Movement Disorders
Vol. 21, No. 7, 2006, pp. 944 –949
© 2006 Movement Disorder Society

Atypical Movement Disorders in Antiphospholipid Syndrome
Davide Martino, MD,1,2 Nee-Kong Chew, MBBS,1,3 Pablo Mir, MD,1,4 Mark J. Edwards, MBBS,1
Niall P. Quinn, MD,1 and Kailash P. Bhatia, MD1*
1

Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom
2
Department of Neurological and Psychiatric Sciences, University of Bari, Bari, Italy
3
Division of Neurology, Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia
4
Servicio de Neurologı́a, Hospital Universitario Virgen del Rocı́o, Seville, Spain

Abstract: Movement disorders have only rarely been reported
in association with antiphospholipid syndrome (APS). In such
cases, chorea is the most common disorder observed, with
occasional reports of hemidystonia, Parkinsonism, and hemiballism. We report here on 3 cases of APS (3 women ages 16,
46, and 56 years) who presented with movement disorders,
including tics, tremor, myoclonus, and a corticobasal syndrome, never or rarely reported in association with this disease.
Mild executive dysfunction was observed in all 3 patients. We

also report the successful treatment of two of these patients
with mild oral anticoagulation (INR 2–3). Movement disorders
in APS seem more clinically heterogeneous than previously
thought. Oral anticoagulation should be considered in the treatment of movement disorders associated with APS. © 2006
Movement Disorder Society
Key words: antiphospholipid syndrome; tics; tremor; corticobasal syndrome; oral anticoagulation

The antiphospholipid antibody syndrome (APS) is defined as a hypercoagulable state characterized by recurrent venous and/or arterial thrombosis and/or pregnancy
complications in the presence of elevated titers of antiphospholipid antibodies.1 It may exist as an independent condition (primary APS) or in conjunction with a
variety of autoimmune, infectious, and neoplastic diseases (secondary APS), among which systemic lupus
erythematosus (SLE) is the most frequently reported.2
Since its original description,3 APS has manifested with
a remarkably wide array of neurological and psychiatric

features, including cerebrovascular disease, epilepsy,
headache, and movement disorders, usually chorea.4
The pathophysiology of APS-related central nervous
system involvement is still incompletely defined, although the main target of antiphospholipid antibodies
has been identified in a phospholipid-binding protein
named ␤2-glycoprotein I, involved in the priming of
vascular endothelial cells to thrombosis and leukocyte
adhesion.5 Although it has been speculated that a direct
binding of antiphospholipid antibodies might affect neuronal or glial function,6 most of the manifestations of
APS are believed to be thrombotic in origin.
Chorea has been the most frequently described movement disorder in this condition, reported in 1% to 4% of
patients with primary or secondary, mainly SLE-related,
APS.7,8 Nevertheless, current evidence suggests that the
full clinical spectrum of basal ganglia dysfunction in this
syndrome might be broader than previously thought, to
include dystonia, ballism, Parkinsonism,9,10 and paroxysmal dyskinesias.11 Movement disorders other than

This article includes Supplementary Video, available online at http://
www.interscience.wiley.com/jpages/0885-3185/suppmat
*Correspondence to: Dr. Kailash P. Bhatia, Sobell Department of
Motor Neuroscience and Movement Disorders, Institute of Neurology,
Queen Square, London, WC1N 3BG, United Kingdom.
E-mail: k.bhatia@ion.ucl.ac.uk
Received 27 May 2005; Revised 27 August 2005; Accepted 4
October 2005
Published online 14 March 2006 in Wiley InterScience (www.
interscience.wiley.com). DOI: 10.1002/mds.20842

