Typical EEG Findings Research Articles

Hashimoto's encephalitis - A case report

A 50 year-old woman with a background of focal epilepsy presented to Emergency Department (ED) in convulsive status epilepticus.After 1 year seizure-free on levetiracetam,she initially presented with headache, tremor, fatigue and general malaise. She was discharged, then re-presented to ED several days later in status epilepticus. Two doses of diazepam administered pre-hospital failed to terminate the seizure. She was loaded with phenytoin,intubated and ventilated and commenced on a midazolam infusion. She spent 7 days in neuro-intensive care and was discharged home on levetiracetam 1.5 g twice daily and phyentoin 250 mg twice daily.One month later, she re-presented to ED with 4 days of worsening tremors and reduced mobility. Physical examination revealed multifocal myoclonus. She had experienced significant cognitive decline and neuropsychiatric symptoms including severe anxiety, delusions and paranoia.InvestigationsImagingInitial MRI head with gadolinium showed marked symmetric abnormality within the white matter of both hemispheres extending into subcorticol regions, most predominant at the vertex and involving U fibres. The neuro-radiologist commented that the differential for these changes was broad, including an encephalitis, PML, meningitis or a leukoencephalopathy.Repeat MRI head 1 month later showed complete resolution of the white matter changes leaving no sequelae.Blood testsHer past medical history included subclinical hypothyroidism. Thyroid function tests showed an elevated TSH of 21.33 and thyroid peroxidase(TPO) antibody level of 188.0 IU/mL, with FT4 levels within normal range.Extensive relevant blood screen, including the following was all negative• NMDA, glycine receptor and anti-cardiolipin antibody• Connective tissue screen, anti-MPO and anti-PR3• Serum electrophoresis• Paraneoplastic antibody screen including Cerebellum IIF, MA2, MA1, amphiphysin, CV2(CRMP5), Anti-Ri (ANNA 2) Ab, Anti Yo Ab and Anti Hu Ab• HIV and syphilis serology• JC virus• Plasma amino acid, urine organic acid and ammoniaLumbar punctureCSF WCC 1/mm3CSF protein- 816 mg/LCSF viral PCR (including JC virus) and oligoclonal bands negativeEEG- normal background activity with no clear focal or diagnostic epileptiform abnormalities.Treatment and follow-upThe clinical picture, elevated TPO antibodies and absence of other relevant investigation abnormality raised Hashimoto's encephalitis as a potential unifying diagnosis. She had a good response to inpatient treatment with steroids, her neuropsychiatric features resolved and she has remained seizure-free since discharge. She remains under neurology outpatient follow-up.Discussion:It can be challenging to make a diagnosis of Hashimoto encephalitis, and other potential causes such as infection, metabolic or paraneoplastic processes must be rigorously ruled out (1). In this case, high titres of TPO alongside the clinical presentation with recurrent seizures and cognitive decline were key in making the diagnosis (2,3). Due to an overlap of features, including myoclonus and rapid cognitive decline, it was important to consider Creutzfeldt-Jakob disease (CJD) in the differential. We ruled out CJD due to the rapid response to steroid therapy and lack of typical EEG and MRI findings (4). Overall, due to low prevalence and non-specific MRI and EEG features, this diagnosis can be difficult to make and requires thorough systemic clinical assessment.

Transient epileptic amnesia-A rare phenomenon in temporal lobe epilepsies

Transient epileptic amnesia (TEA) is arare phenomenon in temporal lobe epilepsy that is often unrecognized or misdiagnosed as transient global amnesia (TGA). It is postulated that TEA is due to both ictal and postictal disturbances. Response to antiseizure medication underlines its epileptic nature. In view of the increasing incidence of new-onset epilepsies in old age, an increase in TEA can be expected in the future. Analysis of TEA features in amonocentric case series. Asearch in our electronic patient data base yielded 10patients with TEA out of 7899patients over aperiod of 8years. Clinical and paraclinical features as well as findings of additional examinations were retrospectively collected. Data are given as mean ± SD. All 10patients were diagnosed with temporal lobe epilepsy. The mean age at manifestation of TEA was 59.1 ± 6.7years, the diagnosis was made with adelay of 21.9 ± 26.3months. The TEA lasted on average 56 ± 37 min, and 16 ± 9.9 TEA episodes per year were reported by the patients; out of the 10patients 6 reported that TEA usually occurred upon awakening. In 9of 10patients, there was evidence of typical seizure symptoms or other semiological elements during TEA. Interictal neuropsychological disturbances of temporal functions were seen in 8of 10patients and evidence of depressive disorder in 6of 10patients. Video EEG recordings revealed epileptiform activity during sleep in 4patients over the left and in 2patients over both temporal regions. In 3patients, magnetic resonance imaging displayed typical alterations of the temporomesial structures (in 2patients on the left and in 1the right side). Antiseizure medication improved seizure control in 7of 10patients (seizure freedom in 6patients), 3patients were lost to follow-up. TEA is rare, occurs in older adults and is correctly diagnosed after about 2years. Thorough assessment of additional symptoms and circumstances, the recurrent occurrence as well as typical EEG and imaging findings of temporal lobe epilepsy enables the distinction between TEA and TGA.

