Cross-sectional, hospital-based analysis of headache types using ICHD-3 criteria in the Middle East, Asia, and Africa: the Head-MENAA study.

BACKGROUND: Headaches are frequent neurological disorders that are yet to be unveiled and treated comprehensively worldwide. Bearing in mind that the distribution of headache subtypes in neurology clinics (NC) is essential for planning appropriate diagnostic and therapeutic approaches, the primary goals of this multi-centric study are to carry out inter-regional comparisons by using current diagnostic criteria with evaluations of neurologists to delineate headache burden. METHODS: A cross-sectional study between April 1 and May 16, 2022 was conducted with the participation of 13 countries from the Middle East, Asia, and Africa. Patients were included in the study on a specific day each week during five consecutive weeks. All volunteers over the age of 18 and whose primary cause for admission was headache were examined. The patients admitted to NC or referred from emergency services/other services were evaluated by neurologists by means of the International Classification of Headache Disorders (ICHD-3) criteria. RESULTS: Among the 13,794 patients encountered in NC, headache was the primary complaint in 30.04%. The headache patients' mean age was 42.85 ± 14.89 (18-95 years), and 74.3% were female. According to the ICHD-3 criteria, 86.7% of the main group had primary headache disorders, 33.5% had secondary headaches, 4% had painful cranial neuropathies along with other facial and headaches, and 5.2% had headaches included in the appendix part showing some overlapping conditions. While the most common primary headache was migraine without aura (36.8%), the most common secondary headache was medication-overuse headache (MOH) (9.8%). Headaches attributed to COVID-19, its secondary complications, or vaccines continue to occur at rates of 1.2%-3.5% in current neurology practice. Pain severity was significantly lower in Ivory Coast and Sudan than in Türkiye, Turkish Republic of Northern Cyprus, Iran, Egypt, Senegal, Tatarstan, and Azerbaijan (p < 0.001). CONCLUSIONS: The study showed that migraine is still the most common motive for admissions to NC in different regions. Furthermore, MOH, an avoidable disorder, is the most common secondary headache type and appears to be a significant problem in all regions. Remarkably, pain perception differs between regions, and pain intensity is lower in Africa than in other regions.

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.

Revolutionizing migraine management: advances and challenges in CGRP-targeted therapies and their clinical implications.

Migraine, a prevalent neurological disorder, affects approximately 14.1% of the global population and disproportionately impacts females. This debilitating condition significantly compromises quality of life, productivity, and incurs high healthcare costs, presenting a challenge not only to individuals but to societal structures as a whole. Despite advances in our understanding of migraine pathophysiology, treatment options remain limited, necessitating ongoing research into effective therapies. This review delves into the complexity of migraine management, examining the roles of genetic predisposition, environmental influences, personalized treatment approaches, comorbidities, efficacy and safety of existing acute and preventive treatments. It further explores the continuum between migraine and tension-type headaches and discusses the intricacies of treating various migraine subtypes, including those with and without aura. We emphasize the recent paradigm shift toward trigeminovascular activation and the release of vasoactive substances, such as calcitonin gene-related peptide (CGRP), which offer novel therapeutic targets. We assess groundbreaking clinical trials, pharmacokinetic and pharmacodynamic perspectives, safety, tolerability, and the real-world application of CGRP monoclonal antibodies and gepants. In the face of persisting treatment barriers such as misdiagnosis, medication overuse headaches, and limited access to specialist care, we discuss innovative CGRP-targeted strategies, the high cost and scarcity of long-term efficacy data, and suggest comprehensive solutions tailored to Turkiye and developing countries. The review offers strategic recommendations including the formulation of primary care guidelines, establishment of specialized outpatient clinics, updating physicians on novel treatments, enhancing global accessibility to advanced therapies, and fostering patient education. Emphasizing the importance of lifestyle modifications and holistic approaches, the review underscores the potential of mass media and patient groups in disseminating critical health information and shaping the future of migraine management.

Other primary headache disorders: Data from the HEAD-MENA-A study in Africa, Asia, and the Middle East.

