Evaluating the prevalence and risk factors for depression in patients with temporal lobe epilepsy with hippocampal sclerosis: A cross-sectional multicenter study.

BACKGROUND: Epilepsy frequently accompanies Major Depressive Disorder (MDD). Notably, people with temporal lobe epilepsy and hippocampal sclerosis may face an increased susceptibility to MDD, as evidence indicates the involvement of the limbic system in the development of emotional symptoms. OBJECTIVES: To determine the prevalence and predictors of depression in temporal lobe epilepsy with hippocampal sclerosis (TLE-HS) and compare them to those of other epilepsy types. METHODS: A sample of 293 epilepsy patients, including 159 non-TLE-HS and 134 TLE-HS, were recruited from three hospitals. Of these, 215 completed a two-section electronic survey. The first section collected demographic and epilepsy data, while the second used the Arabic version of the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E). RESULTS: Of 215 patients, 104 (48%) had TLE-HS-38 with right TLE-HS (37%), 56 with left TLE-HS (54%), and 10 with bilateral TLE-HS (10%). The prevalence and severity of depression was assessed with an NDDI-E score of 15 or higher identified 35 patients (16%) with MDD. Valproic acid and lamotrigine were associated with higher NDDI-E scores. No such associations were found for levetiracetam or carbamazepine. Polytherapy in TLE-HS showed a significant correlation with daily poor concentration. CONCLUSION: We explored the differences in depression prevalence between TLE-HS and other epilepsy types and concluded they are minimal but slightly higher in TLE-HS. Predictors of depression such as seizure frequency and disease duration influenced MDD prevalence in TLE-HS. Lamotrigine and valproate were linked to higher NDDI-E scores.

Longitudinal Comparison of PNES spell and ASM reduction in PNES Patients with and without Epilepsy Discharged from an Epilepsy Monitoring Unit.

OBJECTIVE: To examine trends of Antiseizure Medication (ASM) reduction and discontinuation, as well as Psychogenic Non-Epileptic Seizure (PNES) spell reduction and resolution in patients with PNES, with and without comorbid epileptic seizures (ES). METHODS: A retrospective analysis was conducted on data from 145 patients with PNES, including 109 with PNES alone and 36 with PNES plus comorbid epilepsy. Patients were admitted to the Epilepsy Monitoring Unit (EMU) between May 2000 and April 2008, with follow-up clinical data collected until September 2015. Clinical records were thoroughly examined, encompassing the period preceding the PNES diagnosis until either loss to follow-up or September 2015. A subsequent chart review was conducted by two neurologists, covering the period following the diagnosis of PNES until either loss to follow-up or September 2015, which ever came first. RESULTS: Patients with PNES alone had higher rates of ASM reduction for all variables of ASM reduction measured compared to those with comorbid epilepsy (all at p < 001). Among patients with PNES alone, reductions in ASMs were observed after EMU discharge, but an uptick and plateau were seen in later follow-up years (100% of patients free of ASMs at years 2-3, 20% on at least one ASM by year 7). This pattern differs greatly in PNES + ES patients, in which the only time point at which any patient was able to discontinue all ASMs was at EMU discharge (4.5% of patients), with all patients taking at least one ASM for every other follow-up time point. Reductions in PNES spell frequency did not differ significantly between the two groups (for example PNES spells reduced at final FU 47.2% vs 42.9%, p = 0.65). In both groups, despite an initial drop in variables of PNES spell reduction and resolution in the early years post discharge, there is an eventual rebound and plateau (for example in PNES only patients, 33.9% of patients having no resolution in 1st year FU, which rises to 78% at years 4-5, and plateus around 52.8% at more than 7 years follow-up.) SIGNIFICANCE: This study contributes to the growing body of research focused on improving the current approach to management and prognostic outlook of PNES. Although PNES only patients had higher rates of ASM reduction, the uptick and plateau observed in later years highlights the challenges in managing PNES. Similarly, the continued persistence and rebound of PNES spells underline the continued poor prognostic outcomes associated with this condition.

Psychogenic non-epileptic seizures with and without epilepsy: Exploring the influence of co-existing psychiatric disorders on clinical characteristics and outcomes.

