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Autoimmune Cerebellar Ataxia

Subacute or chronic cerebellar dysfunction from autoantibody-mediated cerebellar injury.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Autoimmune Cerebellar Ataxia?

Autoimmune cerebellar ataxia (ACA) encompasses paraneoplastic and non-paraneoplastic immune-mediated cerebellar disorders. Recognized antibody-associated forms include anti-Yo (PCD with breast and gynecologic cancers), anti-Hu, anti-Tr/DNER (Hodgkin lymphoma), anti-mGluR1, anti-CASPR2, anti-VGCC (Lambert-Eaton overlap), anti-GAD65 (often with stiff person syndrome and type 1 diabetes), and anti-gliadin/anti-transglutaminase 6 (gluten ataxia).

Patients present with subacute (weeks to months) gait ataxia, limb dysmetria, dysarthria, downbeat or other nystagmus, and oculomotor abnormalities. Brain MRI may show cerebellar atrophy in chronic disease and inflammatory enhancement in acute presentation. CSF often reveals lymphocytic pleocytosis and oligoclonal bands.

Diagnosis combines clinical phenotype, neural antibody panel in serum and CSF, and tumor screening with whole-body PET-CT and gynecologic exam in paraneoplastic suspicions. Treatment is high-dose corticosteroids, IVIG, plasma exchange, and rituximab or cyclophosphamide for refractory disease, alongside oncologic therapy and gluten-free diet for gluten ataxia. Early treatment correlates with better outcomes; established cerebellar atrophy responds poorly.

Symptoms

Subacute progressive gait ataxia
Limb dysmetria and intention tremor
Dysarthria and dysphagia
Downbeat or pendular nystagmus
Oculomotor saccadic intrusions
Cognitive cerebellar affective syndrome
Symptoms of underlying malignancy

Risk Factors

Underlying breast or gynecologic cancer
Hodgkin lymphoma (anti-Tr)
Type 1 diabetes (anti-GAD65)
Stiff person syndrome overlap
Celiac disease and gluten sensitivity
Coexisting autoimmune disorders
Smoking-associated SCLC (anti-Hu/VGCC)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Subacute progressive ataxia
  • Cerebellar signs with cancer history
  • Ataxia with type 1 diabetes
  • Falls and dysarthria over weeks
  • Cerebellar signs in celiac patient

Treatment Methods

01
Comprehensive neural antibody panel
02
Whole-body PET-CT for occult malignancy
03
High-dose IV methylprednisolone induction
04
IVIG or plasma exchange
05
Rituximab or cyclophosphamide for refractory
06
Tumor-specific oncologic treatment
07
Strict gluten-free diet for gluten ataxia

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.