Lyme borreliosis and other tick-borne diseases. Guidelines from the French scientific societies (II). Biological diagnosis, treatment, persistent symptoms after documented or suspected Lyme borreliosis.

The serodiagnosis of Lyme borreliosis is based on a two-tier strategy: a screening test using an immunoenzymatic technique (ELISA), followed if positive by a confirmatory test with a western blot technique for its better specificity. Lyme serology has poor sensitivity (30-40%) for erythema migrans and should not be performed. The seroconversion occurs after approximately 6 weeks, with IgG detection (sensitivity and specificity both>90%). Serological follow-up is not recommended as therapeutic success is defined by clinical criteria only. For neuroborreliosis, it is recommended to simultaneously perform ELISA tests in samples of blood and cerebrospinal fluid to test for intrathecal synthesis of Lyme antibodies. Given the continuum between early localized and disseminated borreliosis, and the efficacy of doxycycline for the treatment of neuroborreliosis, doxycycline is preferred as the first-line regimen of erythema migrans (duration, 14 days; alternative: amoxicillin) and neuroborreliosis (duration, 14 days if early, 21 days if late; alternative: ceftriaxone). Treatment of articular manifestations of Lyme borreliosis is based on doxycycline, ceftriaxone, or amoxicillin for 28 days. Patients with persistent symptoms after appropriate treatment of Lyme borreliosis should not be prescribed repeated or prolonged antibacterial treatment. Some patients present with persistent and pleomorphic symptoms after documented or suspected Lyme borreliosis. Another condition is eventually diagnosed in 80% of them.

Lyme borreliosis and other tick-borne diseases. Guidelines from the French Scientific Societies (I): prevention, epidemiology, diagnosis.

Lyme borreliosis is transmitted en France by the tick Ixodes ricinus, endemic in metropolitan France. In the absence of vaccine licensed for use in humans, primary prevention mostly relies on mechanical protection (clothes covering most parts of the body) that may be completed by chemical protection (repulsives). Secondary prevention relies on early detection of ticks after exposure, and mechanical extraction. There is currently no situation in France when prophylactic antibiotics would be recommended. The incidence of Lyme borreliosis in France, estimated through a network of general practitioners (réseau Sentinelles), and nationwide coding system for hospital stays, has not significantly changed between 2009 and 2017, with a mean incidence estimated at 53 cases/100,000 inhabitants/year, leading to 1.3 hospital admission/100,000 inhabitants/year. Other tick-borne diseases are much more seldom in France: tick-borne encephalitis (around 20 cases/year), spotted-fever rickettsiosis (primarily mediterranean spotted fever, around 10 cases/year), tularemia (50-100 cases/year, of which 20% are transmitted by ticks), human granulocytic anaplasmosis (<10 cases/year), and babesiosis (<5 cases/year). The main circumstances of diagnosis for Lyme borreliosis are cutaneous manifestations (primarily erythema migrans, much more rarely borrelial lymphocytoma and atrophic chronic acrodermatitis), neurological (<15% of cases, mostly meningoradiculitis and cranial nerve palsy, especially facial nerve) and rheumatologic (mostly knee monoarthritis, with recurrences). Cardiac and ophtalmologic manifestations are very rarely encountered.

Aseptic meningitis and ischaemic stroke in Fabry disease.

BACKGROUND: Fabry disease (OMIM 301 500) is an X-linked lysosomal storage disease. Neurological symptoms in Fabry disease mainly include stroke, acroparesthesia, cranial nerve palsies and autonomic dysfunction. We report on aseptic meningitis in Fabry patients. METHODS: Clinical analysis, brain magnetic resonance imaging, cerebrospinal fluid analysis, treatment and outcome data were analysed in three cases of meningitis associated with Fabry disease. FINDINGS: Mean age at meningitis onset was 26.6 (24-28) years. Headache was present in all cases and fever in two cases. Meningitis was always diagnosed before Fabry disease. A familial history of Fabry disease was present in two cases. Non-neurological symptoms caused by Fabry disease were present in all cases. All patients also suffered stroke and sensorineural hearing loss. Cerebrospinal fluid (CSF) analysis showed pleocytosis (mean, 36; range: 8-76 cells/mm(3)) and a high protein level (mean, 63; range, 47-70 mg/dl). C-reactive protein blood levels and erythrocyte sedimentation rate were raised. Diagnosis was assessed by low alpha-galactosidase A dosage and/or gene mutation analysis in all cases. All patients were treated with enzyme replacement therapy (ERT). In two cases, lumbar puncture was repeatedly performed and there was no normalisation of CSF under ERT alone, at 9 and 24 months of follow-up, respectively. One patient who suffered intracranial hypertension was treated efficiently with steroids, associated with azathioprine. The fact that Fabry disease could be an auto-inflammatory disorder is discussed. INTERPRETATION: Fabry disease may cause aseptic meningitis.

