Dimethyl fumarate and teriflunomide for multiple sclerosis in a real-life setting: a French retrospective cohort study.

BACKGROUND AND PURPOSE: Dimethyl fumarate (DMF) and teriflunomide are approved oral disease-modifying treatments for relapsing-remitting multiple sclerosis (MS). Phase 3 trials established these agents to be effective and generally well tolerated, although comparative efficacy and discontinuation rates are still unknown. The aim of this study was to assess real-world efficacy and discontinuation of DMF and teriflunomide in patients with relapsing-remitting MS. METHODS: This retrospective observational cohort study was carried out in a French administrative region between March 2014 and July 2017. Patients who were followed by private or hospital neurologists were included. Efficacy and tolerance of the two treatments were assessed and compared by multivariate analysis, considering the duration of MS, annualized relapse rate and Expanded Disability Status Scale score at treatment initiation, treatment duration, type of prescriber and tobacco use. RESULTS: We identified 189 DMF- and 157 teriflunomide-treated patients who had been treated for 22 ± 10 months. After correction for confounders, DMF more efficiently reduced the annualized relapse rate after 2 years than teriflunomide (0.06 vs. 0.21; P = 0.03). DMF-treated patients had more clinical and biological adverse events, resulting in a higher rate of treatment discontinuation (28% vs. 12%, P = 0.03). CONCLUSION: In this retrospective cohort study, DMF demonstrated significantly better efficacy over 2 years than teriflunomide, but tolerance to teriflunomide was better.

[Late onset visual aura associated with a carotid artery stenosis reversible after endarterectomy]. / Migraine avec aura à début tardif résolutive après endartérectomie sur sténose carotide serrée.

BACKGROUND: Visual aura is the most common type of aura. Onset of visual aura occurring for the first time in an elderly person is atypical, even though late-life migraine accompaniments are well-recognized. OBSERVATION: We report the case of a 72-year-old woman with a past history of migraine without aura since adolescence (one attack per month), who experienced for the first time of her life multiple visual aura over a period of more than one year. These auras were always left homonymous hemianopia which developed gradually over 5 mins followed by severe migraine headache without strict lateralization. The patient was known to have a moderate right carotid artery stenosis. In October 2007, she suffered a minor ischemic stroke in the right carotid artery territory due to progression to severe stenosis of the right internal carotid artery. After carotid endarterectomy, she had no further migraine attacks with aura. DISCUSSION: We discuss the mechanism of aura in our case with probable microemboli due to the severe right internal carotid artery stenosis responsible for repeated cortical spreading depression. CONCLUSION: Clinicians should bear in mind to systematically explore all patients with late onset aura including patients who have previously suffered from migraine without aura and also to explore patients who have a dramatic increase of aura even if they are already migrainers with aura.

[Tako-Tsubo syndrome during normal human immunoglobolin perfusion]. / Syndrome Tako-tsubo au décours d'une perfusion d'immunoglobuline humaine normale (Tégeline(®)).

This is a case of an 82 year old female patient with myasthenia gravis, who following treatment with Human Normal Immunoglobulin (Tegeline(®)), developed dyspnoea, chest pain without cardiac insufficiency, inverted T wave on ECG with slight increase in Troponine T 0.43ng/mL (<0.2ng/mL normal value in our hospital) and marked increase in Pro-BNP 4900 (Nl≤450pg/mL for an age greater than 65 years old). Her coronary angiogram showed hypokinesia of apical area but was otherwise normal. Also, MRI ruled out inflammatory and ischemic cardiac diseases. The most likely diagnosis for us was Tako-Tsubo syndrome in relation with injection of Human Normal Immunoglobulin (Tegeline(®)) according to the Mayo clinic criteria.

[Quality of life and multiple sclerosis: validation of the french version of the self-questionnaire (SEP-59)]. / Qualité de vie et sclérose en plaques: validation de la version francophone d'un auto-questionnaire, le SEP-59.

We conducted a prospective study among 166 multiple sclerosis (MS) patients (103 from an university hospital, 63 from a MS rehabilitation center) to assess the properties of the French version of the Multiple Sclerosis Quality Of Life - 54 items (MS QOL-54) which combines the MOS SF36 together with MS specific items. The SF-36 had been translated into French through the IQOLA project. We translated and adapted the MS specific items with the help of three different teams. The translation into French has an addition of five items, because we kept the MS specific items of an earlier unpublished form. Acceptability is excellent with a response rate over 90p.100. Test-retest reliability is good except for the "role limitation-emotional" scale of the SF-36. Construct validity, based on factor analysis, shows no change in the SF-36 internal consistency and the specific items provided their own information. External validity, tested against both medical (Expanded Disability Status Scale, Kurtzke scale, Mini-Mental-State and disease stage) and rehabilitation (Functional Independence Measure) parameters is excellent. The French MS QOL questionnaire contains 59 items including both the SF-36 and the MS QOL-54 items. This will permit international comparisons of MS patients' care and therapy.

[Neurogenic muscle hypertrophy: 3 cases]. / L'hypertrophie musculaire neurogène: 3 cas.

Three cases of neurogenic muscular hypertrophy are reported. First case presented a hypertrophy of one calf following S1 radiculopathy; the second had a hypertrophy of tibialis anterior muscle, five years after a compressive injury of the common peroneal nerve. The third case is a global hypertrophy of leg muscles after intensive care or legionella neuropathy. Previous cases of the literature are reviewed and the various pathophysiological hypothesis are considered. Among these hypothesis, the role of abnormal electrophysiological activities in most previous cases, as well as in ours (namely complex repetitive discharges and fasciculations), seems the most consistent.

[Isolated proximal muscular weakness disclosing myasthenic syndrome]. / Faiblesse musculaire proximale isolée révélatrice d'un syndrome myasthénique.

Three cases of chronic limb-girdle myasthenia gravis (one man and two women) are reported. The duration of the disease was three, five and 20 years respectively. None had oculobulbar weakness during this period nor fluctuating symptoms. The primary diagnosis was myopathy in two cases and myelopathy in the other one. In view of this diagnosis, several features were atypical: absence of marked wasting, preservation of tendon reflexes and normal serum creatine kinase activity. Finally, diagnosis of myasthenia gravis was established by the occurrence of a characteristic decremental muscular response to repetitive stimulation and by positive edrophonium test. It seems that the rate of positivity of acetylcholine receptor antibody is lower than in the common form of the disease. Two patients responded to acetylcholinesterase inhibitors and one to high-doses intravenous human immunoglobulins. These and previous cases described in the literature raise the problem of the definition of an unusual form of myasthenia gravis or of a new type of neuromuscular disease characterized by the presence of distinctive changes on muscular biopsies, specially tubular aggregates as in one of our cases. However, this unusual form of chronic limb-girdle weakness has to be recognized because treatments are effective in most cases.

[Polymyalgia induced by topical minoxidil]. / Etats polyalgiques induits par le minoxidil topique.

Topical minoxidil, used in the treatment of baldness, has been commercially available since 1987. Its systemic side effects are rare. We observed an as yet unreported "polymyalgia syndrome" in four otherwise healthy males whose sole medication was topically applied minoxidil. They experienced fatigue, weight loss and severe pain in the shoulders and pelvic girdle, suggesting connective tissue disease. Three patients had a transient rise in liver enzymes, while other laboratory analyses remained normal. Tritanomaly was detected in two patients who underwent systematic color vision testing. All symptoms disappeared after withdrawal of minoxidil. Rechallenge was positive once in one patient and twice in another. The mechanism of this side effect remains to be determined.