Nruropsychofogm,
Printed in Great

Vol. 29, No. II, pp. lll~ll27,
Britain.

RIGHT-SIDED

1991
0

0028-3932/91 $3.00~0.00
1991 Pergamon
Press plc

ANARCHIC (ALIEN) HAND: A LONGITUDINAL
STUDY

SERGIO DELLA SALA,*? CLELIA MARCHETTI~ and HANS SPINNLER~
*Department
of Clinical Neurophysiology,
San Paolo Hospital, Milan; tNeuropsychology
Unit, Clinica del
Lavoro Foundation,
Medical Centre of Rehabilitation,
Veruno (NO); and fFirst Department
of Neurology,
University of Milan, San Paolo Hospital, Milan
(Received 29 January 1991; accepted 2 June 1991)
Abstract-A
patient with a bilateral frontal vascular lesion, encroaching
upon the mesial cortex on
the left and damage of the corpus callosum showed the ‘alien hand’ phenomenon
on the right.
The various hypotheses as to the nature of the lesion for the alien hand phenomenon
to appear are
discussed. It is proposed that an acute clinical condition, following a lesion of the corpus callosum
only, should be differentiated
from a chronic condition resulting from the additional fronto-mesial
lesion.

INTRODUCTION
THE PHENOMENONcalled ‘alien hand’ (AH) consists of the occurrence of movements of an
upper limb that are unintended
although clearly directed to some purpose. The ‘alien’ hand,
seems to act autonomously,
carrying out complex movements against the subject’s verbally
reported will, that can interfere,with the development
of an intentional
action that the other
hand has begun. The subject is fully aware of the abnormal behaviour of one hand but he is
not able to inhibit it. This is both unpleasant
and frightening for him.
Doubts abound as to the crucial site of the lesion and how large it has to be for AH to
appear.
Interpretations based on callosal lesion only
All the reported cases of AH that have developed after only a callosal interruption
show
the phenomenon
on the left side [l, 9, 541. This was interpreted
as the result of the
disconnection
of the area devoted to complex motor activities in the left hemisphere
[ 17, 30, 313 from the area on the right in which the motor cortex for the left hand is located.
This would exclude right-handed
subjects from having AH on the right. In support of this
interpretation
there are some patients reported with left AH whose neuro-radiological
examination
failed to show any additional involvement
of the cortical areas [26, 511.
Interpretations based on a requirement of a double, callosal and cortical, lesion
After GOLDBERG [21] described two patients with AH on the right side, the explanation
involving the corpus callosum (CC) only, appeared inadequate
and it was proposed that a
SAddress for correspondence:
Hans Spinnler, Clinica Neurologica
I, Polo Didattico
Studi, Ospedale San Paolo alla Barona, Via A. Stanabba di Rudini 8, 20142 Milano.
III3

S. Paolo,

Universita

degli

1114

S. DELLA SALA. C. MARCHETTI and H. SPINNLER

double lesion, namely, both of the CC and of the fronto-mesial cortex (precisely, of the
Supplementary Motor Area, SMA) contralateral to the AH meets the essential pathological
requirement of AH.
Since this condition seldom occurs, it is worth describing each case, as this may contribute
to a clearer definition and interpretation of AH.and will possibly enable us to develop a
metanalysis. We describe a 3-year follow-up of a right-handed patient with callosal and
bilateral frontal vascular lesion presenting with a stable right AH.

