Human prion disease surveillance in Spain, 1993-2018: an overview

Figures & data

Figure 1. Diagram flow from suspect TSE notifications for 1993–2018. TSE indicates transmissible spongiform encephalopathy; sCJD, sporadic Creutzfeldt-Jakob disease (CJD); gCJD, genetic CJD; atCJD, accidentally transmitted CJD; vCJD, variant CJD; FFI, fatal familial insomnia; GSSS, Gerstmann-Sträussler-Scheinker syndrome

Figure 2. Annual number of notifications as at 31 December 2018 and different diagnostic categories by year of diagnosis. sCJD indicates sporadic Creutzfeldt-Jakob disease (CJD); gCJD, genetic CJD; atCJD, accidentally transmitted CJD; vCJD, variant CJD; FFI, fatal familial insomnia; GSSS, Gerstmann-Sträussler-Scheinker syndrome

Table 1. Annual numbers, notifications and subsequently confirmed and probable Creutzfeldt-Jakob disease cases and deaths: 1993–2018 (cases in 1993 and 1994 in retrospect)

Year	1993	1994	1995	1996	1997	1998	1999	2000	2001	2002	2003	2004	2005	2006	2007	2008	2009	2010	2011	2012	2013	2014	2015	2016	2017	2018	Total
Number of reports*			33	60	37	70	55	96	101	82	79	77	105	76	102	122	84	113	96	100	121	108	126	97	102	95	2137
Cases by diagnostic year	30	29	27	36	51	79	76	73	94	80	73	78	104	100	102	108	98	106	95	116	108	114	111	113	92	44	2137
Cases by year of death	24	19	28	36	33	70	67	61	84	70	68	74	91	81	101	99	86	89	85	103	102	93	97	87	89	46	1883
Men, sCJD
Definite	6	7	3	5	2	15	8	10	17	18	15	25	31	25	18	17	20	16	15	25	24	17	20	15	18	3	395
Probable	4	5	3	11	12	16	9	12	12	8	4	4	7	11	7	8	9	13	15	14	14	20	13	23	15	13	282
Probable + definite	10	12	6	16	14	31	17	22	29	26	19	29	38	36	25	25	29	29	30	39	38	37	33	38	33	16	677
Possible	1		1	5		2	2	2	2	1	1	3	1	1	3	1	3	1	1	4	7	3	4	8	7	1	65
All	11	12	7	21	14	33	19	24	31	27	20	32	39	37	28	26	32	30	31	43	45	40	37	46	40	17	742
Women, sCJD
Definite	6	8	6	4	10	13	10	14	21	20	22	16	23	27	28	27	25	22	19	26	19	19	17	25	17	5	449
Probable	6	5	6	8	18	21	20	16	14	13	11	15	14	15	11	11	12	16	17	16	14	19	15	19	24	15	371
Probable + definite	12	13	12	12	28	34	30	30	35	33	33	31	37	42	39	38	37	38	36	42	33	38	32	44	41	20	820
Possible	1			1		1		1	2	1	1			2	2	1	3	2	3	5	6	1	2				35
All	13	13	12	13	28	35	30	31	37	34	34	31	37	44	41	39	40	40	39	47	39	39	34	44	41	20	855
VV/MM	0/0	0/2	0/1	2/1	2/10	8/14	4/17	6/13	6/29	8/23	8/26	13/29	7/37	8/40	10/24	9/17	8/29	9/19	10/17	5/18	5/21	8/24	6/18	4/22	6/21	5/11	157/483
VV/MM (ratio)	0	0	0	2	0.2	0.57	0.24	0.46	0.21	0.35	0.31	0.45	0.19	0.20	0.42	0.53	0.28	0.47	0.59	0.28	0.24	0.33	0.33	0.18	0.29	0.45	0.33
gTSE, all
Definite	1	2	0	1	0	4	5	3	9	5	0	4	4	5	6	4	3	5	4	5	7	5	7	4	2	0	95
Probable	0	0	4	1	2	2	6	3	2	3	1	2	2	3	2	4	3	4	4	8	4	5	3	1	2	2	73
Accidentally transmitted, All	2							1	1	1		1								1						1	8
Variant CJD													1		1	3											5
Non-TSE cases^a	3	0	3	1	7	5	16	11	16	10	17	7	20	13	26	31	21	28	17	15	16	21	26	17	5	4	356
Post-mortem: neurodegenerative/vascular	1	0	1	0	1	1	7	6	8	3	7	2	8	6	13	13	7	13	7	2	2	1	9	1	1	1	121
Other clinically confirmed diagnoses	1	0	0	0	2	1	4	4	4	1	3	0	1	4	2	2	3	1	1	3	0	2	2	1	0	0	42

CJD indicates Creutzfeldt-Jakob disease; sCJD, sporadic Creutzfeldt-Jakob disease; gTSE, genetic transmissible spongiform encephalopathy; MM, methionine-methionine; VV, valine-valine; MV, methionine-valine; TSE, transmissible spongiform encephalopathy; ^aThe difference between total non-TSE cases and the addition of the last two rows is the number of cases, 193 in all, whose diagnosis was never registered.

