New-onset musicogenic epilepsy after temporal lobe epilepsy surgery.

Musicogenic epilepsy is a reflex epilepsy provoked by listening to or playing music. The epileptogenic network involves temporal regions, usually mesiotemporal structures. We present a 31-year-old female patient who experienced musicogenic seizures after a right temporal lobectomy with amygdalohippocampectomy that was performed in order to treat preexisting right mesio-temporal epilepsy.

[State of the art in musicogenic epilepsy: a review of the scientific literature]. / Conocimiento actual de la epilepsia musicogena: revision de la literatura cientifica.

INTRODUCTION: Musicogenic epilepsy is a type of reflex complex epilepsy and is evoked by musical stimulus, specially music with high emotional content to the patient. AIM: To review the state of the art of the musicogenic epilepsy in the current literature. DEVELOPMENT: We have made an analysis of all the patients that have been described with musicogenic epilepsy. Among the most studied variables we have included: the nature of the sound that evoke the seizure, the musical education of the patients, the changes between the different neurophysiological and neuroimaging techniques, the most frequent location and the hemispheric side implication in seizures, and comparison between the different neuroimaging techniques. Most of the patients had seizures when they were listening to music with emotional content. The right temporal lobe is the most affected area in this type of epilepsy. In these studies the structural neuroimaging were normal in most of the patients. CONCLUSIONS: Musicogenic epilepsy is reflex and complex and music with emotional content to the patients is the most frequent stimulus that evoke this type of epilepsy. The right temporal lobe is the most affected area. It seems that exists a correlation between emotional and auditory cerebral processing networks.

TITLE: Conocimiento actual de la epilepsia musicogena: revision de la literatura cientifica.Introduccion. La epilepsia musicogena se clasifica dentro del grupo de las epilepsias reflejas complejas y se caracteriza por desencadenarse con estimulos musicales, especialmente con fragmentos musicales con alta emotividad en la melodia. Objetivo. Revisar el conocimiento actual de las epilepsias musicogenas en la literatura cientifica. Desarrollo. Se ha realizado una revision detallada de los articulos que versan sobre epilepsia musicogena. Para ello se ha llevado a cabo un analisis de todos los casos clinicos descritos en la bibliografia hasta febrero de 2019. Entre las variables mas frecuentes se han estudiado: el tipo de estimulo sonoro desencadenante de las crisis, la presencia o no de educacion musical del paciente, los cambios en las diferentes pruebas complementarias neurofisiologicas y de neuroimagen, y la localizacion y lateralidad hemisferica implicada en este fenomeno. La mayoria de los pacientes tiene crisis evocadas por musica con contenido emocional para ellos. El lobulo temporal derecho es el mas frecuentemente implicado en este tipo de crisis, pero en la mayoria de los casos no se han observado alteraciones estructurales en las pruebas de neuroimagen. Conclusiones. La epilepsia musicogena es refleja y compleja y se caracteriza por desencadenarse con estimulos musicales. La zona que mas se afecta en las pruebas funcionales es el lobulo temporal derecho. Se piensa que existe una relacion entre las redes cerebrales que se encargan del procesamiento de las emociones a traves del estimulo auditivo y el desarrollo de este tipo de epilepsia.

Successful treatment of reflex epilepsy with praxis induction by stimulus avoidance only.

PURPOSE: Reflex epilepsy is a type of epilepsy with seizures that are consistently triggered by a specific stimulus. Zipai is a Chinese ancient card game which has been popular in Southern China for hundreds of years. We sought to report and characterize clinical features of patients with reflex epilepsy evoked by playing Zipai. METHODS: We collected and analyzed clinical data of patients with Zipai-induced epilepsy. Patients were regarded as having Zipai-induced epilepsy if they suffered at least two seizure attack during the course of playing Zipai. Prolonged electroencephalography (EEG) and brain magnetic resonance imaging (MRI) were applied to all patients. All patients were advised to avoid watching and playing Zipai games in daily life, instead of using antiepileptic drugs. The seizure outcome was assessed during outpatient visits and by telephone contact. RESULTS: Five patients were included in this study. No spontaneous seizures occurred in all five patients. No patients had experienced myoclonic and coexistent absences with generalized tonic-clonic seizures (GTCS). All patients had normal MRI and prolonged EEG findings. All patients were advised to avoid the Zipai game, and became seizure-free without medication during the follow-up period (mean 5.4 years, range 3.5-7 years). CONCLUSION: Zipai-induced epilepsy may be an unreported subtype form of reflex epilepsy with praxis induction. Nonpharmacological conservative treatment plays a significant role in the treatment of reflex epilepsy.

