Oromandibular dystonia, mental distress and oro-facial dysfunction-A follow-up 8-10 years after start of treatment with botulinum toxin.

BACKGROUND: Oromandibular dystonia (OMD) with involuntary jaw and tongue movements may be misdiagnosed as temporomandibular disorders (TMD) and because of the complex muscle activity and involvement of several small muscles, OMD is also considered difficult to treat. OBJECTIVES: The aim was to evaluate OMD in patients 8-10 years after start of treatment with botulinum toxin (BoNT) by self-reported and standardised global scales and questionnaires. METHODS: Of 21 previously reported patients with OMD, 14 responded to a mail health questionnaire to describe the disease course and treatment effect as well as the overall impact of OMD by a visual analogue scale (VAS), the Patient Health Questionnaire (PHQ) for depression and anxiety, and the Jaw Functional Limitation Scale (JFLS). The results were analysed with non-parametric statistical analysis (Wilcoxon matched-pairs test and Spearman's rank-order correlations). RESULTS: The OMD was still present in 13 patients. In nine patients, the BoNT treatment had continued as monotherapy or combined with oral medication. VAS for OMD was significantly reduced (P < 0.04) over the years, and most patients felt improvement from the treatment. However, the patients had still some functional limitations, typically regarding jaw mobility and communication, and both JFLS and mental distress (PHQ) were significantly correlated with the OMD VAS (rS 0.77 and 0.74). CONCLUSION: The results showed marked reduction of the experienced OMD with treatment and over time, and also stressed similarities between OMD and TMD. Both dentists and neurologists should be aware of this overlap and reduce misdiagnosis by applying an interdisciplinary approach.

Treatment of severe drooling with botulinum toxin in amyotrophic lateral sclerosis and Parkinson's disease: efficacy and possible mechanisms.

OBJECTIVE: Drooling in neurodegenerative diseases is associated with social impediment. Previous treatments of drooling have little effect or are effective but with severe side effects. Therefore, there is a need to test new methods such as the use of botulinum toxin type A (BTX-A). MATERIAL AND METHODS: This open, prospective study deals with treatment of drooling in 12 patients with amyotrophic lateral sclerosis and three with Parkinson's disease. Injections of BTX-A (Botox) were given into the parotid (25-40 units) and submandibular (15-30 units) glands with ultrasonographic guidance. After BTX-A treatment, the patients were followed for 2 months with evaluations every second week by means of self-assessed rating scales for drooling intensity, discomfort and treatment effect, and determination of unstimulated whole saliva (UWS) flow rate, and inorganic and organic UWS composition. The treatment was repeated up to four times, but seven patients dropped out shortly after the first treatment due to marked worsening of their disease-related condition. RESULTS: Drooling and flow were reduced (P < 0.05) 2 weeks after treatment, without side-effects. The maximal reductions during the observation period were 40% for drooling and 30% for flow. There was a systematic variation in flow during the observation period, with an initial decrease and then an increase followed by a second decrease. Amylase activity and total protein concentration generally increased with decreasing flow (P ≤ 0.03). CONCLUSION: Inhibition of acetylcholine release from postganglionic parasympathetic nerve endings by injection of BTX-A into salivary glands seemed useful for secondary sialorrhoea, although cyclic variations in flow may occur, possibly due to transitory sprouting and regeneration.

Interdisciplinary recognizing and managing of drug-induced tardive oromandibular dystonia: two case reports.

Tardive dystonia is a risk factor in medical antipsychotic treatment. It often begins with repetitive involuntary jaw and tongue movements resulting in impaired chewing and detrimental effect on the dentition. The orofacial dysfunction may go unrecognized in a neurological setting. The diagnosis may be difficult so we suggest interdisciplinary collaboration.

Chewing in patients with severe neurological impairment.

OBJECTIVE AND DESIGN: As degenerative disease, infarction and hemorrhage in the CNS may compromise chewing, the aim was to classify and analyse such chewing disturbances. The case series included clinical and electromyographic recordings from 10 patients (ages 12-78 years) with neurological disorders. RESULTS: Classifications were involuntary munching (two women with dystonia which was abolished during mastication), ceased chewing function (three men with impaired volitional motor control and spasticity from locked-in syndrome, restricted chewing range (two men and one woman with reduced jaw opening due to paradoxical activity after brainstem lesions), and distorted chewing pattern (two men with dystonia resulting in blockings during chewing). CONCLUSION: The effect of the neurological impairment illustrated the complex control of mastication and the interaction between central and peripheral mechanisms, and the variation of the chewing disturbances was surprisingly great, even with similar diagnosis.

