Neurologische Manifestationen beim Sjögren-Syndrom

Zusammenfassung

Das primäre Sjögren-Syndrom (PSS) ist eine nicht ganz seltene Autoimmunerkrankung mit einer Prävalenz zwischen 0,2 % und 2 % und gekennzeichnet durch Xerophthalmie mit verminderter Tränendrüsensekretion (pathologischer Schirmer-Test), Xerostomie mit verminderter Speicheldrüsensekretion (verminderte Nuklidaufnahme in der Speicheldrüsenszintigraphie), SS-A (Ro) oder SS-B(La)-Antikörpern und dem histopathologischen Nachweis mononukleärer Zellen in der Speicheldrüsenbiopsie. Daneben kommt es beim Sjögren-Syndrom in einem nicht unerheblichen Anteil zu neurologischen Manifestationen, die das periphere (sensible Polyneuropathie, autonome Polyneuropathie, Hirnnervenneuropathie) und zentrale Nervensystem (Myelitis, chronische Myelopathie, Optikusneuritis, zerebrale Ischämien, psychiatrische Symptome) betreffen. Da die neurologischen Befunde zunächst ganz im Vordergrund des klinischen Bildes stehen können, ist die Differenzialdiagnose eines PSS häufig zu berücksichtigen.

Abstract

The primary Sjögren-syndrome (PSS) is a chronic autoimmune disorder with a prevalence of 0.2 % and 2 % that is manifestated clinically by xerophthalmia with reduced lacrimation (pathological Schirmer's test), xerostomia with reduced salivary flow (pathological salivary scintigraphy), antibodies to Ro/SSA and La/SSB, labial salivary gland biopsy with mononuclear infiltrations. In a considerable percentage, PSS is associated with neurological complications which may affect the peripheral nervous system (sensory polyneuropathy, autonomic polyneuropathy, cranial nerve neuropathy) and to a lesser extent the central nervous system (acute myelitis, progressive myelopathy, optic neuritis, ischemic infarction, psychiatric disturbances). Since neurological findings may dominate the clinical picture, PSS should be considered frequently as differential diagnosis.

Literatur

• 1 Alexander E. Central nervous system disease in Sjögren's syndrome: new insights into immunopathogenesis.  Rheum Dis Clin North Am. 1992;  18 637-672
• 2 Kaplan J, Rosenberg R, Reinitz E. et al . Peripheral neuropathy in Sjögren's syndrome.  Muscle Nerve. 1990;  13 570-579
• 3 Vitali C, Bomardieri S, Moutsopoulos H M. et al . Assessment of the European classification criteria for Sjögren's syndrome in a series of clinically defined cases: results of a prospective multicentre study.  Ann Rheum Dis. 1996;  55 116-121
• 4 Fox R, Saita I. Criteria for diagnosis of Sjögren's syndrome.  Rheum Dis Clin North Am. 1994;  20 391-407
• 5 Fox R. Sjögren's syndrome.  In: Primer on the Rheumatic Diseases. Atlanta; Arthritis Foundation 1998: 283-286
• 6 Fox R I, Törnwall J, Maruyama T, Stern M. Evolving concepts of diagnosis, pathogenesis, and therapy of Sjögren's syndrome.  Curr Opin Rheumatol. 1998;  10 446-456
• 7 Williams C S, Butler E, Roman G C. Treatment of myelopathy in Sjögren syndrome with a combination of prednisone and cyclophosphamide.  Arch Neurol. 2001;  58 815-819
• 8 Bjerrum K B. Keratoconjunctivitis sicca and primary Sjögren's syndrome in a Danish population aged 30 - 60 years.  Acta Ophthalmol Scand. 1997;  75 281-286
• 9 Manabe Y, Sasaki C, Warita H. et al . Sjögren's syndrome with acute transverse myelopathy as the initial manifestation.  J Neurol Sci. 2000;  176 158-161
• 10 Tan E M, Feltkamp T E, Smolen J S. et al . Range of antinuclear antibodies in „healthy” individuals.  Arthritis Rheum. 1997;  40 1601-1611
• 11 Leydhecker W. Augenheilkunde. Berlin; Springer 1982: 40
• 12 Hietaharju A, Yli-Kerttula U, Häkkinen V, Frey H. Nervous system manifestations in Sjögren's syndrome.  Acta Neurol Scand. 1990;  81 144-152
• 13 Hettenkofer H-J. Rheumatologie. Stuttgart, New York; Thieme 1984: 126
• 14 Kraus A, Caballero-Uribe C, Jakez J. et al . Raynaud's phenomenon in primari Sjögren's syndrome.  J Rheumatol. 1992;  19 1572-1574
