eMedicine Specialties > Neurology > Pediatric Neurology

Benign Neonatal Convulsions: Differential Diagnoses & Workup

Author: Nancy Theresa Rodgers-Neame, MD, Assistant Professor, Department of Molecular Pharmacology and Physiology, University of South Florida; Director, Florida Comprehensive Epilepsy and Seizure Disorders Program
Contributor Information and Disclosures

Updated: Apr 8, 2009

Differential Diagnoses

Arteriovenous Malformations
Neonatal Injuries in Child Abuse
Aseptic Meningitis
Neonatal Meningitis
Epilepsy in Children with Mental Retardation
Neonatal Seizures
Febrile Seizures
Partial Epilepsies
Frontal Lobe Epilepsy
Tuberous Sclerosis
Infantile Spasm (West Syndrome)
Viral Encephalitis

Other Problems to Be Considered

Vitamin B-6 deficiency
Maternal drug abuse

Workup

Laboratory Studies

  • The reason for ordering tests in benign familial and idiopathic neonatal convulsions is to exclude the presence of any etiology for the seizures. The diagnosis of benign infantile convulsions of either type requires that no other explanation exist for the seizures. Order tests for individual patients with a plan for that patient in mind. While a "shotgun" approach is wasteful and unreasonable, it is also important not to miss a diagnosis of a treatable meningoencephalitis in the early stages or intracranial hemorrhage. Both of these conditions in neonates lack the typical findings observed in older infants and children, and the only early symptom may be seizures. Also entertain a healthy suspicion for child abuse in neonates, who often have just arrived home from the hospital following delivery. Review of basic screening laboratory studies performed at delivery may also be helpful.
    • Chemistries - Basic metabolic panel plus calcium, magnesium, phosphorus, thyroid function tests, and possibly B vitamin levels
    • Basic hematologic labs - CBC, prothrombin time, activated partial thromboplastin time
    • Lumbar puncture - Cerebrospinal fluid examination to exclude neonatal meningoencephalitis or occult blood
  • Any abnormalities found that are inconsistent with a diagnosis of benign neonatal convulsions require the appropriate further workup and treatment.

Imaging Studies

  • MRI or CT scan of the brain
    • Perform one or both of these tests in every patient with neonatal seizures to exclude structural lesions and intracranial hemorrhage.
    • An argument can be made that both are needed since CT scan yields better information on acute hemorrhage and skull fracture, and MRI shows better brain structural detail.

Other Tests

  • Electroencephalography
    • The classic EEG observed in 60% of patients with BINC is a rolandic discharge in the theta range that is alternating or discontinuous with intermixed sharp activity. Interhemispheric asymmetry is observed frequently; it is unresponsive to stimulation of any kind. This pattern often is termed theta pointu altérnant.3,7,29
    • The remaining patients with benign idiopathic neonatal convulsions have either a normal interictal EEG or focal abnormalities. The EEG during seizures is most often high-voltage (200-400 µV) generalized discharges, which may appear to have a focal onset.
  • In benign familial neonatal convulsions, the interictal EEG is most commonly normal (50-70% of patients). The theta pointu altérnant pattern also is observed but only in approximately 25% of patients. In a small percentage of patients, focal, often rolandic, discharges or spikes may be present.41,42,3
  • In selected patients, continuous video-EEG can be used to confirm behavioral events concordant with abnormal EEG and to confirm that treatment is effective. The state of the patient and improvement or deterioration can make decision-making easier and facilitate accurate communication with an often anxious family.7

More on Benign Neonatal Convulsions

Overview: Benign Neonatal Convulsions
Differential Diagnoses & Workup: Benign Neonatal Convulsions
Treatment & Medication: Benign Neonatal Convulsions
Follow-up: Benign Neonatal Convulsions
References

References

  1. Quattlebaum TG. Benign familial convulsions in the neonatal period and early infancy. J Pediatr. Aug 1979;95(2):257-9. [Medline].

  2. Mizrahi EM. Seizure disorders in children. Curr Opin Pediatr. Dec 1994;6(6):642-6. [Medline].

  3. Plouin P. Benign idiopathic neonatal convulsions (familial and non-familial): open questions about these syndromes. Epileptic Seizures and Syndromes. 1994;193-201.

  4. Pettit RE, Fenichel GM. Benign familial neonatal seizures. Arch Neurol. Jan 1980;37(1):47-8. [Medline].

  5. Singh NA, Westenskow P, Charlier C, et al. KCNQ2 and KCNQ3 potassium channel genes in benign familial neonatal convulsions: expansion of the functional and mutation spectrum. Brain. Dec 2003;126:2726-37. [Medline].