OBJECTIVE: Other primary headache disorders (OPHD) are under-investigated compared to frequent primary headache types like migraine, tension-type headache, and trigeminal autonomic cephalalgias. Knowledge of the distribution and characteristics of OPHD subtypes is crucial for their recognition. We aimed to determine the prevalence at the hospital and headache clinics and clinical characteristics of OPHDs in patients from 13 countries. METHODS: We analyzed a large dataset from the cross-sectional study Head-MENA-A (Middle East, North Africa, Asia). Consecutive patients over 10 years of age presenting with headaches were included from outpatient, inpatient, and emergency settings. A structured questionnaire addressing demographics, headache characteristics, accompanying symptoms, and triggers was administered. Headache subtypes were diagnosed according to the ICHD-3 criteria. RESULTS: Among patients complaining of headaches (n = 3722), 106 (2.9%) were diagnosed with OPHD. Fifty-two patients (1.4% of all headache patients) had only OPHD, while 54 (1.5%) had both OPHD and a co-existing primary headache (mostly migraine). All OPHDs were more common in females. The most frequent subtypes were new daily persistent headache and primary stabbing headache (0.2% each among all admitted patients). Photophobia and phonophobia were the most frequent accompanying symptoms, while physical activity (28.8%), stress (15.4%), and the Valsalva maneuver (15.4%) were the most common triggering factors. The majority of triggering factors were more pronounced in patients with both migraine and OPHD. CONCLUSIONS: Other primary headaches are rare and heterogeneous. Their high co-existence with migraine suggests shared predisposing factors, hinting at a "headache continuum" concept for primary headaches.

Global uncertainty in the diagnosis of neurological complications of SARS-CoV-2 infection by both neurologists and non-neurologists: An international inter-observer variability study.

INTRODUCTION: Uniform case definitions are required to ensure harmonised reporting of neurological syndromes associated with SARS-CoV-2. Moreover, it is unclear how clinicians perceive the relative importance of SARS-CoV-2 in neurological syndromes, which risks under- or over-reporting. METHODS: We invited clinicians through global networks, including the World Federation of Neurology, to assess ten anonymised vignettes of SARS-CoV-2 neurological syndromes. Using standardised case definitions, clinicians assigned a diagnosis and ranked association with SARS-CoV-2. We compared diagnostic accuracy and assigned association ranks between different settings and specialties and calculated inter-rater agreement for case definitions as "poor" (κ ≤ 0.4), "moderate" or "good" (κ > 0.6). RESULTS: 1265 diagnoses were assigned by 146 participants from 45 countries on six continents. The highest correct proportion were cerebral venous sinus thrombosis (CVST, 95.8%), Guillain-Barré syndrome (GBS, 92.4%) and headache (91.6%) and the lowest encephalitis (72.8%), psychosis (53.8%) and encephalopathy (43.2%). Diagnostic accuracy was similar between neurologists and non-neurologists (median score 8 vs. 7/10, p = 0.1). Good inter-rater agreement was observed for five diagnoses: cranial neuropathy, headache, myelitis, CVST, and GBS and poor agreement for encephalopathy. In 13% of vignettes, clinicians incorrectly assigned lowest association ranks, regardless of setting and specialty. CONCLUSION: The case definitions can help with reporting of neurological complications of SARS-CoV-2, also in settings with few neurologists. However, encephalopathy, encephalitis, and psychosis were often misdiagnosed, and clinicians underestimated the association with SARS-CoV-2. Future work should refine the case definitions and provide training if global reporting of neurological syndromes associated with SARS-CoV-2 is to be robust.

Sleep characteristics of patients with epilepsy with pure sleep-related seizures.

OBJECTIVE: Our aim was to investigate the clinical features and sleep characteristics of patients with pure sleep-related seizures. METHODS: Patients with pure sleep epilepsy were prospectively enrolled and their clinical, EEG, and MRI findings investigated. The Medical Outcomes Study Sleep Scale (MOS-SS) was administered after receiving consent. RESULTS: Thirty-nine of 1401 consecutive patients (2.7%) with pure sleep-related seizures were included. Of these, 30 (76.9%) had epilepsy of unknown cause and 7 had epilepsy with known structural etiologies. Twenty-seven patients reported less than one seizure per month and 19 had been seizure free for at least 1 year. Thirty-four patients participated in our MOS-SS study. Comparison of sleep problems between those with epilepsy and healthy controls and between the subgroups with frequent and rare seizures did not reveal significant differences. CONCLUSION: Patients with pure sleep seizures had mostly undetermined etiology usually with a good prognosis, and this rare condition did not seem to affect their sleep quality.