Clinical and electrophysiological findings in patients with phenylketonuria and epilepsy: Reflex features.

OBJECTIVE: Phenylketonuria (PKU) is the most common form of amino acid metabolism disorders with autosomal recessive inheritance. The brain damage can be prevented by early diagnosis and a phenylalanine-restricted diet. Untreated or late-treated patients may show mental retardation and other cognitive dysfunctions, as well as motor disability and/or epilepsy. METHODS: Three patients with PKU and epilepsy were recognized to have reflex epileptic features, and there were ten consecutive adult patients with PKU and epilepsy who were evaluated retrospectively. Medical history, ages at diagnosis and therapy onset, age at seizure onset, seizure types and reflex features, neurological findings, cranial imaging, electroencephalography (EEG) findings, and final clinical condition were evaluated. Reflex epilepsy features were examined in detail. RESULTS: The cases (6 females, 4 males) were diagnosed at ages between 3.5months and 12years. All patients had various degrees of mental-motor retardation and focal or generalized seizures with age at seizure onset varied between neonatal period and 15years. Three patients had febrile seizure, 3 patients had myoclonia, and 3 patients had status epilepticus. All patients had abnormal EEG findings except one. There was a slowing of background activity, and generalized discharges were observed in 7 patients; 3 of them had asymmetrical discharges. One patient had right hippocampal sclerosis (HS), and another patient had hypointensities in the basal ganglia and corpus callosum. Reflex features were clinically observed in 3 of the patients; however, EEG results did not show any related findings. One patient had reflex seizures triggered by photic stimuli, hot water, and startling; one by photic stimuli; and the other one by startling. CONCLUSION: Reports on the clinical and electrophysiological features of adult patients with PKU were scant. We emphasized that reflex clinical features may be observed in this metabolic disease, and focal epileptiform abnormalities and asymmetry may be present in electrophysiological evaluation besides the rare association with HS.

Photosensitive epilepsy is associated with reduced inhibition of alpha rhythm generating networks.

See Hamandi (doi:10.1093/awx049) for a scientific commentary on this article.Photosensitivity is a condition in which lights induce epileptiform activities. This abnormal electroencephalographic response has been associated with hyperexcitability of the visuo-motor system. Here, we evaluate if intrinsic dysfunction of this network is present in brain activity at rest, independently of any stimulus and of any paroxysmal electroencephalographic activity. To address this issue, we investigated the haemodynamic correlates of the spontaneous alpha rhythm, which is considered the hallmark of the brain resting state, in photosensitive patients and in people without photosensitivity. Second, we evaluated the whole-brain functional connectivity of the visual thalamic nuclei in the various populations of subjects under investigation. Forty-four patients with epilepsy and 16 healthy control subjects underwent an electroencephalography-correlated functional magnetic resonance imaging study, during an eyes-closed condition. The following patient groups were included: (i) genetic generalized epilepsy with photosensitivity, 16 subjects (mean age 25 ± 10 years); (ii) genetic generalized epilepsy without photosensitivity, 13 patients (mean age 25 ± 11 years); (iii) focal epilepsy, 15 patients (mean age 25 ± 9 years). For each subject, the posterior alpha power variations were convolved with the standard haemodynamic response function and used as a regressor. Within- and between-groups second level analyses were performed. Whole brain functional connectivity was evaluated for two thalamic regions of interest, based on the haemodynamic findings, which included the posterior thalamus (pulvinar) and the medio-dorsal thalamic nuclei. Genetic generalized epilepsy with photosensitivity demonstrated significantly greater mean alpha-power with respect to controls and other epilepsy groups. In photosensitive epilepsy, alpha-related blood oxygen level-dependent signal changes demonstrated lower decreases relative to all other groups in the occipital, sensory-motor, anterior cingulate and supplementary motor cortices. Coherently, the same brain regions demonstrated abnormal connectivity with the visual thalamus only in epilepsy patients with photosensitivity. As predicted, our findings indicate that the cortical-subcortical network generating the alpha oscillation at rest is different in people with epilepsy and visual sensitivity. This difference consists of a decreased alpha-related inhibition of the visual cortex and sensory-motor networks at rest. These findings represent the substrate of the clinical manifestations (i.e. myoclonus) of the photoparoxysmal response. Moreover, our results provide the first evidence of the existence of a functional link between the circuits that trigger the visual sensitivity phenomenon and those that generate the posterior alpha rhythm.

Recurrence of Epileptic Spasms as Reflex Seizures Induced by Eating: A Case Report and Literature Review.

