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

2017 ◽  
Vol 25 (1-2) ◽  
pp. 53-58
Author(s):  
Matthias Wittstock ◽  
Uwe Walter ◽  
Daniela Schirrmeister ◽  
Kyrylo Kurtieiev ◽  
Jan Klinke ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Clonic Seizure ◽  
Convulsive Status Epilepticus ◽  
Spatiotemporal Evolution ◽  
Diagnostic Dilemma ◽  
Epileptiform Discharges ◽  
Antiepileptic Medication ◽  
Epileptic Discharges ◽  
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.

scholarly journals The Importance of Rapid Consideration of Creutzfeldt- Jakob Disease in the Differential Diagnosis of Progressive Neurodegenerative Disease: A Case Report

2016 ◽  
Vol 4 (2) ◽  
pp. 72-75
Author(s):  
Arthur Joseph ◽  
Jacob Core ◽  
Daniel Solano ◽  
Marquand Patton ◽  
Shaun Smart
Keyword(s):  
Differential Diagnosis ◽  
Status Epilepticus ◽  
High Signal ◽  
Convulsive Status Epilepticus ◽  
Rare Presentation ◽  
Progressive Encephalopathy ◽  
Jakob Disease ◽  
Low Incidence ◽  
Patient’S History ◽  
Sporadic Cjd

Background: Creutzfeldt-Jakob disease (CJD) is a prion disease characterized by misfolded proteins that lead to neurodegeneration and inevitable death. Classic sporadic CJD presents primarily with cognitive symptoms and ataxia without visual impairment at the onset of the illness. Seizure activity is a rare presentation of patients with sporadic CJD. Case: We present a rare case of rapidly progressive encephalopathy in a 57-year-old female who presented to the emergency department with bizarre behavior and vision deterioration. Imaging was unrevealing, and infectious and organic causes were ruled out. Electroencephalogram showed evidence of encephalopathy and non-convulsive status epilepticus. Magnetic resonance imaging conducted later displayed high signal intensity in centrum ovale. The patient’s history, results from diagnostic analyses, and clinical presentation suggested the diagnosis of CJD (sporadic type). Conclusion: Due to the low incidence and varying clinical presentations, it is difficult to include CJD in a differential diagnosis without specific analytic measures. However, for the benefit of the patient and healthcare resources, CJD needs to be quickly considered when rapid neurological decline or non-convulsive status epilepticus is not suggestive of another entity

scholarly journals Electrical Status Epilepticus in Sleep: Is this a Focal or Generalized Pattern?

2020 ◽  
Vol 1 (1) ◽  
Author(s):  
Raphael R. Almeida ◽  
Ana Carolina Coan ◽  
Marilisa M Guerreiro
Keyword(s):  
Status Epilepticus ◽  
Clinical Data ◽  
Epileptiform Activity ◽  
Rolandic Epilepsy ◽  
Epileptiform Discharges ◽  
Epileptic Syndromes ◽  
Epileptic Discharges ◽  
Genetic Epilepsies ◽  
Rolandic Region ◽  
Correlate Data

Objectives: To evaluate the spatial distribution of the epileptiform activity in electrical status epilepticus in sleep (ESES) and to correlate data from electroencephalograms (EEGs) with clinical and neuroimaging variables. Methods: From 2008 to 2015, 162 reports (1.01%) out of 16,000 EEGs, from 23 patients, showed ESES. We selected one representative EEG per patient. Clinical data was collected retrospectively. Neuroimaging examinations were reviewed. The EEGs were classified as generalized ESES (ESESg) and focal ESES (ESESf) according to the distribution of epileptiform discharges. Results: From the 23 patients, 5 were classified as ESESg and 18 as ESESf. In ESESf, there was a prevalence of focal epileptic discharges in the centrotemporal regions. Abnormal neuroimaging occurred in 100% of the patients with ESESg and in 38.9% of the patients with ESESf (p=0.037). Other clinical data did not show significant differences between the groups. All patients with ESESg had structural etiology, while only 39% of patients with ESESf had structural etiology and the remaining 61% potentially genetic epilepsies of the rolandic spectrum. Conclusion: ESESg occurred predominantly in patients with structural lesions, while most patients with ESESf had normal neuroimaging scans and electrical dysfunction mainly in the rolandic region. Significance: ESESg seems to occur mostly in structural epilepsies. Distinctly, ESESf occurs in epileptic syndromes within the functional spectrum of rolandic epilepsy.

scholarly journals 078 A case of non-convulsive status epilepticus (NCSE) as first presentation of sporadic creutzfeldt-jakob Disease (sCJD)

2021 ◽  
Author(s):  
Luke Gagen ◽  
Kristen Lefever ◽  
Daniel Schweitzer ◽  
Laura Clarke ◽  
Cullen O’Gorman ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Convulsive Status Epilepticus ◽  
Jakob Disease

scholarly journals Non-convulsive status epilepticus: COVID-19 or clozapine induced?

