Sleep in juvenile myoclonic epilepsy: A systematic review.

Sleep disturbances significantly impact the lives of individuals with Juvenile Myoclonic Epilepsy (JME). This study aimed to investigate sleep studies, disturbances, and the impact of anti-seizure drugs on sleep in JME patients. Relevant studies were retrieved from the National Library of Medicine (Pubmed) database and the Cochrane Library utilizing the search terms "Juvenile Myoclonic Epilepsy" and "sleep". A total of 160 papers' review, data extraction, and resolution of discrepancies were performed independently by two reviewers according to the PRISMA protocol and were registered in PROSPERO (CRD42023472439). A systematic review of 31 studies was conducted, encompassing various methodologies, including sleep questionnaires (Pittsburgh Sleep Quality Index (n = 13), Epworth Sleepiness Scale (n = 10)), polysomnography (n = 8), EEG (n = 9), actigraphy (n = 1), and transcranial magnetic stimulation (n = 1). Most studies were hospital-based (n = 31), cross-sectional (n = 11), and prospective (n = 25). Patients with JME exhibit a higher prevalence of sleep disturbances, worse quality of sleep (n = 4), daytime sleepiness (n = 2), sleep efficiency (n = 7), and increased sleep latency (n = 1) compared to controls. These disruptions are characterized by increased wakefulness (n = 3), frequent arousals (n = 3), decreased REM sleep (n = 2), and conflicting NREM sleep findings (n = 3). Additional sleep-related issues observed in JME patients include insomnia (n = 1) and increased prevalence of parasomnias such as nightmares and sleep talking. Periodic limb movement and obstructive sleep apnea are similar or less frequent (3/28). REM behavioral disorders and sleepwalking were not seen. Valproate showed conflicting effects on sleep (n = 7), while levetiracetam did not impact sleep (n = 1). These findings underlined the need for more sufficient evidence of sleep studies in JME. Future research should prioritize understanding the nature of sleep in JME and its impact on management.

Sarcopenia and anti-seizure medication response in juvenile myoclonic epilepsy.

INTRODUCTION: This study aimed to investigate the presence of sarcopenia in patients with juvenile myoclonic epilepsy (JME) and the association between sarcopenia and response to anti-seizure medication (ASM) in patients with JME. METHODS: We enrolled 42 patients with JME and 42 healthy controls who underwent brain magnetic resonance imaging with three-dimensional T1-weighted imaging. We measured the temporal muscle thickness (TMT), a radiographic marker for sarcopenia, using T1-weighted imaging. We compared the TMT between patients with JME and healthy controls and analyzed it according to the ASM response in patients with JME. We also performed a receiver operating characteristic (ROC) curve analysis to evaluate how well the TMT differentiated the groups. RESULTS: The TMT in patients with JME did not differ from that in healthy controls (9.630 vs. 9.956 mm, p = .306); however, ASM poor responders had a lower TMT than ASM good responders (9.109 vs. 10.104 mm, p = .023). ROC curve analysis revealed that the TMT exhibited a poor performance in differentiating patients with JME from healthy controls, with an area under the ROC curve of .570 (p = .270), but good performance in differentiating between ASM good and poor responders, with an area under the ROC curve of .700 (p = .015). CONCLUSION: The TMT did not differ between patients with JME and healthy controls; however, it was reduced in ASM poor responders compared to ASM good responders, suggesting a link between ASM response and sarcopenia in patients with JME. TMT can be used to investigate sarcopenia in various neurological disorders.

A comparison of comorbid headache between patients with temporal lobe epilepsy and juvenile myoclonic epilepsy.

