ALIEN LIMB SYNDROME AND MOYA–MOYA DISEASE
23. Boroojerdi B, Kopylev L, Battaglia F, et al. Reproducibility of
intracortical inhibition and facilitation using the paired-pulse paradigm. Muscle Nerve 2000;23:1594 –1597.
24. Levy LM, Hallett M. Impaired brain GABA in focal dystonia. Ann
Neurol 2002;51:93–101.
25. Gilio F, Curra A, Lorenzano C, Modugno N, Manfredi M, Berardelli A. Effects of botulinum toxin type A on intracortical
inhibition in patients with dystonia. Ann Neurol 2000;48:20 –26.
26. Chen R, Wassermann EM, Canos M, Hallett M. Impaired inhibition in writer’s cramp during voluntary muscle activation. Neurology 1997;49:1054 –1059.
27. Orth M, Snijders AH, Trimble MR, Rothwell JC. Variability of
TMS paired pulse data within subjects and between subjectsmodification of stimulation parameters increases accuracy. Proceedings of Transcranial Magnetic Stimulation in Movement Disorders Conference; 2003 Mar 14 –15; Santa Margherita - Ligure,
Italy: Human Movement Disorder Society.

Alien Limb Syndrome and
Moya–Moya Disease
Omid Rabbani, MD,1 Lennon E. Bowen, MD,1
Robert T. Watson, MD,1 Edward Valenstein, MD,1
and Michael S. Okun, MD1,2*
1

Department of Neurology, McKnight Brain Institute,
University of Florida, Gainesville, Florida, USA;
2
Departments of Neurosurgery and Psychiatry, University of
Florida, Gainesville, Florida, USA

Abstract: We present the case of a 29-year-old woman
with an alien limb and callosal disconnection syndrome
who was diagnosed with moya–moya disease. We discuss
the potential mechanisms responsible for the alien limb
syndrome. © 2004 Movement Disorder Society
Key words: alien limb syndrome; callosal disconnection
syndrome; moya–moya disease; mechanism

Alien limb syndrome, which has been well described
previously,1–3 may result from various underlying pathological conditions. Symptoms may vary, but the hallmark
of the disorder is a limb that performs actions contrary to
the intention of the patient.4 In many cases, the normal

This article contains Supplementary Video Clips, available online at
http://www.interscience.wiley.com/jpages/0885-3185/suppmat.
*Correspondence to: Dr. Michael S. Okun, Movement Disorders
Center, University of Florida, Department of Neurology, McKnight
Brain Institute, P.O. Box 100236, Gainesville, FL 32610.
E-mail: okun@neurology.ufl.edu
Received 28 February 2003; Revised 26 January 2004; Accepted 1
March 2004
Published online 26 May 2004 in Wiley InterScience (www.
interscience.wiley.com). DOI: 10.1002/mds.20155

1317

limb attempts to restrain the alien limb. Alien limb
syndrome usually results from lesions of the corpus
callosum and/or lesions of the anteromedial cortex, including the supplementary motor area.5 Here, we report
another cause of corpus callosum pathological state resulting in alien limb: a disconnection syndrome resulting
from moya–moya disease.
CASE REPORT
A 29-year-old, right-handed woman presented initially
with problems speaking, performing complex calculations, and reading. She also complained of frequent involuntary movements of her left arm. She described
trying to remove her underwear with her right hand and
being thwarted by her left hand uncontrollably bringing
her underwear back up. During her hospitalization when
showering, her right hand would turn the water off while
her left hand would turn it back on. She could not exit the
bathroom because her right hand would open the door
and her left hand would close it. At initial presentation,
the patient also complained of left-sided pain and dysesthesias, as well as headaches and unsteadiness. Examination revealed a unilateral left-sided ideomotor apraxia
characterized by an inability to pantomime the use of
objects to command, which improved with imitation.
The patient showed further improvement with actual
object use, although remained slow when demonstrating
object use with her left hand compared to her right hand.
She had normal repetition but was anomic for objects
placed in her left hand. She demonstrated apparent lefthand agraphesthesia and astereognosis, both secondary
to left tactile anomia. The patient was able to correctly
identify an object placed in her left hand when asked to
choose this object from among a series of objects subsequently placed in the left hand. She had a left alien
limb syndrome (see Video, Segment 1). She also had
finger agnosia, acalculia, and right–left confusion. She
had mild motor impersistence but did not display a grasp
reflex or Myerson’s sign. Motor examination and deep
tendon reflexes were normal, but she had an extensor
plantar reflex on the left. Cerebellar function and gait
were normal.
Lumbar puncture studies and visual evoked potentials
were unrevealing. Angiography revealed bilateral highgrade stenosis of the distal internal carotid arteries and
collateralization of the lenticulostriate vessels through
the posterior communicating arteries and meningeal
branches. These findings were consistent with adult-type
moya–moya disease (Fig. 1A,B). Figure 1C and D show
axial and coronal views demonstrating an enhancing
lesion in the anterior mid-body of the corpus callosum
(also affecting the septal area and head of caudate) and

