Clin Rheumatol (2004) 23: 364–367
DOI 10.1007/s10067-004-0897-4

CASE REPORT

Rika Kuriwaka Æ Makoto Kunishige
Haruhito Nakahira Æ Hideo Inoue Æ Tatsuo Higashi
Yoshiaki Tokumoto Æ Takao Mitsui

Neuro-Behçet’s disease with chorea after remission
of intestinal Behçet’s disease
Received: 27 October 2003 / Accepted: 23 January 2004 / Published online: 17 April 2004
Ó Clinical Rheumatology 2004

Abstract Neuro-Behçet’s disease shows various neuropsychiatric symptoms, but chorea has rarely been reported. We report a case of neuro-Behçet’s disease in a
67-year-old woman with depression and chorea that
occurred 22 years after the onset of intestinal Behçet’s
disease. Brain magnetic resonance imaging (MRI) using
a fluid-attenuated inversion-recovery (FLAIR) sequence
demonstrated lesions more clearly than did T2-weighted
MRI. Some of the lesions appeared as small ring-like
foci, i.e. low-intensity spots rimmed with remarkable
hyperintense signals, in the periventricular white matter
and basal ganglia. A review of the literature revealed
that the onset of chorea in cases of Behçet’s disease
varied from the time of onset of Behçet’s disease to 31
years after onset of the disease. Psychiatric manifestations have often been associated with neuro-Behçet’s
disease. In the present patient, treatment with prednisolone resolved the chorea, suggesting that the chorea
was caused by an autoimmune mechanism. It seems
likely that the long-term development of vasculitis in
patients with Behçet’s disease results in the formation of
these particular brain lesions on FLAIR MR images.
Chorea should be taken into consideration as one of the
manifestations of Behçet’s disease, even many years after
remission of the disease.

R. Kuriwaka Æ M. Kunishige (&) Æ H. Nakahira Æ H. Inoue
Department of Internal Medicine,
Anan Kyoei Hospital, 3-18-15 Kuramoto,
770-8503 Tokushima, Japan
E-mail: kuni@clin.med.tokushima-u.ac.jp
Tel.: +81-88-6339265
Fax: +81-88-6337121
T. Higashi Æ Y. Tokumoto
Department of Radiology,
Anan Kyoei Hospital,
3-18-15 Kuramoto,
770-8503 Tokushima, Japan
T. Mitsui
Department of Medicine and Bioregulatory Sciences,
University of Tokushima, Graduate School of Medicine,
Tokushima, Japan

Keywords Chorea Æ Depression Æ FLAIR MRI Æ
Neuro-Behçet’s disease Æ Vasculitis
Abbreviations ANCA: Antineutrophil cytoplasmic antibodies Æ FLAIR: Fluid-attenuated inversion-recovery Æ
MRI: Magnetic resonance imaging

Introduction
Behçet’s disease is a systemic inflammatory disorder
characterized by recurrent oral and genital ulcers,
uveitis and skin lesions, and is sometimes associated
with neurological, intestinal or vascular involvement
[1]. Chronic, progressive deficit of the central nervous
system (CNS) is known as neuro-Behçet’s disease [1, 2].
Psychiatric symptoms, including psychoneurosis/
depression, memory disturbance, attention deficit and
behavioral change, as well as neurological manifestations, including central motor paralysis and brain stem
signs, are often observed [1, 2], but chorea has rarely
been reported [3, 4, 5, 6, 7, 8]. Furthermore, only a few
patients with Behçet’s disease have been reported as
having both neurological and intestinal involvement [9,
10]. Brain magnetic resonance imaging (MRI) is useful
for the diagnosis of neuro-Behçet’s disease, which
shows lesions usually in the brain stem, basal ganglia
and/or white matter [11, 12]. However, it is often difficult to distinguish a small brain lesion from lacunar
infarction. We report unique findings of brain MRI
using a fluid-attenuated inversion-recovery (FLAIR)
sequence in a case of neuro-Behçet’s disease that presented with depression and chorea after remission of
intestinal Behçet’s disease. The lesions may be differentiated from those in lacunar infarctions.

