[Cerebral vasculitis secondary to Varicella-Zoster virus infection]. / Vasculite cérébrale secondaire à une infection à VZV.

INTRODUCTION: Central nervous system infection by the varicella-zoster virus (VZV) can be responsible for myelitis, meningitis, ventriculitis and large and small-vessels encephalitis. CASE REPORT: We report the case of a 57-year-old-man hospitalized for deteriorating general health. Physical examination revealed likely encephalitis associated with headache without meningeal syndrome. Successive cerebral MRIs showed bilateral necrosis of the amygdaloid bodies and multiple deep and sub-cortical infarcts suggestive of vasculitis. Cerebral arteriography was normal. Three cerebral fluid examinations disclosed mononuclear pleiocytosis with few red blood cells. PCR analysis for VZV was only positive at the third time. DISCUSSION: The diagnosis of VZV encephalitis is difficult without the rash typical of zoster and because of the low sensitivity of PCR VZV in comparison with PCR HSV. CONCLUSION: In active viral disease, where the prognosis depends on early treatment, we highlight the usefulness of repeated PCR analysis and the search for antibodies in blood and cerebrospinal fluid.

[Carcinomatous meningitis presenting with isolated headache]. / Céphalées isolées révélatrices d'une méningite carcinomateuse.

INTRODUCTION: Carcinomatous meningitis reveals a solid cancer in 10 percent of cases. OBSERVATION: Our patient developed isolated headache which progressively worsened. Cranial Computerized Tomography (CT) was normal. Brain MRI showed multiples areas of contrast enhancements meningeal tissue associated with small nodulars deposits. Repeated cerebrospinal fluid (CSF) examinations revealed elevated tumor markers suspect cells. The diagnosis of pulmonary adenocarcinoma was established during systematic follow-up. CONCLUSION: The diagnosis of carcinomatous meningitis can be difficult to establish because of the non-specific clinical presentation and the absence of suggestive context; negative CSF-cytology is frequent. MRI and elevated tumor markers in the CSF compared with the serum level contribute significantly to diagnosis.

Treatment of progressive forms of multiple sclerosis by cyclophosphamide: a cohort study of 490 patients.

There are no generally effective disease-modifying drugs for progressive forms of multiple sclerosis (MS). Some MS centres use cyclophosphamide (CYC) in secondary progressive (SP) forms of MS, especially after interferon beta-1b (INFbeta-1b) treatment failure. Moreover, there are currently no approved drugs for primary progressive (PP) MS. Using the collected data of patients with progressive MS, we studied clinical patterns that predicted a good response to CYC treatment. Secondly, we compared the therapeutic response of SPMS and PPMS patients to the treatment. Data from 490 MS patients were collected. All patients presented an SP (n = 362) or PP (n = 128) form of the disease and 476 had been treated for at least one year with a monthly pulse of CYC associated with methylprednisolone (MP). CYC treatment was justified because of at least a 1-point worsening on the Expanded Disability Status Scale (EDSS) during the previous year. The EDSS score was assessed at baseline and after 6 months (M6) and 12 months (M12) of treatment. After 12 months of CYC treatment, 78.6% of SPMS and 73.5% of PPMS patients had stabilised or had an improved EDSS score. Response to CYC was not significantly different in the two progressive forms of MS. Twenty-two patients presented noticeable drug side effects, one of whom withdrew from the treatment due to intolerance. Patients with an improved EDSS at M12 had a shorter mean progressive time course (5.1 years) than patients who stabilised or worsened (7.1 years) (p = 0.02). We also observed that poor responders at M6 were also poor responders at M12 (p < 0.001). This large cohort study showed that CYC treatment was well tolerated and suggested that a better response occurred in cases with a short progressive time course. We did not find any difference in treatment response between the two progressive forms of MS. To date, no treatment is approved for PPMS and we therefore propose a trial to test the use of CYC treatment early in the course of the disease in PPMS patients with disability progression.

[Treatment of progressive multiple sclerosis with cyclophosphamide]. / Traitement des formes progressives de sclérose en plaques par le cyclophosphamide.

Intravenous (IV) cyclophosphamide is currently used in secondary progressive (SP) and Primary progressive (PP) Multiple Sclerosis (MS) but its efficacy remains uncertain. Furthermore, it is necessary to determine which MS should be successfully treated with IV cyclophosphamide. We retrospectively investigated 111 consecutive patients with progressive MS (21 PPMS and 90 SPMS) treated in an open label fashion with IV cyclophosphamide. We analysed clinical data (gender, age, duration of progression, primary versus secondary MS). The treatment response was assessed by EDSS change after 6 months and 1 year of treatment. The annual relapse average decreased from 1.92 before treatment to 0.39 during the treatment. Age and gender did not influence response to therapy. We did not find any difference of response between PPMS and SPMS. Duration of the progressive phase in SPMS was not a predictive factor of efficacy. A better response was noted in SPMS patients with surimposed relapses than in patients without relapses during the year before treatment (p<0.05). Furthermore, the better response in SPMS patients with relapses before treatment suggests that it is necessary to treat when MS is still in an inflammatory stage.

[Sunct syndrome: case report and literature review]. / Sunct syndrome. Un cas.

The case of a woman with short neuralgiform paroxysmal attacks located in orbital-periorbital area and associated with autonomic features of ten years duration is reported. This headache syndrome is compared with trigeminal neuralgia involving the first branch of the nerve. Duration, intensity, spreading of the pain and presence of accompanying ipsilateral vasomotor phenomena may be of help in the differential diagnosis. According to the latest reports, sex distribution which passed from 17 men/2 women to 18/6 and effect of the carbamazepine on pain would not appear to have an effect. Nevertheless other reports are needed to distinguish these two clinical syndromes and to develop an etiological and pathogenesis hypothesis.