Headache in idiopathic/genetic epilepsy: Cluster analysis in a large cohort.

OBJECTIVE: The link between headache and epilepsy is more prominent in patients with idiopathic/genetic epilepsy (I/GE). We aimed to investigate the prevalence of headache and to cluster patients with regard to their headache and epilepsy features. METHODS: Patients aged 6-40 years, with a definite diagnosis of I/GE, were consecutively enrolled. The patients were interviewed using standardized epilepsy and headache questionnaires, and their headache characteristics were investigated by experts in headache. Demographic and clinical variables were analyzed, and patients were clustered according to their epilepsy and headache characteristics using an unsupervised K-means algorithm. RESULTS: Among 809 patients, 508 (62.8%) reported having any type of headache; 87.4% had interictal headache, and 41.2% had migraine. Cluster analysis revealed two distinct groups for both adults and children/adolescents. In adults, subjects having a family history of headache, ≥5 headache attacks, duration of headache ≥ 24 months, headaches lasting ≥1 h, and visual analog scale scores > 5 were grouped in one cluster, and subjects with juvenile myoclonic epilepsy (JME), myoclonic seizures, and generalized tonic-clonic seizures (GTCS) were clustered in this group (Cluster 1). Self-limited epilepsy with centrotemporal spikes and epilepsy with GTCS alone were clustered in Cluster 2 with the opposite characteristics. For children/adolescents, the same features as in adult Cluster 1 were clustered in a separate group, except for the presence of JME syndrome and GTCS alone as a seizure type. Focal seizures were clustered in another group with the opposite characteristics. In the entire group, the model revealed an additional cluster, including patients with the syndrome of GTCS alone (50.51%), with ≥5 attacks, headache lasting >4 h, and throbbing headache; 65.66% of patients had a family history of headache in this third cluster (n = 99). SIGNIFICANCE: Patients with I/GE can be clustered into distinct groups according to headache features along with seizures. Our findings may help in management and planning for future studies.

Relationship between Status Epilepticus Severity Score and etiology in adult NCSE patients.

BACKGROUND AND PURPOSE: Nonconvulsive status epilepticus (NCSE) is a heterogeneous, severe neurological disorder of different etiologies. In this study, the outcomes of NCSE episodes was assessed in a large series of adult patients. Our objective was to evaluate relationship between Status Epilepticus Severity Score (STESS) and etiology and the role of etiological factors on predicting the outcomes. METHODS: In this retrospective study, the medical records of 95 patients over 18 years of age who were diagnosed with NCSE between June 2011 and December 2015 were reviewed. Their treatment and follow-up for NCSE was performed at the Epilepsy Unit in Department of Neurology, Antalya Research and Training Hospital. Etiological factors thought to be responsible for NCSE episodes as well as the prognostic data were retrieved. The etiological factors were classified into three groups as those with a known history of epilepsy (Group 1), primary neurological disorder (Group 2), or systemic/unknown etiology (Group 3). STESS was retrospectively applied to patients. RESULTS: There were 95 participants, 59 of whom were female. Group 1, Group 2, and Group 3 consisted of 11 (7 female), 54 (33 female), and 30 (19 female) patients, respectively. Of the 18 total deaths, 12 occurred in Group 2, and 6 in Group 3. The negative predictive value for a STESS score of ≤ 2 was 93.88% (+LR 2.05 95% CI: 1.44-2.9 and -LR 0.3 95% CI 0.10-0.84 ) in the overall study group. While the corresponding values for Group 1 (patients with epilepsy), Group 2 (patients with primary neurological disorder), and group 3 (patients with systemic or unknown etiology) were 100%, 92.59% (+LR 2.06 95%CI: 1.32-3.21 and -LR 0.28 95% CI 0.08-1.02 ) 83.33% (+LR 1.14 95%CI: 0.59-2.9 and -LR 0.80 95% CI 0.23-2.73). CONCLUSION: This study included the one of the largest patients series ever reported in whom STESS, a clinical scoring system proposed for use in patients with status epilepticus, has been implemented. Although STESS appeared to be quite useful for predicting a favorable outcome in NCSE patients with epilepsy and primary neurological disorders, its predictive value in patients with systemic or unknown etiology was lower. Further prospective studies including larger NCSE samples are warranted.

Marriage and childbearing in patients with epilepsy in Turkey.

