[Neurological manifestations of Behcet's disease: 22 cases among 170 patients].
OBJECTIVE
To present neurological involvement in Behçet's disease, its prognosis and treatment.


METHODS
Our study was retrospective and was done at Hotel-Dieu Hospital of Beirut between 1980 and 2005. All these patients fulfilled the International Study Group criteria for diagnosis of Behçet's disease.


RESULTS
Neurological involvement was observed in 13% (22/170) of our patients and was more frequent in men (sex-ratio: 1,75). The mean age of onset for Behçet's disease and NeuroBehçet's syndrome was 26+/-6 and 30+/-8 years respectively. Central nervous system involvement was found in 21 patients and peripheral nervous system involvement in one. Meningoencephalitis and/or transverse myelitis were found in 57% (12/21) of cases (in association with brainstem syndrome in 2 of these cases), brainstem syndrome without meningoencephalitis in 5 cases, tumor-like syndrome in 2 cases, repetitive ischemic attacks in 1 case and cerebral venous thrombosis in one. Focal deficits were the major presenting signs (16 cases) and external oculomotor nerve paralysis was observed in 4 patients. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level. CT Scan, performed in 6 patients, was normal in 33% of cases. MRI, performed in 9 patients, was abnormal in 6 and showed abnormal signals distributed over the brainstem and the thalamus in 4, a tumor-like lesion and thrombosis of the left lateral sinus one each. Corticosteroids were usually efficacious but, when used alone, relapse was observed in 31% of patients. One patient who had brainstem syndrome died within 18 months because of a delayed corticosteroid treatment.


CONCLUSIONS
Within central neurological involvement in Behçet's disease, we can individualize 4 clinical aspects: meningoencephalitis (and/or myelitis), brainstem syndrome, tumor-like features and cerebral venous thrombosis. Abnormalities, observed on CT Scan and MRI, by their brainstem localization and their multiplicity, should evoke the diagnosis. Corticosteroids, when prescribed early, are useful and are associated with better prognosis; their association to immunosuppressant agents should be considered in the parenchymatous forms.
Tohmé
Aline
A
Service de médecine interne et de maladies infectieuses, Hôpital Hôtel-Dieu de France, Université Saint Joseph, Faculté de médecine, Beyrouth, Liban. atohme2@inco.com.lb
Koussa
Salam
S
Haddad-Zébouni
Soha
S
El-Rassi
Bassam
B
Ghayad
Elie
E
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001528:Behcet Syndrome / Q000150:complications*; D001921:Brain / Q000473:pathology; D002493:Central Nervous System Diseases / Q000188:drug therapy / Q000209:etiology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy / Q000209:etiology*; D012189:Retrospective Studies; D012737:Sex Factors
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents