Table 1.
Literature | Population | Number/Sex | Age range | Clinical features | FDG-PET findings |
---|---|---|---|---|---|
Henkel K,2002 [11] | Germany | 8 (5F 3M) | 35–78 years | dementia, cerebellar, myoclonus, pyramidal/extrapyramida, visual, speech | spread hypometabolism in CJD, with a reduction of cerebral glucose metabolism in at least one temporal or parietal region |
Engler H,2003[12] | Sweden | 9(5F 4M) | 56 ± 8 years | dementia, aphasia, apraxia, ataxia, myoclonus, rigidity, chorea, akinetic mutism, pyramidal symptoms | pronounced regional decrease in glucose brain metabolism, which were most pronounced in the cerebellum and the frontal, occipital and parietal cortices |
Hamaguchi T,2005[13] | Japan | 8(4F 4M) | 30–75 years | dementia, ataxia, and pyramidal and extrapyramidal signs | thalamic hypoperfusion or hypometabolism on cerebral blood flow |
Okamura N,2010 [14] | Japan | 2(1F 1M) | 58–63 years | subacutely progressive dementia, gait disturbance, psychotic symptoms, dysarthria and myoclonus | no obvious retention of BF-227 with positron emission tomography |
Zhang WJ,2011 [15] | USA | 1F | 72 year | falls, ataxia, bizarre behavior and rapid cognitive deterioration | basal ganglia and patchy cortical hypometabolism |
Zhang Y,2012 [16] | USA. | 1F | 58 year | left-sided decreased sensation, ataxia, and left “alien” hand and leg. | hypoperfusion and hypometabolism in the right frontoparietal cortices, including the primary sensorimotor cortex, and temporal cortex |
Xing XW,2012 [17] | China | 14 (6F 8M) | 36–68 years | NA | hypometabolism was observed in the basal ganglia in 12 patients (85.71%), and 8 patients (57.14%) had decreased FDG uptake in the thalamic nuclei on PET scans |
Kim EJ, 2012 [18] | Korea | 11 (6F 5M) | 36–75 years | gait disturbance, visual disturbance, obtundation, dysarthria, memory impairment, tremor, dementia | decreased glucose metabolism in bilateral parietal, frontal, and occipital cortices. |
Engler H, 2012 [19] | Uruguay | 1M | 64 year | dizziness, memory problems, disorientated, visual hallucinations, speech difficulties, ataxia, myoclonic jerks | extreme low glucose uptake in the left brain hemisphere |
Zhao W,2013 [20] | China | 57(24F33M) | 36–75 years | sub-acute onset with progressive dementia | abnormalities of hypo-metabolism in the cerebral cortex were clearly detected by PET. |
Renard D,2013 [3] | Belgium | 9 (4 F 5M) | 52–86 years | rigidity and dystonia, shuffling gait, postural tremor, Shuffling gait, monotone speech, chorea, bradykinesia | hypometabolism in the medial parietal cortex bilaterally, and in the left-sided lateral and medial frontal and the lateral parietal cortex |
Furukawa F,2014 [21] | Japan | 1 M | 52 year | gait instability, gait unsteadiness, cognitive impairment, dysarthria and hallucinations | low glucose metabolism in the thalami and cerebellar cortices |
Euskirchen P,2014 [22] | Germany | 1F | 65 year | gait disturbance and vertigo, difficulties to speak and write | reduced FDG metabolism in the basal ganglia with spatial correlation to MR lesions |
Ortega-Cubero S, 2015 [23] | Spain | 9 (2M7F) | 39–77 years | psychological disorder, cognitive impairment, cerebellar syndrome | characterised by a cortical-subcortical pattern |
Prieto E,2015 [24] | Spain | 12 (8F 4M) | 39–77 years | psychiatric symptoms, ataxia, cognitive decline, vestibular symptoms | hypometabolism that affected both subcortical (bilateral caudate, thalamus) and cortical (frontal cortex) structures |
Matias-Guiu JA,2017 [10] | Spain | 1 M | 80 year | gait disturbance, unsteadiness and weakness of right limbs, disturbance in smooth pursuit and saccadic ocular movements, mild hemiparesis and bilateral ataxia, spontaneous and action myoclonus | large regions of hypometabolism including left frontoparietal lobes as well as bilateral thalamus |
Miyazawa N,2017 [25] | Japan | 1M | 78 year | memory impairment, severe aphasia and apraxia. | hypometabolism in the posterior cingulate and precuneus cortex, and putamen and caudate nucleus |
Miyazawa N,2017 [25] | Japan | 1F | 68 year | myoclonus and disturbance of consciousness | hypometabolism in the same locations as the foci on MRI and in the cerebellum. |
Renard D,2017 [26] | France | 15 (10F 5M) | 52–86 years | cerebellar ataxia, visual signs, pyramidal signs, myoclonus, limb apraxia, limb dystonia, sensory loss, parkinsonism, corticobasal syndrome | entire CJD group showed lateralized frontal and parietal hypometabolism |
Mente KP, 2017 [27] | USA | 8 (4F 4M) | 2–32 years | dementia, insomnia, myoclonus, pyramidal ataxia, visual, behavior, extrapyramidal | (1) hypometabolism was found in a few regions, most frequently in the posterior cingulate gyrus and precuneus, both of which are in the parietal lobe. (2) hypermetabolism was identified in the limbic and mesolimbic structures, including insula, medial temporal lobe, nucleus accumbens, olfactory cortex, and parahippocampal gyrus. |
Duignan J,2018 [28] | Ireland | 1F | 71 year | cognitive decline, progressive dysphasia and visual hallucinations. | hypometabolism in the temporal and parietal lobes with involvement of the posterior cingulate gyrus and precuneus bilaterall |
sCJD, sporadic Creutzfeldt-Jakob disease. FDG-PET, fluorodeoxyglucose positron emission tomography. NA, not available.