Surgical treatment in startle epilepsy

Hong, Tao; Shi, Xiaoyan; Zuo, Huancong

doi:10.4103/0028-3886.98526

Cited by 3 publications

(7 citation statements)

References 9 publications

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“…For Chauvel and colleagues, startle epilepsy seizures thus originate in the motor and premotor cortices. Other recent studies reported a seizure onset zone located in the premotor cortex [21][22], the prefrontal cortex, and anterior and middle cingulate cortex [23]. The SMA was involved in the epileptic network of the majority of cases [21][22][23][24].…”

Section: Intracerebral Eeg Recordingsmentioning

confidence: 86%

“…Other recent studies reported a seizure onset zone located in the premotor cortex [21][22], the prefrontal cortex, and anterior and middle cingulate cortex [23]. The SMA was involved in the epileptic network of the majority of cases [21][22][23][24]. Using the Epileptogenicity Maps method, Job et al [18] identified an increase of high frequency oscillations in the premotor and prefrontal areas during startle epilepsy seizures and a constant involvement of the SMA.…”

Section: Intracerebral Eeg Recordingsmentioning

confidence: 97%

“…This device produces a background sound that does not disturb hearing but induces a relaxing mood and a feeling of control over the sound environment. Surgery was effective in many studies [7,18,[21][22][23][24]29,30]. However, the proximity to the primary motor cortex requires rigorous preoperative evaluation and the use of intracranial recordings.…”

Section: Treatmentmentioning

confidence: 99%

“…However, the proximity to the primary motor cortex requires rigorous preoperative evaluation and the use of intracranial recordings. Different types of procedures are proposed with similar results: corpus callostomy [7,29], hemispherotomy [30] or focal resection [7,18,[21][22][23][24]. In these studies, postoperative outcomes at one year (or more) were satisfactory with a majority of Engel class 1 [7,18,[21][22][23][24]29,30], and showed no associated motor or cognitive deficit.…”

Section: Treatmentmentioning

confidence: 99%

“…Different types of procedures are proposed with similar results: corpus callostomy [7,29], hemispherotomy [30] or focal resection [7,18,[21][22][23][24]. In these studies, postoperative outcomes at one year (or more) were satisfactory with a majority of Engel class 1 [7,18,[21][22][23][24]29,30], and showed no associated motor or cognitive deficit. Post-operative motor deficit could also rapidly improve in a few months [24].…”

Section: Treatmentmentioning

confidence: 99%

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Clinical and electrophysiological pattern of startle epilepsy: a comprehensive literature review

Cheval

Jonas

2022

Emerg Neurol

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Startle epilepsy is a rare form of epilepsy characterized by recurrent seizures that are triggered by a surprising stimulus. It affects children between 10 months and 14 years of age, with a history of brain damage due to ante- or neonatal hypoxia, and hemiparesis. The most common triggers for these seizures are auditory stimulation, followed by somesthetic stimulation and visual stimulation. Typical startle seizures are rare, and they have a large variety of clinical manifestations. The most frequent type is short hemitonic flexion seizures, followed by myoclonic seizures, and bilateral tonic seizures. Few studies have focused on the electrophysiological features of this condition and the literature is controversial regarding the EEG pattern. Interictal EEG is often normal. The most frequent interictal abnormalities are a slowing of the background rhythm, associated with focal spikes in the frontal, central, and parietal regions. Several ictal EEG patterns have been reported: diffuse flattening of the background rhythm, focal fast activity, an isolated focal spike followed by a discharge of spikes. Intracerebral EEG recording shows primary and secondary motor cortex involvement as initial discharge areas. The most efficient antiepileptic drugs are Lamotrigine, Oxcarbazepine, and benzodiazepines. But most patients are often drug-resistant. Surgery is a possible albeit rare therapeutic option. We describe the case of a 13-year-old female patient with hemitonic seizures triggered by surprising auditory stimuli. The video EEG allowed us to record and define an EEG pattern of her seizures. Interictal EEG showed right fronto-central abundant spikes. The ictal EEG showed right fronto-central and medial spikes followed by a fronto-central fast rhythm discharge. The clinical and electrographic data permitted the diagnosis of startle epilepsy. Our patient was treated with Levetiracetam during 6 months followed by Oxcarbazepine and Clobazam with a partial improvement (reduction of the frequency of focal to bilateral tonic-clonic seizures). Considering the usual pharmaco-resistance of this type of epilepsy, we proposed our patient a presurgical evaluation. This case shows that the diagnosis should be considered in paroxysmal dystonic movements with a stereotyped triggering factor, and underlines the importance of repeating the EEG recordings in this type of epilepsy. In this article we will review the literature, discuss the underlying pathophysiology, and identify the common electrophysiological characteristics associated with startle epilepsy.