Genetic Epilepsy Syndromes.

This article reviews the clinical features, typical EEG findings, treatment, prognosis, and underlying molecular etiologies of the more common genetic epilepsy syndromes. Genetic generalized epilepsy, self-limited focal epilepsy of childhood, self-limited neonatal and infantile epilepsy, select developmental and epileptic encephalopathies, progressive myoclonus epilepsies, sleep-related hypermotor epilepsy, photosensitive occipital lobe epilepsy, and focal epilepsy with auditory features are discussed. Also reviewed are two familial epilepsy syndromes: genetic epilepsy with febrile seizures plus and familial focal epilepsy with variable foci. Recent years have seen considerable advances in our understanding of the genetic factors underlying genetic epilepsy syndromes. New therapies are emerging for some of these conditions; in some cases, these precision medicine approaches may dramatically improve the prognosis. Many recognizable genetic epilepsy syndromes exist, the identification of which is a crucial skill for neurologists, particularly those who work with children. Proper diagnosis of the electroclinical syndrome allows for appropriate treatment choices and counseling regarding prognosis and possible comorbidities.

Neurophysiological Findings in Neuronal Ceroid Lipofuscinoses.

Neuronal ceroid lipofuscinoses (NCLs) are a heterogeneous group of neurodegenerative diseases, characterized by progressive cerebral atrophy due to lysosomal storage disorder. Common clinical features include epileptic seizures, progressive cognitive and motor decline, and visual failure, which occur over different time courses according to subtypes. During the latest years, many advances have been done in the field of targeted treatments, and in the next future, gene therapies and enzyme replacement treatments may be available for several NCL variants. Considering that there is rapid disease progression in NCLs, an early diagnosis is crucial, and neurophysiological features might have a key role for this purpose. Across the different subtypes of NCLs, electroencephalogram (EEG) is characterized by a progressive deterioration of cerebral activity with slowing of background activity and disappearance of spindles during sleep. Some types of heterogeneous abnormalities, diffuse or focal, prevalent over temporal and occipital regions, are described in many NCL variants. Photoparoxysmal response to low-frequency intermittent photic stimulation (IPS) is a typical EEG finding, mostly described in CLN2, CLN5, and CLN6 diseases. Visual evoked potentials (VEPs) allow to monitor the visual functions, and the lack of response at electroretinogram (ERG) reflects retinal neurodegeneration. Taken together, EEG, VEPs, and ERG may represent essential tools toward an early diagnosis of NCLs.

Epilepsy and Electroencephalogram Characteristics in Children with Neurofibromatosis Type 1, What We Have Learned from a Tertiary Center Five Years' Experience.

Introduction: Neurofibromatosis type 1(NF-1) is the commonest neurocutaneous phacomatosis in children. Epilepsy is an infrequent comorbidity. Reports of seizure and Electroencephalogram (EEG) characteristics in children are sparse. Methods: A retrospective review was performed on patients with NF-1 seen between 2016–2020. Patients with co-existing epilepsy were identified. Demographic, clinical, radiological and neurophysiological data were reviewed and analyzed. Results: Out of 118 children with NF1, 16 had epilepsy. 11 patients had focal onset seizures, whereas 5 had generalized onset seizures. Most patients had easy seizure control. Focal epileptiform discharges were the most prevalent EEG abnormality. There was no significant correlation between seizure patterns and presence of intracranial tumors. Conclusion: Epilepsy is a relatively uncommon in pediatric NF-1. Seizures are often of focal semiology and likely to be easily controlled. Focal and multifocal spike epileptiform discharges are the typical interictal EEG findings. Correlation of clinical and EEG findings with intracranial lesions is poor.