944

ATYPICAL MOVEMENT DISORDERS

945

chorea, however, have so far been reported only in a
small proportion of patients with APS, and their true
prevalence is uncertain.
In this article, we further extend the clinical spectrum
of APS-related movement disorders, reporting three patients with APS who presented with movement disorders,
including tics, tremor, myoclonus, and a corticobasal
degeneration (CBD)-like syndrome, never or only rarely
described in association with this condition. We also
report the successful treatment of two of these patients
with oral anticoagulation.
PATIENTS AND METHODS
Case 1
This young woman was admitted at the age of 16 with
a 2-month history of involuntary orofacial movements of
acute onset, associated with speech difficulties. A few
months prior to admission, her mood became low and
she appeared socially withdrawn. Following the onset of
her involuntary movements, she received a presumptive
diagnosis of Tourette’s syndrome by another neurologist,
and her movements had only mildly improved during a
short course of haloperidol, given at small doses (1 mg
bid).
There was no family history of medical, neurological,
or psychiatric disorders, including Tourette’s syndrome
and obsessive– compulsive disorder. There was no personal history of head trauma, infection, or pharmacological treatments prior to symptom onset. Her past medical
history was unremarkable; particularly, there was no
report of previous vascular problems, including peripheral thrombosis. On admission, she exhibited orofacial
involuntary movements, characterized by tongue protrusion, teeth clenching, grimacing, blinking, and raising of
the eyebrows, which did not decrease with distraction
maneuvers, but were partially suppressible under her
volition (see Video, Segment 1). Bite marks were noticeable on the lips and oral mucosae. Intermittent gasping
and coughing sounds, similar to phonic tics, were recorded. Her speech was slurred, but still intelligible, and
verbal fluency was severely impaired. Her general and
neurological examination was otherwise unremarkable.
She scored 28/30 on the Mini Mental State Examination. Neuropsychological assessment demonstrated impaired performance on tests sensitive to frontal executive
dysfunction (Trail Making Test, Wisconsin Card Sorting
Test) and poor performance on verbal fluency tasks.
Asperger’s syndrome, autism, or other pervasive developmental disorders were excluded by history and psychological assessment. Affect seemed bizarre and

FIG. 1. The MRI scan of Case 1 showed a few hyperintense areas on
T2-weighted images, localized in the white matter of the centrum
semiovale of both hemispheres and of the right occipital lobe. The
lesions did not enhance following Gd-DTPA IV infusion and were
interpreted as abnormal, given the young age of the patient.

changeable, and she exhibited a range of expressions
from smiling to crying to perplexity.
Conventional MRI of the brain revealed a few white
matter T2-weighted hyperintense lesions at the centrum
semiovale bilaterally and in the right occipital lobe,
without Gd-DTPA enhancement (Fig. 1). Diffusion- and
perfusion-weighted MRI studies were not performed.
Routine blood chemistry was normal; there was mild
thrombocytopenia (92,000/␮L). A peripheral blood film
for acanthocyte search (repeated three times during 4
months), serum urate, anti–streptolysin O titer (ASOT),
white cell/plasma enzymes, long-chain fatty acids, serum
copper/ceruloplasmin, creatine kinase, autoimmune
screen (including a full autoantibodies screening, comprehensive of antinuclear antibodies), and genetic analysis for Huntington’s disease (HD), and dentatorubropallidoluysian
atrophy
(DRPLA)
were
normal.
Cerebrospinal fluid examination, including anti-Borrelia
antibodies, was normal; oligoclonal bands were absent.
The only laboratory abnormalities were a prolonged activated partial thromboplastin time (aPTT), a moderately
raised anticardiolipin (aCL) IgG antibody titer (50.1 gpl/
mL; normal, 0 –5; weak positive, 5–15; moderate positive, 15– 80; strong positive, ⬎ 80) and a positive diluted
Russel Viper Venom test (DRVVT) for lupus anticoagulant (LA). Anticardiolipin IgM titer was normal. A
provisional diagnosis of possible primary APS was formulated. She was initially treated with aspirin 100 mg/
day, which was ineffective, and she was readmitted 5
months later. On this occasion, the MRI findings were
unchanged, and there was no EEG correlate to her facial

