Second edition

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lesions of the temporal lobe (Mazzucchi et al. 1982; Sparr
2002). Expressive amusia has been noted with lesions of
the superior temporal cortex (McFarland and Fortin 1982)
and of the frontal cortex (Botez and Werheim 1959).
Global amusia has been seen with lesions of the superior
temporal cortex (Piccirilli et al. 2000) and also of Heschl’s
gyrus (Russell and Golfinos 2003). Typically, lesions are
found in the non-dominant hemisphere.
Expressive amusia has also been reported as a manifestation
of a simple partial seizure, with the seizure focus being
found in the right temporo-occipital region (Bautista and
Ciampetti 2003).
Global acquired amusia of gradual onset and slow progression
has also been reported secondary to a neurodegenerative
disorder characterized by bilateral atrophy of
the frontotemporal cortices (Confavreux et al. 1992).
Developmental, or ‘congenital’ amusia, is not uncommon
and such ‘tone deaf’ individuals have a variable
degree of difficulty in recognizing tunes and singing/playing
instruments (Ayotte et al. 2001).
Differential diagnosis
Aprosodia is distinguished from amusia by the fact that
aprosodia is related to the emotional tone with which one
speaks, whether happy, angry or sad, whereas amusia is
related to the tune with which one sings.
Treatment
There are no established treatments.
4.21 FOREIGN ACCENT SYNDROME
The foreign accent syndrome, wherein patients speak with
an accent foreign to their native tongue, is a rare syndrome
that typically evolves out of either a motor aphasia, or an
aphemia, in stroke patients.
Clinical features
As noted, patients present initially with either a motor
aphasia (Christoph et al. 2004) (or its transcortical variant
[Graff-Radford et al. 1986]) or an aphemic mutism (Schiff
et al. 1983; Takayama et al. 1993): as the motor aphasia or
the mutism resolve, patients begin to speak intelligibly but
with a foreign accent. One native English speaker spoke
with an Irish brogue (Seliger et al. 1992), another with a
French accent (Hall et al. 2003), and another with a Chinese
accent (Schiff et al. 1983). In one most unfortunate case, a
Norwegian, who developed the syndrome during World
War II, spoke in such a convincing German accent that she
was suspected of being a traitor (Monrad-Krohn 1947).
Etiology
4.22 Cataplexy 137
Lesions are found in the left hemisphere, typically in the
posterior–inferior portion of the frontal cortex (Schiff et al.
1983; Takayama et al. 1993) or subjacent white matter
(Blumstein et al. 1987); cases have also been reported secondary
to infarction of the left basal ganglia (Fridriksson et
al. 2005), and there is one report of the syndrome occurring
with infarction of the mid-portion of the body of the
corpus callosum (Hall et al. 2003).
There are also report of the syndrome occurring in the
course of schizophrenia (Reeves and Norton 2001): in one
such case the patient, who had the delusion that he was
connected with British royalty, spoke with a British accent
(Reeves et al. 2007).
Differential diagnosis
Motor aphasia is distinguished by the characteristic effortful
speech that stands in contrast with the fluent and effortless
speech of patients with the foreign accent syndrome; it
is distinguished from dysarthria by the fact that in the foreign
accent syndrome there is simply no slurring of speech.
Treatment
There is no known treatment.
4.22 CATAPLEXY
Cataplexy is a condition characterized by the occurrence of
cataplectic attacks, that is to say episodes of a greater or
lesser degree of muscle atonia.
Clinical features
Cataplectic attacks (Adie 1926; Dyken et al. 1996;
Guilleminault et al. 1974; Kales et al. 1982; Parkes et al.
1975; Wilson 1928) are generally precipitated by some
strong emotion, such as laughter or anger, and are characterized
by the paroxysmal onset of more or less generalized
weakness lasting on the order of seconds to a minute or so:
subsequent recovery is rapid and complete. During the
attack, all voluntary muscle power, with the exception of
the diaphragm and, at times, the extraocular muscles, is
lost, and patients may fall to the ground or slump in a
chair. In some cases, the muscle weakness, although generalized,
may be of minor degree, and such patients may
merely experience their heads lolling forward, their jaws
slackening, and their knees beginning to buckle. Limited
attacks, confined perhaps to an arm or leg, have also
been reported. Attacks that last much longer than a minute
may be joined by visual or auditory hallucinations