Case report
The patient was a 67-year-old woman with a history
of recurrent stomatitis, genital ulcer and ileocecal

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Fig. 1 Axial brain MRI
revealed multiple foci in the
white matter and basal ganglia,
appearing hyperintense on
T2-weighted image (a, b). MRI
using the FLAIR sequence
(c, d) demonstrated lesions
more clearly, and some of the
lesions appeared as lowintensity spots rimmed with
high-intensity signals in the
periventricular white matter
(arrowhead) and putamen
(arrow)

resection necessitated by intestinal Behçet’s disease that
had occurred 22 years ago. She had no family history
of Huntington’s disease and involuntary movement.
Chorea suddenly occurred in her lower extremities, and
she visited the emergency room of our hospital in
October 1999. Her oral and genital ulcers had improved over the previous 15 years. She had been treated for headache and depression at a mental hospital.
She had not recently received dopamine antagonists.
On admission, she had stopped taking her medication
several days earlier. She exhibited a prominent behavior disorder, including attention deficit, delusion of
persecution, hallucination and depression, resulting in
social withdrawal. Mini-Mental State Examination
score was 23. Ophthalmologic examination revealed no
evidence of uveitis. Cranial nerve function and muscle
power were spared. Deep tendon reflexes showed
bilateral hyperreflexia with a positive jaw jerk and
Chaddock sign. Results of routine laboratory examinations were normal except for elevations of serum IgG
level (2701 mg/dl; normal, 870–1700) and antinuculear
antibody titer (640; normal, <20). Immune complex,
C3 and C4 levels were normal. Antineutrophil cytoplasmic antibodies (ANCA) and antiphospholipid antibodies were negative. Her HLA type was B51. There
were no findings of fever, hypertension, coagulopathy,

hyperlipidemia or other potential causes of cerebrovascular accident. Serum homocysteine concentration was normal. Results of laboratory examinations
did not satisfy the criteria for other collagen diseases.
Examination of the cerebrospinal fluid (CSF) showed
that the cell count was 2 cells/mm3, protein level was
55 mg/dl (normal, <45) and IgG-albumin index was
0.776 (normal, <0.7). EEG showed diffuse slowing.
Conventional brain MRI revealed multiple foci in the
basal ganglia and white matter, appearing hypointense
on T1-weighted images and hyperintense on T2-weighted images (Fig. 1a, b). MRI using the FLAIR sequence revealed lesions more clearly, and some of them
appeared as low-intensity spots rimmed with highintensity signals (Fig. 1c, d). MR angiography was
unremarkable. Her choreic movement improved after
treatment with diazepam, but subsequently recurred.
Anticonvulsants did not resolve the repeated chorea,
but 60 mg/day prednisolone induced relief. Despite the
administration of antidepressant, anxiolytic and sedative drugs, including maprotiline, zopiclone, diazepam
and etizolam (none of which are dopamine antagonists), her headache persisted and her psychotic features initially showed a slight improvement, but later
became more serious. She sometimes asked us to terminate her life, and in May 2001 she attempted suicide.

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She refused repeated lumbar puncture and brain MRI
studies.

