Henry Meige: The man and his understanding of dystonia, at the turn of the 19th to 20th century.

BACKGROUND: Henry Meige (1866-1940), a French neurologist, the pupil of Charcot, is remembered for the eponym, the Meige syndrome, describing the clinical picture of craniocervical dystonia. This historical review highlights the controversies from his essay on "Le Juif Errant" (the Wandering Jew), and the evolution of his understanding of the movement disorders of the face and neck at the time of the encephalitis lethargica. RESULTS: His thesis reported 5 patients from Eastern Europe, presenting with functional neurological disorders following traumatic life experiences. He wrote with Feindel the first book on movement disorders "Les tics et leur traitement". He pioneered the concept of focal dystonia and distinguished the facial median spasm as a dystonic movement disorder of the face. He highlighted the co-existence of psychopathology and the influence of the mental on tics and dystonia. He coined with Brissaud and Feindel the term "geste antagoniste" in cervical dystonia. He emphasized the importance of self-management and psycho-motor retraining for focal dystonia. CONCLUSION: Meige made an invaluable contribution to our understanding of movement disorders, during his long medical career. The eponym Meige syndrome should be retained to describe an individual clinical entity.

[Problems of treating writer's cramp with botulinum toxin injections: results from 10 years of experience]. / Traitement de la crampe des écrivains par la toxine botulique: efficacité et limite de cette technique basée sur 10 ans d'expérience.

We studied the efficacy of botulinum toxin (BTX-A) injections in 167 patients, from a large cohort of 259 patients, presenting with writer's cramp (WC) and followed up to 10 years. The selection of the muscle was based on a careful physical examination, using up to 6 manoeuvres whilst attempting to write in order to bring out the original dystonic posture. The injection technique had to be precise, under EMG guidance, with a hollow recording needle to detect muscle or finger fascicle. The results showed a good efficacy and tolerance of this treatment in the long term with recovery of normal writing in 46 per cent, partial benefit in 10 per cent, failure in 21 per cent, and loss to follow-up after the first injection in 23 per cent. Among the responders, 27 per cent carried on the treatment every 9 months on average, with a duration of benefit of 6 months with follow-up between 3 and 9 years. Mirror dystonia had no prognostic value. Secondary dystonia, tremulous WC, long duration WC and progressive WC were associated with poor outcome.

[Treatment of dystonias]. / Traitement des dystonies.

SYMPTOMATIC TREATMENT: Treatment of idiopathic dystonia depends on the age of onset and the extent of the disease. Symptomatic treatment has made many advances over the last 20 years. CHILDHOOD ONSET GENERALIZED DYSTONIA: In childhood onset cases, it is important first to exclude dopa-responsive dystonia. Most of these patients will respond subsequently to high dose anticholinergics. ADULT ONSET FOCAL DYSTONIA: Treatment of this form has been transformed by botulinum toxin therapy. TREATMENT: The treatment of each focal dystonia (blepharospasm, spasmodic torticollis, oromandibular dystonia, laryngeal dystonia, writer's cramp, foot dystonia and axial dystonia) are reviewed. Treatments must be titrated carefully. Botulinum toxin injections require precision and must be repeated regularly. A rehabilitation program must be adapted to each individual. Surgery, in very severe cases, requires an extensive well-planned discussion of indications.

[Botulinum toxin in the treatment of frontoglabellar and periorbital wrinkles. An initial study]. / La toxine botulique dans le traitement des rides fronto-glabellaires et de la région orbitaire. Etude préliminaire.

Glabellar frown lines and crow's feet are wrinkles of facial expression related to an underlying muscular activity, which is particularly strong during facial expression. Classic treatments of these wrinkles only give partially satisfactory are associated with results, and secondary effects, whether they involve skin and muscle lifting, surgical section of muscles, dermal stimulation by thread or injectable fillers, chemical or mechanical abrasion, transient or permanent soft tissue augmentation with various materials. The authors studied the efficacy and safety of intramuscular injections botulinum A Exotoxin in glabellar and crow's feet areas in 19 well-informed and consenting patients. Botulinum toxin injections have been used since 1980 in the treatment of focal dystonia (blepharospasm, oromandibular dystonia, spasmodic torticollis, spasmodic dysphonia and writer's cramp) and safety hemifacial spasm. Their wide use in these indications has highlighted their excellent and efficacy, and the need to repeat injections every 3 to 4 months. The dose required was progressively adjusted around glabellar and orbital areas, while injections of the peri-buccal and forehead areas are still being evaluated. The 19 patients were examined clinically, filmed and photographed every month over a period of 12 to 24 months, and skin prints were performed. Evaluation criteria included the percentage improvement as assessed by the patients themselves, and also evaluation by the investigators of the data of clinical examination, and blind comparison of photographic, videoscopic, and prints. The authors obtained a significant decrease of wrinkles of the areas studied, with a "smoothing" effect during the period of activity of the toxin, which lased an average of 3 to 4 months at the beginning, and 6 to 9 months after several injections. No secondary effects, either general or local, were observed. The product's specificity means that the operator must have a complete mastery of the injection technique and a thorough knowledge of its pharmacology.

