Z
Brain Dev 2017 Jun;39(6):506-514. Epub 2017 Feb 21 doi: 10.1016/j.braindev.2017.01.011. PMID: 28238390
Steve TA, Gross DW
Epileptic Disord 2014 Mar;16(1):107-11. doi: 10.1684/epd.2014.0626. PMID: 24556427
Granata T
Neurol Sci 2003 Oct;24 Suppl 4:S239-43. doi: 10.1007/s10072-003-0086-2. PMID: 14598051
Kristiansen K, Kaada BR, Henriksen GF
Epilepsia 1971 Sep;12(3):263-7. doi: 10.1111/j.1528-1157.1971.tb04933.x. PMID: 5004983

Prognosis

Jagtap SA, Deshmukh Y, Joshi A, Patil S, Kurwale N, Nilegaonkar S
Epileptic Disord 2023 Jun;25(3):321-330. Epub 2023 Apr 20 doi: 10.1002/epd2.20033. PMID: 36939721
Gutiérrez-Viedma Á, Romeral-Jiménez M, Serrano-García I, Parejo-Carbonell B, Cuadrado-Pérez ML, Sanz-Graciani I, García-Morales I
Neurologia (Engl Ed) 2022 May;37(4):263-270. Epub 2021 Apr 6 doi: 10.1016/j.nrleng.2019.03.020. PMID: 35595402
Cruz-Flores S
Curr Neurol Neurosci Rep 2021 Mar 11;21(5):21. doi: 10.1007/s11910-021-01105-2. PMID: 33709163
Orsini A, Foiadelli T, Carli N, Costagliola G, Masini B, Bonuccelli A, Savasta S, Peroni D, Consolini R, Striano P
Seizure 2020 Oct;81:76-83. Epub 2020 Jul 25 doi: 10.1016/j.seizure.2020.07.023. PMID: 32769034
Atmaca MM, Bebek N, Kocasoy-Orhan E, Gürses C
Clin Neurol Neurosurg 2018 Aug;171:143-150. Epub 2018 Jun 9 doi: 10.1016/j.clineuro.2018.06.004. PMID: 29909187

Clinical prediction guides

Jagtap SA, Deshmukh Y, Joshi A, Patil S, Kurwale N, Nilegaonkar S
Epileptic Disord 2023 Jun;25(3):321-330. Epub 2023 Apr 20 doi: 10.1002/epd2.20033. PMID: 36939721
Gutiérrez-Viedma Á, Romeral-Jiménez M, Serrano-García I, Parejo-Carbonell B, Cuadrado-Pérez ML, Sanz-Graciani I, García-Morales I
Neurologia (Engl Ed) 2022 May;37(4):263-270. Epub 2021 Apr 6 doi: 10.1016/j.nrleng.2019.03.020. PMID: 35595402
Orsini A, Foiadelli T, Carli N, Costagliola G, Masini B, Bonuccelli A, Savasta S, Peroni D, Consolini R, Striano P
Seizure 2020 Oct;81:76-83. Epub 2020 Jul 25 doi: 10.1016/j.seizure.2020.07.023. PMID: 32769034
Vogrig A, Gigli GL, Nilo A, Pessa ME, Volpetti S, Pegolo E, Valente M
J Neurovirol 2020 Jun;26(3):437-441. Epub 2019 Dec 5 doi: 10.1007/s13365-019-00821-3. PMID: 31807988
Bentes C, Franco AC, Peralta AR, Viana P, Martins H, Morgado C, Casimiro C, Fonseca C, Geraldes R, Canhão P, Pinho E Melo T, Paiva T, Ferro JM
Eur J Neurol 2017 Jul;24(7):929-934. Epub 2017 May 12 doi: 10.1111/ene.13310. PMID: 28497610

Recent systematic reviews

Licchetta L, Ferri L, Morsillo F, Faustini-Fustini M, Toni F, Pondrelli F, Nonino F, Bisulli F, Tinuper P
Seizure 2023 Mar;106:50-57. Epub 2023 Jan 27 doi: 10.1016/j.seizure.2023.01.018. PMID: 36773573
Dibué-Adjei M, Brigo F, Yamamoto T, Vonck K, Trinka E
Brain Stimul 2019 Sep-Oct;12(5):1101-1110. Epub 2019 May 14 doi: 10.1016/j.brs.2019.05.011. PMID: 31126871
Dupont S, Gales A, Sammey S, Vidailhet M, Lambrecq V
Autoimmun Rev 2017 Aug;16(8):803-810. Epub 2017 May 29 doi: 10.1016/j.autrev.2017.05.022. PMID: 28572051
Sweeney M, Sweney M, Soldán MM, Clardy SL
Pediatr Neurol 2016 Dec;65:86-89. Epub 2016 Sep 2 doi: 10.1016/j.pediatrneurol.2016.08.024. PMID: 27707529
Pereira LS, Müller VT, da Mota Gomes M, Rotenberg A, Fregni F
Epilepsy Behav 2016 Apr;57(Pt A):167-176. Epub 2016 Mar 10 doi: 10.1016/j.yebeh.2016.01.015. PMID: 26970993

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