Introduction: For epilepsy, a common neurological disorder, brings psychosocial challenges like stigma, employment difficulties, and barriers to marriage and childbearing. Stigma often stems from misconceptions and societal beliefs, particularly in less developed regions like Turkey. However, research on the marital and childbearing experiences of epilepsy patients in such settings is limited. We aimed to research the marriage and childbearing behaviors of men and women with epilepsy. Methods: We conducted a cross-sectional study involving 215 adult epilepsy patients at Antalya Training and Research Hospital between 2019 and 2022. Patients were asked questions about marriage and having children on prepared questionnaires. Result: The gender distribution of the 215 patients included in the study was revealed to be 62.3% (134) females and 37.7% (81) males. 71.6% of patients were married, and 12.7% had no children. 33.3% of these patients stated that they did not desire children because of the disease. A statistically significant correlation was observed between the duration of the disease and being unmarried. A significant correlation was observed between age at disease onset and number of children. Conclusion: Our study revealed the effects of individuals with epilepsy on marriage and childbearing, and as we know, it is the first study conducted in Turkey on childbearing attitudes in individuals with epilepsy. Despite medical and social developments, epilepsy is still one of the most stigmatized diseases, and the disease has considerable negative effects on marriage and fertility. Our study supported the findings of a small number of previous similar studies on this subject and additionally showed that the likelihood of having children decreased in patients using multiple ASM, and on the other hand, it showed that marriage positively affected patients in terms of social support.

Buccopalpebral reflex in Parkinson disease and blink reflex study.

OBJECTIVE: To define a new primitive reflex named the buccopalpebral reflex (BPR), and to investigate this reflex clinically and neurophysiologically in patients with Parkinson disease. METHODS: This prospectively designed study included 17 patients, 9 BPR positive patients, and 8 BPR negative patients in Ankara Research and Training Hospital, Ankara, Turkey, and was carried out between January and December 2008. All patients had Parkinson disease without any medication. Using the blink reflex technique, 3 branches of the trigeminal nerve were stimulated. Additionally, the Mini Mental State Examination (MMSE), the Unified Parkinson`s Disease Rating Scale (UPDRS), the Hoehn and Yahr Score (HYS), the blink frequency, and the duration of Parkinson disease was also matched between the 2 groups. RESULTS: In patients with positive BPR, 5 had tremor and the remaining 4 had bradykinesia as a dominant symptom, while all other patients with negative BPR had only tremor. When blink reflex findings were compared between the 2 groups, R2 and contralateral R2 latencies that were taken by supraorbital stimulus were significantly shorter in the BPR positive patients. There were no statistically significant differences in terms of MMSE, UPDRS, HYS, and frequency of blinking, and duration of illness between the 2 groups. CONCLUSION: This reflex may be an indicator of sensitivity or decrease of threshold level such as Myerson`s sign, in which there is no inhibition in glabella reflex. The blink reflex findings support this hypothesis.

Colour vision in juvenile myoclonic epilepsy.

To determine the integrity of colour perception, related to photic sensitivity, in patients with juvenile myoclonic epilepsy. Twenty-four patients with photoparoxysmal response, 27 patients without photoparoxysmal response, and 32 healthy individuals were investigated using the Farnsworth Munsell-100 Hue test to calculate error scores for total colour, blue/yellow, and red/green. No significant differences were observed regarding blue/yellow, red/green or total error score between juvenile myoclonic epilepsy patients with or without photoparoxysmal response. However, the data for all three scores were significantly higher in both patient groups compared to the healthy control group. In both patient groups, the blue/yellow error score was significantly higher than the red/green error score. We were unable to identify a relationship between photoparoxysmal response and colour vision in patients with juvenile myoclonic epilepsy. We believe that the underlying reason why juvenile myoclonic epilepsy patients had significantly higher blue/yellow, red/green, and total error score compared to the healthy control group may be due to GABA dysfunction, which is considered to play a role in the pathophysiology of this disease as well as the physiology of colour vision.

Color vision testing versus pattern visual evoked potentials and optical coherence tomography parameters in subclinical optic nerve involvement in multiple sclerosis.

Acute idiopathic demyelinating optic neuritis is frequently the initial manifestation of multiple sclerosis (MS). We aimed to discuss the value of color vision testing to detect possible optic nerve involvement in patients with MS who had no history of optic neuritis. We evaluated color vision with Farnsworth-Munsell 100 (FM-100) hue test. Total error scores (TES), partial error scores for the red-green axis (RGS) and blue-yellow axis (BYS) were calculated. Topographic optic disc parameters (RNFL, RA, DA, CV, RV, and vertical C/D ratio), total macular volume (TMV), central macular thickness (CMT), and retinal ganglion cell layer (RGCL) were determined using spectral domain optical coherence tomography (SD-OCT). Choroidal thickness (CT) was measured using enhanced depth imaging optical coherence tomography (EDI-OCT). Pattern visual evoked potentials (PVEP) were also performed. Twenty-eight patients with RRMS (56 eyes) and 25 healthy controls (50 eyes) were included. P100 latencies were significantly delayed and P100 amplitudes were significantly reduced in the patient group compared with the controls (p ≤ 0.05). Statistically significant thinning was found in temporal quadrant in the patient group compared with the controls (p = 0.002). TES RGS, and BYS were all increased in the patient group but this was not statistically significant. We found no correlation between TES, RGS, BYS, and P100 latencies or OCT parameters. In our investigation as to whether color vision testing could be a simple biomarker for showing neurodegeneration of the anterior visual pathway regardless of optic neuritis, PVEP and OCT-assessed RNFL thickness seemed to be a more valuable biomarker than color vision testing.

