LETTER TO THE EDITOR
Pitfalls in the diagnosis of Neuro-Behcet presentingwith psychiatric symptoms at onset: a case report!
Rocco Salvatore Calabro • Rosario Grugno •
Nunzio Muscara • Placido Bramanti
Received: 12 October 2012 / Accepted: 9 January 2013
� Springer-Verlag Italia 2013
Keywords Neuro-psycho-Behcet � Somatoform
disorders � Limbic system
Dear Sir,
Behcet’s Disease (BD) is a multi-system, vascular-inflam-
matory disease of unknown origin, characterized by
recurrent oral and genital ulcerations and uveitis with
hypopyon, and involving the nervous system in a subgroup
of patients. The majority of Neuro-Behcet’s (NBD) cases
can be characterized as a vascular-inflammatory central
nervous system (CNS) disease, which commonly presents
with the onset of a subacute brainstem syndrome, unilateral
or bilateral cortico-spinal tract signs, cerebellar findings
and mild confusion with or without disturbance in con-
sciousness [1]. Nevertheless, hemiparesis, cognitive,
behavioural and emotional disturbances, extrapyramidal
signs and seizures may be observed. However, neuropsy-
chiatric symptoms at onset are very rare, often leading to
misdiagnosis.
An otherwise healthy 31-year-old woman had behav-
ioural disturbances, excessive anxiety, weakness, and loss
of appetite with some episode of visual and auditory hal-
lucinations for about 3 months. Physical and neurological
examination, as well as EEG and brain CT were normal.
The severity of psychotic symptoms was assessed using the
Brief Psychiatric Rating Scale (BPRS). As somatoform
disorder was diagnosed, venlafaxine (75 mg/day) and
amisulpiride (50 mg/day) prescribed. Although psychiatric
symptoms ameliorated (BPRS score from 56 to 26) within
3 weeks, the patient, after around 2 months of treatment,
showed upper limb weakness and paresthesias, and thus
underwent a more specific evaluation. General examina-
tion, including ophthalmological exam, was normal while
neurological examination showed a mild hemiparesis. MR
flair images showed hyperintensities in the right amygdala
(see Fig. 1) and cingulate anterior gyrus and the left pos-
terior limb of the internal capsule. Laboratory tests,
including common autoantibodies (i.e. lupus anticoagulant,
anticardiolipin and antiphosphatidylserine antibodies,
antibeta 2 glycoprotein I, anti-nuclear, anti-smooth muscle
and anti-neutrophil cytoplasm antibodies, extractable
nuclear antigens), VES, PCR and coagulation (i.e. anti-
thrombin III, protein S and C), urinalyses and cerebrospinal
fluid examination did not reveal any abnormalities.
Since the most common alternative causes of such brain
parenchymal lesion have been ruled out (i.e. CNS infec-
tions with regards to tubercolosis, syphilis and Lyme bor-
reliosis, sarcoidosis, systemic and brain immune disorders,
including the anti-NMDA receptor Encephalitis) and
pathergy test was positive, NBD was suspected (although
the B51 HLA genotype was not detected) and the patient
treated with colchicine (1 mg/day) and antiplatelets (aspi-
rin 100 mg/day). No other brain MRI lesions were identi-
fied at 2-year follow-up.
However, during the follow-up period the patient pre-
sented with recurrent genital ulcerations, further confirm-
ing our hypothesis.
Because neurological involvement usually occurs
1–10 years after the first symptom of BD and because the
first symptom of BD is neurological/psychiatric in only
3 % of cases, NBD is sometimes very difficult to diagnose
[2]. Indeed, our patient has been misdiagnosed as affected
by somatoform disorder since, at the onset of the disease,
she presented with unspecific psychiatric symptoms and
R. S. Calabro (&) � R. Grugno � N. Muscara � P. Bramanti
IRCCS Centro Neurolesi ‘‘Bonino-Pulejo’’, S.S. 113,
Contrada Casazza, 98124 Messina, Italy
e-mail: [email protected]
123
Neurol Sci
DOI 10.1007/s10072-013-1304-1
CT was also negative. Moreover, she had no oral or genital
ulcerations, eye or skin lesions, leading to the proper
diagnosis. The diagnosis of neurological involvement in
BD is done mainly by clinical means since no specific test
for NBD, to date, exist, although an association with HLA
type B51 has been reported (in 60–70 % of Turkish and
Japanese patients and only 10–20 % of Europeans). Also
brain MRI lesions are not specific, although the involve-
ment of the upper brainstem, thalamus and basal ganglia is
more common [2].
To the best of our knowledge, only a few cases of NBD
presenting with psychiatric symptoms has been so far
described [3–5].
In the case report by Aydin et al. [4], Behcet’s disease
presented with affective symptoms, which recovered after
corticosteroid therapy, while Nakano et al. [5] described a
patient affected by Neuro-Behcet’s disease with early onset
of bipolar mood disorder.
Interestingly, both the patients’ brain MRI showed clear
swelling of the brain stem area, especially in the pons,
confirming the important role of the brain stem aminergic
nuclei in determining mood and anxiety disorders.
On the other hand, in the report by Deniz et al. [3], the
authors have hypothesized that their patient psychotic
symptoms could be due to the involvement of the limbic
system, as in our case. Indeed, the limbic system, including
the amygdala, has long been implicated in the pathogenesis of
psychotic spectrum disorders as well as mood disorders. Both
positive and negative symptoms can be attributed to functions
supported by the limbic system or its networks. The limbic
system has extensive afferent and efferent projections to
multiple brain regions, and it has been supposed that the
significant and selective disruption of fronto-temporal
connectivity is sufficient to determine a phenocopy of
schizophrenia [6].
In conclusion, with our report we want to underline the
importance of considering NBD as a possible factor in psy-
chiatric symptom when an organic aetiology is suspected.
References
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Fig. 1 A T2-hyperintense lesion in the right amygdala
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