Headache is one of the most common symptoms of epilepsy comorbidities. However, the relationship between the epilepsy and headache still needs clarification. Previous studies mostly investigated the overall incidence and clinical features of the headache in patients with the epilepsy. Temporal lobe epilepsy (TLE) and juvenile myoclonic epilepsy (JME) are the common types of focal epilepsy and generalized epilepsy, respectively. Nevertheless, there was no study comparing the clinical features of headache between TLE and JME. This study aimed to analyze the headache features of these two types of epilepsy. Patients with either TLE or JME diagnosed with headache and referred to the West China Hospital of Sichuan University were consecutively recruited from June 2021 to June 2022. The duration of epilepsy was longer than 6 months in these patients. Data on headache and epilepsy were obtained through face-to-face questionnaires. The headache was classified according to the International Classification Headache Disorders-3rd edition (ICHD-III) criteria. χ2-test, t-test, rank-sum test, logistic regression modeling and Mann Whitney test were used to compare the clinical differences of the headache in TLE and JME. A total of 151 TLE patients and 30 JME patients were enrolled in this study. There was no significant difference in the family history of headache, epilepsy durations, headache types, proportion receiving analgesic therapy, the frequency of inter-ictal headache (inter-IH), and the quality of life in epilepsy -10 inventory (QOLIE-10) between the TLE and JME patients. Patients in the TLE group were significantly older (p = 0.004), and a lower percentage of them had a family history of epilepsy (p = 0.007) compared with the JME patients. The proportion of cases with refractory epilepsy was higher in the TLE group than that in the JME group (p < 0.001). The types of seizures in the TLE group varied from those in the JME group (p < 0.001). The composition of the antiseizure medications (ASM) applied in the TLE group differed from that in the JME group (p = 0.047), and the usage of oxcarbazepine was more frequently in the TLE group than in the JME group (p = 0.003). There was no difference in the headache types among patients with TLE or JME. Specifically, 67 (44.37%), 12 (7.95%), and 118 (7.95%) patients were found with inter-IH, pre-ictal headache (Pre-IH) and post-ictal headache (Post-IH) in the TLE group; while 8 (26.67%), 4 (13.33%) and 26 (86.67%) patients had inter-IH, Pre-IH and Post-IH in the JME group. Thirty-nine patients in the TLE group and 4 patients in the JME group were identified with more than one type of headaches, respectively. Tension-type headache (TTH) were found in 38 patients (25.17%) in the TLE group and 3 patients (10.00%) in the JME group, respectively; migraines were found in 10 patients (6.62%) in the TLE group and in 2 patients (6.67%) in the JME group. Patients in the TLE group had a higher headache-attributed lost time-90 days (HLT-90) score than those in the JME group (p = 0.019). The proportion of patients with inter-IH accompanied by nausea in the TLE group was higher than that in the JME group (p = 0.029), while the proportion of patients with frontal headache was lower than that in the JME group (p < 0.05). There was no significant difference in headache severity, quality, headache nature, unilateral/bilateral, and headache duration either in inter-IH or peri-ictal headache (Peri-IH) between the two groups. The logistic regression analysis suggested that except for HLT-90 (AUC = 0.622, p = 0.027), other factors were not found to be correlated with refractory epilepsy. The clinical features of headache differed between TLE and JME patients. TLE patients had a higher ratio of refractory epilepsy, more headache time loss compared with JME patients. HLT-90 was associated with the occurrence of refractory epilepsy in TLE patients. Taken together, we suggested that the comorbid headache may essentially be different between TLE and JME patients.

Two-year efficacy of lacosamide as adjunctive therapy for generalized tonic-clonic seizures in patients with juvenile myoclonic epilepsy.

OBJECTIVE: To report the long-term efficacy of adjunctive lacosamide therapy in patients with juvenile myoclonic epilepsy whose generalized tonic-clonic seizures were significantly reduced by treatment. METHODS: A retrospective study was conducted in patients who visited the Department of Child Neurology, National Hospital Organization Nishiniigata Chuo Hospital and the Department of Pediatrics, National Hospital Organization Nagasaki Medical Center. Among patients who had been diagnosed with juvenile myoclonic epilepsy, those who received lacosamide as adjunctive therapy for refractory generalized tonic-clonic seizures for at least 2 years from January 2017 to December 2022, and who achieved seizure freedom or >50% seizure reduction in tonic-clonic seizures were included. The medical records and neurophysiological data of the patients were reviewed retrospectively. RESULTS: Four patients met the inclusion criteria. The mean age at the onset of epilepsy was 11.3 years (range 10-12), and the mean age of starting lacosamide was 17.5 years (range 16-21). All patients received two or more antiseizure medications prior to lacosamide. Three of four patients had seizure freedom for more than 2 years, and the one remaining patient had >50% seizure reduction for more than one year. Only one patient had recurrent myoclonic seizures after starting lacosamide. The mean lacosamide dose at the last visit was 425 mg/day (range 300-600). CONCLUSION: Adjunctive lacosamide therapy might be a treatment option for juvenile myoclonic epilepsy with generalized tonic-clonic seizures, which are not responsive to standard antiseizure medications.

