Second edition

04.qxd 3/10/08 9:33 AM Page 119
limb and, if the stimulus is maintained, slow, feeble,
voluntary movements of the limbs may occur in an
attempt to remove the source of the stimulation, but
usually without tears, noise, or other manifestations of
pain or displeasure. The patient swallows readily but
has to be fed. Food seen may be recognized as such,
but there is evidently little appreciation of its taste and
other characteristics: objects normally chewed or
sucked may be swallowed whole. There is total incontinence
of urine and feces.
Cairns’ cases, occurring with third-ventricular tumors,
were generally of gradual or subacute onset. Another case
(Nielsen 1951), secondary to infarction of the cingulate
gyri, bears detailed reporting given the remarkable abruptness
of its onset. The patient was a 46-year-old woman who:
was ironing when she suddenly stopped on the spot,
complained of a severe headache, but remained standing.
Her son put her to bed, where she lay motionless,
without even speaking. She stared at the ceiling and
did not ask for anything, not even for a drink. After 9
days she was hospitalized and on the ward she continued
akinetic and mute. Under strong stimulation she
did say, ‘It hurts,’ and ‘Water,’ but that was all. She was
obviously conscious and took note of her environment
but lay day after day motionless, not deigning to call
for bed pan or food.
Etiology
Akinetic mutism has been noted with intraventricular masses
of the third ventricle, which compressed the surrounding
diencephalon (Cairns et al. 1941), following surgical damage
to the hypothalamus (Ross and Stewart 1981), in obstructive
hydrocephalus (Messert et al. 1966), and with infarction
of the anterior cingulate gyri (Barris and Schuman 1953;
Faris 1969; Freemon 1971; Nielsen 1951). Akinetic mutism
has also been seen as a side-effect of cyclosporine (Bird et al.
1990) and in a patient treated with total body irradiation
and amphotericin B (Devinsky et al. 1987).
Differential diagnosis
Akinetic mutism must be distinguished from other conditions
capable of causing immobility and mutism, including
catatonia, depression, the persistent vegetative state, the
locked-in syndrome, and stupor.
Catatonia is distinguished by the presence of waxy flexibility,
a sign not seen in akinetic mutism. Depression, when
severe and characterized by psychomotor retardation, may
resemble akinetic mutism; however, in this instance history
will reveal a typical depressive syndrome preceding the
evolution of immobility and mutism, with such symptoms
as depressed mood, anergia, anhedonia, and changes in sleep
and appetite.
4.3 Stuttering 119
The persistent vegetative state may also appear similar to
akinetic mutism; however, in this condition the eye movements
– although at times seeming purposeful – are less consistently
‘lively’; furthermore, in patients with the persistent
vegetative state one always finds a history of coma secondary
to severe traumatic brain injury, global hypoxia, etc.
The locked-in syndrome, occurring secondary to high
brainstem infarction or other lesions, such as central pontine
myelinolysis, is characterized by tetraparesis, bilateral
facial paresis, and mutism, and thus may appear similar to
akinetic mutism. Examination of eye movements, however,
will enable the correct diagnosis. In contrast with akinetic
mutism, wherein extraocular movements are full, in the
locked-in syndrome there is a paralysis of lateral gaze, with
only vertical gaze being left intact. Furthermore, and again
in contrast with akinetic mutism, in the locked-in syndrome
the patient is often desperate for communication and will
attempt to do so by utilizing the remaining vertical eye
movements in a sort of ‘Morse code’.
Stupor of various causes may leave patients immobile
and mute; however, in stupor patients are not alert and eye
movements, rather than being ‘lively’, are generally roving
or dysconjugate.
Abulia may also be considered on the differential; however,
the distinction is readily apparent upon merely
instructing the patient to do something. When left undisturbed,
the abulic patient is mute and immobile like the
akinetic mute; however, when the abulic patient is given
directions he or she follows them, in contrast with the akinetic
mute, who remains immobile.
Treatment
Anecdotally, bromocriptine is effective (Psarros et al. 2003;
Ross and Stewart 1981); treatment may be initiated at
2.5 mg/day (given in two divided doses: once in the early
morning and once in the early afternoon), and increased in
similar increments every few days until significant
improvement or limiting side-effects occur or a maximum
dose of 40 mg is reached.
4.3 STUTTERING
Stuttering, a speech dysfluency familiar to most ears,
although most commonly seen on a developmental basis,
may also, as noted below, occur on an acquired basis.
Clinical features
The phenomenology of stuttering differs according to
whether it occurs on a developmental or an acquired basis
(Helm et al. 1978).
In developmental stuttering, patients find themselves
‘blocked’ as they attempt to speak the first letter or syllable of
a word and have particular difficulty getting past the letters