BACKGROUND AND OBJECTIVES: Psychogenic non-epileptic seizures (PNES) are commonly associated with co-existing psychiatric disorders. The relationship between psychiatric factors and PNES episodes with and without epilepsy remains understudied. We reviewed co-existing psychiatric disorders in PNES-only, PNES with epilepsy aiming to examine whether these co-existing disorders associated with PNES clinical presentation and long-term outcomes. METHODS: We conducted a retrospective, longitudinal cohort study of patients with PNES diagnosed at our EMU from May 2000 to April 2008, with follow-up clinical data until September 2015. We categorized patients into three groups: PNES-only, PNES+ definite epilepsy, and PNES+ possible/probable epilepsy. RESULTS: In total, 271 patients with PNES were identified: 194 had PNES-only, 30 had PNES+ possible or probable epilepsy, and 47 had PNES+ definite epilepsy. No significant differences were observed in the prevalence of depression, anxiety, post-traumatic stress disorder (PTSD), substance abuse, or suicidal thoughts among the three groups. Similarly, no differences in co-existing psychiatric disorders characteristics were discovered among patients grouped by various durations and frequencies of PNES episodes. At EMU admission, for PNES-only patients total of 130/194 patients (67%) were on ASMs, and 64/194 (32.9%) were not. PNES-only not on ASM were the most likely to report at least two of the three main psychiatric disorders (depression, anxiety, and PTSD; p = 0.01). At the final follow-up, 68/130 (52.3%) and 92/130 (70.8%) patients were able to discontinue or reduce their ASM intake, respectively, with no significant differences in co-existing psychiatric disorders among them (p < 0.001). Overall, 51.6% or 31.3% of patients reported reduced or resolved PNES episodes, respectively. Further, this reduction and resolution of PNES episode were not affected by any psychological variable. CONCLUSIONS: Co-existing psychiatric disorders prevalence did not differ between patients with PNES-only and those with coexisting epilepsy. Further, co-existing psychiatric disorders characteristics did not reliably predict PNES episode duration, frequency, reduction, or resolution. For patients with PNES-only, the presence of co-existing psychiatric disorders did not predict the rate at which ASMs could be reduced or discontinued.

Patients with psychogenic nonepileptic seizures and suspected epilepsy: An antiseizure medication reduction study.

OBJECTIVE: To examine predictors of ASM reduction/discontinuation and PNES reduction/resolution in patients with PNES with a confirmed or strong suspicion of comorbid ES. METHODS: A retrospective analysis of 271 newly diagnosed Patients with PNESs admitted to the EMU between May 2000 and April 2008, with follow-up clinical data collected until September 2015. Forty-seven patients met our criteria of PNES with either confirmed or probable ES. RESULTS: Patients with PNES reduction were significantly more likely to have come off all ASMs by the time of final follow-up (21.7 vs. 0.0%, p = 0.018), while documented generalized (i.e. epileptic) seizures were much more common in patients with no reduction in PNES frequency (47.8 vs 8.7%, p = 0.003). When comparing patients that reduced their ASMs (n = 18) with those that did not (n = 27), the former were more likely to have neurological comorbid disorders (p = 0.004). When comparing patients with PNES resolution (n = 12) vs not (n = 34), those with PNES resolution were more likely to have a neurological comorbid disorder (p = 0.027), had a younger age at EMU admission (29.8 vs 37.4, p = 0.05) and a greater proportion of patients with ASMs reduced in EMU (66.7% vs 30.3%, p = 0.028). Similarly, those with ASM reduction had more unknown (non-generalized, non-focal) seizures (33.3 vs 3.7%, p = 0.029). On hierarchical regression analysis, a higher level of education and absence of generalized epilepsy remain as positive predictors of PNES reduction (p = 0.042, 0.015), while the presence of some other neurological disorder besides epilepsy (p = 0.04) and being on more ASMs at EMU admission (p = 0.03) were positive predictors of ASM reduction by final follow-up. SIGNIFICANCE: Patients with PNES and epilepsy have distinct demographic predictors of PNES frequency and ASM reduction by final follow-up. Patients with PNES reduction and resolution had higher level education, less generalized epileptic seizures, younger age at EMU admission, more likely to have presence of a neurological disorder besides epilepsy, and a greater proportion of patients had a reduction in the number of ASMs in the EMU. Similarly, patients with ASM reduction and discontinuation were on more ASMs at initial EMU admission and also were more likely to have a neurological disorder besides epilepsy. The positive relationship between reduction in psychogenic nonepileptic seizure frequency and discontinuation of ASMs at final follow-up elucidates that tapering medication in a safe environment may reinforce psychogenic nonepileptic seizure diagnosis. This can be reassuring to both patients and clinicians, resulting in the observed improvements at the final follow-up.

