Focal Slow Waves Research Articles

Electroencephalography can Ubiquitously Delineate the Brain Dysfunction of Neurodegenerative Dementia by Both Visual and Automatic Analysis Methods: A Preliminary Study.

Introduction: The aim was to examine the differences in electroencephalography (EEG) findings by visual and automated quantitative analyses between Alzheimer's disease (AD) and dementia with Lewy bodies (DLB) and Parkinson's disease with dementia (PDD). Methods: EEG data of 20 patients with AD and 24 with DLB/PDD (12 DLB and 12 PDD) were retrospectively analyzed. Based on the awake EEG, the posterior dominant rhythm frequency and proportion of patients who showed intermittent focal and diffuse slow waves (IDS) were visually and automatically compared between the AD and DLB/PDD groups. Results: On visual analysis, patients with DLB/PDD showed a lower PDR frequency than patients with AD. In patients with PDR <8 Hz and occipital slow waves or patients with PDR <8 Hz and IDS, DLB/PDD was highly suspected (PPV 100%) and AD was unlikely (PPV 0%). On automatic analysis, the findings of the PDR were similar to those on visual analysis. Comparisons between visual and automatic analysis showed an overlap in the focal slow wave commonly detected by both methods in 10 of 44 patients, and concordant presence or absence of IDS in 29 of 43 patients. With respect to PDR <8 Hz and the combination of PDR <8 Hz and IDS, PPV and NPV in DLB/PDD and AD were not different between visual and automatic analysis. Conclusions: As the noninvasive, widely available clinical tool of low expense, visual analysis of EEG findings provided highly sufficient information to delineate different brain dysfunction in AD and DLB/PDD, and automatic EEG analysis could support visual analysis especially about PD.

Clinical Features and Electroencephalogram Analysis ofBrain Network Functional Connectivityin Anti-Leucine-RichGlioma-Inactivated1Antibody Encephalitis.

To summarize the clinical manifestations, laboratory findings, and magnetic resonance imaging (MRI) characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis (anti-LGI1 antibody encephalitis) and explore the electroencephalogram (EEG) features. We retrospectively analyzed the medical history of 16 patients diagnosed with anti-LGI1 antibody encephalitis at the First Hospital of Hebei Medical University from 2021 to 2023. EEGs of patients with anti-LGI1 antibody encephalitis and healthy individuals were analyzed. Based on Video-EEG signal analysis of EEG δ, θ, α, β frequency bands, weighted phase lag index values were calculated to form brain network matrices, studying differences in coherence between brain regions of patients with anti-LGI1 antibody encephalitis and healthy individuals. Patients with anti-LGI1 antibody encephalitis often presented with subacute onset seizures and cognitive decline. Routine test of cerebrospinal fluid was mostly normal. Serum testing revealed hyponatremia in 62.50% of patients, along with positive serum antinuclear antibodies, decreased vitamin B12, and abnormal cytokines such as interleukin-6. Head MRI revealed abnormal lesions related to the disease in seven cases (43.75%), mainly located in the unilateral or bilateral frontal and temporal lobes of the hippocampus. The EEG mainly showed generalized and focal slow waves, sometimes with focal discharges. Brain network functional connectivity analysis found a significant weakening of functional connections in the frontal-temporal lobe in the δ and β frequency bands. Intravenous pulse corticosteroids and intravenous immunoglobulin are first-line immunotherapies for anti-LGI1 antibody-related encephalitis. The disease recovery and cognitive decline improved in most patients. Anti-LGI1 antibody encephalitis is characterized by seizures and cognitive dysfunction. Serum may show abnormalities in immune indicators such as cytokines. Head MRI mainly reveals abnormal signals in the frontal-temporal lobes and the hippocampus. EEG brain network connectivity analysis reveals characteristic weakening of functional connections in the frontal-temporal lobe in the δ and β frequency bands.

Outcomes and predictors of seizure recurrence in post-stroke epilepsy, a retrospective hospital-based study

