Dr. Weimer of Columbia University has received consulting fees from Roche.)
This article includes discussion of polyneuropathy associated with antisulfatide antibodies and sulfatide neuropathies. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Antisulfatide neuropathy is an immune-mediated neuropathic disorder. It presents most commonly as an axonal sensory predominant polyneuropathy. However, demyelination may be seen in some cases. GALOP syndrome, a variant of antisulfatide neuropathy, is a disabling gait disorder in the elderly. Patients typically have gait ataxia and a distal sensory predominant polyneuropathy. This neuropathy is mostly demyelinating. Clinical testing for antisulfatide and anti-GALOP antibodies is useful in slowly progressive sensory or sensorimotor neuropathies affecting patients older than 50 years of age. Treatment for both involves neuropathic pain management and immunosuppression in the presence of demyelination.
Historical note and terminology
Antibodies against sulfatide, the major acidic glycosphingolipid in myelin, have been reported in a variety of systemic and neurologic disorders. These antibodies have been found in disorders such as idiopathic thrombocytopenic purpura (van Vliet et al 1987), autoimmune chronic active hepatitis (Toda et al 1990), HIV (Petratos et al 1999b; Lopate 2005), multiple sclerosis (Ryberg 1978), Guillain-Barré syndrome (Fredman et al 1991; Ilyas et al 1991; Van den Berg et al 1993), and chronic inflammatory demyelinating polyradiculoneuropathy (Pestronk et al 1991; Melendez-Vasquez et al 1997). Most studies have shown an association between highly elevated titers of antisulfatide antibodies and peripheral neuropathy (Nemni et al 1993; Latov 1995; Shigeta et al 1997; Ferrari et al 1998; Dabby et al 2000). In the first report describing antibodies to sulfatide in neuropathy, high titers were found in patients with chronic axonal, predominantly sensory neuropathy (Pestronk et al 1991). Other reports followed, but patients did not appear to constitute a single clinical syndrome, and some patients had demyelination, sometimes in association with antibodies against other myelin antigens (Ilyas et al 1992; Fredman et al 1993; Nobile-Orazio et al 1994).
Pestronk and colleagues identified extremely high titers of IgM binding to a CNS myelin antigen that co-purified with myelin associated glycoprotein in patients with gait ataxia and sensorimotor polyneuropathy. This clinical syndrome was termed the GALOP syndrome (gait disorder, autoantibody to a neural antigen, late-age onset, and polyneuropathy of mild to moderate severity) (Pestronk et al 1994). The anti-CNS myelin antigen antibody was also found to cross react with both sulfatide and myelin associated glycoprotein. Pestronk's studies suggested that titers greater than 1:10,000, with no cross-reactivity to GM1 ganglioside, have specificity for this syndrome. However, since that report, GALOP syndrome is now considered a variant of the antisulfatide syndrome, as the initially unknown antigen that co-purified with myelin associated glycoprotein was subsequently determined to be sulfatide (Donofrio 2003; Kornberg and Pestronk 2003). Meehan and colleagues created some new and further refined antisulfatide antibodies that show additional reactivity; further study is needed to assess the utility (Meehan et al 2018).
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