Neurologic Complications Associated with Sjögren’s Disease: Case Reports and Modern Pathogenic Dilemma

Table 1

Review of the literature regarding Sjögren's syndrome (SS) patients with cranial neuritis.

First authors/year	Ref.	No. pts.	Age/gender	Onset of neuritis versus diagnosis of SS (years)*	Nerves involved/clinical features	Follow-up
Sjogren/1935	[10]	1	n.d.	n.a.	bil. VII	n.a.
Weber/1945	[11]	1	n.a.	n.a.	III	n.a.
Spillane/1959	[12]	1	58 F	n.a.	bil. V	n.a.
Attwood/1961	[13]	1	50 F	n.a.	III–V–VII–IX	Improv. with Cs
Kaltreider/1969	[14]	4	45 F, 48 F,; 52 F, 73 F	+6; +2; +5; +14	sV (3), bil. (pt. 3); bil. I + PNS involv. (pt. 4)	1: chronic course, onset during Cs; 2, 4: Cs inefficacy; 3: response to Cs
		1	55 F	+8	I	remission within 1 year
Whaley/1972	[15]	1	27 F	n.a.	V	Response to Cs and P.E.
Hull/1984	[16]	1	33 F	0	V + PNS	n.a.
Vincent/1985	[17]	1	53 F	+7	V–VII–IX-diplopia	Recurrent ep. (6 in 7 years)
Alexander/1986	[18]	16	Mean 50 F	n.a.	7 II, 2 III, 1 V, 6 VI, 1 VII, 1 VIII	n.a.
Shimode/1986	[19]	1	n.a.	n.a.	II	n.a.
Serratrice/1986	[20]	1	58 F	+4	V	Cs 20 mg/day ineffective
Malinow/1986	[21]	1	F	n.a.	V + d.r. ganglionitis	n.a.
Mauch/1994	[22]	1	n.a.	n.a.	V	n.a.
Hankey/1987	[23]	1	78 F	+ (several years)	V + PNS involv.	n.a.
Wise/1988	[24]	3	F	− (1–6)	II	n.a.
Laloux/1988	[25]	1	81 F	n.a.	V + d.r. ganglionitis	n.a.
Graus/1988	[26]	2	58 F, 75 F	n.a.	sV	n.a.
Phanthumchinda/; 1989	[27]	1	28 F	0	Multiple	Resolved with Cs
Uchihara/1989	[28]	1	n.a.	n.a.	bil. VII	n.a.
Mellgren/1989	[29]	5	n.a.	n.a.	V	n.a.
Andonopoulos/; 1990	[30]	3	n.a.	n.a.	V	n.a.
Flint/1990	[31]	1	n.a.	n.a.	bil. sV	n.a.
Mukai/1990	[32]	5	n.a.	n.a.	V	n.a.
Semah/1990	[33]	1	57 F	−11	V	Chronic course
Berman/1990	[34]	1	10 F	0	II + CNS vasculitis	Improv. with immunosuppressors
Berault-Dupont/1992	[35]	1	59 F	0	VII	Improv. to high dosage Cs
Tesar/1992	[36]	3	20 F, 35 F, 41 F	0; −1; −2	II, bil. III-IV–VI (pt. 1), bil. II (pt. 2), II–IX-diplopia + CNS involv. (pt. 3)	Resolution with high dosage Cs (pt. 1); improv. with high dosage Cs (pt. 2); recurrent, resolution of neuritis with Cs/CYC within 6 months (pt. 3)
Soubrier/1993	[37]	4	n.a.	n.a.	V	n.a.
Pou Serradell/1993	[38]	1	63 F	− (n.a.)	III (8 ep.), multiple (5 ep.)	Recurrent, responsive to Cs
Güell/1993	[39]	1	n.a.	0	V	n.a.
Mauch/1987	[40]	1	n.a.	n.a.	sV	n.a.