A comparative ID migraine screener study in ophthalmology, ENT and neurology out-patient clinics.

Migraine is more likely to be misdiagnosed in patients with comorbid diseases. Not only primary care physicians, but also specialists might misdiagnose it due to the lack of diagnostic criteria awareness. The ID migraine test is a reliable screening instrument that may facilitate and accelerate migraine recognition. This study aimed to compare the prevalence and characteristics of migraine in a large sample of patients admitted to clinics of ophthalmology (OC), ear, nose and throat diseases (ENTC) and neurology (NC), as well as to validate the use of the ID migraine test in OC and ENTC settings. This was a multicentre (11 cites) study of out-patients admitting either to NC, ENTC or OC of the study sites during five consecutive working days within 1 week. From each of the clinics, 100 patients were planned to be recruited. All recruited patients were interviewed and those having a headache complaint received an ID migraine test and were examined for headache diagnosis by a neurologist, blinded to the ID migraine test result. A total of 2625 subjects were recruited. Only 1.3% of OC patients and 5.4% of ENTC patients have been admitted with a primary complaint of headache, whereas the percentage of NC patients suffering from headache was 37.6%. Whereas 138 patients (19.3%) in OC, 154 (17.3%) in ENTC and 347 (34%) in NC were found to be ID migraine test positive, 149 patients (20.8%) in OC, 142 (16%) in ENTC and 338 (33.1%) in NC were diagnosed with migraine. The sensitivity, specificity, and positive and negative predictive ratios of the ID migraine test were found to be similar in all clinics. An important fraction of the patients admitted to NC, as well as to OC and ENTC, for headache and/or other complaints were found out to have migraine by means of a simple screening test. This study validated the ID migraine test as a sensitive and specific tool in OC and ENTC, encouraging its use as a screening instrument.

Nose wiping: an unrecognized automatism in absence seizures.

Nose wiping is a well-known postictal automatism in complex partial seizures, considered very important for lateralization. We report a 24-year-old woman selected from 32 patients with video-EEG investigations of absence seizures. The patient, with normal examination and imaging findings, had typical absence seizures from the age of 5 years, which were controlled with either ethosuximide or valproate and worsened by carbamazepine. In two of her recorded typical absence seizures associated with generalized 3 Hz symmetric spike-wave discharges, she wiped her nose as an automatism in the ictal and post-ictal period. Our case showed that nose wiping could be associated with generalized seizures, in contrast to other recent reports.

Correlation between clinical stages and EEG findings of subacute sclerosing panencephalitis.

In this retrospective study 67 patients with SSPE seen between the years 1980 and 1998 were reviewed. Using the criteria of SSPE diagnosis (clinical signs, characteristic EEG patterns, high titres of measles antibodies in the serum and CSF), the patients were divided into two groups. Group A fulfilled all criteria, however, due to the inability of measuring measles antibody before 1987, it was not possible to observe the third criterion in Group B. Among 67 patients, groups A and B consisted of 51 boys and 16 girls ranging in age between 1 to 23 years, mean age 13.1. The male/female ratio was 3.1. The periodic EEG complexes (PCs) were usually bilateral, synchronous and symmetrical. PC amplitude asymmetry was seen in 12 patients and 2 patients had no PC synchronization between the hemispheres. Six patients had more than one form of PC. Delta activity in anterior hemispheres after PC was seen in 40 patients, mostly in stage 2A. Thirty-two patients had focal epileptiform abnormalities in multiple locations at every stage but most frequently in frontal, central and temporal regions. One patient had PC over both hemispheres and periodic lateralized epileptiform discharges (PLEDs) over the right hemisphere. The EEG findings described and observed in our study do not seem to be specific to SSPE but these findings were not atypical or unusual.

