Prevalence and characterisation of vocal fold motion impairment (VFMI) in patients with Multiple system atrophy compared with Parkinson's disease.

INTRODUCTION: Multiple system atrophy (MSA) is a neurodegenerative disorder in which vocal fold mobility can be affected, sometimes leading to life-threatening situations. Our aim was to know if laryngeal examination could help differentiate MSA from Parkinson's disease (PD). MATERIALS AND METHODS: Between 2004 to 2014, all consecutive patients diagnosed with probable MSA were included in this retrospective, monocentric study. Flexible laryngoscopy was obtained in 51 MSA patients and compared with 27 patients with Parkinson's disease (PD). Laryngeal muscles EMG was available in 6 MSA patients. RESULTS: Vocal fold motion impairments (VFMI) was found in 35 (68.6%) MSA patients: 15 (29.4%) had uni- or bilateral vocal fold abnormal movement (VFAM), 13 (25.5%) had uni- or bilateral vocal fold abductor paresis (VFABP), 4 (7.8%) had uni- or bilateral vocal fold adductor paresis (VFADP), 10 (19.6%) had bilateral vocal fold paralysis (BVFP). VFMI was found in 13 PD patients (48.1%) all of whom had VFADP. Presence of BVFP was found associated with stridor (P<0.001) and dysphagia (P=0.002). In all muscles examined in 6 MSA patients, the EMG showed neuropathic patterns. CONCLUSIONS: Our data support that VFMI may be encountered in two-thirds of MSA with a variable degree of gravity. Laryngological examination should be considered as a supplementary tool for the diagnosis and prognosis of MSA. VFMI in particular VFAM, VFABD and BVFP should be discussed as an additional possible red flag even at an early stage of MSA and could help discriminate MSA from PD.

[Treating writer's cramp: 14 years' experience with botulinum toxin]. / Prise en charge de la crampe de l'écrivain: 14 ans d'expérience de la toxine botulique.

INTRODUCTION: Writer's cramp is a focal dystonia; treatment remains disappointing. We report our 14-year experience with a population of 119 patients aged between 18 and 85 years (average age 43 years). METHODS: Treatment was based on botulinum toxin injections (Dysport) and physiotherapy. Patients were reviewed every four to six months with clinical and video evaluation by three different observers and subjective analysis of the treatment efficiency by the patient (score of 1 to 3). The post-injection deficit, if present, was also quantified. RESULTS: In the group treated with toxin and physiotherapy, cramps improved (score 2 and 3) in 61.6% of patients; a majority of patients (n=14) reported they were moderately satisfied (score 2). In the group treated with toxin alone, 37.9% of patients were improved (score 2 and 3) with a majority (n=18) very satisfied reporting normal writing (score 3). Age was not a predictor of therapeutic response. Good results were obtained with injections of the flexor carpi radialis followed by flexor digitorum profundus II and III and the flexor pollicis longus. Seventy-one per cent of injections caused moderate muscle weakness, minimally disabling compared to the benefit of injections. Twenty-seven patients were followed for more than two years and three patients, who had achieved score 3 with excellent response, were followed for 14 years with very efficient repeated injections. If the injections were not effective the first time, we re-assessed the situation and adjusted the injections; we considered that toxin treatment was unsuccessful after three injections without benefit. CONCLUSION: The choice treatment for writer's cramp remains well-targeted injections of botulinum toxin. Physiotherapy is useful when the toxin injections are ineffective in completely improving writing. This requires close cooperation between the injector, the physiotherapist and the patient.

Postural control and sensory integration in cervical dystonia.

OBJECTIVE: Postural control and sensory integration were assessed in 12 patients with Cervical Dystonia (CD) and 11 healthy control subjects (CS), who were asked to maintain their posture as vertical as possible with their eyes open and closed while standing on a platform tilting laterally at angular accelerations below the vestibular activation threshold. METHODS: Data were collected with a three-dimensional acquisition system. The orientation and stabilization components were studied using specific indexes. We also tested the subjective visual vertical. RESULTS: CD does not affect whole body postural control. CD patients were able to control the orientation of their head. CS stabilized their head in space, especially when their eyes were open. This head stabilization strategy was lost in the CD patients, and the presence of visual inputs made no difference. CD patients seemed to neglect the visual information when controlling their head. The RFT confirmed this visual independence. CONCLUSION: CD patients seem to ignore the sensory information skewed by the disease: their reference segment shifts from head to trunk and they tend to preferentially use proprioceptive information. SIGNIFICANCE: As the proprioceptive chain remains functional in CD patients, it seems likely that the vestibular system might rather be involved in the pathophysiology of CD.

Facial reflex myoclonus induced by language: a neuropsychological and neurophysiological study.

We studied a 53 year old right-handed patient who presented isolated myoclonus of right facial muscles induced exclusively by language. Twitching significantly hindered speaking and reading performance. MRI and CT-scan revealed no brain lesion. Conventional EEG showed a few spike-waves predominantly in the left hemisphere. Spike-waves increased during drowsiness. An EEG-EMG polygraphic study was performed during stimulation tests which included linguistic tasks and non-verbal/non-linguistic tests. Myoclonus was triggered by speaking and writing but not by non-linguistic tasks. The severity of myoclonus was dependent on the complexity of the language task. Back-averaging of right facial EMG bursts failed to show a reliable EEG-EMG correlation. However, the facial reflex myoclonus might have originated from the left rolandicopercular cortex, as it was triggered by complex language activities. Findings in this case are compared with those reported for other forms of reflex seizure and myoclonus.

[Treatments by local injections of botulinum toxin in neurology. Indications and results]. / Traitements par injections locales de toxine botulique en neurologie. Indications et résultats.

Local injections of botulinum toxin constitute the only truly effective treatment of certain abnormal movements and focal dystonias. The authors describe its indications and report on their personal experience. One hundred and seventeen patients were treated: 48 for blepharospasm, 46 for hemifacial spasm and 23 for spasmodic torticollis. The results were evaluated by means of a score taking into account the effectiveness of treatment, the duration of this effectiveness, the side-effects, if any, observed, and the course of the neurological disorder after several series of injections. The results were good or excellent in 91 percent of patients with hemifacial spasm and 79 percent of patients with blepharospasm. Spasmodic torticollis was much improved in 35 percent of the cases and less, but satisfactorily, improved in 48 percent. In this disease, the muscles which antagonize those responsible for the dystonia must absolutely be re-educated.

Epilepsy, antiepileptic drugs, and malformations in children of women with epilepsy: a French prospective cohort study.

We conducted a prospective study of teratogenic effects of antiepileptic drugs (AEDs) in pregnant women with epilepsy in southeast France, comparing malformation rates with those collected by a birth defects registry. We evaluated isolated microcephalies separately. Malformations were seen in 7% of infants of mothers with epilepsy (IME) and in 1.36% of the general population. No significant relationship was found between type and severity of epilepsy and occurrence of malformations or isolated microcephaly. Valproate and phenytoin were the most teratogenic (all malformations). None of the malformations observed in IME whose mothers received valproate, phenytoin, or phenobarbital was seen in IME not exposed to the respective AEDs. Phenytoin plus phenobarbital was more teratogenic than phenobarbital alone. Benzodiazepines, prescribed only in combinations, had a borderline, nonspecific effect on microcephaly.