Risk factors for comorbid epilepsy in patients with psychogenic non-epileptic seizures. Dataset of a large cohort study.

Psychogenic non-epileptic seizures (PNES) are the main differential diagnosis of pharmacorresistant epilepsy. Achieving the certainty in the diagnosis of PNES may be challenging, especially in the 10-22% of cases in which PNES and epilepsy co-exist. This difficulty hampers the management of these patients. Unfortunately, published series with this combined pathology are scarce and small in size. This article presents the dataset of our article "Factors associated with comorbid epilepsy in patients with psychogenic non-epileptic seizures: a large cohort study" (Massot-Tarrús et al. 2022). It is composed by a detailed demographic and clinical data of 271 consecutive patients diagnosed with PNES in our epilepsy monitoring unit (EMU) between May 2001 and February 2011, and followed until September 2016. Based on the clinical, neuroimaging and vEEG findings, 47 of these patients were diagnosed with definite comorbid epilepsy, and 30 with possible or probable comorbid epilepsy. All data was collected retrospectively from chart review. The cohort is depicted by means of demographic variables; age at PNES onset; years with PNES; frequency of PNES; duration of longest PNES seizure; self-reported history of minor head trauma (not associated with an increased risk of epilepsy) immediately preceding the first PNES; history of substance abuse; past or present history of active suicidal ideation; neuropsychological evaluation with the Minnesota Multiphasic Personality Inventory test; number and nature of risk factors for epilepsy; co-morbid degenerative brain disease or other neurological or psychiatric medical conditions; semiology of the seizures and triggers; EEG findings; type of epilepsy; number of past EMU admissions and epilepsy clinic visits and re-referrals; number of Anti-Seizure Medications (ASM) at EMU admission and discharge; and the outcome of the spells and ASM after the EMU discharge. Those ASM prescribed for reasons other than the treatment of the seizures (e.g., psychiatric disorders, migraine, pain syndromes, etc.) were not counted. The presented baseline data can be used in studies evaluating the characteristics of patients with PNES and comorbid epilepsy, and in the creation of algorithms to identify them. It could facilitate the prioritization of this subgroup of patients for prolonged video-EEG monitorization to confirm the co-existence of both types of seizures and treat them accordingly.

Factors associated with comorbid epilepsy in patients with psychogenic nonepileptic seizures: A large cohort study.

OBJECTIVE: Comorbid epilepsy and psychogenic nonepileptic seizures (PNES) occur in 12-22% of cases and the diagnosis of both simultaneous disorders is challenging. We aimed to identify baseline characteristics that may help distinguish patients with PNES-only from those with comorbid epilepsy. METHODS: We performed a longitudinal cohort study on those patients diagnosed with PNES in our epilepsy monitoring unit (EMU) between May 2001 and February 2011, prospectively followed up until September 2016. Patients were classified into PNES-only, PNES + possible or probable epilepsy, and PNES + definite epilepsy based on the clinical, vEEG, and neuroimaging data. Demographic and basal clinical data were obtained from chart review. Multiple regression models were performed to identify significant predictors of PNES + definite epilepsy, excluding patients with only possible or probable epilepsy for this specific analysis. RESULTS: One-hundred and ninety four patients with PNES-only, 30 with PNES + possible or probable epilepsy and 47 with PNES + definite epilepsy were included. 73.8% were female and the mean age at EMU admission was 37.4 ±â€¯standard deviation 13.5 years. Patients with PNES + definite epilepsy most likely had never worked, had history of febrile seizures, structural brain lesions, developmental disabilities, and maximum reported seizure duration between 0.5 and 2 min. Patients with PNES-only were on fewer anti-seizure medications (ASM), reported more frequently an initial minor head trauma, seizures longer than 10 min, and a higher number of neurological and medical illnesses - being migraine (18.1%), other types of headaches (18.5%), and asthma (15.5%) the most prevalent ones. All p < 0.05. On the hierarchical regression analysis, history of febrile seizures, developmental disabilities, brain lesions, longest reported seizure duration between 0.5 and 2 min, and lack of neurological comorbidity, remained as significant predictors of PNES + epilepsy. The model's performance of a 5-fold cross-validation analysis showed an overall accuracy of 84.7% to classify patients correctly. CONCLUSIONS: Some demographic and clinical characteristics may support the presence of comorbid epilepsy in patients with PNES, being unemployment, the presence of brain lesions, developmental disabilities, history of febrile seizures, seizure duration and lack of comorbid headaches the most relevant ones.

