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The epileptology of alternating hemiplegia of childhood.

Publication ,  Journal Article
Uchitel, J; Helseth, A; Prange, L; McLean, M; Ghusayni, R; Sachdev, M; Hunanyan, A; Mikati, MA
Published in: Neurology
September 24, 2019

OBJECTIVE: To report our experience and investigate 5 original hypotheses: (1) multiple types of epileptic seizures occur in alternating hemiplegia of childhood (AHC), and these can be the initial presentation; (2) epileptiform abnormalities often appear well after clinical seizures; (3) nonepileptic reduced awareness spells (RAS) occur frequently; (4) epilepsy is commonly drug resistant but may respond to vagal nerve stimulation (VNS); and (5) status epilepticus (SE) is common and is usually refractory and recurrent. METHODS: We analyzed a cohort of 51 consecutive patients with AHC. RESULTS: Thirty-two of 51 patients had epilepsy: 18 focal seizures, frontal more frequently than temporal, and then posterior. Eleven had primary generalized seizures (tonic-clonic, myoclonic, and/or absence). Epileptic seizures preceded other AHC paroxysmal events in 8 (lag 5.63 ± 6.55 months; p = 0.0365). In 7 of 32, initial EEGs were normal, with the first epileptiform EEG lagging behind by 3.53 ± 4.65 years (p = 0.0484). RAS occurred equally in patients with epilepsy (16 of 32) and patients without epilepsy (10 of 19, p = 1.0). Twenty-eight patients had video-EEG; captured RAS showed no concomitant EEG changes. Nineteen patients (59%) were drug resistant. VNS resulted in >50% reduction in seizures in 5 of 6 (p < 0.04). Twelve patients (38%) had SE (9 of 12 multiple episodes), refractory/superrefractory in all (p < 0.001), and 4 of 12 had regression after SE. CONCLUSIONS: Epilepsy in AHC can be focal or generalized. Epileptic seizures may be the first paroxysmal symptom. EEG may become epileptiform only on follow-up. Epilepsy, although frequently drug resistant, can respond to VNS. RAS are frequent and nonepileptic. SE often recurs and is usually refractory/superrefractory. Our observations are consistent with current data on AHC-ATP1A3 pathophysiology.

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Published In

Neurology

DOI

EISSN

1526-632X

Publication Date

September 24, 2019

Volume

93

Issue

13

Start / End Page

e1248 / e1259

Location

United States

Related Subject Headings

  • Young Adult
  • Vagus Nerve Stimulation
  • Status Epilepticus
  • Sodium-Potassium-Exchanging ATPase
  • Seizures
  • Neurology & Neurosurgery
  • Male
  • Infant, Newborn
  • Infant
  • Humans
 

Citation

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ICMJE
MLA
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Uchitel, J., Helseth, A., Prange, L., McLean, M., Ghusayni, R., Sachdev, M., … Mikati, M. A. (2019). The epileptology of alternating hemiplegia of childhood. Neurology, 93(13), e1248–e1259. https://doi.org/10.1212/WNL.0000000000008159
Uchitel, Julie, Ashley Helseth, Lyndsey Prange, Melissa McLean, Ryan Ghusayni, Monisha Sachdev, Arsen Hunanyan, and Mohamad A. Mikati. “The epileptology of alternating hemiplegia of childhood.Neurology 93, no. 13 (September 24, 2019): e1248–59. https://doi.org/10.1212/WNL.0000000000008159.
Uchitel J, Helseth A, Prange L, McLean M, Ghusayni R, Sachdev M, et al. The epileptology of alternating hemiplegia of childhood. Neurology. 2019 Sep 24;93(13):e1248–59.
Uchitel, Julie, et al. “The epileptology of alternating hemiplegia of childhood.Neurology, vol. 93, no. 13, Sept. 2019, pp. e1248–59. Pubmed, doi:10.1212/WNL.0000000000008159.
Uchitel J, Helseth A, Prange L, McLean M, Ghusayni R, Sachdev M, Hunanyan A, Mikati MA. The epileptology of alternating hemiplegia of childhood. Neurology. 2019 Sep 24;93(13):e1248–e1259.

Published In

Neurology

DOI

EISSN

1526-632X

Publication Date

September 24, 2019

Volume

93

Issue

13

Start / End Page

e1248 / e1259

Location

United States

Related Subject Headings

  • Young Adult
  • Vagus Nerve Stimulation
  • Status Epilepticus
  • Sodium-Potassium-Exchanging ATPase
  • Seizures
  • Neurology & Neurosurgery
  • Male
  • Infant, Newborn
  • Infant
  • Humans