CASE REPORT
G.C. is a 56-year-old right-handed
woman who first came to us in January 1988. A year before she suffered a
subarachnoid
haemorrhage
and was operated on 2 days later to clip two aneurisms of the anterior communicating
artery. After she roused from a 20-day coma, the patient had language disorders and right hemiplegia.
The language disorders
(comprehension
well-preserved,
spontaneous
speech nearly nonexistent,
repetition
possible and correct) was compatible with a transcortical
motor aphasia syndrome. The disorder cleared up within a
few months. The motor deficit also improved and was all but gone when we first saw her.
In the first months after the operation her behavioural characteristics
fitted both an adynamic (abulic-apatheticakinetic) and moriatic (‘Witzelsucht’) syndrome. Both of these features subsided with time and when we began to
study G.C. only slight stable abulia remained. She was forgetful in everyday life with a clear-cut deficit of ongoing
memory.
As the motor symptoms cleared up, severe disturbances
of organization
of voluntary gestures and of bimanual
coordination
became apparent. The right hand frequently carried out complex activities that were not willed by
G.C. These activities were clearly goal-directed
and were well executed, but undesired by the patient, who used her
left hand to try to stop them. For example, when the patient had a steaming cup of tea in front of her, the right hand
proceeded to pick it up and bring it to her mouth, even though the patient knew that it was too hot and had just said
she would wait a few moments until it had cooled. Nevertheless it needed the intervention of her left hand to replace
the cup on the table. The will to do so was not sufficient to modify the directed but inappropriate
behaviour of the
right hand. On other occasions, when G.C. had a genital itch, the right hand scratched it vigorously, in view of other
people, causing considerable embarrassment
to the patient, who tried to stop the right hand with her left. These are
only two examples ofmany such manifestations
ofimpulsive, context-dependent
autonomous
behaviour of the right
hand. At other times, the right hand interfered with intentional movements of the left hand, carrying out complex
and organized activities that did not appear to be so closely context-dependent.
For example, while the left hand was
using some object, the right hand would try to grab it and use it, against the will of the patient, in some way different
from the original plan. The patient considered her left hand to be the one she could trust, while the right hand, which
could make motions completely without her wanting it to, was the untrustworthy
one that ‘always does what it
wants to do’. The patient often experienced this problem with the right hand during her daily activities and in order
to complete the activity she wanted to complete, she had to prevent the ‘wayward’ right hand from interfering,
sometimes by force (by hitting it or by sitting on it). The need to take these precautions,
which had only fleeting
effects, and the frequent failures produced a marked feeling of frustration.
In spite of the inconveniences
caused by
this motor behaviour of the right hand, G.C. never denied (even in response to direct questioning) that this ‘alien’
hand belonged to her (i.e. she had no sign of asomatognosia).
She never had a depressed or anxious reaction, but was
rather annoyed by her symptoms. She was well aware of the problem and that the disturbance consisted exclusively
of the right-hand functioning autonomously
against her will.
Neurological examination
The neurological examination in January 1988 (i.e. a year after the rupture of the aneurism) showed slight paresis
of the right limbs, which could still carry out all the movements. Movements for reaching or picking up objects
placed in the peri-personal
space were normal. Two-handed
tasks, even relatively complex ones (e.g. making a
package, folding a letter and placing it in an envelope), were done correctly and with good coordination
of the two
hands. Sensation was normal in all modalities. The right hand showed tonic grasping; there were no other cortical
disinhibition
signs or perseveration
of motor acts.
Neuroradiology
The CT scan carried out in May 1987, showed a bilateral fronto-basal
paramedian
hypodensity,
involving the
anterior part of the CC and extending more to the left so as to presumably encroach upon the supplementary
motor
area of that side (Fig. 1). This could not be confirmed by Nuclear Magnetic Resonance because of the metal clips in
the patient’s head.

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1117

Neuropsychological examination
A standard language examination,
the Aachener Aphasie Test [ 14,331 (January 1988), showed no aphasic deficit.
Writing with the right hand was normal. Neither writing nor drawing evidenced perseveration.
The neuropsychological
examination
(see Table 1, first session) excluded global cognitive deterioration.
There
were memory deficits that did not fit into any classical amnesia syndrome, involving both short-term and long-term
spatial and verbal memory. Autobiographical
memory was also impaired; G.C. did not produce, however,
confabulations.
The patient scored below the cut-offs in some tests (Weigl Sorting Test, Elithorn Perceptual Maze)
115, 531 that are traditionally
considered to be sensitive indices of frontal damage [48].

