Akut motor aksonal nöropati ile giden bir Guillain barre varyantı
Akut motor aksonal nöropati, akut motor paralizi, arefleksi ya da hiporefleksi, pek az duysal kayıp, beyin-omurilik sıvısında albumino-sitolojik uyumsuzluk ve elektrofizyolojik çalışmalarda demiyelinizan bulgular olmaksızın periferal aksonal tutulum ile giden bir Guillain Barre sendromu varyantıdır ve etiyolojide camphylobacter jejuni, sitomegalovirüs, Epstein Barr virus, influenza, mikoplasma, koksaki virus ve hepatit virüsleri, geçirilmiş cerrahi, doğum ve bazı aşılar suçlanmaktadır. Alt ekstremitelerde başlayan ilerleyici kas güçsüzlüğü, hiporefleksi ve beyin-omurilik sıvısında albumino-sitolojik uyumsuzluğu olan, elektrofizyolojik çalışmasında akut motor aksonal nöropati ile uyumlu bulgular saptanan ve intravenöz immunglobulin tedavisine iyi yanıt veren 16 yaşında erkek olgu sunuldu.
A Guillain-Barre variant with acute motor axonal neuropathy
Acute motor axonal neuropathy is a Guillain-Barre syndrome variant, characterized by acute motor paralysis, areflexia or hyporeflexia, minimal sensory loss, albumino-cytologic dissociation in the cerebrospinal fluid and evidence of peripheral axonal involvement without evident demyelination findings in electrophysiologic studies. It is most frequent in the rural areas of China during summer months. Campylobacter jejuni, cytomegalovirus, Epstein Barr virus, influenza, mycoplasma, coxsackie viruses, hepatitis viruses, antecedent surgery, childbirth and some vaccines are considered as etiologic factors. We present a 16-year-old boy who presented with progressive weakness that initiated in lower extremities, had hyporeflexia, albumino-cytologic dissociation in cerebrospinal fluid, evidence consistent with acute motor axonal neuropathy in electrophysiologic studies and responded well to intravenous immunoglobulin therapy.
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- 1- Hiraga A, Mori M, Ogawara K, et al. Recovery patterns and long term prognosis for axonal Guillian-Barre syndrome. J Neurol Neurosurg Psychiatry 2005; 76:719-722.
- 2- Phillips JR Kincaid JC, Bhuwan PC Acute motor axonal neuropathy in childhood: clinical MRI findings. Pediatr Neurol 1997; 16:152-155.
- 3- Levin KH, Variants and mimics of Guillain Barre syndrome. The Neurol 2004; 10:61-74.
- 4- Kuwabara S, Ogawara K, AAizobuchi K, et al. Mechanisms of early and late recovery in acute motor axonal neuropathy. Muscle Nerve 2001; 24:288-291.
- 5- Alam TA, Chaudhry V, Cornblath DV Electrophysiological studies in the Guillain-Barre syndrome: distinguishing subtypes by published criteria. Muscle Nerve 1998; 21:1275-1279.
- 6- Susuki K, Nishimoto YYamada M, et al. Acute motor axonal neuropathy rabbit model: immune attack on nerve root axons. Ann Neurol 2003; 54:383-388.
- 7- Kuwabara S, Bostock H, Ogawara K, et al. The refractory period of transmission is impaired in axonalTjuillain-Barre syndrome. Muscle Nerve 2003;28:683-689.
- 8- Kuwabara S, Ogawara K, Sung J, et al. Differences in membrane properties of axonal and demyelinating Guillain-Barre syndromes. Ann Neurol 2002; 52:180-187.
- 9- Hughes R.A.C, Hadden R.D.M, Gregson N.A, et al. Pathogenesis of Guillain-Barre syndrome. J Neuroimmunol 1999; 100:74-97.
- 10-Yuki N, Kuwabara S, Koga M, et al. Acute motor axonal neuropathy and acute motor-sensory axonal neuropathy share a common immunological profile. J Neurol Scie 1999; 168:121-126.
- 11-Kuwabara Sr Ogawara K, Misawa S, et al. Does campylobacter jejuni infection elicit "demyelinating" Guillain Barre syndrome? Neurology 2004; 63(3):529-33.
- 12-Susuki K, Odaka M, Mori Mr et al. Acute motor axonal neuropathy after Mycoplasma infection: Evidence of molecular mimicry. Neurology 2004; 62:949-56.
- 13-Hiraga A, Kuwabara S, Ogawara K, et al. Patterns and serial changes in electro diagnostic abnormalities of axonal Guillain Barre syndrome. Neurology 2005; 64:856-60.
- 14-Kuwabara S. Guillan-Barre syndrome: epidemiology, pathophysiology and management. Drugs 2004; 64:597-610.
- ISSN: 1300-0853
- Yayın Aralığı: Yılda 6 Sayı
- Başlangıç: 1992
- Yayıncı: TÜRK TABİPLERİ BİRLİĞİ