Prevalence, nature, and severity of the psychiatric comorbidities and their impact on quality of life in adolescents with Juvenile myoclonic epilepsy.

INTRODUCTION: Adults with Juvenile myoclonic epilepsy (JME) are at increased risk for psychiatric comorbidities, personality traits, and abnormality in executive function. But studies on adolescents and their impact on quality of life are scarce in the literature. MATERIALS AND METHODS: This cross-sectional study was performed between August 2019 and October 2022 to compare the prevalence of psychiatric comorbidities in adolescents with JME and age and gender-matched healthy controls. After completing DSM-5 Structured Clinical Interview (SCID-5) initially in all patients, we measured the severity of individual psychiatric problems like anxiety, depression, and somatic symptoms by using an appropriate psychometric scale. We also measured both groups' intelligence quotient (IQ), executive function, and quality of life. RESULTS: One hundred patients with JME (14.3 ± 2.5 years, 48 boys) and 100 controls were enrolled. Psychiatric disorders were observed in 46% of JME and 6% of controls (p < 0.01). Psychiatric comorbidities noted in the patients with JME were: somatic symptom and related disorders(n = 14), anxiety (n = 13), adjustment disorders (n = 12), depression (n = 11), oppositional defiant disorder (n = 6), conduct disorder (n = 5), anorexia nervosa (n = 3), narcissistic (n = 3), histrionic (n = 1), substance-related disorder (n = 1), borderline (n = 2) and antisocial personality disorder (n = 2). The prevalence of depressive disorders, anxiety disorders, adjustment disorders, somatic symptoms, related disorders, and any personality disorder was significantly more in the JME group (p < 0.01 for all). Female gender, higher Epilepsy Stigma Scale score, and lower Epilepsy Outcome Expectancy Scale were significantly associated with depressive disorders (p = 0.04, 0.03, 0.03 respectively). Similarly, for anxiety, only female gender and lower Epilepsy Outcome Expectancy Scale were significant associated factors (p = 0.03, 0.02 respectively). CONCLUSIONS: Psychiatric disorders like anxiety, depression, and personality disorders are more frequent in adolescents with JME than in controls.

Prevalence of headache disorders in patients living with epilepsy in rural region in western part of India.

BACKGROUND AND AIMS: Headache disorders cause significant distress in patients living with epilepsy (PWE) and are underreported. This study aimed to evaluate the prevalence of various forms of headache in PWE. METHODOLOGY: Two hundred and three PWE were evaluated for the presence and type of headache as per the International Classification of Headache Disorders (ICHD)-3 classification criteria. The severity was graded using the Headache Under Response to Treatment (HURT)-3. A subgroup analysis of headache and epilepsy was done. The World Health Organization-5 (WHO-5) questionnaire was used to assess mental well-being. The Mini International Neuropsychiatric Interview (MINI) questionnaire was used to study the psychiatric comorbidities, which were classified according to the Diagnostic and Statistical Manual of Mental Disorders (DSM)-IV classification. RESULTS: The prevalence of headaches in PWE was 45% [60% females]. Female gender and younger age were significantly associated with migraine (p values of 0.03 and 0.05, respectively). Migraine was the most common type of headache (71%), followed by tension-type headache (TTH) (23%) in PWE. The headache was inter-ictal in 80% of PWE. In PWE with migraine, both juvenile myoclonic epilepsy (JME) and frontal lobe epilepsy (FLE) had a 28% prevalence. In PWE with TTH, FLE was more common (43%). The prevalence of migralepsy in PWE was 4% [n = 4; 2 each of occipital lobe epilepsy (OLE) and idiopathic generalized epilepsy (IGE)]. A psychiatric illness was more than two times more likely in PWE with headache (n = 34; 37%) as compared to PWE without headache (n = 19; 17%). Patients living with epilepsy with headaches and psychiatric comorbidities had significantly lower mental well-being (p = 0.001). Forty five percent of PWE with headaches required acute management, and 35% required prophylactic management for their headache. CONCLUSION: Headache is frequently ignored in PWE. It can affect their mental health and quality of life. Evaluation and management of headache in PWE is very important.

