McLeod syndrome (MLS) belongs to the heterogeneous group of neuroacanthocy-tosis (NA) syndromes that are characterized by involvement of both the hematological and nervous system [8, 9, 16, 28, 29]. Central nervous system symptoms of MLS are similar to Huntington's disease (HD) and comprise a choreatic movement disorder, psychiatric abnormalities, cognitive decline, and generalized seizures [9, 16]. About 150 MLS patients are reported worldwide, but data on brain pathology consist of only three cases published in either the form of a book chapter, abstracts or small case series [4, 5, 13, 22, 23]. Here we summarize the neuropathological features of these cases as reported in the literature and compare them with choreoacanthocytosis (ChAc), which is another NA syndrome of differential diagnostic interest, and HD.

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