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Purpose: Leptomeningeal carcinomatosis (LC) is a devastating condition in patients with systemic malignancies or primary brain tumors. Although much is known about neuro-radiologic investigations, there is very little information about EEG findings in these patients. Whether EEG is correlated with cranial magnetic resonance imaging (MRI) results and survival has not been investigated. Methods: Medical records of 2340 adult patients with the diagnosis of brain tumor, either metastatic (Group 1) or primary (Group 2), between 2000-2021 were reviewed for the presence of LC and seizures. Demographic and clinical features, laboratory results and Karnofsky performance scores of included patients were noted. Available routine EEG recordings were re-evaluated. Any possible correlation between EEG findings-MRI and EEG findings-survival were investigated statistically. Results: Sixty-six patients with LC and seizures were identified. The most common malignancies were lung cancer and glioblastoma multiforme. Twenty-six EEG recordings of 17 patients in Group 1, and 13 EEGs of 9 patients in Group 2 were available for final analysis. The most common EEG characteristic was background slowing (73%). The most frequent findings were rhythmic periodic patterns or spike wave activity (27%). Sporadic epileptiform discharges (8%) or ictal recordings (4%) were very rare. None of the EEG features correlated with MRI results or survival. Conclusion: There are various EEG patterns in patients with LC and seizures. The most common findings are related to background activity, with rhythmic periodic patterns or spike wave activity being observed less commonly. EEG characteristics do not predict MRI findings or survival.

Refractory/super-refractory nonconvulsive status epilepticus (r/srNCSE) is an acute life-threatening neurocritical entity with significant morbidity. Failure to control SE in its earlier stages leads to multiple molecular alterations in the brain such as downregulation of GABA-A and upregulation of NMDA receptors. Recently ketamine, an NMDA receptor antagonist, has gained increased attention as a therapeutic choice in controlling refractory/super-refractory SE. We aimed to analyze the efficacy and safety profile of ketamine in our center. We retrospectively identified all the patients with nonconvulsive SE who received ketamine during their follow-up in our neurological intensive care unit between 2009 and 2019. Information about demographic, clinical, and laboratory findings; concurrent antiseizure and anesthetic medications; time of initiation, dose and duration of ketamine infusion; any adverse effects and finally prognosis were collected. The effect of day of ketamine initiation and duration of infusion on ketamine efficacy were analyzed statistically. Seven patients (4 males, 3 females; age: 44-86 years) were included in the study. Encephalitis was the most common etiology. Concurrent antiseizure medications varied between 2 and 5. Six patients received midazolam before/during ketamine infusion. Ketamine was initiated 2 to 7 days after the onset of EEG monitoring and lasted 3 to 24 days with a maximum infusion dose ranging between 1 to 5 mg/kg/h. It was definitely effective in 4 patients, and possibly effective in an additional patient. Earlier initiation was correlated with higher efficacy ( P = .047). There was a trend toward higher efficacy with longer duration of infusion ( P = .285). Overall prognosis was poor with 29% mortality rate. Temporary hepatic failure occurred in 1 patient. Ketamine appears to be a promising drug in r/srNCSE. Earlier and prolonged infusion, as well as combination with benzodiazepines may increase its efficacy. Adverse events are rarely observed.

Objectives. The frequency and types of complications in patients with nonconvulsive status epilepticus (NCSE) who are followed up in the intensive care unit (ICU), and the impact of these complications on outcome are not well-known. We investigated the complications and their effects on prognosis in NCSE patients. Methods. After reviewing the video-EEG monitoring (VEEGM) reports of all the consecutive patients who were followed up in our ICU between 2009 and 2019, we identified two groups of patients: 1-patients with NCSE (study group) and 2-patients who underwent VEEGM for possible NCSE but did not have ictal recordings (no-NCSE group). Electronic health records were reviewed to identify demographic and clinical data, duration of ICU care, medical and surgical complications, pharmacologic treatment, and outcome. These parameters were compared statistically between the groups. We also investigated the parameters affecting prognosis at discharge. Results. Thirty-two patients with NCSE comprised the study group. Infection developed in 84%. More than half were intubated, had tracheostomy or percutaneous endoscopic gastrostomy application. Refractory NCSE was associated with significantly more frequent complications and worse outcome. There was a higher tendency of infections in the study group ( P = .059). Higher organ failure scores and prolonged stay in ICU predicted worse outcome ( P

Lateralized periodic discharges (LPDs) are unilateral electroencephalography (EEG) waveforms, recurring at regular intervals. There has been a long-lasting debate about whether they represent ictal or interictal phenomena. Very few patients in the literature have been investigated with multimodal functional imaging techniques. Here, we present a 58-year-old male patient with symptomatic epilepsy who had cerebral venous sinus thrombosis in the right temporo-parietal area and dural arteriovenous fistula (dAVF) over the left fronto-parietal region. He developed acute speech disturbances and altered mental status after a generalized tonic–clonic seizure. Video-EEG monitoring (VEEGM) demonstrated LPDs over the left fronto-central area, overlapping in part with the dAVF. Diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) sequences revealed restricted diffusion compatible with cytotoxic edema, whereas single-photon emission computed tomography (SPECT) indicated hyperperfusion in the same region, leading to the conclusion that he was having possible nonconvulsive status epilepticus (NCSE). An increase in antiseizure medications led to gradual improvement in clinical status and the disappearance of LPDs.