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Section: Intracerebral Eeg Recordingsmentioning

confidence: 86%

Section: Intracerebral Eeg Recordingsmentioning

confidence: 97%

Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

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Clinical and electrophysiological pattern of startle epilepsy: a comprehensive literature review

Cheval

Jonas

2022

Emerg Neurol

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SEEG re‐exploration in a patient with complex frontal epilepsy with rapid perisylvian propagation and mixed “startle ‐ reflex” seizures

Mălîia¹,

Kahane²,

Nica³

et al. 2021

Epileptic Disorders

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The SEEG International Course, organised in 2017, focused on the investigation and surgery of insulo-perisylvian epilepsies. We present one representative complex case that was discussed. The patient had seizures displaying startle/ reflex components. He was MRI negative, while other non-invasive investigations offered only partially concordant data. Initial SEEG exploration resulted in an incomplete definition of the epileptogenic zone. A second SEEG followed, which led to a thorough assessment of the seizure onset zone and the epileptic network, localised to the lateral inferior premotor cortex, explaining the incongruent data obtained beforehand. This was the basis of a tailored resection with a favourable outcome. The patient has been seizure-free for five years without any motor nor cognitive deficits, but with pharmacodependence to one AED. The electroclinical reasoning is presented, accompanied by relevant commentaries and recommendations from the tutors.

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Startle-Induced Epileptic Spasms: A Clinical and Video-EEG Study

Jiao²,

Gong³

et al. 2022

Front. Neurol.

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ObjectiveThis study aimed to delineate the detailed characteristics of startle-induced epileptic spasms (ES) and explore the brain regions where startle-induced ES originated.MethodsAmong 581 patients with ES registered in our database, 30 were diagnosed with startle-induced ES according to video-electroencephalogram (EEG) and seizure semiology and were included in this study. Patients' clinical characteristics and ictal high-frequency oscillations (HFOs) were analyzed.ResultsMean age at the onset of startle-induced ES was 28.1 months. Half of the patients had structural etiology, two of whom were diagnosed with co-existing structural and genetic etiologies. The focal neuroimaging abnormalities were predominant in the frontal cortex (9/15, 60.0%). Fifteen patients (50%) had prominent interictal epileptiform discharges in the frontal and anterior temporal. Ictal HFOs counts of the startle-induced ES in the anterior region were significantly higher than those in the posterior regions (p < 0.05). Five patients (16.7%) became seizure-free ≥6 months, and ten (33.3%) showed startle-induced ES cessation ≥6 months. All patients except one had mild to severe psychomotor developmental delay after the onset of seizures.ConclusionPatients with startle-induced ES typically had brain lesions and showed drug-resistant. The neuroimaging and EEG findings, including ictal HFOs, support that startle-induced ES often originates from the frontal cortex.

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Surgical treatment in startle epilepsy

Cited by 3 publications

References 9 publications

Clinical and electrophysiological pattern of startle epilepsy: a comprehensive literature review

Clinical and electrophysiological pattern of startle epilepsy: a comprehensive literature review

SEEG re‐exploration in a patient with complex frontal epilepsy with rapid perisylvian propagation and mixed “startle ‐ reflex” seizures

Startle-Induced Epileptic Spasms: A Clinical and Video-EEG Study

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