Evaluation of Eeg Pattern and Seizure Types of Genetic Dysmorphic Syndromes: Report of 50 Patients and Review of the Literature

Many chromosomal anomalies manifest with epilepsy. Only few typical EEG and seizure type have been identified in genetic syndromes. Identification of typical seizure and EEG findings of certain genetic syndromes may serve as a guide for genetic analysis. This study aims to find typical EEG paterns of spesific genetic syndromes. The study enrolled 50 patients aged 0-16 years with a diagnosis of epilepsy and genetic syndrome in between 2014-2017 at the Dr. Behçet UZ Children’s Hospital Pediatric Neurology and Medical Genetics departments. Patients' characteristics and dysmorphic features were retrieved from Medical Genetic outpatient clinic patient files, while seizure type, epileptic syndromic classification, EEG and brain MRI findings, age at onset and frequency of seizure were determined from pediatric neurology follow-ups. Fifty patients (29 girls) with a mean age of 6.52 ±3.67 years (max=16, min=1) were enrolled. Twenty-two patients had microdeletion-duplication (44%), 12 had chromosomal anomalies (24%) and 16 had monogenic syndrome (32%). Pathology was present in the EEGs of 40 patients (80%). Focal epileptic disorder was determined in 28 subjects (56%), epileptic encephalopathy in 7 (14%), and generalized epileptic disorder in 5 (10%) Identification of seizure type and EEG pattern specific to each genetic dysmorphic syndrome may give clues to clinicians in recognizing these syndromes. However, in order to detect other specific EEG patterns, there is a need for multicentre studies with more patients.

Triphasic Waves in EEG, an Atypical Finding in a Subacute Sclerosing Panencephalitis (SSPE) Adult Patient

Subacute sclerosing panencephalitis (SSPE) is a known rare cause of progressive myoclonic encephalitis of childhood and young adults due to a persistent measles virus infection. It is usually characterised by myoclonic jerks, cognitive decline , typical EEG findings that clinches its diagnosis and further supported by a high CSF and serum measles antibody titre.

Electroencephalogram findings in patients with posterior cortical atrophy

Aim of the studyThe aim of this study is to evaluate standard scalp EEG findings in patients with posterior cortical atrophy (PCA), an atypical variant of Alzheimer's disease (AD). Clinical rationalePCA is a topographically selective variant of AD. Patients with typical AD have an increased likelihood of seizures, which may negatively impact overall functional performance and cognition. It is currently unknown what the typical EEG findings are for patients with PCA. Materials and methodsA retrospective chart review was performed on patients identified either with autopsy confirmed (n=13) or clinically (n=126) as PCA. Results139 patients were included though only 23 (16.5%) had undergone EEG recording. The EEG was normal in 6 (26%), while an abnormal EEG was present in 17 (74%). Interictal epileptic discharges (IEDs) were found in 2 of the 23 patients (9%). ConclusionsThis study of limited sample size suggests that there may be an increased predilection to find IEDs within PCA when compared to typical AD. Larger cohorts are required to determine frequency of abnormal EEGs in PCA, roles of AEDs in therapy, and in the selection of preferred AED. Clinical implicationsPatients with PCA would potentially benefit from an EEG for assessment of IEDs which may provide the clinician with a therapeutic opportunity.

F17. EEG findings and seizure patterns in three infants with type 1 citrullinemia

