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Benign infantile focal epilepsy with midline spikes and wave during sleep

MedGen UID:
1663004
•Concept ID:
C4749346
•
Disease or Syndrome
Synonyms: benign infantile focal epilepsy with midline spikes and wave during sleep; Benign infantile focal epilepsy with midline spikes and waves during sleep; benign infantile focal epilepsy with midline spikes and waves during sleep; BIMSE; BIMSE - benign infantile focal epilepsy with midline spikes and waves during sleep
SNOMED CT: Benign infantile focal epilepsy with midline spikes and waves during sleep (770622009); BIMSE - benign infantile focal epilepsy with midline spikes and waves during sleep (770622009)
 
Monarch Initiative: MONDO:0015641
Orphanet: ORPHA166308

Definition

A rare infantile epilepsy syndrome characterized by age of onset between 4 and 30 months, partial sporadic seizures presenting with motion arrest, staring, cyanosis and, less common, automatisms and lateralizing signs, and characteristic interictal sleep EEG changes consisting of a spike followed by a bell-shaped slow wave in the midline region. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBenign infantile focal epilepsy with midline spikes and wave during sleep

Recent clinical studies

Etiology

'Benign focal epilepsy in infancy with vertex spikes and waves during sleep'. Delineation of the syndrome and recalling as 'benign infantile focal epilepsy with midline spikes and waves during sleep' (BIMSE).
Capovilla G, Beccaria F, Montagnini A
Brain Dev 2006 Mar;28(2):85-91. Epub 2005 Jun 20 doi: 10.1016/j.braindev.2005.05.001. PMID: 15967619

Diagnosis

'Benign focal epilepsy in infancy with vertex spikes and waves during sleep'. Delineation of the syndrome and recalling as 'benign infantile focal epilepsy with midline spikes and waves during sleep' (BIMSE).
Capovilla G, Beccaria F, Montagnini A
Brain Dev 2006 Mar;28(2):85-91. Epub 2005 Jun 20 doi: 10.1016/j.braindev.2005.05.001. PMID: 15967619

Prognosis

'Benign focal epilepsy in infancy with vertex spikes and waves during sleep'. Delineation of the syndrome and recalling as 'benign infantile focal epilepsy with midline spikes and waves during sleep' (BIMSE).
Capovilla G, Beccaria F, Montagnini A
Brain Dev 2006 Mar;28(2):85-91. Epub 2005 Jun 20 doi: 10.1016/j.braindev.2005.05.001. PMID: 15967619

Clinical prediction guides

'Benign focal epilepsy in infancy with vertex spikes and waves during sleep'. Delineation of the syndrome and recalling as 'benign infantile focal epilepsy with midline spikes and waves during sleep' (BIMSE).
Capovilla G, Beccaria F, Montagnini A
Brain Dev 2006 Mar;28(2):85-91. Epub 2005 Jun 20 doi: 10.1016/j.braindev.2005.05.001. PMID: 15967619

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