EEG is an important tool for neurologists in both diagnosis and classification of seizures. It is not uncommon in clinical practice to see patients who were erroneously diagnosed as epileptic. Most of the time incorrect interpretation of EEG contributes significantly to this problem. In this study, we aimed to investigate the success rate of neurology residents in EEG interpretation after formal training. Eleven neurology residents were included in the study. Duration of EEG training (3 vs 4 months) and time since completion of EEG education were determined. Residents were randomly presented 30 different slides of representative EEG screenshots. They received 1 point for each correct response. The effect of training duration and time since training were investigated statistically. Besides, we looked at the success rate of each question to see whether certain patterns were more readily recognized than others. EEG training duration ( P = .93) and time since completion of training ( P = .16) did not influence the results. The success rate of residents for correct responses was between 17% and 50%. On the other hand, the success rate for each question varied between 0% and 91%. Overall, benign variants and focal ictal onset patterns were the most difficult to recognize. On 13 occasions (6.5%) nonepileptiform patterns were thought to represent epileptiform abnormalities. After formal training, neurology residents could identify ≤50% of the EEG patterns correctly. The wide variation in success rate among residents and also between questions implies that both personal characteristics and inherent EEG features influence successful EEG interpretation.

Video-EEG monitoring is often used to detect nonconvulsive status epilepticus (NCSE) in critical care patients. Short recording durations may fail to detect seizures. In this study, we investigated the time required to record the first ictal event, and whether it could be correlated with some clinical or EEG parameters. Video-EEG recordings of patients who were followed up in our neurological intensive care unit were evaluated retrospectively. The EEG recordings of patients with NCSE were reviewed to determine the timing of the first seizure occurrence. Demographic data and EEG findings were obtained from patient charts and EEG reports. Possible correlations between the presence of periodic discharges (PD), Glasgow Coma Scale (GCS) score and early seizure detection (defined as a seizure within the first hour of recording) were explored statistically. Out of 200 patients who underwent video-EEG monitoring, we identified 30 cases (15%; 18 male, 12 female; age 24-86 years; mean recording duration 99 hours) with NCSE. The first seizure was recorded within 0 to 1 hour in 22 patients (73%) and within 1 to 12 hours in 6 patients (22%). Interictal PDs were identified in 19 patients (63%). GCS score was ≤8 in 16 patients (53%). There was no correlation between early seizure detection and PDs (p=1.0) or GCS score ( P = .22). In our study, >90% of the seizures were captured within 12 hours. This finding suggests that most of the NCSE cases can be identified even in centers with limited resources. The presence or absence of PDs or GCS score does not predict the timing of the first seizure.

Introduction. Frontal intermittent rhythmic delta activity (FIRDA), a transient rhythmic slow wave pattern over the anterior EEG leads, has been reported in a wide variety of cerebral lesions and different metabolic disturbances. Few authors have analyzed the frequency and clinical significance of FIRDA in the critical care setting. We aimed to better understand these issues in our intensive care cohort and if possible, try to delineate its underlying mechanisms. Methods. Video-EEG reports of consecutive adult patients in the neurological intensive care unit (NICU) since 2009 were retrospectively reviewed to identify cases with FIRDA. Demographic, clinical, and laboratory data were obtained from EEG reports and patient charts. Age- and sex-matched patients with acute stroke, hospitalized in NICU and no FIRDA on video-EEG monitoring served as the control group. Results. Among 162 patients who underwent video-EEG monitoring, FIRDA was documented in 17%. Female prevalence was 50% and age ranged from 23 to 82 years. Twenty-three (82%) of patients with FIRDA had a diagnosis of stroke. Comparison of demographic characteristics, EEG findings, metabolic disturbances and prognoses revealed no differences between stroke cases with and without FIRDA, except for higher frequency of acute and chronic isolated posterior circulation infarcts in patients with FIRDA. Conclusion. FIRDA is more commonly encountered in the neurocritical care setting as compared with outpatient EEG clinics. Our findings in stroke patients indicate that involved vascular territories may be related to the generation of FIRDA.

INTRODUCTION Specialized centers, in the management and surgical treatment of medically refractory epilepsy, emphasize the importance of differentiating the varieties of localization related epilepsies. There has been considerable recent interest in temporal and frontal lobe epileptic syndromes and less attention has been paid to parietal and occipital lobe epilepsies. METHODS Here we report the clinical, electroencephalographic and neuroimaging characteristics of 46 patients with medically refractory lesional parietal lobe epilepsy who have been followed up for 1-10 years. RESULTS In this study auras were reported in 78.3% of the patients and included sensory symptoms (72.2%), headache (36.1%), nausea and vomiting (36.1%), psychic symptoms (36.1%) and visual symptoms (16.6%). The most common ictal behavioral changes were paresthesia (69.6%) and focal clonic activity (39.1%). Tonic posture, various automatisms, head deviation, staring, sensation of pain and speech disturbances occurred to a lesser extent. Simple partial seizures were present in 69.6%. Complex partial seizures occurred in 43.5% and secondary generalized tonic clonic seizures were reported in 58.7% of the patients. Interictal routine EEG disclosed abnormal background activity in 1/3 of the patients. Nonlocalising epileptiform abnormalities were found in 34.8% of the patients. EEG findings were normal in 34.8% of the patients. The most common presumed etiologic factors were as follows: posttraumatic encephalomalacia, stroke, tumor, malformation of cortical development, atrophy, and arteriovenous malformation. CONCLUSION Clinical, electrophysiological and neuroimaging features of the lesional symptomatic partial epilepsy patients may help us to localize the seizure focus in some patients with cryptogenic partial epilepsy. So that, the timing decision of the parietal lobe sampling with more invasive techniques like intracranial electrodes prior to epilepsy surgery would be easier.

