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Head drops in advanced Huntington's disease

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Introduction

Recently, Spampinato U et al. described head drops in two molecular confirmed positive HD patients [1].

“Head drops” has been described as a movement disorder characterized by flexion of the neck that could be associated to sudden striking backward extension [2]. Although its nature and pathophysiology remain under discussion, different patterns of movement including chorea, tics or dystonia, secondary to basal ganglia involvement have been proposed.

The occurrence of head drops was previously reported as a characteristic feature of “advanced” choreic disorders, such as McLeod syndrome and autosomal recessive chorea-acanthocytosis [2], [3]. On this way, Schneider SA et al. proposed this sign as a useful clue toward the differential diagnosis between chorea-acanthocytosis (ChA) and Huntington's disease (HD) [3].

Herein, we report a new case of head drops in a 71 year-old man with advanced HD.

Section snippets

Case 1

This 71-year-old man developed slowly progressive motor, behavioral and cognitive dysfunction at age 55. His family history was positive for Huntington's disease with at least two relatives affected (his mother, who was clinically diagnosed of HD and a premanifest son with a positive genetic testing with 42 nCAG repeats). He has a personal history of rheumatoid arthritis and cardiac arrhythmia. Genetic analysis at age 60 confirmed HD, with 41 nCAG repeats in the expanded allele and 19 nCAG

Financial disclosure

Authors have nothing to disclose related to the manuscript.

Conflict of interest

Authors have nothing to disclose related to the manuscript.

Acknowledgment

We thank to the patient and his family for their contribution to this report.

References (3)

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