Brain (1999), 122, 21832194

Neurological complications in Behcets syndrome

D. Kidd,1 A Steuer,2,3 A. M. Denman2,3 and P. Rudge1
1The

National Hospital for Neurology and Neurosurgery,

London and the Departments of 2Immunology and
3Rheumatology, Northwick Park Hospital, Harrow, UK

Correspondence to: Dr Desmond Kidd, Department of

Clinical Neurosciences, Royal Free Hospital, Pond Street,
London NW3 2QG, UK
E-mail: d.kidd@rfhsm.ac.uk

Summary
The neurological complications of Behcets syndrome have
not been characterized with clarity. We present the clinical
features, imaging characteristics and CSF findings of a
series of 50 patients seen at the National Hospital for
Neurology and Neurosurgery over the past 10 years. In
this series, vascular complications had a low prevalence,
whereas involvement of the brainstem was common;
spinal cord lesions, hemisphere lesions and meningoencephalitis also occurred. Optic neuropathy, vestibulocochlear and peripheral nerve involvement occurred, but
were rare. The prognosis for recovery was in general

good, and the majority of those followed-up over a median

of 3 years (range 119 years) had only single attacks.
One-third of patients underwent further attacks, and four
underwent progressive deterioration leading to disability.
Factors suggesting a poor prognosis are repeated attacks,
incomplete recovery, progressive disease course and a
high level of CSF leucocytosis during acute attack. These
data should be of help in the further definition of the
clinical characteristics of this rare neurological disorder
and in the planning of treatment trials.

Keywords: neurological diseases; Behcets syndrome; MRI; CSF

Introduction
Behcets syndrome is an episodic disorder of unknown
aetiology or pathogenesis, characterized by recurrent oral and
genital ulceration and panuveitis. Constitutional disturbance is
common, and there is malaise, fatigue and loss of weight. Skin
involvement, characterized by erythema nodosum, pustular
eruptions or pseudofolliculitis, is also common, and there is
an oligoarthropathy of large joints such as the knees, ankles
and shoulders. Involvement of the lungs, gastrointestinal tract
and kidneys has also been noted, but is rare (ODuffy, 1994).

were sufficiently high have suggested a prevalence of 5.3%

in Istanbul (Serdaroglu et al., 1989), 16% in Casablanca
(Benamour et al., 1990), 25% in Alexandria (Assaad-Khalil
et al., 1993) and 3.3% in a nationwide survey in Iran
(Davitchi et al., 1997). In an autopsy series, 20% of 170
cases of patients with Behcets syndrome showed pathological
evidence for neurological involvement (Lakhanpal et al.,
1985).

Diagnosis
Epidemiology
Behcets syndrome is most common in the countries around
the eastern shores of the Mediterranean, the Middle East and
Eastern Asia, particularly Japan, where the prevalence was
7/105 in 1974 (Yamamoto et al., 1974). In Turkey the
prevalence was found to be higher in rural than in urban
areas (37/105 versus 8/105) (Yurdakul et al., 1988). There
has been only one published survey of the disease in the UK
(Chamberlain, 1977), in which the prevalence was found to
be 0.4/105. There has been no epidemiological study of cases
of Behcets syndrome with neurological complications, other
than those that have reported the prevalence of such
complications in hospital populations attending specialist
Behcets syndrome clinics. Authors whose patient populations
Oxford University Press 1999

The diagnostic criteria are summarized in Table 1

(International Study Group for Behcets Disease, 1990).
These are based on a cohort of 914 patients from several
centres around the world. Clinical features were compared
with those of 308 patients with connective tissue diseases
who had also had recurrent oral ulceration. Use of the new
criteria offered an increase in diagnostic sensitivity and
specificity compared with previously published diagnostic
criteria. Although it was specified that recurrent oral
ulceration was a prerequisite, it was acknowledged that cases
existed in which there was pathologically proven Behcets
syndrome without oral ulceration; it was stated that the
criteria would exclude only 3% of patients in whom recurrent
oral ulceration was not a feature.

