A Case of Senile Chorea:  Considering Huntington’s Disease and Neuroacanthocytosis in differential diagnosis

Ayşe Deniz Elmalı; Ayşegül Gündüz; Zafer Başlar; Fatoş Sibel Ertan

doi:10.4274/tnd.34545

Ayşe Deniz Elmalı¹, Ayşegül Gündüz¹, Zafer Başlar², Fatoş Sibel Ertan¹

¹Istanbul University, Cerrahpasa Medical Faculty, Department Of Neurology
²Istanbul University, Cerrahpasa Medical Faculty, Department Of Internal Medicine, Division Of Hematology

Keywords: chorea, senile chorea, Huntington Disease, chorea-acanthocytosis, neuroacanthocytosis

Abstract

Sporadic chorea presenting after the age of 50 is called “senile chorea”. Senile chorea is a rare entity with a wide differential diagnosis list. Causes of senile chorea include vascular and metabolic diseases, adverse events related to medications, hematologic and immune system diseases, genetic and sporadic neurodegenerative syndromes, and paraneoplastic disorders. Although the most common etiologies are vascular and metabolic disorders, neuroacanthocytosis, Wilson and Huntington diseases are included in the differential diagnosis.

Here, we discuss differential diagnosis and approach to late onset chorea based on a case with late onset chorea, whose clinical findings suggested chorea-acanthocytosis at first, but revealed to be Huntington disease after detailed laboratory studies.