  6. Singh NA, Charlier C, Stauffer D, et al. A novel potassium channel gene, KCNQ2, is mutated in an inherited epilepsy of newborns. Nat Genet. Jan 1998;18(1):25-9. [Medline].

  7. Plouin P. [Value of video electroencephalography in neonatology]. Arch Pediatr. May 2000;7 Suppl 2:332s-333s. [Medline].

  8. Biervert C, Schroeder BC, Kubisch C, et al. A potassium channel mutation in neonatal human epilepsy. Science. Jan 16 1998;279(5349):403-6. [Medline].

  9. Hirose S, Zenri F, Akiyoshi H, et al. A novel mutation of KCNQ3 (c.925T-->C) in a Japanese family with benign familial neonatal convulsions. Ann Neurol. Jun 2000;47(6):822-6. [Medline].

  10. Schroeder BC, Kubisch C, Stein V, et al. Moderate loss of function of cyclic-AMP-modulated KCNQ2/KCNQ3 K+ channels causes epilepsy. Nature. Dec 17 1998;396(6712):687-90. [Medline].

  11. Schwake M, Pusch M, Kharkovets T, et al. Surface expression and single channel properties of KCNQ2/KCNQ3, M-type K+ channels involved in epilepsy. J Biol Chem. May 5 2000;275(18):13343-8. [Medline].

  12. Singh NA, Charlier C, Stauffer D, et al. A novel potassium channel gene, KCNQ2, is mutated in an inherited epilepsy of newborns. Nat Genet. Jan 1998;18(1):25-9. [Medline].

  13. Kananura C, Biervert C, Hechenberger M, et al. The new voltage gated potassium channel KCNQ5 and neonatal convulsions. Neuroreport. Jun 26 2000;11(9):2063-7. [Medline].

  14. Beck C, Moulard B, Steinlein O, et al. A nonsense mutation in the alpha4 subunit of the nicotinic acetylcholine receptor (CHRNA4) cosegregates with 20q-linked benign neonatal familial convulsions (EBNI). Neurobiol Dis. Nov 1994;1(1-2):95-9. [Medline].

  15. Steinlein O, Sander T, Stoodt J, et al. Possible association of a silent polymorphism in the neuronal nicotinic acetylcholine receptor subunit alpha4 with common idiopathic generalized epilepsies. Am J Med Genet. Jul 25 1997;74(4):445-9. [Medline].

  16. Lerche H, Biervert C, Alekov AK, et al. A reduced K+ current due to a novel mutation in KCNQ2 causes neonatal convulsions. Ann Neurol. Sep 1999;46(3):305-12. [Medline].

  17. Zhu G, Okada M, Murakami T, et al. Dysfunction of M-channel enhances propagation of neuronal excitability in rat hippocampus monitored by multielectrode dish and microdialysis systems. Neurosci Lett. Nov 10 2000;294(1):53-7. [Medline].

  18. Burgess DL. Neonatal epilepsy syndromes and GEFS+: mechanistic considerations. Epilepsia. 2005;46 Suppl 10:51-8. [Medline].

  19. Lehmann-Horn F, Jurkat-Rott K. Voltage-gated ion channels and hereditary disease. Physiol Rev. Oct 1999;79(4):1317-72.

  20. Mulley JC, Scheffer IE, Petrou S, et al. Channelopathies as a genetic cause of epilepsy. Curr Opin Neurol. Apr 2003;16(2):171-6. [Medline].

  21. Webb R, Bobele G. 'Benign' familial neonatal convulsions. J Child Neurol. Oct 1990;5(4):295-8. [Medline].

  22. Tharp BR. Neonatal seizures and syndromes. Epilepsia. 2002;43 Suppl 3:2-10. [Medline].

  23. DeLorenzo RJ. The challenging genetics of epilepsy. Epilepsy Res Suppl. 1991;4:3-17. [Medline].

  24. Hirose S, Mitsudome A, Okada M, et al. Genetics of idiopathic epilepsies. Epilepsia. 2005;46 Suppl 1:38-43. [Medline].

  25. Ben-Ari Y, Khazipov R, Leinekugel X, et al. GABAA, NMDA and AMPA receptors: a developmentally regulated 'ménage à trois'. Trends Neurosci. Nov 1997;20(11):523-9. [Medline].