2184

D. Kidd et al.

Table 1 International Study Group for Behcets disease

criteria
Recurrent major or minor aphthous or herpetiform ulceration of
the mouth
Plus
recurrent genital ulceration
erythema nodosum
pseudofolliculitis
papulopustular eruption
acneiform nodules
pathergy test positivity
anterior or posterior uveitis
retinal vasculitis

Aetiology
The aetiology is unknown; an infective agent has long
been postulated but has never been identified. Neutrophil
hyperfunction and an increase in the CD81/CD41 cell ratio
occur. There is an increase in circulating T cells bearing
receptors; indeed, peptides derived from the 65 kDa heat
shock proteins have been shown to stimulate T cells
specifically from patients with the disease (Suzuki et al.,
1992; Lehner, 1997). Such cells have been shown to be
uveitogenic to Lewis rats (Stanford et al., 1994). The
association with HLA B51 is well known and this is
particularly associated with uveitis, although even in Japanese
series the prevalence of HLA B51 is only 57% (Mizuki et
al., 1997). There is some evidence that HLA B51, as well
as B12, DR7 and DR2, may bear a relationship with the
tissue location of the disease. Vascular complications may
be related to abnormal procoagulant activity, which may have
a primary role, or they may be secondary to endothelial cell
activation.

Clinical picture of neurological Behcets

syndrome
Neurological involvement in Behcets syndrome was first
described in the early decades of this century. Pallis and
Fudge (1956) and Wadia and Williams (1957) described the
clinical syndromes and classified them into three types.
(i) Brainstem disturbance associated with systemic symptoms
such as fever, arthralgia and skin eruption; the brainstem
signs developed subacutely with evolving cranial neuropathies, ocular motor dysfunction, nystagmus and gaze
palsies, dysarthria and ataxia and, on occasions, bulbar muscle
weakness. Headache with meningism was commonly cited,
and in these cases CSF pleocytosis and high protein content
were observed. (ii) Meningomyelitis, comprising a meningitis
with varying neurological signs, including spinal cord and
hemisphere signs (Silfverskiold, 1951). (iii) A confusional
syndrome, in which meningoencephalitis without focal
neurological signs arose, which was in some instances chronic
and progressive, resulting ultimately in the development of
dementia, Parkinsonism, pseudobulbar palsy and quadriparesis. Pallis and Fudge (1956) also described a case with
acute myelitis. Wadia and Williams (1957) were the first to

describe a response to corticosteroids, which Evans and

colleagues confirmed (Evans et al., 1957), although others
noted some cases in which no response was evident. All had
noted a propensity for relapse and remission. Further reviews
have been published from time to time (Wolf et al., 1965;
ODuffy and Goldstein, 1976; Al-Kawi, 1992; Serdaroglu,
1998).
Bienenstock and Margulies (1961) described a case in
which cerebral angiography revealed venous sinus thrombosis
as the cause of their patients syndrome. Vascular complications as a result of dural venous sinus thrombosis occur
commonly in some series (Bousser et al., 1980; el-Ramahi
and Al-Kawi, 1991; Serdaroglu, 1998). Others present with
isolated intracranial hypertension (Pamir et al., 1981; Shakir
et al., 1990; Akman-Demir et al., 1996a); dural venous sinus
thrombosis is found in association with this in most (but not
all) cases, and the CSF is in general inactive. Arterial
thrombosis is rarer, and aneurysm formation has been reported
(Wechsler et al., 1989).
Involvement of muscle (Arkin et al., 1980; Lang et al.,
1990), causing a polymyositis, appears to be rare. Peripheral
neuropathy has been reported (ODuffy and Carney, 1971;
Rougemont et al., 1982), with nerve biopsy and electrophysiological characteristics of a non-vasculitic axonal neuropathy.
A predominately motor polyradiculopathy has also been
reported (Bakouche and Guillard, 1984).

Neuropathology
The first paper to describe the neuropathology of such cases
was in 1944 (Berlin, 1944); in this report the basal meninges
were seen to be thickened and multiple foci of cellular
infiltration within the meninges and parenchymal lesions
were observed. Silfverskiold described findings in a patient
who had died within 5 days of the onset of a severe brainstem
syndrome and saw that there was considerable swelling of
the brainstem with multiple foci of cellular infiltration and
perivascular inflammatory cell cuffs (Silfverskiold, 1951).
McMenemey and Lawrence described these cases and two
others; in these, as in the others published previously, the
brainstem was predominately involved, and areas of softening
with cellular infiltration and perivascular cuffing were seen
(McMenemey and Lawrence, 1957). Lesions also involved
the cortex and other grey matter structures, in which loss of
neurons was seen. Rubinstein and Urich reported a further
case in which the patient died after a 6 year disease course
characterized initially by relapses and then progressive
deterioration (Rubinstein and Urich, 1961). Their findings
were typical of those previously described: there was a chronic
meningoencephalitis with inflammatory cell infiltration and
circumscribed areas of necrosis with loss of all tissue
elements, accumulation of lipid-laden macrophages and
gliosis. Although the white matter was more often involved,
there was clear evidence for lesion formation within the grey
structures, including the cortex, basal ganglia and brainstem
nuclei. A large series from Japan (Kawakita et al., 1967)