Sleep quality and circadian rhythm profile of persons with juvenile myoclonic epilepsy in a tertiary epilepsy center: A case-control study.

PURPOSE: This study evaluated sleep quality, chronotype, and excessive diurnal somnolence in persons with Juvenile Myoclonic Epilepsy (JME) and their possible association with clinical variables. METHODS: This cross-sectional controlled study evaluated 49 consecutive patients (65% females, mean age 27.53 years) with an electroclinical diagnosis of JME and 49 healthy controls (55% females, mean age 28.55 years). The Pittsburgh Sleep Quality Inventory (PSQI) was used to assess sleep quality and the Epworth Sleepiness Scale (ESS) to evaluate excessive daytime sleepiness. The patients' chronotype was evaluated by the Morningness-Eveningness Questionnaire (MEQ). Epilepsy-related factors gathered from the medical chart and personal interview were epilepsy duration, age at onset, frequency of myoclonic (Mcl), generalized tonic-clonic (GTC) and absence (ABS) seizures, pharmacoresponse, and current antiseizure medication (ASM). RESULTS: Persons with JME did not differ from the control group regarding daytime sleepiness (p=0.840); however, the JME group had worse sleep quality (p=0.01) than the controls. Persons with JME presented a more evening chronotype than controls (p = 0.003). The age at onset, epilepsy duration, frequency of Mcl seizure, frequency of GTC seizure, frequency of ABS seizure, and drug response did not predict ESS and MEQ scales. Pharmacoresponsive patients had lower PSQI scores compared with pharmacoresistant patients (p=0.036). CONCLUSION: Persons with JME have worse sleep quality and a more evening chronotype. Notably, pharmacoresistant patients present a worse sleep quality that deserves attention and special care due to the relationship between sleep deprivation and seizure worsening.

Impulsivity traits in eyelid myoclonia with absences.

PURPOSE: Eyelid myoclonia with absences (EMA) shares some clinical characteristics with juvenile myoclonic epilepsy (JME), in which impulsivity traits have been described. Aim of the study was to evaluate whether EMA patients could present a peculiar behavioural profile. METHODS: Patients with EMA, JME and healthy controls (HCs) were enrolled. Subjects with intellectual quotient <80 were excluded from the study. All the enrolled subjects underwent the Italian version of the Barratt Impulsiveness Scale (BIS-11) and the three dimensions of impulsivity (motor, attentional-cognitive and nonplanning impulsivity) were considered. RESULTS: Seventeen patients with EMA (12 females [70.6%], age 30.8±10 years), 29 patients with JME (17 females [58.6%], age 29.1±9.7 years) and 31 HCs (15 females [48.4%], age 27.6±5.8 years) were enrolled. Both EMA and JME patients presented a borderline significantly higher BIS total score than HCs (p=0.064). EMA patients presented a significantly higher BIS nonplanning subscore than JME patients and HCs (p=0.001). CONCLUSION: The study showed the presence of peculiar behavioral characteristics in EMA patients, slightly different from patients with JME.

Functional connectivity disturbances of ascending reticular activating system and posterior thalamus in juvenile myoclonic epilepsy in relation with photosensitivity: A resting-state fMRI study.

OBJECTIVE: Juvenile myoclonic epilepsy (JME) is typified by the occurrence of myoclonic seizures after awakening, though another common trait is myoclonic seizures triggered by photic stimulation. We aimed to investigate the functional connectivity (FC) of nuclei in the ascending reticular activating system (ARAS), thalamus and visual cortex in JME with and without photosensitivity. METHODS: We examined 29 patients with JME (16 photosensitive (PS), 13 non- photosensitive-(NPS)) and 28 healthy controls (HCs) using resting-state functional magnetic resonance imaging (rs-fMRI). Seed-to-voxel FC analyses were performed using 25 seeds, including the thalamus, visual cortex, and ARAS nuclei. RESULTS: Mesencephalic reticular formation seed revealed significant hyperconnectivity between the bilateral paracingulate gyrus and anterior cingulate cortex in JME group, and in both JME-PS and JME-NPS subgroups compared to HCs (pFWE-corr < 0.001; pFWE-corr < 0.001; pFWE-corr = 0.002, respectively). Locus coeruleus seed displayed significant hyperconnectivity with the bilateral lingual gyri, intracalcarine cortices, occipital poles and left occipital fusiform gyrus in JME-PS group compared to HCs (pFWE-corr <0.001). Additionally, locus coeruleus seed showed significant hyperconnectivity in JME-PS group compared to JME-NPS group with a cluster corresponding to the bilateral lingual gyri and right intracalcarine cortex (pFWE-corr < 0.001). Lastly, the right posterior nuclei of thalamus revealed significant hyperconnectivity with the right superior lateral occipital cortex in JME-PS group compared to HCs (pFWE-corr < 0.002). CONCLUSIONS: In JME, altered functional connectivity of the arousal networks might contribute to the understanding of myoclonia after awakening, whereas increased connectivity of posterior thalamus might explain photosensitivity.