Citrullinemia is a urea cycle disorder which leads to toxic accumulation of ammonia and other metabolites. While it is widely accepted that citrullinemia is associated with seizures, there is no literature describing the typical EEG findings. Furthermore, there are no data available on long-term seizure outcomes. We report herein EEG findings and clinical courses in 3 patients with type 1 citrullinemia. We reviewed EEG records to identify patients with type 1 citrullinemia who had EEG studies at the Montefiore Medical Center. The EEG findings, clinical history and course in these patients were reviewed. We identified 3 patients with type 1 citrullinemia with infantile onset of symptoms who had EEG studies. The initial EEG studies were done between 4 days and 3 weeks of life; all patients had subsequent follow-up EEGs. Patient 1 presented with abnormal movements and severe hyperammonemia (921 μmol/L) at 3 weeks of life. EEG demonstrated independent left and right central electrographic and electroclinical seizures. She received emergent dialysis, lorazepam, levetiracetam, sodium phenylacetate and sodium benzoate. She had no further seizures, and at last follow-up at age 9 months, she was seizure-free off all anti-epileptic drugs (AEDs), on dietary modification and treatment with glycerol phenylbutyrate. Patient 2 presented on day 4 of life with lethargy and poor feeding. She suffered a cardiac arrest and was found to have severe hyperammonemia (>2000 μmol/L). EEG demonstrated many independent left and right temporal electrographic seizures requiring phenobarbital boluses and midazolam infusion. She had no further seizures and was tapered off of phenobarbital at 6 months of age. She remains on dietary modification and treatment with arginine and sodium phenylbutyrate. Patient 3 presented with neonatal seizures. An EEG performed on day 7 of life demonstrated independent left and right central electrographic seizures. She was initially treated with levetiracetam, phenobarbital, and hemodialysis. She is now 10 years old and has been seizure-free for 3 years. She is currently on levetiracetam, dietary modification and treatment with arginine and sodium phenylbutyrate. Our 3 patients with type 1 citrullinemia all presented in the neonatal period with independent electrographic seizures in homologous regions in both hemispheres. One patient also had bilateral independent electroclinical seizures. Our findings suggest that bilateral independent electrographic seizures are a characteristic EEG finding in neonatal-onset type 1 citrullinemia. Two of our patients had no further seizures after the initial presentation and are now off AEDs. The third patient has been seizure-free for 3 years on levetiracetam as the only AED. These findings suggest that patients with citrullinemia may not have further seizures once their metabolic state is controlled and, therefore, may not require long-term AEDs.

Evaluation of Eeg Pattern and Seizure Types of Genetic Dysmorphic Syndromes: Report of 50 Patients and Review of the Literature

Many chromosomal anomalies manifest with epilepsy. Only few typical EEG and seizure type have been identified in genetic syndromes. Identification of typical seizure and EEG findings of certain genetic syndromes may serve as a guide for genetic analysis. This study aims to find typical EEG paterns of spesific genetic syndromes. The study enrolled 50 patients aged 0-16 years with a diagnosis of epilepsy and genetic syndrome in between 2014-2017 at the Dr. Behçet UZ Children’s Hospital Pediatric Neurology and Medical Genetics departments. Patients' characteristics and dysmorphic features were retrieved from Medical Genetic outpatient clinic patient files, while seizure type, epileptic syndromic classification, EEG and brain MRI findings, age at onset and frequency of seizure were determined from pediatric neurology follow-ups. Fifty patients (29 girls) with a mean age of 6.52 ±3.67 years (max=16, min=1) were enrolled. Twenty-two patients had microdeletion-duplication (44%), 12 had chromosomal anomalies (24%) and 16 had monogenic syndrome (32%). Pathology was present in the EEGs of 40 patients (80%). Focal epileptic disorder was determined in 28 subjects (56%), epileptic encephalopathy in 7 (14%), and generalized epileptic disorder in 5 (10%) Identification of seizure type and EEG pattern specific to each genetic dysmorphic syndrome may give clues to clinicians in recognizing these syndromes. However, in order to detect other specific EEG patterns, there is a need for multicentre studies with more patients.

Influence of epileptic activity during sleep on cognitive performance in benign childhood epilepsy with centrotemporal spikes.

Benign childhood epilepsy with centrotemporal spikes is benign childhood epilepsy, presenting between 4 and 10 years of age, characterized by typical clinical and EEG findings. Despite excellent prognosis, there are reports of mild cognitive, language, fine motor and behavioral difficulties. In its atypical form - electrical status epilepticus during slow wave sleep, continuous epileptiform activity during sleep lead to severe neurocognitive deterioration. Our objective was to investigate the influence of abundant sleep epileptiform activity, not fulfilling the criteria for electrical status epilepticus during Slow Wave Sleep, discovered randomly in children without overt intellectual impairment. We retrospectively reviewed the charts and EEG's of 34 children with benign childhood epilepsy with centrotemporal spikes, who underwent neurocognitive evaluation. The neurocognitive battery included items in the following domains: attention span, memory, language, fine motor and behavior. Patients were divided into two groups according to the spike wave index on sleep EEG, with a cut-off point of 50%. The groups were compared regarding to neurocognitive performance. Children with epileptiform activity of more than 50%, were diagnosed at a significantly younger age (5.13±1.94 years vs. 7.17±2.45, p=0.014 T test), had less controlled seizures and received more antiepileptic drugs. However, there was no difference in neurocognitive performance, except in fine motor tasks (Pegboard), where children with more abundant activity were scored lower (-0.79±0.96 vs. 0.20±1.05, p=0.011, T test). Our study did not show negative cognitive effect of abundant epileptiform activity discovered randomly in children with benign childhood epilepsy with centrotemporal spikes, warranting aggressive treatment.

