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. 2014 Sep 8;14:179. doi: 10.1186/s12883-014-0179-y

Table 1.

Previously reported cases with the feature of CJD and HE in need of differential diagnosis

Year/author Final diagnosis Clinical findings Corticosteroid response EEG finding Brain image CJD work-up Hashimoto encephalopathy work-up
CSF study Biopsy/gene Lab US/SCAN/FANA
2004/Cho et al. [14]. CJD 66/F Bilateral frontal SWC (0.5–2 Hz) MRI (DWI): HSI at cortex, caudate nucleus, putamen 14-3-3: + TG-AB: WNL US: Chronic thyroiditis
Dementia, parkinsonism, visual symptom, ataxia, myoclonus, akinetic mutism SPECT/PET: Rt. Hemisphere: ↓ TPO-AB: ↑ FNA: PMN, lymphocyte
2003/Cossu et al. [2]. CJD 61/F Periodic triphasic wave MRI: normal 14-3-3: + Biopsy: typical CJD pattern, PRNP + codon 210, 129 TG-AB: ↑
Visual symptom, ataxia, myoclonus, mental change TPO-AB: ↑
TSH : ↑
T3, T4 : WNL
2012/Kondziella et al. [10]. CJD 67/F Anti-thyroid antibody (+)
HE 63/F +
Dementia, ataxia, myoclonus
2008/Cerqueira et al. [8]. HE 68/F + Occasional sharp waves (2–3 Hz) MRI (T2WI): HSI at corona radiata, centrum semiovale T3, T4: WNL
Cognitive decline, insomnia, poor appetite, visual hallucination, tremor, gait disturbance, decreased mentality, myoclonus TSH: ↑
TPO-AB: ↑
TG-AB: WNL
2002/Doherty et al. [9]. HE 57/F + Bihemispheric slowing, triphasic wave MRI (T2WI): HIS at Lt. medial frontal region 14-3-3: − Biopsy: spongiform change TPO-AB: ↑ US: hypoechoic area
Generalized seizure, hallucination
2004/Vander et al. [13]. HE 58/M + Slow background, generalized delta activity MRI: normal 14-3-3: + TSH: ↑
Confusion, myoclonus, bilateral hyperreflexia, babinski (+) generalized seizure TG-AB: ↑
TPO-AB: ↑
2011/Santoro et al. [12]. HE 66/M + Slow theta and delta waves MRI (DWI, T2WI): HSI at left white matter and bilateral cortical region 14-3-3: −− TG-AB: ↑
Confusion, GTC, fluctuating alertness, myoclonic jerks
2004/Sakuria et al. [11]. HE 79/F + Diffuse slowing, periodic synchronous discharge MRI (T2WI): HSI at periventricular and basal ganglia lesion 14-3-3: + TG-AB: ↑
TPO-AB: ↑
Dementia, gait, inactivity, myoclonus TSH-receptor AB: ↑
TPO-AB: ↑
TSH-receptor AB: ↑

CJD Creutzfeldt-Jakob disease; HE Hashimoto’s encephalopathy; GTC generalized tonic-clonic seizure; DWI diffusion-weighted images; HSI high signal intensity; Tg-Ab anti-thyroglobulin antibody; TPO-Ab anti-thyroid peroxidase antibody; US ultrasonography; FNA fine needle aspiration; PMN polymorphonuclear leukocytes.