Neurological complications in Behcets syndrome

summarized the findings in nine cases from that country and
compared them with the 13 published from Europe and North
America. No difference was noted. Miyakawa and colleagues
and Yamamori and colleagues described cases in which
marked cerebral atrophy was seen; in these cases involvement
of the cortex with marked loss of neurons was observed in
addition to the white matter findings noted in previous reports
(Miyakawa et al., 1976; Yamamori et al., 1994). Lueck and
co-authors, from this institution, published the post-mortem
findings of their case, who had presented with a relapsing then
progressive meningoencephalitis without systemic features in
which the neuropathology showed features characteristic of
Behcets disease (Lueck et al., 1993). Hadfield and colleagues
have shown that there is a marked infiltration by neutrophils
and eosinophils as well as by lymphocytes, and there is at
times marked axonal degeneration within lesions (Hadfield
et al., 1996). No evidence for vasculitis has been found in
this or other pathological studies.

Current study
The purpose of this study was to define further the various
clinical syndromes associated with this disorder, to
characterize the various immunological and imaging
abnormalities seen and to attempt to identify clinical and
immunological prognostic factors for subsequent disease
activity. We also investigated possible differences in these
characteristics between patients of European stock and those
born in regions with higher disease prevalence.

Patients and methods

The medical notes of all patients admitted to the National
Hospital for neurological investigation and treatment with a
diagnosis of Behcets syndrome over the past 15 years were
reviewed. Most had been under the care of one of the authors
(P.R.). Many had been referred by another (A.M.D.), and in
order to follow-up their cases the medical notes of such
patients were scrutinized at Northwick Park Hospital. The
diagnosis of Behcets syndrome was accepted if the clinical
course and features fulfilled the International Study Group
criteria (International Study Group for Behcets Disease,
1990). Statistical comparison of groups used the Mann
Whitney test.

Results
Fifty patients were studied; their mean age was 31 years (SD
6 years). Thirty-one (62%) were male. Thirty-nine were of
European stock, three were born in Iran, three in Turkey,
two in the Indian subcontinent and three in North Africa. All
but one had had mouth ulcers, 41 had had orogenital ulceration
and 29 had developed uveitis. Systemic symptoms, such as
oligoarthritis (20 cases) and skin lesions (22 cases), were
relatively common, although in 30 cases the neurological
syndrome arose in conjunction only with orogenital ulceration

2185

Table 2 Clinical presentation in the 50 patients

Meningoencephalitis with brainstem involvement
Spinal cord involvement
Hemisphere involvement
Meningitis
Isolated intracranial hypertension
Cerebral venous sinus thrombosis*
Cranial neuropathy*

II
V 1 VII
VII
VIII

25
7
5
4
4
2
1
2
2
3

*Some patients shared different clinical subgroups.

and/or uveitis. The median latency from onset of systemic

symptoms to neurological presentation was three (028)
years. Twelve patients presented first with neurological
symptoms. In one case, published previously (Lueck et al.,
1993), the diagnosis was made only at autopsy, following a
neurological illness without any evidence of a systemic
disturbance at all.

Neurological presentation (Table 2)