Background Eating epilepsy (EE) is a rare form of reflex epilepsy in which seizures are induced by eating. It is known that most patients with eating seizures, in fact, suffer from symptomatic temporal lobe epilepsy (TLE), whereas only a few patients with epileptic spasms induced by eating (E-ES) have been reported. Patient Description The patient was an 8-year-old girl whose magnetic resonance imaging (MRI) of the head detected dysgenesis of the corpus callosum, cerebellar hypogenesis, marked cerebral asymmetry, broad polymicrogyria, periventricular heterotopia, and closed lip-type schizencephaly. She experienced E-ES as the second form of recurrent seizures after the first recurrence of spontaneous ES. After E-ES occurred, the EEG findings in the right hemisphere, predominantly over the right centrotemporal region, were clearly exacerbated, although the interictal EEG originally showed left-side-dominant asymmetric hypsarrhythmia. The ictal EEG of the E-ES showed diffuse large triphasic (negative-positive-negative) potentials, predominantly over the right centrotemporoparietal region. Conclusions This is a unique case because the E-ES were recurrent ES, although the previous ES were spontaneous, which may provide insight into the mechanism of E-ES.

Voxel-based morphometry in epileptic baboons: Parallels to human juvenile myoclonic epilepsy.

The epileptic baboon represents a natural model for genetic generalized epilepsy (GGE), closely resembling juvenile myoclonic epilepsy (JME). Due to functional neuroimaging and pathological differences between epileptic (SZ+) and asymptomatic control (CTL) baboons, we expected structural differences in gray matter concentration (GMC) using voxel-based morphometry (VBM). Standard anatomical (MP-RAGE) MRI scans using a 3T Siemens TIM Trio (Siemens, Erlangen, Germany) were available in 107 baboons (67 females; mean age 16±6years) with documented clinical histories and scalp-electroencephalography (EEG) results. For neuroimaging, baboons were anesthetized with isoflurane 1% (1-1.5 MAC) and paralyzed with vecuronium (0.1-0.3mg/kg). Data processing and analysis were performed using FSL's VBM toolbox. GMC was compared between CTL and SZ+ baboons, epileptic baboons with interictal epileptic discharges on scalp EEG (SZ+/IED+), asymptomatic baboons with abnormal EEGs (SZ-/IED+), and IED+ baboons with (IED+/PS+) and without (IED+/PS-) photosensitivity, and the subgroups amongst themselves. Age and gender related changes in gray matter volumes were also included as confound regressors in the VBM analyses of each animal group. Significant increases in GMC were noted in the SZ+/IED+ subgroup compared to the CTL group, including bilaterally in the frontopolar, orbitofrontal and anterolateral temporal cortices, while decreases in GMC were noted in the right more than left primary visual cortices and in the specific nuclei of the thalamus, including reticular, anterior and medial dorsal nuclei. No significant differences were noted otherwise, except that SZ+/IED+ baboons demonstrated increased GMC in the globus pallidae bilaterally compared to the SZ-/IED+ group. Similar to human studies of JME, the epileptic baboons demonstrated GMC decreases in the thalami and occipital cortices, suggesting secondary injury due to chronic epilepsy. Cortical GMC, on the other hand, was increased in the anterior frontal and temporal lobes, also consistent with human JME studies. This VBM study may indicate a combination of developmental and acquired structural changes in the epileptic baboon.

The Pivotal Role of the Parieto-Occipital Lobe in Card Game-Induced Reflex Epilepsy: A Voxel-Based Morphometry Study.

BACKGROUND: The pathogenesis of card game-induced reflex epilepsy has not been determined so far. The aim of this study was to evaluate structural abnormalities using voxel-based morphometry (VBM) analysis, which may give some clue about the pathogenesis in card game-induced reflex epilepsy. METHODS: The 3 subjects were diagnosed with card game-induced reflex epilepsy. Evaluation involved a structured interview to obtain clinical information and brain MRI. In VBM analysis, Statistical Parametric Mapping 8 running on the MATLAB platform was employed to analyze the structural differences between patients with card game-induced reflex epilepsy and age- and sex-matched control subjects. RESULTS: The results of VBM analysis revealed that patients with card game-induced reflex epilepsy had significantly increased gray matter volume in the right occipital and parietal lobe. However, there were no structures with decreased gray matter volume in patients with card game-induced reflex epilepsy compared with control subjects. In addition, we found that the patients with card game-induced reflex epilepsy had onset of seizures in adulthood rather than in adolescence, and all of the patients were men. CONCLUSIONS: The parieto-occipital lobes might be partially involved in the neuronal network responsible for card game-induced reflex epilepsy.