The alien hand
The audio-videotape
of the test sessions was evaluated by two independent
observers.
During some of the neuropsychological
tests which required manipulation
or utilization of
objects, a conflict between the two hands became evident, with each of them acting according
to separate and often competing programmes. The patient always felt that the right hand was
a disturbing element, even when it carried out an activity more adequately than the left. The
feeling that the movements of the right hand were disturbing appeared to arise from the fact
that the patient consciously willed (as was clear from her spoken descriptions) only what the
left hand was doing, while what the right hand was doing was always unexpected. The
activities of the right hand did not lessen when the arm was moved over the midline to the left
hemispace, as they did in patient B.D. described by GOLDBERG [23], suggesting that our case
did not have ‘hemispatial motor neglect’ [37].
There were many ‘callosal phenomena’; they are listed in Table 2. Whenever possible, we
used the terminology
of BOGEN [9].
(1) Aphasic errors when writing with the left hand in response to dictation
(aphasic
agraphia of the left hand) (Fig. 2). The patient herself was aware of these errors and was
surprised by them, but could not correct them with the left hand.
(2) Errors of naming objects placed in the left hand and manipulated
without visual
control (tactile anomia of the left hand): a pen was called a knife, a key was called a spoon, a
band was called a tennis racket, a fork a spoon, a ring a lid. The objects were manipulated
correctly and they were recognized, though not named, when later presented mixed with new
objects. Objects were named correctly when manipulated
by the right hand.
(3) Verbal orders executed by the left hand were defective (apraxia of the left hand) both
when G.C. was asked to mime the action without using the real object and when she was
asked to carry out gesture without significance or meaningful gestures (e.g. make the sign of
the cross, give a military salute, etc.).
(4) Relatively
simple arithmetic
calculations,
which could sometimes
be performed
successfully vocally, were frequently erroneous when written (spatial discalculia).
(5) A geometrical figure was copied correctly by both hands, but the proportions
and
dimensions
were closer to those of the original in the copy made with the left hand.
(6) Transfer of kinesthetic information from one arm to the other outside the visual control
(for example, arm extended with the forearm flexed and the closed fist placed on the
homolateral shoulder) was virtually impossible for both the left and right side (9 failures in 10
proposals, whether from right to left or left to right).
Follow-up
G.C’s AH stabilized over time, which constituted a chronic right AH. The patient herself
said that it appeared within 2 months after her recovery from postoperative
coma and we
observed it for the first time 1 year later. The sign had not changed at an examination
2 years
later.

1118

S. DELLA SALA, C. MARCHETTI and H. SPINNLER

TABLE 1. CC’s performance
in a formal psychometric
battery and healthy baselines. All scores have been
adjusted for age, education and sex. Range in brackets. The tests utilized are described in SPINNLER and
TOGNONI [47] except for the test for prose memory [S], the test of autobiographical
memory [lo] and the test
of the Towers of Hanoi [2]

G.C’s
adjusted
scores (1st
session)

G.C:s
adjusted
scores (2nd
session,
interval =
29 months)

Controls’
inferential
5th centile

Controls’
median score

30*

41.5

30

49

24

17.25

18.25

23.25

43.25
16.75

58.25
20.75

32
14.75

50
28.75

2*
0*
17.86
9.75
4.25

4*
4.75s
18.02
12.25
7.75

4.25
7.25
9
7
3.15

10.75
14.25
18
17
9.50

2.50*
3*
8*

3.5
5
40

2.75
3.M
36

4.50
4.75
111

5.25*

9.07

6.50

20

12
Il.50
3*
14*

7
16.50
6*

4.50
7.50
24
19

15.50
18.50
60
38

26.15
14

32.25
14

26.25
6.25

33
13.75

62
52*

62
56

52
52

69
69

21
23
7.50

26
19
8.5

17
20.75
2

26
31.25
7.25

Control functions
Attention
Selective attention:
Digit cancellation
test ((r60)
Inverted copying of gestures
Intelligence

and executive

(&24)

functions

Verbal judgement (t&60)
Raven’s progressive matrices set A, B, C,
D (w8)
Weigl’s sorting test (O-15)
Elithorn’s perceptual maze test
Towers of Hanoi test ((r18.80)
Fluency on semantic cue (&inf)
Free word association
(t%inf)
Instrumental functions
Memory
Verbal forward span (t%inf)
Spatial forward span (O-10)
Supra-span
verbal learning
Buschke-Fuld’s
selective reminding
technique (6180)
Supra-span
spatial learning (Corsi’s test)
(G29.16)
Serial position curve
Primacy (G70)
Recency (O-30)
Prose memory (&150)
Autobiographical
memory (&45)

Language
Token test (&36)
Sentence generation
nouns (O-18)

from two unrelated

Praxis
Ideomotor apraxia
Right hand (O-72)
Left hand (&72)
Visual perception
Length of segment discrimination
Scrawls discrimination
(t&32)
Street’s completion test (&14)
*Pathological

(O-32)

score, i.e. below the 5th centile of the normal

distribution.

