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

(5 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…After the disease progressed, the patient developed seizures. A perfusion SPECT scan showed hypoperfusion of the right frontoparietal cortex, consistent with the study by Henkel and colleagues (Nagasaka et al 2011). DAT binding was bilaterally reduced in the striatum of patients with CJD (Chen et al 2010;Ragno et al 2009) and tracer binding to the GABA-A receptor was reduced throughout the entire cerebral cortex, but not in the cerebellum (Itoh et al 1998).…”

Section: Prion Diseasesupporting

confidence: 87%

PET and SPECT Imaging in Hyperkinetic Movement Disorders

Zoons

Sharifi

Booij³

et al. 2020

PET and SPECT in Neurology

View full text Add to dashboard Cite

Movement disorders can be classified in hypokinetic (e.g., Parkinson's disease, PD) and hyperkinetic disorders (e.g., dystonia, chorea, tremor, tics, myoclonus, and restless legs syndrome). In this chapter, we will discuss results from positron emission tomography (PET) and single photon emission computed tomography (SPECT) imaging studies in patients with tremor, tics, myoclonus, and restless legs syndrome.Most studies in patients with tremor included patients with essential tremor (ET): a bilateral, largely symmetric, postural or kinetic tremor mainly involving the upper limbs and sometimes the head. Other studies evaluated patients with orthostatic tremor (OT): an unusually high frequent tremor in the legs that mainly occurs when patients are standing still. Increased regional cerebral blood flow (rCBF) and increased glucose metabolism have been found in the cerebellum, sensorimotor cortex, and thalamus in both patients with ET and OT compared to controls. Both PET and SPECT studies have evaluated the dopamine system in patients with ET and OT. Most imaging studies in patients with ET showed no, or only subtle loss of striatal tracer binding to the dopamine transporter indicating that ET is not characterized by nigrostriatal cell loss. The serotonin and/or gamma-aminobutyric acid (GABA) systems may play a role in the pathophysiology of ET. PET and SPECT imaging of the dopamine and serotonin system in patients with OT showed no abnormalities.Tics, the clinical hallmark of Gilles de la Tourette syndrome (TS), are relatively brief and intermittent involuntary movements (motor tic) and sounds (phonic tic). The essential features of tics are that (1) they can be temporarily suppressed; after suppression a rebound usually occurs with a flurry of tics; (2) the patient experiences an urge to tic, and (3) the tic is followed by a short moment of relief. Using 18 F-FDG PET, it was shown that TS is a network disor-E. Zoons et al.

show abstract

Section: Prion Diseasesupporting

confidence: 87%

PET and SPECT Imaging in Hyperkinetic Movement Disorders

Zoons

Sharifi

Booij³

et al. 2020

PET and SPECT in Neurology

View full text Add to dashboard Cite

show abstract

“…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.

216

186

View full text Add to dashboard Cite

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.

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

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

Cited by 11 publications

References 12 publications

PET and SPECT Imaging in Hyperkinetic Movement Disorders

PET and SPECT Imaging in Hyperkinetic Movement Disorders

Insulin Resistance and Alzheimer’s Disease: Bioenergetic Linkages

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

Contact Info

Product

Resources

About