Prevalence of photoparoxysmal response in patients with epilepsy: Effect of the underlying syndrome and treatment status.

OBJECTIVE: To prospectively study the prevalence of photoparoxysmal response (PPR) and its determinants in epilepsy patients. METHODS: Consecutive patients, older than 2 years, undergoing EEG from January 2016 to December 2019 were prospectively studied for the presence of PPR. Patients with emergent EEG and those with only sleep record were excluded. Intermittent photic stimulation was performed as per standard techniques with frequencies from 1-30 Hz. RESULTS: Of the 1893 subjects included, 1492 (78%) patients had epilepsy while 401 (22%) had other diagnoses. In epilepsy group, 1028 (68.7%) had focal epilepsy, 343 (21.6%) had generalized epilepsy, while (9.7%) patients had unclassified epilepsy. Overall, 36 (2.2%) patients with epilepsy had PPR. The mean age of these patients was 19.5 ± 9.4 years and 75% were females. PPR was noted in 5 (0.5%) patients with focal epilepsy and 31 (9%) patients with generalized epilepsies [p < 0.0001; Odds ratio: 20.3 (95% CI, 7.8 - 52.7)]. PPR was noted in 1.5% of treated and 18% of untreated patients with genetic generalized epilepsy (n = 145) and 22% of untreated patients with juvenile myoclonic epilepsy (n = 86). Patients with untreated epilepsy had 17 times higher odds of having PPR [p < 0.0001; Odds ratio: 17.6 (95% CI, 4.1 - 75.6)]. CONCLUSION: Underlying epilepsy syndrome and treatment status are the two most important determinants of PPR. Variability in these two factors is largely responsible for the variable reported prevalence of PPR.

Trait impulsivity correlates with active myoclonic seizures in genetic generalized epilepsy.

BACKGROUND: Juvenile myoclonic epilepsy (JME) is a common subtype of genetic generalized epilepsy (GGE) arising in adolescence and is often associated with executive function (EF) deficits. Some EF components like response inhibition have been extensively evaluated in JME, but few studies have focused upon trait impulsivity or compared between GGE subtypes. The aim of the present study was to compare the association of trait impulsivity in JME with other GGE subtypes. METHODS: Patients with GGE aged between 14 and 40 years (n = 137) were divided into those with JME (n = 92) and those with other GGEs (n = 45: 8 childhood absence epilepsy (CAE), 22 juvenile absence epilepsy (JAE), and 15 epilepsy with generalized tonic-clonic seizures only (EGTCS)). The study participants were recruited through medical records of the general population of Buskerud County and the neighboring municipalities, covering 477,000 people or 9.1% of Norway's total population. All participants underwent a clinical interview including the Barratt Impulsiveness Scale (BIS), an established measure of trait impulsivity. We controlled for other potential predictors of BIS score using analysis of covariance (ANCOVA). RESULTS: There were no differences between JME and other types of GGE for BIS scores, but the presence of myoclonic seizures within the last year, irrespective of GGE subtype, was independently associated with significantly increased behavioral impulsivity. CONCLUSIONS: This study demonstrates that trait impulsivity in GGE is most strongly related to the recent occurrence of myoclonic seizures rather than GGE subtype.