"Apraxia of lid opening," a focal eyelid dystonia: clinical study of 32 patients.

We have seen 32 patients with "apraxia of lid opening" (ALO) in the following clinical settings: as an isolated condition (3 patients), idiopathic blepharospasm (BSP, 20 patients, including 4 familial cases), progressive supranuclear palsy (PSP, 7 patients), and dystonic parkinsonian syndrome (2 patients). Twenty-nine patients treated with botulinum toxin into the orbicularis oculi muscle were rated before and after treatment and 83% of the patients improved on a clinical scale. Best results were obtained with injections directed toward the junction of the preseptal and pretarsal parts of the palpebral orbicularis oculi. Several patients also improved on anticholinergic drugs. Besides medical treatment, lid crutches, in conjunction with botulinum toxin injections, were useful in some patients. ALO is not a true apraxia; it constitutes an eyelid dystonia as shown by its clinical and electrophysiological features as well as pharmacological reactions and is encountered in a clinical spectrum ranging from an isolated form to predominant BSP. It was an important cause of treatment failures in botulinum toxin-treated BSP but by modifying our injection strategy and by adding anticholinergic drugs and also lid crutches, we obtained a good functional benefit.

[Indications for botulinum toxin in laryngectomy]. / Indication de la toxine botulique en laryngologie.

The botulinum toxin is a highly potent neurotoxin, used since several years in the treatment of the focal musculary dystonias. We define the laryngeal dystonia as a clinical entity, which is represented by a spasmodic dysphonia or an inspiratory dyspnea without dysphonia, related to a specific dystonia of the thyroarytenoid muscles. The laryngeal functional exploration (i.e. fibroscopy, videostroboscopy, acoustic analysis, computerized voice analysis), the neurological and electrophysiological assessment allow to make diagnosis and evaluation of the therapeutical results of this rare laryngeal neurologic disease which was relatively misunderstood until now. There are several clinical types of spasmodic dysphonia: adductor form (with a jerky voice, pitch beaks, vocal arrests and pneumophonatory incoordination); abductor form (with a breathy voice of very low intensity) and mixed types which can be difficult to identify. Since 1989, we have treated 55 laryngeal dystonias with local intra-muscular botulinum toxin injection: 48 spasmodic dysphonia and 7 inspiratory dyspnea without dysphonia, with hyperactivity of the thyroaryténoïd muscles. We present our therapeutical protocol and results which are good in 87% of cases.

Effect of a macrolide (spiramycin) on the pharmacokinetics of L-dopa and carbidopa in healthy volunteers.

In one well-equilibrated parkinsonian patient treated with combined L-dopa and carbidopa (Sinemet), we have observed changes in treatment efficacy while receiving spiramycin (Rovamycine) for an intercurrent respiratory infection. A preliminary study of the pharmacokinetics of L-dopa and its main metabolites 3-O-methyldopa (3-OMD) and dihydroxyphenylacetic acid (dopac) in two parkinsonian patients treated with Sinemet has revealed a marked decrease in the AUC0-360 of these two metabolites after a 3-day course of Rovamycine. In order to confirm this interaction, we have studied the modifications of the pharmacokinetics of L-dopa, 3-OMD, dopac, and carbidopa in eight male healthy volunteers after a single dose of Sinemet 250 (L-dopa, 250 mg and carbidopa, 25 mg) before and after a 3-day course of Rovamycine. Our study confirms this interaction. After spiramycin, we observed a marked reduction in AUC0-360 for L-dopa (p less than 0.001), 3-OMD (p less than 0.001), and carbidopa (p less than 0.001), and an increase in AUC0-360 for dopac (p less than 0.01). The L-dopa elimination half-life was increased (p less than 0.012); differences in peak plasma concentrations did not attain statistical significance. We think that these modifications in L-dopa pharmacokinetics after spiramycin are due to nonabsorption of carbidopa secondary to modified gastrointestinal motility.

