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.