RIGHT-SIDED

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TABLE 2. G.C.‘s performance

(ALIEN)

HAND:

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in tasks devised to show a callosal

Agraphia
Tactile anomia
Ideomotor apraxia in response to verbal
command:
Pretend object utilization
Intransive movements
Meaningful gestures
Constructional
apraxia
Spatial acalculia
Cross-replication
of arm and hand postures
out of visual control

1119

syndrome

Right hand
No

Left hand
Yes

IO/l0

S/l0

9110
9.5/10
lO/lO
14/14

o/10
7110
4.5110
14114
Yes

l/l0

l/10

The patient was not able to find strategies that would avoid or stop the conflict between the
two hands, which could be resolved only temporarily
by using force. Even the suggestion
[21,23] that she should ‘talk to the hand’ did not prove effective.
Three years after the subarachnoid
hemorrhage, all the signs of callosal disconnection
that
we have described were still present. The ideomotor apraxia on imitation [lS], of the left
hand, seen in the first examination,
had improved noticeably (Table 1). The cognitive profile
showed marked improvement
in memory function and partial improvement
in the so-called
frontal tests (Table 1). However, there was still everyday evidence of disturbances of ongoing
memory and her behaviour varied between inertia and facetiousness.

DISCUSSION
The anarchic hand
Our patient with chronic right-sided AH had an extensive lesion of the corpus callosum
(CC) and the fronto-mesial
cortex on both sides. It is reasonable
to think that the
supplementary
motor area (SMA) was more likely to be compromised
on the left, given the
larger mesial lesion on that side. The activities of the right hand were considered by the
patient to be unwanted,
and this feeling did not change with time. The autonomous
movements were correct from the praxic viewpoint, they were neither aimless nor ataxic, not
identifiable as dyskinesias. The patient disavowed the movements carried out by the right
hand even when they fit the requirements
of the tasks carried out. This behaviour cannot be
interpreted as the consequence of hemispheric cognitive incompetence
for some responses of
the right hand, as was considered to be the case for LORING et al.? case J.M. [32].
G.C. never considered that the AH that she could not control was not really hers; in fact, as
in most cases in the literature,
she always recognized the arm as hers. We agree with
GOLDBERG
[23] that the term ‘alien hand’ is less appropriate
than the term ‘wayward hand’
or, in view of the complete autonomy of the arm and its disruptive effect on the daily activities
of patients who have one, perhaps the best would be ‘anarchic hand’.
The interpretation
of AH is made complicated even by linguistic misunderstanding.
This
same term has been used to describe phenomena that have little in common. BOGEN[9], for
example, defined AH as a hand that ‘behaves in a way which the patient finds foreign or at
least uncooperative’
(p. 313) and, with almost imperceptible
shades of meaning, differentiates this from ‘intermanual
conflict’ (p. 312), defined as a situation in which one hand ‘is
acting at cross purposes to the other’, sometimes to the point of engaging in a real struggle

S. DELLA SALA, C. MARCHETTI and H. SPINNLER

FIG. 2. Some examples of words written by G.C. with the left hand. In addition to the reasonable
clumsiness due to lack of experience in writing with the nondominant
hand, there are many obvious
paragraphias.
It is noteworthy that all resulting responses are non-words. The patient correctly read
what she had wrongly written, but was not able to correct it graphically,
with the left hand.