Movement Disorders, Vol. 19, No. 11, 2004

1318

O. RABBANI ET AL.

FIG. 1. A and B depict the typical “puff of
smoke” appearance (arrows) of moya–moya
disease (left carotid injection, lateral view).
An axial (C) and coronal (D) magnetic resonance image depicting the corpus callosum
infarction (arrows) at two sites: anterior midbody comprising the septal area and head of
caudate (coronal section) and splenium (axial
section). The rostrum and posterior mid-body
were also affected (not shown).

the splenium of the corpus callosum, more on the left. In
addition, the rostrum and posterior mid-body of the corpus callosum were also affected (not shown).
The patient was examined again at approximately 6
months after her initial presentation. She did not exhibit
any interval change upon neurological examination and
continued to demonstrate signs of callosal disconnection
along with alien limb features.
DISCUSSION
The unilateral tactile anomia and ideomotor apraxia
displayed by our patient, along with dissociative phenomena such as intermanual conflict and alien limb, are
consistent with a callosal disconnection syndrome.6
Brion and Jedynak believed the alien hand sign7 was a
clinical sign of corpus callosum disconnection. They
described a sensory disconnection phenomenon, in
which the patient did not recognize his own hand when
both palms were placed together out of view, either
behind the back, or in front of the patient with closed
eyes.7 The scope of alien limb syndrome has broadened
to include “involuntary” movements that the patients

Movement Disorders, Vol. 19, No. 11, 2004

consider alien to their intent.5 Thought to be due to a
disruption of the transcallosal fibers and associated supplementary motor areas,1 the alien hand syndrome was
further classified into two types by Feinberg and colleagues.2 The first, the frontal alien hand syndrome, may
result from damage to the supplementary motor area,
anterior cingulate gyrus, medial prefrontal cortex of the
dominant hemisphere, and anterior corpus callosum. The
symptoms of frontal alien hand syndrome consist of
reflexive grasping and groping in the dominant hand. The
second type of alien limb requires only a solitary callosal
lesion. These cases are characterized by intermanual
conflict and apraxia of the nondominant hand. Recently,
a posterior alien hand syndrome has been proposed,
which spares the corpus callosum and occurs with parietal8,9 or thalamic10 infarcts. In this syndrome, the acting
hemisphere is disconnected from sensory input, resulting
in purposeless, involuntary behavior such as arm levitation or writhing fingers.11 Additionally, variant cases of
alien limb syndrome have been found in patients with
partial seizures,12 Creutzfeldt–Jakob disease,13–15 corti-

ALIEN LIMB SYNDROME AND MOYA–MOYA DISEASE
cobasal ganglionic degeneration,16 –18 Alzheimer’s disease,19 corpus callosum infarction,1,20,21 corpus callosectomy,6 bifrontal penetrating cerebral injury,3 anterior
cerebral artery territory infarction,22–27 anterior communicating artery rupture,3,28 –30 parietal lobe lesions,8,9 posterior cerebral artery territory infarction,31,32 capsulothalamic hemorrhage,33 thalamic infarction,10 and seizure.34
We report on a case of alien limb resulting from
vascular damage secondary to moya–moya disease. Our
patient was found to have an ischemic event in the
setting of moya–moya disease, which resulted in a callosal disconnection syndrome. Moya–moya disease is a
cerebral vasculopathy of unknown etiology35 that is characterized by progressive occlusion of the proximal portion of the carotid arteries, and the development of compensatory collateral vessels. The name moya–moya
means a “puff of smoke” and has been used to describe
the angiographic appearance of this extensive collateral
network of vessels. Although initially thought of as a
disease of childhood, moya–moya is well known to occur
in adults.36,37 The neurological deficits typically result
from ischemia produced by arterial occlusion or hemorrhage. To our knowledge, this is the first report of moya–
moya disease and alien limb. The patient had nondominant arm involvement and no motor deficits or reflexive
grasping. She demonstrated alien limb symptoms similar
to the patient of Akelaitis who had undergone callosotomy for intractable epileptic seizures.38 In Akelaitis’
report, the patient would attempt to put on clothes with
the right hand while pulling them off with the left hand.
Similarly, the patient would open a door with the right
hand and simultaneously push it shut with the left hand.
In another report,39 a patient with callosotomy is described pulling down clothes with one hand and pulling
them up with the other, as evidenced in our patient.
Akelaitis termed this behavior “diagonistic dyspraxia” to
describe the conflict between the intended act and the act
actually performed.38 Another patient we observed with
a callosal infarction also demonstrated problems with the
left hand pulling a blouse off and the right hand putting
the blouse back on.20 In these cases and the one reported
here, the patients were aware of the alien limb behavior
and would express frustration over its interference with
routine activities.
The finding of an “alien limb,” as in our patient
should direct the attention of the treating physician not
toward a specific diagnosis, but rather toward a localization of the problem which can be caused by a
multitude of pathological conditions. It is not surprising, therefore, that moya–moya disease can result in
this phenomenon.

