Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Epileptic and Epileptiform Encephalopathies Clinical Presentation

  • Author: Dean Patrick Sarco, MD; Chief Editor: Amy Kao, MD  more...
 
Updated: Dec 15, 2015
 

History

The history in patients with epileptic encephalopathy varies with the specific syndrome.

Early infantile epileptic encephalopathy (Ohtahara syndrome)

Early infantile epileptic encephalopathy (EIEE) is a rare disorder characterized by early-onset seizures in the neonatal period, which may begin as early as the first few days of life. Brief generalized tonic seizures typically occur first, occasionally in clusters. Focal motor and hemiconvulsive seizures may occur in up to half of cases.[32]

Most children are symptomatic from structural brain abnormalities such as cerebral dysgenesis, although metabolic disorders are also reported.[42]

Early myoclonic encephalopathy

Early myoclonic encephalopathy (EME) is a rare disorder characterized by neonatal-onset seizures, usually within the first month of life.

Seizures are mostly myoclonic and partial motor seizures. Myoclonic seizures may be focal, occasionally very subtle, and may become frequent. Tonic spasms may develop later. This is different from EIEE, in which tonic seizures appear early.

Infantile spasms (West syndrome)

West syndrome usually occurs in the first year of life and consists of the triad of infantile spasms, developmental deterioration, and a hypsarrhythmia pattern on EEG.

The epileptic spasms are brief, generalized seizures involving extension and/or flexion axially and of the extremities. An individual spasm lasts seconds, often longer than typical myoclonic seizures, though not as long as most tonic seizures.

The spasms may be subtle and may be isolated at onset, typically clustering later in the course. Several clusters per day, particularly in drowsiness, are characteristic.

Malignant epilepsy with migrating partial seizures in infancy

Onset of this rare syndrome occurs in the first year of life, in some cases in the neonatal period. It is characterized by frequent partial seizures of multifocal onset, with autonomic or motor involvement. The seizures increase in frequency and may become nearly continuous.

Severe myoclonic epilepsy of infancy (Dravet syndrome)

Severe myoclonic epilepsy of infancy (SMEI) is an uncommon disorder with onset between 3 months and 2 years of age. The epilepsy begins with recurrent simple febrile seizures, which later become of longer duration and occur when the patient is afebrile.

Myoclonic seizures, either focal or generalized, appear after age 1 year. Multiple seizure types develop, including hemiclonic, simple motor, complex partial, and atypical absence seizures. Episodes of status epilepticus are common.

Myoclonic status in nonprogressive encephalopathies

This rarely reported disorder has onset in infancy or early childhood, with onset usually during the first year of life.[26] Seizures typically begin with partial motor seizures, although myoclonic status may occur at onset. Myoclonic absences, massive myoclonias, and rarely generalized or hemiclonic seizures may occur.

Myoclonias may be multifocal and occur with startles. Myoclonic status epilepticus may be recurrent. Motor abnormalities and movement disorders are common.

Myoclonic-astatic epilepsy (Doose syndrome)

Myoclonic-astatic epilepsy (MAE) is a rare syndrome occurring in early childhood, usually before age 5 years. Children are previously normal, and there is a slight male predominance to the syndrome. A history of febrile seizures or generalized epilepsy with febrile seizures "plus" (GEFS+) may be present.

Initial seizures are generalized tonic-clonic (GTC), followed by myoclonic seizures that increase in frequency. Frequent falls are characteristic and are due to myoclonic or atonic seizures or both. Multiple seizure types, including atypical absence and tonic seizures in addition to myoclonic, atonic, and GTC seizures, may occur. Nonconvulsive status epilepticus (NCSE) is common.

Lennox-Gastaut syndrome (LGS)

Lennox-Gastaut syndrome (LGS) is a mixed seizure disorder with onset in early childhood and a very refractory course resulting in significant cognitive impairment. Onset is often before age 5 years.