The diagnostic dilemma of non-convulsive status epilepticus in sporadic Creutzfeldt-Jakob disease

SummaryIntroduction.The differentiation between the clinical and electroencephalographic changes in nonconvulsive status epilepticus (NCSE) and those in sporadic Creutzfeldt-Jakob disease (sCJD) is a crucial question.Case report.A 77-year old woman was admitted because of fluctuating behavioural chancaseges, adynamia and apraxia since several months for diagnostic. The diagnosis of sCJD was suggested. Subsequently, she had a generalized tonic clonic seizure (GTCS) and the EEG revealed periodic lateralized epileptiform discharges and NCSE was considered.Discussion.The presented case illustrates the dilemma in the differential diagnosis of sCJD and (symptomatic) NCSE in the light of the recently published new Salzburg consensus criteria and unified EEG terminology. Concerning these criteria, the patient showed after an initial generalized seizure and substantial clinical improvement after administration of antiepileptic drugs, persisting epileptic discharges and only subtle clinical ictal phenomena during the EEG patterns with typical spatiotemporal evolution as a correlate of a symptomatic NCSE. During the further course of the disease in the presented patient the picture changed into an encephalopathic pattern.Conclusion.EEG criteria for the diagnosis of NCSE are complex. In our case the EEG resembled the pattern of NCSE in the postictal phase of a GTCS according to a classification of NCSE in use at this time. After initial responsiveness to antiepileptic medication the patient lost responsiveness to therapy displaying the typical encephalopathic EEG findings in sCJD. These findings may support the hypothesis of initial NCSE and transformation into prion protein induced encephalopathic EEG and demonstrated clinical usefulness of the Salzburg consensus criteria for NCSE.

Role of Electroencephalography in the Diagnosis and Treatment of Neuropsychiatric Border Zone Syndromes.

Border zone disorders involve neurological disorders with psychiatric symptoms and signs as well as psychiatric disorders with soft neurological features. This becomes a cause for great diagnostic and therapeutic concerns. We, in this paper, analyzed some of the imitators such as epilepsy, dementia, some forms of encephalitis, and pure psychiatric diseases which produce problems in decision making due to soft neurological features and the utility of electroencephalography (EEG) as a simple diagnostic tool in differentiating some of these conditions from each other as well as the therapeutic role of EEG in some of these disorders. We retrospectively took index cases which produced problems for us in decision making in the last 5 years and correlated with the final diagnosis, EEG parameters as well as literature available by PubMed search using specific key words based on the conditions identified. EEG can be normal in organic diseases and abnormal in psychiatric diseases. Typical EEG findings in neuropsychiatric syndromes point to specific diagnosis. Soft EEG changes are common in psychiatric disorders and do not indicate organicity. EEG can be used to assess efficacy and toxicity of therapeutic agents in psychiatry. Biofeedback-based training to keep the brain in particular rhythm is of use in psychiatric disorders as a pharmaco-sparing agent.

EEG in Gastaut Type-Idiopathic Childhood Occipital Epilepsy.

Gastaut type-idiopathic childhood occipital epilepsy (G-ICOE) is a rare form of idiopathic childhood occipital epilepsy. It is characterized by late-onset, brief, frequent, usually diurnal, elementary visual auras which may be associated with hemi-clonic  or generalized convulsion. Occasionally the clinical presentation can be overlapped with Panayiotopoulos syndrome. Typical EEG findings are occipital paroxysms. The prognosis remains uncertain, but most seizures are often responsive to antiepileptic treatment.

