TABLE 1.
Clinical findings of CJD-type of Hashimoto's encephalopathy and CJD with anti-NAE antibodies.
| Author (year) | Age of onset (y) / sex | Consciouness disturbance/ progressive dementia | Myoclonus | Convulsion/ seizure | Serum anti-thyroid Abs | Serum anti-NAE Abs | EEG | Hyper-intense area on DWI | 14-3-3 protein/ total tau protein in the CSF | Result of RT-QUIC assay of the CSF | PRNP gene information | Reponsivenss to corticosteroids | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| HE 1 | Wilhelm-Gössling (1998) | 58/F | +/N.A. | + | +/+ | TPO+, Tg+ | N.A. | Triphagic wave | N.A. | N.A. | N.A. | N.A. | + | |
| HE 2 | Sakurai (2007) | 76/F | −/+ → +/+ (early-phase) | + | +/+ | TPO+, Tg+ | + | PSWCs | − | −/− | N.A. | No muration, codon 129 M/M, 219 E/E | + | |
| HE 3 | de Cerqueria (2008) | 68/F | +/+ | + | +/+ | TPO+ | N.A. | Triphagic wave | − | N.A. | N.A. | N.A. | + | |
| HE 4 | Muramatsu (2013) | 57/M | +/+ | + | +/+ | TPO+, Tg+ | + | PSWCs | Bilateral frontal cortices and thalamus | +/N.A. | N.A. | No mutation | + | |
| CJD with anti-NAE Abs |
Present case |
76/F |
−/+ |
− (early-phase) → + (terminal-phase) |
−/− |
TPO+, Tg+ |
+ |
slow wave (early-phase) → PSWCs (terminal-phase) |
Bilateral occipital, parietal, left frontal and temporal cortices |
−/− |
+ |
No mutation, codon 129 M/M, 219 E/E |
− |
|
| MM2C sCJD | 64.3 (49-77)1) | −/+(relatively slow progressive course) → +/+ | + (67%)1) | N.A. | N.A. | N.A. | +or − (0-42%)1,20,23) | + (～100%)11,20) | + (30-91%)11,20, 21, 23, 24)/ + (40%)21) | + (44.4%)21) | no mutation, codon 129 M/M | − |
Abs: antibodies; anti-NAE Abs: anti-N-terminal alpha enolase antibodies; CJD: Creutzfeldt-Jakob disease; CSF: cerebrospinal fluid; DWI: diffusion-weighted image; EEG: electroencephalogram; F: female; HE: Hashimoto's encephalopathy; M: male; N.A.; not available or no discription; PSWCs: periodic sharp-wave complexes; RT-QUIC: real-time quaking-induced conversion; y: year-old; +: positive; -: negative.