Onabotulinumtoxin A Treatment of Drooling in Children with Cerebral Palsy: A Prospective, Longitudinal Open-Label Study.

The aim of this prospective open-label study was to treat disabling drooling in children with cerebral palsy (CP) with onabotulinumtoxin A (A/Ona, Botox®) into submandibular and parotid glands and find the lowest effective dosage and least invasive method. A/Ona was injected in 14 children, Mean age 9 years, SD 3 years, under ultrasonic guidance in six successive Series, with at least six months between injections. Doses and gland involvement increased from Series A to F (units (U) per submandibular/parotid gland: A, 10/0; B, 15/0; C, 20/0; D, 20/20; E, 30/20; and F, 30/30). The effect was assessed 2, 4, 8, 12, and 20 weeks after A/Ona (drooling problems (VAS), impact (0-7), treatment effect (0-5), unstimulated whole saliva (UWS) flow and composition)) and analyzed by two-way ANOVA. The effect was unchanged-moderate in A to moderate-marked in F. Changes in all parameters were significant in E and F, but with swallowing problems ≤5 weeks in 3 of 28 treatments. F had largest VAS and UWS reduction (64% and 49%). We recommend: Start with dose D A/Ona (both submandibular and parotid glands and a total of 80 U) and increase to E and eventually F (total 120 U) without sufficient response.

Action of some pericranial muscles during provoked attacks of common migraine.

In this electromyographic study the activity in the temporal, masseter, sternocleidomastoid and nuchal muscles was recorded bilaterally in 2 males and 2 females during attacks of common migraine precipitated with specific beverages. Muscle activity continuously picked up with bipolar surface electrodes was supplemented by pain ratings at regular intervals, and registrations of similar duration were obtained with the patients free of headache. Recordings were divided into sections of 10 min, and during the last 2 min of each section the average level and the peak of the mean voltage were assessed with intervals of 5 sec. The average of these measurements in per cent of maximal activity was used to characterize the activity in a section. The time course of muscle activity varied between patients and muscles. In general, the migraine attack was characterized by a rise of activity from control level shortly before the patients experienced maximal pain, and location of the hyperactivity corresponded reasonably well with the spatial distribution of pain and tenderness. In two patients activity was sufficiently strong to account for a substantial part of their pain.

Electromyography of pericranial muscles during treatment of spontaneous common migraine attacks.

In 7 patients given a standard treatment during spontaneous attacks of common migraine, activity in the temporal and sternocleidomastoid muscles was studied for 140 min. Pain and nausea ratings were taken with 20 min intervals. Baseline recordings were obtained from all patients during a period without headache and from 8 dental students without a history of migraine. During the attack of migraine, activity in the anterior temporal muscles significantly exceeded the patient's own baseline recordings and all muscles were activated more strongly than in the control sample. The increase in per cent of maximal muscle activity was insufficient to account for ischemic pain. Following treatment the activity of the temporal and sternocleidomastoid muscles decreased in 5 patients at the same time as the pain and nausea to the level of the controls. It is suggested that the moderate increase of activity on admission was a residual from stronger contractions earlier in the attack. In addition to cessation of the attack, placement in supine position and intake of diazepam may have contributed to the decline of muscle activity.

Treatment of perioral dystonia with botulinum toxin in 4 cases of Meige's syndrome.

OBJECTIVE: We describe the treatment of 4 patients (median age, 53.5 years) with incapacitating perioral dystonia and insufficient response to peroral medication. Their general treatment with clonazepam and anticholinergics was supplemented by intramuscular injections with botulinum toxin A (20-40 U) in the orbicularis oris muscle, guided by electromyography (EMG). STUDY DESIGN: Perioral dystonia and treatment effect were assessed by using subjective global and visual analog scales, examiner-based video movement counts and rating scales, and quantitative EMG. t Tests were used for statistical analysis. RESULTS: The result of the intramuscular botulinum toxin A injections was characterized by the patients as "much improved"; correspondingly, dystonia was significantly reduced in visual analog scale scores, on examiner-based assessments, and in recordings of EMG. The side effects were few and short-lasting. CONCLUSION: Incapacitating perioral dystonia in Meige's syndrome may be safely controlled by recurrent EMG-guided botulinum toxin A injections in the orbicularis oris muscle, in combination with general medication.

Three-dimensional magnetic resonance image of the mandible and masticatory muscles in a case of juvenile chronic arthritis treated with the Herbst appliance.

