Birth outcomes in pregnant women with epilepsy: A Nationwide multicenter study from Türkiye.

OBJECTIVE: The present study was aimed at investigating the effects of anti-seizure medications (ASMs), patient demographic characteristics, and the seizure type and frequency on the development of congenital malformations (CMs) in the infants of pregnant women with epilepsy (PWWE). METHODS: PWWE followed up at the neurology outpatient clinic of 21 centers between 2014 and 2019 were included in this prospective study. The follow-up of PWWE was conducted using structured, general pregnant follow-up forms prepared by the Pregnancy and Epilepsy Study Committee. The newborns were examined by a neonatologist after delivery and at 1 and 3 months postpartum. RESULTS: Of the infants of 759 PWWE, 7.2% had CMs, with 5.6% having major CMs. Polytherapy, monotherapy, and no medications were received by 168 (22.1%), 548 (72.2 %), and 43 (5.7 %) patients, respectively. CMs were detected at an incidence of 2.3% in infants of PWWE who did not receive medication, 5.7% in infants of PWWE who received monotherapy, and 13.7% in infants of PWWE who received polytherapy. The risk of malformation was 2.31-fold (95% confidence interval (CI): 1.48-4.61, p < .001) higher in infants of PWWE who received polytherapy. Levetiracetam was the most frequently used seizure medication as monotherapy, with the highest incidence of CMs occurring with valproic acid (VPA) use (8.5%) and the lowest with lamotrigine use (2.1%). The incidence of CMs was 5% at a carbamazepine dose <700 mg, 10% at a carbamazepine dose ≥700 mg, 5.5% at a VPA dose <750 mg, and 14.8% at a VPA dose ≥750 mg. Thus the risk of malformation increased 2.33 times (p = .041) in infants of PWWE receiving high-dose ASMs. SIGNIFICANCE: Birth outcomes of PWWE receiving and not receiving ASMs were evaluated. The risk of CMs occurrence was higher, particularly in infants of PWWE using VPA and receiving polytherapy. The incidence of CMs was found to be lower in infants of PWWE receiving lamotrigine.

Eye closure sensitivity and genetic generalized epilepsies: A prospective study of 123 cases.

PURPOSE: To investigate the possible relationship between eye closure sensitivity (ECS) and genetic generalized epilepsy (GGE) in terms of epilepsy syndromes, photosensitivity (PS), and prognosis. METHOD: One hundred and twenty-three patients diagnosed with GGE were classified according to epilepsy syndromes. Among them, ECS and PS were detected in repeated video-EEGs. In patients with ECS, the impact of sleep deprived EEG on awakening on ECS was evaluated. To explore the ECS as a possible accurate predictor designating the prognosis in GGEs, we defined the ECS rate in poor prognosis group (n = 21) and in patients without antiseizure drug (ASD) treatment and seizure-free for at least for 2-5 years (n = 20). RESULTS: ECS was found in almost all types of GGE but at different rates. ECS was detected in all groups with highest rate in eyelid myoclonia with or without absences (EMA). Sleep deprived EEG on awakening was performed in 44 of 59 patients with ECS and enhanced ECS prominently. In all groups except EMA, PS rate was lower compared with ECS. Both ECS and PS were observed in 15 patients with poor prognosis (71 %) and one patient with good prognosis (5%). CONCLUSIONS: ECS can occur in all types of the GGEs. We claim that ECS can be asserted as a syndrome-specific feature for EMA among GGEs. Sleep deprived EEG on awakening can enhance ECS prominently in all types of GGE. ECS and PS might overlap but their impact on prognosis is different. In the poor prognosis group, the number of patients having ECS together with PS was high compared to the group without ASD and excellent prognosis. Therefore, we suggest that this combination predicts worse outcome in GGEs.

Panayiotopoulos Syndrome with a special emphasis of ictal semiologic features.

PURPOSE: The aim was to identify the demographic, electroencephalographic findings, clinical features, especially ictal semiological symptoms of patients diagnosed with Panayiotopoulos Syndrome. METHOD: The semiologic and EEG findings of 42 patients were reviewed. Statistical analysis was used to determine the frequencies of semiologic features and to define the symptom clusters. RESULTS: The most common clinical features were nausea (54.8%), vomiting (50.0%), pallor (50.0%), deviation of head and eyes (45.2%), generalized seizures (35.7%), ictal syncope (33.3%) and stomach ache (26.2%). Visual manifestations which were observed in 38.9% of the patients were amaurosis, flashes of colourful lights, flashes of brightful lights, visual hallucinations and blurring of vision. Autonomic status epilepticus was observed in 21.4% of the patients. Cluster analysis of 13 most common symptoms showed a tendency toward 3 clusters. Pallor constituted a subgroup on its own whereas nausea, vomiting, deviation of head and eyes tended to occur together in the second subgroup and the third subgroup included semiologic features some of which were atypical for PS. CONCLUSIONS: A broad spectrum of typical and atypical clinical features might be observed in patients with PS. Some of the semiologic features of PS might have tendency to occur together. Overlapping of typical autonomic features with some of the atypical features might suggest that PS, RE and ICOE-G were the constituents of the same neurobiological spectrum. Our detailed data might contribute to the awareness of a wide range of variabilites in the electroclinical features of PS.

