Dataset Information

Isolated nostril myoclonus: A novel ictal presentation in structural generalised epilepsy.

ABSTRACT: Introduction:Perioral myoclonus (POM) is a rare seizure manifestation which may present in either idiopathic or structural epilepsies. There has been little description of the rarer ictal manifestations in POM in generalised epilepsy. It is important during Electroencephalography (EEG) testing to carefully monitor clinical change during inter-ictal bursts, as this condition, demonstrated in this case, can exhibit extremely subtle seizure semiology which can allude typical clinical examination. Case:Presented is a four-year-old boy with a complex medical history, referred following episodes up to six times per day consisting of perioral myoclonus at a rate of ?3p/s alongside behavioural arrest lasting up to thirty seconds. Electroencephalography (EEG) recording captured nine seizures within a twenty-five-minute period, where only one seizure was of his stereotyped semiology. Additional seizures commonly adopted a novel semiology of isolated nostril ("flaring") myoclonus, on some occasions with concomitant head bobbing. Surface EMG and high resolution zoomed video revealed time-locked myoclonus to the generalised spike and wave discharges seen in on EEG. Significance:The findings demonstrate a novel epileptic seizure manifestation of nostril myoclonus, in which detailed electroencephalographic and video correlation was essential to minimise risk of underestimating seizure frequency in this rare and complex epilepsy disorder.

SUBMITTER: Marmoy OR

PROVIDER: S-EPMC6449652 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Isolated nostril myoclonus: A novel ictal presentation in structural generalised epilepsy.

Marmoy Oliver R OR

Clinical neurophysiology practice 20190314

<h4>Introduction</h4>Perioral myoclonus (POM) is a rare seizure manifestation which may present in either idiopathic or structural epilepsies. There has been little description of the rarer ictal manifestations in POM in generalised epilepsy. It is important during Electroencephalography (EEG) testing to carefully monitor clinical change during inter-ictal bursts, as this condition, demonstrated in this case, can exhibit extremely subtle seizure semiology which can allude typical clinical examin ...[more]

PMID: 30989152

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Project description:Hypersynchronous (HYP) and low voltage fast (LVF) activity are two separate ictal depth EEG onsets patterns often recorded in presurgical patients with MTLE. Evidence suggests the mechanisms generating HYP and LVF onset seizures are distinct, including differential involvement of hippocampal and extra-hippocampal sites. Yet the extent of extra-hippocampal structural alterations, which could support these two common seizures, is not known. In the current study, preoperative MRI from 24 patients with HYP or LVF onset seizures were analyzed to determine changes in cortical thickness and relate structural changes to spatiotemporal properties of the ictal EEG. Overall, onset and initial ipsilateral spread of HYP onset seizures involved mesial temporal structures, whereas LVF onset seizures involved mesial and lateral temporal as well as orbitofrontal cortex. MRI analysis found reduced cortical thickness correlated with longer duration of epilepsy. However, in patients with HYP onsets, the most affected areas were on the medial surface of each hemisphere, including parahippocampal regions and cingulate gyrus, whereas in patients with LVF onsets, the lateral surface of the anterior temporal lobe and orbitofrontal cortex showed the greatest effect. Most patients with HYP onset seizures were seizure-free after resective surgery, while a higher proportion of patients with LVF onset seizures had only worthwhile improvement. Our findings confirm the view that recurrent seizures cause progressive changes in cortical thickness, and provide information concerning the structural basis of two different epileptogenic networks responsible for MTLE. One, identified by HYP ictal onsets, chiefly involves hippocampus and is associated with excellent outcome after standardized anteromedial temporal resection, while the other also involves lateral temporal and orbitofrontal cortex and a seizure-free surgical outcome occurs less after this procedure. These results suggest that a more extensive tailored resection may be required for patients with the second type of MTLE.

Dataset Information

Isolated nostril myoclonus: A novel ictal presentation in structural generalised epilepsy.

Publications

Isolated nostril myoclonus: A novel ictal presentation in structural generalised epilepsy.

Similar Datasets

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