with the other, and from ‘autocriticism’,
which consists of an ‘expression of astonishment’
(p. 313) by the patient who sees that one hand appears to be provided with its own
autonomous
volition and refers to it as it is not driven by his will: ‘the hand did such and
such’.
In reviewing previous reports, it turns out that the above-mentioned
terms are often used
interchangeably
for very different situations: spontaneous
and involuntary
grasping by one
hand of everything near it (with a need for the other hand to intervene to stop it from taking
up things that the subject does not want) [24, 353, intervention
of one hand to correct
activities that are or are considered to be wrong [32], the experience of the impossibility
of
exerting voluntary control of uncoordinated
movements by one hand in the case of optic

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1121

ataxia [29] or even the involuntary
movements of one hand during the course of corticobasal
degeneration
[20,40,41].
Usually these phenomena
are described within variegated picture of acute interhemispheric disconnection
symptoms and they are usually related to the ‘Main Etranghe’ Of ORION
and JEDYNAK [l l] or to the ‘Diagnostic Dyspraxia’ of AKELAITIS [l]. However, there are
obvious differences. The main ttrangdre described by BRION and JEDYNAK [ 1 l] consisted of a
lack of recognition by the subject of his or her left hand when asked to touch it with the right
hand without visual control. That is, the patients recognized one hand, but were not aware
that they were dealing with their own left hand, in a kind of hemisomatoagnosia
limited to
the hand and to the tactile modality only. No movements that were not voluntary and not
controlled
by the subject were included in this picture. The ‘Diagnostic
Dyspraxia’ of
AKELAITIS [l] consisted,
instead,
of a fleeting appearance,
during the short period
immediately
following a callosotomy,
of motor activity that contradicted
what the subject
wanted to do. This phenomenon
appeared in the patient as a strong conflict between the two
hands, with the left hand systematically undoing what the right hand had just finished doing.
Anatomo-functional

correlating

hypothesis

of AH

Table 3 summarizes the information
about many of the patients with AH described in the
literature. It also includes cases for whom it is not possible to speak of AH in the strict sense,
but who had one or more of the phenomena associated with it. There is an extensive Japanese
literature that has not been mentioned on the table; the reader is referred to a recent paper by
TANAKA et al. [49].
We considered it useful to include the largest amount of clinical information
for each
patient in this table. Attribution
of a given symptom to a patient was based on the description
of the authors rather than on what they named the symptom. When the authors did not
include enough information to make this possible (e.g. CHAN et al. [ 12]), we used the term the
authors had used. We used the above-mentioned
definitions
of BOGEN [9] for AH,
intermanual
conflict and autocriticism.
By ‘mirror movements’ we mean situations in which
both arms cannot carry out different and complementary
activities harmoniously,
but are
bound to mirror each other. The ‘callosal signs’ are those commonly
reported for the
syndrome of interhemispheric
disconnection
(see BOGEN, 1979 [9]). In detail, for the patients
considered in Table 3 they were: tactile anomia of the left hand [8, 11,24,26,28,36,49-513,
apraxia of the left hand [8, 12,24,26,36,51],
agraphia of the left hand [8,11,12,26,28,36,
511, right constructive apraxia [ll, 25,28,51], left relative pseudo-hemianopsia
[ 111, lack of
ability in cross-replicating
hand postures [ll, 12, 281, extinction
in dichotic hearing of
stimuli presented to the left ear [25], spatial discalculia
[28] and anomia for objects
presented in the left visual field [4]. The patient described by SHAHANI [45] is not set forth in
Table 3 inasmuch as he did not present with clear-cut alien behaviour. This is at odds with
the frequent report of this case [21] among the ‘alien’ references.
Analysing the data in Table 3, it is possible to formulate some localizing hypotheses from
the literature on AH, hypotheses already mentioned in the Introduction.
(1) The hypothesis that a circumscribed
lesion of the CC is sufficient is based essentially on
the fact that the earliest descriptions
of AH were made after surgical callosotomy
and the
phenomenon
was found along with signs of acute interhemispheric
disconnection
[ 1,9,54].
This hypothesis seemed to have been confirmed by some cases with vascular lesions [6,28,
511 which did not have cortical lesions that could be detected by neuroradiological
methods.
Cases with left AH fit within a theoretical explanation,
stemming from the apraxia domain,