• 15 Kuhl V, Urban P P, Mayet W J, Hopf H C. Isolated trochlear nerve palsy and repetitive Raynaud's phenomenon of the tongue in primary Sjögren's syndrome.  J Neurol. 1999;  246 974-975
• 16 Viergever P, Swaak K. Renal tubular dysfunction in primary Sjögren's syndrome: clinical studies in 27 patients.  Clin Rheumatol. 1991;  10 23-27
• 17 Siamopoulos K C, Elisaf M, Drosos A. et al . Renal tubular acidosis in primary Sjögren's syndrome.  Clin Rheumatol. 1992;  11 226-230
• 18 Zimhony O, Sthoeger Z, Ben David D. et al . Sjögren's syndrome presenting as hypokalemic paralysis due to distal renal tubular acidosis.  J Rheumatol. 1995;  22 2366-2368
• 19 Perez B, Kraus A, Lope B. Autoimmune thyroid disease in primary Sjögren's syndrome.  Am J Med. 1995;  99 480-484
• 20 Königsmann V, Müller-Jensen A, Stanc A, Müller-Plathe O. Primäres Sjögren-Syndrom - eine Kasuistik.  Akt Neurol. 1998;  25 245-247
• 21 Yasuda M, Nobunaga M. The association of myasthenia gravis and connective tissue diseases: the role of Sjögren's syndrome.  Fukuoka Igaku Zasshi. 1994;  85 38-51
• 22 Govoni M, Bajocchi G, Rizzo N. et al . Neurological involvement in primari Sjögren's syndrome: clinical and instrumental evaluation in a cohort of italian patients.  Clin Rheumatol. 1999;  18 299-303
• 23 Kassan S S, Thomas T L, Moutsopoulos H M. et al . Increased risk of lymphoma in sicca snydrome.  Ann Intern Med. 1978;  89 888-892
• 24 Valesini G, Priori R, Bavoillot D. et al . Differential risk of non-Hodgkin's lymphoma in Italian patients with primary Sjögren's syndrome.  J Rheumatol. 1997;  24 2376-2380
• 25 Alexander E, Provost T T, Stevens M B, Alexander G E. Neurologic complications of primary Sjögren's syndrome.  Medicine. 1982;  61 247-257
• 26 Mellgren S J, Conn D L, Stevens J C, Dyck P J. Peripheral neuropathy in primary Sjögren's syndrome.  Neurology. 1989;  39 390-394
• 27 Gemignani F, Marbini A, Pavesi G. et al . Peripheral neuropathy associated with primary Sjögren's syndrome.  J Neurol Neurosurg Psychiatry. 1994;  57 983-986
• 28 Andouopoulos A P, Lagos G, Drosos A A, Moutsopoulos H M. The spectrum of neurological involvement in Sjögren's syndrome.  Br J Rheumatol. 1990;  29 21-23
• 29 Grant I A, Hunder G G, Homburger H A, Dyck P J. Peripheral neuropathy associated with sicca complex.  Neurology. 1997;  48 855-862
• 30 van Dijk G W, Notermans N C, Kater L. et al . Sjögren's syndrome in patients with chronic idiopathic axonal polyneuropathy.  J Neurol Neurosurg Psychiatry. 1997;  63 376-378
• 31 Mori K, Koike H, Misu K. et al . Spinal cord magnetic resonance imaging demonstrate sensory neuronal involvement and clinical severity in neuronopathy associated with Sjögren's syndrome.  J Neurol Neurosurg Psychiatry. 2001;  71 488-492
• 32 Denislic M, Meh D. Early asymmetric neuropathy in primary Sjögren's syndrome.  J Neurol. 1997;  244 383-387
• 33 Sorajja P, Poirier M K, Bundrick J B, Matteson E L. Autonomic failure and proximal skeletal myopathy in a patient with primary Sjögren Syndrome.  Mayo Clin Proc. 1999;  74 695-697
• 34 Goto H, Matsuo H, Fukudome T, Shibuya N. Chronic autonomic neuropathy in a patient with primary Sjögren's syndrome.  J Neurol Neurosurg Psychiatry. 2000;  69 134-141
• 35 Bansal S K, Sawhney I M, Chopra J S. Epilepsia partialis continua in Sjögren's syndrome.  Epilepsia. 1987;  28 362-363
• 36 Mauch E, Völk C, Kratzsch G. et al . Neurologic and neuropsychiatric dysfunction in primary Sjögren's syndrome.  Acta Neurol Scand. 1994;  89 31-35
• 37 Lafitte C, Amoura Z, Cacoub B. et al . Neurological complications of primary Sjögren's syndrome.  J Neurol. 2001;  248 577-584
• 38 Alexander E. Neurologic disease in Sjögren's syndrome: mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle.  Rheum Dis Clin North Am. 1993;  19 869-908
• 39 Pou-Serradell A, Vinas-Gaya J. Trois cas de neuropathies périphériques rares associées au syndrome de Gougerot-Sjögren primitif.  Rev Neurol. 1993;  149 481-484