Discussion
Chorea is usually caused by ischemic brain lesions,
toxins, neuroleptic drugs, dopamine antagonists and
heritable metabolic disorders such as Huntington’s disease [13]. In our patient, drug-induced chorea was less
likely because none of the previous psychiatric treatments included dopamine antagonists, and she had
stopped taking her medication several days before the
onset of the chorea. Huntington’s disease is also unlikely
because there was no family history of involuntary
movements or compatible MRI findings. Brain MRI
revealed multiple foci in the white matter and basal
ganglia. Because chorea has been associated mainly with
lesions in the caudate nucleus or putamen [13], the brain
lesions in the putamen may have been responsible for
her chorea. In a review of 200 patients with neuroBehçet’s disease [2], attacks of neurologic deficits with
headache and gradual behavioral changes were a characteristic clinical picture of neuro-Behçet’s disease, and
6% of patients had movement disorders such as hemichorea, hemiballismus and hemidystonia. To the best of
our knowledge, only 6 cases of neuro-Behçet’s disease
with chorea have been reported (age range of the
patients, 39–55 years; 3 males and 3 females) [5, 6, 7, 8].
Oral and/or genital ulcers were noted in 4 patients [3, 5,
7, 8], but no uveitis was evident in any of the patients at
the time of presentation of chorea. The disease duration
between onset of Behçet’s disease and occurrence of
chorea was varied. Three patients presented with chorea
at the onset of Behçet’s disease [5, 7, 8], and three
developed chorea 7 months [6], 6 years [3], and 31 years
[4] after onset of the disease. Psychiatric manifestations,
including memory disturbance [5, 8], dementia [6],
inattention [7, 8] and psychomotor agitation [7], have
been noted. Brain MRI was performed in 4 patients, and
all of the detected lesions included the caudate nucleus
[5, 6, 7, 8]; however, the FLAIR sequence was not used.
Neuropsychiatric symptoms were improved by treatment with prednisolone in 3 patients [3, 5, 7], but 2 required the additional administration of colchicine [6] or
haloperidol [8]. The present patient had developed
headache, depression, delusion and hallucination followed by chorea with no recurrent ulcers on admission,
22 years after remission of intestinal Behçet’s disease.
The long-lasting clinical course was similar to that of a
patient reported by Bussone et al. [4], who developed
choreic dyskinesia, anxiety, suspicion and restlessness
after improvement of ulcers, and who had been diagnosed as having Behçet’s disease 31 years earlier. Neuropsychiatric involvement may progress insidiously and
become prominent several years after the onset of
Behçet’s disease. In the present patient, treatment with
prednisolone resolved the chorea, suggesting that the
cause was an inflammatory mechanism associated with

Behçet’s disease. Therefore, it seems likely that the longterm development of vasculitis results in a unique
appearance on brain MRI scans using the FLAIR sequence. The FLAIR sequence allows heavily T2-weighted images to be obtained with no or considerably
reduced CSF signals obscuring adjacent brain regions.
In a previous study on neuro-Behçet’s disease, MRI
using the FLAIR sequence revealed significantly more
lesions than did MRI using the conventional dual-echo
spin-echo (SE) sequence [11]. In the present patient,
MRI using the FLAIR sequence also demonstrated
brain lesions more clearly than did T2-weighted MRI.
Moreover, some of the lesions appeared as small ringlike foci, i.e. low-intensity spots rimmed with remarkable
hyperintense signals, in the periventricular white matter
and basal ganglia. It is difficult to distinguish a neuroBehçet’s lesion from lacunar infarction on conventional
T1- or T2-weighted MR images if the lesion is small. On
the FLAIR images, lacunar infarction initially appears
as a hyperintense spot, and chronic lacunar infarction
appears as a hypointense spot occasionally with a hyperintense rim [14]. The latter finding seems to depend
on the consequent encephalomalacia in chronic lacunar
infarction. Therefore, a hypointense lesion has an
equivocal margin with a slightly hyperintense rim,
whereas the present case showed small ring-like foci, i.e.
low-intensity spots rimmed with remarkable hyperintense signals. The primary pathologic change in Behçet’s
disease is based on vasculitis, and the neuropathologic
features in the brain of a patient with neuro-Behçet’s
disease are necrotic foci with accumulated microglial
phagocytes and focal cuffing of blood vessels by lymphocytes [1]. It has been suggested that cerebral vasculitis should be considered in neuro-Behçet’s disease,
although low-grade chronic meningoencephalitis is the
principal neuropathological process [15]. On FLAIR
MR images in the present case, the remarkable hyperintense rim could represent vasculitis and the hypointense spot may represent central necrosis. A similar
appearance on brain FLAIR MR images has been in a
case of primary vasculitis of the CNS [16].
In conclusion, chorea should be taken into consideration as one of the manifestations of Behçet’s disease,
even many years after remission of the disease. Brain
MRI using the FLAIR sequence has a potential
advantage to demonstrate cerebrovascular involvement
caused by chronic vasculitis in neuro-Behçet’s disease.

