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.

Effects of dopaminergic treatment on bladder function in Parkinson's disease.

AIMS: To evaluate the nature of voiding complaints, findings on urodynamics and the effects of medication on bladder control in a group of Parkinson's disease (PD) patients between the ages of 30-60 years. METHODS: Thirty-two patients with PD were evaluated by questionnaires, PD rating scales, and full urodynamics evaluation. To evaluate lower urinary tract symptoms (LUTS), we used the Danish Prostate Symptom Score (Dan-PSS). PD was evaluated using Unified Parkinson's Disease Rating Scale (UPDRS), and Hoehn and Yahr rating scale for PD. Urodynamics were performed while the patients were on dopaminergic therapy, and after stopping this for three half lives. RESULTS: There was a significant correlation between the Dan-PSS score and UPDRS, Spearman's rho = 0.50, (P = 0.004), and between the score of irritative symptoms in Dan-PSS and motor symptoms (UPDRS(III)) is seen (Spearman's rho = 0.50, P = 0.003). Patients with significant bladder symptoms (> or =10 points in Dan-PSS) had a significantly higher bladder capacity in the medicated state than after wash out (P = 0.04), while patients with out bladder symptoms had no difference. Patients with a significant increase in bladder capacity on medication had a significantly higher interval between the volume of first sensation of bladder filling and bladder capacity when medicated (P = 0.01) than after wash out. Patient who did not benefit from medication had a significant lower interval off medication (P = 0.008). CONCLUSIONS: The effects of dopaminergic treatment on bladder control and urodynamic parameters are unpredictable in the individual patient, though most patients experience significant changes. We have also demonstrated the complexity of bladder control, and that patients with PD may be particularly susceptible to develop complex micturitional dysfunction. Our data indicates that cortical dysfunction may play a significant role in bladder dysfunction in PD parallel to the pontine lesion.

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.

Clinical impact of diagnostic SPET investigations with a dopamine re-uptake ligand.

The diagnosis of Parkinson's disease is based on clinical features with pathological verification. However, autopsy has been found to confirm a specialist diagnosis in only about 75% of cases. Especially early in the course of the disease, the clinical diagnosis can be difficult. Imaging of presynaptic dopamine transporters (DAT receptors) has provided a possible diagnostic probe in the evaluation of Parkinson's disease. The cocaine analogue [(123)I]-2-beta-carboxymethoxy-3-beta(4-iodophenyl)tropane ([(123)I]-beta-CIT) is one of several radioligands that have been developed for single-photon emission tomography (SPET). The purpose of this study was to evaluate the impact of [(123)I]-beta-CIT SPET on the diagnosis and clinical management of patients with a primary, tentative diagnosis of parkinsonism. We undertook a retrospective evaluation of the clinical records of 90 consecutive patients referred to [(123)I]-beta-CIT SPET from the neurological department, Bispebjerg Hospital. In 58 subjects the scans revealed altered tracer uptake consistent with Parkinson's disease, progressive supranuclear palsy and multiple system atrophy. A significant change in the management or treatment because of the scan was found in 25 patients (28%). The sensitivity of the examination was 97% and the specificity 83%. In conclusion, a significant clinical impact of DAT receptor SPET imaging was found. DAT receptor imaging is a useful diagnostic probe in patients with a possible diagnosis of parkinsonism.