Right cerebral hemiatrophy: neurocognitive and electroclinical features.

The purpose of this study was to retrospectively evaluate the cognitive and electroclinical characteristics of right cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome [DDMS]). Cognitive assessments with a particular emphasis on visuospatial functions, electroclinical features, and neuroimaging characteristics were analyzed for five patients with a clinically and neuroradiologically confirmed diagnosis of right-sided DDMS. Intelligence tests revealed mental retardation in all but one. Neuropsychological assessments demonstrated consistent impairments in tasks that have a spatial component (spatial processing and orientation discrimination), whereas attention, executive functions and verbal memory domains were variably impaired. Electroclinically, the main seizure types were simple partial motor, complex partial, and secondarily generalized seizures. Interictal EEG delineated lower amplitudes and slow background activity in the affected hemisphere. Overall, the cognitive performance of patients with DDMS encompasses a broad spectrum of impairments affecting multiple domains. Our findings support the concept that dorsal visual pathways responsible for spatial processing may be lateralized to the right hemisphere.

Panayiotopoulos syndrome with coincidental brain lesions.

PURPOSE: To describe the clinical and electroencephalographic features of three patients diagnosed with Panayiotopoulos syndrome with different lesions identified by cranial MRI investigation. METHODS: Our study was based on patients from the Epilepsy Outpatient Clinic of Sisli Etfal Education Hospital in Istanbul, where a prospective study of Panayiotopoulos syndrome was initiated in 1995. Records of our Epilepsy Outpatient Clinic revealed 53 patients with Panayiotopoulos syndrome. Among them were three with cranial lesions identified by MRI. RESULTS: The onset of the seizures in our patients ranged between five and eight years. The seizures included mainly autonomic symptoms such as nausea, vomiting, pallor, mydriasis, urinary and fecal incontinence, and rarely hypersalivation. Autonomic partial status was detected in one patient. The personal history of our patients revealed head trauma in two and difficult birth history in one patient. Two patients described simple febrile seizures. All patients had occipital spike or spike-wave complexes in their EEGs. The background activity was normal. From the cranial MRI, one patient had a neuroepithelial cyst, the second patient had a right occipital encephalomalasic lesion and the third patient had an arachnoid cyst located in the cisterna magna associated with colpocephaly. Seizure frequency was low in general. All patients except for one received carbamazepine treatment 450-600 mg/day. None of our patients had seizures under antiepileptic treatment. CONCLUSIONS: Children with Panayiotopoulos syndrome may have static MRI brain findings which are likely to be coincidental and do not affect prognosis.

Hot water epilepsy: clinical and electroencephalographic features of 25 cases.

The aim of this study was to analyze the clinical and electroencephalographic findings for 25 patients with hot water epilepsy. Personal and family history, neurological state, age at onset and types of seizures, provoking factors, bathing habits, EEGs, and neuroimages were all reviewed. Age at onset of seizures ranged from 6 months to 37 years. Twenty patients had complex partial seizures. Eight patients had spontaneous seizures as well; one was a typical case of idiopathic photosensitive occipital lobe epilepsy. One patient described an episode interpreted as nonconvulsive status. In our study group, the pouring of the water over the head and the temperature of the water were the most common triggering factors. A special kind of soap and entry of water into the mouth were determined to be unusual triggering factors. Interictal EEGs revealed epileptogenic abnormalities located over the temporal regions in nine patients. Seventeen patients underwent neuroimaging, mostly cranial magnetic resonance imaging. One had right mesial temporal sclerosis and one cortical atrophy; the others had normal findings. Sixteen patients received antiepileptic drugs, mainly carbamazepine, and remained seizure-free. The high rate of epileptogenic abnormalities localized in the temporal region and the complex partial seizures observed in most of our patients indicate the considerable role of the temporal lobe in hot water epilepsy.

Evidence against association between arachnoid cysts and epilepsy.

Arachnoid cysts are often discovered incidentally in MRs performed for a variety of reasons. In this study, we have attempted to determine a possible relationship between the arachnoid cyst, seizure type and EEG abnormalities. Eight patients were classified according to the Classification of Epilepsies and Epileptic Syndromes of ILAE as idiopathic generalized or localized epilepsy: three as idiopathic generalized epilepsy, three as Rolandic epilepsy and two as juvenile myoclonic epilepsy. Two patients with rare nocturnal seizures had normal EEGs in the awaking and sleep stage. The EEGs of five patients with simple or complex partial seizures revealed focal epileptiform abnormalities, but only one patient had the same location with the arachnoid cyst. Five patients with symptomatic epilepsy had diffuse slowing in their EEGs and only one patient had seizure focus in the EEG that was contralateral to the arachnoid cyst. According to our study, seizure type and EEG abnormality corresponds to arachnoid cyst location in only one patient. Therefore, we suggest that arachnoid cysts may not be related to a specific seizure type and EEG focus.