Focal EEG Abnormalities in Heidenhain's Variant of Jakob-Creutzfeldt Disease

Furlan, Anthony J.; Henry, Charles E.; Sweeney, Patrick J.; Mitsumoto, Hiroshi

doi:10.1001/archneur.1981.00510050078015

Cited by 33 publications

(17 citation statements)

References 6 publications

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“…Occipital impairment and visual symptoms are most prominently found in the Heidenhain variant [14,29]. Visual impairment was correlated to hypometabolism in this region in three of four own cases.…”

Section: Discussionmentioning

confidence: 75%

Positron emission tomography with [ 18 F]FDG in the diagnosis of Creutzfeldt-Jakob disease (CJD)

Henkel

Zerr

Hertel

et al. 2002

Journal of Neurology

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The aim of this study was to explore the sites of metabolic changes with [(18)F]2-fluoro-2-desoxy-D-glucose (FDG) and positron emission tomography (PET) in patients with Creutzfeldt-Jakob disease and to correlate the findings with clinical symptoms. Static [(18)F]FDG-PET studies of eight patients with the diagnosis of confirmed or probable CJD were retrospectively analysed by two physicians from departments of nuclear medicine independently with a strong interrater agreement (kappa=0,98). The clinical data of the patients, based on a standardized evaluation by physicians from the German Creutzfeldt-Jakob disease surveillance study, was correlated with the PET findings. [(18)F]FDG-PET shows widespread hypometabolism in CJD. All patients had a reduction of cerebral glucose metabolism in at least one temporal or parietal region. Additionally in 7 of our own 8 cases and 3 of 4 cases from the literature the occipital lobe, the cerebellum or the basal ganglia were involved. These findings differ from typical patterns of hypometabolism in Alzheimer's disease and other neurodegenerative disorders. In two thirds of the cases the distribution was markedly asymmetric. Myoclonus was present in five out of our eight own cases. Our data suggest that myoclonus might correlate with metabolic impairment of contralateral parietal and temporal lobes. In three of four patients with visual symptoms FDG uptake was reduced in the visual cortex bilaterally. Typical hyperintensities on MRI were only found in two of the eight cases at the time of PET-studies. Our results demonstrate that [(18)F]FDG-PET appears to be a sensitive investigation in CJD and could be useful to differentiate CJD from other neurodegenerative disorders.

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Section: Discussionmentioning

confidence: 75%

Positron emission tomography with [ 18 F]FDG in the diagnosis of Creutzfeldt-Jakob disease (CJD)

Henkel

Zerr

Hertel

et al. 2002

Journal of Neurology

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“…PSWCs in CJD may be unilateral 6, 24–28. This may reflect a state of the disease when the commissural progress, which has been shown in experiments,29 has not yet led to the diffuse cortical disease 27, 28.…”

Section: Discussionmentioning

confidence: 89%

“…Because the diagnosis is proved only post mortem and sCJD always has a fatal course, it is essential to establish diagnostic intra vitam criteria as sensitive and especially as specific as possible. For decades, along with the widely accepted clinical criteria,4 periodic sharp‐wave complexes (PSWCs) in the electroencephalogram (EEG) were reported to represent the most typical finding in the course of sCJD5–9 and therefore were included in the World Health Organization classification criteria of sCJD 4…”

mentioning

confidence: 99%

Diagnostic value of periodic complexes in Creutzfeldt–Jakob disease

Steinhoff

Zerr

Glatting

et al. 2004

Annals of Neurology

198

111

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In 1996, our group published objective electroencephalogram (EEG) criteria to define periodic sharp-wave complexes (PSWCs) suggestive for Creutzfeldt-Jakob disease (CJD). These criteria have since then been strictly applied in all cases reported to us as possible CJD in the course of the German CJD surveillance study. Furthermore, EEG analysis of the records was performed without any additional information on complementary clinical and laboratory data. In this study, we investigated sensitivity, specificity, and the predictive values of these EEG criteria exclusively in cases in which autopsy confirmed (n=150) or excluded (n=56) CJD. EEG criteria were positive in 64% (n=96) of the CJD cases and falsely positive in 9% (n=5) of other dementias. The resulting figures for sensitivity, specificity, and positive and negative predictive values were 64%, 91%, 95%, and 49%, respectively. In the falsely positive cases, Alzheimer's disease (n=4) and vascular dementia (n=1) were the underlying diseases. However, only in one of these five cases both clinical and EEG data would have led to the false-positive result to diagnose probable CJD. These data prove the high diagnostic value of our objective EEG criteria in CJD.

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“…With regards to the EEG, a periodic sharp wave complex (PSWC) is detected in approximately 67% of sCJD patients, and is especially noted for those patients whose codon 129 polymorphism contains methionine (i.e. MM, MV) (Furlan et al ., 1981;Levy et al ., 1986;Steinhoff et al ., 1996;Wieser et al ., 2006). The appearance of PSWC on the EEG is not specifi c for CJD, as this pattern has been noted on EEG examination of other forms of dementia (e.g.…”

Section: Non-surgical Diagnostic Evaluation Of the Patientmentioning

confidence: 99%

Prions and endotoxins: reprocessing strategies for reusable medical devices

Sehulster

2012

Sterilisation of Biomaterials and Medical Devices

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Focal EEG Abnormalities in Heidenhain's Variant of Jakob-Creutzfeldt Disease

Cited by 33 publications

References 6 publications

Positron emission tomography with [ 18 F]FDG in the diagnosis of Creutzfeldt-Jakob disease (CJD)

Positron emission tomography with [ 18 F]FDG in the diagnosis of Creutzfeldt-Jakob disease (CJD)

Diagnostic value of periodic complexes in Creutzfeldt–Jakob disease

Prions and endotoxins: reprocessing strategies for reusable medical devices

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