Acute complex neuroplegia and ophthalmoplegia associated with anti-gq1b antibodies

Yirmi dört yaşında bir erkek boğaz ağrısını takip eden zonklayıcı tarzda şiddetli baş ağrısı şikayeti ile başvurdu. Burundan konuşma ve disfaji şikayetleri olduğunu belirtti. Hastada progresif olarak tüm dört ekstremitede güçsüzlükle birlikte oftalmopleji gelişti. Ardından solunum kasları tutulumu nedeniyle entübe edildi ve ventilasyon yapıldı. Hastanın anti-GQ1b antikorları pozitif geldi ve hastada Guillain-Barre sendromunun üstüne eklendiği Miller Fisher Sendromu ile birlikte Bickerstaff beyinsapı ensefaliti tanısı teyit edildi. Hasta 5 gün intravenöz immünüglobin 0.4 mg/kg/gün ile tedavi edildi ve nörolojik zayıflığı yavaş yavaş düzeldi.

Anti-gq1b antikorları ile ilişkili akut kompleks nöroloji ve oftalmopleji

A 24-year-old gentleman presented with a history of severe throbbing headache preceded by sore throat. He was noted to have a nasal twang and dysphagia. He progressively developed weakness of all four limbs associated with ophthalmoplegia. He, subse- quently, had to be intubated and ventilated in view of the involvement of the respiratory muscles. His anti-GQ1b antibodies were positive and the diagnosis of Bickerstaff brainstem encephalitis associated with Miller Fisher Syndrome overlapping with Guillain- Barre syndrome was confirmed. He was treated with intravenous Immunoglobulins 0.4 gm/kg/day for 5 days which resulted in a gradual recovery from his neurologic weakness.

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1. Ropper AH. The Guillain-Barre Syndrome. N Engl J Med 1992; 326:11306.
2.Mori M, Kuwabara S, Fukutake T, Yuki N, Hattori T. Clinical features and prognosis of Miller Fisher syndrome. Neurology 2001; 56:1104-6.
3.Fisher M. An usual variant of acute idiopathic polyneuritis (Syndrome of ophthalmoplegia, ataxia and areflexia) N Eng J Med 1956; 255: 57-65.
4.Bickerstaff, ER. Brainstem Encephalitis: further observa- tions on a grave syndrome with benign prognosis. Br Med J 1957; 1: 1384-7.
5. Bickerstaff ER, Cloake PC. Mesencephalitis and rhomben- cephalitis. BMJ 1951; ii:77-81.
6.Al-Din AN, Anderson M, Bickerstaff ER, Harvey I. Brainstem encephalitis and the syndrome of Miller Fisher: a case study. Brain 1982; 105: 481495.
7.Chiba A, Kusunoki S, Obata H, et al. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher Syndrome and Guillain-Barre syndrome: clinical and immunohistochemical studies. Neurology 1993; 43: 1911-7.
8. Odaka M, Yuki N, Yamada M, et al. Bickerstaffs brainstem encephalitis : clinical features of 62 cases and a subgroup associated with Guillain-Barre syndrome. Brain 2003; 126: 2279-90.
9. Snyder LA, Rismondo V, Miller NR. The Fisher vari- ant of Guillain-Barré syndrome (Fisher syndrome). J Neuroophthalmol 2009 Dec;29(4):312-24.
10. Overell JR, Willison HJ. Recent developments in Miller Fisher Syndrome and related disorders. Curr Opin Neurol 2005; 18: 562-6.
11. Hughes RA, Cornblath DR. Guillain-Barre syndrome. Lancet 2005; 366: 1653-66.
12. Overell JR, Hsieh ST, Odaka M, et al. Treatment of Fisher syndrome, Bickerstaffs brainstem encephalitis and re- lated disorders. Cochrane Database Syst Rev 2007; 1; CD004761.
13. Ogawara, K; Kuwabara, S; Yuki, N. Fisher syndrome or Bickerstaff brainstem encephalitis? Anti-GQ1b IgG anti- body syndrome involving both the peripheral and central nervous systems. Muscle and Nerve 2002; 26 (6):845-9.
14. Matsuo, M; Odaka, M; Koga, M; Tsuchiya, K; Hamasaki, Y; Yuki, N. Bickerstaffs brainstem encephalitis associated with a IgM antibodies to GM1b and Gal-NAcGD1a. J Neurol Sci 2004; 217 (2): 225-8.
15. Ogawara , K; Kuwabara, S; Mori, M; Hatori, T; Koga, M; Yuki, N. Axonal Guillain-Barre syndrome: relation to anti- ganglioside antibodies and Compylobacter jejuni infec- tion in Japan. Ann Neurol 2000; 48 (4): 624-31.
16. Odaka, M; Yuki, N; Hirata, K. Anti-GQ1b IgG antibody syndrome: clinical and immunologic range. J Neurol Nerosurg Psychiatry 2001; 70 (1):50-5.