The effect of transcranial direct current stimulation on seizure frequency of patients with mesial temporal lobe epilepsy with hippocampal sclerosis

ObjectivesTranscranial direct current stimulation (tDCS) is a non-invasive and safe method tried in drug-resistant epilepsies, in recent years. Our aim was to evaluate the effect of tDCS in patients diagnosed with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) which is a well-known drug-resistant focal epilepsy syndrome. Patients and methodsTwelve MTLE-HS patients diagnosed with their typical clinical, EEG and MRI findings fulfilling the criteria for drug-resistance as suggested by the ILAE commission were included after Ethics Committee approval and their signed consent. All patients received modulated cathodal stimulation; 2mA for 30min on 3 consecutive days. All patients also received sham stimulation with the same electrode positions; designed as 60s stimulation gradually decreasing in 15s with placement of the electrodes for 30min over the stimulation side. They were followed up by standard seizure diaries and their medical treatment was not changed during the study period. Their seizure frequencies both before and after cathodal tDCS and sham stimulation were compared statistically. Adverse effects were also questioned. ResultsMean age of our study group was 35.42±6.96 (6 males; median: 35.50). The mean seizure frequency was 10.58±9.91 (median=8; min-max=2-30) at the baseline and significantly decreased to 1.67±2.50 (median=0.5; min-max=0-8) after cathodal tDCS application (p=0.003). Ten patients (83.33%) had more than 50% decrease in their seizure frequencies after cathodal tDCS. Two patients (16.67%) also showed positive sham effect. Six patients (50%) were seizure-free in the post-cathodal tDCS period of one month. No adverse effect has been reported except tingling sensation during cathodal stimulation. ConclusionOur small series suggested that cathodal tDCS may be used as an additional treatment option in MTLE-HS. It may be tried in TLE-HS patients waiting for or rejecting epilepsy surgery or even with ineffective surgery results. More studies are needed with large series of patients to investigate the effects of tDCS in drug resistant epilepsies.

P20 – 2476: Atypical and fulminant clinical course subacute sclerosing panencephalitis

Objective Subacute sclerosing panensefalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. Patient usually have behavioral changes, myoclonus, demantia, visual disturbances, and pyramidal and extrapyramidal sign. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demostration of increased antibody titre againist measles in the plasma and cerebrospinal fluid. Neuroimaging has limited role in the early diagnosis of SSPE. Methods 17 year old girl presented with progressively increasing myoclonic jerks, psychiatric manifestations and cognitive decline for two weeks. There was no fever and vomiting. She had measles at age 1 year. Her developmental milestones were normal. On her examination, patient interact poorly with the examiner and was partial oriented to place or time. She had frequent myoclonic jerks involving limbs, face, head, and gait instability. Tone was increased and deep tendon reflexes were exaggerated in all limbs and bilateral plantar reflexes were extensor. Fundus examination showed papilledema and bilateral macular retinitis. Complete blood count and serum chemistries including lactate and ammonia were normal. Serologies for Herpes simplex virus, and mycoplasma were negative. Cerebrospinal fluid (CSF) examination showed no cells, and normal protein (35.7 mg/dL), and glucose (65 mg/dL). Initial MRI was normal but after 20 days showed nonspesific white matter chances on posterior periventricular area. Electroencephalogram (EEG) showed bilateral symmetrical, syncronous periodic high voltage bursts of polyphasic, steretyped delta waves. The Measles Ig G antibody in CSF was 185194 U/ml, and Ig G index was higher than normal value. Results We diagnosed SSPE to based on clinical feature, typical EEG finding, and laboratory result. Conclusion SSPE is still an important medical problem in the developing countries. So SSPE should be considered when a patient admitted with atypical clinical features like loss of consciousness, acute partial-generalized convulsion, visual loss.