BackgroundThe occurrence of seizures following a stroke is a well-recognized complication associated with a significant increase in morbidity and mortality. Despite the numerous studies examining outcomes and risk factors related to post-stroke seizures (PSS), there remains a lack of clarity regarding the clinical characteristics, treatment, and PSS recurrence (PSSR) rates in patients experiencing their initial episode of PSS. PurposeThis study aimed to determine the risk factors for developing recurrent seizures after first PSS and their effects on functional outcomes and mortality. MethodsAll patients underwent an electroencephalography (EEG) and were monitored for a minimum of 24 months following the first PSS. The primary endpoint was the recurrence of seizures. Predictive factors for PSSR were determined by using the Cox-proportional hazards model, and the cumulative latency of recurrence at 90, 180, 360, and 720 days was estimated using Kaplan-Meier analysis. ResultsSeizure recurred in 36.8% (39/106). Significant association of PSSR was noted with female gender, use of older anti-seizure medications (ASMs) (p<0.001), EEG findings as focal slow wave activity (p<0.001), Ictal epileptiform abnormalities (p=0.015), status epilepticus (p=0.015), and with severe disability (p=0.008). However, multivariate cox-proportional hazards model showed significant association of female gender (HR=3.28; 95% CI: 1.42–7.58; p=0.006). Hazard ratio (HR) was increased with older ASMs use, focal aware seizure types, Ictal EAs, and periodic discharges on EEG; though, statistically significant. ConclusionFactors such as the type of ASMs, EEG findings, and seizure type were significantly linked to PSSR. Female gender was the only independent predictor established. Additionally, significant functional decline was reported with recurrence.

Clinical Implications of Various Electroencephalographic Patterns in Post-Stroke Seizures. The Utility of Routine Electroencephalogram.

Objective: Post-stroke seizures (PSS) are one of the major stroke-related complications. Early therapeutic interventions are critical therefore using electroencephalography (EEG) as a predictive tool for future recurrence may be helpful. We aimed to assess frequencies of different EEG patterns in patients with PSS and their association with seizure recurrence and functional outcomes. Methods: All patients admitted with PSS were included and underwent interictal EEG recording during their admission and monitored for seizure recurrence for 24 months. Results: PSS was reported in 106 patients. Generalized slow wave activity (GSWA) was the most frequent EEG pattern observed (n = 62, 58.5%), followed by Focal sharp wave discharges (FSWDs) (n = 57, 55.8%), focal slow wave activity (FSWA) (n = 56, 52.8%), periodic discharges (PDs) (n = 13, 12.3%), and ictal epileptiform abnormalities (n = 6, 5.7%). FSWA and ictal EAs were positively associated with seizure recurrence (p < .001 and p = .015 respectively) and it remained significant even after adjusting for age, sex, stroke severity, stroke subtype, or use of anti-seizure medications (ASMs). Other positive associations were status epilepticus (SE) (p = .015), and use of older ASM (p < .001). FSWA and GSWA in EEG were positively associated with severe functional disability (p = .055, p = .015 respectively). Other associations were; Diabetes Mellitus (p = .034), Chronic Kidney Disease (p = .002), use of older ASMs (p = .037), presence of late PSS (p = .021), and those with Ischemic stroke (p = .010). Conclusions: Recognition and documentation of PSS-related EEG characteristics are important, as certain EEG patterns may help to identify the patients who are at risk of developing recurrence or worse functional outcomes.

Electroencephalogram characteristics of 26 children with febrile infection-related epilepsy syndrome

To investigate the electroencephalogram (EEG) characteristics and progression of febrile infection-related epilepsy syndrome (FIRES) in children, aiming to enhance diagnosis and treatment approaches. A retrospective analysis was conducted on 26 children with FIRES between May 2017 and December 2021. All 26 children (100%) presented with fever at the onset, followed by frequent convulsions that rapidly progressed into convulsive status. Ventilator support was required for 22 cases (85%). During the acute phase, EEG features demonstrated the disappearance of background activity and physiological sleep cycles in all children. Diffuse slow waves and multifocal slow spike slow waves were observed as abnormal waves during the interictal period. A characteristic pattern of focal low amplitude fast wave initiation was detected in all children during seizure episodes. In the chronic phase, the background EEG activity gradually recovered, and the presence of abnormal waves was relatively limited. The characteristic pattern of focal slow wave rhythm initiation was evident during seizure episodes. Additionally, extreme δ brushes were observed in four cases (15%). These findings suggest that EEG manifestations in children with FIRES exhibit distinctive patterns during the acute and chronic stages, providing significant value for early diagnosis and clinical staging. Extreme δ brushes may be one of the distinctive markers of children with FIRES.

