Table 2.

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

	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

Epidemiological data are cited from our previous Consensus paper [2]

Abbreviations: SCLC, small cell lung carcinoma, TG2 Ab and TG6 Ab anti-transglutaminase 2 and 6 Abs