technetium-tc-99m-bicisate and Creutzfeldt-Jakob-Syndrome

To clinically diagnose MM2-cortical (MM2C) and MM2-thalamic (MM2T)-type sporadic Creutzfeldt-Jakob disease (sCJD) at early stage with high sensitivity and specificity.. We reviewed the results of Creutzfeldt-Jakob disease Surveillance Study in Japan between April 1999 and September 2019, which included 254 patients with pathologically confirmed prion diseases, including 9 with MM2C-type sCJD (MM2C-sCJD) and 10 with MM2T-type sCJD (MM2T-sCJD), and 607 with non-prion diseases.. According to the conventional criteria of sCJD, 4 of 9 patients with MM2C- and 7 of 10 patients with MM2T-sCJD could not be diagnosed with probable sCJD until their death. Compared with other types of sCJD, patients with MM2C-sCJD showed slower progression of the disease and cortical distribution of hyperintensity lesions on diffusion-weighted images of brain MRI. Patients with MM2T-sCJD also showed relatively slow progression and negative results for most of currently established investigations for diagnosis of sCJD. To clinically diagnose MM2C-sCJD, we propose the new criteria; diagnostic sensitivity and specificity to distinguish 'probable' MM2C-sCJD from other subtypes of sCJD, genetic or acquired prion diseases and non-prion disease controls were 77.8% and 98.5%, respectively. As for MM2T-sCJD, clinical and laboratory features are not characterised enough to develop its diagnostic criteria.. MM2C-sCJD can be diagnosed at earlier stage using the new criteria with high sensitivity and specificity, although it is still difficult to diagnose MM2T-sCJD clinically.

Topics: Adult; Aged; Aged, 80 and over; Case-Control Studies; Cerebral Cortex; Cerebrovascular Circulation; Creutzfeldt-Jakob Syndrome; Cysteine; Diffusion Magnetic Resonance Imaging; Disease Progression; Female; Fluorodeoxyglucose F18; Humans; Iofetamine; Male; Middle Aged; Organotechnetium Compounds; Prion Proteins; PrPSc Proteins; Radiopharmaceuticals; Sensitivity and Specificity; Thalamus; Tomography, Emission-Computed, Single-Photon

We report a case of autopsy-verified MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) in a 46-year-old patient with a 16-month history of abnormal behavior, progressive dementia, insomnia, and speech disturbances without family history. Neurological examination revealed progressive dementia, frontal signs, insomnia, speech disturbance, gait disturbance and bilaterally exaggerated tendon reflexes. Both brain MRI and cerebrospinal fluid examinations, including 14-3-3 protein, yielded normal results. An easy Z-score (eZIS) analysis for (99m)Tc-ethyl cysteinate dimer-single photon emission computed tomography ((99m)Tc-ECD-SPECT) revealed decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata. PRNP gene analysis revealed methionine homozygosity at codon 129 without mutation. Neuropathological examinations revealed severe neuronal loss, gliosis, and hypertrophic astrocytosis in the medial thalamus and inferior olivary nucleus. A slight depletion of Purkinje cells was observed. PrP immunostaining showed no obvious PrP deposits in the basal ganglia, thalamus, cerebellum, or brainstem; however, mild synaptic-type PrP deposits with some smaller plaque-like structures were only partially observed in the localized region of the frontal lobe with the spongiform change. Western blot analyses of protease-resistant PrP showed a type 2 pattern. In conclusion, eZIS analysis of (99m)Tc-ECD-SPECT images is useful for detecting both thalamic and medullary lesions. This is the first case of medullary lesions detected in a live patient with MM2-thalamic-type sCJD using SPECT.

Topics: Cerebrovascular Circulation; Creutzfeldt-Jakob Syndrome; Cysteine; Humans; Male; Medulla Oblongata; Middle Aged; Organotechnetium Compounds; Radiopharmaceuticals; Thalamus; Tomography, Emission-Computed, Single-Photon

Creutzfeldt-Jakob disease (CJD) is a subacute spongiform encephalopathy characterized by rapidly progressive dementia, hard to diagnose during life.. We present a case of a patient with pathologically confirmed sporadic form of CJD in whom initial diagnostic tests were negative. Two sequential brain single-photon emission computed tomography with Tc-99m ethyl-cysteinate dimer were performed, the first one was performed few days after the admission into hospital and the second, 1 month later.. Both studies revealed a decrease in regional cerebral blood flow indicative of neuronal dysfunction, more pronounced in the second study.. Current radionuclide scintigraphy can be an useful tool for the investigation of CJD.