Clinical and electroencephalography characteristics of 41 children with epileptic spasms onset after 1 year of age

The incidence of epileptic spasms (ES) that begin after the first year of life is much lower than that before 1 year of age. The aim of this study was to identify clinical and electroencephalography (EEG) characteristics, etiologies, treatments, and prognoses in pediatric patients with ES onset after 1 year of age. Forty-one children were retrospectively identified in Children's Hospital of Chongqing Medical University between January 1, 2020 and December 1, 2021. ES onset after 1 year of age have diverse presentations. Although most occur in clusters, are symmetrical and flexional, and occur frequently during awakening, some are characterized as isolated and asymmetrical, have a tonic component, and can also occur during sleep. The hypsarrhythmia variants and focal or multifocal discharges occur alternately in the interictal period, and the focal spikes and slow waves predominated in the unilateral temporal or frontotemporal areas. These patients had diverse etiologies, including structural (51.2 % of patients) and genetic (22.0 %) ones, and 11 patients (26.8 %) had an unknown etiology. No patients in our study had an infectious or immune-mediated etiology. Forty-eight percent of patients responded to hydrocortisone and/or adrenocorticotropic hormone. The efficacy of antiepileptic drug therapy was lower in patients who did not receive concurrent steroid therapy. However, ES onset after 1 year of age caused by a tumor, brain malformation, or other focal lesions, may be cured by focal cortical resection despite a lack of clearly localized EEG surface anomalies. Delays in motor, language, and cognitive development, or behavioral problems were observed in all but three patients.

P 92 Limbic encephalitis with Contactin-associated protein like-2 (CASPR2) -antibodies: acute psychosis with late onset and abnormal EEG

Introduction: Contactin-associated protein like-2 (CASPR2)- autoantibodies are associated with distinctive syndromes 1 , such as agrypnia excitata, psychosis, neuromytonia and limbic encephalitis. All of them are highly responsive to immunotherapy. The target of these directly pathogenetic autoantibodies are surface-exposed domains of CASPR2 2 . Due to the fact of multiple variations of clinical presentations, it may be challenging to distinguish particular an autoimmune encephalitis associated with anti-neuronal autoantibodies, from primary psychiatric disorders such as psychosis. First medical treatment should be immediate immunotherapy, such as steroids, intravenous antibodies or plasma exchange given fast diagnosis of encephalitis is made. In our case the EEG showed focal slow waves with the clinical presentation of an acute psychosis which leaded to an admission to the neurology ward and further investigations. Patient and methods: A 75-years-old man was hospitalized on a psychiatric ward for psychotic symptoms characterized by psychomotor agitation, thought disorganization, persecutory delusions and auditory hallucinations with commanding voices and global insomnia. Cerebral Imaging with cMRI and cCT scan showed no acute abnormalities. EEG showed focal intermittent slow delta-theta wave activity (4-6 Hz) on the left temporal lobe, reactive to visual stimuli and provided focal cerebral dysfunction and pathology. CSF did not reveal any evidence of infection, but CASPR2- autoantibodies (1:10.000) were indentified and the diagnosis of limbic encephalitis with CASPR2-antibodies was made. Treatment with high dose dexamethasone and intravenous immunoglobulin (IVIG) was made and the clinical conditions improved. Conclusion: Fast and first medical treatment of autoimmune encephalitis should be initiated in early stages. To avoid an unnecessary delay in diagnosis and treatment, EEG, cMRI and expanded CSF examination should be performed as soon as possible to prevent severe disability in patients with limbic encephalitis with CASPR2-antibodies.

Electroencephalographic findings after convulsive seizures due to cerebral arterial air embolism secondary to lung cancer: a case report

BackgroundCerebral arterial air embolism is often associated with an invasive iatrogenic etiology and a high rate of convulsive seizures. There are only a few descriptions of electroencephalogram findings in convulsive seizures due to cerebral arterial air embolism of noniatrogenic etiology. Herein, we describe the case of a patient with lung cancer and convulsive seizures with abnormalities detected on electroencephalogram caused by cerebral arterial air embolism of noniatrogenic etiology.Case presentationA 55-year-old Japanese man underwent radiotherapy and chemotherapy for cancer in the hilum of the left lung that was diagnosed after hemoptysis. One year after the diagnosis, he developed fever and chest pain that required hospitalization. At admission, he was in shock, and chest computed tomography revealed invasion of the left atrium and left main bronchus by the hilar cancer. Chest and abdominal computed tomography revealed small low-density areas within the tumor and around the intestinal membrane, which were interpreted as the presence of air due to invasion of the lung cancer. He was diagnosed with septic shock due to necrotic infection secondary to cancer invasion into the left atrium. The following day, he complained of difficulty in speaking and weakness in the left side of his body. A head computed tomography scan revealed multiple small low-density areas in the right cortex and bilateral subcortex, which were interpreted as air emboli. On day 3, he experienced generalized tonic–clonic seizures for approximately 1 minute, followed by myoclonus-like convulsions in the left lower limb and a right-sided gaze. The electroencephalogram findings after the convulsive seizures revealed partial epilepsy-like waves with intermittent spikes in the bilateral cerebral hemispheres and a diffuse slow wave in the left frontal lobe. He recovered from sepsis without recurrence of convulsive seizures; however, he died of hemoptysis on day 50 after discharge.ConclusionsElectroencephalogram findings of focal spike activities and diffuse slow waves were detected in early seizures due to cerebral arterial air embolism of noniatrogenic etiology associated with lung cancer. Additional case descriptions are warranted to establish patterns in electroencephalogram findings specific to cerebral arterial air embolism.

