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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