2020 ◽  
Vol 13 (10) ◽  
pp. e239015 ◽  
Author(s):  
Elisaveta Sokolov ◽  
Shahrzad Hadavi ◽  
Laura Mantoan Ritter ◽  
Franz Brunnhuber
Keyword(s):  
Differential Diagnosis ◽  
Status Epilepticus ◽  
Schizoaffective Disorder ◽  
Convulsive Status Epilepticus ◽  
Neurological Manifestations ◽  
Diagnosis And Management ◽  
Global Pandemic ◽  
Antiepileptic Medication ◽  
Eeg Changes ◽  
Electroencephalogram Eeg

We present a case of non-convulsive status epilepticus in a 57-year-old woman with a schizoaffective disorder, without an antecedent seizure history, with two possible aetiologies including SARS-CoV-2 infection and clozapine uptitration. We discuss the presentation, investigations, differential diagnosis and management. In particular, we focus on the electroencephalogram (EEG) findings seen in this case and the electroclinical response to antiepileptic medication. We review the literature and discuss the relevance of this case to the SARS-CoV-2 global pandemic. We emphasise the importance of considering possible neurological manifestations of SARS-CoV-2 infection and highlight seizure disorder as one of the possible presentations. In addition, we discuss the possible effects of clozapine on the electroclinical presentation by way of possible seizure induction as well as discuss the possible EEG changes and we highlight that this needs to be kept in mind especially during rapid titration.

Status Epilepticus in Children: Update and Review

1994 ◽  
Vol 9 (2_suppl) ◽  
pp. 2S27-2S35 ◽  
Author(s):  
John M. Pellock
Keyword(s):  
Status Epilepticus ◽  
Young Children ◽  
Medical Therapy ◽  
Morbidity And Mortality ◽  
Convulsive Status Epilepticus ◽  
Elderly Adults ◽  
Neurologic Emergency ◽  
Improved Outcome

This report presents a brief description and classification of status epilepticus. This neurologic emergency occurs more frequently in young children and elderly adults, although patients of all ages may experience prolonged seizures. The pathophysiology and prognosis of the disorder are presented, as well as a review of drugs commonly used for the treatment of convulsive status epilepticus. The morbidity and mortality of status epilepticus are significant and directly dependent upon prompt and appropriate medical therapy. Advances in understanding various aspects of status epilepticus may lead to improved outcome for affected patients. (J Child Neurol 1994;9(Suppl):2S27-2S35).

scholarly journals Non-Convulsive Status Epilepticus in Medical ICU Masking Creutzfeld Jakob Disease

2020 ◽  
Author(s):  
T. Henson ◽  
E. Matayeva ◽  
R. Yusufani ◽  
R. Sanivarapu ◽  
J. Iqbal ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Convulsive Status Epilepticus ◽  
Jakob Disease

scholarly journals Periodic EEG patterns in sporadic Creutzfeld-Jakob-Disease can be benzodiazepine-responsive and be difficult to distinguish from non-convulsive status epilepticus

Seizure ◽  
2017 ◽  
Vol 53 ◽  
pp. 47-50 ◽  
Author(s):  
Justus Marquetand ◽  
Susanne Knake ◽  
Adam Strzelczyk ◽  
Bernhard J. Steinhoff ◽  
Holger Lerche ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Convulsive Status Epilepticus ◽  
Jakob Disease

scholarly journals Hyperperfusion in the thalamus on arterial spin labeling indicates non-convulsive status epilepticus

2020 ◽  
Author(s):  
Satoru Ohtomo ◽  
Hiroshi Otsubo ◽  
Hiroaki Arai ◽  
Yoshiteru Shimoda ◽  
Yoichiro Homma ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Critically Ill Patients ◽  
Arterial Spin Labeling ◽  
Spin Labeling ◽  
Convulsive Status Epilepticus ◽  
Impaired Consciousness ◽  
Periodic Discharges ◽  
Epileptiform Discharges ◽  
Delta Activity ◽  
Rhythmic Delta Activity