1122
TABLE 3. Lay-out

SERGIO DELLA SALA, CLELIA MARCHETTI and

of findings

reported

in 35 patients

presenting

HANS SPINNLER

‘alien hand’

mnin twang&e,

and/or

intermanual

Lesion
AKELAITIS,
1944 [I]

I

BRIONdi JEUINAK,1972 [I I]
WlLsoNet ol., 1977 1541

I Callosal tumor
2 Callosal f”mOrS
1 Left pericallosal an&ma
1 Surgical division of the CC

BARBIZET
et al., 1978 [4]
BEUWELMAN
er al., 1980 [S]

I Clinical dwgnosis of Marchiafava Bignami dxase
I CT: diminished density between the anterior horns of

Go~ue~nc; et al., 1981 [Zl]
WATSON
& HEILMAN
1963 [51]
GOLOENBERO
er ol., 1985 [24]
WATSON
et al., 1986 [52]

LEVINE
k RINN,1986 1291

Surgical division of body and posterior half of genu of
the CC

the lateral ventricles
1 CT: left fronto-mesial cortex infarction
1 CT: left fronto-mesial cortex infarction
1 CT: hemorrhage extending from the genu to the
spenium of the CC. Coronal reconstructions showed
this to extend above the CC
1 Cl: infarction of the anterior two thirds of the CC. of
both cingulate gyri and the white matter underlvinh the
upper and mesial parts of frontal lobes
I CT: low densitv of the left mesial hemisohere and eenu
and anterior body of the CC
1 CT: low density in the distribution of the left anterior
cerebral artery not Involving the CC
1 CT: large right basal temporo-ocapital and small ventral postero-lateral thalamic infarcts

GOLDBERG,
1987 [23]

I CT, NMR: extensive infarction of the mesial surface of

GRAFT-RADFORD
or al., 1987 [26]

I NMR: hemorrhage in the genu and most of the body of

MAGNANIer al., 1987 [35]

I CT: reduced density in the mesial face of the left

+
+
R
R
R

+ (RI

R

+ (BIL) + (L)

+ (RI

+ (RI

+ CR)
+ CR)

(R)

+ (RI

+ CL)

+

+

+ (RI

R

the right frontal lobe and of the body of the CC

+ CL)

+

R

the CC: no evidence of infarction in the SMA

MCNABBet al., 1988 [36]

hemisphere, two thirds of the anterior portion of the
CC and part of the parietal lobe
1 CT: recent ischemic lesion of the white matter in the
left fronto-mesial region; a previous lesion in the left
caudate nucleus and cortical atrophy
1 CT: extensive infarction of the left suoerior. frontome&l and parietal cortex and of thegenu and body of
cc
1 No information

+ CR) + CR)
+ (BIL) + (BIL)
R

I
CHANB Ross, 1988 [I21

BANKSer of.. 1989 [3]

LEtcunnuA er al.. 1989 [28]

Lonlw

1 CT: ischemic infarctloo involving right anterior
cinaulate wrus and SMA. CC onlv minimallv involved
3 CT: infarction on the left SMA, anterior &g&e
gyrus and anterior CC
1 CT: infarction in the right SMA, anterior cingulate
gyros and anterior CC
1 CT: infarction m the riaht SMA, anterior cineulate
gyrus and anterior CC1 Autoosv:_ bilateral frontal damaee
_ side1
_ (z on the rieht
and callosal damage; additional damage on the right
basal ganglia and thalamus
1 CT: hemorrhage in the fronto-mesial lobes bilaterallv:
damage to thegenu and anterior portion of the CC.
1 CT: hemorrhage involvine the CC from the dorsorostra1 position of the g& to the most caudal part of
the body
1 CT. hemorrhage extendmg from the dorsal part of the
genu to the caudal third of the CC
1 CT: extensive hemorrhage from the rostra1 Dart of the
genu to the dorsal splenium of the CC
I Sureical section of the CC
NGR: division of the CC, no additional lesion
1 NMR: small ischemic stroke of the anterior two-thuds
of the CC of the cingulate gyrus and of the mesial
superior frontal gyrus of both hemispheres (SMAs
claimed 10 be spared)
I CT: bdateral frontal infarction involving the CC. On
the left side, lesion extending to SMA

+ CR) + CR)

Coordination
problems

+

Coordination
problems
Coordination
problems

+?

