Table 1.

Clinical features of sporadic CJD patients and endpoint RT-QuIC analysis in tissues. All four female patients fulfilled the WHO diagnostic criteria for CJD and were considered to be definite cases and autopsy cases. The clinical findings and clinical course of disease were consistent with classical types of sporadic CJD. EEG results showed diffuse slowing of background with periodic sharp wave complexes (PSWC). The CSF samples had elevated 14-3-3 protein and t-tau protein, and prion seeding activities were identified by RT-QuIC assay. Western blotting and genetic analysis revealed that these 4 sCJD patients were all MM1 subtype.

N.D.: not detected; N.A.: not available.

		Patient 1				Patient 2				Patient 3				Patient 4
Age at onset (years)					69			70				59				62
Period from onset to death (months)					27			18				78				60
Period from onset to akinetic mutism (months)					3			2				3				4
End-point RT-QUIC (Log SD50/g tissue)		1st	2nd	3rd	mean ± S.D.	1st	2nd	3rd	mean ± S.D.	1st	2nd	3rd	mean ± S.D.	1st	2nd	3rd	mean ± S.D.
Central nervous system	Brain	10.25	10	10	10.08 ± 0.12	9.25	9.5	9.5	9.42 ± 0.12	9.75	9	8.75	9.17 ± 0.42	9.75	10.5	9.75	10.00 ± 0.35
Non-neuronal tissues	Spleen	6	≤ 5.25	≤ 6.25	≤ 5.83	6.25	6.5	5.75	6.17 ± 0.31	N.D.	N.D.	N.D.	N.D.	6	5.75	6	5.92 ± 0.12
	Kidney	5.5	5.5	N.D.	5.5	≤ 5.50	6.5	6.75	≤ 6.25	≤ 5.50	≤ 4.75	7	≤ 5.92	5.75	≤ 6.00	6.5	≤ 6.08
	Lung	≤ 5.25	≤ 5.50	6	≤ 5.58	6.25	6.25	≤ 5.75	≤ 6.08	N.D.	N.D.	N.D.	N.D.	6.75	7	6.75	6.83 ± 0.12
	Liver	≤ 5.25	≤ 6.50	≤ 6.00	≤ 5.92	N.D.	N.D.	N.D.	N.D.	≤ 6.25	≤ 6.50	≤ 6.25	≤ 6.33	6.75	6.75	7.25	6.92 ± 0.24
	Adrenal grand	≤ 5.00	N.D.	≤ 5.25	≤ 5.13	7.25	7.5	7.5	7.42 ± 0.11	7	6.5	5.75	6.42 ± 0.51	6.75	6.5	6.25	6.5 ± 0.20

N.D. = not detected.