Stridor and focal laryngeal dystonia.

Fibreoptic laryngoscopy in 6 patients with laryngeal stridor showed immobile vocal cords in a paramedian position but no other local cause. Thus a diagnosis of Gerhardt's syndrome, usually ascribed to paralysis of vocal-cord abductor muscles, was made in 3 patients who had no other signs or symptoms of dystonia, and in 3 patients who had multifocal dystonia. Electromyography (EMG) showed evidence of overactivity of vocal-cord adductors, with no evidence of denervation in the abductor muscles. Botulinum toxin injection of the overactive thyroarytenoid muscles abolished stridor. These clinical and EMG findings indicate that Gerhardt's syndrome is not caused by paralysis of vocal-cord abductors, but represents a focal laryngeal dystonia which may be treatable by botulinum toxin injections of vocal-cord adductor muscles rather than by arytenoidopexy or tracheostomy.

[Spasmodic dysphonia. Investigation and therapeutic methods]. / Les dysphonies spasmodiques: méthodes d'investigation et de traitement.

We report the successful treatment of three patients with adduction spasmodic dysphonia by direct injection of botulinum toxin into the vocal cord(s). This was achieved under electromyographic control, and this and other otolaryngeal techniques were used to monitor treatment and study this often puzzling condition.

[Treatment of spasmodic dysphonia with botulinum toxin]. / Traitement de la dysphonie spasmodique par la toxine botulique.

Spasmodic dysphonia is a focal laryngeal dystonia, a rare form of dystonia. Videostroboscopy, acoustic analysis, computerized voice analysis and over all electrophysiological analysis allow for the study of the different muscles involved in this dysphonia. There are two types of spasmodic dysphonia: adductor spasmodic dysphonia and abductor spasmodic dysphonia. The most efficient therapy nowadays is the injection of botulinum toxin into the thyroarytenoid muscle under fiberoptic visualization. We report 6 patient's cases of spasmodic dysphonia that we have been treating for about 2 years by direct injection of botulinum toxin in the vocal cords.

Natural history of adult-onset idiopathic torticollis.

The rates of spontaneous remission and progression of dystonia to other sites were studied in 72 patients who first presented with adult-onset torticollis, and who were followed up for a mean of 7.7 years. Dystonia had progressed to sites other than the neck (mainly the face and upper limbs) in 23 patients (32%). The latter cases were not differentiated from those with isolated torticollis in terms of any of the demographic or clinical features studied, although they tended to have suffered from torticollis longer. Fifteen patients (20.8%) had experienced a spontaneous remission of their torticollis, which was sustained for a median period of 3 years in 9 cases (12.5%). Eighty-seven percent of the 15 remissions had occurred during the first 5 years of the illness. In the 9 cases with sustained remission, the duration of torticollis before spontaneous remission was significantly longer and remission had mostly occurred after 2 years of illness compared with the 6 who had relapsed. The 15 cases with spontaneous remission tended to have an earlier age of onset compared with those with no remission. Sixty-five percent of cases were correctly classified on the basis of age at onset, which emerged as the only salient variable in the discrimination of the 15 patients with spontaneous remission from the 57 without spontaneous remission. Age at onset, form of torticollis, gender, and direction of head deviation resulted in a correct classification rate of 70%, in the discrimination of the 9 cases with sustained remission from those with no remission.(ABSTRACT TRUNCATED AT 250 WORDS)

[Objective tinnitus and palatal myoclonus. A new therapeutic approach]. / Acouphène objectif et myoclonies vélaires. Nouvelle approche thérapeutique.

The syndrome combining velar myoclonia with objective tinnitus is rare and, in adults, it corresponds most of the times to a neurological lesion of the dento-olivary tractus. Its functional manifestations are often invalidating and propounded medical treatments uneffective. The authors report a case in which local injection of botulinus toxin allowed to obtain satisfactory functional improvement. Such therapy may prove useful when conventional management is failing.

Spasms of amputation stumps: a report of 2 cases.

The occurrence of involuntary movements of stumps following amputation is described in two patients. Although recognised for over 100 years, this phenomenon has received little attention in the modern literature. The condition appears to represent a variant within the spectrum of movement disorders induced by injury to the peripheral nervous system.