EEG–EMG polygraphic study of dystonia and myoclonus in a case of Creutzfeldt–Jakob disease

Hashimoto, Takao; Iwahashi, Teruaki; Ishii, Wataru; Yamamoto, Kanji; Ikeda, Shu‐ichi

doi:10.1016/j.ebcr.2015.05.002

Cited by 4 publications

(3 citation statements)

References 11 publications

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“…Periodic rhythmic synchronized myoclonus has been suspected due to a hyperexcitable cortico-subcortical loop ( 26 , 27 ). Furthermore, myoclonus occurred more frequently and earlier in MM type at codon 129 of the prion protein gene compared to other codon 129 polymorphism ( 9 , 28 ). This genotype was considered to have the shortest survival compared to MV and VV forms of the human PRNP gene ( 7 , 9 ).…”

Section: Discussionmentioning

confidence: 83%

Predictive Factors for Early Initiation of Artificial Feeding in Patients With Sporadic Creutzfeldt-Jakob Disease

Hsieh

Chang

et al. 2018

Front. Neurol.

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Background: Akinetic mutism has often been used as the predictor of sporadic Creutzfeldt-Jacob disease (sCJD) endpoints, but it may be difficult for general physcians to assess. Nasogastric (NG) tube insertion is indicated for many neurodegenerative diseases with a clinical course of swallowing failure, and can be more easily identified than akinetic mutism by general physicians. Therefore, the aim of this study was to identify whether there are predictive factors for early initiation of artificial feeding in patients with sCJD who require enteral nutrition due to swallowing failure.Methods: We retrospectively reviewed the medical records of all patients diagnosed with probable sCJD who were admitted to the neurology ward at a medical center in Taiwan from January 2002 to July 2017. We used Pearson's chi-squared test to detect the correlation of initial symptoms, neurological signs, brain magnetic resonance imaging (MRI), electroencephalography (EEG), and increased levels of 14-3-3 protein in cerebrospinal fluid (CSF) analysis. The Cox proportional hazards model was used to detect prognostic factors for early initiation of NG tube insertion in sCJD patients.Results: The onset age ranged from 51 to 83 years, and mostly ranged from 60 to 79 years. Akinetic mutism was correlated with pyramidal tract signs, myoclonus, and extrapyramidal signs. Furthermore, myoclonus was revealed to be associated with pyramidal tract signs. Multivariate Cox regression analysis showed that myoclonus and elevated CSF levels of 14-3-3 protein are predictive of early NG insertion.Conclusions: Increased levels of 14-3-3 protein in CSF and the presence of myoclonus at diagnosis are predictive of early swallowing difficulty and indicate rapid deterioration in probable sCJD. In addition to akinetic mutism, early initiation of artificial feeding can be used to predict early deterioration in sCJD.

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Section: Discussionmentioning

confidence: 83%

Predictive Factors for Early Initiation of Artificial Feeding in Patients With Sporadic Creutzfeldt-Jakob Disease

Hsieh

Chang

et al. 2018

Front. Neurol.

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“…The three cases of the present study were subjected to serial EEG throughout the course of the disease and not all of them had typical PSWC; in the first case, PSWC was not detected, while it rapidly developed in the other two cases at different stages. According to a previous study, EEGs are normal in numerous cases of CJD at the early stage; however abnormal EEG findings may differ among different CJD subtypes (23). Abnormal EEG findings occur in patients with M homozygosis and M/V heterozygosis but rarely in patients with V homozygosis at codon 129 of the PRNP (24).…”

Section: Discussionmentioning

confidence: 99%

Three sporadic cases of Creutzfeldt-Jakob disease in China and their clinical analysis