Clinical Neurophysiology of Zika Virus Encephalitis.

Zika virus (ZIKV) has been shown to be highly neurotropic; neurologic disorders are a common complication of this infection. Encephalitis-an inflammation of the brain parenchyma associated with neurologic dysfunction-is a rare complication of ZIKV infections. It affects patients from young to elderly ages. Clinical presentation of ZIKV encephalitis may be heterogeneous, including altered mental status (decreased or altered level of consciousness, lethargy, or personality change), seizures, and focal deficits. Complementary diagnostic investigation should include neuroimaging, lumbar puncture, and EEG. Neuroimaging findings in ZIKV encephalitis are not specific and may be diverse, including normal findings, hyperintense lesions on MRI involving cortical or subcortical structures, symmetric or asymmetric lesions involving supra or infratentorial regions, and more widespread involvement such as brain swelling. A remarkable scarcity of neurophysiological data on ZIKV encephalitis was found in the literature. In line with other diagnostic examinations, there are no neurophysiological findings suggestive or specific of the disease. EEG in ZIKV encephalitis showed different results: normal or diffuse disorganization of background activity, asymmetry with abnormal focal slow waves, focal epileptic discharges or generalized spike-wave and multispike-wave complexes, and periods of generalized voltage attenuation.

Predictors and prognoses of epilepsy after anti-neuronal antibody-positive autoimmune encephalitis

PurposeThis study aimed to investigate the incidence and predictors of epilepsy after anti-neuronal antibody-positive autoimmune encephalitis (AIE). The clinical outcomes of patients with epilepsy after AIE were also explored. MethodsA total of 111 AIE patients were retrospectively evaluated. Post-AIE epilepsy (PAEE) was defined as at least one unprovoked seizure occurring six or more months after discharge from hospital. ResultsThe incidence of acute symptomatic seizures was 80.2% (89/111) in our AIE patients. Furthermore, of the 89 AIE patients with seizures, 29 (32.6%) presented with seizures as the initial symptom. Overall, 44 out of 111 AIE patients (39.6%) had unprovoked seizures after six months, meeting our definition of PAEE. The independent risk factors for PAEE incidence included an initial presentation with new-onset refractory status epilepticus (NORSE), delayed immunotherapy treatment, the complication of a lung infection during admission, the requirement for mechanical ventilation during hospitalization, parietal lesions observed in magnetic resonance imaging (MRI), and focal slow waves on electroencephalographic (EEG) monitoring. ConclusionsEarly initiation of immunotherapy and lung infection treatment may reduce the risk of conversion of symptomatic seizures to chronic epilepsy in the acute phase of AIE. In general, PAEE patients could have a good prognosis if treated properly and in a timely fashion.

Clinical Correlates of Electroencephalographic Patterns in Critically Ill Patients.

The objective of this study was to determine the frequency and clinical correlates of different electroencephalographic patterns and their association with clinical outcomes in critically ill patients. This retrospective cross-sectional study was performed in the Neurology Department of King Fahd Hospital of the University, Kingdom of Saudi Arabia and involved a review and analysis of medical records pertaining to 179 intensive care unit patients who underwent electroencephalography (EEG) in the June to November 2018 period. Among the different etiologies, presence of spike and wave or sharp wave (SWs) was associated with encephalitis (P = .01) and large artery stroke (P = .01), whereas markedly attenuated EEG activity (p = .04) and burst suppression (P = .01) were associated with large artery stroke and hypoxic ischemic encephalopathy (HIE), respectively. Generalized theta activity (P = .01) was significantly found in patients of septic encephalopathy, while generalized delta activity (P = .02) and asymmetrical background (P = .04) were significantly associated with traumatic brain injury. Presence of periodic discharges in EEG was significantly associated with more adverse clinical outcomes (P = .001), whereas rhythmic delta activity (RDA) (P = .03), persistent focal slow wave activity (P = .01), and asymmetric background (P = .002) were found in patients who were discharged from hospital with sequelae of current illness. Certain EEG patterns are associated with particular underlying etiologies like SWs for encephalitis, markedly attenuated EEG activity and burst suppression with large artery stroke and HIE, respectively. Whereas few EEG patterns, including periodic discharges, RDA, persistent focal slow wave activity have some prognostic value in critically ill patients. However, they cannot be used as markers for prognostic assessment of patients without considering other clinical and diagnostic variables. Further larger prospective studies with continuous EEG monitoring with control of confounding factors are needed.

