Creutzfeldt-Jakob disease: Which diffusion-weighted imaging abnormality is associated with periodic EEG complexes?

Kandiah, Nagaendran; K, Nagaendran; Tan, Kevin; Pan, Andrew; Au, Wing Lok; Venketasubramanian, Narayanaswamy; Lim, C. C. Tchoyoson; Tan, Ngan Meng

doi:10.1007/s00415-008-0934-3

Cited by 20 publications

(20 citation statements)

References 9 publications

(10 reference statements)

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“…Our patients with probable CJD showed a wide range of age at onset (range 54–80 years, mean 73.5 years). The results are fairly consistent with those of previous studies that focused on asymmetry in clinical, EEG, and MRI abnormalities [2,8,9], but the mean age at symptom onset of our patients was somewhat higher than that of the patients in the previous studies. The asymmetry has been detected at an early stage in 40% of the patients with the MM1 and MV1 subtypes [7].…”

Section: Discussionsupporting

confidence: 92%

“…In addition, a high consistent correlation was noted among clinical presentation and PSWC and brain MRI-DWI findings in our patients. Kandiah et al [8] suggested that EEG-PSWC findings were well correlated with cortical lesions on brain MRI-DWI scans, but not with striatum lesions in probable and possible CJD patients. In addition, they also found that 11 out of 14 patients showed an asymmetric distribution of the lesions on DWI.…”

Section: Discussionmentioning

confidence: 99%

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Asymmetric Involvement in Sporadic Creutzfeldt-Jakob Disease: Clinical, Brain Imaging, and Electroencephalographic Studies

Huang

et al. 2010

Eur Neurol

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Objective: To ascertain the characteristics of patients with sporadic Creutzfeldt-Jakob disease (CJD) and to determine the findings of electroencephalography (EEG) and brain magnetic resonance imaging (MRI). Methods: We pooled patients at a hospital from 2000 to 2008, and classified them according to WHO diagnostic criteria as having probable or possible CJD. We retrospectively analyzed their clinical manifestations, brain MRI, and EEG findings to evaluate correlations among them. Results: In this study, 12 probable and 4 possible CJD patients were identified. Ten patients with probable CJD had asymmetric manifestations with hemiparesis, focal myoclonus, dystonia or apraxia; 9 had clinical manifestations mimicking the corticobasal syndrome. In contrast, neurological examinations did not show asymmetric signs in 4 patients with possible CJD. EEG showed a typical periodic sharp wave complex (PSWC) in 12 patients with probable CJD; most of them had bright signal intensity on diffusion-weighted imaging of the cortex and/or basal ganglia. There was a high tendency for asymmetric clinical manifestations that correlated with the presentation of PSWC and cortical lesions observed on the brain MRI scan. Conclusions: Our study indicates that asymmetric extrapyramidal symptoms/signs, in clinical features with characteristic abnormalities on MRI and EEG findings, might contribute to early diagnosis of sporadic CJD.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Asymmetric Involvement in Sporadic Creutzfeldt-Jakob Disease: Clinical, Brain Imaging, and Electroencephalographic Studies

Huang

et al. 2010

Eur Neurol

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“…Restricted diffusion and DW-MRI signal hyperintensity have been found in the basal ganglia, thalamus, and cortex of CJD patients (60). Again, the proposed explanations for restricted diffusion vary with the CJD subtype but may be related to intraneuronal microvacuolation (57), prion protein deposition, and spongiform change (61). The combination of DW-MRI with FLAIR imaging has sensitivity and specificity reported at Ͼ91 to 98% (59,62).…”

Section: Discussionmentioning

confidence: 99%

“…The combination of DW-MRI with FLAIR imaging has sensitivity and specificity reported at Ͼ91 to 98% (59,62). Those sequences are important for early detection to inform appropriate diagnostic testing (61) and to enable timely intervention as new therapies are developed (59). Although only a small number of studies have used MRI to investigate animal models of prion disease, it has been shown that focal blood-brain barrier disruption occurs in a hamster scrapie model (63), that there are increases in T2 measurements in the septum, hippocampus, thalamus, and cortex of mice inoculated with 139A scrapie (64), and that the diffusion of tissue water was significantly reduced in the late preclinical period in mice inoculated with ME7 scrapie (65).…”

Section: Discussionmentioning

confidence: 99%

Susceptibility of Domestic Cats to Chronic Wasting Disease

Mathiason

Nalls

Seelig

et al. 2013

J Virol

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“…[16] Difüzyon MRG'de kortikal sinyal artışı olan hastaların hepsinde EEG'de periyodik deşarjlar gözlenmektedir. [20] Beyin omurilik sıvısında 14-3-3 proteininin saptanması CJH tanısını destekler. [2] Çeşitli çalışmaların sonucunda 14-3-3 proteininin hastalık için ortalama duyarlılık ve özgüllüğü %92 olarak hesaplanmıştır.…”

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Atypical Sporadic Creutzfeldt-Jakob disease mimicking nonconvulsive status epilepticus

Uca¹,

Genç²,

Güney³

et al. 2014

Epilepsi

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ÖzetSporadik Creutzfeldt-Jakob hastalığı (sCJH) prion hastalıkları (insan bulaşıcı subakut spongioform ensefalopatiler) arasında en sık görülenidir. Miyoklonus, ilerleyici demans ve periyodik elektroensefalografi aktivitesi gibi karakteristik bulguların birlikte olduğu hastada tanıyı koymak kolay olabilir. Olağandışı belirti ve bulguların ilave olduğu olgularda tanı koymada sorunlar ortaya çıkabilir. Bu yazıda, depresyon, demans, refrakter nonkonvulzif status epileptikus ön tanılarıyla izlenen ve daha sonra sCJH tanısı konan 65 yaşında bir kadın hasta sunuldu.Anahtar sözcükler: Creutzfeldt-Jakob hastalığı; nonkonvulzif status epileptikus; prion hastalığı. SummarySporadic Creutzfeldt-Jakob disease (sCJD) is the most common disorder among the very rare prion diseases (human transmissible subacute spongiform encephalopathies). The diagnosis might be easy if a patient with characteristics features of myoclonus, progressive dementia and periodic electroencephalography activity. Diagnosis of some patients with unusual symptoms and signs may be challenging. The reported case is a 65 years old female patient who was finally diagnosed as CJD, after previously being misdiagnosed with depression, dementia and refractory nonconvulsive status epilepticus.

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Creutzfeldt-Jakob disease: Which diffusion-weighted imaging abnormality is associated with periodic EEG complexes?

Abstract: Serial EEGs are not useful for patients with isolated striatal DWI hyperintensity but will increase diagnostic certainty from "possible" to probable" CJD for patients with cortical DWI hyperintensity.

Cited by 20 publications

References 9 publications

Asymmetric Involvement in Sporadic Creutzfeldt-Jakob Disease: Clinical, Brain Imaging, and Electroencephalographic Studies

Asymmetric Involvement in Sporadic Creutzfeldt-Jakob Disease: Clinical, Brain Imaging, and Electroencephalographic Studies

Susceptibility of Domestic Cats to Chronic Wasting Disease

Atypical Sporadic Creutzfeldt-Jakob disease mimicking nonconvulsive status epilepticus

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