The most commonly reported seizure types are tonic, atonic, and atypical absences. Myoclonic, GTC, and focal seizures may also occur. Seizures may begin with infantile spasms, which then evolve into multiple seizure types. Nocturnal tonic seizures are most characteristic, with atypical absences and atonic seizures also occurring in most patients.

Tonic and atonic seizures may cause frequent falls and injury, resulting in the need for protective helmets for some patients. Seizures are very frequent, and episodes of convulsive and nonconvulsive status epilepticus are common.

A broad range of acquired and developmental etiologies has been described, including cerebral malformations, encephalitis, and hypoxic-ischemic injury.[27] From 70-78% of LGS cases are symptomatic (ie, have an identified cause). Development is often delayed in symptomatic cases, whereas development may be normal in idiopathic cases.

Landau-Kleffner syndrome and epilepsy with continuous spikes-waves during slow sleep

Landau-Kleffner syndrome (LKS) is a rare epilepsy syndrome occurring in early childhood, with onset usually between 3 and 10 years of age.[43] It was first described in 1957, when Landau and Kleffner reported on 6 children who presented with aphasia after apparently normal language acquisition. Since then, LKS has been recognized as an epileptic syndrome characterized by language regression, an abnormal EEG, and absence of specific underlying brain pathology.

The disorder is more common in boys, and most children have previously normal development. Patients develop an acquired verbal auditory agnosia early in the course, mimicking difficulty hearing, or "word deafness." Aphasia and language regression follow, along with seizures and behavioral problems in most children. Most have normal preceding language development, and the loss of language function is considered to be secondary to the near continuous epileptiform discharges in the superior temporal gyrus and adjacent cortical areas. Behavioral problems reported include aggression, emotional lability, disinhibition, and hyperactivity.

Epilepsy with continuous spike-waves during slow sleep (CSWS), like LKS, is a rare epilepsy syndrome occurring in early childhood. Peak onset is between 3 and 5 years of age.[44] Most children have normal development until the syndrome manifests. In contrast to LKS, which primarily affects language, children with CSWS develop more global cognitive impairment.

CSWS and LKS are not always completely distinct; overlap is seen in some cases. In CSWS, deficits in attention, language, memory, and visuospatial skills are reported. As in LKS, behavioral problems may occur, including aggression, emotional lability, disinhibition, and hyperactivity.

With LKS and CSWS, seizures may be either rare or very frequent and difficult to control. Multiple seizures types are characteristic. Atonic, absence, partial motor, and generalized convulsive seizures may occur.

Benign childhood epilepsy with centro-temporal spike discharges (benign rolandic epilepsy)

Benign rolandic epilepsy (BRE) is the most common epilepsy syndrome of childhood and has a peak onset between 7 and 10 years of age, with resolution by adolescence. The most common seizures are brief partial motor seizures involving the face and pharyngeal muscles, usually occurring at night. A tendency toward secondary generalized tonic-clonic seizures also exists.

Autistic regression with epileptiform EEG findings

An increased risk of epilepsy is associated with autism, but the role of epilepsy in this disorder remains unclear. In most autistic children, including the approximately one third with developmental regression, epilepsy does not play an obvious role in their symptoms.

In children with autism, there is no difference in the incidence of regression between children with and without epilepsy, suggesting that epilepsy does not increase the risk of regression in autism.[45] Epilepsy is present in up to 38% of autistic children and more may have epileptiform abnormalities present on their EEG.[46]

Next

Physical Examination

A thorough general and neurological evaluation may aid in identifying a specific underlying etiology; however, findings may be normal in some cases. An evaluation by a geneticist may be useful when dysmorphic features are present.

Previous
Next

Complications

The complications of epileptic and epileptiform encephalopathies usually are secondary to the treatment, especially with antiepileptic drugs (AEDs) or high-dose steroids. However, the psychiatric and psychological problems associated with a neurological handicap, especially a neurodegenerative process, can have a great impact on both the child and the family. In LKS and some cases of CSWS, behavioral and emotional disturbances are the major problems encountered.