Twenty-five patients presented with a meningoencephalitis
involving the brainstem, seven with spinal cord syndromes
and five with hemisphere signs. Four presented with
symptoms and signs of meningitis and encephalopathy alone.
One patient had signs of an optic neuropathy for which no
other cause has been found. One presented with bilateral
facial weakness, two with acute vestibular disturbance due
to an isolated vestibular lesion, and one with isolated bilateral
sensorineural deafness. Vascular complications were rare in
our series; two presented with cortical venous thrombosis.
Four patients presented with intracranial hypertension alone.
In the patients with isolated brainstem disturbance, the
disorder had a tendency to arise subacutely over days. Twenty
patients presented with ataxia and ocular motor dysfunction,
one having signs indicating a pontine disturbance and two
having bulbar symptoms, with dysarthria and dysphagia. The
severity of the accompanying long tract signs was variable.
In two cases the patient presented acutely with a severe
brainstem encephalitis requiring intensive and ventilatory
care. Isolated cranial nerve lesions also occurred; in these
cases lesion of nerve VII was the most prevalent (three cases,
of which two also had trigeminal involvement).
Seven patients presented with spinal cord disease; two of
these patients presented with a severe transverse myelitis
with paraplegia, two with a BrownSequard syndrome and
the others with sensory disturbance with increased reflexes
and sphincter disturbance but without weakness.
Of the patients with hemisphere disturbance, four presented
with hemiparesis and one with hemisensory disturbance
alone. Three had seizures, including one patient who presented
de novo with hippocampal complex partial seizures.
Symptoms of meningitis that preceded or were an important

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D. Kidd et al.
of CSF and imaging abnormalities was also non-significantly
different.

Imaging results

Fig. 1 FLAIR (fluid attenuated inversion recovery) sequence MRI

[1.0 T; TR (repetition time) 7000 ms, TE (echo time) 150 ms] of
a patient who presented with seizures and hemiparesis due to
cortical vein thrombosis with infarction.

part of the clinical syndrome in those with parenchymal

involvement were common, arising in 16 out of the 39 cases.
Two patients presented with an isolated vestibulopathy;
one of these patients showed evidence for canal paresis and
a preponderance of directional rotation on electronystagmography.
Cortical vein thrombosis was seen in two cases, one
of whom showed evidence of concurrent meningitis. One
presented with a hemisphere lesion with seizures due to
cortical vein thrombosis (Fig. 1) and the other with intracranial
hypertension. Isolated intracranial hypertension was seen in
three other cases, in whom no evidence for meningitis
was found.

Ethnic differences
Eleven (22%) patients were not born of Northern European
stock. There was no difference in the age of onset of the
disease [23 years (range 1052 years) versus 27 years (range
1044 years), P 5 0.34], age at onset of neurological
complications [31 years (range 1452 years) versus 28 years
(range 1844 years), P 5 0.54] (non-European and European
subjects, respectively), characteristics of the systemic disease
and the characteristics of the neurological disorder. Similar
conclusions have been drawn in a series from France
(Wechsler et al., 1988). Six patients had had a brainstem
syndrome, two a cord lesion, two a hemisphere disturbance
and one isolated intracranial hypertension. The prevalence

Imaging showed MRI abnormalities in the clinically affected

area in all but one case of those presenting with a brainstem
syndrome (Table 3). The other was a normal CT scan. In
three cases in addition to the brainstem lesion, multiple
predominantly periventricular white-matter lesions were seen
in the hemispheres bilaterally. Lesions correlated well with
the clinical syndrome; 20 showed lesions within the midbrain
or superior cerebellar peduncle (Fig. 2), sometimes extending
to the diencephalon, one showed a single lesion within the
pons, and two showed medullary lesions. Severe atrophy was
noted in cases with progressive disease (Fig. 2). In two cases,
in whom the clinical syndrome was of a severe acute
brainstem disturbance, the entire brainstem was seen to be
involved (Fig. 3) and considerable brainstem swelling was
observed in the acute phase (Fig. 4).
All those with hemisphere syndromes showed multiple
white matter lesions in both hemispheres, which tended not
to be localized to the periventricular regions. Among patients
with cord syndromes, spinal cord imaging was undertaken
in three cases; the image was abnormal in two cases, one
showing a high signal intensity lesion with and one without
adjacent cord swelling (Fig. 5). In two other cases the brain
alone was scanned, and this was normal in both cases.
In the single patient with an isolated optic neuropathy,
imaging showed no abnormality, including the optic nerves.
In the two patients with isolated vestibulopathy and the single
case with bilateral deafness, imaging of the brain was normal.
In patients presenting with meningitis alone or isolated
intracranial hypertension, imaging of the brain was normal
in each case.

