Systematic literature review and meta-analysis of the prevalence of secondary progressive multiple sclerosis in the USA, Europe, Canada, Australia, and Brazil

Vasanthaprasad, Vijayalakshmi; Khurana, Vivek; Vadapalle, Sreelatha; Palace, Jackie; Adlard, Nicholas

doi:10.1186/s12883-022-02820-0

Table 1 Summary of SPMS prevalence studies and quality assessments

From: Systematic literature review and meta-analysis of the prevalence of secondary progressive multiple sclerosis in the USA, Europe, Canada, Australia, and Brazil

	Studies	Region	Study year/ prevalence day	Loney score	Diagnostic criteria	General population denominator	SPMS prevalent cases	SPMS prevalence (per 100,000)	MS prevalence (per 100,000)
Europe
Bosnia and Herzegovina
1	Klupka-Saric et al., 2007 [19]	Western Herzegovina	Dec 31, 2003	5	McDonald criteria	300,746	29	9.6	26.9
2	Klupka-Saric and Galic, 2010 [20]	Western Herzegovina Canton and Herzegovina-Neretva Canton	Dec 31, 2006	5	McDonald criteria	309,712	29	9.4	31
Bulgaria
3	Milanov et al., 1999 [21]	Sofia	Mar 31, 1998	5	Poser criteria	74,334	12	16.1	43.1
3	Milanov et al., 1999 [21]	Samokov	Mar 31, 1998	5	Poser criteria	44,616	10	22.4	38.1
Croatia
4	Perkovic et al., 2010 [22]	The town of Cabar	Dec 31, 2001	3	NR	4387	3	59.5	205.7
Croatia, Slovenia
5	Peterlin et al., 2006 [23]	Gorski Kotar, Croatia and Kocˇevje, Slovenia	Jun 1, 1999	5	Poser criteria	57,258	35	61.8	151.9
Finland
6	Laakso et al., 2019 [24]	Helsinki and Uusimaa, Southwest Finland, Tavastia Proper, Northern Savonia, and Central Finland	Dec 31, 2018	5	McDonald criteria	2,804,616	723	25.8	191.3
France
7	Berr et al., 1989 [25]	Hautes-Pyrenees	Jun 1, 1983	2	Poser criteria	227,942	5	2.2	40.0
8	Debouverie, 2009 [26]	Lorraine	2002	4	Diagnosed by a neurologist	2,310,376	933	40.4	109.0
Germany
9	Fasbender and Kolmel, 2008 [27]	Urban Area of Erfurt, Thuringia	Jan 31, 2006	4	Poser criteria	201,267	97	48.3	127.2
10	Hoer et al., 2014 [28]	Bavaria	2009	2	Diagnosed by a neurologist	10,400,000	1363	13.1	174.8
Greece
11	Papathanasopoulos et al., 2008 [29]	Rion-Patras	Dec 31, 2006	2	Poser and McDonald criteria	652,108	172	26.4	119.61
12	Piperidou et al., 2003 [30]	Province of Evros	Dec 31, 1999	1	Poser criteria	143,752	14	9.7	38.9
Hungary
13	Bencsik et al., 1998 [31]	Szeged city, Csongra’d County	1997	4	Poser criteria	198,682	5	2.6	65.0
14	Bencsik et al., 2001 [32]	Csongrad County	Jul 1, 1999	4	Poser criteria	400,128	48	12.0	62.0
15	Zsiros et al., 2014 [33]	Csongrad County	Jan 1, 2013	4	McDonald criteria	421,827	52	12.3	89.8
16	Biernacki et al., 2020 [34]	Csongrad County	Jan 1, 2019	4	McDonald criteria	399,012	102	25.6	105.3
Ireland
17	McDonnell and Hawkins, 1998 [35]	Ballymena, Coleraine, Ballymoney, and Moyle districts spanning the counties of Antrim and Derry in Northern Ireland	July 1, 1996	5	Poser criteria	151,000	111	73.5	190.0
18	McGuigan et al., 2004 [36]	Wexford	Jan 1, 2001	5	Poser criteria	104,372	49	46.6	120.7
18	McGuigan et al., 2004 [36]	Donegal	Jan 1, 2001	5	Poser criteria	129,994	92	70.5	184.6
19	Gray et al., 2008 [37]	Northeast of Northern Ireland	Jan 1, 2004	6	Poser/McDonald criteria	160,446	112	69.8	230.6
