Evidence map›Paper›PMID 41733016›Full record

ArticleEuropean journal of neurology2026

Chronic Ataxic Neuropathy With Disialosyl Antibodies Responsive to Zanubrutinib.

Benedetta Tierro, Andrea Visentin, Alessandro Salvalaggio, Gabriella Cacchiò, Nicolò Danesin, Susanna Ruggero, Francesco Logullo, Chiara Briani

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Article in European journal of neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Benedetta TierroNeurology Unit, Department of Neurosciences, University of Padova, Padova, Italy.
Andrea VisentinHematology Unit, Department of Medicine, University of Padova, Padova, Italy.ORCID 0000-0003-0271-7200
Alessandro SalvalaggioNeurology Unit, Department of Neurosciences, University of Padova, Padova, Italy.
Gabriella CacchiòNeurology Unit AST AP, Mazzoni Hospital, Ascoli Piceno, Italy.
Nicolò DanesinHematology Unit, Department of Medicine, University of Padova, Padova, Italy.
Susanna RuggeroNeurology Unit, Department of Neurosciences, University of Padova, Padova, Italy.
Francesco LogulloNeurology Unit, Pesaro Hospital, Pesaro, Italy.
Chiara BrianiNeurology Unit, Department of Neurosciences, University of Padova, Padova, Italy.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesTo describe a patient with chronic ataxic neuropathy with disialosyl antibodies (CANDA) with clinical and neurophysiological improvement after zanubrutinib therapy.

methodsA 62-year-old man started complaining of sensory symptoms in his hands and feet extending over time to the knees and elbows. Nerve conduction studies were consistent with diffuse sensory axonal ganglionopathy. Blood tests revealed an IgM 𝜆 paraprotein expression of Waldenström's macroglobulinemia. Anti-MAG and anti-neuronal antibodies were negative. Anti-ganglioside IgM antibodies (GD1b; GD2, GD3, GT1a, GT1b, GQ1b) were positive. Neurological examination disclosed pinprick and tactile hypoesthesia with stocking-glove distribution. The patient was responsive to intravenous immunoglobulin (IVIg) but reported end-dose fluctuations. Rituximab was started but soon discontinued for clinical worsening, so IVIg therapy was resumed.

resultsThe patient was started on zanubrutinib, a second generation Bruton's tyrosine kinase (BTK) inhibitor, while continuing IVIg with global improvement and absence of end of dose efficacy. After 15 months, besides a clinical and hematological amelioration, a dramatic neurophysiological improvement also occurred. Antibodies to IgM GM1 and GM2 were not detectable, whereas anti-disialosyl antibodies GD1a and GD1b persisted unchanged. DISCUSSION: To the best of our knowledge, this is the first patient with Waldenström-associated CANDA with clinical, hematological, and neurophysiological benefit after zanubrutinib therapy.

Indexed as

AtaxiaAutoantibodiesGangliosidesWaldenstrom MacroglobulinemiaHumansImmunoglobulins, IntravenousMaleMiddle AgedAutoantibodiesGangliosidesImmunoglobulins, IntravenousCANDACANOMADzanubrutinib

Identifiers

PMID41733016
PMCPMC12930296

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