CSF results
CSF examination was carried out in 18 of 25 patients with
brainstem disturbance; CSF was active in 17 cases, in which
leucocytosis was seen. In 11 of the latter cases there was an
increase in protein concentration (Table 3). Those with
meningitis and encephalopathy had abnormal CSF in all four
cases, whilst half of the samples examined were abnormal
in patients with hemisphere and spinal cord disturbance.
Among those presenting with isolated intracranial hypertension the CSF was abnormal in one case, and in the single
cases of those with dural vein thrombosis, optic neuropathy
and isolated vestibulopathy, CSF was normal. Intrathecal
synthesis of immunoglobulin was not observed in the cases
studied; matched serum/CSF bands were seen in seven cases
(18%). In one case a monoclonal band was seen which had
disappeared 6 months later when the CSF examination had
been repeated during a reappraisal of the diagnosis.
The difference between median CSF protein concentration
in the brainstem group and the meningoencephalitis group

Neurological complications in Behcets syndrome

2187

Table 3 MRI and CSF results in patient subgroups, divided into the clinical syndromes with which they presented
Patient
subgroup

Number

MRI

CSF

No data

Normal

Local

Local 1
No data
periventricular

Protein
(mg/mm3)
median
(range)

Total WCC %
(cells/mm3) polymorphs
median
(range)

%
%
lymphocytes monocytes

OB
/

OB
1/

OB
1/1

25

19

0.51
(0.17-3.70)

40
(1360)

9.5
(080)

80
(20100)

0
(050)

14

Cord

3*

0.57
(0.372.4)

67
(10103)

0
(050)

75
(0100)

0
(0100)

Hemisphere

0.69
(0.280.79)

2
(122)

6.0
(553)

67
(4090)

5
(05)

Meningitis

n/a

1.01
(0.622.5)

15
(2368)

82
(090)

10
(10100)

0
(08)

ICH

0.53
(0.30.66)

2
(128)

4.0
(020)

95
(80100)

0
0

Brainstem

OB x/x 5 oligoclonal immunoglobulin bands in CSF/serum; WCC 5 CSF white cell count; ICH 5 intercranial hypertension. *Two
normal brain MRI, one normal cord MRI.

Fig. 2 T2-weighted MRI (1.5 T; TR 3000 ms, TE 91 ms) of a patient with multiple brainstem attacks
and a progressive disease course, showing a lesion within the midbrain, and brainstem and cerebellar
atrophy.

2188

D. Kidd et al.

Fig. 3 T2-weighted MRI (0.5 T; TR 3800 ms, TE 92 ms) of a patient who presented with an acute
brainstem syndrome with apnoea requiring prolonged ventilatory support, showing very pronounced
high signal intensity abnormalities throughout the brainstem.

was significant (P 5 0.042); in all other cases CSF protein

and white cell count were not significantly different between
groups (P . 0.05).

Evoked potentials
The visual evoked potential was measured in 10 cases; it
was abnormal in one case. Sensory evoked potentials were
measured in eight cases and were normal in each case.
Brainstem auditory evoked potentials were measured in 12
cases and abnormalities were detected in two cases, in which
there was a delay in conduction at the level of the pons in
patients with brainstem dysfunction.

Clinical follow-up
Thirty-five (70%) patients have been followed-up for a
median of 3 years (range 119 years). All four cases of
intracranial hypertension underwent shunting procedures and
have remained well. Recovery from attacks with parenchymal
lesions was in general good; the majority of patients recovered

well without residual disability. Three patients (one with

transverse myelitis and two with a brainstem disturbance),
however, made no improvement and are left with severe
neurological impairments. Further attacks occurred in 12
patients, in whom a median of one relapse (range 16) has
arisen to date. Relapse occurred frequently in patients with
brainstem involvement at onset (Table 4), and eight patients
had further brainstem attacks. One patient with cord
involvement initially had a further cord syndrome, and one
each with cord involvement and hemisphere involvement had
a brainstem attack at a later point. Four patients have
subsequently undergone progressive deterioration leading to
severe impairment and disability; all of these patients had
brainstem disturbance initially and all have had further
attacks. There were two deaths, both owing to aspiration
pneumonia with severe brainstem impairment.
In an attempt to identify significant prognostic factors
during the first neurological attack, CSF protein and white
cell count were measured. There was no significant difference
in CSF protein or white cell count between patients followed
up who had had a single attack and those whose attacks were

Neurological complications in Behcets syndrome

2189

Fig. 4 T1-weighted MRI (0.5 T; TR 420 ms, TE 15 ms) following injection of gadoliniumDTPA of
the same patient as in Fig.3, showing swelling of the brainstem and enhancement of the lesion within
the pons.

recurrent. However, the median CSF white cell count was

higher in patients who subsequently became disabled or died
[103 (range 5368)] than in those who were not disabled at
follow-up [12 (range 1180)] (P 5 0.02).