EEG and MRI Abnormalities in Patients With Psychogenic Nonepileptic Seizures.

OBJECTIVE: To compare the rate of EEG and MRI abnormalities in psychogenic nonepileptic seizures (PNES) patients with and without suspected epilepsy. Patients were also compared in terms of their demographic and clinical profiles. METHODS: A retrospective analysis of 271 newly diagnosed PNES patients admitted to the epilepsy monitoring unit between May 2000 and April 2008, with follow-up clinical data collected until September 2015. RESULTS: One hundred ninety-four patients were determined to have PNES alone, 16 PNES plus possible epilepsy, 14 PNES plus probable epilepsy, and 47 PNES plus confirmed epilepsy. Fifty-seven of the 77 patients (74.0%) with possible, probable, or definite epilepsy exhibited epileptiform activity on EEG, versus only 16 of the 194 patients (8.2%) in whom epilepsy was excluded. Twenty-four of these 194 patients (12.4%) had MRI abnormalities. Three of 38 patients (7.9%) with both EEG and MRI abnormalities were confirmed not to have epilepsy. In PNES patients with EEG or MRI abnormalities compared with those without, patients with abnormalities were more likely to have epilepsy risk factors, such as central nervous system structural abnormalities, and less likely to report minor head trauma. The presence of EEG abnormalities in PNES-only patients did not influence antiseizure medication reduction, whereas those with MRI abnormalities were less likely to have their antiseizure medications reduced. CONCLUSIONS: Psychogenic nonepileptic seizure patients without MRI or EEG abnormalities are less likely to have associated epilepsy, risk factors for epilepsy, and had different demographic profiles. There is a higher-than-expected level of EEG and MRI abnormalities in PNES patients without epilepsy.

Prevalence, incidence, and risk factors of epilepsy in arab countries: A systematic review.

OBJECTIVE: To describe the prevalence, incidence, and risk factors of epilepsy in Arab countries. METHODS: In this systematic review, electronic databases including PubMed, ProQuest Public Health and Cochrane Library were searched to identify relevant English articles published until April 2020. The search was conducted to cover all 22 Arab countries. Studies were eligible for inclusion if they assessed any of these epidemiological parameters of epilepsy: prevalence, incidence, and/or risk factors and focused on at least one of the Arab countries. The quality of the studies was evaluated using standardized quality assessment tools. RESULTS: Twenty-nine articles met the inclusion criteria. Out of the 22 Arab countries, only 11 were found to have published studies reporting on the epidemiology of epilepsy. The median lifetime prevalence of epilepsy in Arab countries was found to be 6.9 per 1000. The median incidence is 89.5 per 100000. The most frequently identified risk factors were parental consanguinity, family history of epilepsy, and a history of perinatal infections/insults. CONCLUSION: The prevalence and incidence of epilepsy varies in Arab countries. The most frequently reported risk factor is parental consanguinity. The lack of epidemiological studies on epilepsy in half of the Arab countries calls for more studies on this aspect to identify the burden and risk factors of epilepsy in this region.

Predicting outcome of patients with psychogenic nonepileptic seizures after diagnosis in an epilepsy monitoring unit.

OBJECTIVE: To identify predictors of Psychogenic NonEpileptic Seizure (PNES) improvement and anti-seizure medication (ASM) discontinuation in patients with PNES only. METHODS: This is a retrospective study of a consecutively enrolled cohort of 271 patients diagnosed with PNES by video-EEG (vEEG) telemetry in our Epilepsy Monitoring Unit (EMU) between May 2000 and February 2010. Patients with any possibility of past or present comorbid epilepsy based on clinical, EEG, and neuroimaging, or less than one year of follow-up after discharge were excluded. RESULTS: A total of 109 subjects were included. The mean age at PNES onset was 33 (range 6-89), mean age at EMU admission was 38.3 (16-89.8), 70.6% were female mean video-EEG length was 6.1 days, and the median time of final follow-up 3.3 (CI 1.6-6.4) years. 51/108 patients (47.2%) reported a PNES decrease and 29 (26.8%) experienced PNES resolution. 59/73 (81.9%) subjects on ASM at the time of EMU admission were able to discontinue them by the final visit. On univariate analysis, patients whose PNES frequency improved were significantly younger at time of admission, more likely married or cohabiting, less likely unemployed, less likely to have migraine, and had a higher frequency of PNES. On hierarchical regression analysis, younger age and employment remained significant predictors of PNES improvement and resolution. Patients who achieved ASM discontinuation had significantly more children and subsequent EMU visits, were less likely to have history of minor head trauma immediately preceding PNES onset and structural brain lesions, experienced a greater reduction of ASMs during the EMU admission, and had a greater improvement of their PNES frequency at the final visit (p ≤ 0.05). On hierarchical regression analysis, higher number of children, absence of structural brain lesions, fewer ASMs at EMU discharge, and improvement of PNES frequency remained significant predictors of ASM discontinuation. CONCLUSION: The outcome of PNES is positively correlated with earlier age of diagnosis in an EMU, especially in patients with better social resources. Furthermore, discontinuation of ASM is more likely if the process is initiated during the EMU stay and in the absence of structural brain lesions.

