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Table 2 Clinical features of the main types of immune-mediated cerebellar ataxias

From: Immune-mediated cerebellar ataxias: from bench to bedside

 

Gluten ataxia

Paraneoplastic cerebellar degeneration

Anti-GAD65 Ab-associated cerebellar ataxia

Gender

Women in 50–60% of patients

 

Mostly women (80–90%)

Mean age (years)

Mostly 40–50 (median 48)

26–85 (median 61)

Mostly 50–60 (mean 58)

Clinical course

Chronic/insidious

Subacute

Subacute or chronic/insidious

Cerebellar signs

Gait ataxia is predominant (100%), accompanied by upper limb (75%) and lower limb ataxia (90%), dysarthria (656%), and nystagmus (84%).

Pancerebellar cerebellar ataxias, which are sometimes preceded by nausea, vomiting and dizziness.

Gait ataxia is predominant (100%), accompanied by limb ataxia (71%), dysarthria (66%), and nystagmus (64%)

Other symptoms

Sensorimotor axonal neuropathy, gluten-sensitive enteropathy, gastrointestinal symptoms, focal myoclonus, palatal tremor, and opsoclonus

Malignancy, e.g. breast, uterus, ovaries, SCLC, Hodgkin’s disease, thymoma

Stiff-person syndrome, epilepsy, myasthenia gravis

Associated autoimmune diseases

Thyroiditis, T1DM, pernicious anemia

Not correlated

T1DM, thyroiditis, hemolytic anemia

Autoantibodies

Anti-gliadin Ab, TG2 Ab, TG6 Ab

Anti-Yo, Hu, Tr, CV2, Ri, Ma2, and VGCC(P/Qtype) Abs

Anti-GAD65 Ab, TPO, TG, ANA: 30/41 (73%)

Cerebellar atrophy on MRI

Normal or mild atrophy

Initially normal (during subacute phase)

Normal or mild atrophy

  1. Epidemiological data are cited from our previous Consensus paper [2]
  2. Abbreviations: SCLC, small cell lung carcinoma, TG2 Ab and TG6 Ab anti-transglutaminase 2 and 6 Abs