Previous
 
 
Contributor Information and Disclosures
Author

Dean Patrick Sarco, MD Instructor, Department of Neurology, Harvard Medical School; Assistant Physician, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Children's Hospital Boston

Dean Patrick Sarco, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Masanori Takeoka, MD Assistant Professor, Department of Neurology, Harvard Medical School; Staff Physician, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Boston Children's Hospital

Masanori Takeoka, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Medical Association, Child Neurology Society, Massachusetts Medical Society

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.

Acknowledgements

Robert J Baumann, MD Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, and Child Neurology Society

Disclosure: Nothing to disclose.

Jose E Cavazos, MD, PhD, FAAN Associate Professor with Tenure, Departments of Neurology, Pharmacology, and Physiology, Program Director, Clinical Neurophysiology Fellowship, University of Texas School of Medicine at San Antonio; Co-Director, South Texas Comprehensive Epilepsy Center, University Hospital System; Director of the Epilepsy and Neurodiagnostic Centers, Audie L Murphy Veterans Affairs Medical Center

Jose E Cavazos, MD, PhD, FAAN is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, American Neurological Association, and Society for Neuroscience

Disclosure: GXC Global, Inc. Intellectual property rights Medical Director - company is to develop a seizure detecting device.

Stavros M Hadjiloizou, MD Instructor, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Children's Hospital, Harvard University Medical School

Stavros Michael Hadjiloizou is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, Child Neurology Society, and Massachusetts Medical Society

Disclosure: Nothing to disclose.

James J Riviello Jr, MD George Peterkin Endowed Chair in Pediatrics, Professor of Pediatrics, Section of Neurology and Developmental Neuroscience, Professor of Neurology, Peter Kellaway Section of Neurophysiology, Baylor College of Medicine; Chief of Neurophysiology, Director of the Epilepsy and Neurophysiology Program, Texas Children's Hospital

James J Riviello Jr, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Up To Date Royalty Section Editor

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

References
  1. Khan S, Al Baradie R. Epileptic encephalopathies: an overview. Epilepsy Res Treat. 2012. 2012:403592. [Medline]. [Full Text].

  2. Kural Z, Ozer AF. Epileptic encephalopathies in adults and childhood. Epilepsy Res Treat. 2012. 2012:205131. [Medline]. [Full Text].

  3. Engel J Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia. 2001 Jun. 42(6):796-803. [Medline].

  4. Drislane FW. Overlap of Encephalopathies and Epileptic Seizures. J Clin Neurophysiol. 2013 Oct. 30(5):468-476. [Medline].

  5. Coppola G, Plouin P, Chiron C, Robain O, Dulac O. Migrating partial seizures in infancy: a malignant disorder with developmental arrest. Epilepsia. 1995 Oct. 36(10):1017-24. [Medline].

  6. A glossary of terms most commonly used by clinical electroencephalographers. Electroencephalogr Clin Neurophysiol. 1974 Nov. 37(5):538-48. [Medline].

  7. Moruzzi G, Magoun HW. Brain stem reticular formation and activation of the EEG. 1949. J Neuropsychiatry Clin Neurosci. 1995 Spring. 7(2):251-67. [Medline].

  8. Holmes GL, Lenck-Santini PP. Role of interictal epileptiform abnormalities in cognitive impairment. Epilepsy Behav. 2006 May. 8(3):504-15. [Medline].

  9. Shewmon DA, Erwin RJ. Transient impairment of visual perception induced by single interictal occipital spikes. J Clin Exp Neuropsychol. 1989 Oct. 11(5):675-91. [Medline].

  10. Shewmon DA, Erwin RJ. The effect of focal interictal spikes on perception and reaction time. II. Neuroanatomic specificity. Electroencephalogr Clin Neurophysiol. 1988 Apr. 69(4):338-52. [Medline].

  11. Kasteleijn-Nolst Trenité DG. Transient cognitive impairment during subclinical epileptiform electroencephalographic discharges. Semin Pediatr Neurol. 1995 Dec. 2(4):246-53. [Medline].

