A longitudinal observational study of a cohort of patients with relapsing-remitting multiple sclerosis treated with glatiramer acetate.

Immunomodulatory treatments for relapsing-remitting multiple sclerosis (RRMS) are not efficacious or tolerated in all patients. It is important to evaluate alternative classes of treatment in patients failing first-line therapy. The objective of this prospective observational study was to evaluate the efficacy and safety of glatiramer acetate in patients, to whom beta-interferons could not be administered. The study included patients with RRMS who were intolerant to or had contraindications to beta-interferon. After initiation of glatiramer acetate treatment, follow-up visits were made every 3 months, when data on neurologist-ascertained relapses and disability [Expanded Disability Status Scale (EDSS) score] were collected. Tolerability was evaluated by spontaneous adverse event reporting. Overall, 205 patients were studied and 113 (55.1%) treated for at least 4 years. The proportion of patients presenting over three relapses per year decreased from 51.2% to 8.4% in the 2 years following treatment initiation. Over 5 years of treatment, mean annualized relapse rates and mean EDSS scores remained stable (0.4-0.6 relapses/year and 3.6 +/- 1.8-3.3 +/- 2.1 respectively). Adverse events were reported by 179 patients, leading to discontinuation of treatment in 10 patients. Patients with RRMS to whom beta-interferons cannot be prescribed can benefit from treatment with glatiramer acetate.

[Pharmaco-epidemiological study on the prophylactic treatment of migraine. National inquiry on attitude to prescription practices by primary care physicians and neurologists in Franceø]. / Etude pharmaco-épidémiologique sur le traitement de fond de la migraine.

A pharmacoepidemiological survey was conducted in order to understand the pattern of migraine prophylactic drug utilization by French physicians. Neurologists and primary care physicians completed a phone-mail-phone questionnaire which inquired about migraine prophylactic treatment. French neurologists and PCP made the same use of migraine prophylaxis in terms of indication, time interval between treatment onset and evaluation, and duration. The two most commonly chosen migraine prophylactic agents were dihydroergotamine and beta-blockers. This study also showed the importance of considering quality of life to evaluate efficacy of migraine prophylaxis.

[Cerebellar imitation synkineses]. / Syncinésies d'imitation cérébelleuses.

Two patients with autosomal dominant pure cortical cerebellar atrophy, belonging to the same family, exhibited imitation synkineses of hands and feet when the contralateral extremity was moved. The phenomenon was observed particularly when alternate movements of one hand were performed, but it also existed when flexion-extension movements of one foot took place. The induced synkinetic movements were mainly observed on the right side in one patient and exclusively observed on the right side in the other one. At electromyography, the imitation synkinesis took place about 200 milliseconds after the first inducing movement, but tended to be simultaneous with the following ones. Imitation synkinesis appeared to be shared by other cerebellar conditions: 8 cases of sporadic pure cerebellar atrophy and 2 cases of post-surgical injury of the anterior lobe vermis. In the 2 genetic cases, there was no pyramidal sign nor sensitive disturbance, the somesthesic evoked potentials being normal. Thus, the imitation synkinesis was considered as having a cerebellar origin. The cerebellar imitation synkinesis might be provoked by the lack of a physiologic cerebellar inhibition located in the paleo- and/or neo-cerebellum. The predominance of imitation synkineses on the right side suggests that cerebellar inhibition is stronger for the dominant side, in order to liberate it from archaic synkineses.

[Value of somatosensory evoked potentials in thoraco- brachial outlet syndrome]. / Intérêt des potentiels évoqués somesthésiques dans les syndromes de la traversée thoraco-brachiale.

INTRODUCTION: Brachial plexus involvement in symptoms of thoracic outlet syndrome (TOS) is often difficult to assess from clinical data. Conventional EMG and nerve conduction studies (NCS) do not seem reliable to all authors. For this reason, our investigations of this syndrome were complemented by study of somatosensory evoked potentials (SEP) in order to compare the results of these different techniques. PATIENTS AND METHODS: Ten patients were studied, all of whom had prominent vascular symptoms which led to their consulting a vascular surgeon. Only one had hand wasting without hypoesthesia. None had cervical rib or cervical spine anomaly. In all cases, diagnosis was confirmed by arteriography or phlebography. Operations were decided on clinical data and results of vascular investigations. Patients were tested with conventional motor and sensitive NCS F-wave studies. Needle EMG was performed in abductor pollicis brevis, first dorsal interosseus or abductor digitiminimi. Their SEP were performed as for controls. Ten controls were studied whose SEP were obtained at Erb's point (N9) and C2 cervical spine level (N13) after percutaneous stimulation of median and ulnar nerves at the wrist on both sides. The criterion of abnormality was the mean of controls + 2.5 SD for latencies. Amplitude was considered as low when it was less than 50% of the contralateral one. RESULTS: For 2 patients EMG, NCS and SEP were abnormal. One had hand wasting and denervation in hand muscles as well as slowed median and ulnar sensory conduction with low amplitude responses. SEP at Erb's point were slightly delayed after ulnar stimulation. No cervical response was obtained after ulnar stimulation. The second one had normal responses at Erb's point but delayed responses at the cervical level. In addition, N13 amplitude after ulnar stimulation was low. Four patients had normal EMG, NCS and SEP. Two patients had normal EMG and NCS, but their SEP was questionable since latencies were normal, even though amplitude was low after median and ulnar stimulation. This was not considered this to be abnormal since it was bilateral. For the remaining 2 patients (F.1), EMG and NCS and Erb's point SEP were normal, but C2 median and ulnar responses were delayed in one case and C2 ulnar response amplitude was very low on one side only in the other. In conclusion, SEP were abnormal for 4 patients out of 10 but gave more information than conventional EMG and NCS for only 2 patients. SEP abnormalities prevailed after ulnar stimulation.

Improvement of cerebellar ataxia with levorotatory form of 5-hydroxytryptophan. A double-blind study with quantified data processing.

Some features of cerebellar ataxia have been reported to regress partially with long-term administration of 5-hydroxytryptophan or levorotatory form of 5-hydroxytryptophan. To test this effect further, 30 patients with various inherited or acquired cerebellar ataxias underwent a randomized, double-blind trial of placebo, and the levorotatory form of 5-hydroxytryptophan taken orally for four months. The levorotatory form of 5-hydroxytryptophan significantly improved the ataxia score. It also significantly modified the time of standing upright, the spread of feet, the speed of walking, speaking, and writing. In five cases in which the levorotatory form of 5-hydroxytryptophan therapy was maintained for 12 months, the effect continued progressively.

[Peripheral neuropathies simulating amyotrophic lateral sclerosis in gammopathies]. / Neuropathies périphériques simulant une sclérose latérale amyotrophique au cours des gammapathies.

Particular clinical pictures of ALS may occur during the course of some "benign gammapathies". We observed 12 patients (age range 57 to 74 years; 9 men-3 women) with initially benign gammapathy (9 IgG, 1 IgA, 2 IgM) associated to a clinical picture of progressive anterior horn and pyramidal tract involvement. These cases led us to recognize some particularities of gammapathy-associated ALS: Relative frequency of asymmetrical clinical manifestations, rarity of bulbar signs; Decrease of sensory nerve conduction velocities without evidence of sensory clinical symptoms (8 out 9); Increase CSF protein content including the monoclonal component, axonal degeneration and immunostaining evidence of the paraprotein fixation observed on nerve biopsies (5 out 7 cases). Association of ALS and gammapathy is not fortuitous as shown by epidemiology, experiments, pathology and effects of different immunological treatments as related in this study. Demonstration of infra-clinical neuropathy face to a clinical syndrome of ALS should prompt too careful screening for a gammapathy.

[Clinical amyotrophic lateral sclerosis syndromes in gammapathies: clinical, electrophysiologic and histoimmunologic aspects]. / Syndromes cliniques de SLA au cours des gammapathies: aspects cliniques, électrophysiologiques et histoimmunologiques.

Eleven patients with motor neuron disease associated with a monoclonal gammopathy were studied. One patient had a previously known multiple myeloma. In the other patients neurological symptoms preceded diagnosis of the gammopathy by one month to 8 years. They were 5 multiple myeloma, 1 macroglobulinemia, 1 chronic lymphatic leukemia, 1 lymphoma and 3 benign monoclonal gammopathies. EMG and motor conduction velocities results were consistent with anterior horn cell disease but sensory conduction studies were abnormal in seven cases out of eight. Superficial peroneal nerve biopsy performed in all patients confirmed peripheral nerve involvement. The relations between gammopathy, clinical symptoms and electrophysiological data are discussed. The interest of sensory conduction studies in every patient with motor neuron disease is emphasized.

[Parathyrotoxic encephalopathy without hypercalcemia]. / Encéphalopathie parathyrotoxique sans hypercalcémie.

Two patients with severe, repeated confusional episodes of apparent fluctuating dementia type, and electroencephalographic alterations (permanent, diffuse slow waves) on recordings, were found to have elevated parathormonemia and slightly raised or normal levels of calcemia. The only other sign of hyperparathyroidism was intermittent hypophosphoremia. Exploratory cervicotomy identified a parathyroid microadenoma in one case and a parathyroid adenoma in the other. Recovery of intellectual functions was slow but complete. The possible direct role of parathormone in the genesis of this parathyrotoxic encephalopathy is discussed.