Differences in diffusion tensor imaging changes between narcolepsy with and without cataplexy.

OBJECTIVE: The distinctive clinical finding of Type 1 narcolepsy compared to Type 2 is the presence of cataplexy. Several neuroimaging studies have also reported abnormalities in narcolepsy patients with or without cataplexy. However, there are conflicting results to differentiate them. In this study, we aimed to clarify the white matter changes in narcolepsy patients both with and without cataplexy and compared them with healthy adults to evaluate microstructural differences in the brain. METHODS: Eleven narcolepsy patients with cataplexy (NC), 12 narcolepsy patients without cataplexy (NOC) and healthy age- and gender-matched controls (N = 16) were studied. Whole-brain diffusion tensor imaging (DTI) was obtained and tract-based spatial statistics were used to localize white matter abnormalities. RESULTS: Compared with the healthy controls, both NC and NOC patients exhibited significant fractional anisotropy (FA) decreases in the bilateral cerebellar hemispheres, bilateral thalami, the corpus callosum and left anterior-medial temporal white matter. Compared with the controls, the NC patients' FA values were also decreased in the midbrain. No significant correlations were found between FA values and clinical-polysomnographic variables. CONCLUSION: This DTI study has demonstrated white matter abnormalities in the midbrain-brainstem regions as a distinctive finding of narcolepsy patients with cataplexy. Involvement of bilateral temporal lobes with greater changes on the left lobe is also a supporting finding of patients with cataplexy. DTI changes in the midbrain-brainstem and bilateral temporal lobes can be signs of different pathological mechanisms in these patients.

The temporal relation between seizure onset and arousal-awakening in temporal lobe seizures.

PURPOSE: Our main aim was to determine the time interval between the seizure onsets and arousal-awakening related to these seizures in patients with temporal lobe epilepsy (TLE) and to discuss the role of lateralization on arousal-awakening mechanisms. METHODS: Thirty-three TLE patients who underwent video-EEG monitoring with simultaneous polysomnography (PSG) and had recorded nocturnal seizures were retrospectively examined. These TLE patients had 64 seizures during sleep. The onsets of seizures and arousal-awakening related to these seizures were marked according to clinical and electrophysiological features. The time interval between the seizure onset and arousal-awakening related to the seizure was compared in patients with right- or left-sided temporal lobe seizures. RESULTS: In our TLE patients nocturnal seizures mostly followed arousal-awakening (64%). The time interval between the seizure onset and arousal-awakening related to the seizure was significantly shorter in patients with left-sided temporal lobe seizures (p=0.01). CONCLUSION: Video-EEG monitoring and PSG with scalp electrodes in our TLE patients showed that nocturnal seizures mostly followed arousal-awakening, and it was more pronounced in those with left-sided seizures. Arousal-awakening might be a signal for subsequent seizures in patients with TLE.

TAVI in a Patient with Single Coronary Artery: The Choice of Self-Expandable Valve may be Reasonable.

BACKGROUND: Single coronary artery is a rare congenital coronary artery anomaly with the incidence of approximately 0.03%-0.05%. Its coexistence with severe aortic stenosis (AS) is even rarer. Patients who undergo transcatheter aortic valve implantation (TAVI) have a risk of coronary obstruction (1%) which will be more lethal when the origin of the coronary artery is single. CASE REPORT: An 81 year-old female was referred to our institution for severe AS. Her coronary angiography revealed a single coronary artery originating from right sinus of valsalva. Since the patient had a high risk for aortic valve surgery, the cardiac team decided to perform transfemoral TAVI with a self-expandable valve. CONCLUSION: We presented a successful case of TAVI with Medtronic CoreValve (Medtronic; Minnesota, USA) in a patient with single coronary artery after discussing the base of our strategy for preferring self-expandable valve. To the best of our knowledge, this is the second case of CoreValve implantation in such a patient in the literature. We suggest that the self-expandable valve may be the first choice in these patients because of its recapturable design.