Although it is an orphan disease, isolated central nervous system Whipple's disease is one of the "must be known" conditions in neurology because it belongs to the list of "treatable disorders". Here, we present two cases which highlight the importance of early diagnosis. Additionally, we provide a discussion on up to date diagnostic approach to this life-threatening disorder.

Sensory neuronopathy is a well-established presentation in paraneoplastic neurological syndromes that is mostly associated with small cell lung cancer and anti-Hu antibodies. Motor neuronopathy, on the other hand, is an extremely rare observation in this syndrome. A 56-year-old man presented with asymmetric brachial diparesis and sensory ataxia. Electrophysiological studies revealed sensory ganglionopathy and progressive anterior horn degeneration in cervical segments. Small cell lung carcinoma with associated anti-Hu antibodies was later diagnosed. The patient did not improve despite the administration of steroids and chemotherapy. Paraneoplastic syndromes may exceptionally present with a bilateral arm weakness. Cases accompanied by sensory ganglionopathy should therefore be promptly investigated for any underlying malignancy.

Purpose: There is evidence of autonomic dysregulation in temporal lobe epilepsy. The structures removed during temporal lobectomy are important centers of central cardiovascular control; therefore surgery may conceivably alter the cardiovascular autonomic function. The effects of temporal lobectomy on autonomic cardiac control are controversial. We investigated the effects of temporal lobectomy on heart rate variability (HRV) in the early and late postoperative periods. Methods: We used 1-h ECG recordings to assess heart rate variability by spectral analysis in 24 consecutive patients who underwent temporal lobectomy due to intractable temporal lobe epilepsy. ECG recordings were performed before and twice (early and late) after surgery. The results were compared with age and sex matched controls. Results: When compared with controls, all the time and frequency domain indices (SDRR, RMSSD, TP, LF and HF) were significantly lower in the patient group before surgery. Findings were similar in the early and late post-operative periods except that the LF/HF ratio increased in the patient group after the late post-operative period. Within the patient group, compared to pre-operative results, normalized HF was increased in the early post-operative period; however in the late post-operative period, LF/HF ratio was increased. Conclusions: These findings show that in patients with intractable temporal lobe epilepsy, HRV is decreased globally in both sympathetic and parasympathetic domains. While the total HRV remains reduced throughout the postoperative periods, the LF/HF ratio, i.e., sympathovagal balance is altered, in favor of parasympathetic side early after surgery, but towards the sympathetic side after the first postoperative month. (C) 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Anti-NMDA receptor encephalitis is a severe disorder characterised clinically by seizures, autonomic instability, and severe disturbances of memory, behaviour, and cognition. Due to the severity of symptoms, many patients are admitted to the intensive care unit. For some patients, the presence of various movement disorders and abnormal autonomic signs, as well as a history of seizures, lead to a false impression of status epilepticus, which is reported in 6% of the cases. Here, we present two young female patients, one of whom had ovarian teratoma. Both patients were referred to our neurological intensive care unit with a diagnosis of status epilepticus. However, prolonged video-EEG findings were compatible with encephalopathy. We avoided aggressive treatment with intravenous anaesthetics and both patients recovered after immunotherapy, one of whom received surgery. Physicians should be cautious in interpreting abnormal movements and autonomic signs in such patients and video-EEG monitoring is advised when status epilepticus is suspected. [Published with video sequences].

Mesial temporal lobe epilepsy (MTLE) is often characterized pathologically by severe neuronal loss in the hippocampus. In this study we investigated concomitant appearance of the pro-apoptotic and anti-apoptotic mechanisms in injured neurons in epileptic human hippocampi. Postsurgical hippocampal specimens of randomly selected 25 patients with MTLE were studied with standard immunohistochemical techniques to detect the below markers of cell death pathways: truncated Bid – tBid, mitochondrial translocation of Bax (markers of pro-apoptotic Bcl-2 protein activation) and nuclear translocation of AIF (caspase-independent pro-apoptotic pathway). For cell survival pathways, we investigated the expression of c-IAP1, c-IAP2 and Hsp70 (heat shock protein). Immunopositive cells were counted in different regions of the hippocampus. We also verified IAP (inhibitor of apoptosis) expression with Western blotting. The results were statistically compared with hippocampi from non-epileptic autopsy controls. In patient hippocampi, Bax and tBid immunoreactivity were significantly increased and Bax staining was consistent with mitochondrial translocation. AIF was not translocated to the nucleus. c-IAP1 and c-IAP2 were barely detectable in control hippocampi, whereas their expression was dramatically increased in the patients in all hippocampal subfields. Interestingly, these neurons were also positively co-labeled for tBid and translocated Bax. Hsp70 immunreactivity was significantly increased in all surviving neurons in patient hippocampi whereas degenerating neurons failed to express Hsp70. Our findings are consistent with both pro-apoptotic and anti-apoptotic mechanisms being active within the same hippocampal neurons of patients with MTLE, illustrating an ongoing struggle between cell death and survival mechanisms in neurons under stress.

