Noninvasive treatment alternative for intractable startle epilepsy

Klinkenberg, Sylvia; Ubbink, Sander W J; Vles, J. S. H.; Louw, Anton de; Hall, Mariette H. J. A. Debeij‐van; Scheijen, Dyon J. W. M.; Brokx, J.P.L.

doi:10.1016/j.ebcr.2014.02.002

Cited by 5 publications

(4 citation statements)

References 14 publications

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“…Seizures without a startle component also occurred. However, our patient presented only mild cognitive impairment (mostly regarding executive dysfunction), normal development, and no perinatal injuries, in contrast to other cases stated in the literature (Klinkenberg et al ., ). Our patient did not have any visible lesion on MRI.…”

Section: Discussionmentioning

confidence: 97%

Successful epilepsy surgery in frontal lobe epilepsy with startle seizures: a SEEG studyFrontal epilepsy with startle seizures

Ciurea

Popa

Mălîia

et al. 2015

Epileptic Disorders

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Pre‐surgical assessment and surgical management of frontal epilepsy with normal MRI is often challenging. We present a case of a 33‐year‐old, right‐handed, educated male. During childhood, his seizures presented with mandibular myoclonus and no particular trigger. As a young adult, he developed seizures with a startle component, triggered by unexpected noises. During his ictal episodes, he felt fear and grimaced with sudden head flexion and tonic axial posturing. Similar seizures also occurred without startle. Neuropsychological assessment showed executive dysfunction and verbal memory deficit. The cerebral MRI was normal. Electro‐clinical reasoning, investigations performed, the results obtained and follow‐up are discussed in detail. [Published with video sequence]

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Section: Discussionmentioning

confidence: 97%

Successful epilepsy surgery in frontal lobe epilepsy with startle seizures: a SEEG studyFrontal epilepsy with startle seizures

Ciurea

Popa

Mălîia

et al. 2015

Epileptic Disorders

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“…16,17 Klinkenberg et al reported noninvasive treatment method using psychoeducational counselling and sound generators for startle epileptic seizures in four children, resulting in a seizure frequency reduction of 50% in two of them. 18 In our patient, due to the disabling seizures, established right hemiparesis and severe left hemispheric gliosis, we planned a left functional hemispherotomy.…”

Section: Managementmentioning

confidence: 93%

Symptomatic Case of Startle Epilepsy with Infantile Brain Damage Treated with Hemispherotomy: A Case Report and Review of Literature

et al. 2016

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We are reporting a case of rare reflex epilepsy??startle? epilepsy effectively treated with hemispherotomy. A 6-year-old boy, a child of nonconsanguineous parentage with infantile hemiparesis presented to us with recurrent right focal seizures along with secondary generalization provoked by sudden and unexpected auditory stimuli for the last 1 year. The boy had a minimum of four episodes per day despite maximal medical management with multiple antiepileptic drugs. Magnetic resonance imaging of the brain showed severe atrophy of the whole of the left hemisphere. Interictal electroencelphographic (EEG) data showed predominantly left central epileptiform discharges. Long-term video-EEG monitoring record showed seizures of the left hemispheric origin. He underwent a left functional hemispherotomy and has been totally seizure free in the 12-month follow-up period with an improved quality of life both for him and for his caregivers. We feel that surgical management should be considered in startle epilepsy with infantile hemiparesis when seizures are refractory to the optimal medical treatment.

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“…Vagus nerve stimulation was effective in isolated case reports [27]. Non-invasive sound generator treatment [28] could reduce 50% of seizure-frequency in two patients out of four. 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.…”

Section: Treatmentmentioning

confidence: 96%

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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Noninvasive treatment alternative for intractable startle epilepsy

Cited by 5 publications

References 14 publications

Successful epilepsy surgery in frontal lobe epilepsy with startle seizures: a SEEG studyFrontal epilepsy with startle seizures

Successful epilepsy surgery in frontal lobe epilepsy with startle seizures: a SEEG studyFrontal epilepsy with startle seizures

Symptomatic Case of Startle Epilepsy with Infantile Brain Damage Treated with Hemispherotomy: A Case Report and Review of Literature

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

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