scholarly journals Psychiatric Symptoms as Bases for Differential Diagnosis of Pre-mortem Sporadic Creutzfeldt-Jakob Disease and Anti-N-Methyl D-aspartate Receptor Encephalitis

2018 ◽  
Vol 08 (01) ◽  
Author(s):  
Yujia Luo ◽  
Xi Cheng ◽  
Yang Jiao ◽  
Huibin Wang ◽  
Yifan Liang ◽  
...  
2020 ◽  
Vol 11 ◽  
Author(s):  
Jiao Liu ◽  
Liyan Chen ◽  
Jing Yang ◽  
Lan Wang ◽  
Huifang Shang ◽  
...  

Objectives: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and sporadic Creutzfeldt–Jakob disease (sCJD) share similar clinical features. Here, we present two unusual cases of anti-NMDAR encephalitis who were misdiagnosed as sCJD at first.Methods: We described two patients' clinical manifestations, as well as the string of symptomatological evolution, treatments, and follow-up results.Results: Our patients presented with rapidly progressive dementia, memory problems, psychiatric symptoms, and movement disorders, and we considered all these symptoms as a presenting feature of sCJD at first, but the cerebrospinal fluid examination showed positive results for both the 14-3-3 protein and antibodies against NMDAR. Immunomodulatory treatment led to a resolution of these deficits, and both of them remained in remission after treatment.Conclusion: Anti-NMDAR encephalitis can present with rapidly progressive cognitive decline, and sometimes laboratory investigations can be misleading. The examination for the presence of NMDAR antibodies is necessary, even with the presence of 14-3-3 protein. Early immunomodulatory therapy should be considered, especially for patients with high titers of NMDAR antibodies.


2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Iria Grande ◽  
Juan Fortea ◽  
Ellen Gelpi ◽  
Itziar Flamarique ◽  
Marc Udina ◽  
...  

We describe a case report of an 80-year-old woman who presented with symptomatology compatible with an episode of major depression with catatonia. After psychiatric admission, electroconvulsive therapy (ECT) was applied, but symptoms progressed with cognitive impairment, bradykinesia, widespread stiffness, postural tremor, and gait disturbance. After compatible magnetic resonance imaging (MRI), diffusion changes, and electroencephalogram (EEG) findings the case was reoriented to Creutzfeldt-Jakob disease (CJD). The genetic study found a methionine/valine heterozygosity at codon 129 of the prion protein gene PrPSc. On followup, a significant clinical recovery turned out. For this reason, EEG and MRI were repeated and confirmed the findings. The patient subsequently demonstrated progressive clinical deterioration and died 21 months later. The diagnosis was verified postmortem by neuropathology. The vCJD subtype MV2 is indeed characterized by early and prominent psychiatric symptoms and a prolonged disease duration however no frank clinical recovery has before been reported.


2016 ◽  
Vol 29 (6) ◽  
pp. 436-439 ◽  
Author(s):  
Pierre-Luc Gamache ◽  
Maude-Marie Gagnon ◽  
Martin Savard ◽  
François Émond

This article reports the case of a 68-year-old patient with anti-HU antibodies paraneoplastic encephalitis. The clinical manifestations were atypical and the paraclinical work-up, notably the magnetic resonance imaging (MRI) showing bilateral posterior thalamic hyperintensities (pulvinar sign), misleadingly pointed towards a variant Creutzfeld–Jakob disease. After presenting the case, the differential diagnosis of the pulvinar sign is discussed along with other important diagnostic considerations.