20	Lonergan et al., 2011 [38]	Donegal County	Dec 31, 2007	6	McDonald criteria	113,347	124	109.1	290.3
		Wexford				119,442	75	62.6	144.8
		Southeast Dublin city				101,721	51	50.1	127.8
		All three areas				334,510	251	75.0	188.9
Italy
21	Bellantonio et al., 2013 [39]	Campobasso, chief town of Molise region	Sep 30, 2009	5	Diagnosed by a neurologist	51,633	17	32.9	91.0
22	Bergamaschi et al., 2020 [40]	Pavia, Northern Italy	Dec 31, 2016	5	McDonald criteria	547,251	295	53.9	169.4
23	Caniglia-Tenaglia et al., 2018 [41]	Republic of San Marino	Dec 31, 2014	5	Diagnosed by a neurologist	32,789	12	36.8	204.3
24	Cavalletti et al., 1994 [42]	Province of Modena, Northern Italy	Dec 31, 1990	5	McAlpine and Confavreux	603,989	53	8.8	38.9
25	Granieri et al., 1996 [43]	Ferrara	Dec 31, 1993	5	Poser criteria	358,808	73	20.3	69.4
26	Granieri et al., 2007 [44]	Province of Ferrara, Northern Italy	Dec 31, 2004	5	Poser criteria	349,777	147	41.9	120.9
27	Granieri et al., 2008 [45]	Republic of San Marino	Dec 31, 2005	4	Poser criteria	29,999	5	16.7	166.7
28	Granieri et al., 2018 [46]	Province of Ferrara, Northern Italy	Dec 31, 2016	4	McDonald 2010 criteria	351,436	162	45.9	197.5
29	Grimaldi et al., 2007 [47]	Caltanissetta (Sicily), Southern Italy	Dec 31, 2002	4	Poser criteria	60,919	16	26.3	165.8
30	Guidetti et al., 1995 [48]	Provinces of Reggio Emilia and Modena	Dec 31, 1990	4	McAlpine criteria	1,024,223	30	2.9	40.2
31	Iuliano et al., 2014 [49]	Salerno (Southern Italy)	Dec 31, 2010	5	McDonald criteria	366,025	79	21.6	85.2
32	Millefiorini et al., 2010 [50]	Province of Frosinone	Jan 1, 2007	5	Poser criteria	491,548	85	17.2	95.0
33	Nicoletti et al., 2001 [51]	Catania, Sicily	Jan 1, 1995	5	Poser criteria	333,075	68	20.4	58.5
34	Nicoletti et al., 2005 [52]	Catania, Sicily	Dec 31, 1999	5	Poser criteria	313,110	77	24.6	92.0
35	Nicoletti et al., 2011 [53]	Catania, Sicily	Dec 31, 2004	5	Poser criteria	313,110	90	28.7	127.1
36	Patti et al., 2019 [54]	Sicily	Dec 31, 2018	4	McDonald criteria and Thompson criteria	23,948	5	20.9	292.3
37	Solaro et al., 2005 [55]	Province of Genoa	Dec 31, 1997	5	Poser criteria	913,218	150	16.4	94.0
38	Totaro et al., 2000 [56]	L’Aquila	Dec 31, 1996	5	Poser criteria	297,828	29	9.7	53.0
Kosovo
39	Zeqiraj et al., 2014 [57]	Prishtina	2003–2012	2	McDonald criteria	2,102,041	93	4.4	19.6
Netherlands
40	Minderhoud et al., 1988 [58]	Groningen	NR	2	Poser criteria	560,000	108	19.3	61.1
Norway
41	Dahl et al., 2004 [59]	Nord-Trøndelag County	Jan 1, 2000	6	Diagnosed by a neurologist	127,108	59	46.4	163.6
42	Gronning and Mellgren, 1985 [60]	Troms and Finnmark	Jan 1, 1983	3	Rose criteria	225,073	23	10.4	31.5
43	Risberg et al., 2011 [61]	Oppland County hospitals, Gjøvik, and Lillehammer	Jan 1, 2002	4	Poser criteria	183,235	95	51.8	174.1
Poland
44	Brola et al., 2016 [62]	Swietokrzyskie Province	Dec 31, 2014	5	McDonald criteria	1,263,176	317	25.1	115.7
45	Brola et al., 2017 [63]	Swietokrzyskie Province	Dec 31, 2015	5	McDonald criteria	1,257,179	360	28.6	121.3
46	Kapica-Topczewska et al., 2018 [64]	Central Poland	Dec 31, 2013	5	McDonald criteria	1,268,239	311	24.6	109.1