Objectives and Background: In patients with medically refractory seizures 20–30% do not have epilepsy, and the majority are diagnosed with psychogenic non-epileptic seizures (PNES). Almost one-third of this group has a prolonged episode that is termed as PNES status epilepticus (PNES-SE). The aim of this study was to describe the characteristics of patients with (1) PNES-SE only and (2) PNES-SE with concurrent epileptic seizures.Methods: We screened retrospectively all patients who underwent video-electroencephalogram (video-EEG) long-term monitoring at our institution between 1996 and 2009. Patients with PNES were identified by typical clinical features with no ictal EEG changes. Patients with continuous episodes for at least 30 min that led to intubation were identified as PNES-SE. They were divided into two groups: group 1, pure PNES-SE and group 2, PNES-SE with concurrent epilepsy. Group characteristics were defined with respect to demographic, clinical, electroencephalographic, neuroimaging and psychiatric variables.Results: PNES were recorded in 88/916 patients (10%) of whom 6 (7%) were treated intensively due to misdiagnosis of status epilepticus (SE), with two having coexistent epileptic seizures. All patients had concurrent psychiatric disorder(s), diagnosed by a psychiatrist. Duration of time until correct diagnosis was lengthy in all cases, and particularly for patients with PNES-SE with coexisting epileptic seizures (more than 10 years).Conclusion: To avoid iatrogenic complications and to treat the underlying psychiatric conditions, early diagnosis of PNES is necessary. Understanding the clinical characteristics of PNES-SE leads to early diagnoses, which may avoid iatrogenesis and economic burden.

The most common pathology in adult patients with temporal lobe epilepsy is hippocampal sclerosis (HS), the etiology of which is not clear. There is a conflicting evidence in literature regarding whether HS is genetic or acquired. Twin studies can help to clarify the mechanisms of HS, but limited numbers of twins have been studied. We describe two monozygotic pairs, in whom the affected twin had mesial temporal lobe epilepsy and HS. The unaffected twin remained seizure free on long-term follow-up. HS was confirmed pathologically in one of the affected twins. Our data and other limited twin studies indicate that HS occurs as a consequence of prolonged repeated seizures or other events during childhood. In other words, some acquired factors may be more important than genetic ones in the etiology of HS (Fig. 2, Ref. 16).

Background The potential complications of the subdural electrode implantation providing identification of the seizure focus and direct stimulation of the cerebral cortex for defining the eloquent cortical areas are epidural and subdural hematoma, cortical contusions, infection, brain edema, raised intracranial pressure, CSF leakage, and venous infarction have been previously reported in the literature. Purpose To present the first case of subelectrode hematoma without subdural component that was detected during invasive EEG monitoring after subdural electrode implantation. Case A 19-year-old female with drug resistant seizures was decided to undergo invasive monitoring with subdural electrodes. While good quality recordings had been initially obtained from all electrodes placed on the right parietal convexity, no cerebral cortical activity could be obtained from one electrode 2 days after the first operation.Explorative surgery revealed a circumscribed subelectrode hematoma without a subdural component. Conclusion Awareness of the potential complications of subdural electrode implantation and close follow-up of the clinical findings of the patient are of highest value for early detection and successful management.

Summary Purpose The C3435T polymorphism in the gene coding for P-glycoprotein (ABCB1) has been correlated with drug resistance in patients with epilepsy. However, replication studies have revealed conflicting results and the reason for this is not clear. We investigated the frequency of C3435T polymorphism in epileptic Turkish patients who underwent resective epilepsy surgery and compared our results with healthy controls. Methods DNA samples were obtained from 100 healthy controls and 89 consecutive adult patients who underwent resective brain surgery due to refractory seizures at our epilepsy center. Genotypes for the C3435T polymorphism were determined by PCR and restriction analysis. Results Comparison of drug-resistant patients and healthy controls revealed no significant difference in allele frequency (C vs. T; χ2 = 0.015, p = 0.90) and genotype frequency (χ2 = 2.05, p = 0.36). The findings in the pure hippocampal sclerosis (HS) group (n = 73) were not significantly different from control subjects, either (allele frequency: χ2 = 0.29, p = 0.59; genotype frequency: χ2 = 2.14, p = 0.34). Conclusions Our findings failed to prove an association between C3435T polymorphism and drug resistance in a sample of Turkish patients with refractory epilepsy who underwent resective brain surgery.

Various movement disorders have been described following hypoxic-ischaemic brain injury. Here, we present a 72-year-old female patient who developed periodic opening and upward deviation of the eyes as an isolated clinical finding, within 24 hours after cardio-pulmonary arrest. These movements were accompanied by burst-suppression on EEG, and both clinical and electrophysiological findings were suppressed 18 hours after intravenous levetiracetam infusion. The strictly periodic nature of both EEG discharges and eye opening with vertical deviation suggest a cause due to either activation of a subcortical/brainstem pacemaker reciprocally stimulating the cortex, or, alternatively, post-anoxic burst activity of viable cortical neural networks, somehow stimulating the relevant oculomotor nuclei. Together with previous similar cases, our case expands the spectrum of post-resuscitation myoclonus syndromes with the addition of this rare isolated oculopalpebral subtype. [Published with video sequence].

