CorrespondenceHead drops in advanced Huntington's disease
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)
- et al.
Head drops are also observed in advanced Huntington disease
Parkinsonism Relat Disord
(2013)
Cited by (2)
Head drops: electromyography may give the way
2017, Sleep MedicineCitation Excerpt :Among choreic syndromes, intermittent head drops have been reported as a discrete sign in patients with chorea-acanthocytosis [2]. Subsequently, they have been described also in patients with advanced HD [3,4]. EMG recordings in HD patients have shown random and usually long duration bursts and co-contraction of antagonist neck muscles, thus suggesting that sudden head drops in HD might be due to chorea affecting the neck muscles [3].
Intermittent head drops: The differential spectrum
2016, Journal of Neurology, Neurosurgery and Psychiatry