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 Non-convulsive status epilepticus: an often-overlooked etiology of syndrome of inappropriate antidiuretic hormone secretion

2017 ◽  
Vol 5 (5) ◽  
pp. 2251
Author(s):  
Elizabeth Davis ◽  
Rima Chakraborty
Keyword(s):  
Differential Diagnosis ◽  
Status Epilepticus ◽  
Mental Status ◽  
Hormone Secretion ◽  
Altered Mental Status ◽  
Convulsive Status Epilepticus ◽  
Chronic Medical Conditions ◽  
Inappropriate Antidiuretic Hormone Secretion ◽  
Antidiuretic Hormone Secretion ◽  
Chronic Hyponatremia

Altered mental status is a common presenting complaint in adult medicine with a broad differential diagnosis. When found in the context of chronic medical conditions, less common etiologies can be overlooked. We present a case of acute altered mental status thought to be secondary to acute on chronic hyponatremia in the context of syndrome of inappropriate antidiuretic hormone secretion (SIADH), eventually diagnosed as non-convulsive status epilepticus, partial type. We report the case of a 67-year-old patient with known SIADH of unknown etiology, hypertension, chronic pancreatitis and chronic obstructive pulmonary disease (COPD) who presented with fatigue, myalgia, decreased urine output. On presentation patient also had profound acute on chronic hyponatremia. During sodium correction, the patient developed an acute, progressive decline in mental status. Vital signs remained stable and workup including LP and MRI were negative. Initial electroencephalographic (EEG) showed no definitive seizure activity, but did show bifrontal focal continuous slowing. The patient’s mental status continued to decline and upon further evaluation it was suggested that the EEG findings and the patient’s progressive AMS could be compatible with non-convulsive status epilepticus. The patient received loading doses of IV lorazepam and levetiracetam and within 48 hours after initial treatment was back to baseline. Non-convulsive status epilepticus is a common, but heterogeneous subclass of status epilepticus that is difficult to diagnose. This case demonstrates the difficulty of diagnosing normalized corrected Shannon entropy (NCSE) in the context of other chronic medical conditions and the importance of including it on any differential diagnosis for acute change in mental status. 

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.

scholarly journals An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob Disease

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Parmvir Parmar ◽  
Curtis L. Cooper ◽  
Daniel Kobewka
Keyword(s):  
Differential Diagnosis ◽  
Cognitive Deficits ◽  
Autoimmune Encephalitis ◽  
Illustrative Case ◽  
Visual Hallucinations ◽  
Progressive Dementia ◽  
History Of ◽  
Jakob Disease ◽  
Clinical Description ◽  
Sporadic Cjd

Rapidly progressive dementia is a curious and elusive clinical description of a pattern of cognitive deficits that progresses faster than typical dementia syndromes. The differential diagnosis and clinical workup for rapidly progressive dementia are quite extensive and involve searching for infectious, inflammatory, autoimmune, neoplastic, metabolic, and neurodegenerative causes. We present the case of a previously highly functional 76-year-old individual who presented with a 6-month history of rapidly progressive dementia. His most prominent symptoms were cognitive impairment, aphasia, visual hallucinations, and ataxia. Following an extensive battery of tests in hospital, the differential diagnosis remained probable CJD versus autoimmune encephalitis. He clinically deteriorated and progressed to akinetic mutism and myoclonus. He passed away 8 weeks after his initial presentation to hospital, and an autopsy confirmed a diagnosis of sporadic CJD. We use this illustrative case as a framework to discuss the clinical and diagnostic considerations in the workup for rapidly progressive dementia. We also discuss CJD and autoimmune encephalitis, the two main diagnostic possibilities in our patient, in more detail.

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

Dura mater graft-associated Creutzfeldt–Jakob disease with an incubation period of 30 years, mimicking non-convulsive status epilepticus

2019 ◽  
Vol 119 (3) ◽  
pp. 497-499 ◽  
Author(s):  
Vincent Van Iseghem ◽  
Anne Sieben ◽  
Alexander Verhaeghe ◽  
Bart De Vil ◽  
Alfred Meurs
Keyword(s):  
Status Epilepticus ◽  
Incubation Period ◽  
Dura Mater ◽  
Convulsive Status Epilepticus ◽  
Jakob Disease ◽  
Dura Mater Graft

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 Etiology and outcomes of convulsive status epilepticus in children

2019 ◽  
Vol 35 (3) ◽  
Author(s):  
Muhammad Uzair ◽  
Asif Ibrahim ◽  
Faisal Zafar ◽  
Tipu Sultan
Keyword(s):  
Mental Retardation ◽  
Status Epilepticus ◽  
Open Access ◽  
Original Work ◽  
P Value ◽  
Convulsive Status Epilepticus ◽  
Common Cause ◽  
Creative Commons ◽  
Progressive Encephalopathy ◽  
Open Access Article

Objective: The study aimed to ascertain different causes and outcomes of convulsive status epilepticus in children. Methods: From January 2018 to June 2018, seventy three patients who presented with status epilepticus were studied. Data were recorded with the help of a pre-formed performa. Etiological factors and outcomes in terms of recovery, morbidity and mortality were studied. Results: Out of 73 children, forty one (56%) were males and 32(44%) were females with median age of 1.09±0.27 years. Etiologies were acute symptomatic 25(34%), febrile 19(26%), progressive encephalopathy 10(14%), remote symptomatic 10(14%) and idiopathic 7 (9%) with p-value 0.005. Status epilepticus was controlled within one hour in 42(57%), within 1-6 hours in 21(29%) and more than 6 hours in 10(14%) patients with p-value 0.027. During hospitals stay, twenty one (29%) patients recovered completely, seizure recurred in 12(16%), Twelve (16%) became mentally retarded, Twelve (16%) developed mental retardation along with seizures and 16(22%) died. Eight (10.9%) deaths were attributed to acute symptomatic etiology with p-value less than 0.001. Conclusion: This study concluded that acute symptomatic etiology was more common cause of status epilepticus as compared to other etiologies and it is associated with poorer outcomes as compared to other etiologies. doi: https://doi.org/10.12669/pjms.35.3.120 How to cite this:Uzair M, Ibrahim A, Zafar F, Sultan T. Etiology and outcomes of convulsive status epilepticus in children. Pak J Med Sci. 2019;35(3):---------. doi: https://doi.org/10.12669/pjms.35.3.120 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Sign in / Sign up

Export Citation Format

Share Document