Blue rubber bleb nevus syndrome associated with tuberous sclerosis complex and CNS involvement.

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder that is characterized by multiple dome-shaped cutaneous venous malformations on the skin and visceral organs. Typical extra-cutaneous lesions have the appearance of blueish nipple-shaped nodules that can easily compress and refill. We described a rare case of a 23-year-old female with BRBNS and tuberous sclerosis complex (TSC) that presented with central nervous system (CNS) involvement including unprovoked focal impaired awareness seizure. Her BRBNS presents with hemangiomas involving multiple organs in the body including the brain, gastrointestinal (GI) system, and skin. This case highlights the importance of studying and understanding the association between BRBNS and TSC as it may lead to improved understanding.

Seizure outcome after epilepsy surgery for patients with normal MRI: A Single center experience.

OBJECTIVE: To analyze the surgical outcome in non-lesional intractable focal epilepsies in our center and to find possible predictors for better outcome. METHODS: This is a retrospective study for 40 adult patients with intractable focal epilepsy following at KFSHRC-Riyadh, who underwent presurgical evaluation followed by resective surgery and continued follow up for a minimum of 2 years. The surgery outcome was evaluated based on the type of surgical procedure and histopathology results. RESULTS: Out of all 40 patients studied, seizure freedom was achieved in 19 (47.5 %) and 17 (42.5 %) patients at the first and second year respectively in all non-lesional cases. Seizure freedom in non-lesional temporal lobe surgery was achieved in 10 (45 %) of patients at 2 years, 5 (38 %) in non-lesional frontal lobe patients at 2 years and 8 (44 %), 7 (38 %) for all extratemporal at 1 and 2 years respectively. Good prognosis was seen in patients with localized positron emission tomography (PET), had no aura and had a clear ictal onset either on scalp electroencephalogram (EEG) or subdural invasive electroencephalogram. SIGNIFICANCE: The best surgical outcome is achievable in patients with non-lesional focal epilepsy. This study highlights the prognostic value of the PET scan and ictal scalp/subdural invasive EEG.

Lateralization value of peri-ictal headache in drug-resistant focal epilepsy.

OBJECTIVE: To examine the lateralizing value of unilateral peri-ictal and interictal headaches in patients with drug-resistant focal epilepsy (DRFE). METHODS: Four-hundred consecutive patients undergoing presurgical evaluation for DRFE were interviewed. Patients with headache were broadly divided into two groups: peri-ictal and interictal headache. The lateralizing value of unilateral headache was compared in each group between three diagnoses: temporal lobe epilepsy (TLE), extratemporal lobe epilepsy (ETLE), and temporal-plus epilepsy (TEMP+ epilepsy). RESULTS: Out of 400 patients, 169 (42.25%) had headaches. Peri-ictal headaches were experienced in 106 patients (26.5%) and interictal headaches were experienced in 63 (15.75%). In the peri-ictal group, unilateral headaches were present in 48 out of 60 patients (80%) with TLE; they were ipsilateral to the seizure focus in 45 out of 48 patients (93.75%). Unilateral headaches in patients with ETLE were present in 20 out of 31 patients (64.5%) and were ipsilateral to the seizure focus in 14 out of 20 patients (70%). In patients with TEMP + epilepsy, unilateral peri-ictal headaches were present in 9 out of 15 patients (60%); they were ipsilateral to the seizure focus in all 9 patients (100%). In the interictal headache group, unilateral headaches were ipsilateral the seizure focus in 9 out of 10 patients (90%) with TLE and 5 out of 6 patients (83.3%) with ETLE. None of the TEMP + epilepsy patients had a unilateral interictal headache. CONCLUSION: Headache is a frequently encountered symptom in patients with DRFE. When occurring in a unilateral fashion, it has a high lateralizing value in temporal and extratemporal lobe epilepsies. This has been demonstrated to be true for both peri-ictal and interictal headaches. In the vast majority of patients with DRFE, unilateral headache occurs ipsilateral to the seizure focus.