  12. Aarts JH, Binnie CD, Smit AM, Wilkins AJ. Selective cognitive impairment during focal and generalized epileptiform EEG activity. Brain. 1984 Mar. 107 ( Pt 1):293-308. [Medline].

  13. Binnie CD. Cognitive impairment during epileptiform discharges: is it ever justifiable to treat the EEG?. Lancet Neurol. 2003 Dec. 2(12):725-30. [Medline].

  14. Binnie CD. Significance and management of transitory cognitive impairment due to subclinical EEG discharges in children. Brain Dev. 1993 Jan-Feb. 15(1):23-30. [Medline].

  15. Binnie CD, Kasteleijn-Nolst Trenité DG, Smit AM, Wilkins AJ. Interactions of epileptiform EEG discharges and cognition. Epilepsy Res. 1987 Jul. 1(4):239-45. [Medline].

  16. Aldenkamp AP, Overweg J, Gutter T, Beun AM, Diepman L, Mulder OG. Effect of epilepsy, seizures and epileptiform EEG discharges on cognitive function. Acta Neurol Scand. 1996 Apr. 93(4):253-9. [Medline].

  17. Nicolai J, Aldenkamp AP, Arends J, Weber JW, Vles JS. Cognitive and behavioral effects of nocturnal epileptiform discharges in children with benign childhood epilepsy with centrotemporal spikes. Epilepsy Behav. 2006 Feb. 8(1):56-70. [Medline].

  18. Massa R, de Saint-Martin A, Carcangiu R, Rudolf G, Seegmuller C, Kleitz C, et al. EEG criteria predictive of complicated evolution in idiopathic rolandic epilepsy. Neurology. 2001 Sep 25. 57(6):1071-9. [Medline].

  19. Nabbout R, Dulac O. Epileptic encephalopathies: a brief overview. J Clin Neurophysiol. 2003 Nov-Dec. 20(6):393-7. [Medline].

  20. Morrell F. Secondary epileptogenesis in man. Arch Neurol. 1985 Apr. 42(4):318-35. [Medline].

  21. Morrell F, Whisler WW, Smith MC, Hoeppner TJ, de Toledo-Morrell L, Pierre-Louis SJ, et al. Landau-Kleffner syndrome. Treatment with subpial intracortical transection. Brain. 1995 Dec. 118 ( Pt 6):1529-46. [Medline].

  22. Kobayashi K, Murakami N, Yoshinaga H, Enoki H, Ohtsuka Y, Ohtahara S. Nonconvulsive status epilepticus with continuous diffuse spike-and-wave discharges during sleep in childhood. Jpn J Psychiatry Neurol. 1988 Sep. 42(3):509-14. [Medline].

  23. Guzzetta F, Battaglia D, Veredice C, Donvito V, Pane M, Lettori D, et al. Early thalamic injury associated with epilepsy and continuous spike-wave during slow sleep. Epilepsia. 2005 Jun. 46(6):889-900. [Medline].

  24. Hrachovy RA, Frost JD. Severe Encephalopathic Epilepsy in Infants: Infantile Spasms (West Syndrome). In: Pellock JM, Bourgeois BFD, Dodson WE. Undefined. Pediatric Epilepsy. Third Edition. New York, NY: Demos Medical Publishing; 2008:16.

  25. Saitoh M, Shinohara M, Hoshino H, Kubota M, Amemiya K, Takanashi JL, et al. Mutations of the SCN1A gene in acute encephalopathy. Epilepsia. 2012 Feb 6. [Medline].

  26. } Dalla Bernardina B, Fontana E, Darra F. Myoclonic status in non-progressive encephalopathies. International Leauge Against Epilepsy. Available at http://www.ilae.org/ctf/myoclon_stat_nonpro_enceph.html. Accessed: February 15, 2011.

  27. Morita DA, Glauser TA. Lennox-Gastaut Syndrome. In: Pellock JM, Bourgeois BFD, Dodson E. Third. Undefined. Pediatric Epilepsy: Diagnosis and Therapy. New York, NY: Demos Medical Publishing; 2008:Chapter 21.