Movement Disorders, Vol. 21, No. 7, 2006

946

D. MARTINO ET AL.

dyskinesias on EEG telemetry. Given the persistence of
raised aCL IgG antibody titers (63.3 gpl/mL), the diagnosis of primary APS was confirmed, and oral anticoagulants (warfarin, target INR 2.5–3) were administered.
Four months later, the movements had completely resolved and her speech and cognitive state were significantly ameliorated. In the following months, initial withdrawal of warfarin caused a recurrence of her facial
movements; after its prompt reintroduction, a marked
and stable improvement of her involuntary movements
was observed.
Case 2
This 46-year-old woman was admitted with a 2-year
history of complex involuntary movements involving
face and limbs. During the same period, she developed a
blistering rash of the axillary and neck regions, a petechial rash of the upper limbs, and oronasal mucosal
ulcers, which all fluctuated in severity. She also suffered
from migraine and arterial hypertension; there was no
history of peripheral thrombosis. The patient had never
been pregnant.
On admission, she exhibited a complex movement
disorder, characterized by a 10-Hz bilateral arm tremor,
more severe on the right, strongly related to posture and
partially distractible; superimposed myoclonic jerks of
all four limbs, predominant on the right side, and frequently associated with twitching of the right corner of
the mouth; tremulous dyskinesias on protrusion of the
tongue. The neurological examination was otherwise unremarkable. General examination was normal, apart from
the rash; there was no goiter, lymph node enlargement,
or joint deformity and tenderness. Her neuropsychological profile was characterized by good functioning in the
high average range on the verbal and performance scales
of the Wechsler Adult Intelligence Scale Revised
(WAIS-R), mild to moderate inefficiency on tests sensitive to executive dysfunction (Trail Making Test and
Stroop Colour Word Task), and evidence for slowed
speed of cognition (Symbol Digit Modalities Test); these
findings were suggestive of anterior and subcortical region involvement. Frequency domain EMG analysis
gave strong evidence for an organic type of movement
disorder due to the detection of short EMG bursts (50 –70
milliseconds) of 8 to 12 Hz frequency, occurring in the
absence of corticomuscular coherence (EEG–EMG).
Motor and sensory nerve conduction studies and central
motor conduction times were normal, as was a standard
EEG. MRI of the brain showed several hyperintense
areas on T2-weighted images in the subcortical white
matter bilaterally (mainly frontal lobes) and small bilateral cerebellar infarcts. Routine blood chemistry and

Movement Disorders, Vol. 21, No. 7, 2006

blood counts were normal. Antinuclear antibody titer
was positive on repeated determinations between 1/40
and 1/160 (homogenous pattern); aCL antibodies were
moderately raised (26.6 gpl/mL), and DRVVT for lupus
anticoagulant was also positive. The rest of the autoimmune screen (comprehensive of full autoantibody
screening) and complement factor determinations were
normal. Prothrombin time (PT) was slightly prolonged.
Search for acanthocytes was negative. CSF studies were
normal, including screening for syphilis and neuroborreliosis. Genetic testing for HD, DRPLA, and prion protein
gene mutations was negative. To exclude the possibility
of lupus vasculitis, a cerebral biopsy was proposed,
which the patient refused.
Her involuntary movements responded partially to
small doses (0.5 mg bid) of clonazepam. During the
following months, she developed multiple nonerosive
arthritides, accompanied by exacerbation of the mucosal
ulcers. Moreover, she developed several generalized
tonic– clonic seizures within a period of 2 months. At
that stage, she fulfilled the diagnostic criteria for SLE
and, given the persistently elevated aCL titers, she received a diagnosis of SLE-related APS. Eight months
after first admission, she was started on warfarin (INR
2–3). Six months later, at her last follow-up visit, her
seizures and complex movement disorder had significantly improved. A follow-up MRI study was refused by
the patient.
Case 3
This 56-year-old woman presented with a 6-year history of progressive memory problems, difficulties in
using her right arm, particularly in fine tasks (e.g., doing
up buttons, using cutlery), and depression. She noticed
that her right arm was involuntarily kept in an abnormal
posture and tended to move about while she walked, as if
it had “a life of its own.” She suffered from longstanding hypertension and migraine. No difficulties during pregnancy were reported.
On examination, she showed poor verbal fluency, but
comprehension was only mildly impaired. She exhibited
ideomotor and limb– kinetic apraxia bilaterally, manifesting mainly with spatial-type errors (body-part-asobject errors on pantomime tasks and difficulty in imitating finger postures; see Video, Segment 2); her right
arm was the most affected, having become functionally
useless. She exhibited also dyscalculia and difficulty in
distinguishing left from right. She walked with short
steps, stooped posture, and a flexed dystonic posture of
the right arm. On the pull test, she tended to fall backward, with retropulsion. She was hypomimic and slow
on alternate repetitive movements, her right side being

