Table 2. Differential diagnosis of ACA.

Metabolic	Infections	Hereditary	Other autoimmune disease	Nutrition/toxic	Others
Mitochondrial disorders	Viral (EBV, VZV, JCV, prion, HIV)	Channelopathies	SLE	Drugs	Stoke
Amino acid/organic acid metabolism disorders: MSUD, MMA	Bacterial (Streptococcus, Tropheryma whipplei)	Autosomal-dominant/recessive ataxias	Behcet’s	Alcohol abuse	Trauma
Storage disease: NPC	Mycoplasma		CNS demyelinating diseases	Vitamin deficiencies: Wernicke encephalopathy, others	Posterior cranial fossa tumor
GLUT1-DS	Others (syphilis, tuberculosis, borreliosis)				Endocrine (hypothyroidism, hypoparathyroidism)

ACA, autoimmune cerebellar ataxia; EBV, Epstein-Barr virus; VZV, Varicella-zoster virus; JCV, JC virus; SLE, systemic lupus erythematosus; MSUD, maple syrup urine disease; MMA, methylmalonic acidemia; NPC, Niemann-Pick disease type C; CNS, central nervous system; GLUTI-DS, glucose transporter type 1 deficiency syndrome.