  28. Takeoka M, Riviello JJ Jr, Duffy FH, Kim F, Kennedy DN, Makris N, et al. Bilateral volume reduction of the superior temporal areas in Landau-Kleffner syndrome. Neurology. 2004 Oct 12. 63(7):1289-92. [Medline].

  29. Strug LJ, Clarke T, Chiang T, Chien M, Baskurt Z, Li W, et al. Centrotemporal sharp wave EEG trait in rolandic epilepsy maps to Elongator Protein Complex 4 (ELP4). Eur J Hum Genet. 2009 Sep. 17(9):1171-81. [Medline]. [Full Text].

  30. Deonna T, Roulet E. Autistic spectrum disorder: evaluating a possible contributing or causal role of epilepsy. Epilepsia. 2006. 47 Suppl 2:79-82. [Medline].

  31. Kramer U, Nevo Y, Neufeld MY, Fatal A, Leitner Y, Harel S. Epidemiology of epilepsy in childhood: a cohort of 440 consecutive patients. Pediatr Neurol. 1998 Jan. 18(1):46-50. [Medline].

  32. Aicardi J, Ohtahara S. Severe neonatal epilepsies with suppression-burst pattern. In: Roger J, Thomas P, Bureau M, Hirsch D, Dravet C, et al. Undefined. Syndromes in Infancy, Childhood and Adolescence. Fourth Edition. John Libbey Eurotext; 2005:Chapter 3.

  33. Hurst DL. Epidemiology of severe myoclonic epilepsy of infancy. Epilepsia. 1990 Jul-Aug. 31(4):397-400. [Medline].

  34. Guerrini R, Parmeggiani L, Bonanni P, Kaminska A, Dulac O. Myoclonic astatic epilepsy. In: Roger J, Bureau M, et al. Undefined. Syndromes in Infancy, Childhood and Adolescence. Fourth Edition. Montrouge, France: John Libbey Eurotext; 2005:Chapter 8.

  35. Oguni H, Hayashi K, Imai K, Funatsuka M, Sakauchi M, Shirakawa S, et al. Idiopathic myoclonic-astatic epilepsy of early childhood--nosology based on electrophysiologic and long-term follow-up study of patients. Adv Neurol. 2005. 95:157-74. [Medline].

  36. Oguni H, Hayashi K, Osawa M. Long-term prognosis of Lennox-Gastaut syndrome. Epilepsia. 1996. 37 Suppl 3:44-7. [Medline].

  37. Weglage J, Demsky A, Pietsch M, Kurlemann G. Neuropsychological, intellectual, and behavioral findings in patients with centrotemporal spikes with and without seizures. Dev Med Child Neurol. 1997 Oct. 39(10):646-51. [Medline].

  38. Staden U, Isaacs E, Boyd SG, Brandl U, Neville BG. Language dysfunction in children with Rolandic epilepsy. Neuropediatrics. 1998 Oct. 29(5):242-8. [Medline].

  39. Nicolai J, van der Linden I, Arends JB, van Mil SG, Weber JW, Vles JS, et al. EEG characteristics related to educational impairments in children with benign childhood epilepsy with centrotemporal spikes. Epilepsia. 2007 Nov. 48(11):2093-100. [Medline].

  40. Saint-Martin AD, Seegmuller C, Carcangiu R, Kleitz C, Hirsch E, Marescaux C, et al. [Cognitive consequences of Rolandic Epilepsy]. Epileptic Disord. 2001. 3 Spec No 2:SI59-65. [Medline].

  41. Metz-Lutz MN, Filippini M. Neuropsychological findings in Rolandic epilepsy and Landau-Kleffner syndrome. Epilepsia. 2006. 47 Suppl 2:71-5. [Medline].

  42. Ohtahara S, Yamatogi Y. Ohtahara syndrome: with special reference to its developmental aspects for differentiating from early myoclonic encephalopathy. Epilepsy Res. 2006 Aug. 70 Suppl 1:S58-67. [Medline].