ATYPICAL MOVEMENT DISORDERS

947

dopa, up to 600 mg/day, without benefit. She was discharged with a diagnosis of CBD-like syndrome associated with primary APS, and aspirin and warfarin were
separately tried without significant benefit. After 8
months of progressive deterioration, she developed a
new stroke, related to a new infarct in the right internal
capsule. Repeated aCL IgG was still raised (23.6 gpl/
mL). She became bedridden and died from pneumonia 2
years later. A postmortem study was not obtained.

FIG. 2. The MRI scan (T2-weighted images) of Case 3 revealed
several cortical and subcortical infarcts in both hemispheres, extensive
periventricular white matter changes, and a marked diffuse cerebral
cortico–subcortical atrophy. Infarcts were located in multiple cerebral
vascular territories. Lacunar infarcts were observed also in the striatum
bilaterally, particularly in the right putamen.

more affected. No tremor or myoclonus was observed.
She had mild limitation of upgaze and impersistence on
horizontal gaze; saccades were hypometric, particularly
in the vertical axis. Perimetry revealed a right homonymous superior quadrantanopia. General examination revealed livedo reticularis of torso and limbs.
Her neuropsychological profile showed a significant
degree of intellectual deterioration, mainly on tests with
a nonverbal component, and evidence of severe underfunctioning of the nondominant hemisphere. Brain MRI
showed extensive periventricular white matter changes,
marked diffuse cerebral cortico–subcortical atrophy, and
several infarcts in both hemispheres, involving multiple
vascular territories, including the striatum bilaterally
(Fig. 2). MR angiography, carotid doppler, and transthoracic and transesophageal echocardiography were all
normal. Extensive blood and CSF investigations were all
negative (including serology for syphilis and full autoantibody screening), except for moderately raised levels
of aCL IgG (30.9 gpl/mL), a positive DRVVT for lupus
anticoagulant, and a slightly prolonged aPTT (39.6 seconds). A skin biopsy did not suggest systemic vasculitis
or cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy (CADASIL).
A frontal lobe biopsy revealed nonspecific severe gliosis
of cortex, white matter and deep gray nuclei, without
evidence of vasculitis. Immunohistochemical stains for
amyloid and tangles (A4 and Tau) were negative.
Before biopsy, she had a 2-month trial of steroids, in
the assumption of cerebral vasculitis, and a trial of levo-

DISCUSSION
The three patients presented here developed a complex
movement disorder associated with persistently elevated
serum levels of aCL antibodies and positive LA, which
were the only abnormal finding after extensive diagnostic work-up. Patient 2 had APS secondary to SLE, while
the others fulfilled established criteria for possible primary APS.12 Two of the three patients presented with a
complex hyperkinetic syndrome, in which tics and an
unusual form of tremor were the most striking features.
We interpreted the involuntary movements of Patient
1 as motor and phonic tics, similar to those in Gilles de
la Tourette’s syndrome (GTS) patients, being repetitive,
rapid, and stereotyped in their quality and localization.
However, their transient duration, rapid progression in
severity, and dramatic response to anticoagulants make
the diagnosis of GTS in this patient unlikely. We excluded the vast majority of the reported causes for secondary tourettism. A subacute onset of tics has been
observed following streptococcal infections.13 Our patient’s ASOT was in the normal range, and she did not
report a history of pharyngotonsillitis. The striking response to oral anticoagulation and the persistently elevated aCL titer suggest a causal link between APS and
the movement disorder in this patient. To the best of our
knowledge, this is the first case ever described of tics
occurring as a manifestation of APS.
Patient 2 presented with a complex disorder, characterized by postural tremor of all four limbs, with superimposed lightning-like myoclonic jerks of higher amplitude. A possible subcortical origin of the involuntary
movements was suggested by electrophysiological testing. Brain imaging revealed several white matter and
cerebellar lesions, compatible either with an inflammatory process or with a small vessel disease. Although
conventional MRI did not allow a definitive diagnostic
discrimination of the true pathology of these lesions, we
thought that the lack of inflammatory findings in the
CSF, together with the final diagnosis of SLE associated
with APS, was more in favor of a vascular origin. Moreover, the surprisingly good response to oral anticoagulants further suggests that an underlying vasculopathy,