• 40 Chu K, Kang D-W, Song Y-W, Yoon B-W. Trochlear nerve palsy in Sjögren's syndrome.  J Neurol Sci. 2000;  177 157-159
• 41 Kaltreider H B, Talal N. The neuropathy of Sjögren's syndrome: trigeminal nerve involvement.  Ann Int Med. 1969;  70 751-762
• 42 Ashworth B, Tait G BW. Trigeminal neuropathy in connective tissue disease.  Neurology. 1971;  21 609-614
• 43 Lecky B RF, Hughes R AC, Murray N MF. Trigeminal sensory neuropathy.  Brain. 1987;  110 1463-1485
• 44 Matsukawa Y, Nishinarita S, Morie T. Abducent and trochlear palsies in a patient with Sjögren's syndrome.  Br J Rheumatol. 1995;  34 484-485
• 45 Touzé E, Blanche P, Zuber M. A 35-year history of recurrent multiple cranial neuropathy due to primari Sjögren's syndrome.  J Neurol. 1999;  246 968-969
• 46 Vincent D, Loron P, Awada A, Gautier J C. Paralysies multiples et récidivantes des nerfs craniens. Syndrome de Gougerot-Sjögren.  Rev Neurol. 1984;  4 318-321
• 47 Bakouche P, Ferroir J P, Guillard A. Paralysies multiples et récidivantes des nerfs crâniens syndrome de Goujerot-Sjögren primitif.  Rev Neurol. 1994;  150 728-731
• 48 Urban P P, Keilmann A, Hopf H C. Sensory neuropathy of the trigeminal, glossopharyngeal, and vagal nerves in Sjögren's syndrome.  J Neurol Sci. 2001;  186 59-63
• 49 Süß W, Hopf H C, Ludwig B, Poralla T. Okuläre Myosistis, Erstsymptom eines Sjögren-Syndroms.  Akt Neurol. 1984;  11 23-24
• 50 Kraus A, Cifuentes M, Villa A R. et al . Myositis in primari Sjögren's syndrome. Report of 3 cases.  J Rheumatol. 1994;  21 649-653
• 51 Dowd J E, Lipsky P E. Sjögren's syndrome presenting as hypokalemic periodic paralysis.  Arthritis Rheum. 1993;  36 1735-1738
• 52 Thomas N, Ramakrishna B, Seshadri M S. Hypokalemic periodic paralysis: an unusual cause.  J Assoc Physicians India. 1996;  44 207-208
• 53 Alexander E, Malinow K, Lijewski J. Primary Sjögren's syndrome with central nervous system disease mimicking multiple sclerosis.  Ann Intern Med. 1986;  104 323-330
• 54 Tsai K-Y, Tsai C-P, Liao N. Sjögren's syndrome with central nervous system involvement presenting as multiple sclerosis with failure response to Beta-Interferon.  Eur Neurol. 2001;  45 59-60
• 55 de Seze J, Devos D, Castelnovo G. et al . The prevalence of Sjögren syndrome in patients with primary progressive multiple sclerosis.  Neurology. 2001;  57 1359-1363
• 56 Mochizuki A, Hayashi A, Hisahara S, Shoji S. Steroid-responsive Devic's variant in Sjögren's syndrome.  Neurology. 2000;  54 1391-1392
• 57 Shimode K, Kobayashi S, Kitani M. et al . Optic neuritis in primary Sjögren's syndrome.  Rinsho Shinkeigaku. 1986;  26 433-436
• 58 Tesar J T, Mcmillian V, Molina R, Armstrong J. Optic neuropathy and central nervous system disease associated with primary Sjögren syndrome.  Am J Med. 1992;  92 686-692
• 59 Bragoni M, di Piero V, Priori R. et al . Sjögren's syndrome presenting as ischemic stroke.  Stroke. 1994;  25 2276-2279
• 60 Nagahiro S, Mantani A, Yamada K, Ushio Y. Multiple cerebral arterial occlusions in a young patient with Sjögren's syndrome: case report.  Neurosurgery. 1996;  38 592-595
• 61 Berlit P, Klötzsch C, Henningsen H. Polyneuropathie bei Sjögren-Syndrom.  Akt Neurol. 1992;  19 54-56
• 62 Niemelä R K, Hakala M. Primary Sjögren's syndrome with severe central nervous system disease.  Semin Arthritis Rheum. 1999;  29 4-13
• 63 Selnes O A, Barry G, Malinow K L, Alexander E L. Cognitive dysfunction in primary Sjögren's syndrome.  Neurology. 1985;  35 (Suppl) 179
• 64 Malinow K L, Molina R, Gordon B. et al . Neuropsychiatric dysfunction in primary Sjögren's syndrome.  Ann Intern Med. 1985;  103 344-349
• 65 Drosos A A, Andonopoulos A P, Lagos G. et al . Neuropsychiatric abnormalities in primary Sjögren's syndrome.  Clin Exp Rheumatol. 1989;  7 1-3
