Clinical and serological features of acute sensory ataxic neuropathy with antiganglioside antibodies

Abstract

There is as yet no consensus for considering pure acute sensory ataxic neuropathy (ASAN) as a variant of Guillain-Barré syndrome (GBS). Reactivity against gangliosides sharing disialosyl epitopes has been reported in these patients. The aim of this study was to determine the spectrum of reactivity against gangliosides in ASAN and to define the clinical pattern. From our database we identified patients with suspicion of ASAN. We defined ASAN as the presence of ataxia of peripheral origin with loss of proprioception, and areflexia, absence of ophthalmoplegia and no or minimal muscle weakness. Patients who met these criteria were retrospectively reviewed for their spectrum of reactivity against gangliosides and clinical features. We identified 12 patients fulfilling pre-defined criteria for ASAN. Reactivity against gangliosides containing disialosyl epitopes was present in seven patients. Concomitant reactivity against other gangliosides was present in 6/7 patients. All patients presented good prognosis and an antecedent illness was present in nine. Our results support the previously described clinico-immunological association between ASAN and disialosyl specificity, and widen the spectrum of reactivity against gangliosides. The acute presentation with a monophasic course, and good prognosis in all cases, together with transient immunoglobulin G antiganglioside antibodies and infectious antecedent in 7/12 patients support the inclusion of ASAN as a GBS variant.

Diagnosis and management of sensory polyneuropathy

“…In the past, ASAN was not considered to be a GBS variant because affected patients do not meet the diagnostic criteria for sensory GBS and lack demyelinating features on electrodiagnostic studies. Both ASAN and ataxic GBS, however, share many features with MFS including acute ataxia, areflexia, antecedent infection, and antiganglioside antibodies but lack the typical ophthalmoplegia 110112. The presence of a Romberg sign helps differentiate ASAN from ataxic GBS.…”

“…Patients with ASAN may harbor anti-disialosyl antibodies to GD1b alone or in combination with antibodies to CD3, GQ1b, or GT1a. Autoantibodies against gangliosides without disialosyl epitopes (GD1a and GM3) may also be present 112. Given that patients with ASAN typically have an antecedent infection, monophasic course, and excellent recovery, they should be considered under the rubric of GBS, in the subcategory of acute ataxic neuropathy 112.…”

“…Autoantibodies against gangliosides without disialosyl epitopes (GD1a and GM3) may also be present 112. Given that patients with ASAN typically have an antecedent infection, monophasic course, and excellent recovery, they should be considered under the rubric of GBS, in the subcategory of acute ataxic neuropathy 112. Ataxic GBS is distinguished by cerebellar-like ataxia and absence of a Romberg sign 113.…”

Mechanism and spectrum of anti‐glycolipid antibody‐mediated chronic inflammatory demyelinating polyneuropathy

“…In 1994, a study reported a patient with acute self‐limiting ataxic neuropathy who had IgG antibodies to GD1b and GD3, but not to GT1a and GQ1b . Since then, several cases with acute sensory ataxic neuropathy with IgG antibodies to GD1b alone or some of the glycolipids with at least one disialosyl residue, including GD1b, have been reported . GD1b is present in large neurons of dorsal root ganglia .…”

“…32 Since then, several cases with acute sensory ataxic neuropathy with IgG antibodies to GD1b alone or some of the glycolipids with at least one disialosyl residue, including GD1b, have been reported. 33,34 GD1b is present in large neurons of dorsal root ganglia. 35 In 1996, a study reported the induction of sensory ataxic neuropathy in rabbits sensitized with GD1b.…”