Discussion
In this large clinical series of patients with neurological
involvement in Behcets syndrome the incidence of brainstem
involvement was high (52%) and that of vascular
complications low (4%). Involvement of the hemisphere,
spinal cord and optic nerves was also seen. No difference
was noted between the small number of non-Northern
European-born patients and the others. Patients with active
disease showed a mixed lymphocyte and neutrophil
pleocytosis and there was no evidence for intrathecal synthesis
of immunoglobulin. Imaging revealed lesions localized to
the clinically affected areas in most cases examined, with
evidence for lesion dissemination in a further three cases.
The prognosis for recovery following acute relapse was in
general good, the majority of patients becoming symptomfree. However, of those who were followed-up 28%
underwent further attacks and 14% became significantly
disabled as a consequence of either the absence of recovery
or the development of a progressive disease course. It should
be noted, however, that in this series the median follow-up
was 3 years (range 119 years); a longer follow-up may
reveal a more frequently relapsing disease than these data

suggest. This may also account for the lower incidence of

progressive disease than that noted in other studies, although
we did take care to follow up patients who returned to the
rheumatology clinics in order to minimize this potential bias.
One patient presented with signs of optic neuropathy, for
which no alternative cause has been found; imaging was
normal and CSF immunoglobulin oligoclonal bands were
absent. Optic neuropathy is rare in Behcets syndrome, only
a handful of cases having been published (Colvard et al.,
1977; Kansu et al., 1989). Pathological examination of one
case has shown gliosis and demyelination within the nerve
(Colvard et al., 1977).
Of particular interest are the three patients who presented
with or developed vertigo or hearing loss. There has been
only one report of auditory findings, in a small series of
patients with Behcets syndrome (Brama and Fainaru, 1980).
In two of our patients, initial asymmetrical hearing loss
progressed to involve both ears, but there were fluctuations,
often over a period of weeks, in the magnitude of the hearing
deficit. Extensive tests of auditory function showed that the
hearing decline was due to hair cell loss; both patients
showed recruitment on assessment of loudness discomfort
level and the stapedius reflex measurement; they had normal
brainstem auditory evoked potentials and absent echoes. The
intolerance of loud sounds reported by one patient is also
consistent with hair cell loss. Both had recurrent attacks of
severe vertigo that resolved over 14 weeks. Only one of
the patients had abnormal caloric responses, indicating loss

2190

D. Kidd et al.

Fig. 5 T2-weighted MRI (1.5 T; TR 5000 ms, TE 130 ms) of a patient who presented with an acute
disturbance of sensation in the upper and lower limbs, with urgency of micturition, showing a cord
lesion adjacent to C2.

of horizontal canal function on one side. Ultimately this

patient developed complete vestibular failure. Both patients
had normal vestibulo-ocular reflex suppression on rotational
and caloric testing, indicating a peripheral origin of the
dysfunction. Thus the three patients with auditory vestibular
abnormalities or symptoms all had a progressive peripheral
rather than a central nervous system disorder, which to some
extent mimicked Menie`res syndrome.
Previous clinical series have been small with a variable
follow-up. In one of the larger series (Serdaroglu et al., 1989;
Akman-Demir et al., 1996b), which followed-up 15 patients
who had had abnormal neurological signs out of a series of
45 who had had neurological symptoms, seven were stable
and three had died (one during a relapse); of the remaining
five, one had had two further relapses and four had undergone
progressive deterioration without superimposed relapse. In
this group of patients the incidence of CSF abnormalities
was higher than in those who were stable, and the authors

submitted that this may offer prognostic information. In our

series, disabled patients at follow-up had a higher median
CSF white cell count during the acute attack than those who
had improved and remained stable. More recent reports
(Akman-Demir et al., 1996c; Bohlega, 1996; Siva et al.,
1998), in which larger patient groups have been studied,
have shown a frequency of relapse similar to that in our own
series, but a greater frequency of progressive disease course
(3040%). They suggest that adverse prognostic factors
include young age of onset, brainstem involvement, high
frequency of relapse and the presence of CSF abnormalities. In
addition, one group has suggested that a primarily progressive
(progressive from symptom onset) disease course may exist
(Akman-Demir et al., 1996c). Our series and those of others
suggest that in general the prognosis for isolated intracranial
hypertension and dural venous sinus thrombosis is good
following satisfactory treatment. For those with parenchymal
involvement it appears that prognosis is related to the

