Second edition

04.qxd 3/10/08 9:33 AM Page 124
124 Other signs and symptoms
Cantu and Drew 1966) and also with compression of the
brainstem, as for example by a meningioma (Motomura
et al. 1980).
Differential diagnosis
‘Emotionalism’ is not at all uncommon after strokes but it
is readily distinguished from the emotional incontinence
of pseudobulbar palsy by the fact that ‘emotional’ patients
experience an affect congruent with their facial expression,
whereas the emotionally incontinent patient feels neither
sadness nor mirth and is often as surprised at the emotional
display as is the observer.
Lability of affect, as may be seen in mania, is, like emotionalism,
distinguished from emotional incontinence by the
presence of an affect congruent with the facial expression.
Episodes of mirthless laughter may also occur in an isolated
fashion as le fou rire prodromique and as a simple partial
seizure in gelastic epilepsy. Le fou rire prodromique, as
discussed in Section 4.9, represents a prodrome to stroke
and is distinguished from the emotional incontinence of
pseudobulbar palsy in that the mirthless laughter of emotional
incontinence occurs after stroke. Gelastic seizures
are suggested by a history of other seizure types, such as
grand mal or complex partial seizures.
Treatment
In placebo-controlled, double-blind studies, several medications
have been shown to be effective for emotional
incontinence. For cases occurring secondary to infarction
nortriptyline (in doses of from 50 to 100 mg) (Robinson
et al. 1993), imipramine (in doses of 10–20 mg) (Lawson
and McLeod 1969), and citalopram (in doses of 10–20 mg)
(Andersen et al. 1993) are each effective. For emotional
incontinence in ALS (Brooks et al. 2004) a combination of
dextromethorphan (30 mg) and quinidine (30 mg), given
twice daily, is also effective: in this preparation, quinidine
is used merely in order to inhibit the metabolism of dextromethorphan,
which is the active agent. In cases of MS,
both amitriptyline (Schiffer et al. 1985) and the combination
of dextromethorphan and quinidine are effective
(Panitch et al. 2006).
It must be stressed that although amitriptyline, nortriptyline,
imipramine, and citalpram are all antidepressants, they
are effective regardless of whether patients are depressed or
not; furthermore, although the response to both amitriptyline
and nortriptyline may take weeks, the response to
citalopram may be very rapid, with some patients getting
relief within a day or two.
It is not clear whether the agents that are found to be
effective in stroke cases would be effective in amyotrophic
lateral sclerosis or multiple sclerosis, and vice versa, and it is
not clear whether any of these agents would be effective in
emotional incontinence of other causes. In choosing among
these agents, amitriptyline, nortriptyline and imipramine
are probably second choices, given their side-effect burdens;
citalopram, in contrast, is extraordinarily easy to use.
In cases when citalopram is ineffective, one might consider
dextromethorphan or one of the other antidepressants;
nortriptyline would probably be the best of these, given its
lower side-effect burden.
4.8 EMOTIONAL FACIAL PALSY
Most central facial palsies are of the ‘voluntary’ sort, in that
when patients are asked to voluntarily perform a facial
maneuver, such as showing their teeth, there is a droop evident
on one side; less commonly one finds an ‘emotional’
facial paresis wherein, although voluntary movements are
intact, a ‘droop’ becomes evident when the patient smiles
in response, say, to a joke. This emotional facial paresis has
also been termed ‘mimetic’ or ‘involuntary’ facial paresis.
Clinical features
Facial palsies may be either peripheral or central. In a
peripheral facial palsy, there is no movement in either the
forehead or the lower face, whether on voluntary command
or in response to a joke. Central facial palsies are
immediately distinguished from peripheral palsies in that
this palsy affects only the lower half of the face, with forehead
movements being spared.
The two forms of central facial palsy, namely voluntary
and emotional, may be distinguished by first noting facial
movements when patients are instructed to voluntarily
show their teeth and then, at some point in the examination,
by closely observing facial movements when the
patient spontaneously smiles, for example in response to a
joke or recollection of some happy memory (Monrad-
Krohn 1924).
In voluntary central facial paresis there is a droop of one
side of face when patients are asked to show their teeth;
however, when these patients are observed smiling at a
joke, one sees full facial movements on both sides.
In emotional facial paresis there is full movement bilaterally
when patients are asked to show their teeth; however,
when one observes these patients smiling at a joke, there is
drooping of one side of the face; this produces what might
be called a ‘hemismile’.
Etiology
Voluntary and emotional facial paresis are dissociated
because the corticobulbar fibers subserving these two functions
are separate.
Corticobulbar fibers for voluntary facial movement
arise in the posterior portion of the frontal cortex, descend
through the corona radiata to the posterior limb of the
internal capsule and then travel through the ventral mesencephalon
in the crus cerebri to the basis pontis. At this