Periodic lateralized epileptiform discharges: association with seizures.

The clinical features and EEGs of 45 consecutive patients (40 adults and 5 children) who had periodic lateralized epileptiform discharges (PLEDs) were reviewed to determine the relationship between seizures and PLEDs. Focal encephalitis and ischemic stroke were the most frequent underlying processes for adult patients. All of the children, but only six of the adults, had long-lasting cerebral disorders whereas the remaining adults had acute or subacute illness. There were 38 patients (84.4%) experiencing a seizure disorder. Twenty-six of them had their first seizure during their acute illness, as the pattern of PLED was encountered. Eight cases had status epilepticus, and seven of them had epilepsia partialis continua. Nineteen patients had a recent seizure in the day when PLEDs were observed but not during EEG recording; 12 patients had their seizures within 10 days before the observation of PLED. PLEDs were grouped into three categories with respect to their extensions: lateralized to one hemisphere (n = 22), localized in one region (n = 17) and being prominent over one side with contralateral spread (n = 6). The last group was found to be more closely associated with frequent seizures or status epilepticus than the other two groups. Our results showed that PLEDs were highly correlated with recent seizures in the majority of the patients. These EEG findings may be considered as a manifestation of an increased neuronal excitability caused by different etiologies; but not an ictal pattern.

Longterm follow-up of electroencephalographic and clinical findings of a case with Gaucher's disease type 3a.

Among three recognised clinical phenotypes, type 3a Gaucher's disease is characterised by mild to severe systemic disease, neurological manifestations and myoclonic seizures. We report the long term clinical and electrophysiological follow-up of a 27-year old man with a diagnosis of type 3a Gaucher's disease, which was confirmed by bone marrow biopsy examination and leukocyte glucocerebrosidase level measurement. His neurological examination was normal throughout the follow-up period. EEG examination, recorded five days after the first seizure, revealed generalised nonrhythmic paroxysmal rapid spikes with occipital predominance increased by photic stimulation and normal background activity. The frequency of seizures increased from 3-4/year to 1-2/month within a follow-up period of 12 years and a repeat EEG examination on the eight year of diagnosis revealed additional background slowing. A giant potential was obtained in somatosensory evoked potential (SEP) examination. EEG findings of this case demonstrate a specific pattern with rapid spike activity, photosensitivity, eye closure sensitivity and gradual background slowing.

Perioral myoclonia with absence seizures: a rare epileptic syndrome.

We present the clinical and video-EEG data on an epileptic boy whose absence seizures with marked perioral movements had started at the age of 1.5 years. From age 12 years, he experienced frequent episodes of typical absence status epilepticus (ASE) lasting 1-2 hours with marked perioral myoclonia and moderate confusion. Initial therapy with carbamazepine was substituted by valproate because of worsening of the absence seizures. At the age of 17, the patient was admitted to our clinic with his usual, but long lasting ASE attack, accompanied by 2 generalized tonic-clonic convulsions. ASE was confirmed with the EEG which showed continuous 3 Hz spike and wave paroxysms with occasional normal intervals of 1-5 seconds. IV injection of clonazepam improved the clinical and EEG findings immediately. Video- EEG examination performed after a few weeks demonstrated typical absence seizures with perioral myoclonia. Based on the characteristics of seizure semiology, other clinical data and EEG findings, the patient was diagnosed as having the syndrome of "perioral myoclonia with absence seizures" described by Panayiotopoulos.

Identifying SNP targeted pathways in partial epilepsies with genome-wide association study data.