Neuroacanthocytosis is a group of disorders, clinically characterized with movement disorders, self-mutilation and seizures. There is little information in the literature regarding the clinical and EEG findings of the accompanying seizures in this neurodegenerative disorder. A 46-year-old man who was diagnosed as chorea-acanthocytosis, a subgroup of neuroacanthocytosis, was investigated for severe drug resistant seizures. Continuous video-EEG monitoring revealed an active left temporal epileptogenic focus with left temporal rhythmic discharges detected during his complex partial seizures. T2-weighted cranial MRI indicated hyperintensity of the left amygdala as well as hyperintensity and atrophy of bilateral basal ganglia. We discuss the development of focal seizures in a patient who has a neurodegenerative disease, namely neuroacanthocytosis.

The role of cerebral lesions associated with absence seizures, outside the frontal lobe, has not been demonstrated by both electroencephalography and neuroimaging techniques until now, to our knowledge. We present a case with absence seizures and EEGs with generalized 3 Hz spike-wave patterns that were preceded by a burst of spike-waves on the right parieto-occipital region. The patient had a history of head trauma, and cranial MRI revealed lesions that might be responsible for the seizures. The patient's lateralized and localized EEG findings were probably a representation of secondary bilateral synchrony (SBS). The mechanism of SBS is considerably more complex than a simple triggering of generalized spike-wave complexes from a single cortical focus.

Limbic encephalitis (LE) is characterized by a subacute onset of episodic memory impairment, disorientation, agitation, seizures and histological evidence of mesial temporal lobe inflammation. It is usually considered to have a paraneoplastic origin and is associated with specific autoantibodies in various cancers [1]. The non-paraneoplastic form has similar clinical and neuroradiological presentation. In these cases antibodies usually target voltage-gated potassium channels (VGKC) or glutamic acid decarboxylase and are, therefore, accepted as immunotherapyresponsive syndromes. Potassium channel antibodies were reported in two patients with reversible LE for the first time in 2001 [2]. Very recently, however, Dalmau et al. showed that the autoantigen associated with LE previously attributed to VGKC is, in fact, leucine-rich, glioma inactivated 1 (LGI1), a neuronal secreted protein [3]. Since this is a relatively rare condition, there have been very few publications on the clinical findings, treatment and follow-up results in this group of patients [3–6] and the optimum treatment regimen is still unknown. In this case report we present a young male patient who did not respond to plasma exchange therapy, but improved rapidly and significantly after IV pulse steroid infusion. A 31-year-old man was admitted for confusional state that had started 1 month ago. He had fever, vomiting and headache at the onset. His past medical history was unremarkable. Physical and neurologic examinations were normal apart from confusion and short-term memory deficits. Cranial MRI revealed hyperintense signals in bilateral hippocampi and the amygdaloid nuclei with little contrast enhancement (Fig. 1). At the onset of his complaints he was admitted to another hospital where he was diagnosed with herpes encephalitis and was given acyclovir without any benefit. In our clinic, blood studies including complete blood count, liver and renal function tests, tumor markers and paraneoplastic antibody analysis (anti-Hu, -Yo, -Ri, -Ma, -CV2) were all normal. Serum electrolyte analysis revealed mild hyponatremia (132 mEq/L). Cerebrospinal fluid (CSF) protein and glucose levels were normal. Analysis of CSF cytology showed increased mononuclear cells. Herpes PCR was negative. Thorax– abdomen CT scans, scrotal-pelvic ultrasonography and whole body fluorodeoxyglucose-positron emission tomography were negative for malignancy. He had two temporal lobe seizures during his follow up. Electroencephalography revealed bilateral generalized mild slow wave paroxysms, without epileptiform discharges. The patient was put on oxcarbazepine (OXC), and did not have any further seizures. In order to investigate the presence of nonparaneoplastic LE, serum and CSF samples were sent to Dr. J. Dalmau’s laboratory where antibodies for NMDA receptors and VGKC antibodies were studied. Meanwhile, the patient had six exchanges of 1.5 plasma volumes on alternate days. We could not give intravenous immunoglobulin (IVIg) because his IgA levels were very low. No clinical response was obtained in the first week after treatment with plasmapheresis and the patient was discharged from hospital. One month later we were informed that he had antibodies against LGI1. The patient was hospitalized again for IV pulse steroid treatment. At that time, his orientation in time and space had partially improved; however, he still had severe memory deficits. He had difficulty in remembering some recent events. After the B. Kaymakamzade T. Kansu E. Tan N. Dericioglu (&) Department of Neurology, Faculty of Medicine, Hacettepe University, Ankara, Turkey e-mail: noroloji2011@gmail.com