Diagnostic Yield of Neuroimaging and Electroencephalography in Children with Recurrent Headaches

AbstractNeuroimaging and electroencephalography (EEG) are commonly used for pediatric recurrent headache evaluation, although not routinely recommended. In this study, data of 517 children with recurrent headaches were analyzed to evaluate the diagnostic yield of neuroimaging and EEG. Neuroimaging was performed in 55% (n = 283) of children, 61% with magnetic resonance imaging and 39% with computed tomography. Abnormal findings were reported in 17% (n = 48), and 5% (n = 13) were significant abnormalities altering headache management. Among children with significant imaging abnormalities, 85% had alarming signs on history, physical, or neurological examination. The diagnostic yield of imaging was 0.7% (n = 2), picking a cavernous hemangioma and a giant arachnoid cyst. EEG was performed in 69% (n = 356) of children, significant findings were reported in 8% (n = 29), with sharp waves/spikes in 4.1% (n = 15), and intermittent focal slow waves in 3.9% (n = 14). EEG was diagnostic for epilepsy in 1.6% (n = 6) of children with recurrent headaches. When there are no red flags on history or physical examination, diagnostic yield of neuroimaging is low for pediatric recurrent headaches. EEG can be helpful in selective cases, and when performed during and at headache-free periods.

Clinical and Electroencephalographic Features of the Seizures in Neuronal Surface Antibody-Associated Autoimmune Encephalitis.

Objectives: To investigate clinical and electroencephalographic features of the seizures in different types of neuronal surface antibody (NSAb)-associated autoimmune encephalitis (AE).Methods: The clinical data of the seizures were analyzed in 18 patients with NSAb-associated AEs diagnosed in the First Affiliated Hospital of Dalian Medical University.Results: From May 2013 to April 2019, a total of 18 cases of NSAb-associated AE were diagnosed, including 9 cases of leucine-rich glioma-inactivated 1 protein (LGI1) antibody-associated encephalitis, 7 cases of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, and 2 cases of anti-γ-aminobutyric acid B receptor (GABABR) encephalitis. All nine cases (100%) with LGI1 AE had seizures manifesting in three types: faciobranchial dystonia seizure (FBDS) (44.4%), mesial temporal lobe epilepsy (MTLE)-like seizure (66.7%), and focal to bilateral tonic–clonic seizure (FBTCS) (77.8%). Six of nine (66.7%) showed abnormal signal on hippocampus or basal ganglia in brain MRI. Five of seven cases (71%) with anti-NMDAR encephalitis had seizures manifesting in three types: focal aware seizure (40%), focal-impaired awareness seizure (20%), generalized tonic–clonic seizure (GTCS) (100%), and status epilepticus (SE) (40%). Three of seven (42.8%) showed abnormalities in brain MRI. Both patients with anti-GABABR encephalitis had seizures manifesting in two types: GTCS and MTLE-like seizure, one with SE. One showed abnormal signal on left hippocampus in brain MRI. All patients (100%) with three types of AE had abnormalities in electroencephalogram (EEG), showing diffuse (4/18) or focal slow waves (14/18) in background, interictal (10/18), or ictal (6/18) epileptic discharges in the temporal or other regions; two patients with anti-NMDAR encephalitis showed delta activity or rhythm in frontotemporal region. All patients with seizures showed good response to immunotherapy except one with LGI1 AE.Conclusions: Most patients with NSAb-associated AE had seizures; seizure types varied between different types of AE. In LGI1 AE, the hippocampus and basal ganglia were two main targets; the corresponding seizure type was MTLE-like seizure and FBDS, respectively. Anti-NMDAR encephalitis had more generalized than focal seizures. Delta activity or rhythm in the frontotemporal region in EEG was helpful for diagnosis. Anti-GABABR encephalitis was characterized by refractory seizures as initial symptom, mainly GTCS or MTLE-like seizure. Most seizures in NSAb-associated AE showed good response to immunotherapy, and antiepileptic drugs should be considered as an add-on symptomatic treatment.

Clinical features of nine cases of leucine-rich glioma inactivated 1 protein antibody-associated encephalitis.