PURPOSE: In a recent genome-wide association study for partial epilepsies in the European population, a common genetic variation has been reported to affect partial epilepsy only modestly. However, in complex diseases such as partial epilepsy, multiple factors (e.g. single nucleotide polymorphisms, microRNAs, metabolic and epigenetic factors) may target different sets of genes in the same pathway, affecting its function and thus causing the disease development. In this regard, we hypothesize that the pathways are critical for elucidating the mechanisms underlying partial epilepsy. METHODS: Previously we had developed a novel methodology with the aim of identifying the disease-related pathways. We had combined evidence of genetic association with current knowledge of (i) biochemical pathways, (ii) protein-protein interaction networks, and (iii) the functional information of selected single nucleotide polymorphisms. In our present study, we apply this methodology to a data set on partial epilepsy, including 3445 cases and 6935 controls of European ancestry. RESULTS: We have identified 30 overrepresented pathways with corrected p-values smaller than 10(-12). These pathways include complement and coagulation cascades, cell cycle, focal adhesion, extra cellular matrix-receptor interaction, JAK-STAT signaling pathway, MAPK signaling pathway, proteasome, ribosome, calcium signaling and regulation of actin cytoskeleton pathways. Most of these pathways have growing scientific support in the literature as being associated with partial epilepsy. We also demonstrate that different factors affect distinct parts of the pathways, as shown here on complement and coagulation cascades pathway with a comparison of gene expression vs. genome-wide association study. CONCLUSIONS: Traditional studies on genome-wide association have not revealed strong associations in epilepsies, since these single nucleotide polymorphisms are not shared by most of the patients. Our results suggest that it is more effective to incorporate the functional effect of a single nucleotide polymorphism on the gene product, protein-protein interaction networks and functional enrichment tools into genome-wide association studies. These can then be used to determine leading molecular pathways, which cannot be detected through traditional analyses. We hope that this type of analysis brings the research community one step closer to unraveling the complex genetic structure of epilepsies.

Expression analysis and clinical correlation of aquaporin 1 and 4 genes in human hippocampal sclerosis.

Mesial temporal sclerosis (MTS) is the most frequent cause of drug resistant symptomatic partial epilepsy. The mechanism and genetic background of this unique pathology are not well understood. Aquaporins (AQP) are regulators of water homeostasis in the brain and are expressed in the human hippocampus. We explored the role of AQP genes in the pathogenetic mechanisms of MTS through an evaluation of gene expression in surgically removed human brain tissue. We analyzed AQP1 and 4 mRNA levels by quantitative real-time polymerase chain reaction and normalized to ABL and cyclophilin genes, followed by immunohistochemistry for AQP4. Relative expressions were calculated according to the delta Ct method and the results were compared using the Mann-Whitney U test. Brain specimens of 23 patients with epilepsy who had undergone surgery for MTS and seven control autopsy specimens were investigated. Clinical findings were concordant with previous studies and 61% of the patients were seizure-free in the postoperative period. AQP1 and 4 gene expression levels did not differ between MTS patients and control groups. Immunofluorescence analysis of AQP4 supported the expression results, showing no difference. Previous studies have reported contradictory results about the expression levels of AQP in MTS. To our knowledge, only one study has suggested upregulation whereas the other indicated downregulation of perivascular AQP4. Our study did not support these findings and may rule out the involvement of AQP in human MTS.

Whole-genome linkage scan for epilepsy-related photosensitivity: a mega-analysis.

Photoparoxysmal response (PPR) is considered to be a risk factor for idiopathic generalised epilepsy (IGE) and it has a strong genetic basis. Two genome-wide linkage studies have been published before and they identified loci for PPR at 6p21, 7q32, 13q13, 13q31 and 16p13. Here we combine these studies, augmented with additional families, in a mega-analysis of 100 families. Non-parametric linkage analysis identified three suggestive peaks for photosensitivity, two of which are novel (5q35.3 and 8q21.13) and one has been found before (16p13.3). We found no evidence for linkage at four previously detected loci (6p21, 7q32, 13q13 and 13q31). Our results suggest that the different family data sets are not linked to a shared locus. Detailed analysis showed that the peak at 16p13 was mainly supported by a single subset of families, while the peaks at 5q35 and 8q21 had weak support from multiple subsets. Family studies clearly support the role of PPR as a risk factor for IGE. This mega-analysis shows that distinct loci seem to be linked to subsets of PPR-positive families that may differ in subtle clinical phenotypes or geographic origin. Further linkage studies of PPR should therefore include in-depth phenotyping to make appropriate subsets and increase genetic homogeneity.