Frontal disconnection surgery for drug-resistant epilepsy: Outcome in a series of 16 patients.

OBJECTIVE: To evaluate the effectiveness of frontal disconnection surgery in seizure control and related consequences in a consecutive patient series. METHODS: We conducted a retrospective analysis of patients who underwent frontal disconnection surgery for drug-resistant epilepsy (DRE). Baseline epilepsy characteristics, detailed presurgical evaluation including epileptogenic zone (EZ) localization, magnetic resonance imaging (MRI) detection of epileptogenic lesion, and pathological findings were reviewed. Patients were followed postoperatively for seizure outcome at 1 year. RESULTS: A total of 16 patients were identified (six children and 10 adults). Most patients had a childhood onset of DRE with a median duration of epilepsy of 6.5 years (interquartile range 3.5-17.5 years) before surgery. In 10 (62.5%) patients, the EZ was localized to the frontal lobe, while in six patients, the EZ involved also adjacent lobes or consisted of multiple foci. In 10 (62.5%) patients, an epileptogenic lesion was detected on presurgical MRI, four of which (40%) had all MRI abnormalities confined to the frontal lobe. Two-thirds of the patients (11/16; 68.8%) underwent isolated frontal disconnection procedure, while remaining patients had frontal disconnection combined with resection of an adjacent lobe. Of the 12 patients in whom biopsy was taken from the disconnected frontal lobe, six (50%) had pathology-proven focal cortical dysplasia. We observed surgical-related complications in three (18.8%) cases, neurological deficits in other three (18.8%) patients, and worsening cognitive abilities in one (6.3%) patient. Overall, eight (50%) patients became completely seizure-free (ILAE 1) at one-year follow-up. SIGNIFICANCE: Frontal disconnection surgery for DRE can result in seizure freedom in certain patients, especially when the EZ is strictly limited to the ipsilateral frontal region, and the MRI shows an epileptogenic lesion that is purely frontal in location. Frontal lobe disconnection procedure is safe and has a limited complication rate. However, further studies with larger patient population will yield more significance.

Seronegative Neuromyelitis Optica Spectrum Disorder: An Unusual Presentation of Acute Brainstem Syndrome.

BACKGROUND Neuromyelitis optica (NMO) is an autoimmune, demyelinating, inflammatory disorder affecting the central nervous system, mostly targeting optic nerves and the spinal cord. NMO spectrum disorder (NMOSD) is a newly revised nomenclature in which new diagnostic criteria have been developed, including serological testing of serum aquaporin-4 immunoglobulin G (AQP4-IgG) antibodies. Results of a negative antibody will group the patient in a seronegative subgroup. CASE REPORT We describe the case of a 27-year-old female who presented to our hospital with new onset of sudden unexplained vomiting, dysphagia, dysphonia, and food regurgitation. Extensive investigations were done and brain magnetic resonance imaging (MRI) showed a small nonspecific area of signal abnormality in the right dorsal medulla. Aquaporin-4 antibodies were negative, and the patient was diagnosed with seronegative NMOSD with acute brainstem syndrome after meeting the diagnostic criteria. The patient's condition improved after steroids administration. CONCLUSIONS We report an unusual presentation of seronegative NMOSD presenting with acute brainstem syndrome.

Six adult patients with septo-optic dysplasia and drug-resistant epilepsy: Clinical findings and course.

Septo-optic dysplasia (SOD) is a rare disorder associated with optic nerve hypoplasia, pituitary abnormalities and agenesis/dysgenesis of midline brain structures including the septum pellucidum and corpus callosum. Though sometimes associated with drug-resistant epilepsy, this association has not been well studied. We report six SOD patients with associated malformation of cortical development (MCD) and drug-resistant epilepsy who underwent video-EEG telemetry at our centre between 1998 and 2016 for drug-resistant epilepsy. ‬Three then underwent surgery; right temporal neocortical resection, right functional hemispherectomy and placement of a vagus nerve stimulator. Clinical findings and the patients' ultimate courses are discussed.