To investigate clinical features of leucine-rich glioma inactivated 1 protein (LGI1) antibody-associated autoimmune encephalitis (AE). The clinical data were collected and analyzed in nine patients with LGI1 AE. All nine patients (100%) presented acute/subacute onset, had seizures, cognitive impairment, mental/behavioral abnormalities, six had sleep disorders and seven showed hyponatremia. Seizures manifested in three types: faciobranchial dystonia seizure (FBDS) (44%), mesial temporal lobe epilepsy (MTLE)-like seizure (66%), and focal to bilateral tonic-clonic seizure (FBTCS) (77%). Six of nine cases (66%) showed abnormalities in brain MRI, among them four showed high T2/flair signal on unilateral/bilateral hippocampus, two showed high T1/T2 signal on unilateral basal ganglia. All nine patients (100%) showed abnormalities in EEG, among them 1 (11%) showed diffuse slow waves, 8 (88%) showed focal slow waves; 6 (66%) revealed interictal epileptic discharges; ictal EEG was recorded in five patients, two were FBDS, three were MTLE-like seizure.LGI1 antibodies in serum and cerebrospinal fluid were both positive. No signs of tumor were found in all patients. Eight of nine patients received immunotherapy and antiepileptic drug (AED) treatment, one only treated with AED without immunotherapy. Eight patients improved significantly with seizure-free after immunotherapy, only one still had FBDS after immunotherapy and AED treatment. In LGI1 AE hippocampus and basal ganglia were two main targets, the corresponding seizure type was MTLE-like seizure and FBDS respectively. Diagnosis depended on detection of LGI1 antibodies in CSF. The incidence of tumor was low. The effect of immunotherapy was good and AEDs should be considered as add-on symptomatic treatment.

Clinical and Radiological Correlates of Different Electroencephalographic Pattern in Hospitalized Patients.

Objective. The present study aimed to determine the clinical and radiological correlates of different electroencephalographic (EEG) patterns in hospitalized patients. Subjects and Methods. In this retrospective study performed at the Neurology Department, King Fahd University Hospital, Kingdom of Saudi Arabia (KSA), data of 374 patients who underwent EEG were reviewed and analyzed. Results. Presence of focal spike and wave or sharp wave (SW) (P = .00), generalized theta activity (P = .00), generalized delta activity (P = .04), persistent focal slow wave activity (SWA) (P = .003) and asymmetric background (P = .01) in the EEG record was significantly associated with abnormal imaging findings. Specifically, generalized theta delta activity (P = .01) and markedly attenuated EEG activity (P = .007) were associated with presence of cortical lesions; whereas, triphasic waves (TWs) (P = .009), and generalized theta activity (P = .001) were found to be related with presence of subcortical lesions. While, generalized delta activity (P = .01) was the only correlate with extra-axial lesions. Conclusion. At present, certain EEG patterns cannot be precisely correlated with imaging findings, suggesting that intercurrent metabolic, infectious, and/or toxic contributors could be the confounding factors. Nonetheless, when EEG patterns are examined alongside magnetic resonance imaging findings and other clinically relevant data, these might be indicative of a group of diseases in some pertinent situations. Thus, further larger prospective clinical studies that incorporate continuous EEG monitoring, advanced radiology techniques, and laboratory analyses would be beneficial to elucidate their interplay for better firm up the correlations.

Multidisciplinary Study Based on Clinical, Electrophysiological and Psycological Evaluations Combined with Advanced Neuroimaging in Gaucher Disease Patients