Eating epilepsy: phenotype, MRI, SPECT and video-EEG observations.

BACKGROUND: Eating epilepsy is one of the rare forms of reflex epilepsy precipitated by eating. Previous studies have demonstrated lesions due to variable aetiology involving the temporolimbic and suprasylvian regions. OBJECTIVE: To study anatomical correlates of reflex eating epilepsy using multimodality investigations (MR imaging, video-EEG and SPECT). METHODOLOGY: Six patients (M:F=3:3; mean age: 20.7±4.9 years) with eating epilepsy were subjected to MRI of brain, video-EEG studies and SPECT scan. These were correlated with phenotypic presentations. RESULTS: Among the five patients with ictal recording of eating epilepsy during video-EEG, semiology was characterized by behavioural arrest followed by either flexion or extension of trunk and neck and two patients had speech arrest and four had salivation from angle of mouth. Another patient had EEG changes during "thought about eating". Four patients had perisylvian frontal lobe lesions and one had high frontal lesion on MRI. Ictal EEG (n=6) showed ictal rhythmic slowing/fast activity in parieto-temporal (n=2) or fronto-temporal (n=4) regions with subsequent secondary generalization in three. Ictal and interictal SPECT imaging showed changes in frontal lobe (n=1), anterior temporal lobe (n=1), and parieto-insular region (n=1) suggesting it to be seizure onset zone. Three of four patients with structural lesions in MRI had concordant ictal EEG and ictal SPECT changes. CONCLUSION: Lesions near the perisylvian region might play a major role in eating epilepsy.

Somatosensory rub evoked reflex epilepsy of a temporal lobe origin.

Somatosensory rub reflex epilepsy, evoked by prolonged or repetitive cutaneous contact of a circumscribed body area, is an unusual form of reflex epilepsy. The peculiar complaints and the negative interictal electroencephalographic (EEG) recordings make it difficult to identify the epileptic origin. Here we report an unusual case whose seizures would be evoked by a touch or rub on a unilateral arm and shoulder, with a contralateral temporal lobe origin, demonstrated in immediate postictal single-photon emission computed tomography (SPECT).

Reading epilepsy from the dominant temporo-occipital region.

BACKGROUND: Reading epilepsy is a rare form of epilepsy, classified among idiopathic, age- and localisation-related (partial) epilepsies as a reflex epilepsy syndrome. Seizures usually consist of myoclonic jerks restricted to the jaw. However, distinct ictal features including visual symptoms and paroxysmal a- or dyslexia are described in some patients. The anatomical substrate of ictogenesis in reading epilepsy remains poorly understood. METHODS: The authors report here the case of a primary reading epilepsy for which ictal semiology was characterised by visual symptoms and dyslexia, investigated by MRI, interictal high-resolution EEG and PET, ictal video-EEG and SPECT. Brain MRI was normal. Interictal high-resolution EEG was performed with 64 scalp channels, a realistic head model and different algorithms to solve the inverse problem. RESULTS: Interictal source localisations highlighted the left occipito-temporal junction. Interictal PET demonstrated bilateral occipito-temporal hypometabolism with left-sided predominance. Ictal EEG showed a rhythmic discharge in left temporo-parieto-occipital junction channels, with left occipito-temporal predominance. MRI fusion of the coregistered subtraction between ictal and interictal SPECT individualised relative hyperperfusion affecting (a) the left occipito-parietal junction area, (b) the left lateral middle and inferior temporal gyri and (c) the left inferior frontal area. CONCLUSION: Besides reading-induced myoclonic jerks of the jaw, a second variant of reading epilepsy exists with clearly partial seizures manifested by visual symptoms and a- or dyslexia. These seizures originate from the occipito-temporal region of the dominant hemisphere, corresponding to the posterior part of the neural network that underlies the function of reading.

Seizure propagation in a patient with musicogenic epilepsy.

Musicogenic epilepsy (ME) is a condition in which seizures are triggered by music. We describe a novel investigation of the mechanisms of ME using noninvasive (ictal 2-flurodeoxyglucose (2-FDG) positron emission tomography (PET)) and invasive (subdural arrays and depth electrodes) methodology in one patient with ME. Ictal PET defined onsets in the right mesial temporal lobe. This was confirmed by intracranial electrode recordings, where there was onset in the right mesial temporal lobe, followed by sequential spread to the lateral temporal cortex, Heschel's gyrus, insula, and frontal lobes. Right temporal lobectomy resulted in Engel class 1 outcome.