The association between temperament features and childhood traumas in patients with juvenile myoclonic epilepsy

Background/aim: Epilepsy is a common chronic neurological problem that impairs daily activities, functionality, and quality of life. Childhood traumas (CTs) are known to be critical factors in the onset or development of many psychiatric and medical disorders. They also play a critical role in the development of temperament and personality. This study aimed to investigate the association between CTs and common temperament patterns and features seen in epilepsy patients. Materials and methods: The study included 38 patients who were diagnosed with juvenile myoclonic epilepsy (JME) and volunteered to participate in the study. In addition to the sociodemographic form and questions on disease features, Structured Clinical Interview for DSM-IV Axis I Disorders, Temperament Evaluation of Memphis, Pisa, Paris and San Diego Questionnaire (TEMPS-A), Childhood Trauma Questionnaire (CTQ), Beck Depression Inventory (BDI), and Beck Anxiety Inventory (BAI) were administered to all participants. In the present study, a cut-off value of 35 was used for the CTQ scale. The patients with CTQ scores lower than 35 (50%, n = 19, Group 1) and the patients with CTQ scores above 35 (50%, n = 19, Group 2) were compared. Results: The comparison of TEMPS-A and its subscale scores in the JME patients in the groups with CTQ scores above or below a cut-off value detected significant differences between the groups in depressive and irritable temperament scores. The mean BDI scores were also different between the two groups. Furthermore, a significant positive correlation was detected between the disease duration, anxiety, and depression scores in the JME patients. A significant relationship was detected between the emotional neglect subscale score of the JME patients and the BDI scores. A significant positive correlation was found between the total disease duration, BDI, and BAI. Significant moderate-level relationships were found between the BDI score and irritable, depressive, cyclothymic, and anxious temperaments and between the BAI score and irritable, depressive, cyclothymic, and anxious temperaments. Conclusion: Several temperamental features of JME patients are related to CTs. More depressive symptoms are seen in JME patients with higher disease durations.

Psychosocial complications in juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy (JME) constitutes about 10% of all epilepsies. Because of executive dysfunction, people with JME may be prone to impulsivity and risk-taking behavior. Our aim was to investigate whether psychosocial issues associated with impulsivity are more prominent in people with JME than in those with other types of genetic generalized epilepsy (GGE). Patients with GGE were recruited retrospectively through the Drammen Hospital records in Buskerud County, Norway, 1999-2013. They were invited to a semi-structured interview, either at the hospital or at home. Ninety-two patients with JME and 45 with other types of GGE were interviewed. Variables were evaluated in terms of their association with JME versus other GGE diagnosis using a logistic regression model. Juvenile myoclonic epilepsy was associated with use of illicit recreational drugs and police charges, although with borderline significance (odds ratio [OR] 3.4, p = 0.087 and OR 4.2, p = 0.095); JME was also associated with being examined for attention-deficit hyperactivity disorder (ADHD) in females (OR 15.5, p = 0.015), a biological parent with challenges like addiction or violent behavior (OR 3.5, p = 0.032), and use of levetiracetam (OR 5.1, p = 0.014). After controlling for group differences, we found psychosocial complications to be associated with JME, potentially influencing the lives of the individuals and their families to a greater extent than the seizures per se. Thus, JME should be considered a disorder of the brain in a broader sense than a condition with seizures only.

Praxis-induced myoclonia: From the neurophysiologist to the patient perspective.

PURPOSE: To characterize semiology and EEG features of praxis-induced (PI) myoclonia and to describe the subjective perception of juvenile myoclonic epilepsy (JME) patients with this reflex trait. METHODS: Patients with JME who presented myoclonia during a Video-EEG Neuropsychological Protocol were selected. We analyzed the semiology of upper limbs myoclonia and the ictal EEG patterns on Video-EEG. We explored the subjective aspects of PI by performing a semi-structured interview to each patient. RESULTS: 15 patients experienced 59 upper limbs myoclonia. Jerks were more frequently asymmetric or unilateral (32/59); a bilaterally symmetric pattern of all myoclonia was observed in only five patients. Ictal pattern was polyspike-wave (PSW) in 31/59 myoclonic seizures and spike-and-wave (SW) in 28/59. Six patients started perceiving myoclonia while learning a new skill or practicing a previously learned skill in a more stressful context. For most patients, PI-myoclonia were a source of anxiety. PI persisted despite antiepileptic medications in 10 patients. CONCLUSIONS: Electroclinical features of PI-myoclonia were more heterogeneous than traditionally described. Ictal pattern of SW was almost as frequent as classical PSW. Patients described the influence of learning new skills and anxiety on PI. Their subjective perception let us understand the impact of this reflex trait.