References
1. Goodin DS (1998) Behçet’s disease. In: Aminoff MJ, Goetz
CG, eds. Handbook of clinical neurology, vol. 27(71), Systemic
Diseases, Part III. Elsevier Science, Amsterdam, pp/ 209–231
2. Akman-Demir G, Serdaroglu P, Tasçi B, the Neuro-Behçet
Study Group (1999) Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. Brain
122:2171–2181
3. Schotland DL (1963) Neurologic aspects of Behçet’s disease.
Case report and review of the literature. Am J Med 34:544–553

367
4. Bussone G, La Mantia L, Boiardi A, Giovannini P (1982)
Chorea in Behçet’s syndrome. J Neurol 1982;227:89–92
5. Kojima S, Mori M, Nakai R, Tsuboi Y, Kitano K (1996)
Chorea in neuro-Behçet’s syndrome. A case report. Shinkeinaika 45:115–120 [in Japanese]
6. Iwatsuki K, Deguchi K, Narai H et al. (2000) A case of neuroBehçet’s disease with HLA B54 and predominant cerebral
white matter lesions. Rinsho Shinkeigaku 40:921–925 [in Japanese]
7. Revilla FJ, Racette BA, Perlmutter JS (2000) Chorea and jawopening dystonia as a manifestation of neuroBehçet’s syndrome. Mov Disord 15:741–744
8. Kimura N, Sugihara R, Kimura A, Kumamoto T, Tsuda T
(2001) A case of neuro-Behçet’s disease presenting with chorea.
Rinsho Shinkeigaku 41:45–49 [in Japanese]
9. Kozin F, Haughton V, Bernhard GC (1977) Neuro-Behçet’s
disease: two cases and neuroradiologic findings. Neurology
27:1148–1152
10. Ikezoe K, Yamashita Y, Sakai Y, Yamamoto I (1998) NeuroBehçet’s disease with unilateral multiple brain lesions during
the active phase of entero-Behçet’s disease. A case report.
Shinkeinaika 49:537–540 [in Japanese]

11. Jäger HR, Albrecht T, Curati-Alasonatti WL, Williams EJ,
Haskard DO (1999) MRI in neuro-Behçet’s syndrome: comparison of conventional spin-echo and FLAIR pulse sequences.
Neuroradiology 41:750–758
12. Coban O, Bahar B, Akman-Demir G et al. (1999) Masked
assessment of MRI findings: is it possible to differentiate neuroBehçet’s disease from other central nervous system diseases?
Neuroradiology 41:255–260
13. Janavs JL, Aminoff MJ (1998) Dystonia and chorea in acquired
systemic disorders. J Neurol Neurosurg Psychiatry 65:436–445
14. Alexander JA, Sheppard S, Davis PC, Salverda P (1996) Adult
cerebrovascular disease: role of modified rapid fluid-attenuated
inversion-recovery sequences. Am J Neuroradiol 17:1507–1513
15. Krepsi Y, Akman-Demir G, Poyraz M et al. (2001) Cerebral
vasculitis and ischaemic stroke in Behçet’s disease: report of
one case and review of the literature. Eur J Neurol 8:719–722
16. Pomper MG, Miller TJ, Stone JH, Tidmore WC, Hellmann DB
(1999) CNS vasculitis in autoimmune disease: MR imaging
findings and correlation with angiography. Am J Neuroradiol
20:75–85