Movement Disorders, Vol. 21, No. 7, 2006

948

D. MARTINO ET AL.

involving the cerebellum and/or the frontal white matter,
and likely related to the presence of antiphospholipid
antibodies, played a major role in the causation of her
symptoms. Interestingly, the occurrence of a generalized
slow-frequency tremor, related to posture and associated
with T2-weighted diffuse subcortical hyperintensities on
brain MRI, has been recently reported in a middle-aged
woman who received a diagnosis of Sneddon’s syndrome, a condition characterized by livedo reticularis
and cerebrovascular disease, and often presenting with a
raised titer of antiphospholipid antibodies.14
Patient 3 is the second case ever described of corticobasal syndrome (CBS) in a patient with APS and multiple cerebral infarcts.15 Her picture resembled corticobasal degeneration (CBD), a tauopathy characterized by
asymmetric cortical and basal ganglionic destruction.
CBD typically manifests with a CBS, which classically
comprises levodopa-resistant Parkinsonism, dystonia,
myoclonus, apraxia, cortical sensory loss, and the alien
limb sign. However, not all cardinal signs of CBD were
present here: cortical sensory loss and cortical myoclonus were absent. On examination, her right arm had
become functionally useless, and therefore we could not
observe clear involuntary alien limb activity, although a
subjective feeling of a foreign right arm and autonomous
motor activity were present in her history. Finally, the
time course and the manifestation of a clear visual field
deficit raised the suspicion that her CBS had a different
etiology from CBD. Many CBD look-alike syndromes
have been reported.16 The majority of these alternative
causes (Progressive Supranuclear Palsy,16 Pick’s disease,16 Creutzfeld–Jakob disease,17 sudanophilic leukodystrophy,16 meningovascular syphilis18) were ruled out
by a thorough diagnostic work-up, including a frontal
lobe biopsy, which did not reveal tau deposits. This case
indicates that APS should be excluded in patients presenting with a CBS associated with multiple cerebrovascular lesions.
Our patients showed mild executive dysfunction, suggestive of subcortical region involvement. Cognitive impairment is a very common CNS manifestation of APS.19
It is likely that in our patients both movement disorders
and executive dysfunction were related to frontosubcortical abnormalities.
We cannot exclude the possibility that the co-occurrence of movement disorders and elevated levels of aCL
antibodies was due to chance; indeed, abnormal aCL IgG
and IgM titers have been reported in approximately 4%
to 9% of different populations of healthy donors, although their true prevalence in the general population
remains uncertain.20,21 However, we believe a coincidental finding of elevated aCL titers to be unlikely in our

Movement Disorders, Vol. 21, No. 7, 2006

three patients, due to the observed response to anticoagulants and the exclusion of other differential diagnoses. Anticardiolipin IgM may also rise after viral infections.22 All our patients had repeatedly positive aCL
IgG antibody, whereas two had undetectable aCL IgM
levels, suggesting that abnormal aCL titers were unlikely
to be secondary to an infectious process.
Cerebral ischemia due to microthrombosis seems the
main cause of CNS disease in neuropsychiatric lupus and
APS.1,23 In two patients, we could not detect infarcts in
the basal ganglia or thalamus. Patients with APS-related
chorea often do not show cerebral infarcts7 or manifest
only diffuse white matter changes on brain imaging.24 In
Patients 1 and 2, the hyperkinesias might have been
caused by altered connectivity between the basal ganglia
and the cortex, secondary to lobar white matter and/or
cerebellar lesions. Alternatively, small thrombotic lesions of the basal ganglia might have been overlooked on
brain MRI due to the long period between the movement
disorder onset and the neuroradiological investigation.
Oral anticoagulation is considered the first-choice
treatment in patients with APS suffering from cerebral
ischemia, and discontinuation of warfarin can lead to
major recurrent thrombosis.1 Although potentially
useful, the use of steroids and immunosuppressive
drugs in treating these patients remains empirical.1
Furthermore, it has been recently stated that, in patients with cognitive dysfunction related to APS, immunosuppression should be regarded as a second-line
treatment, to be introduced following failure of oral
anticoagulants.19 Given the absence of reference
guidelines for the use of immunosuppressants in APS,
steroids were not administered in our patients, apart
from Case 3, in whom they were used only before
biopsy excluded a vasculitic process. However, in two
of our patients, who displayed multiple vascular lesions on brain MRI, their movement disorder significantly improved after a brief period of anticoagulation, and no other treatment was required to achieve
good control of their symptoms. Several reports indicate that patients with APS may significantly improve
after anticoagulation, both clinically and radiologically.25–28 In an interesting analogy to our first two patients, a recent case report described a 29-year-old
woman with a paroxysmal nonkinesigenic dyskinesia,
occurring as the first manifestation of primary APS,
who significantly improved after the introduction of
acenocoumarol (INR 2–3).11
In conclusion, the movement disorders in APS seem
more heterogeneous than previously thought. APS as the
underlying cause of motor disturbances should be suspected in the presence of systemic manifestations of APS