Ictal Cardiorespiratory Arrest Associated with Status Epilepticus in Panayiotopoulos Syndrome.

A healthy 10-year-old boy vomited during sleep and later complained of abdominal pain; he became drowsy and uncommunicative. At the nearby hospital E.R., he deteriorated rapidly, and his respiratory movements were absent with cardiac arrest. He was immediately resuscitated. Brain MRI showed no abnormalities. EEG revealed an abnormal pattern with recurrent multifocal epileptiform activity over the bilateral occipital and frontal regions during sleep. Based on the clinical/radiological findings we diagnosed Panayiotopoulos syndrome (PS), a benign form of early-onset pediatric epilepsy characterized by autonomic symptoms. Lifethreating cardiopulmonary arrest is rare in PS, but long seizure duration of PS may associate with apnea and bradycardia.

Impact of sleep disorders on the risk of seizure recurrence in juvenile myoclonic epilepsy.

OBJECTIVE: The aim of this study was to investigate the presence of sleep disturbances in patients with juvenile myoclonic epilepsy (JME) using sleep questionnaires. Further, we tried to evaluate whether alterations in sleep quality may influence the clinical expression of JME. METHODS: Sixty-two patients with JME treated with levetiracetam were included. Demographic and clinical variables were collected. Moreover, all patients submitted the Pittsburgh Sleep Quality index (PSQI) and the Epworth Sleepiness Scale (ESS) in order to respectively assess sleep quality during the last month and daytime sleepiness. All patients were followed up for a 6-month period and divided in two groups: seizure-free (Group 1) and seizure recurrence (Group 2). The PSQI and ESS scores were synthesized as binary variables <5/≥5 and <10/≥10, respectively. A comprehensive analysis was performed to evaluate the independent effect of the sleep quality and daytime sleepiness on the risk of having seizures during the follow-up. RESULTS: Both reduced sleep quality during the last month and daytime sleepiness were associated with an increased risk of suffering from seizures during the follow-up period. In fact, a PSQI score<5 or an ESS score<10 resulted significantly associated with the absence of seizure recurrence (p<0.004 and p<0.001, respectively). Increasing age had a significantly protective effect in the risk of seizure relapse. CONCLUSIONS: Our findings show that reduced sleep quality and daytime sleepiness in patients with JME increase the risk of seizure occurrence in spite of an appropriate pharmacological treatment. This negative effect seems to be more relevant in younger patients. Sleep disorders and their specific correction should be taken into consideration for the management of patients with JME.

Prevalence and clinical characteristics of headache in juvenile myoclonic epilepsy: experience from a tertiary epilepsy center.

The comorbidity of headache and epilepsy is often seen in neurological practice. The objective of this study was to assess the prevalence, types of, and risk factors for headache in juvenile myoclonic epilepsy (JME). We assessed a total of 200 patients and 100 healthy controls in our study. Headache was classified in participants using a self-administered questionnaire. Demographical, clinical features and headache characteristics were recorded. Seizure and headache temporal profiles were noted. Headache was present in 111 (56%) patients and 50 (50%) healthy participants. From these patients, 47 (42.3%) JME patients had migraine [30 (27%) migraine without aura (MO), 17 (15.3%) migraine with aura (MA)], 52 (46.8%) had tension type headache (TTH), 4 (3.6%) had both migraine and TTH, and 8 (7.2%) had other non-primary headaches. In the healthy control group, migraine was detected in 16 (32%) subjects, TTH in 33 (66%), both migraine and TTH in 1 (2%) subject. A positive migraine family history and symptom relief with sleep were more frequent in JME patients (p = 0.01). Headache was classified as inter-ictal in 82 (79.6%) patients and peri-ictal in 21 (20.4%) patients. In conclusion, the present study revealed that headache frequency was not significantly different between JME patients and healthy controls (p > 0.05). However, migraine frequency was higher in JME patients than healthy controls. Some migraine and TTH characteristics were different in between groups. We suggest that our results support both genetic relationship and shared underlying hypothetical pathopysiological mechanisms between JME and headache, especially migraine.