Bakouche/1994	[41]	1	49 M	− (n.a.)	Diplopia-V-tinnitus	Recurrent (3 ep.) solving within 3 weeks
Matsukawa/1995	[42]	1	56 F	0	V then IV–VI	The first ep. autoresolved; the second with Cs
Harada/1995	[43]	1	n.a.	− (n.a.)	II + acute transv. myelopathy	Resistant to Cs
Tajima/1997	[44]	9	Mean 54.9 F; 51 F	n.a.	8 V, 4 bil.; 1 II	n.a.
Rojas-Rodriguez/1998	[45]	1	8 F	− (n.a.)	II	Visual impairment not responsive to Cs, IVIG, CYC pulses
Govoni/1999	[4]	2	51 F, 24 F	+6; +2	VIII; III + cerebellar ataxia	n.a.
Dumas/1999	[46]	1	41 F	n.a.	sV	n.a.
Kuhl/1999	[47]	1	54 F	+8	IV	Improv. spontaneously within few weeks
Touze/1999	[48]	1	34 F (first ep.)	−35; −2; 0; +2; + (n.a.)	VI, VII, IX, laryngeal	Recurrent ep., not improv. with Cs
Oketani/1999	[49]	1	52 F	+2	II	Improv. to pulse Cs
Wingerchuk/1999	[104]	4	n.a.	n.a.	II	n.a.
Chu/2000	[50]	1	54 F	0	IV-V	Resolution with Cs and Aza within 4 weeks
Urban/2001	[51]	1	53 F	+1	V–IX-X	Chronic course
Hadithi/2001	[52]	1	41 F	0	VII	Autoresolution after several days
Lafitte/2001	[53]	1	n.a.	n.a.	V	n.a.
Kadota/2002	[54]	1	63 F	0	II	Autoresolution within 6 months
Maeda/2002	[55]	1	21 F	0	II	Autoresolution
Anaya/2002	[56]	1	n.a.	n.a.	II	n.a.
Yanagihara/2002	[57]	1	39 F	0	III	n.a.
Font/2003	[58]	6	Mean 58 F	−4 to 0	V	Chronic course besides oral Cs
Delalande/2004	[59]	30	n.a.	n.a.	2 I, 13 II, 5 V, 4 VII, 6 VIII	n.a.
Mori/2005	[60]	20	Mean 55.6	− (n.a.)	15 V, 1 VII, 5 multiple (III, V, VI, VII, IX, X, XII)	4/7 patients improv. with Cs
Rousso/2005	[61]	1	40 F	0	VII	Recurrent ep. that autoresolved, in 1 pt. with Cs/vit. B12 within 12 days
Oishi/2007	[62]	1	65 M	0	VI	Autoresolved within 5 months
Cardoso/2006	[63]	1	9 F	−9; 0	bil. II	2 ep. 9 years apart, irreversible visual loss after initial response to Cs
Galbussera/2007	[64]	1	79 F	0	III	Resolution with Cs and IGIV within 2 months
Pournaras/2007	[65]	1	38 M	0	bil. II	Improv. with Cs
Gökçay/2007	[66]	1	20 F	−10	II	Cs/Aza resistant, switch to CYC
Arabshahi/2006	[67]	1	11 F	0	bil. II + transv. myelitis	Recurrent ep., transient improv. with Cs
Barroso/2007	[68]	1	34 F	−9	II + CNS involv.	Recurrent ep., responsive to iv Cs
Teo/2008	[69]	1	52 F	0	III	n.a.
Béjot/2008	[70]	1	53 F	+ (n.a.)	bil. II + aseptic meningitis	Improv. with CYC
Lui/2008	[71]	1	59 M	0	III	Resolved with Cs/Aza within 2 weeks
Ii/2008	[72]	1	49 F	0	II	Improv. with IVIG
Javed/2008	[73]	2	n.a.	n.a.	II	n.a.
Pittock/2008	[74]	6	n.a.	n.a.	II	n.a.
Min/2009	[75]	6	n.a.	n.a.	II	n.a.
Ashraf/2009	[76]	1	47 F	0	V–IX–XII	Resolved with Cs and MTX
Kato/2009	[77]	1	25 F	+ (2)	II + CNS involv.	Improv. with high-dosage Cs
Dellavance/2009	[78]	2	n.a.	n.a.	II	n.a.
