A five-year-old, developmentally-normal girl
presented with involuntary, intermittent trembling of the chin noted
since early infancy (Web Video 1 and
Fig. 1). There were no triggering-factors. These movements could
not be initiated or suppressed voluntarily. These were absent during
sleep. No abnormal movement of jaw, face, head, or any other body part
were noted. There was no functional or social impairment. Physical
examination, neuroimaging and electro-encephalography (EEG) was
unremarkable. Father of the child had similar chin- trembling; the
intensity and duration of which had decreased with age.
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Fig. 1 Spontaneous, involuntary,
intermittent movements of the mentalis muscle; chin myoclonus.
(see video at website)
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Chin-trembling or geniospasm is a rare movement
disorder, caused by continuous or intermittent tremulous activity of the
mentalis muscle. It may be familial (autosomal-dominant) or sporadic. It
is characterized by paroxysmal, rhythmic, up-and-down movements of the
chin and/or lower-lip with episodes lasting from seconds to hours.
Episodes may occur spontaneously or be precipitated by stress,
concentration, and emotion. These typically become apparent in infancy
or in early-life, and the episodes tend to reduce in frequency/intensity
with advancing age. Impairments include social embarrass-ment, and
interference with speech, feeding or sleep.
Electrophysiological studies have suggested these to
be a form of subcortical myoclonus, and Hereditary chin myoclonus has
been proposed to be a better term to describe these movements. Treatment
is usually not required; benzodiazepines, haloperidol, phenytoin and
botulinum toxin have been tried in desperate situations with limited
success.
Similar-looking movements may be observed in
association with essential tremor, palatal tremor and facial myokymia.
Close mimickers also include Essential tremor of the jaw and
tremors-of-the-smile. The clinical and electrophysiological features may
help in differentiation.