Neurological complications in Behcets syndrome

Table 4 Frequency of relapse and site of further attacks
according to clinical subgroup at presentation
Clinical subgroup

No. of No. of cases Site of

cases with relapse relapse

Brainstem
Cord

25
7

Hemisphere
Meningitis

5
4

Intracranial
hypertension
Cranial neuropathy II
VII
VIII

8
1
1
1
1
1
0

1
4
3

0
0
1

Brainstem
Cord
Brainstem
Brainstem
Meningitis
Brainstem

Progressive
vestibular failure

frequency and number of further attacks and the development

of a progressive disease course. The pathophysiology of this
is not clear but is likely to be linked to progressive axonal
degeneration leading to atrophy, as has been intimated in
multiple sclerosis (Kidd et al., 1996, 1998; Trapp et al.,
1998). Further prospective studies are required in order to
define further the pathophysiology of the disease course and
the prognostic features.
In our series no patient showed intrathecal synthesis of
immunoglobulin. Other papers (Sharief et al., 1991; McLean
et al., 1995; Serdaroglu, 1998) have reported series in
which the majority showed evidence of bloodbrain barrier
dysfunction but few papers have shown intrathecal synthesis
of IgG, and Gille and colleagues reported that oligoclonal
immunoglobulin bands disappeared from CSF following an
acute attack (Gille et al., 1990). This is in stark contrast to
multiple sclerosis, in which persistent oligoclonal bands are
present in the CSF of 97% of patients (McLean et al., 1990;
Zeman et al., 1996). Sharief and colleagues found a greater
incidence of IgA and IgM bands (Sharief et al., 1991), which
may suggest an alternative antigenic stimulus, and Jongen
and colleagues in a single case found that IgM and IgA
bands disappeared following recovery from episodes of
meningoencephalitis (Jongen et al., 1992). In neurosarcoidosis, oligoclonal bands are also infrequent (McLean et al.,
1995); indeed, there is recent evidence to suggest that they
may also be absent, as in Behcets syndrome (V. Chamoun
and L. J. Thompson, personal communication). The mixed
pleocytosis and high frequency of neutrophils was noted in
early publications and was subsequently confirmed
(Nakamura et al., 1980). These authors also noted that the CSF
cell count diminished in response to steroid administration,
prompting speculation that there is an anti-inflammatory role
for steroids rather than simply an anti-oedema mode of action.
Our series confirms the findings of previous MRI studies
that have shown that involvement of the clinically affected
region alone is most common. Most prevalent is the lesion
of the midbrain extending to the basal ganglia or internal
capsules (Kermode et al., 1989; Morrissey et al., 1993;

2191

Wechsler et al., 1993; Coban et al., 1996; Tali et al., 1997).