46	Kapica-Topczewska et al., 2018 [64]	Northeastern Poland	Dec 31, 2013	5	McDonald criteria	1,195,625	318	26.6	108.6
47	Kułakowskaa et al., 2017 [65]	Northeastern Poland (Podlaskie voivodeship)	NR	3	McDonald criteria	750,460	200	26.6	108.6
48	Potemkowski and Jasinska, 2015 [66]	Kielce, Central Poland	NR	4	McDonald criteria	200,938	71	35.4	98.53
Portugal
49	Branco et al., 2020 [67]	Entre Douro e Vouga region	Jul 1, 2014	5	McDonald criteria	274,859	36	13.1	64.4
50	De Sa et al., 2006 [68]	District of Santarém	Nov 1, 1998	6	Poser criteria	62,621	6	9.6	46.3
51	Figueiredo et al., 2015 [69]	Braga	Dec 31, 2009	5	Diagnosed by a neurologist	866,012	49	5.7	39.8
52	Lopes et al., 2020 [70]	Sao Miguel	Jul 1, 2019	4	McDonald criteria	137,150	4	2.9	34.3
53	Ruano et al., 2014 [71]	Entre Douro-e-Vouga	Jan 1, 2013	4	McDonald criteria	274,859	34	12.3	58.6
Romania
54	Becus and Popoviciu, 1994 [72]	Mures County	Dec 31, 1986	2	Diagnosed by a neurologist	615,032	8	1.3	21.0
55	Cornea et al., 2016 [73]	Timis County	Aug 16, 2016	3	McDonald criteria	486,420	45	9.3	69.1
Serbia
56	Pekmezovic et al., 2019 [74]	Belgrade	Dec 31, 2018	4	McDonald criteria	1,685,673	586	34.7	136.8
57	Toncev et al., 2011 [75]	Sumadija	Dec 31, 2006	4	McDonald criteria	298,778	62	20.8	64.9
Spain
58	Aladro et al., 2005 [76]	Las Palmas, Canary Islands	Dec 31, 2002	5	Poser criteria/McDonald criteria	82,623	12	14.5	73.8
59	Benito-Leon et al., 1998 [77]	Mostoles	Feb 1, 1998	5	Poser criteria	195,979	9	4.6	43.4
60	Bufill et al., 1995 [78]	Region of Osona in northern Catalonia	Dec 31, 1991	4	Poser criteria	71,985	6	8.3	58.0
61	Candeliere-Merlicco et al., 2016 [79]	Health District III, Murcia	Dec 13, 1999	5	McDonald criteria	171,040	27	15.8	71.9
62	Casquero et al., 2001 [80]	Menorca (Balearic Islands)	Dec 31, 1996	5	Poser criteria	67,009	9	13.4	68.6
63	Costa Arpin et al., 2020 [81]	Santiago de Compostela	Dec 31, 2015	5	McDonald criteria	95,612	24	25.1	152
64	Hernandez, 2002 [82]	Island of La Palma, Canary Islands	Dec 15, 1998	4	Poser criteria	81,507	11	13.5	41.7
65	Izquierdo et al., 2015 [83]	Northern Seville	Dec 31, 2011	5	Poser criteria	163,324	24	14.7	90.2
66	Modrego Pardo et al., 1997 [84]	Province of Teruel	Mar 1, 1996	5	Poser criteria	143,680	4	2.8	32.0
67	Modrego and Pina, 2003 [85]	Bajo Aragon, province of Teruel, Northeastern Spain	Jan 1, 2003	5	Poser criteria	58,666	5	8.5	75.0
68	Perez-Carmona et al., 2017 [18]^a	San Vicente del Raspeig	Apr 10, 2017	3	2010 McDonald criteria- MS diagnosis, Lublin criteria (2013 revisions) – MS subtypes	56,696	NR	SPMS with progression but without activity: 3.4%; SPMS without progression or activity: 6.9%	102.3
69	Perez-Carmona et al., 2019 [86]	San Vicente del Raspeig	Dec 31, 2018	5	McDonald criteria	1,685,673	586	34.7	136.8
70	Pina et al., 1998 [87]	Sanitary District of Calatayud	Apr 1, 1995	5	NR	58,591	18	30.7	58.0
71	Tola et al., 1999 [88]	Valladolid	Mar 1, 1997	5	Poser criteria	92,632	6	6.5	58.3
Sweden
72	Bostrom et al., 2009 [89]	County of Varmland in Western Sweden	Dec 31, 2002	4	Poser criteria	273,419	205	75.0	170.1
Turkey
73	Akdemir et al., 2017 [90]	Middle Black Sea Region	Aug 2010–May 2011	4	McDonald criteria	3,666,667	74	2.0	43.2