+ (RI

_

+

+ (L)

_

+

+ (L)

_

+ (R)

+ (RI

_

_

+ (L)

+

R
R

+ (RI

+

+ (BIL)

+

+ (R)

+ (L)

+
+
t (2

(RI

-

J : Sign present: - : sign absent; +?: questionable sign presence: (R): n&-sided; (L): left-sided; (BIL): bilaterally sided; A: ambidextrous: (R)*:
the right hand also occasronally showed ahen behawour.

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.wum

conflict and autocriticism. When information is missing, corresponding space is left blank

speech.

Left hemiparests and sensny changes: slurring of
Schizoid pattern on Rorschach test. Sometimes she would
set OK in bne directton but then she would find herself going
in the opposite direction

+

+

+ (L)
+ (L)
+ (L)

(R&g)
+ (U

+ (fleeting)

+
+

+ CR)
+ CR)
+ CL)

21 days: +
21 days. +
3 days: +

+ (L)

180 days: +

+ (RI

and cerebral CT normal

Activities of the left extremities were not improved
cueing
Right arm paresis, right leg paralysis

by verbal

35 days:

+

44 days: +

+

+
+
+

+

+

Bilateral leg paresis; double mcontinence

+

Increased reflexes of the right leg and right extensor plantar
response
Right leg weakness

+

40 days: +

+ (R)
+ (R)

90 days:

+

+ +&I
+ CL)
+

+ CR)

+ (R)

42 days: +

+ (RI

+?

+ CR)

+?

Bilateral
left

weakness of legs, the right being weaker than the

Ataxic left hand: wual control did not improve petfotmawe. Left hemianopia, left spatial neglect; left sensory loss.
The patient first thought of the left hand as naughty, then
conceived it as a baby
The pabent felt as d her left leg did not belong to her. Left
leg weakness and ‘inconsistent’ ability to move the left arm

+ 0-j

+ (R)

Angiography

Mutism for
4 weeks
+

Right leg weakness, right extensor plantar

response

+

Patient mutacic, as in a state ol catatonia with improvement
of vigilance, could repeat words and phrases

+ (Ll

+

Right arm paresis, right leg paralysis

+ (BIL)

Hesitation

Right leg weakness. In bimanual task, the two hands
appeared to act quite independently 01 each other
Right leg weakness
Left leg paralysis, left extensor plantar response. Tendency
to make fewer movements with the left hand

+ (L)
+ (L)

+

_
+ a,

+

13 years: -

+ (L)

+ &I CR)* 15 days: +

+

_

+ u-1

+

Abulic.

Left ahen foot. Bdateral plantar extensor response

1

Bilateral

leg weaknesss. left extensor plantar response

+

Left crural hyperreflexia,

left extensor plantar response

+

Left crural hyperreflexia.

left extensor plantar

+

Intermanual competition: the right hand corrected written
verbal errors made by the left hand, and vice versa
Left hand apraxia
Right hand dysgraphia
Extinction of the right ear on dichotic listening

+
+

+ (L)

+(Rl

3 months:

1 year: +

+ I months:

3 years: +

-

+

+

~~

to the site of the

+ u-1

+

+ (L)