Optical coherence tomography parameters in patients with photosensitive juvenile myoclonic epilepsy.

PURPOSE: Juvenile myoclonic epilepsy (JME) is commonly associated with photoparoxysmal response (PPR) with a reported prevalence of 25-42%. In this study, we aim to explore the relationship between the PPR and Optical Coherence Tomography (OCT) parameters in order to determine whether optic nerve fiber layer or other structural differences have a pathophysiological role of photosensitivity in patients with JME. METHODS: We studied 53 consecutive patients with Juvenile myoclonic epilepsy (JME) at our outpatient department. The interictal electroencephalogram (EEG) findings for each patient were analyzed for the presence of photoparoxysmal features. The peripapillary Retina Nerve Fiber Layer (RNFL) thickness, ganglion cell thickness, macular thickness and choroid thickness levels were analyzed using OCT. RESULTS: We classified the patients into two groups as those with PPR (Group 1) and those without PPR (Group 2). There were statistically significant differences in the average RNFL thickness values of the left eye between the two groups (p<0.001). Although the RNFL thickness of the right eye was higher in Group 1, no statistically significant difference was observed between the two groups. The RFNL thickness of the superior quadrants both in the right and the left eyes was significantly higher in Group 1 patients (p<0.001). Macular thickness of the right and left eyes were significantly thinner in Group 1 patients (p<0.001). Choroid thickness of the left eye was significantly higher in Group 1 than in Group 2 patients (p<0.001). Although the choroid thickness of the right eye was higher in Group 1 patients, no statistically significant difference was observed between the two groups. CONCLUSION: This is the first study to our knowledge which has investigated the relation between the OCT parameters and photosensitivity in patients with JME. We concluded that these microstructural features may be related to photosensitivity in patients with JME.

Epileptic seizures in Neuro-Behcet disease: why some patients develop seizure and others not?

PURPOSE: Behcet disease (BD) is a chronic relapsing inflammatory disorder. Neuro BD (NBD) is seen in approximately 5% of all patients. The aim of this study is to investigate the frequency, type and prognosis of epileptic seizures in different forms of NBD. METHODS: All files of 42 patients with NBD were evaluated between 2006 and 2012, retrospectively. The demographic data, the presentation of NBD, clinical findings including seizures, EEG and neuroimaging findings were reviewed. RESULTS: The mean age of patients was 35.02±8.43 years. Thirty (71.4%) patients were male; the remaining 12 of them were female. Twenty-four patients had brainstem lesions; 16 patients had cerebral venous thrombosis. Spinal cord involvement was seen in two patients. Seven patients had epileptic seizures (six partial onset seizures with or without secondary generalization). Six of them had cerebral sinus thrombosis (CVT). Four patients had a seizure as the first symptom of the thrombosis. One patient had late onset seizure due to chronic venous infarct. The other patient with seizure had brainstem involvement. The remaining was diagnosed as epilepsy before the determination of NBD. CONCLUSION: CVT seen in BD seems to be the main risk factor for epileptic seizures in patients with NBD. The prognosis is usually good especially in patients with CVT. Epileptic seizures in patients with brainstem involvement may be an indicator for poor prognosis. Superior sagittal thrombosis or cortical infarct would be predictor of seizures occurrence because of the high ratio in patients with seizures.

Reversible encephalopathy due to sulfasalazine.

BACKGROUND: Sulfasalazine was devised by Swedish physician in the late 1930s in an attempt to treat "rheumatic polyarthritis." It is still a widely used anti-inflammatory agent especially in the treatment of rheumatologic disorders and inflammatory bowel diseases. Most of its side effects are benign, tolerable, and dose dependent, but less common severe systemic reactions have also been revealed. CASE PRESENTATION: A 42-year-old woman diagnosed with rheumatoid arthritis was admitted to emergency service because of status epilepticus. Hepatitis and myelotoxicity were also present after laboratory investigations. The patient was on sulfasalazine treatment for 3 weeks with a daily dose of 2g. Cranial magnetic resonance imaging (MRI) revealed bilateral periventricular and subcortical lesions in the white matter of especially temporal and occipital regions. Cerebrospinal fluid (CSF) examination showed very high protein level (564 mg/L). After discontinuation of treatment, the clinical, CSF, and MRI findings regressed rapidly. CONCLUSIONS: Side effects of sulfasalazine include neurotoxicity such as status epilepticus, cranial MRI lesions, and CSF abnormalities that were diagnosed in our patient after excluding other etiologic factors causing encephalitis.