Clinicopathologic features and outcomes of neuro-Behçet disease in Spain: a study of 20 patients.

BACKGROUND: To describe the clinical characteristics and evolution of a series of adult patients hospitalized for neuro-Behçet disease (NBD). METHODS: Consecutive patients admitted for NBD in a teaching hospital were retrospectively selected. Disability at discharge and during follow-up was graded with the modified Rankin Scale, and outcome classified as good or poor (grades 3-6). RESULTS: Twenty patients were included (M/F, 13/7). Mean age at NBD diagnosis was 36.3 years. Nineteen patients had other manifestations of Behçet disease (BD) before NBD developed, but only 7 met the complete diagnostic criteria for BD. Fever, headache, motor weakness, and cranial nerve palsy were each present in approximately 60% of patients. There was a low prevalence of behavioral changes (5%), seizures (5%), and sphincter incontinence (0%), and a relatively high prevalence of meningism (25%). Non-neurologic manifestations of BD were concurrently detected in 15 patients (75%). 80% had parenchymal involvement. Brain biopsies during 5 attacks showed perivascular lymphocytic infiltration with reactive astrocytosis, but no frank vasculitis. During a mean follow-up of 6.3 years per patient, 12 had at least one relapse. In total, there were 22 relapses; all but two were in the same location and were symptomatically similar in each patient. At the end of follow-up, 7 patients (35%) had a poor outcome, including 4 who died. CONCLUSION: Recording of previous manifestations of BD and a physical examination to detect concomitant systemic manifestations of BD may help establish an early diagnosis of NBD. Relapses frequently occurred in the same location. No frank vasculitis was present in brain biopsies.

Bilateral carotid and bilateral vertebral artery dissection following facial massage.

A 50-year-old woman underwent facial massage. After 13 days, she experienced left retro-orbital pain, ptosis, and miosis. Magnetic resonance imaging (MRI) showed stenotic dissection of bilateral cervical internal carotid and vertebral arteries. The intracranial vasculature was intact. She was treated conservatively with long-term oral anticoagulation and remains asymptomatic 18 months later.

An analysis of the etiology of cervical artery dissections: 1994 to 2003.

OBJECTIVE: To provide a literature review of the etiologic breakdown of cervical artery dissections. METHODS: A literature search of the MEDLINE database was conducted for English-language articles published from 1994 to 2003 using the search terms cervical artery dissection (CAD), vertebral artery dissection, and internal carotid artery dissection. Articles were selected for inclusion only if they incorporated a minimum of 5 case reports of CAD and contained sufficient information to ascertain a plausible etiology. RESULTS: One thousand fourteen citations were identified; 20 met the selection criteria. There were 606 CAD cases reported in these studies; 321 (54%) were internal carotid artery dissection and 253 (46%) were vertebral artery dissection, not including cases with both. Three hundred seventy-one (61%) were classified as spontaneous, 178 (30%) were associated with trauma/trivial trauma, and 53 (9%) were associated with cervical spinal manipulation. If one apparently biased study is dropped from the data pool, the percentage of CADs related to cervical spinal manipulation drops to approximately 6%. CONCLUSIONS: The case series that were reviewed in this article indicated that most CADs reported in the previous decade were spontaneous but that some were associated with trauma/trivial trauma, and a minority with cervical spine manipulation. This etiologic breakdown of CAD does not differ significantly from what has been portrayed by most other authors.

Cervical artery dissection--clinical features, risk factors, therapy and outcome in 126 patients.

The highly variable clinical course of cervical artery dissections still poses a major challenge to the treating physician. This study was conducted (1) to describe the differences in clinical and angiographic presentation of patients with carotid and vertebral artery dissections (CAD, VAD), (2) to define the circumstances that are related to bilateral arterial dissections, and (3) to determine factors that predict a poor outcome. Retrospectively and by standardised interview, we studied 126 patients with cervical artery dissections. Preceding traumata, vascular risk factors, presenting local and ischemic symptoms, and patient-outcome were evaluated. Patients with CAD presented more often with a partial Horner's syndrome and had a higher prevalence of fibromuscular dysplasia than patients with VAD. Patients with VAD complained more often of neck pain, more frequently reported a preceding chiropractic manipulation and had a higher incidence of bilateral dissections than patients with CAD. Bilateral VAD was significantly related to a preceding chiropractic manipulation. Multivariate analysis showed that the variables stroke and arterial occlusion were the only independent factors associated with a poor outcome. This study emphasises the potential dangers of chiropractic manipulation of the cervical spine. Probably owing to the systematic use of forceful neck-rotation to both sides, this treatment was significantly associated with bilateral VAD. Patients with dissection-related cervical artery occlusion had a significantly increased risk of suffering a disabling stroke.