If genetic testing for FA is negative, the following disorders should be considered: ataxia with vitamin E deficiency, ataxia with coenzyme Q 10 deficiency, autosomal-recessive spastic ataxia Charlevoix-Saguenay (ARSACS), spastic paraplegia (SP), ataxia with ocular apraxia type 1 and 2 (AOA1 and 2), ataxia telangiectasia (AT), ataxias associated with POLG mutations, Aß-lipoproteinemia, Refsum’s disease, late onset Tay-Sachs disease, cerebrotendinous xanthomatosis, hereditary sensory and motor neuropathies. Here, POLG is linked to cerebellar ataxia.