Summary Video-EEG monitoring is the ultimate way to diagnose non-convulsive status epilepticus (NCSE) in intensive care units (ICU). Usually EEG recordings are evaluated once a day by an electrophysiologist, which may lead to delay in diagnosis. Digital EEG trend analysis methods like amplitude integrated EEG (aEEG) and density spectral array (DSA) have been developed to facilitate recognition of seizures. In this study, we aimed to investigate the diagnostic utility of these methods by non-expert physicians and ICU nurses for NCSE identification in an adult neurological ICU. Ten patients with NCSE and ten control patients without seizures were included in the study. The raw EEG recordings of all subjects were converted to both aEEG and DSA and displayed simultaneously without conventional EEG. After training for seizure recognition with both methods, two physicians and two nurses analyzed the visual displays individually, and marked seizure timings. Their results were compared with those of a study epileptologist. Participants analyzed 615h of EEG data with 700 seizures. Overall, 63% of the seizures were recognized by all, 15.6% by three, 11.6% by two, 8.3% by one rater and only 1.5% were missed by all of them (sensitivity was 88–99%, and specificity was 89–95% when the ratings were assessed as 1-h epochs). False positive rates were 1 per 2h in the study and 1 per 6h in the control groups. Interrater agreement was high ( κ =0.79–0.81). Bilateral independent seizures and ictal recordings with lower amplitude and shorter duration were more likely to be missed. There was no difference in performance between the rating of physicians and nurses. Our study demonstrates that bedside nurses, ICU fellows and residents can achieve acceptable level of accuracy for seizure identification using the digital EEG trend analysis methods following brief training. This may help earlier notification of the electrophysiologist who is not always available in ICUs.

Phenylketonuria (PKU) is one of the most common inherited metabolic disorders, which is characterized by excessive accumulation of phenylalanine (Phe) in tissues. Generalized seizures occur in 25% of the patients. Little is known about seizures and their treatment in adult PKU patients, and information with newer antiepileptic drugs is lacking. Here we report an adult patient who developed generalized seizures later in life, despite strict dietary control, and whose seizures were aggravated by levetiracetam (LEV). Convulsions ceased after discontinuation of LEV and the patient has been seizure free on topiramate 125 mg/day.

In dacrystic seizures lacrimation has previously been reported as an ictal autonomic event accompanying emotional and somatic signs. However, it has not been observed as an isolated event in autonomic seizures. We report a patient experiencing complex partial seizures characterized by lacrimation as the initial and most prominent ictal event, with no sign of crying. The patient’s ictal electroencephalogram (EEG) suggested a focus in the right hemisphere, along with interictal single-photon emission computed tomographic (SPECT) and positron emission tomographic (PET) studies demonstrating hypoperfusion-hypometabolism in the right temporoparietal lobe. Magnetic resonance imaging (MRI) did not show any structural abnormalities. Involvement of the temporal lobe has been suggested for dacrystic seizures, and both dominant and nondominant hemispheric foci were detected in such cases. Therefore, partially in concordance with previous reports, our case may point to neurobiologic interaction, between the temporoparietal region and the hypothalamus, for mediating lacrimation.

The availability of video electroencephalography monitoring (VEEGM) in neurological intensive care units has allowed the recognition and treatment of nonconvulsive status epilepticus (NCSE). However, little is known about characteristics, management, and outcomes in patients with NCSE in developing countries. We retrospectively reviewed the video-EEG reports of 120 patients who were monitored from November 2009 to March 2013. Indications for video-EEG were mostly unexplained alterations of consciousness or witnessed convulsive seizures. We identified the clinical characteristics, treatment regimes, and outcomes of patients with NCSE and tried to determine which parameters were associated with prognosis. NCSE was detected in 12/120 (10%) patients (3 females, 9 males; age 24-86 years). Admission diagnoses were: stroke (3), epilepsy (3), autoimmune limbic encephalitis (3), herpes encephalitis (1), presumed encephalitis-cardiac arrest (1), and malignancy (1). Eight patients had witnessed convulsive seizures before video-EEG. Interictal periodic epileptiform discharges were detected in 9 patients. In one-third of patients, ≥2 EEG recordings were required to capture seizures. In addition to anticonvulsants, 3 patients received immunosuppressive therapy, while intravenous anesthetics were given to 7 patients. Four patients (33.3%; 1 female, 3 males; age 51-67 years; etiology: stroke, autoimmune encephalitis, encephalitis-cardiac arrest, and malignancy; Glasgow coma scale (GCS) score

erebral autosomal dominant arteriopathy with subcorti-cal infarcts and leukoencephalopathy (CADASIL) is arare hereditary disease that presents with various neurologicaland/or psychiatric symptoms, such as stroke, migraine with aura,cognitive changes, mood disorders, and dementia. Mutations inthe Notch3 gene have been documented in most of the patients.Clinically, the most useful diagnostic tool is cranial magneticresonance (MR) imaging, which demonstrates widespread sub-cortical leukoencephalopathy associated with hyperintensities inbasal ganglia, thalamus, and brainstem. Psychiatric symptomsare seen in 20%–41% of the patients, and are rarely the initialpresenting symptom. Here we present two siblings in their 40swith prominent psychiatric complaints. Clinical diagnosis ofCADASIL was made years later, after a careful personal andfamily history combined with typical MR imaging findings.Both patients had a missense mutation in exon 4 of Notch3. Ifpatients with various psychiatric symptoms have a history ofheadache, stroke-like episodes, or cognitive changes, and a pos-itive family history of similar complaints, they should undergocranial MR imaging. In case the imaging demonstrates unex-plained leukoencephalopathy, patients should be investigated forCADASIL.Cerebral autosomal dominant arteriopathy with subcor-tical infarcts and leukoencephalopathy (CADASIL) is a rarehereditary brain disease that in most patients occurs due tomutations of the notch 3 gene on chromosome 19q12.

