Miller Fisherov sindrom

Miller Fisherov sindrom (MFS) neuropatija je nazvana po neurologu Charlesu Milleru Fisheru koji je 1956. godine opisao tri pacijenta s kliničkim trijasom kojeg su sačinjavali oftalmoplegija, ataksija i arefleksija bez istaknutih znakova periferne neuropatije. Ovaj sindrom opisan je kao varijanta Guillain-Barréova sindroma. Kod Miller Fisher varijante, oko 96% bolesnika pozitivno je za anti-GQ1b antitijela (protutijela na glikolipid izražen na aksolemi kranijalnih živaca). MFS je rijetka bolest. Incidencija je procijenjena na otprilike 1-2 slučaj na milijun stanovnika godišnje. Pogađa svaku životnu dob, uključujući i djecu. Dvostruko se češće javlja kod muškaraca nego kod žena. Osim karakterističnog trijasa (oftalmoplegija, ataksija i arefleksija), mogu se javiti i facijalna pareza te senzorni ispadi. MFS obično prati infekciju, najčešće Campylobacter jejuni. U podlozi nastanka bolesti su antigangliozidna antitijela koja križno reagiraju s lipopolisaharidnim antigenima prisutnim kod infektivnih uzročnika. Kod većine pacijenata s MFS-om, dijagnoza se može postaviti klinički na temelju karakterističnog trijasa simptoma. Pretrage koje su od pomoći su: određivanje serumskih antitijela, MRI, nalaz cerebrospinalne tekućine, elektrofiziološki nalazi, elektromiografija te ostali laboratorijski nalazi. MFS je samoograničavajuća bolest i oporavak je u većini slučajeva spontan. Prognoza je dobra, oporavak se očekuje u prosjeku nakon 10 tjedana.Miller Fisher syndrome (MFS) is a neuropathy named after neurologist Charles Miller Fisher who in 1956 described three patients with clinical triad comprising of ophthalmoplegia, ataxia and areflexia without prominent signs of peripheral neuropathy. This syndrome has been described as a variant of Guillain-Barre syndrome. In Miller Fisher variant, about 96% of patients are positive for anti-GQ1b antibodies (antibodies to glycolipid expressed on cranial nerves). MFS is a rare disease. The incidence is estimated to be about 1-2 cases per million inhabitants per year. It affects every age, including children. It occurs more frequently in men than in women. In addition to the characteristic clinical triad (ophthalmoplegia, ataxia and areflexia), facial paresis and sensory dropouts also may occur. MFS usually follows an infection, most commonly Campylobacter jejuni. In the background of disease are anti-ganglioside antibodies that cross-react with the lipopolysaccharide antigen present in infectious agents. In most patients with MFS, the diagnosis can be set on the basis of clinical symptoms of clinical triad, and investigations that can be helpful are determination of serum antibodies, MRI, CSF analysis, electrophysiological findings, electromyography and other laboratory findings. MFS is a self-limiting disease and recovery is spontaneous in most cases. The prognosis is good, the recovery is expected after about 10 weeks