Paresis more severe tn the leg controlateral
lesion

+

response

1124

S. DELLASALA,C. MARCHETTI
and H. SPINNLER

of a disconnection between the areas of programming on the left and those on the right for
carrying out movements with the left hand. However, this does not apply to right AH of
right-handed people.
All patients with right AH have been found to have lesions that extend beyond the CC. In
Table 3 there is only one partial exception to this: LEIGUARDA et al.% [28] patient No. 3 had a
right hand that occasionally behaved like the left AH.
(2) The hypothesis of a double, contemporaneous lesion of the CC and of the fronto-mesial
cortex of the hemisphere controlateral to the AH was proposed after the first description [21]
of two patients with right AH. This hypothesis states that AH is the result of an imbalance, in
the hemisphere controlateral to the affected hand [22], between activity of the SMA-that
directs intentional movements due to the inner context (so-called ‘predictive’ movements) [7,
18, 27]-and the activity of a lateral system, probably sited in the arcuate premotor area,
which organizes automatic movements in response to external stimuli (so-called ‘responsive’
movements) [39,42]. There are some clinical confirmations of this in Table 3 [21, 35, 521,
especially one anatomopathological finding [3] and one electrophysiological finding [36].
(3) There is a final hypothesis that seems to be a compromise between the two, put forward,
though incidentally, by LEIGUARDA et al. [28] (p. 1033). AH might occur because of an
isolated callosal interruption and in this case it would be seen in the arm ipsilateral to the
language and praxis dominant hemisphere, whereas the phenomenon would appear in the
arm controlateral to the lesioned hemisphere if the lesion was also cortical. This possibility,
however, appears to be contradicted by many cases and among them also by case No. 3 of the
same authors.
Except for our case G.C. who had marked stability of the right AH (follow-up of 3 years),
BANKS et al.3 [3] case No. 1 and TANAKA et d’s [49] no other case to our knowledge (see
Table 3) has been studied longitudinally: sometimes AH is described as fleeting and
sometimes as persistent. GOLDBERG et al. [21] suggested that the fleeting AH that appears
after surgical callosotomy [55] might be the result of cortical damage due to displacement
during the operation of hemispheric structures contiguous to the CC. A similar explanation
might be given for stroke patients with acute AH, hypothesizing passing functional damage
(compression due to oedema) of the frontal structures: this was confirmed by ‘cortical signs’
in the patients described above.
We propose the alternative hypothesis that only the callosal lesion is necessary for the
appearance of AH, but that to continue there must also be a fronto-mesial lesion encroaching
upon the SMA controlateral to the AH. The fronto-mesial lesion would be the basis of the
lack of compensation of the acute syndrome and cause the ‘chronic AH syndrome’.
Analogous mechanisms of compensation, possibly subcortical [34, 43, 441, have been
hypothesized for other signs of callosal disconnection that tend to be compensated to a
remarkable degree within a few months after operation [9]. However, no ideal case to
demonstrate this hypothesis has as yet been reported. Ideally such a case would be one with
two-step damage: appearance of and regression of AH after a callosal lesion; reappearance
and stabilization of AH after a later fronto-mesial lesion. This would be analogous to the
situation that helped to demonstrate the partial right hemispheric compensation of an
aphasia that appeared and partially regressed after a lesion of the left hemisphere and
worsened again after a later lesion in the right hemisphere [6,25, 381 or the existence of a
distributed network subserving the direction of spatial attention instead of a single parietal
locus [13].
Our case suggests that AH is a ‘frontal hand’, being characterized by a ‘frontal’ context-

RIGHT-SIDEDANARCHIC (ALIEN) HAND: A L~NGI~DINAL

STUDY

1125

driven disinhibition (impulsiveness) [46]. We are not dealing with two wills, that of the right
hemisphere and that of the left hemisphere, in which only one is accessible to the patient is
verbal report and the other acts as a ‘well-kept secret’, but with an erroneous control of
actions: in one hemisphere the internal/external balance being normal and the other
imbalanced because the internal is virtually absent.
In conclusion, the AH can be conceived as a fragment of the frontal syndrome,
demonstrating the possibility of separate lesions within a complex and extensive system of
functional representation; such a system is very likely to encompass the fronto-mesial areas.
Analysis of the cases in the literature and of our longitudinally studied case supports the
hypothesis of a fractionation of AH in an acute and a chronic form. It is only for the
appearance of chronic form that, in agreement with GOLDBERG [21]it is necessary to have a
double lesion, that is a lesion of the CC along with a lesion of the fronto-mesial cortical areas
(possibly of the SMA).
Acknowledgements-We
thank Mirella Crevacore for her editorial help, Gillian Jarvis who helped with the English
version of the paper, Anna Basso, Ph.D., who kindly addressed the patients to us, and two anonymous referees for
their comments. Research partly supported by grant to H.S. Nr. 87.00233.04.115.12234
of the Consiglio Nazionale
delle Ricerche.

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