Gaucher Disease (GD) is an autosomal recessive metabolic disorder due to glucocerebrosidase deficit. There are three main clinical phenotypes: type I (GD1: non-neuronopathic form), characterized by a visceral involvement that can mimic a hematologic disease; type II (GD2: acute neuropathic form) and type III (GD3) characterized by a slower and progressive neurological involvement. Hematologists have often involved in diagnosis and management of the GD1 patients. Increasing data and our experience show that patients with GD1 may present manifestations of Parkinson's disease or Parkinsonism symptoms (tremor, bradykinesia and rigidity), frequently combined with cognitive impairment and behavioral alterations. The aim of this study is to deeply investigate the neurological and neuropsychiatric aspects in GD1 patients in order to identify clinical and subclinical neurological manifestations, including cognitive impairment and behavioral alterations in this category of patients. This observational, monocentric, and prospective study is planned to enroll 22 GD patients (19 GD1 patients and 3 GD3 patients with or without active neurological signs) aged &gt;12 years. Neurological assessments include: clinical evaluation including Severity Scoring Tool Unified Parkinson's Disease Rating scale and Epworth Sleepiness scale, psycho-diagnostic tests and psychiatric evaluation using cognitive test battery and two psychiatric (BPRS) and psychological (CBA 2.0) questionnaires, Somatosensory and Motors Evoked Potentials, Electroencephalography (EEG) and magnetic resonance (MR) 3Tesla, at baseline, after 12 months and 24 months. Preliminary results concerning the baseline evaluation of first cohort of patients are here reported. Since June 2019, 11 patients (10 GD1 and 1 symptomatic GD3) have been enrolled. Neurological impairment according to the major features (Gaucher severity score tool) was found in 4/11 patients: signs of Parkinson disease in 1 GD1 patient, motoneuron disease in 1 GD3 patients, abnormalities in ocular motility and psycho-cognitive disturbances in 2 GD1 patients. Minor features of a neurological impairment was found as follows: bradykinesia in 5 GD1 patients, excessive daytime sleepiness in 5 GD1 patients, and saccadic slowness in 1 GD1 patient. EEG revealed focal or diffuse slow waves in 6 patien patients (5 GD1 and 1 GD3). Cognitive and psychological evaluations revealed the presence of significant psychiatric symptoms such as depression, anxiety, and physical concerns. Six patients (5 GD1and 1 GD3) surprisingly had sensorineural hearing loss. Auditory Brainstem Response revealed abnormal disyncrony-like morphology and central speech processing in noise in 3 GD1 and in 4 (3 GD1 and 1 GD3) patients, respectively. Ophthalmological evaluation highlighted a normal visus and intraocular pressure in all patients. Moderate alterations of the fundus were present in 2 patients (1 GD1 and 1 GD3) and superficial corneal dystrophies in 3 (2 GD1 and 1 GD3) patients. An increased latency and moderate reduction in the amplitudes of optic nerve function were found in all tested patients. The retinal responses to the Electroretinogram exam were of excellent quality in all but one GD1 patient, who showed slightly reduced responses. The spectral domain-optical coherence tomography (SD-OCT) examination showed posterior pole alterations in 3 patients (2 GD1 and 1 GD3) and borderline nerve fiber layers of the optic nerve (RNFL) in 3 (2 GD1 and 1 GD3) cases. Our clinical study represents quite an innovative one, since neurological investigations are focused on GD2 and GD3 phenotypes. Neurological involvement with different phenotypical aspects were also found in GD1 patients. Sensorineural hearing loss and central alteration of auditory processing were present in higher incidence compared to the unaffected population. Psychological data din't show significant cognitive impairments, but pointed out some psychiatric aspects, such as anxiety, depression and somatic concerns. MR 3Tesla can be an important tool to better understand these data. Evaluation of the remaining cohort of patients and longer follow-up could confirm that neurological involvement is not exclusive of the type 3 GD. Disclosures No relevant conflicts of interest to declare.

Clinical features of cerebral amyloid angiopathy characterized by cortical superficial siderosis

Objective: To study the clinical and imaging characteristics of cerebral amyloid angiopathy characterized by cortical superficial siderosis and improve clinicians' understanding of the disease. Methods: Retrospective analysis was performed on 16 patients with cerebral amyloid angiopathy characterized by cortical superficial siderosis from June 2013 to August 2016 in Beijing Hospital, and the information including epidemiological data, clinical features, cranial MRI and electroencephalogram (EEG) results were analyzed. Results: The ratio of male to female in 16 patients was 1.67∶1, and the average age of onset was 73 (69-79) years. The most common clinical symptoms were transient focal neurological episodes (TFNEs)(12/16). Cranial MRI showed localized (9/16) and diffuse type cortical superficial siderosis (7/16); few of them were associated with different degrees of cerebral microbleeds. Most of the EEG findings were normal (6/9) and a few showed focal slow waves (3/9). During a mean follow-up of 17 (17±11) months, 5 patients developed repeated TFNE, of which 1 had cerebral hemorrhage. Conclusions: Cerebral amyloid angiopathy characterized by cortical superficial siderosis occurs predominantly in the elderly. TFNE is the most common clinical manifestation. Cranial MRI is the most important diagnostic method, and antithrombotic therapy should be avoided as much as possible.

Grand Total EEG Score Can Differentiate Parkinson's Disease From Parkinson-Related Disorders.