In acute lesions there may be oedema, and enhancement is
present in the majority of cases (Kazui et al., 1991; Tali
et al., 1997). T1-weighted hypointensity is seen in chronic
lesions. There is a close clinicalradiological correlation
(Morrissey et al., 1993; Wechsler et al., 1993; Akman-Demir
et al., 1998). A striking feature noted by many (Kermode
et al., 1989; Al-Kawi et al., 1991; Nussell et al., 1991;
Gerber et al., 1996; Akman-Demir et al., 1998) is that T2weighted white matter lesions reduce in size markedly in
conjunction with clinical recovery and often become invisible,
at least on low-strength magnets (Kermode et al., 1989; Tali
et al., 1997). Brainstem atrophy may be striking, as evident
in several of our cases (Fig. 2) and noted by others (Morrissey
et al., 1993; Wechsler et al., 1993; Coban et al., 1996).
The largest published study involved 15 patients with
parenchymal involvement; atrophy was seen in three cases,
involvement of the brainstem and basal ganglia alone in eight
cases, and in three further cases periventricular lesions were
seen in addition to brainstem lesions. MRIs were normal in
two cases, scanned at least 11 months after resolution of the
clinical attack (Wechsler et al., 1993). In a further 10 patients
with cerebral vein thrombosis, MRI of the brain was normal
and the imaging abnormality was restricted to the venous
system. In a further five cases with headache alone without
intracranial hypertension, three of whom had CSF pleocytosis,
MRI was normal. More recently (Akman-Demir et al., 1998),
a study of a series of 59 patients with brainstem involvement
and 14 with intracranial hypertension has shown similar
findings.
Magnetic resonance spectroscopy investigations have been
published for only three cases (Nussel et al., 1991), in
which a reduced NAA/Cr (N-acetyl aspartate/creatine 1
phosphocreatine) ratio was seen in the acute phase, which
subsequently normalized following clinical recovery; in all
three cases substantial radiological recovery had occurred.
No long-term or systematic studies have been published.
Few electrophysiological studies have been published
(Nakamura et al., 1980; Besana et al., 1989; Rizzo et al.,
1989; Stigsby et al., 1994); abnormalities of brainstem
auditory evoked potentials are most frequent, and are more
prevalent in patients in acute attacks than in patients studied
in remission. Visual evoked potential abnormalities are also
common in other series, although not in ours; this is interesting
in view of the fact that the optic nerve is involved clinically
in a small minority of cases (Colvard et al., 1977; Kansu
et al., 1989). Characteristically, recordings show reduced
amplitude with little or no prolongation in latency. This is in
contrast to that of demyelination, in which it is characteristic
to see prolonged latency with temporal dispersion but normal
amplitude (Halliday et al., 1972). Visual evoked potential
abnormalities occurred in patients without uveitis with
frequency equal to that in patients who had had ocular
involvement (Stigsby et al., 1994). A study of central motor
conduction times in 20 patients, 13 of whom had neurological
symptoms and signs, has shown that abnormalities comprising

2192

D. Kidd et al.

mild conduction slowing and reduction in amplitude were

present in half of those with abnormal neurological signs,
and also arose in those without signs (Parisi et al., 1996). In
our series the prevalence of evoked potential abnormalities
was low (two out of 12 patients in whom data were available).
There has been no prospective placebo-controlled trial of
any form of treatment in Behcets syndrome with neurological
complications. Anecdotal evidence has pressed forward the
opinion that use of corticosteroids is mandatory in acute
attacks and possibly thereafter. Intravenous methylprednisolone is often given in acute attacks. Other forms of
immunosuppressive treatment, such as azathioprine, cyclophosphamide, chlorambucil and cyclosporin A, have been
advocated as maintenance therapy (Serdaroglu, 1998).
Colchicine is thought to act by inhibiting neutrophil
chemotaxis (Matsumara and Mizushima, 1975). For adequate
treatment trials one must turn to the ophthalmologists, who
have shown clearly that the use of cyclosporin A in uveitis
results in an alteration of the visual prognosis in this
complication of the disease (Graham et al., 1985; Masuda
et al., 1989). More recently, reports of beneficial effects on
systemic features of the disease with tacrolimus (Ishioka
et al., 1994; Sakane et al., 1995) and interferon -2a (Alpsoy
et al., 1994; Azizlerli et al., 1996) have been published.
Treatment for dural venous sinus thrombosis involves
anticoagulation; Wechsler and colleagues advocate the
concurrent use of corticosteroids (Wechsler et al., 1992),
although again this has not been established by means of a
prospective clinical trial.
In summary, this clinical series of patients with Behcets
syndrome with neurological complications has shown and
confirmed that, in general, patients develop subacute episodes
of neurological dysfunction predominantly situated within
the brainstem, but also the hemisphere and the spinal cord.
Involvement of the optic nerve was seen, but is exceedingly
rare; involvement of the spinal roots, peripheral nerves and
muscle is also rare and was not seen in this series. MRI reveals
lesions that enhance and show close clinicalradiological
correlation. Evidence for lesion dissemination is uncommon,
in contrast to multiple sclerosis. The spinal fluid shows a
mixed pleocytosis with predominance of lymphocytes but
also neutrophils, and intrathecal synthesis of immunoglobulin
is not or very rarely seen. The majority of patients do not
undergo relapses, but those who do have a poor prognosis
for the development of fixed impairments and disability, and
a proportion develop a progressive course with accumulating
disability. No formal trial of treatment in this disorder has
been published, and there is now an urgent need to do so
through multicentre clinical trials.

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Received February 8, 1999. Revised June 6, 1999.

Accepted June 14, 1999