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behet 's disease ( bd ) is a heterogeneous , recurrent multisystem inflammatory disorder whose etiology and pathogenesis are fairly unknown . vessels ( particularly veins ) , joints , gastrointestinal system , and central nervous system involvement may also be observed , except for the classic triad of oral aphtaes , genital ulcerations , and uveitis2 ) .
the prevalence of neuro - behet 's disease ( nb ) , presenting two types of involvements as parenchymal and non - parenchymal , is at the rate of 2.2% to 50%3 ) .
findings such as elevated intracranial pressure , behavioral disorders , or isolated headache progressing as a result of aseptic meningitis or venous sinus thrombosis may be experienced in cases with non - parenchymal involvement2 ) .
a neuro - behet 's case with parenchymal involvement has been examined in light of literatures in terms of differentiation from tumoral and demyelinating diseases .
the 25-year - old male patient diagnosed with bd three years ago was admitted to our clinic with complaints of recurrent throbbing headache accompanied by nausea and vomiting for four months .
on the cranial magnetic resonance imaging ( mri ) , a lesion that was thought to be accordant with choroiditis or a tumor with a diameter of one cm was detected near the left lateral ventricle posterior horn and near the left temporal lobe uncus ( fig .
lumbar punction ( lp ) could not performed because the patient did not give permission for an lp .
the patient was then discharged . on the basis of the intracranial mass ( malignancy ? )
diagnosis , a tumor excision was made on the patient who applied at another center .
the pathological examination was consistent with a definite loss of myelin in the lesion via the immuno - histochemical method .
after the operation , intensive oedema areas were observed in the left temporo - parietal , left pons hemisphere , and left mesencephalic hemisphere on the cranial mri ( fig .
2 ) . right hemiparesis and motor aphasia were observed and three weeks later the patient suffered from secondary generalized tonic - clonic seizures .
triple antiepileptic and 1 gr / day methyl prednisolone were given to the patient who suffered persistent seizures and lethargy .
the patient 's seizures were kept partially under control with this treatment , and he was discharged with the recommendation of coming to the controls .
hulusi behet in 1937 as a classic triad of recurrent oral aphtaes , genital ulcerations , and uveitis disease4 ) .
the mean age range of onset of bd is 20 - 30 and its neurological involvement comes out averaging five years after the systemic findings .
it is observed that bd is three to four times more common among males compared to females6 ) . neurological involvement reveals itself primarily in the form of central nervous system ( cns ) involvement , but peripheral nervous system 's or muscles ' involvement is highly rare . a form of a brainstem syndrome , which develops within days , is frequently seen in parenchymal involvement . in general ataxia , dysarthria , hemiparesis , bipolar pyramidal findings , cognitive disorder , and sphincter defect are detected during the examination .
siva et al . reported that headaches in nb disease is at a high rate of 61%7 ) .
the headaches continuing for four months in our patient is thought to be secondary to bd or to intracranial mass accompanied by nausea and vomiting along with persistence and throbbing .
as stated in the literature , parenchymal cns involvement may first occur as a headache and it is always accompanied by neurological findings , but not accompanied in our patient . in a great majority of cases with nb , headache types such as tension - type headache or migraine can be seen as well .
it may even be discussed that systemic bd exacerbations , for example , oral aphthaes , genital ulcerations , and uveitis , trigger these primary headaches among certain patients .
interestingly , about 5 percent of patients suffer from migrainous headache only during the systemic bd exacerbations and their headaches disappear via the treatment of these systemic bd exacerbations rather than the analgesics7 ) . according to the mri images of our patient with headaches , a contrast - enhanced mass - like lesion existed near the left temporal lobe uncus .
it was thought that this image could be either a tumoral mass or indication of parenchymal involvement of nb . on cases with parenchymal involvement of neuro - behet ,
the mri presents lesions that extend characteristically from brain stem or basal ganglia to diencephalic structures and that appear hyperintense on t2 weighted - sections .
the mri also reports the lesions at the center of which a small contrast involvement is seen and that can be unilateral or bilateral .
it appears that these lesions become smaller by treatment and their sparsed milimetric t2 hyperintensities remain1 ) .
nevertheless , it must be kept in mind that there may be metastatic or primary cns tumor in bd .
the pathology result of the material is reported as a nontumoural demyelinating nature after the excisional surgery that is carried out with the diagnosis of tumor epicenter .
the mri presents particularly white - matter lesions among approximately 10% of the patients with nb and it may be really difficult to differentiate these lesions from ms lesions1 ) .
cerebrospinal fluid examination is auxiliary in differentiating parenchymal neuro - behet 's disease from ms in the diagnosis . in general ,
slightly moderate lymphocyte and polymorphonuclear cell proliferation along with moderate concentrations of protein levels ( generally < 100 mg / dl ) are observed , even though this elevation is sometimes perceived as normal .
oligoclonal igg bands are not usually detected in the cerebrospinal fluid , but the igg index may have increased . apart from supporting inflammatory neurological involvement , abnormal cerebrospinal fluid findings point to poorer prognosis and necessitate more aggressive treatment2 ) .
cerebrospinal fluid analysis could not be made in this case , but temporal uncus lesion rather than the periventricular multiple plaques led us to think of vasculitis or a malignate lesion instead of ms .
for the cases encountering difficulties in differential diagnosis , if it is thought that this type of lesion may represent a finding of nb ( demyelinating , in particular ) or if it is considered that a metastatic or primary tumor may occur coincidentally , then it needs to be followed up as to whether this type of lesion is becoming smaller or not , primarily after the systemic treatment of bd . also , it should be borne in mind that stereotactic surgery is the last option vis -  - vis the lesions that do not get smaller or that get slightly bigger .
behet 's disease ( bd ) is an inflammatory systemic disorder with oral and genital ulcers , as well as ophthalmologic and cutaneous symptoms . 
 neurological manifestations in bd represent between 2.2% to 50% of the cases . 
 the 25-year - old male patient , diagnosed with bd three years earlier , was admitted to our clinic with complaints of recurrent headaches . 
 tumor - like - parenchimal involvement was detected on a cranial magnetic resonance imaging . 
 the lesion was removed surgically and then he suffered from right hemiparesis and epilepsy . 
 pathological examination of the lesion noted a demyelinating non - tumoural etiology . 
 a neuro - behet 's case with parenchymal involvement has been examined in light of the literature , in terms of a tumor and a demyelinating disease differential diagnosis .