Author(s):  
James Badenoch ◽  
Tamara Searle ◽  
Iona Watson ◽  
Andrea E. Cavanna

Abstract Background Movement disorders have been described in the context of different types of encephalitis. Among hyperkinetic manifestations, tics have sporadically been reported in cases of encephalitis resulting from a range of aetiologies. Objective This review aimed to assess the prevalence and characteristics of tics in patients with encephalitis. Methods We conducted a systematic literature review of original studies on the major scientific databases, according to the standards outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Results In addition to the established association between tics and encephalitis lethargica, our literature search identified reports of tics in patients with immune-mediated pathologies (including autoimmune encephalitides affecting the N-methyl-d-aspartate receptor, voltage-gated potassium channels, and glycine receptors) and infective processes (ranging from relatively common viral pathogens, such as herpes simplex, to prions, as in Creutzfeldt-Jakob disease). Tics were most commonly reported in the post-encephalitic period and involvement of the basal ganglia was frequently observed. Discussion The association of new-onset tics and encephalitis, in the background of other neuropsychiatric abnormalities, has practical implications, potentially improving the detection of encephalitis based on clinical features. Future research should focus on the categorisation and treatment of hyperkinetic movement disorders associated with encephalitis.


2018 ◽  
Vol 24 (2) ◽  
pp. 175-176
Author(s):  
Yasemin Dinç ◽  
Ezgi Sezer Eryıldız ◽  
Atilla Özcan Özdemir

2016 ◽  
Vol 4 (2) ◽  
pp. 72-75
Author(s):  
Arthur Joseph ◽  
Jacob Core ◽  
Daniel Solano ◽  
Marquand Patton ◽  
Shaun Smart

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


2016 ◽  
Vol 33 (S1) ◽  
pp. S348-S348
Author(s):  
A. Cercos López ◽  
M.C. Cancino Botello ◽  
V. Chavarria Romero ◽  
G. Sugranyes Ernest

IntroductionAnti-NMDA encephalitis normally appears as a characteristic syndrome with typical symptoms that undergoes with multiphase evolution. However, it sometimes develops atypical symptoms so we must perform a careful differential diagnosis.ObjectivesTo conduct a current review of detection and management of anti-NMDAr encephalitis, and psychiatric manifestations.MethodSystematic review of the literature in English (PubMed), with the following keywords: “Autoimmune encephalitis”, “psychosis”, and “NMDA receptor”.ResultsWe present the case of a 15-year-old boy referred to evaluation for a first psychotic episode. He had no past history of psychiatric illness or substance abuse. The only relevant antecedent is multiple sclerosis in a first degree relative. For the last months, he presented high levels of anxiety symptoms apparently related to college stressful events and fluctuating hypoesthesia of left cranial side. Days later, it appeared autolimited gastrointestinal symptoms, headache and fever. During the next days it appeared psychomotor retardation, choreic movements, suicide ideation and mood-congruent paranoid and nihilistic ideation, auditory and visual hallucinations, perplexity and catatonic symptoms so he was hospitalized. We observed cognitive functions impairment, unsteady gait, dysartria, dysphasia, clonus and left babinsky sign. EEG showed slow waves on right frontal area. CFS showed protein elevation and immunologic study revealed positive anti-NMDA antibodies. Treatment with methylprednisolone and gammaglobuline was started with partial response, needing addition of rituximab.ConclusionsIn this case, we highlight the importance of early detection and a detailed differential diagnosis, to determine whether the etiology of psychiatric symptoms in order to achieve an accurate and early treatment.Disclosure of interestThe authors have not supplied their declaration of competing interest.


Author(s):  
Ellen E. Lee ◽  
Baichun Hou ◽  
Ipsit V. Vahia ◽  
Dilip V. Jeste

Late-onset schizophrenia remains an understudied subtype of schizophrenia, despite growing recognition of its impact and distinction from early-onset schizophrenia. This chapter reviews the existing literature on late-onset schizophrenia including beginning with the nomenclature and epidemiology. Then we provide a review of key risk factors and correlates—including genetic risk, sex differences, comorbid sensory loss and physical illness, cognitive and psychiatric symptoms, sociodemographic factors, adverse life events, neuropathology, and inflammation. The chapter ends with clinical issues, including symptoms, differential diagnosis, treatments, and prognosis. Recent studies have examined the role of oestrogen treatments and a new therapy for tardive dyskinesia therapy as well as inflammatory mechanisms in schizophrenia.


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