Betül ÇEVİK, Dürdane AKSOY, Semiha KURT, Volkan SOLMAZ, Emre ÇEVİK, Elmas PEKDAŞ

A case of Miller Fisher syndrome with progression and early relapse

Miller Fisher sendromu (MFS), Guillain-Barre sendromunun ender varyantlarından bir tanesidir.Charles Miller Fisher tarafından hastalığın ataksi, oftalmoparezi, arefleksi triadıyla giden iyiprognozlu bir Guillain-Barre varyantı olduğu ifade edilmiştir. Genellikle monofazik olmaklabirlikte literatürde rekürrenslerle giden vakalar da bildirilmiştir. Bu yazıda kliniğimize ataksi,oftalmoparezi, arefleksi ve parestezilerle başvurup MFS tanısıyla yatırılan, takip eden birkaç güniçinde tetraparezisi gelişen ve erken dönemde relaps meydana gelen 73 yaşında bir erkek hastasunuldu. Hastanın tam kan sayımı, rutin biyokimya, eritrosit sedimantasyon hızı, C-reaktif protein,tiroid fonksiyon testleri, serum vitamin B12 ve folat düzeyleri, Hepatit B, Hepatit C ve diğer viralbelirteçlerinde anormallik yoktu, ayrıca beyin ve servikal manyetik rezonans imajları (MRI)normaldi. Elektronörografide median, ulnar, peroneal ve tibial F yanıtları elde edilemedi. Serumanti-GQ1 antikoru pozitifti. Hasta MFS tanısı ile yatırıldı, sonraki bir kaç gün içerisinde tetraparezigelişti ve erken dönemde relaps gözlendi. Hastaya servisimize yatışının ardından verilen 7 günlükintravenöz immünglobulin (IVIG) tedavisi, yatışının 30. gününde relaps gelişmesi üzerinetekrarlandı. Bu tedavilerden sonra hastanın belirtilerinde kısmi bir düzelme gözlendi. MFSninprognozunun iyi olduğunu ifade eden çalışmalara rağmen hastalığın seyrinde bu vakada olduğugibi progresyon veya erken relapslar görülebilir ve tekrarlanan IVIG tedavisi hastalığın seyriniolumlu etkileyebilir. Dolayısıyla MFS tanısı alan hastaların takibinde, tanımlanan bu nadirdurumların akılda tutulması önemlidir.

Progresyon ve erken relapsla seyreden bir Miller Fisher sendromu olgusu

Miller Fisher syndrome (MFS) is one of the rare variants of the Guillain-Barre syndrome. CharlesMiller Fisher expressed the disease to be a Guillain-Barre variant with a good prognosis, that isaccompanied by ataxia, ophthalmoplegia and areflexia. While it is generally monophasic, somecases with recurrences were also reported in literature. This article presents a 73-year-old malepatient who was admitted to our clinic with ataxia, ophthalmoplegia, areflexia and paresthesia. Noabnormality was detected in the complete blood count, routine biochemistry, erythrocytesedimentation rate, C-reactive protein, thyroid function tests and serum vitamin B12 and folatelevels, tests for hepatitis A, B and other viral markers; brain and cervical magnetic resonanceimages (MRI) were also normal. Electroneurography revealed the absence of the median, ulnar,peroneal and the tibial F waves. Anti-GQ1b antibody was positive in the patient's serum. He washospitalized with the diagnosis of MFS, and he subsequently developed tetraparesis in thefollowing a few days, and had a relapse in the early period. Seven-day intravenousimmunoglobulin (IVIG) treatment which was given to the patient after hospitalization, wasrepeated upon the patient s relapse on the 30th day of his admission. A partial recovery of thepatient s symptoms was observed after these treatments. Although there have been studiessuggesting that the prognosis of MFS is good, progression or early relapses can be seen during thecourse of the disease; and as in this case, the repeated IVIG treatment can affect the course of thedisease positively. Therefore, it is important to keep these rare conditions in mind during follow- up of patients diagnosed with MFS.

PDF

___

1. Ito M, Kuwabara S, Odaka M, Misawa S, Koga M, Hirata K, Yuki N. Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum: clinical analysis of 581 cases. J Neurol 2008; 255: 674-82.
2. Takano H, Yuki N. Fishers syndrome associated with chickenpox and anti-GQ1b antibody. J Neurol 1995; 242: 255-6.
3. Fisher M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 1956; 255: 57-65.
4. Seneviratne U. Guillain-Barré syndrome. Postgrad Med J 2000; 76: 774-82.
5. Smith J, Clarke L, Severn P, Boyce R. Unilateral external ophthalmoplegia in Miller Fisher syndrome: case report. BMC Ophtalmol 2007; 7: 7.
6. Takahashi M, Koga M, Yokoyama K, Yuki N. Epidemiology of Campylobacter jejuni isolated from Patients with Guillain-Barré and Fisher Syndromes in Japan. J Clin Microbiol 2005; 43: 335-9.
7. Susuki K, Yuki N, Muramatsu M, Hirata K. Unilateral ophthalmoparesis and limb ataxia associated with anti-GQ1b IgG antibody. J Neurol 2000; 247: 652-3.
8. Weiss JA, White JC. Correlation of 1a afferent conduction with the ataxia of Fisher syndrome. Muscle Nerve 1986; 9: 327-32.
9. Snyder LA, Rismondo V, Miller NR. The Fisher variant of Guillain-Barré syndrome (Fisher syndrome). J Neuroophthalmol 2009; 29: 312-24.
10. Nishimoto Y, Odaka M, Hirata K, Yuki N. Usefulness of anti-GQ1b IgG antibody testing in Fisher syndrome compared with cerebrospinal fluid examination. J Neuroimmunol 2004; 148: 200-5.
11. Lo YL. Clinical and immunological spectrum of the Miller Fisher syndrome. Muscle Nerve 2007; 36: 615-27.
12. Li H, Yuan J. Miller Fisher syndrome: toward a more comprehensive understanding. Chin Med J 2001; 114: 235-9.
13. Mori M, Kuwabara S, Fukutake T, Yuki N, Hattori T. Clinical features and prognosis of Miller Fisher Syndrome. Neurology 2001; 56: 1104-6.
14. Mori M, Kuwabara S, Fukutake T, Hattori T. Plasmapheresis and Miller Fisher syndrome: analysis of 50 consecutive cases. J Neurol Neurosurg Psychiatry 2002; 72: 680.