Tracking the source of cerebellar epilepsy: Hemifacial seizures associated with cerebellar cortical dysplasia

Lascano, Agustina Maria; Lemkaddem, Alia; Granziera, Cristina; Korff, Christian; Boëx, Colette; Jenny, Benoit; Schmitt‐Mechelke, Thomas; Thiran, Jean-Philippe; Garibotto, Valentina; Vargas, Maria Isabel; Schaller, Karl; Seeck, Margitta; Vulliémoz, Serge

doi:10.1016/j.eplepsyres.2012.12.010

Cited by 18 publications

(13 citation statements)

References 13 publications

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“…lissencephaly 2 and Walker‐Warburg syndrome; ARX phenotypes). Rare cases of cerebellar dysgenesis in patients with epilepsy have been described, however (Lascano et al ., ). The infrequent cerebellar ganglioglioma merits further consideration, but present evidence does not refute the concept that the cerebellum is synaptically stable with low epileptogenic potential.…”

Section: Synapatic Factors Conducive To Epileptogenesismentioning

confidence: 99%

Might the olfactory bulb be an origin of olfactory auras in focal epilepsy?

Sarnat¹,

Flores‐Sarnat²

2016

Epileptic Disorders

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Olfactory auras (phantosmia) are an infrequent phenomenon in complex focal seizures generated in the mesial temporal lobe. It is generally assumed that all such auras arise from epileptic foci in the entorhinal cortex, amygdala or rostral insula, all of which have major afferent projections from the olfactory bulb or mainly from its relay, the anterior olfactory nucleus. The histological morphology, synaptic circuitry, and foetal development of the olfactory bulb are unique. The olfactory system is the only special sensory system that does not project to the thalamus because its bulb and tract incorporate an intrinsic thalamic equivalent: axonless granular and periglomerular neurons and the anterior olfactory nucleus. The olfactory bulb exhibits continuous synaptic turnover throughout life. Other brain structures with synaptic plasticity (neocortex, hippocampus, and amygdala) are epileptogenic; synaptically stable structures (brainstem, cerebellum, and basal ganglia) are not epileptogenic. Electrophysiological and neuropathological data of the olfactory bulb in epilepsy are sparse. We propose an alternative hypothesis, first hinted in 1954 by Penfield and Jasper, that some epileptic olfactory auras are primarily generated by the olfactory bulb and secondarily mediated by the amygdala and entorhinal cortex.

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Section: Synapatic Factors Conducive To Epileptogenesismentioning

confidence: 99%

Might the olfactory bulb be an origin of olfactory auras in focal epilepsy?

Sarnat¹,

Flores‐Sarnat²

2016

Epileptic Disorders

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“…the finger-nose-finger test). The issue is not exclusive to MEG -to date, few studies with scalp cerebellar EEG have been reported (Muthukumaraswamy et al 2003;Lascano et al 2013;Todd et al 2018). It is widely assumed that scalp EEG recordings for the cerebellum suffer from muscle artefacts (Muthukumaraswamy, 2013).…”

Section: Introductionmentioning

confidence: 99%

Using optically pumped magnetometers to measure magnetoencephalographic signals in the human cerebellum

Lin

Tierney

Holmes

et al. 2019

The Journal of Physiology

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Key points The application of conventional cryogenic magnetoencephalography (MEG) to the study of cerebellar functions is highly limited because typical cryogenic sensor arrays are far away from the cerebellum and naturalistic movement is not allowed in the recording. A new generation of MEG using optically pumped magnetometers (OPMs) that can be worn on the head during movement has opened up an opportunity to image the cerebellar electrophysiological activity non‐invasively. We use OPMs to record human cerebellar MEG signals elicited by air‐puff stimulation to the eye. We demonstrate robust responses in the cerebellum. OPMs pave the way for studying the neurophysiology of the human cerebellum. Abstract We test the feasibility of an optically pumped magnetometer‐based magnetoencephalographic (OP‐MEG) system for the measurement of human cerebellar activity. This is to our knowledge the first study investigating the human cerebellar electrophysiology using optically pumped magnetometers. As a proof of principle, we use an air‐puff stimulus to the eyeball in order to elicit cerebellar activity that is well characterized in non‐human models. In three subjects, we observe an evoked component at approx. 50 ms post‐stimulus, followed by a second component at approx. 85–115 ms post‐stimulus. Source inversion localizes both components in the cerebellum, while control experiments exclude potential sources elsewhere. We also assess the induced oscillations, with time‐frequency decompositions, and identify additional sources in the occipital lobe, a region expected to be active in our paradigm, and in the neck muscles. Neither of these contributes to the stimulus‐evoked responses at 50–115 ms. We conclude that OP‐MEG technology offers a promising way to advance the understanding of the information processing mechanisms in the human cerebellum.

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“…Furthermore, gangliogliomas are among the most common epileptogenic lesions in epilepsy centers [21], harboring a major propensity for spontaneous electrical discharges which probably explains the association of this lesion to this unique syndrome [22]. Not unexpectedly, histological studies of cerebellar or fourth ventricle lesions presenting with subcortical epilepsy were classified as ganglioglioma [2,4,9,10,12,[23][24][25][26][27], hamartoma [8,13,14,28], gangliomatous hamartoma [29], ganglioneurocytoma [30][31][32] or low-grade dense fibrillary astrocytoma [3,15]. Although many of these lesions may carry a developmental origin and actually represent a continuum with FCD, some are not intrinsically epileptogenic [33].…”

Section: Discussionmentioning

confidence: 95%

Dysplastic Cerebellar Epilepsy: Complete Seizure Control Following Resection of a Ganglioglioma

et al. 2015

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Subcortical epilepsy has been a controversial issue, partially settled by evidence showing seizure generation in hypothalamic hamartomas and also by reports of seizures caused by cerebellar lesions. We report 4-year-old girl with right hemifacial seizures and autonomic phenomena, in whom MRI showed an irregular mass in the right cerebellar peduncle. Despite several unremarkable video-EEG recordings, seizure origin in the lesion was hypothesized. Complete resection was feasible, histopathology showed a ganglioglioma, and she has been seizure free for 3 years. A fine line separates these developmental tumors from focal cortical dysplasia, and the homogeneous presentation of this entity led us to propose the terminology dysplastic cerebellar epilepsy.

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Tracking the source of cerebellar epilepsy: Hemifacial seizures associated with cerebellar cortical dysplasia

Cited by 18 publications

References 13 publications

Might the olfactory bulb be an origin of olfactory auras in focal epilepsy?

Might the olfactory bulb be an origin of olfactory auras in focal epilepsy?

Using optically pumped magnetometers to measure magnetoencephalographic signals in the human cerebellum

Dysplastic Cerebellar Epilepsy: Complete Seizure Control Following Resection of a Ganglioglioma

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