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Tremor Other Hyperkinet Mov (N Y). 2017 Mar 25;7:453. doi: 10.7916/D8KW5MRG. eCollection 2017.

Early Head Tremor in Essential Tremor: A Case Series and Commentary.

Author information

1
Division of Movement Disorders, Department of Neurology, Yale School of Medicine, New Haven, CT, USA; Department of Chronic Disease Epidemiology, Yale School of Public Health, New Haven, CT, USA; Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, New Haven, CT, USA.
2
Division of Movement Disorders, Department of Neurology, Yale School of Medicine, New Haven, CT, USA.
3
Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, NY, USA; Department of Pathology and Cell Biology, Columbia University Medical Center, New York, NY, USA.

Abstract

BACKGROUND:

Classically, the onset of head tremor in essential tremor (ET) patients follows that of hand tremor, such that there is a somatotopic spread of involved areas. Here we present a series of seven self-reportedly "unaffected" relatives of ET cases. These seven were clinically asymptomatic and had normal levels of arm tremor on examination, yet each evidenced a transient head wobble on examination. We estimate the prevalence of this phenotype within the two studies from which cases were ascertained.

METHODS:

ET cases and their self-reportedly affected and unaffected relatives, enrolled in two family studies, underwent a medical history and videotaped neurological examination.

RESULTS:

In seven self-reportedly "unaffected" relatives, a transient and subtle head wobble was seen, always during sustained phonation, speech, or reading aloud. Total tremor score (a measure of arm tremor) ranged from 5 to 12 (i.e., mild tremor within the range of normal). The prevalence of this phenotype of early head tremor was 3.7% in one study and 23.1% in the other.

DISCUSSION:

We present a series of seven individuals who had early head tremor in an evolving transition state from normal to ET. These cases raise a number of broad clinical, phenotypic, and pathophysiological issues about ET.

KEYWORDS:

Essential tremor; clinical; head tremor; phenotype

Conflict of interest statement

Funding: This work was supported by the National Institutes of Health (NINDS #R01 NS094607 and NINDS #R01 NS073872). This funding body played no role in the design of the study, the collection, analysis, and interpretation of data, or the writing of the manuscript. Conflict of Interest: The authors declare no conflict of interest. Ethics Statements: All patients that appear on video have provided written informed consent; authorization for the videotaping and for publication of the videotape was provided.

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