الفهرس 
Behest’s sy" ~ "drome
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Abstract
I’he Aim of the ·!..o.£J!;:
Ir~ - -~.
(: j ’
Tor the pa•.
deter:n.:na’”i··• ·.)f G.S.:F·. Lo
’ g A’ ~d th - I ”~ ,,
were d<)ne. to .-=:tnswe.!”
fied as 1 Jllows:
l- Ten Y-f~·:r~J-Be L’~ .-,f ’-”’ __ ·--”.
- .. lfestation~’<.
MG thods:
A- C.Li n aE--: , .
’ 3. c_- -, ’
1- . r 61’:
i. le’ :.\l St’ ·u.rr.Results of t:Ic -~ _inieal assessment:
Ten neuro-Behcet 1 s c·~ses were examined. The
average age at the onset was 25.5 year, and the total
duratianof the disease averaged 4.05 years. Out of the ten,
two,do not fulfilled th& three clinical diagnostic oriteria
of Curth (1965), but all the ten patients fulfilled the
clinica~ ,_:iagnostic criteria of Fadli and Youssef (1973).
The time between the first manifestation and the onset
of neurologic lesion averaged 2.1 year. Two patients
presented with neurological manifestations at the onset
of the disease. ’Lhe duratior, of neurological affection
averaged 1.9 year. The corticospinal tracts were most
c01mnonly involved (five patients). Six patients showed
visual defects. Out of ten neuro-Behcet 1 s cases, three
showed E.E.G. changes. The comnon p<’.ttern for those
three patients was mild generalised theta slow wavesmoderate ri.ght hemispherical cf;eta wave iy:o<rythmta.
Thrombotic tendency was (25%).
2) Results of immunologic study:
Quantitative c.s.F. and serum immunoglobulins disturbances
in the form of polyclonal ga.mmopathy state but
it is not truly polyclonal. C. s.F. measurable Ii~ was
present in two cases only with recent neurological manifestations.
Complement C3 activation is via the classic
pathway.
J) Haemostatic study showed platelet hyperactivity diminished.
fibrinolytic mechanism and normal coagulation
mechanism.
4) Lipid study showed, normal level. of s ’3rum total lipid
and c ”10lesterol, and increased beta-lipoproteins.
Conclusions:
l- An autoimmune etiology for Behest’s syndrome was suggested
from the results ,; thG immunologic study and
the study of the fibrinolytic mechanism.
2- A virus agent ftS a cause for this autoimmunity was
suggested from the resu1 ts of C.S.F. immunoglobulin
study.Hypothetically, <6netif’ factors i.n C··~~bine’”i’m ’>~irh
a virc1s was sup;,;ested to ln; tiate r.tl teration of the
immun.e system in 3ehcet’s syndrome.
4- A separate autoimnune process occurring in the nervous
system independent of other system affection, was
suggested by the raised possibility of local c.s.F.
antibody production.
5- Complement component CJ is activated via the classic
pathway.
6- The results of the haematologic study indicate that,
the t~rombotic tendency in Behest’s syndrome is a
sequence of the disease process and not the primary
etiologic factor.
7- Immune complexes cause basement membrane injury with
subsequent platelet hyperactivity which play the
primary role in the pathogenesis of the thrombotic
manifestations. The site of action is the basement
membrane and the main part of the vascular tissue
affected is the ~icrocirculation.
8- Diminished fibrinolytic activity is due to diminished
activator activity.
:9- It seems that the main role of increased beta-lipoproteins
level, in the pathogenesis of Behcet’s syndrome
is the enhancement of the thrombotic tendency
through its sffect on platelet activity and fibrino10.·- NGurol·)iC:i<”q} ,,ffectioc:J Jr activr•d:i ’!l can be known by
quantitative C.S.i. imlnunoglc·C)~J-lins determination.
ll- Complete haematologic study especially for platelet
function and fibrinolytic mechanism, is essential to
decide whether the disease is generally active or not.
R!icommende.tions:
l- We can depend on the laboratory data investigated in
this study with one or more of the clinical diagnostic
criteria to se;y that there is Behest’s syndrome or not.
2- Cur laboratory data suggestea an autoimmune pathogenesis
possibly due to virus infection.Also there ~s separ~te
autoimmune process occurring in the nervous system independent
of other system affection. However to complete
these evidences for Behcet’s syndrome pathogenesis
we have to do:
a- Future virologic studies which should include the
new techniqLtes demonstrating slow viruses.
b- Searching for C.S.F. inmunologically competent
lymphocytes as a further evidence for local c.s.F.
immunoglobulins production.
c- Searching for serum autoantibodies and amyloid indicating
deposition of denatured gamma-globulin.
d- Irrnunofluorescent study of tissue biopsy and also
LateA test, to detect immune complexes.adviced to us0 anotr,ar method t,, dsterminG thG
active OJ component only. Also the time course
variation must be adjusted befo:c:e taking the serum
samples. Determination of 01 and 04- to give furq
ther support to the activ2.tion of OJ by the classic
pathway is recommended,
f- Controlled genetic study.
3- Haematologic investigations especially for platelet
function and fibrinolytic mechanism are essential to
know whether the disease is generally active or not,
4- To diagnose neuro-Behcet 1 s and to see if the disease
is neurologically active or not, it is recommended to
do complete O.S.F. study especially quantitative immunogl:
Jbulins determinations.
5- Higher incidence of venous thrombosis showed the importance
of early and even prophylactic management.
Our data indicates that the thrombotic tendency is a
sequence of the disease process itself, so our attention
in man8ging these tendencies must be directed
towards the original pe. thoge.’1esis. Als:> it is recommended
to do repeated platelet function study during
the course of treatment to have a good idea about the
progress of the disease and to treat early haemostaticbo tic ma . ni f sstr. tt ems.
6- ’I’reatment trials is &dviced to be done in a C•Jntrolled
collaborative stu_dy in many cent.r6So rl’h6SG lines ccf
treatment are reco~~ended:
a) Antiviral drugs together with the uddi tive effect
of prednisone and cytotoxic agents.
b) Drugs that reduce platelet hyperactivity and drugs
or agents that enhanced the fibrinolytic activity.
7- Lastlywecan say BEr”cet’s disease instead of Behcet•s
syndrome. Now we do not depend only on the clinical
picture to diagnose Behest’s disease. We have the
Lab. data. which we can d spend on it togs ther with one
or more of the clinical criteria to diagnose Behcet•s
disec.se.