  43. McKinney W, McGreal DA. An aphasic syndrome in children. Can Med Assoc J. 1974 Mar 16. 110(6):637-9. [Medline]. [Full Text].

  44. Riviello JJ, Hadjiloizou S. The Landau-Kleffner Syndrome and Continuous Spike-Waves during Sleep. In: Pellock JM, Bourgeois BFD, Dodson WE. Pediatric Epilepsy: Diagnosis and Therapy. Third Edition. New York, NY: Demos Medical Publishing; 2008:Chapter 24.

  45. Tuchman R, Rapin I. Epilepsy in autism. Lancet Neurol. 2002 Oct. 1(6):352-8. [Medline].

  46. Levisohn PM. The autism-epilepsy connection. Epilepsia. 2007. 48 Suppl 9:33-5. [Medline].

  47. Laufs H. Functional imaging of seizures and epilepsy: evolution from zones to networks. Curr Opin Neurol. 2012 Feb 8. [Medline].

  48. Wong-Kisiel LC, Nickels K. Electroencephalogram of Age-Dependent Epileptic Encephalopathies in Infancy and Early Childhood. Epilepsy Res Treat. 2013. 2013:743203. [Medline]. [Full Text].

  49. Patry G, Lyagoubi S, Tassinari CA. Subclinical "electrical status epilepticus" induced by sleep in children. A clinical and electroencephalographic study of six cases. Arch Neurol. 1971 Mar. 24(3):242-52. [Medline].

  50. Van Hirtum-Das M, Licht EA, Koh S, Wu JY, Shields WD, Sankar R. Children with ESES: variability in the syndrome. Epilepsy Res. 2006 Aug. 70 Suppl 1:S248-58. [Medline].

  51. Smith MC, Hoeppner TJ. Epileptic encephalopathy of late childhood: Landau-Kleffner syndrome and the syndrome of continuous spikes and waves during slow-wave sleep. J Clin Neurophysiol. 2003 Nov-Dec. 20(6):462-72. [Medline].

  52. [Guideline] Hirtz D, Ashwal S, Berg A, Bettis D, Camfield C, Camfield P, et al. Practice parameter: evaluating a first nonfebrile seizure in children: report of the quality standards subcommittee of the American Academy of Neurology, The Child Neurology Society, and The American Epilepsy Society. Neurology. 2000 Sep 12. 55(5):616-23. [Medline].

  53. [Guideline] Karis JP, Seidenwurm DJ, Davis PC, Brunberg JA, De La Paz RL, Dormont PD, et al. ACR Appropriateness Criteria epilepsy. Epilepsy. [Full Text].

  54. Cusmai R, Martinelli D, Moavero R, Dionisi Vici C, Vigevano F, Castana C, et al. Ketogenic diet in early myoclonic encephalopathy due to non ketotic hyperglycinemia. Eur J Paediatr Neurol. 2012 Jan 17. [Medline].

  55. Chiron C, Marchand MC, Tran A, Rey E, d'Athis P, Vincent J, et al. Stiripentol in severe myoclonic epilepsy in infancy: a randomised placebo-controlled syndrome-dedicated trial. STICLO study group. Lancet. 2000 Nov 11. 356(9242):1638-42. [Medline].

  56. Sasagawa M, Kioi Y. [A successful treatment with intravenous high doses of gamma globulin for a minor status in a patient with Doose syndrome]. No To Hattatsu. 1997 May. 29(3):261-3. [Medline].

  57. LANDAU WM, KLEFFNER FR. Syndrome of acquired aphasia with convulsive disorder in children. Neurology. 1957 Aug. 7(8):523-30. [Medline].

  58. Deuel RK, Lenn NJ. Treatment of acquired epileptic aphasia. J Pediatr. 1977 Jun. 90(6):959-61. [Medline].

  59. Aeby A, Poznanski N, Verheulpen D, Wetzburger C, Van Bogaert P. Levetiracetam efficacy in epileptic syndromes with continuous spikes and waves during slow sleep: experience in 12 cases. Epilepsia. 2005 Dec. 46(12):1937-42. [Medline].

