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Data from: The epileptology of alternating hemiplegia of childhood

Cite this dataset

Uchitel, Julie et al. (2020). Data from: The epileptology of alternating hemiplegia of childhood [Dataset]. Dryad.


Objective: Report our experience and investigate five 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) Non-epileptic reduced awareness spells (RAS) occur frequently. 4) Epilepsy is commonly drug resistant but may respond to vagal nerve stimulation (VNS). 5) Status epilepticus (SE) is common and is usually refractory and recurrent. Methods: Analyze a cohort of 51 consecutive AHC patients. Results: 1) Seizure types: 32/51 had epilepsy: Eighteen focal seizures, frontal more frequently than temporal, then posterior. Eleven had primary generalized seizures (tonic-clonic, myoclonic, and/or absence). Epileptic seizures preceded other AHC paroxysmal events in eight (lag: 5.63±6.55 months; p=0.0365). 2) EEG: In 7/32, initial EEGs were normal with the first epileptiform EEG lagging behind by 3.53±4.65 years (p=0.0484). RAS occurred equally in epilepsy (16/32) and non-epilepsy patients (10/19, p=1.0). 28/28 video-EEG captured RAS showed no concomitant EEG changes. 4) Therapies: Nineteen (59%) were drug resistant. VNS resulted in >50% reduction in seizures in 5/6 (p<0.04). 5) SE: Twelve (38%) had SE (9/12 multiple episodes), refractory/super-refractory in all (p<0.001). 4/12 had regression after SE. Significance: Epilepsy in AHC can be focal or generalized. Epileptic seizures may be the first paroxysmal symptom. EEG may only become epileptiform on follow-up. Epilepsy, though frequently drug resistant, can respond to VNS. RAS are frequent and non-epileptic. SE often recurs and is, usually, refractory/super-refractory. Our observations are consistent with current data on AHC-ATP1A3 pathophysiology.

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