Abstract Non-convulsive status epilepticus describes the syndrome of unexplained impaired consciousness in critically ill patients. Non-convulsive status epilepticus is very likely to lead to delayed diagnosis and poor outcomes because of the absence of convulsive symptoms. EEG is essential for the diagnosis of non-convulsive status epilepticus to establish the association between periodic discharges and rhythmic delta activity in addition to ictal epileptiform discharges according to the Salzburg criteria. Arterial spin labeling, a type of perfusion MRI, has been applied for rapid and non-invasive evaluation of the ictal state. Ictal cerebral cortical hyperperfusion is the most common finding to demonstrate focal onset seizures. Hyperperfusion of the thalamus on single photon emission computed tomography was found in patients with impaired awareness seizures. We hypothesized that thalamocortical hyperperfusion on arterial spin labeling identifies non-convulsive status epilepticus and such thalamic hyperperfusion specifically associates with periodic/rhythmic discharges producing impaired consciousness without convulsion. We identified 27 patients (17 females; age 58-91 years) who underwent both arterial spin labeling and EEG within 24 hours of suspected non-convulsive status epilepticus. We analyzed 28 episodes of suspected non-convulsive status epilepticus and compared hyperperfusion on arterial spin labeling with periodic/rhythmic discharges. We evaluated 21 episodes as a positive diagnosis of non-convulsive status epilepticus according to the Salzburg criteria. We identified periodic discharges in 15 (12 lateralized and three bilateral independent) episodes and rhythmic delta activity in 13 (10 lateralized, one bilateral independent, two generalized) episodes. Arterial spin labeling showed thalamic hyperperfusion in 16 (11 unilateral, 5 bilateral) episodes and cerebral cortical hyperperfusion in 24 (20 unilateral, 4 bilateral) episodes. Thalamic hyperperfusion was significantly associated with non-convulsive status epilepticus (P = 0.0007; sensitivity, 76.2%; specificity, 100%), periodic discharges (P < 0.0001; 93.3%; 84.6%), and rhythmic delta activity (P = 0.0006; 92.3%; 73.3%). Cerebral cortical hyperperfusion was significantly associated with non-convulsive status epilepticus (P = 0.0017; 100%; 57.1%) and periodic discharges (P = 0.0349; 100%; 30.8%), but not with rhythmic delta activity. Thalamocortical hyperperfusion could be a new biomarker of non-convulsive status epilepticus according to the Salzburg criteria in critically ill patients. Specific thalamic hyperexcitability might modulate the periodic discharges and rhythmic delta activity associated with non-convulsive status epilepticus. Impaired consciousness without convulsions could be caused by predominant thalamic hyperperfusion together with cortical hyperperfusion but without ictal epileptiform discharges.

scholarly journals Hyperosmolar hyperglycaemic state causing atypical status epilepticus with hippocampal involvement

2021 ◽  
pp. practneurol-2021-003222
Author(s):  
Emanuele Bartolini ◽  
Raffaella Valenti ◽  
Josemir W Sander
Keyword(s):  
Status Epilepticus ◽  
Hippocampal Sclerosis ◽  
Convulsive Status Epilepticus ◽  
Epileptiform Discharges ◽  
Symptomatic Seizures ◽  
Mesial Temporal Lobe ◽  
Permanent Brain Damage ◽  
Acute Symptomatic Seizures

Diabetes mellitus may arise abruptly and decompensate suddenly, leading to a hyperglycaemic hyperosmolar state. Coma often ensues, although this usually reverses after the metabolic abnormalities have resolved. Acute symptomatic seizures can also occur in patients who are conscious, although these usually resolve after osmolarity and glycaemia have normalised. We describe an elderly woman who failed to regain vigilance despite prompt treatment; the cause was an unusual non-convulsive status epilepticus arising from the mesial temporal lobe and promoting a progressive and selective hippocampal involvement. During follow-up, her seizures recurred after stopping antiseizure medication and she developed hippocampal sclerosis, although she subsequently became seizure-free with antiseizure medications. Patients who are unresponsive in a hyperglycaemic hyperosmolar state may be having subclinical epileptiform discharges and risk developing permanent brain damage and long-term epilepsy.

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