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Table 1 Previously reported cases with the feature of CJD and HE in need of differential diagnosis

From: Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto's encephalopathy

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: ↑

  1. 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.