Kim/2009	[79]	7	n.a.	n.a.	II	Poor prognosis for high relapse rate
Alhomoud/2009	[80]	10	Mean 40 F	n.a.	9 II + CNS involv.; 1 VII	n.a.
Rabadi/2010	[81]	1	23 F	0	II	n.a.
Sakai/2010	[82]	1	77 M	0	III-bil.V-VI-VII	Improv. with Cs
Imbe/2010	[83]	1	31 F	0	II + acute myelitis	Improv. with Cs/P.E.
Nascimento/2010	[84]	2	n.a.	−10; −0.5	V	n.a.
Chourkani/2010	[85]	2	43 F, 48 F	0	II (bil. 1 pt.)	Improv. with Cs/immunosuppressors
Massara/2010	[86]	3	48, 50, 74 F	+5, +6,; +16 years later	2 II, 1 VII	Resolution with iv Cs
Cojocaru/2011	[87]	1	50 F	+0.7; +2	II then V	1° ep.: improv. with Cs/IVIG; 2° ep.: Cs-resistant
Niţescu/2011	[88]	2	n.a.	+ (n.a.)	II	n.a.
Yadav/2011	[89]	1	26 F	+ (several years)	bil. II	n.a.
Koga/2011	[90]	1	31 F	0	II + acute myelitis	Improv. with P.E., Cs-resistant
Gono/2011	[91]	7	Mean 44 F	n.a.	3 II (1 + CNS involv.), 2 V, 1 VII, 1 IX-X	n.a.
Kolfenbach/2011	[92]	6	n.a.	n.a.	II	Recurrent ep.
Estiasari/2012	[93]	10	n.a.	n.a.	II	n.a.
Horai/2012	[94]	8	63 F, 52 M, 43 F, 61 F,; 51 F, 55 F, 45 F, 35 F	0 (first case); n.a.	V (4 bil.)	Improv. with Cs/tacrolimus (1); Improv. with Cs (1); Improv. with symptomatic treat (3); resist. to Cs (1); recurrent (1)
Tan/2012	[95]	1	56 F	0	bil. II	Permanent visual impairment
Maruta/2012	[96]	1	89 F	0	II	Improv. with iv Cs
Mallucci/2012	[97]	1	74 F	0	bil. II	n.a.
Teixeira/2013	[7]	4	Mean 47.9 F	n.a.	2 II, V, VI, 2 VII	Cs almost effective
Briani/2013	[98]	1	66 F	+5	V	n.a.
Flanagan/2013	[99]	1	64 M	0	V	n.a.
Tang/2013	[100]	8	Mean 34.7 F	0	II	Recurrent (3 pts), response to Cs/immunosuppressors
Present cases		2	40 F, 54 F	−6; +8	II; phrenic nerve	Improvement with Cs (40 F), with IVIG (54 F)
TOTAL		267	F/M: 20.8 Mean age: 𝟒𝟖±𝟏𝟑.𝟐 years		4 I, 123 II, 8 III, 4 IV, 95 V, 17 VI, 23 VII, 9 VIII, 11 IX, 8 X, 0 XI, 6 XII ( § )

(*) 0: diagnosis of SS and neuritis were contemporary; + (yrs): neuritis onset after diagnosis of SS; − (yrs): neuritis onset before diagnosis of SS. (§) for each cranial nerve, all cases of documented involvement have been counted, even if they are included in episodes of multineuritis; therefore the total of SS patients with neuritis does not correspond to the total number of episodes with cranial nerve involvements. n.a. = not available; CNS = central nervous system; PNS = peripheral nervous system; bil. = bilateral; involv. = involvement; sV = pure sensory trigeminal involvement; d.r. ganglionitis = dorsal roots ganglionitis; transv. = transverse; ep. = episode; pt. = patient; Cs = corticosteroids; CYC = cyclophosphamide; Aza = azathioprine; IMTX = methotrexate; VIG = intravenous immunoglobulins; P.E. = plasma exchange.