74	Çelik et al., 2011 [91]	Edirne City	2003	5	McDonald criteria	119,298	8	6.7	33.9
74	Çelik et al., 2011 [91]	Edirne City	2004	5	McDonald criteria	119,298	8	6.7	36.5
75	Gokce et al., 2019 [92]	Sivas Province	Apr 2017–Jan 2018	7	McDonald criteria	6595	4	60.7	288
76	Turk Boru et al., 2006 [93]	Maltepe, Istanbul	Nov 2002–May 2003	7	Poser criteria	32,531	11	33.8	101.4
77	Turk Boru et al., 2011 [94]	Three areas of the Black Sea coast of Turkey (Kandıra, Geyve, Erbaa)	2006 and 2010	8	Poser criteria	53,364	9	16.9	50.6
78	Turk Boru et al., 2018 [95]	Gazipasa (Mediterranean coast)	Apr–May 2012	7	McDonald 2010 criteria	13,451	1	7.4	52.0
		Artvin (Black sea coast)	May–Jun 2012			16,116	1	6.2	18.6
		Ordu (Black sea coast)	Nov–Dec 2012			28,800	4	13.9	55.5
79	Turk Boru et al., 2020 [96]	Eregli	May–Oct 2018	7	McDonald criteria	32,261	5	15.5	96.1
79	Turk Boru et al., 2020 [96]	Devrek	May–Oct 2018	7	McDonald criteria	21,963	2	9.1	45.5
United Kingdom
80	Ford et al., 1998 [97]	Leeds	Apr 30, 1996	4	Poser criteria	732,061	225	30.7	97.3
81	Ford et al., 2002 [98]	Leeds	Oct 31, 1999	5	Poser criteria	732,061	244	33.3	108.7
82	Fox et al., 2004 [99]	Devon	Jun 1, 2001	5	Poser criteria	341,796	121	35.3	117.6
83	Gajofatto et al., 2013 [100]	Verona	Dec 31, 2001	5	McDonald criteria	253,208	59	23.4	106.0
84	Robertson et al., 1995 [101]	Cambridgeshire	Jul 1, 1993	5	Poser, Allison, and Millar criteria	378,959	85	22.4	118.0
85	Simpson et al., 2015 [102]	Isle of Man	2006	5	McDonald criteria	80,058	56	69.9	153.6
85	Simpson et al., 2015 [102]	Isle of Man	2011	5	McDonald criteria	84,497	63	74.6	179.9
86	Visser et al., 2012 [103]	Aberdeen, Orkney, Shetland	Sep 24, 2009	5	Poser and McDonald criteria	24,8102	237	95.5	238.0
Australia
87	Barnett et al., 2003 [104]	Newcastle	1996	5	Diagnosed by a neurologist	133,686	13	9.7	59.1
88	Ribbons et al., 2017 [105]	Newcastle	Aug 9, 2011	5	McDonald criteria	148,535	17	11.8	124.2
Brazil
89	Callegaro et al., 2001 [106]	Sao Paulo	Jul 1, 1997	4	Poser criteria	9,380,000	23	0.2	15.8
90	Calmon et al., 2016 [107]	Volta Redonda	Nov 2012	5	Poser criteria/McDonald criteria	260,180	1	0.4	15.4
91	Negreiros et al., 2015 [108]	João Pessoa, Paraíba	Jul 2013	4	Diagnosed by a neurologist	723,515	19	2.6	12.0
92	Lana-Peixoto et al., 2012 [109]	Belo Horizonte	July 1, 2001	4	Poser criteria	2,238,526	78	3.5	18.1
93	Ribeiro et al., 2011 [110]	Uberaba, Minas Gerais	Aug–Dec 2008	4	Poser and McDonald criteria	287,760	2	0.7	12.5
94	Ribeiro et al., 2019 [111]	Goiânia	Dec 31, 2015	4	Poser or McDonald criteria	1,430,697	27	1.9	22.2
Canada
95	Sloka et al., 2005 [112]	Newfoundland and Labrador	Dec 31, 2001	5	Poser criteria	521,986	94	18.0	94.4
96	Warren and Warren, 1992 [113]	County of Barrhead, Alberta	Jan 1, 1990	5	Poser criteria	9720	9	92.6	196.0
97	Warren and Warren, 1993 [114]	Westlock county	Jan 1, 1991	5	Poser criteria	11,510	9	78.2	200.0

^aAs this is the sub-group analysis, not included in the meta-analysis
MS Multiple sclerosis, NR Not Reported, SPMS Secondary progressive multiple sclerosis

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