Causes for treatment delays in dystonia and hemifacial spasm: a Canadian survey.

BACKGROUND: Dystonia must be accurately diagnosed so that treatment can be administered promptly. However, dystonia is a complex disorder, with variable presentation, which can delay diagnosis. METHODS: Data were gathered by questionnaire from 866 patients with dystonia or hemifacial spasm (HFS) treated in 14 movement disorders centres in Canada injecting botulinum toxin, to better understand the path to diagnosis, wait times and obstacles to treatment. RESULTS: Most participants were female (64.1%), mean age was 58 years, and patients consulted an average of 3.2 physicians before receiving a dystonia or HFS diagnosis. Many patients (34%) received other diagnoses before referral to a movement disorders clinic, most commonly "stress" (42.7%). A variety of treatments were often received without a diagnosis. The mean lag time between symptom onset and diagnosis was 5.4 years. After the decision to use botulinum toxin, patients waited a mean of 3.1 months before treatment. The most common diagnoses were cervical dystonia (51.6% of patients), HFS (20.0%) and blepharospasm (9.8%). CONCLUSIONS: Survey results show that diagnosis of dystonias or of HFS, and therefore, access to treatment, is delayed. An educational program for primary care physicians may be helpful to decrease the time to diagnosis and referral to a specialist centre for treatment.

Is the target for thalamic deep brain stimulation the same as for thalamotomy?

Deep brain stimulation (DBS) has virtually replaced thalamotomy for the treatment of essential tremor. It is thought that the site for DBS is the same as the optimal lesion site; however, this match has not been investigated previously. We sought to determine whether the location of thalamic DBS matched the site at which thalamotomy would be performed. Eleven patients who had detailed microelectrode recording and stimulation for placement of DBS electrodes and subsequent successful tremor control were analysed. An experienced surgeon, blinded to outcome and final electrode position, selected the ideal thalamotomy site based on the reconstructed maps obtained intraoperatively. When the site of long-term clinically used DBS and theoretical thalamotomy location was calculated in three-dimensional space and compared for each of the x, y, and z axes in stereotactic space, there was no significant difference in the mediolateral location of DBS and theoretical lesion site. There was also no difference between the theoretical lesion site and the placement of the tip of the electrode; however, the active electrodes used for chronic stimulation were significantly more anterior (P = 0.005) and dorsal (P = 0.034) to the ideal thalamotomy target. This mismatch may reflect the compromise required between adverse and beneficial effects with chronic stimulation, but it also suggests different mechanisms of effect of DBS and thalamotomy.

Clinical manifestations of Parkinson's disease and parkinsonism.

The most common disorder in a patient presenting to a movement disorder clinic will be parkinsonism. The challenge is to provide the patient with the most accurate diagnosis and prognosis possible. The assumption at the time of initial presentation of the clinical diagnosis of Parkinson's disease is often wrong (20-25%). Waiting to see the pattern of progression, and response to medication provides invaluable additional information. This manuscript summarizes the clinical manifestations of Parkinson's disease and the main akinetic-rigid syndromes (progressive supranuclear palsy, multiple system atrophy, cortical-basal ganglionic degeneration, and dementia with Lewy bodies) that make up the differential diagnosis.