中枢神経症状を伴うBehçet氏症候群—所謂Neuro-Behçet's Syndromeの2例及びその文献的考察

Behçet's Syndrome and its Neuropsychiatric Complications: Socalled "Neuro-Behçet's Syndrome" with some literatural considerations

I．序論

　Behçet氏症候群，即ち，屡々前房蓄膿を伴う虹彩炎と口腔及び外陰部に潰瘍の再発を繰返す症候群に就いては，皮膚科，眼科或は産婦人科医によつて古くから報告されているが，近年，ヴィールス学或はアレルギー学説の発展により，原因不明の本症候群に対する新たな興味が喚起されるに到つた。本症候群は比較的稀有な疾患であるが，時に神経精神科医にとつても甚だ興味深い，重篤な併発症状を示すことがある。吾々は最近，脳腫瘍を疑わせた本症候群の1例と高度の記銘力障害を示した1例とを経験したので，本症候群に於ける中枢神経症状の発生様式を概観する為に，文献上27例を集め，自験例と共に検討を加えてみることにした。

　Two cases of Neuro-Behçet's syndrome were reported in this paper, and the review of the literature concerning history, clinical features, histopathology and etiology of this syndrome was described briefly, too.

　Following are the clinical symptomes and positive findings of author's cases:

　Case 1. : Man, aged 30 : 4 years after the onset, severe headache of left temporal area, nausea, vomiting, dizziness : hypomanic state. Optic edema ; cerebellar sign (+); C.S.F., pressure 430mmH₂O, cell count 12/3, protein reaction (+); E.E.G., diffuse dysrhythmia.

　Admitted for "brain tumor" to be suspected. Left internal hydrocephalus on pneumoencephalogramm, and normal angiographic findings.

　Recovered within two months.

　Case 2. : Man, aged 32 : 10 years after the onset, sudden attacks of severe headache (duration ; about 2 hours) and hyperaesthesia of both legs (duration ; about 30 minutes); severe fatigue and impairment of retention ; optic atrophy ; E.E.G., organic brain damage suspected.

　Admitted in the Ophthalmological clinic because of eye symptomes.

　From author's own 2 and 27 cases previously reported with neurological complications, whichwere selected from 107 cases of Behçet's syndrome found in the literature, the following neuropsychiatric patterns in Behçet's syndrome were established :

　I. Inflammatory or Meningoencephalomyelitic type

　a) meningitic type

　b) encephalitic type

　c) mixed or intermediate type

　( 1 ) meningoencephalitic type

　( 2 ) encephalomyelitic type

　II. Noninflammatory type

　A. Localized or intracranial tumor type

　a) brain stem type

　b) confusional or psychotic type

　c) mixed or intermediate type

　B. Generalized or disseminated sclerosis type