[Acute myelitis and Lyme disease]. / Myélite aiguë et neuroborréliose.

INTRODUCTION: Acute myelitis accounts for 4 to 5 percent of all cases of neuroborreliosis. In the literature, simultaneous spinal MRI and cerebrospinal fluid (CSF) investigations are presented for only 8 cases. We describe here 3 cases of acute Lyme myelitis. METHOD: In a cohort of 45 patients with neuroborreliosis, diagnosed between January 1998 and January 2005, 3 had acute myelitis. Clinical, biological and radiological data were studied. CASE REPORTS: The three patients had motor, sensorial and sphincter involvement. Extra-spinal involvement, such as fever and headache for one, facial nerve palsy for the second and subarachnoid hemorrhage for the third, was also noted. Pleocytosis varied from 10 to 520 white cells per mm3. Lyme serology was positive in CSF for all. Intrathecal anti-Borrelia antibody index was positive or intermediate for all three patients. Spinal cord MRI revealed a large hyperintense zone involving more than 3 vertebral segments. Myelitis was central, posterior or transverse in the axial plane. The clinical course was favorable after a three-week course of appropriate antibiotics. CONCLUSION: These 3 cases and the others from the literature show the diversity of the clinical and radiological features of acute myelitis: transverse, central or posterior myelitis. Thus, Lyme serology in CSF in indicated for patients presenting acute myelitis, particularly in endemic areas.

[Patterns of prescription and usage of triptans in Alsace (France): misuse is frequent and avoidable]. / Modes de prescription et de consommation des triptans en Alsace: un mésusage fréquent mais évitable.

AIMS: To analyze use of triptans in the Alsace region of France: patients, disorders motivating, doses, analgesics and migraine prophylactics associated treatments, contra-indications. To study major consumers (more than 144 intakes per year) and to determine among them the proportion who suffering from chronic headache. METHOD: Data concerning all prescriptions of triptans and analgesics as well as migraine prophylaxis prescriptions were obtained from the computer databases of five of the French National Health's local health agencies in Alsace, recorded between April 1, 2003 and March 31, 2004. Data about motivating disorders and the clinical context were obtained using a questionnaire sent to prescribers. Data about patients with more than 144 intakes per year were provided by medical advisors of French Health insurance. RESULTS: We founded 20686 users: 92.1 percent used between 0 and 6 intakes per month. 11.5 percent of disorders motivating the prescription that were mentioned by prescribers were for off-label use: tension-type headache 2.7 percent, mixed headache, 8.8 percent. Prescribers declared at least one contra-indication for triptan use for 7.8 percent of patients. Over all, prescriptions were off-label for 16.1 percent of patients. Patients who used more than 144 intakes per year accounted for 1.9 percent of the total number and self-medication accounted for 19.2 percent of all triptan intakes. Half of the patients were suffering from daily chronic headache (chronic migraine in 66 percent). 15.6 percent of these patients presented at least one contraindication (high blood pressure or ischemic disease). All in all we estimate that use of triptan is a misuse for 25 percent to 30 percent of the intakes. Quantities of other analgesics used increased simultaneously with triptan use: on average 65, 119 and 244 Defined Daily Doses (DDD)/person/year for patients who used between 1 and 72, 73 and 144 and more than 144 intakes respectively. On average 35.4 percent (in DDD) of analgesics used were opiates (dextropropoxyphene, codeine, tramadol). This proportion increased simultaneously with triptan use: 58.9 percent for major users. Prophylactic treatment for migraine was used by 27.9 percent of the patients: lack of prophylaxis was a prescriber's choice in 90 percent of the cases. CONCLUSIONS: The high rate of triptan misuse emphasizes the importance of improving prescription of these drugs.

[Intravenous immunoglobulins in neurology]. / Immunoglobulines intravenineuses en neurologie.

Immunoglobulins, used at first empirically in the treatment of thrombocytopenic purpura, occupy a prominent place not only in the treatment of antibody deficiencies, but also in that of antoimmune diseases. Their indications in neurology are ever extending; they include myasthenia, chronic inflammatory polyneuropathies with or without monoclonal gammopathy, polymyositis, dermatomyositis and, more recently, disseminated sclerosis and Guillain-Barré syndrome. Even the therapeutic priority of plasmapheresis in this syndrome is disputed by some authors. Immunoglobulins are costly, but they are well tolerated and easy to use. Their effectiveness must be confirmed by controlled, double-blind trials. In neurology such trials are still rare, but those recently published are devoid of methodological errors. Once the effectiveness of immunoglobulins is confirmed, their dosage must be established, and attempts should be made at a better understanding of their mechanisms of action.