  26. Dzhala VI, Talos DM, Sdrulla DA, et al. NKCC1 transporter facilitates seizures in the developing brain. Nat Med. Nov 2005;11(11):1205-13. [Medline].

  27. Leinekugel X, Medina I, Khalilov I, et al. Ca2+ oscillations mediated by the synergistic excitatory actions of GABA(A) and NMDA receptors in the neonatal hippocampus. Neuron. Feb 1997;18(2):243-55. [Medline].

  28. Rivera C, Voipio J, Payne JA, et al. The K+/Cl- co-transporter KCC2 renders GABA hyperpolarizing during neuronal maturation. Nature. Jan 21 1999;397(6716):251-5. [Medline].

  29. Miles DK, Holmes GL. Benign neonatal seizures. J Clin Neurophysiol. Jul 1990;7(3):369-79. [Medline].

  30. Lewis TB, Leach RJ, Ward K, et al. Genetic heterogeneity in benign familial neonatal convulsions: identification of a new locus on chromosome 8q. Am J Hum Genet. Sep 1993;53(3):670-5. [Medline].

  31. Treiman LJ. Genetics of epilepsy: an overview. Epilepsia. 1993;34 Suppl 3:S1-11. [Medline].

  32. Turnbull J, Lohi H, Kearney JA, et al. Sacred disease secrets revealed: the genetics of human epilepsy. Hum Mol Genet. Sep 1 2005;14(17):2491-500. [Medline].

  33. Jallon P, Latour P. Epidemiology of idiopathic generalized epilepsies. Epilepsia. 2005;46 Suppl 9:10-4. [Medline].

  34. Concordance of clinical forms of epilepsy in families with several affected members. Italian League Against Epilepsy Genetic Collaborative Group. Epilepsia. Sep-Oct 1993;34(5):819-26. [Medline].

  35. North KN, Storey, GNB, Henderson-Smart, D.J. Fifth day fits in the newborn. Journal of Paediatrics and Child Health,. March 2008;25(5):284-287.

  36. Leppert M, Anderson VE, Quattlebaum T, et al. Benign familial neonatal convulsions linked to genetic markers on chromosome 20. Nature. Feb 16 1989;337(6208):647-8. [Medline].

  37. Leppert M. Novel K+ channel genes in benign familial neonatal convulsions. Epilepsia. Aug 2000;41(8):1066-7. [Medline].

  38. Lewis TB, Shevell MI, Andermann E, et al. Evidence of a third locus for benign familial convulsions. J Child Neurol. May 1996;11(3):211-4. [Medline].

  39. Elmslie F, Gardiner M. Genetics of the epilepsies. Curr Opin Neurol. Apr 1995;8(2):126-9. [Medline].

  40. Gardiner M. Genetics of idiopathic generalized epilepsies. Epilepsia. 2005;46 Suppl 9:15-20. [Medline].

  41. Camfield PR, Dooley J, Gordon K, et al. Benign familial neonatal convulsions are epileptic. J Child Neurol. Oct 1991;6(4):340-2. [Medline].

  42. Tibbles JA. Dominant benign neonatal seizures. Dev Med Child Neurol. Oct 1980;22(5):664-7. [Medline].

  43. Vigevano F. Benign familial infantile seizures. Brain Dev. Apr 2005;27(3):172-7. [Medline].

  44. Delgado-Escueta AV, Serratosa JM, Liu A, et al. Progress in mapping human epilepsy genes. Epilepsia. 1994;35 Suppl 1:S29-40. [Medline].

Further Reading

Keywords

benign neonatal convulsions, second day seizures, benign neonatal seizures, benign familial neonatal convulsions, benign idiopathic neonatal convulsions, benign familial neonatal seizures, benign idiopathic neonatal seizures, fifth day disease, fifth day fits, seizure epilepsy treatment, symptoms, BFNC, BINC

Contributor Information and Disclosures

Author

Nancy Theresa Rodgers-Neame, MD, Assistant Professor, Department of Molecular Pharmacology and Physiology, University of South Florida; Director, Florida Comprehensive Epilepsy and Seizure Disorders Program
Nancy Theresa Rodgers-Neame, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Medical Women's Association, Society for Neuroscience, Southern Clinical Neurological Society, and Southern Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Robert Baumann, MD, Program Director, Professor, Departments of Neurology and Pediatrics, University of Kentucky
Robert Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American College of Epidemiology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Neurology, Department of Neurology, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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