1319

LEGEND TO THE VIDEO
A disconnection syndrome and apraxia is demonstrated.
Segment 1. The patient explains how one hand tries to
restrain the other when performing actions.
Segment 2. The segment demonstrates left ideomotor
apraxia.
Segment 3. The segment demonstrates the patient’s difficulty performing tasks with her left hand.
Segment 4. This segment demonstrates the apparent lefthand agraphesthesia and astereognosis, both secondary to left
tactile anomia. The patient was able to correctly identify an
object placed in her left hand when asked to choose this object
from among a series of objects subsequently placed in the left
hand. This finding demonstrates that her right hemisphere is
intact and that her apparent agraphesthesia and astereognosis
are secondary to the callosal disconnection, which prevents
input that is correctly perceived by the right hemisphere from
reaching the language region of the left hemisphere (the complete sequence showing intact stereognosis and the ability of
the patient to identify objects from multiple choice is discussed
in the text but not shown in the video).

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Movement Disorders, Vol. 19, No. 11, 2004

Abnormalities of Dopaminergic
Neurotransmission in SCA2:
A Combined 123I-␤CIT and
123
I-IBZM SPECT Study
Sylvia M. Boesch, MD,1* Eveline Donnemiller, MD,2
Jörg Müller, MD,1 Klaus Seppi, MD,1
Helga Weirich-Schwaiger, MD,3 Werner Poewe, MD,1
and Gregor K. Wenning, MD, PhD1
1

Department of Neurology, University of Innsbruck, Austria;
Department of Nuclear Medicine, University of Innsbruck,
Austria; 3Department of Biology and Human Genetics,
University of Innsbruck, Austria

2

Abstract: Extrapyramidal features may occur in spinocerebellar ataxias consistent with neuropathological evidence of nigrostriatal involvement. Recently, striatal dopaminergic neurotransmission was found to be abnormal in the uncommon
parkinsonian presentation of spinocerebellar ataxia type 2
(SCA2). We have investigated, therefore, striatal dopamine
transporter and D2 receptor function in a series of 9 patients
with the more common ataxic presentation of SCA2 using
single photon emission computed tomography and ␤-CIT as
well as IBZM. Age-matched healthy subjects and patients
with Parkinson’s disease (PD) served as controls. All except 1
SCA2 patient exhibited slowness of limb movements without
rigidity or rest tremor. In addition, cervical dystonia was
present in 5 and dystonic head tremor in 2 SCA2 patients.
Striatocerebellar (S/C) ratios of ␤-CIT binding were significantly reduced in SCA2 patients compared to control subjects,
and they were within the range of PD patients. S/C ratios of
IBZM binding were significantly reduced in SCA2 patients
compared to control subjects. We conclude that dopaminergic
neurotransmission is impaired in the ataxic presentation of
SCA2, with a prominent loss of striatal dopamine transporter
function. Both slowness of limb movements as well as dystonia
in the ataxic SCA2 phenotype may reflect dysfunction not only
at cerebellar but also at basal ganglia level. © 2004 Movement
Disorder Society
Key words: SCA2; dopamine; SPECT; ADCA

Spinocerebellar ataxia type 2 (SCA2) is an autosomal
dominant cerebellar ataxia that is progressive and typically associated with slow saccades. The clinical phenotype of SCA2 is caused by an increased number of CAG
repeats in the 5⬘ coding region on chromosome 12.1–3
Intermediate and/or interrupted CAG repeat expansions

*Correspondence to: Dr. Sylvia M. Boesch, Department of Neurology, University of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria. E-mail: sylvia.boesch@uibk.ac.at
Received 10 October 2003; Revised 25 February 2004; Accepted 2
March 2004
Published online 26 May 2004 in Wiley InterScience (www.
interscience.wiley.com). DOI: 10.1002/mds.20159