Background: Semi-quantitative electroencephalogram (EEG) analysis is easy to perform and has been used to differentiate dementias, as well as idiopathic and vascular Parkinson's disease.Purpose: To study whether a semi-quantitative EEG analysis can aid in distinguishing idiopathic Parkinson's disease (IPD) from atypical parkinsonian disorders (APDs), and furthermore, whether it can help to distinguish between APDs.Materials and Methods: A comprehensive retrospective review of charts was performed to include patients with parkinsonian disorders who had at least one EEG recording available. A modified grand total EEG (GTE) score evaluating the posterior background activity, and diffuse and focal slow wave activities was used in further analyses.Results: We analyzed data from 76 patients with a final diagnosis of either IPD, probable corticobasal degeneration (CBD), multiple system atrophy (MSA), or progressive supra-nuclear palsy (PSP). IPD patients had the lowest mean GTE score, followed those with CBD or MSA, while PSP patients scored the highest. However, none of these differences were statistically significant. A GTE score of ≤9 distinguished IPD patients from those with APD (p < 0.01) with a sensitivity of 100% and a specificity of 33.3%.Conclusion: The modified GTE score can distinguish patients with IPD from those with CBD, PSP or MSA at a cut-off score of 9 with excellent sensitivity but poor specificity. However, this score is not able to distinguish a particular form of APD from other forms of the disorder.

The yield of electroencephalography in syncope.

Syncope is defined as a brief transient loss of consciousness due to cerebral hypoperfusion. Although the diagnosis of syncope is based on a thorough history and examination, electroencaphalography (EEG) is also an important investigational tool in the differential diagnosis in this group of patients. In this study we aimed to identify the diagnostic value of EEG in patients with syncope. We retrospectively examined EEG recordings of 288 patients with the diagnosis of syncope referred to the Cankiri State Hospital EEG laboratory, from January 2014 to January 2016. The EEG findings were classified into 6 groups as normal, epileptiform discharges (spike and sharp waves), generalized background slowing, focal slowing, hemispherical asymmetries, and low amplitude EEG tracing. The EEGs were separated according to gender and age. Total of 288 patients were included in this study, 148 were females (51.4%) and 140 (48.6%) were males. Among all the EEG reports, 203 (70.5%) were normal, 8 of them (2.8%) showed generalized background slowing and 7 (2.4%) demonstrated focal slow waves. Epileptiform discharges occured among 13 patients (4.5%). Hemispherical asymmetries were detected in 10 patients (3.5%) and low amplitude EEG tracing in 47 patients (16.3%). There was no significant difference between age groups in EEG findings (p=0.3). Also no significant difference was detected in EEG results by gender (p=0.2). Although the diagnosis of syncope, epilepsy and non-epileptic seizures is clinical diagnosis, EEG still remains additional method.

Seizure characteristics are related to tumor pathology in children with brain tumors

ObjectivesBrain tumors are found in 1–3% of children with newly onset of seizures. Understanding the impact of seizures on pediatric brain tumors and factors between seizures and brain tumors are crucial. This study aimed to evaluate the association of pediatric brain tumors on seizure occurrence and the relationships between seizures and the diagnosis and prognosis of brain tumors. MethodsIn a cohort of 184 children with newly diagnosed brain tumors between August 2012 and September 2017, 56 patients with brain tumor-associated seizures were evaluated for their clinical characteristics, electroencephalography (EEG), tumor pathology, response to anti-epileptic drugs (AEDs) and their seizure outcome. ResultsThe prevalence of seizures in our cohort of children with brain tumors was 30.4% (n = 56/184). Supratentorial tumors were more commonly associated with seizures than were infratentorial tumors (89.3% vs. 10.7%; P < 0.01). Among patients with tumor-associated seizures, the tumors were most commonly located in the temporal lobe (32.1%), followed by the frontal (21.4%) and parietal lobes (16.1%) and other cortices. Thirty-four patients (60.7%) had benign tumor pathology and 22 patients (39.3%) had malignant tumor pathology; low grade astrocytoma (n = 15; 26.8%) was the most common histology type. Seizure onset at initial presentation was more frequently observed in the patients with benign tumors, whereas the patients with malignant tumors tended to have delayed seizure onset (P < 0.01). On EEGs, focal epileptiform discharges were more commonly seen in benign tumors, whereas focal and diffuse slow waves were most commonly associated with malignant tumors. Levetiracetam was the most commonly prescribed AED to treat brain-tumor-associated seizures. ConclusionsIn children with brain tumors, higher seizure frequency with normal neurologic examination was more prevalent in benign tumors, whereas less frequent seizures with focal weakness and signs of increased intracranial pressure were more indicative of malignancy. Delayed onset of seizures was more often observed in the children with malignant brain tumors. Seizure characteristics play an important role in relation to tumor biology and epileptogenesis in pediatric brain tumors.