ATYPICAL MOVEMENT DISORDERS
such as livedo reticularis, skin rash, migraine, and MRI
brain abnormalities (white matter lesions, unexplained
infarcts). Subjects with APS-associated movement disorders can have subclinical or clinically mild cognitive
impairment, consistent with subcortical damage. Importantly, in some of these patients, mild oral anticoagulation may have a role in improving the clinical picture,
sometimes dramatically.
LEGENDS TO THE VIDEO
Segment 1. The patient exhibits mixed involuntary
movements of the face, represented by raising of the
eyebrows, tongue protrusion and twisting, mouth opening, eye rolling, smiling, and blinking. She also produces
coughing, sniffing, and gasping actions, which strongly
resemble phonic tics. All involuntary movements are
partially suppressible by volition.
Segment 2. On gait examination, the patient exhibits
an outward flexed dystonic posture of the right upper
limb. Retropulsion is observed on the pull test. She
manifests dyspraxic spatial-type errors, characterized by
difficulty in imitating finger postures and body-part-asobject errors on pantomime tasks. Errors are present
bilaterally, although more severe on the right side. Intransitive actions are performed normally. There is bradykinesia of both upper limbs on alternate repetitive
movements.
REFERENCES
1. Sanna G, Bertolaccini ML, Cuadrado MJ, Khamashta MA, Hughes
GRV. Central nervous system involvement in the antiphospholipid
(Hughes) syndrome. Rheumatology 2003;42:200 –213.
2. Grossman JM. Primary versus secondary antiphospholipid syndrome: is this lupus or not? Curr Rheumatol Rep 2004;6:445– 450.
3. Hughes GRV. Thrombosis, abortion, cerebral disease, and the
lupus anticoagulant. Br Med J 1983;287:1088 –1089.
4. Sanna G, Bertolaccini ML, Cuadrado MJ, et al. Neuropsychiatric
manifestations in systemic lupus erythematosus: prevalence and
association with antiphospholipid antibodies. J Rheumatol 2003;
30:985–992.
5. Del Papa N, Guidali L, Sala A, et al. Endothelial cells as target for
antiphospholipid antibodies: human polyclonal and monoclonal
anti– beta 2-glycoprotein I antibodies react in vitro with endothelial
cells through adherent beta 2-glycoprotein I and induce endothelial
activation. Arthritis Rheum 1997;40:551–561.
6. Sun KH, Liu WT, Tsai CY, Liao TS, Lin WM, Yu CL. Inhibition
of astrocyte proliferation and binding to brain tissue of anticardiolipin antibodies purified from lupus serum. Ann Rheum Dis 1992;
51:707–712.
7. Cervera R, Asherson RA, Font J, et al. Chorea in the antiphospholipid syndrome: clinical, radiologic and immunologic characteristics of 50 patients from our clinics and the recent literature.
Medicine (Baltimore) 1997;76:203–212.
8. Kiechl-Kohlendorfer U, Ellemunter H, Kiechl S. Chorea as the
presenting clinical feature of primary antiphospholipid syndrome
in childhood. Neuropediatrics 1999;30:96 –98.