The present report documents, in a case of juvenile chronic arthritis (JCA) with mandibular retrognathia, three-dimensional (3D) changes in the mandible and the relationship between the mandible and the masticatory muscles resulting from treatment with the Herbst appliance after cessation of growth. Magnetic resonance scanning of the whole head was carried out before and after treatment. The mandible, the masseter, and the medial and lateral pterygoid muscles were segmented bilaterally and reconstructed in 3D for both stages. Superimposition of the datasets was carried out according to anatomical structures in the brain (cranial base). Mandibular superimposition was performed according to the mandibular symphysis and the lower mandibular border. The mandible moved forward and downward relative to the anterior cranial base. In addition, bone apposition was observed at the superior and posterior surfaces of both mandibular condyles and at the roof of the glenoid fossa. The masticatory muscles remained relatively stable in position in relation to the anterior cranial base. To our knowledge, such information in JCA patients has not previously been published in the literature. Using magnetic resonance imaging (MRI), it was possible to gain improved insight into the 3D morphology including soft tissues without the overlap of the surrounding tissues observed in the conventional radiographs. Accordingly, it is suggested that 3D magnetic resonance analysis is a more useful method for the follow-up of the JCA patients than radiographic techniques.

Oromandibular dystonia--functional and clinical characteristics: a report on 21 cases.

OBJECTIVES: The objectives of this study were to describe subtypes, characteristics, and orofacial function of patients with oromandibular dystonia and report results of special dental importance. STUDY DESIGN: Symptoms, signs, and function were evaluated by questionnaires, video, and clinical and physiological examinations in 21 patients with primary and secondary dystonia (13 focal, 7 segmental, 1 multifocal). RESULTS: A mixture of 2 or more subtypes of jaw movements was most common (43%), and the dystonic electromyographic activity was frequent in the anterior digastric (62%) and temporal and lateral pterygoid (48%) muscles. The impact from the oromandibular dystonia was marked. The prevalence of problems with mastication and swallowing was high, as well as with hyposalivation, dental attrition, and other dental problems. CONCLUSIONS: Patients with oromandibular dystonia may present to dentists with involuntary jaw movements and other severe functional problems. Care must be adapted to the neurological disorder and may be complicated by the condition itself.

Oromandibular dystonia involving the lateral pterygoid muscles: four cases with different complexity.

The report describes oromandibular dystonia (OMD) in four women with involuntary activity of the lateral pterygoid muscles (LP), causing incapacitating protrusive and lateral jaw movements and displacements, and treatment with botulinum toxin type A (BTX). For initial survey and treatment control, OMD was analyzed with several, independent, and standardized methods. OMD severity and functional difficulties were evaluated subjectively and scored from videotapes. Jaw movements were assessed graphically with a magnetic tracking system, and electromyographical activity (EMG) of LP was recorded with needle electrodes using an intraoral approach, whereas activity of masseter muscles was recorded with surface electrodes. EMG-guided BTX injections (25-40 units Botox per muscle) into the muscles were performed with cannula electrodes. Compared with reference values for LP, OMD was associated with a markedly increased level of spontaneous activity, but almost normal level of maximum voluntary activity. The central pattern generator for mastication seemed to override the dystonic activity, as all patients were able to chew despite some distortion. BTX reduced both the spontaneous and the maximum activity for 3-9 months. Concomitantly, a marked reduction of the OMD severity, mandibular movements and functional disturbances were also present with the best effect in localized OMD with late onset.

Treatment of severe temporomandibular joint clicking with botulinum toxin in the lateral pterygoid muscle in two cases of anterior disc displacement.

A novel treatment procedure is introduced for severe clicking of the temporomandibular joint (TMJ) associated with anterior disc displacement (ADD), using injections with botulinum toxin (BTX-A) in the lateral pterygoid muscle (LP). It has been suggested that ADD may be caused, precipitated, or maintained by LP activity, but the role of the LP in the dynamics of the TMJ clicking is uncertain. The case report includes 2 women, followed with clinical examinations, TMJ imaging, and electromyography (EMG), in whom local anesthetics in the LP could abolish the clicking for several days. BTX-A block of the LP (30 U Botox, given twice under EMG guidance with 6-month interval) temporarily reduced the action of the muscle, but the clicking was permanently eliminated and did not return during the observation period of 1 year, and a small but distinctive positional improvement in the disc-condyle relationship was obtained. However, the precise mechanisms behind the favourable treatment outcome are unclear.