[Visual evoked potentials (VEP's) obtained by black and white or red and black checkerboard inversion. Results in healthy subjects and in multilocular sclerosis patients]. / Potentiels évoqués visuels (PEV) obtenus par inversion de damiers noirs et blancs ou rouges et noirs. Résultats chez les sujets normaux et dans la sclérose multiloculaire.

Visual evoked potentials (VEPs) were elicited in a control group and in 125 multiple sclerosis (MS) patients by reversal of TV black and white checkerboard patterns with two different check sizes and contrasts (44'/contrast 50%; 22'/contrast 20%), and also by a display of red light emitting diodes (LEDs) generating a 'true' pattern reversal. In control subjects LED pattern reversal produced responses of lower amplitude and of longer latency compared to responses to TV black and white checkerboards. The interindividual variability of P100 latency was lower with the LED display than with the low contrasted TV pattern. In 18 patients with definite or suspected MS, VEPs were normal with the two TV patterns when delayed or absent with the LED display. Thus LED pattern reversal was a more effective stimulus than black and white pattern reversal for the detection of demyelinating lesions of the optic nerves in MS. LED stimulation proved to be particularly useful in patients with progressive spastic paraparesis or with previous history of isolated optic neuritis.

[Value of electromyography in the child. Apropos of 1,624 examinations performed over a 3 year period]. / Intérêt de l'électromyographie chez l'enfant. A propos de 1624 examens réalisés en 3 ans.

EMG and nerve conduction studies have limitations and require particular consideration in children. The indications and the main results are considered from the study of 1624 EMGs in 1385 children under 15 years old seen over a period of 3 years. Classification of cases was based on clinical criteria. The diagnostic yield of EMG is emphasized in the evaluation of 122 children with hypotonia and weakness (all under 3 years old); abnormalities were demonstrated in 48% leading to the diagnosis of spinal muscular atrophy in 16%, of congenital myopathies in 11%, and of peripheral neuropathies in 8%. In 103 children with hypotonia associated with mental deficiency and/or seizures, evidence of a peripheral neuropathy was shown in 20%. EMG abnormalities allowing the diagnosis of hereditary motor and sensory neuropathies or myopathies were demonstrated in a wide range of clinical conditions. EMG appears to be useful for the early detection of hereditary myopathies or neuropathies in asymptomatic children. It may have also a prognostic value as in adults, especially in nerve traumas.

[Contribution of visual evoked potentials to the diagnosis of isolated progressive paraparesis in the adult]. / Apport des potentiels évoqués visuels au diagnostic des paraparésies progressives isolées de l'adulte.

Pattern reversal VEPs were recorded in 30 adult patients with progressive spastic paraparesis. Abnormal responses were observed in 17 patients (57 per cent) without any clinical evidence of visual impairment. Abnormal VEPs helped to the diagnostic reclassification of these patients by demonstrating the multifocality of the lesions. When the number of lymphocytes and/or the lgG level were abnormal in the cerebrospinal fluid (CSF) abnormal VEPs conforted the diagnosis of multiples sclerosis (MS) in 12 cases out of 20. In five patients out of the ten whose CSF was normal MS was considered as highly probable because of VEPs abnormalities.

[Regression of the cerebellar syndrome under long-term administration of 5-HTP or the combination of 5-HTP and benserazide. 26 cases quantified and treated using computer methods]. / Régression du syndrome cérébelleux sous administration à long terme de 5-HTP ou de l'association 5-HTP-bensérazide.

A quantitative evaluation of cerebellar ataxia, with an ataxia score (total, static, kinetic) and the measurement of objective values related to the major symptoms, is proposed. 21 patients with heredo-ataxias were treated for 12 months with high doses (16 mg/kg/day) of D-L-5-HTP, L-5-HTP or the association D-L-5-HTP (16 mg/kg/day)-Benserazide(6 mg/kg/day). A computerized processing of the data obtained by regular examination was performed. The ataxia showed a significant regression at the 12th month, mainly in the static performances and in the speed of speech. L-5-HTP appeared to be more active than D-L-5-HTP. The regression of the cerebellar ataxia was also observed in non degenerative conditions such as multiple sclerosis and surgical injury of the anterior lobe vermis, showing that 5-HTP was active on the cerebellar syndrome in general. The regression of the cerebellar ataxia was very slow in inherited diseases and continued for 2 or 4 months after the treatment stopped. A serotoninergic cerebellar control of motricity is discussed.