Central neurogenic hyperventilation (CNH) is a rare condition that is generally associated with infiltrative tumors of the brainstem. Respiratory dysfunction, particularly central hypoventilation, is common in anti-N-methyl D-aspartate (NMDA) receptor encephalitis. CNH, to the best of our knowledge, has not been described previously in this disease. A 24-year-old woman was diagnosed with anti-NMDA receptor encephalitis secondary to ovarian teratoma. In addition to the typical symptoms of the disease, recurrent CNH episodes were observed during the course of the illness, which subsided with midazolam and propofol infusion. Supportive and disease-specific treatments, including oopherectomy, plasmapheresis and intravenous immunoglobulin, provided excellent recovery. These observations suggest that NMDA receptors may play a role in the pathophysiology of CNH.

Pallister-Hall syndrome (PHS) is a very rare syndrome characterized by hypothalamic hamartoma (HH), polydactyly, panhypopituitarism, imperforate anus and other visceral anomalies. Contrary to patients with isolated HH, neurological dysfunction and precocious puberty are uncommon and seizures are usually well controlled with anticonvulsant medication. Therefore, conservative management of HH is advised. To the best of our knowledge, seven cases of PHS with surgical resection of the HH have so far been reported. Five patients were either seizure-free or had >90% seizure reduction postoperatively. Here, we present a case of PHS of a patient who also underwent transcallosal endoscopic resection of the HH with a subsequent 70% reduction in seizure frequency.

Concordance of EEG findings and MRI is best correlated with favored surgical outcome in patients with unilateral mesial temporal sclerosis (MTS). If there is no evidence for unilateral focus with scalp EEG, invasive recordings are undertaken. In this report we describe the investigation process for epilepsy surgery in patients with unilateral MTS and contralateral ictal scalp EEG findings.The data of all adult patients who had undergone videoEEG recording with subdural and/or depth electrodes at our center in almost 7.5 years, were reviewed. Four patients with unilateral MTS and contralateral ictal onset on scalp EEG were included. Their invasive EEG recordings and surgical outcomes were examined.Four patients met the inclusion criteria. Invasive recordings demonstrated ictal onset in the mesial temporal lobe ipsilateral to MRI findings. In one patient we have also proven the false lateralization of scalp EEG simultaneously during the recordings with depth electrodes. All operated cases are seizure free during follow-up.Before the decision of epilepsy surgery we have to identify the semiology and ictal EEG findings in patients with unilateral MTS and concordant IEDs. Bilateral depth recordings must be considered to show the ipsilateral hippocampal epileptogenic focus.

Mesial temporal lobe epilepsy syndrome (MTLES) is the most common surgically remediable epileptic syndrome in adults. Its diagnosis is easy when clinical history is supported by positive laboratory findings. However, routine EEG may not be informative in some patients, thus delaying accurate diagnosis. Therefore, we sought to determine how often routine EEGs displayed epileptiform discharges pre-operatively in a group of patients who underwent surgery for MTLES. Retrospectively, we reviewed the outpatient EEG records of MTLES patients who underwent surgery at our epilepsy center between 1997–2008 and had at least one routine pre-operative EEG recording in our outpatient laboratory. For each patient, serial EEGs were coded as normal, displaying nonspecific abnormalities or lateralized and localized interictal epileptiform discharges. Seventy patients were included in the study. We reviewed 230 EEGs. In almost half of the patients (47.1%) all EEGs were normal or revealed nonspecific findings. In patients who had >1 EEG, almost 3 EEGs had to be recorded to detect the epileptiform discharges for the first time and 6.23 years were needed to accomplish this. Sleep deprivation considerably increased the yield. In summary, patients who have a clinical history suggesting MTLES may need at least 3 routine scalp EEG recordings (with at least one of them after sleep deprivation) to detect epileptiform abnormalities and it may take much time. Therefore, a single prolonged outpatient video-EEG monitoring or an overnight inpatient monitoring might be reasonable alternatives to serial EEGs.

Various hippocampal malformations have been described in the context of widespread cortical malformations in patients with epilepsy. Isolated hippocampal malformations however are very rarely identified on MR imaging studies. Little is known about the epileptogenicity of these malformations and their pathologic appearance. We present a case with severe bilateral hippocampal malformations who underwent right temporal lobectomy due to intractable temporal lobe epilepsy. Postoperative examination of the resected hippocampus revealed abnormal shape of the dentate gyrus and an atypical convolution of the CA1 pyramidal cell-subicular layers. After surgery, the patient has been seizure free.

