Cerebral Hypermetabolism Demonstrated by FDG PET in Familial Creutzfeldt-Jakob Disease

Nagasaka, Takamura; Nagasaka, Kaori; Ohta, Emiko; Shindo, Kazumasa; Takiyama, Yoshihisa; Shiozawa, Zenji; Miyazawa, Nobuyuki; Yamasaki, Nobuhiko; Mori, Nobuhiko; Onda, Hideaki; Shinohara, Toyoaki

doi:10.1097/rlu.0b013e31821a2604

Cited by 11 publications

(4 citation statements)

References 12 publications

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“…If this was an isolated phenomenon, it would be easy to dismiss. However, it has been reported in conditions from Alzheimer’s, Parkinson’s, Huntington’s and ALS, to Down Syndrome, Friedrich’s Ataxia and familial Creutzfeldt-Jakob disease in disease-specific patterns (Gilman et al, 1990 ; Haier et al, 2003 ; Nagasaka et al, 2011 ; Borghammer et al, 2012 ; Cistaro et al, 2012 ; Lee et al, 2012 ; Ashraf et al, 2015 ).…”

Section: Discussionmentioning

confidence: 99%

Insulin Resistance and Alzheimer’s Disease: Bioenergetic Linkages

Neth

Craft

2017

Front. Aging Neurosci.

191

176

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Metabolic dysfunction is a well-established feature of Alzheimer’s disease (AD), evidenced by brain glucose hypometabolism that can be observed potentially decades prior to the development of AD symptoms. Furthermore, there is mounting support for an association between metabolic disease and the development of AD and related dementias. Individuals with insulin resistance, type 2 diabetes mellitus (T2D), hyperlipidemia, obesity, or other metabolic disease may have increased risk for the development of AD and similar conditions, such as vascular dementia. This association may in part be due to the systemic mitochondrial dysfunction that is common to these pathologies. Accumulating evidence suggests that mitochondrial dysfunction is a significant feature of AD and may play a fundamental role in its pathogenesis. In fact, aging itself presents a unique challenge due to inherent mitochondrial dysfunction and prevalence of chronic metabolic disease. Despite the progress made in understanding the pathogenesis of AD and in the development of potential therapies, at present we remain without a disease-modifying treatment. In this review, we will discuss insulin resistance as a contributing factor to the pathogenesis of AD, as well as the metabolic and bioenergetic disruptions linking insulin resistance and AD. We will also focus on potential neuroimaging tools for the study of the metabolic dysfunction commonly seen in AD with hopes of developing therapeutic and preventative targets.

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Section: Discussionmentioning

confidence: 99%

Insulin Resistance and Alzheimer’s Disease: Bioenergetic Linkages

Neth

Craft

2017

Front. Aging Neurosci.

191

176

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“…This patient showed abnormal cortical hyperperfusion on ECD‐SPECT and ASL images during the interictal phase. Previously, Nagasaka et al reported cortical hypermetabolism on PET in a patient with familial CJD with an E200K mutation of the prion protein who exhibited focal epileptic seizures 11 . Typical cortical lesions in CJD without seizure show hypoperfusion on SPECT 12 .…”

Section: Discussionmentioning

confidence: 98%

“…Nagasaka et al reported cortical hypermetabolism on PET in a patient with familial CJD with an E200K mutation of the prion protein who exhibited focal epileptic seizures. 11 Typical cortical lesions in CJD without seizure show hypoperfusion on SPECT. 12 Conversely, the epileptic focus shows hyperperfusion during the ictal phase on ECD-SPECT images in patients with epilepsy.…”

Section: Discussionmentioning

confidence: 99%

Bilateral tonic–clonic seizure and focal cortical hyperexcitability in familial Creutzfeldt‐Jakob disease with E200K mutation of the prion protein

Kawaguchi

Motoda

Terada

et al. 2023

Epileptic Disorders

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Convulsive epileptic seizures are rare in Creutzfeldt-Jakob disease (CJD), and their clinical and EEG features have not been reported in detail. We describe a case of familial CJD with an E200K mutation of the prion protein who presented with bilateral tonic-clonic seizures (BTCS) during long-term video-EEG monitoring. Semiologically, BTCS showed focal clinical signs such as head turning and eye deviation to the left. The ictal EEG started with generalized polyspikes. Interictal EEG showed generalized periodic discharges with right fronto-temporal predominance (larger amplitude and earlier onset compared with other regions). MRI showed high-intensity signals persistently in the right temporo-parietal region on diffusion-weighted images (DWI). Interictal single-photon emission computed tomography (SPECT) showed hyperperfusion in the same region. Brain pathology revealed typical spongiform changes in CJD without other pathological findings of rapidly progressive dementia. Our case demonstrates that CJD can cause BTCS with generalized EEG changes and focal semiological/imaging abnormalities, suggesting that diffuse and inhomogeneous cortical and subcortical epileptic networks may develop in familial CJD.

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“…Only a few cases have been reported in terms of the FDG-PET findings of Creutzfeldt-Jakob disease (Engler et al, 2003;Henkel et al, 2002;Nagasaka et al, 2011). Study with FDG-PET only found that all 8 patients had reduction of CMRgl in at least one temporal or parietal region.…”

Section: Creutzfeldt-jakob Diseasementioning

confidence: 99%