Table 2. Notifications, definite and probable sCJD and gTSE cases with age-adjusted incidences per million person-years in 1998–2018 by regions

			sCJD					gTSE
Regions	Population·10^−3	Notifications	n	Incidence per million person-years	Definite/ probable ratio	MM (%)	VV+MV (%)	n	Incidence per million person-years
Andalusia (a)	166 600.03	298	210	0.97	69/122	61.8	38.2	15	0.07
Aragon (b)	26 765.78	64	41	1.08	22/19	63.6	36.4	3	0.11
Asturias (c)	22 152.33	34	30	0.81	19/9	57.1	42.9	1	0.04
Balearic Islands (d)	21 292.74	32	25	1.00	10/13	62.5	37.5	0	0.00
Canary Islands (e)	41 524.34	58	51	1.12	14/36	72.4	27.6	0	0.00
Cantabria (f)	11 825.76	26	23	1.41	16/7	83.3	16.7	1	0.00
Castile la Mancha (g)	40 576.72	95	65	1.16	11/46	55.2	44.8	9	0.17
Castile and León (h)	51 910.37	162	123	1.42	63/56	50.9	49.1	13	0.30
Catalonia (i)	146 771.14	325	290	1.51	209/75	62.2	37.8	32	0.19
Valencian Community (j)	97 879.48	330	219	1.43	85/97	55.2	44.8	13	0.12
Extremadura (k)	22 559.49	37	25	0.78	11/12	50.0	50.0	0	0.00
Galicia (l)	57 047.07	103	84	0.90	46/35	71.8	28.2	2	0.03
Madrid (m)	126 107.99	271	205	1.27	127/70	68.9	31.1	34	0.23
Murcia (n)	28 529.56	48	39	1.22	16/21	82.6	17.4	2	0.00
Navarre (o)	12 600.51	39	32	1.77	25/6	33.3	66.7	5	0.35
Basque Country (p)	44 589.39	195	121	1.72	90/26	50.0	50.0	38	0.69
La Rioja (q)	6 291.76	19	16	1.84	12/4	50.0	50.0	0	0.00
Ceuta (r)	1 596.38	0	0	0.00	0/0	0.0	0.0	0	0.00
Melilla (s)	1 534.93	1	1	0.50	0/1	0.0	0.0	0	0.35
TOTAL	928 155.77	2137	1600	1.25	951/730	61.1	38.9	168	0.16

sCJD indicates sporadic Creutzfeldt-Jakob; gTSE, genetic transmissible spongiform encephalopathy; MM, methionine-methionine; VV, valine-valine; MV, methionine-valine.

Figure 3. (a) Age- and sex-specific incidences of definite and probable sporadic Creutzfeldt-Jakob disease (sCJD) and genetic transmissible spongiform encephalopathy (gTSE), for 1998–2018; and (b) time trends for annual genetic subgroup proportions in percentages

Figure 4. Geographical patterns in Spain of: (a) sporadic Creutzfeldt-Jakob disease (sCJD), number of cases (b) sCJD age-adjusted incidence; (c) genetic transmissible spongiform encephalopathy (gTSE), number of cases; (d) gTSE incidence; (e) variant Creutzfeldt-Jakob disease (vCJD), number of cases; (f) accidentally transmitted Creutzfeldt-Jakob disease (atCJD); and (g) population in millions

Figure 5. Relationship as between incidence, disease duration and onset. The diameter of each bubble is computed as the mean age at onset minus 60 years. Shown in the top right corner are disorders with high incidence, long duration and later onset (AD, AMD, PD and LBD). Shown at the bottom left are disorders that are more rapidly progressive, with earlier onset and lower incidence (sCJD, MSA and ALS). sCJD indicates sporadic Creutzfeldt-Jakob disease; MSA, multisystemic atrophy; FTD, frontotemporal dementia; ALS, amyotrophic lateral sclerosis; LBD, Lewy body dementia; PD, Parkinson´s disease; AMD, age-related macular degeneration; AD, Alzheimer’s disease. Data from figures cited in ref [33]

De Pedro-Cuesta J, Martínez-Martín P, Rábano A, et al. Etiologic framework for the study of neurodegenerative disorders as well as vascular and metabolic comorbidities on the grounds of shared epidemiologic and biologic features. Front Aging Neurosci. 2016;8:138. PMID: 27378910.

 PubMed Web of Science ®Google Scholar