949

9. Milanov I, Bogdanova D. Antiphospholipid syndrome and dystonia–parkinsonism: a case report. Parkinsonism Relat Disord
2001;7:139 –141.
10. Angelini L, Zibordi F, Zorzi G, et al. Neurological disorders, other
than stroke, associated with antiphospholipid antibodies in childhood. Neuropediatrics 1996;27:149 –153.
11. Engelen M, Tijssen MAJ. Paroxysmal non-kinesigenic dyskinesia
in antiphospholipid syndrome. Mov Disord 2005;20:111–113.
12. Asherson RA, Khamashta MA, Ordi-Ros J, et al. The “primary”
antiphospholipid syndrome: major clinical and serological features. Medicine (Baltimore) 1989;68:366 –374.
13. Swedo SE, Leonard HL, Garvey M, et al. Pediatric autoimmune
neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry
1998;155:264 –271.
14. Martins da Silva A, Rocha N, Pinto M, et al. Tremor as the first
neurological manifestation of Sneddon’s syndrome. Mov Disord
2005;20:248 –251.
15. Morris HR, Lees AJ. Primary antiphospholipid syndrome presenting as a corticobasal degeneration syndrome. Mov Disord 1999;
14:530 –531.
16. Bhatia KP, Lee MS, Rinne JO, et al. Corticobasal degeneration
look-alikes. In: Litvan I, Goetz CG, Lang AE, editors. Corticobasal
degeneration and related disorders. Philadelphia: Lippincott Williams and Wilkins; 1999. p 169 –182.
17. Kleiner-Fisman G, Bergeron C, Lang AE. Presentation of
Creutzfeldt–Jakob disease as acute corticobasal degeneration syndrome. Mov Disord 2004;19:948 –949.
18. Benito-Leon J, Alvarez-Linera J, Louis ED. Neurosyphilis
masquerading as corticobasal degeneration. Mov Disord 2004;19:
1367–1370.
19. Gomez-Puerta JA, Cervera R, Calvo LM, et al. Dementia associated with the antiphospholipid syndrome: clinical and radiological
characteristics of 30 patients. Rheumatology (Oxford) 2005;44:95–
99.
20. Shi W, Krilis SA, Chong BH, Gordon S, Chesterman CN. Prevalence of lupus anticoagulant and anticardiolipin antibodies in a
healthy population. Aust NZ J Med 1990;20:231–236.
21. Vila P, Hernandez MC, Lopez-Fernandez MF, Batlle J. Prevalence, follow-up and clinical significance of the anticardiolipin
antibodies in normal subjects. Thromb Haemost 1994;72:209 –213.
22. Vaarala O, Palusuo T, Kleemola M, Aho K. Anticardiolipin response in acute infection. Clin Immunol Immunopathol 1986;41:
8 –15.
23. Ziporen L, Polak-Charcon S, Korczyn DA, et al. Neurological
dysfunction associated with antiphospholipid syndrome: histopathological brain findings of thrombotic changes in a mouse
model. Clin Dev Immunol 2004;11:67–75.
24. Brey RL, Gharavi AE, Lockshin MD. Neurologic complications of
antiphospholipid antibodies. Rheum Dis Clin North Am 1993;19:
833– 850.
25. Ferreira S, D’Cruz DP, Hughes GR. Multiple sclerosis, neuropsychiatric lupus and antiphospholipid syndrome: where do we stand?
Rheumatology (Oxford) 2005;44:434 – 442.
26. Aziz A, Conway MD, Robertson HJ, Espinoza LR, Wilson WA.
Acute optic neuropathy and transverse myelopathy in patients with
antiphospholipid antibody syndrome: favorable outcome after
treatment with anticoagulants and glucocorticoids. Lupus 2000;9:
307–310.
27. Ruiz-Irastorza G, Khamashta MA. Warfarin for multiple sclerosis?
Q J Med 2000;93:497– 499.
28. D’Cruz DP, Mellor-Pita S, Joven B, et al. Transverse myelitis as
the first manifestation of systemic lupus erythematosus or lupuslike disease: good functional outcome and relevance of antiphospholipid antibodies. J Rheumatol 2004;31:280 –285.

Movement Disorders, Vol. 21, No. 7, 2006