  60. De Negri M, Baglietto MG, Battaglia FM, Gaggero R, Pessagno A, Recanati L. Treatment of electrical status epilepticus by short diazepam (DZP) cycles after DZP rectal bolus test. Brain Dev. 1995 Sep-Oct. 17(5):330-3. [Medline].

  61. Hadjiloizou SM, Bourgeois BFD, Duffy FH, et al. Childhood-onset epileptic encephalopathies with sleep activated EEG (EESA_EEG) and high dose diazepam treatment (HDDT): Review of a 5-year experience at Children's Hospital Boston. Undefined. Epilepsia. Suppl. 8; 2005;46:150-151.

  62. Marescaux C, Hirsch E, Finck S, Maquet P, Schlumberger E, Sellal F, et al. Landau-Kleffner syndrome: a pharmacologic study of five cases. Epilepsia. 1990 Nov-Dec. 31(6):768-77. [Medline].

  63. Lerman P, Lerman-Sagie T, Kivity S. Effect of early corticosteroid therapy for Landau-Kleffner syndrome. Dev Med Child Neurol. 1991 Mar. 33(3):257-60. [Medline].

  64. Chez MG, Loeffel M, Buchanan CP, et al. Pulse high-dose steroids as combination therapy with valproic acid in epileptic aphasia patients with pervasive developmental delay or autism. Undefined. Ann Neurol. 1998;44(3):539.

  65. Tsuru T, Mori M, Mizuguchi M, Momoi MY. Effects of high-dose intravenous corticosteroid therapy in Landau-Kleffner syndrome. Pediatr Neurol. 2000 Feb. 22(2):145-7. [Medline].

  66. Sinclair DB, Snyder TJ. Corticosteroids for the treatment of Landau-kleffner syndrome and continuous spike-wave discharge during sleep. Pediatr Neurol. 2005 May. 32(5):300-6. [Medline].

  67. Dagar A, Chandra PS, Chaudhary K, Avnish C, Bal CS, Gaikwad S, et al. Epilepsy Surgery in a Pediatric Population: A Retrospective Study of 129 Children from a Tertiary Care Hospital in a Developing Country along with Assessment of Quality of Life. Pediatr Neurosurg. 2011. 47(3):186-93. [Medline].

  68. Morrell F, Whisler WW, Bleck TP. Multiple subpial transection: a new approach to the surgical treatment of focal epilepsy. J Neurosurg. 1989 Feb. 70(2):231-9. [Medline].

  69. Grote CL, Van Slyke P, Hoeppner JA. Language outcome following multiple subpial transection for Landau-Kleffner syndrome. Brain. 1999 Mar. 122 ( Pt 3):561-6. [Medline].

  70. Irwin K, Birch V, Lees J, Polkey C, Alarcon G, Binnie C, et al. Multiple subpial transection in Landau-Kleffner syndrome. Dev Med Child Neurol. 2001 Apr. 43(4):248-52. [Medline].

 
Previous
Next
 
Epileptic and epileptiform encephalopathies. EEG showing an epileptiform beta frequency burst.
EEG of a patient with Landau-Kleffner syndrome showing electrical status epilepticus of sleep (ESES).
Epileptic and epileptiform encephalopathies. Waking EEG in Landau-Kleffner syndrome, showing left posterior spikes.
Epileptic and epileptiform encephalopathies. EEG in Landau-Kleffner syndrome (LKS), before and after treatment with prednisone. The left EEG tracing shows electrical status epilepticus of sleep. The right tracing, obtained after 6 months of prednisone treatment, is normal.
Epileptic and epileptiform encephalopathies. Frequency-modulated auditory evoked response (FMAER), before and after treatment with prednisone. The left FMAER is absent. The right FMAER is normal following treatment.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.