Topics: Autopsy; Cerebrovascular Circulation; Creutzfeldt-Jakob Syndrome; Cysteine; Humans; Male; Middle Aged; Organotechnetium Compounds; Time Factors; Tomography, Emission-Computed, Single-Photon

Topics: Aged; Aged, 80 and over; Creutzfeldt-Jakob Syndrome; Cysteine; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Female; Humans; Lewy Body Disease; Organotechnetium Compounds; Radiopharmaceuticals; Tomography, Emission-Computed, Single-Photon

Diffusion-weighted imaging (DWI) has been reported to be a useful technique for diagnosing Creutzfeldt-Jakob disease (CJD). The present study reported DWI results in cases of familial CJD with a V180I mutation (CJD180) in the prion protein gene as well as neurological findings.. A retrospective analysis of 3 patients with V180I was performed. Cerebrospinal fluid (CSF) analysis, brain MRI, single-photon emission computed tomography (SPECT), and magnetic resonance spectroscopy (MRS) were included. CSF was analyzed for biochemical markers, and each patient underwent brain MRI, SPECT, and MRS analysis. A brain biopsy from the frontal cortex, which corresponded to the area of increased DWI signals, was utilized for neuropathological analysis.. CSF analysis results revealed elevated total tau protein and the absence of 14-3-3 protein, as well as decreased concentrations of neuron-specific enolase, S100 protein, and prostaglandin E(2). All patients presented with unique MRI features. Brain biopsy showed severe spongiform morphology, but comparatively preserved neurons and mild astrocytic gliosis. Accumulations of PrP(Sc) were not detected using the 3F4 antibody, and microglial activation was subtle. SPECT revealed hypoperfusion throughout both hemispheres. MRS revealed a reduced N-acetyl aspartate/creatine ratio.. Results from this study suggested that increased DWI signals could reflect severe spongiform changes in CJD180 patients.

Topics: Aged; Biomarkers; Brain; Creutzfeldt-Jakob Syndrome; Cysteine; Diffusion Magnetic Resonance Imaging; Enzyme-Linked Immunosorbent Assay; Female; Humans; Image Processing, Computer-Assisted; Immunohistochemistry; Magnetic Resonance Spectroscopy; Male; Memory Disorders; Organotechnetium Compounds; Primary Progressive Nonfluent Aphasia; Pyridines; Radiopharmaceuticals; Tomography, Emission-Computed, Single-Photon

We observed a discrepancy between the perfusion patterns seen in single photon emission computed tomography (SPECT) images obtained using technetium-99m hexamethyl propylene amine oxime (HMPAO) and SPECT images obtained using technetium-99m ethyl cysteinate dimer (ECD) in an 84-year-old man with Creutzfeldt-Jacob disease (CJD). HMPAO-SPECT demonstrated a reduction in perfusion in the parieto-temporal regions, especially the left temporal area. However, ECD-SPECT revealed a significant reduction in the bifrontal regions. At autopsy, the characteristic pathological findings of CJD, spongiform vacuolation, were most prominent in the bifrontal regions. Thus, the clinical features and the pathological findings were more closely correlated with the ECD-SPECT images than the HMPAO-SPECT images.

Topics: Creutzfeldt-Jakob Syndrome; Cysteine; Diagnosis, Differential; Humans; Male; Middle Aged; Organotechnetium Compounds; Radiopharmaceuticals; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon

In a patient with the occipitoparietal form of Creutzfeldt-Jakob disease (CJD) (Heidenhain type) positron emission tomography (PET) demonstrated decreased glucose utilization in the occipital lobes and adjacent cortical regions. Single photon emission computed tomography (SPECT) with 99mTc-bicisate showed a "coupled" decrease in blood flow in identical cortical areas in this patient. In contrast, magnetic resonance imaging (MRI) was normal. In the early stage of CJD, when still no major morphological abnormalities can be observed, functional imaging is useful for differential diagnosis, particularly to exclude other causes of dementia or pathological EEG patterns.

Topics: Aged; Creutzfeldt-Jakob Syndrome; Cysteine; Deoxyglucose; Female; Fluorine Radioisotopes; Fluorodeoxyglucose F18; Glucose; Humans; Organotechnetium Compounds; Tomography, Emission-Computed; Tomography, Emission-Computed, Single-Photon