Neural correlates of musicogenic epilepsy: SISCOM and FDG-PET.

To localize the neural correlates of musicogenic epilepsy, subtraction ictal SPECT coregistered with MRI (SISCOM) and (18)F-fluorodeoxy glucose positron emission tomography (FDG-PET) were performed in a woman who had suffered from frequent musicogenic seizures. She had complex partial seizures consisting of palpitation and an unpleasant feeling, which were followed by staring and oroalimentary automatisms. Ictal EEG showed rhythmic theta waves originated from the right temporal lobe, and SISCOM showed ictal hyperperfusion on right insula, amygdala, hippocampal head, and anterior temporal lobe, whereas interictal FDG-PET showed interictal hypometabolism in the same brain regions, suggesting dysfunction and abnormal activation of right temporo-limbic structures related to an emotional response to music.

PET imaging in the photosensitive baboon: case-controlled study.

PURPOSE: The baboon (Papio hamadryas spp) offers a natural primate animal model of photosensitive generalized epilepsy. This study compared changes in cerebral blood flow (CBF) during intermittent light stimulation (ILS) between photosensitive and asymptomatic baboons. METHODS: Six photosensitive, epileptic (PS) and four nonphotosensitive, asymptomatic (CTL) baboons, matched for age, gender, and weight, were selected based on previous scalp EEG evaluation. Continuous intravenous ketamine (5-13 mg/kg) was used for sedation. Subjects underwent five sequential blood-flow PET studies within 60 min with 20 mCi (15)O-labeled water. Images were acquired in 3D mode (CTI/Siemens HR+ scanner, 63 contiguous slices, 2.4-mm thickness). Three resting scans were alternated with two activation scans during ILS. ILS was performed at 25 Hz for 60 s before to 60 s after the start of an activation scan. PET images were coregistered with MRI (3T Siemens Trio, T(1)-weighted 3D Turboflash sequence; TE/TR/TI, 3.04/2,100/785 ms; flip angle, 13 degrees). PET scans were reviewed and corrected for motion artifact. Resting scans were contrasted with activation scans and averaged independently for both groups. Quantitative CBF analyses were performed for the occipital and motor cortices. RESULTS: The CTL baboons showed greatest ILS-induced activation in the left middle frontal and inferior temporal gyri, left brainstem structures and right postcentral gyrus, bilateral occipital lobes, and in the posterior cingulate gyrus and cerebellum. In contrast, the PS animals showed strongest ILS activation in the right anterior cingulate and medial orbital gyri, amygdala, globus pallidum, and left inferior and superior temporal gyri. A striking finding was the absence of occipital and variable motor cortex activation in the PS animals. Deactivations were noted in the right orbitofrontal and anterior cingulate cortices in the CTL baboons and in the posterior cingulate gyrus, brainstem and cerebellum of the PS animals. CONCLUSIONS: The patterns of ILS-induced CBF changes differed between CTL and PS groups. These differences of activations and inhibitions suggest involvement of specific cortical-subcortical or networks in photosensitivity.

Ictal SPECT in a case of pure musicogenic epilepsy.

A 39-year-old, right-handed woman had seizures for two years which were always triggered by exposure to various types of music: the first occurred while she listened to a tune she particularly liked, Con Te Partiro, by Andrea Boccelli. Other triggering factors were various types of music such as supermarket background music and polyphonic singing or instrumental music played by family members. The seizures had a stereotyped course: she felt anxious, tearful, then occurred slight obtundation, during which she smacked her lips and moved restlessly. There was no complete loss of consciousness, but some degree of amnesia. She never experienced a generalized tonic-clonic seizure, but reported rare spontaneous feelings of déjà-vu that had begun at the same time as the induced seizures. There were no other spontaneous attacks; only one seizure was apparently provoked, not by music but by a loud background noise in her office. She was a music lover and a singer. Interictal EEG showed independent slow waves over the temporal regions. Several seizures with EEG localisation over the right temporal region were elicited after several minutes of exposure to music. Monoauricular stimulation with the same music produced a seizure when applied to the left ear but was ineffective when applied to the right ear. Ictal SPECT demonstrated right temporal hyperperfusion. MRI was normal. On high dose of carbamazepine, seizure frequency decreased. The addition of topiramate resulted in full seizure control. Musicogenic epilepsy is a rare form of reflex epilepsy. Pure cases, when patients do not experience unprovoked seizures, are exceptional. Our report confirms the implication of the right temporal lobe in this epilepsy.