Table 5: Additional neuropathological states and conditions in which FNS is invariably part of the neurological syndrome [2224].

Subcortical gray matter
 HIV dementia
 Wilson’s disease
 Neuroacanthocytosis syndrome
 Prionopathies (Creutzfeldt-Jakob, GSS FFI, and BSE)
 Fahr’s syndrome—calcification of the BG
 Pantothenate kinase 2 associated neurodegeneration (PANK2)
 Adult neuronal ceroid lipofuscinosis
Subcortical white matter
 Leukodystrophy disorders (metachromatic, Krabbe’s, adrenal, orthochromatic)
 Fabry’s disease
 Vanishing white matter disease
Mixed cortical and subcortical pathology
 Alexander’s disease
 Canavan disease
 Cerebrotendinous xanthomatosis
 Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) or Nasu-Hakola disease
 Mitochondrial diseases (MELAS, MERFF, and Kearns Sayre)