Wang

Liu

et al. 2017

Experimental and Therapeutic Medicine

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Abstract. The present study described the characteristics of three cases of Creutzfeldt-Jakob disease (CJD) in China and analyzed their clinical presentations. The clinical information of the three cases was collected and analyzed. Blood and cerebrospinal fluid (CSF) specimens of the patients were collected for detection of the prion protein (PRNP) gene and 14-3-3 protein levels. Dynamic changes of electroencephalograms (EEGs) and brain magnetic resonance images (MRIs) were also observed. All the three cases were sporadic CJD cases. They presented with symptoms including hyposthenia, progressive memory loss, truncal and limb ataxia, dysarthria, lowered vision acuity, bucking, language disorders, myoclonia and akinetic mutism state. One of the three cases was associated with a prolonged duration of >6 years. The EEG of two cases showed slow biphasic waves. The diffusion-weighted MRI sequence revealed abnormal hyperintensity and bilateral ribboning in the cortex. Two patients tested positive for the 14-3-3 protein in the CSF. All patients were of methionine homozygosity at codon 129 in the gene encoding PRNP protein and one patient had a mutation. The CJD cases showed differences in terms of symptoms and disease duration. Subacute onset was common and with attentive nursing and supportive treatments, one of the patients had a prolonged survival time of >6 years. IntroductionCreutzfeldt-Jakob disease (CJD) is a progressive neurodegenerative disorder that is caused by prion protein deposition in the central nervous system. As the most common type of human transmissible spongiform encephalopathy, CJD is incurable and fatal with a duration from a few months to two years (1). Typical CJD triad symptoms include progressive dementia, myoclonus and electroencephalographic (EEG) changes (periodic triphasic sharp wave complexes) (2). Traditionally, CJD is classified as sporadic (sCJD), familial (fCJD), iatrogenic (iCJD) and variant CJD (vCJD) (3). Different subtypes are associated with specific clinical symptoms and pathological features (4,5). sCJD, accounting for ~85% of all cases worldwide, is categorized into six subtypes according to the methionine (M)/valine (V) polymorphism at codon 129 of the PRNP gene (6,7). fCJD accounts for 5-10% of all cases and is an autosomal dominant disorder associated with various mutations of the PRNP gene (8,9). The main causes of iCJD include contaminated human hormone supplements, corneal transplantation and transplantation of cadaveric dura mater (10). At present, vCJD, which is transmitted from cows with bovine spongiform encephalopathy to humans, mainly affects younger adults in Europe with different clinical, etiological and neuropathological features as well as risk factors compared with other types of CJD (11-13).Although final diagnosis of CJD requires neuropathological biopsy examination of affected brain tissues, nonpathological methods are currently used worldwide for the early diagnosis and prevention of CJD (14). At present, the diagnosis of CJD is frequently delayed or CJD ...

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“…Hashimoto, T. Iwahashi, W. Ishii, K. Yamamoto, and S.I. Ikeda, [12] evaluated a patient's sporadic Creutzfeldt-Jakob Disease (CJD) who showed dystonia, periodic myoclonus, and Periodic Sharp Wave Complexes (PSWCs) on EEG. The EEG-EMG polygraphic study revealed that dystonia appeared without relation to periodic myoclonus and PSWCs and that dystonia EMGs were strongly suppressed after periodic myoclonus EMGs.…”

Section: Literature Reviewmentioning

confidence: 99%

Evaluation of Knee Activities Using EMG Signals for Pre-predicting Lower Limb Dystonia Diseases

Balasubramanyam¹,

Kalappan²

2018

IJIES

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Electromyography (EMG) signal is one of the important tools for the detection of skeletal related muscular information. In this scenario, we analyse the normality and abnormality of knee movements for pre-predicting the knee dystonia diseases. It is characterized by involuntary sustained muscle contractions affecting one or more sides of the body, frequently causing twisting and repetitive movements or abnormal postures. In this paper, EMG based knee dystonia pre-prediction is assessed by employing EMG-Lower Limb dataset. Then, transformation is done by using discrete wavelet Transformation (DWT), which delivers a superior time and frequency localization ability. After transformation, hybrid feature extraction is performed by employing yule-walker, burg's, voltage level (minimum and maximum), Renyi entropy, and Peak-Magnitude to Root Mean Square Ratio (PMRS) for achieving optimal feature subsets and also to reject the redundant and irrelevant features. In most of the existing studies, an individual feature or two different features are combined for extracting the feature values from the acquired signal. In this research, five effective entropy and autoregressive features are combined to obtain more active features. After obtaining the active features, a superior binary classifier: Kernel Nearest Neighbour (KNN) is implemented for classifying the normality and abnormality of knee movements. The experimental outcome proves that the proposed methodology effectively distinguishes the normal and abnormal knee movement in terms of sensitivity, specificity, recall, precision, accuracy and E-rate. The proposed methodology improves the classification accuracy in knee movement detection upto 0.785-1.65% compared to the existing methods.

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EEG–EMG polygraphic study of dystonia and myoclonus in a case of Creutzfeldt–Jakob disease

Cited by 4 publications

References 11 publications

Predictive Factors for Early Initiation of Artificial Feeding in Patients With Sporadic Creutzfeldt-Jakob Disease

Predictive Factors for Early Initiation of Artificial Feeding in Patients With Sporadic Creutzfeldt-Jakob Disease

Three sporadic cases of Creutzfeldt-Jakob disease in China and their clinical analysis

Evaluation of Knee Activities Using EMG Signals for Pre-predicting Lower Limb Dystonia Diseases

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