• 66 Alexander E, Beall S S, Gordon B. et al . Magnetic resonance imaging of cerebral lesions in patients with the Sjögren syndrome.  Ann Intern Med. 1988;  108 815-823
• 67 Pierot L, Saure C, Leger J-M. et al . Asymptomatic cerebral involvement in Sjögren's syndrome: MRI findings in 15 cases.  Neuroradiology. 1993;  35 378-380
• 68 Awad I A, Spetzler R F, Hodak J A. et al . Incidental subcortical lesions identified on magnetic resonance imaging in the elderly. I. Correlation with age and cerebrovascular risk factors.  Stroke. 1986;  17 1084-1089
• 69 Coates T, Slavotinek J P, Rischmueller M. et al . Cerebral white matter lesions in primary Sjögren's syndrome: a controlled study.  J Rheumatol. 1999;  26 1301-1305
• 70 McDonald W J, Compston A, Eadn G. et al . Recommended diagnostic criteria for multiple sclerosis: guidelines from the international panel on the diagnosis of multiple sclerosis.  Ann Neurol. 2001;  50 121-127
• 71 Malinow K, Yanakakis G D, Glusman S M. et al . Subacute sensory neuronopathy secondary to dorsal root ganglionitis in primary Sjögren's syndrome.  Ann Neurol. 1986;  20 535-537
• 72 Griffin J W, Cornblath D R, Alexander E. et al . Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome.  Ann Neurol. 1990;  27 304-315
• 73 Satake M, Yoshimura T, Iwaki T. et al . Antidorsal root ganglion neuron antibody in a case of dorsal root ganglionitis associated with Sjögren's syndrome.  J Neurol Sci. 1995;  132 122-125
• 74 Bakchine S, Duyckaerts C, Hassine L. et al . Central and peripheral neurologic lesions in primary Gougerot-Sjögren syndrome: clinicopathological study of a case.  Rev Neurol. 1991;  147 368-375
• 75 Manley G, Wong E, Dalman J. et al . Sera from some patients with antibody-associated paraneoplastic encephalomyelitis/sensory neuronopathy recognize Hu Ro-52 k antigen.  J Neurooncol. 1994;  19 105-112
• 76 Alexander G E, Provost T T, Stevens M B, Alexander E L. Sjögren's syndrome: central nervous system manifestations.  Neurology. 1981;  31 1391-1396
• 77 Alexander E, Arenett F C, Provost T T, Stevens M B. Sjögren's syndrome: association of anti-Ro (SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity.  Ann Intern Med. 1983;  98 155-159
• 78 Alexander E, Craft C, Dorsch C. et al . Necrotizing arteriitis and spinal subarachnoid hemorrhage in Sjögren syndrome.  Ann Neurol. 1982;  11 632-635
• 79 Sato K, Miyasaka N, Nishioka K. et al . Primary Sjögren's syndrome associated with systemic necrotizing vasculitis: a fatal case.  Arthritis Rheumatol. 1987;  6 717-718
• 80 Vissink A, Schaub R MH, van Rijn L J. et al . The efficacy of mucin-containing artificial saliva in alleviating symptoms of xerostomia.  Gerodontology. 1987;  6 4-10
• 81 Wright R A, O'Duffy J D, Rodriguez M. Improvement of myelopathy in Sjögren's syndrome with chlorambucil and prednisone therapy.  Neurology. 1999;  52 386-388
• 82 Gourley M F, Austin H A, Scott D. et al . Methylprednisolone and cyclophosphamide, alone or in combination, in patients with lupus nephritis. A randomized, controlled trial.  Ann Intern Med. 1996;  125 549-557
• 83 Konttinen Y T, Kinnunen E, von Bonsdorff M. et al . Acute transverse myelitis successfully treated with plasmapheresis and prednisone in a patient with primary Sjögren's syndrome.  Arthritis Rheum. 1987;  30 339-344
• 84 Chen W-H, Yeh J-H, Chiu H-C. Plasmapheresis in the tretament of ataxic sensory neuropathy associated with Sjögren's syndrome.  Eur Neurol. 2001;  45 270-274
• 85 Pascual J, Cid C, Berciano J. High-dose IV immunglobulin for peripheral neuropathy associated with Sjögren's syndrome.  Neurology. 1998;  51 650-651
• 86 Molina J A, Benito-Leon J. Intravenous immunoglobulin therapy in sensory neuropathy associated with Sjögren's syndrome.  J Neurol Neurosurg Psychiatry. 1996;  60 699
• 87 Grant I A, Dyck P J. High-dose IV-immunglobulin for peripheral neuropathy associated with Sjögren's syndrome (reply).  Neurology. 1998;  51 651