The syndrome of mesial temporal lobe epilepsy (MTLE) is a well-defined clinical entity that responds to surgical treatment in a considerable number of patients. Although it has been subjected to intensive clinical research, few investigators have published the ictal scalp EEG findings and looked for specific features that might predict postoperative outcome. This study was designed to examine ictal scalp EEG characteristics in detail, in a group of patients with pathologically confirmed hippocampal sclerosis (HS). Patients who underwent long-term video-EEG monitoring at our center during a 3-year period and were diagnosed to have MTLE and pathologically proven HS were included in this retrospective study. All ictal scalp EEGs were investigated in a common reference montage, paying attention to the localization, morphology and frequency of ictal discharges that were accepted to represent a specific phase if the findings were sustained for at least 3 seconds. Any significant change in localization, morphology or frequency of discharges was said to represent a different phase. The ictal EEG patterns in different phases were later compared among seizures of different patients. In addition, the ictal EEG characteristics of the patients in Group I (Engel's classification) were compared with the ictal EEG findings in patients who were included in another group. All the patients have been followed for more than 5 years. Seventy-one ictal EEGs were investigated in 25 adult patients (11 M, 14 F). Onset patterns were lateralized in 81.7% and localized in 76% of the seizures. Thirteen different patterns of onset were detected, the most common of which was the cessation of interictal discharges (35.2%). The most common ictal pattern following the initial changes was ipsilateral temporal rhythmic theta-delta activity (85.2%) that occurred on the average 13.4 seconds after onset. Nonlocalized/lateralized seizure onset of all the seizures or bilateral independent onset was present in 75% of the patients in Groups II-III, whereas this ratio was 14.3% in the patients in Group I (p=0.031). In conclusion, ictal scalp EEG in MTLE allows correct lateralization and localization in most of the seizures. Onset patterns may vary considerably; however, a later significant pattern consisting of rhythmic ipsilateral temporal build-up develops in the majority of seizures. Some ictal EEG characteristics may be related to post-operative outcome.

Kabuki syndrome is a rare multiple congenital anomaly disorder. Although mental retardation is one of the main features, various neurological symptoms such as hypotonia and seizures can occur. Here we report on a 18-year-old Turkish male patient who was diagnosed previously as Kabuki syndrome. Molecular genetic analysis showed a novel de novo heterozygous mutation (c.12964C > T [p.Gln4322*] ) in the MLL2 gene, that leads to the synthesis of a truncated protein. The aim of the present report is to increase the awareness of Kabuki Syndrome among adult neurologists and to present a previously unreported non-sense mutation in the MLL2 gene.

Creutzfeldt-Jakob disease is the most common disorder among the very rare human transmissible subacute spongiform encephalopathies. Sporadic, familial or iatrogenic forms of the disease can be seen. The common presentations of the disease include rapidly progressive cognitive decline, behavioral changes, visual disturbances and cerebellar dysfunction. Hyperparathyroidism and toxicity of lithium and bismuth have been reported to induce similar symptoms and EEG findings, leading to a Creutzfeldt-Jakob like syndrome. We report a very rare case of Creutzfeldt-Jakob disease presenting with hyperparathyroidism and generalized tonic status epilepticus.

Purpose: The determination of prognostic factors is important for predicting outcome after epilepsy surgery. We investigated the factors related to surgical outcome within a homogeneous group of patients suffering from pathologically proven mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS), and compared Engel's outcome classification system with the latest one proposed by the ILAE. Method: We included 109 patients with MTLE-HS who were followed-up for at least 1 year after epilepsy surgery. A retrospective chart review was performed to extract patients' demographic details, and potential pre- postoperative risk factors. Outcome of surgery was defined by the Engel's and ILAE classifications. In addition, the course of prognosis was determined according to the changes in ILAE classifications on an annual basis. Univariate and multivariate logistic regression analyses were used for the latest available outcomes and two different courses of prognosis. Results: The average duration of follow up was 4.78 +/- 2.55 years in the 109 patients with MTLE-HS. The univariate and multiple logistic regression analyses showed that the occurrence of seizures during the first month after surgery was a significant risk factor for a poor outcome. A history of trauma was also significant for patients with late recurrence of postsurgical seizures after at least 1-year seizure remission. Conclusion: The occurrence of seizures during the first month after surgery is a significant prognostic factor in patients with MTLE-HS. Ignoring early postoperative seizures in classification systems may result in difficulty in identifying the course of epilepsy after surgery. (C) 2008 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

The MURCS association is a rare, nonrandom association of müllerian duct aplasia, renal aplasia and cervicothoracic somite dysplasia. The etiology is unknown. Although it is usually a sporadic disorder, familial cases with uterovaginal anomalies have been reported. Occasionally, it may be accompanied by abnormalities involving various other organs or systems. Malformations related to the central nervous system are very rare and the presence of seizures has not been reported previously. We present a 26-year-old female with MURCS association who had late onset, drug resistant partial seizures presumably originating in the frontal lobe.

A 64-year-old woman presented with a 6-month history of intermittent episodes of ataxia and dysarthria. She had been on metronidazole 1,500 mg daily for ulcerative colitis for 10 months. Cranial MRI 3 months before admission revealed hyperintensity in corpus callosum and bilateral dentate nuclei (figure 1, A–C). MRI on admission revealed cystic necrotic degeneration (figure 1, D and E) and cytotoxic edema (figure 2) in corpus callosum, whereas dentate nuclei appeared normal (figure 1F). The cumulative metronidazole dose was 450 g, relatively high,1 which could cause white matter injury by various mechanisms (appendix e-1 on the Neurology® Web site at www.neurology.org). Although early MRI findings are typical for metronidazole toxicity,2 delayed cystic necrosis is unusual.