NI-37 * INCREASED CEREBRAL AMINO ACID UPTAKE DURING AND AFTER EPILEPTIC DISORDERS MIMICS BRAIN TUMOR IN 18F-FET PET

BACKGROUND: O-(2-[F-18]fluoroethyl)-L-tyrosine (18F-FET) PET has become an important diagnostic tool in addition to standard MRI to delineate cerebral gliomas. However, 18F-FET uptake has also been observed in non-tumoral lesions. METHODS AND RESULTS: We report on abnormal non-tumoral 18F-FET uptake in 6 patients with epileptic disorders: Patients A-C with clinically stable anaplastic astrocytoma over years, Patient D with anaplastic oligodendroglioma with stable residual tumor, Patient E with initial diagnosis of glioblastoma, Patient F with subacute cerebral ischemia. All patients presented initially with repeated epileptic seizures or status epilepticus and typical clinical and EEG findings. Standard MRI (T1 + /-contrast, T2/FLAIR) showed cortical and subcortical contrast enhancement and/or T2/FLAIR signal alterations and 18F-FET PET demonstrated a significantly increased cortical amino acid uptake. 18F-FET uptake and contrast enhancement on MRI resolved completely after cessation of epileptic activity. Patient C suffered from a prolonged postictal state. In that patient, EEG monitoring demonstrated a left hemispheric deceleration without signs of epileptic discharges; additionally, 18F-FDG PET and HMPAO SPECT showed cortical glucose hypometabolism and hypoperfusion, not consistent with a status epilepticus. After 14 weeks, clinical symptoms and the cortical 18F-FET uptake resolved completely. CONCLUSION: Abnormal 18F-FET uptake may occur in patients with epileptic disorders in brain areas not infiltrated by tumor. The reason for this phenomenon is yet unknown.

Neurologists' Awareness and Preparedness on Prion Diseases in Korea

Department of Neurology*, Seoul National University Bundang Hospital, Seongnam; Department of Neurology†, Seoul National University Boramae Hospital, Seoul; Department of Neurology‡, Yonsei University Severance Hospital, Seoul; Department of Social and Preventive Medicine§, Sungkyunkwan University School of Medicine, Suwon, Korea Background: Creutzfeldt-Jakob disease (CJD) is very rare human prion disease. But, neurologists take a key role in diagnosis, surveillance and management of the cases because of its complexity and difficulty in diagnosis of the disease. The aim of this study is to investigate the level of awareness and preparedness of Korean neurologists on this rare disease. Methods: Survey sheets of self-administered questionnaire were given to Korean neurologists who participated in the 31st Annual Meeting of the Koran Neurological Association. Data from 133 respondents were conducted by descriptive analysis. Results: Their answers were as follows: About 62% of neurologists have experienced patients of CJD. Forty-four percent of the patients were confirmed by brain biopsy. Most of neurologists (44%) were not confident to diagnose CJD and the reason why they felt hard to diagnose was due to the variable initial clinical manifestations (45.1%) and the lack of clinical experience (51.9%). Heidenheim variant CJD, proteinase sensitive prionopathy, molecular subtypes of sporadic CJD, diagnostic criteria was not familiar term to Korean neurologists (76.7%, 53.4%, 58.6%, and 62.4% respectively). Opinion for the most useful diagnostic tool was brain MRI (45.1%), CSF 14-3-3 protein (30.1%), typical EEG finding (36.8%) and gene (PRNP) test (42.9%). And they consider none of them are specific for the diagnosis of CJD (89.5%, 73.7%, 83.5%, 91.7%, respectively). Most of the neurologist in this survey answered that the opportunity for education of CJD should be increased (67.7%). Conclusions: Most of neurologists have encountered CJD patients although it is very rare disease. Some of the important and fundamental concepts of CJD were not correctly recognized to Korean neurologists, necessitating a persistent support for updating knowledge and information. Received: February 5, 2013 Revision received: March 28, 2013 Accepted: March 28, 2013

Diagnosing Sporadic Creutzfeldt-Jakob Disease in a Patient with a Suspected Status Epilepticus in the Intensive Care Unit

Objective. Several tests are available in the diagnostics of sporadic Creutzfeldt-Jakob disease (sCJD); however, none of these is conclusive. We review the values of these tests, from an intensive care unit (ICU) perspective. Methods. Case report and review of the literature. Results. A 53-year-old woman initially presenting with psychiatric symptoms developed myoclonus and was admitted 1 month later to the ICU with a suspected nonconvulsive status epilepticus and respiratory insufficiency, probably due to extensive antiepileptic drug therapy. Typical MRI and EEG findings and a positive 14-3-3 protein led to the diagnosis of sCJD. All treatments were terminated, and autopsy confirmed sCJD. Conclusions. Clinical signs combined with MRI, EEG, and 14-3-3 and/or tau protein determination might be sufficient to diagnose or exclude sCJD and may therefore prevent the application of unnecessary diagnostic tests.

単純ヘルペス脳炎に特徴的なCT, 脳波像を呈しs-CRNの上昇した1例

単純ヘルペス脳炎に特徴的なCT, 脳波像を呈しs-CRNの上昇した1例