Zeynep ÖZÖZEN AYAS, Dılcan KOTAN, Emel OĞUZ AKARSU, Bekir Enes DEMİRYÜREK

Analysis of the Patients with Autoimmune Neurological Syndromes in a Single Center in Turkey

Autoimmune neurological syndromes (ANS) are a series of diseases that may involve the central, peripheral, and autonomic nervous system wherein the immune system is affected through varying immunological pathways. In the present study, the clinical and radiological features of the patients diagnosed with ANS were discussed. We reviewed the clinical features, laboratory and radiological findings of 15 patients, who were diagnosed with ANS among those being followed at the hospital. All patients underwent tumor screening tests for paraneoplastic syndrome investigations. Total of 15 patients were included; 11 (73.3%) men and 4 (26.7%) women. The mean age of patients was 50.46±8.49 (32–72) years. Six (40%) patients were diagnosed with paraneoplastic cerebellar degeneration (PCD); three (20%) with limbic encephalitis (LE); two (13.3%) with chronic inflammatory demyelinating polyneuropathy; one (6.67%) with sensorimotor neuropathy; one (6.67%) with motor neuron disease (MND); one (6.67%) with transverse myelitis (TM), one (6.67%) with dermatomyositis. Six (40%) patients had subacute-onset. Chronic symptoms were detected in five (33.3%) patients, acute in four (26.7%) patients. Gait and speech disorders were most common symptoms. The cancer was detected in 10 (three breast, three lung, one liver, one prostate, one thyroid, and one nasopharyngeal cancer) of 15 patients (66.7%). Autoantibody tests revealed GAD-Ab positivity in the patient with LE and anti-Ri, anti-Amphiphysin positivity in the patient with PCD. Seven patients’ CSF were analysed. The CSF examination revealed normal findings in three patients with PCD. Pleocytosis was found in patient with TM, while the other three (two LE, one MND) had protein elevation unaccompanied by lymphocytosis. Three of the patients had typical bilateral mesial-temporal involvement in MRI, consistent with LE. Cerebellar atrophy was detected in five of six patients with PCD. ANS span greatly heterogenous neurological disorders. Therefore, a variety of clinical signs and symptoms may be present. Early diagnosis brought about by increased awareness consistently affects treatment. Although the presence of autoantibodies is an important sign, the detection rates are small. Routinely performing cancer screening to facilitate early diagnosis and practicing persistent patient follow-up are the most important factors.

Otoimmün Nörolojik Sendromlu Hastaların Türkiye’deki bir Merkezdeki Analizleri

Otoimmün nörolojik sendromlar (ONS), bağışıklık sisteminin değişik immünolojik yollardan etkilediği santral, periferal ve otonomik sinir sistemini içeren bir dizi hastalıktır. Bu çalışmada ONS tanısı almış hastaların klinik ve radyolojik özellikleri tartışılmıştır. ONS tanısı almış ve hastaneden takip edilmiş 15 hastanın klinik, laboratuvar ve radyolojik özellikleri incelendi. Tüm hastalara, paraneoplastik sendromların araştırılması için tümör tarama testleri yapıldı. Onbiri (%73.3) erkek, 4’ü (%26.7) kadın, toplam 15 hasta dahil edildi. Hastaların ortalama yaşı 50.46±8.49 (32-72) idi. Altı (%40) hastaya paraneoplastik serebellar dejenerasyon (PSD), 3 (%20) limbik ensefalit (LE), 2 (%13.3) kronik inflamatuar demyelizan polinöropati, 1 (%6.67) sensöriomotor nöropati, 1 (%6.67) motor nöron hastalığı (MNH), 1 (%6.67) transvers myelit (TM), 1 (%6.67) dermatomyozit tanısı konuldu. Altı (%40) hasta subakut başlangıçlı idi. Kronik semptomlar 5 (33.3%) hastada, akut 4 (26.7%) hastada saptandı. Yürüme ve konuşma bozuklukları en sık görülen semptomlardı. Kanser 15 hastanın 10’unda (3 meme, 3 akciğer, 1 karaciğer, 1 prostat, 1 tiroid ve 1 nazofarenks kanseri) tespit edildi. Otoantikor testlerinde GAD-Ab pozitifliği LE, anti-Ri ve anti-Amphiphysin pozitifliği PSD’lu hastada saptandı. Yedi hastanın BOS’u analiz edildi. PSD’lu 3 hastanın BOS’u normal bulgular saptandı. TM hastasında pleositoz bulunurken, diğer 3 hastada (2 LE, 1 MNH) lenfositoza eşlik etmeyen protein artışı saptandı. LE’li hastaların üçünde MRG’de tipik bilateral meziotemporal tutulum vardı. PSD’li altı hastanın beşinde serebellar atrofi saptandı. ONS geniş heterojen nörolojik bozukluklara sahiptir. Bu nedenle, çeşitli klinik semptom ve bulgularla ortaya çıkabilmektedir. Artan farkındalığın getirdiği erken tanı, tedaviyi sürekli olarak etkiler. Otoantikorların varlığı önemli bir işaret olmasına rağmen, tespit oranları düşüktür. Erken tanıyı kolaylaştırmak için rutin kanser taraması ve ısrarlı hasta takibi önemli faktörlerdir.

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