Validity of the ID-Migraine screener in the workplace.

OBJECTIVE: The impact of migraine on physical, social, and emotional performance is considerable, yet it remains an underdiagnosed disorder. ID-Migraine is a validated migraine-screening tool developed to facilitate diagnosis. This study evaluated the validity and use of the Turkish version of the ID-Migraine screener (ID-Ms) in the workplace, and measured the impact of headache on disability, productivity, and quality of life among the workforce. METHODS: A total of 465 employees from four companies were interviewed for screening with the ID-Ms. Subjects were included in the study if they reported two or more headaches in the past 3 months and gave a positive answer to one of the two ID-Ms prescreening questions. Eligible subjects completed the ID-Ms, the Migraine Disability Assessment Questionnaire, and the Medical Outcomes Study 36-Item Short Form Health Survey. Subjects were then evaluated for confirmation of their diagnosis according to the International Classification of Headache Disorders, 2nd edition (ICHD-2) criteria. RESULTS: A total of 227 subjects (mean age 31.9 +/- 5.9 years; 65.6% women) completed the study. Migraine was diagnosed in 106 of the 227 subjects (46.7%) according to the ID-Ms and in 117 of the 227 subjects (51.5%) according to ICHD-2 criteria. The sensitivity of the ID-Ms was 70.9%, specificity was 79.1% and Cohen kappa value was 0.50. Workdays lost over the previous 3 months due to headache amounted to 8.7 +/- 9.5 days for migraine-positive and 4.9 +/- 6.6 days for migraine-negative subjects. CONCLUSION: The Turkish version of the ID-Migraine screener is a valid tool for identifying subjects with migraine in the workplace.

A severe neurological sequela in acute intermittent porphyria: presentation of a case from encephalopathy to quadriparesis.

Porphyrias present themselves with autonomic or peripheral neuropathy or central nervous system dysfunction. They are a varied group of inborn errors of metabolism that are characterized by specific inherited enzyme defects in haem biosynthesis. A patient whose mother was diagnosed as having porphyria was admitted to hospital because of her abdominal pains and dysuria. She had three generalized convulsions and, in a few hours, she lost the vision in both of her eyes. As the seizures continued, she became quadriparetic and fell into a coma after gradually losing consciousness. She improved but with sequelae; her serial MRIs, including apparent diffusion coefficient map imaging, diffusion-weighted imaging and angiography, showed ischaemic lesions that were both unlike and more severe than the ones reported in the literature.

Myoclonic seizures subside in the fourth decade in juvenile myoclonic epilepsy.

OBJECTIVE: Our aim was to assess the long-term follow-up of juvenile myoclonic epilepsy (JME), with an emphasis on the course of the myoclonic seizures. METHODS: We enrolled 48 patients with JME (29 F, 19 M; aged 39.9 +/- 9.5 years) followed up for a mean of 19.6 +/- 5.7 years. The remission for 5 years and relapses were evaluated for all seizure types and the changes in severity/frequency of myoclonia were systematically questioned. The clinical and EEG features, antiepileptic drug (AED) treatment regimen, and systemic and psychiatric comorbid diseases were evaluated. RESULTS: We found a benign course in 66.6% whereas 16.7% had pseudo-resistance due to problems in treatment or lifestyle. The true-resistant course observed in the remaining 16.7% was significantly associated with psychiatric disorders and the presence of thyroid diseases. In 54.2% of the patients, myoclonia were in remission for a mean duration of 8.4 +/- 7.7 years, after an average age of 32.9 +/- 9.6. Of these patients, 6 were on a lower dose of AED in comparison to the dosage needed to control the seizures in the beginning, and 5 patients had stopped AED treatment. None of the latter 11 patients except one relapsed during the follow-up. Furthermore, 21 other patients (43.8%) described substantial alleviation after age 31.3 +/- 8.4 in the severity of myoclonia. CONCLUSION: Although a great majority of the patients with juvenile myoclonic epilepsy had continuing seizures after a follow-up of 20 years, almost all had either 5-year remission or a substantial alleviation of the myoclonic seizures.