Prolonged remission of dystonia occurs rarely; however, well-documented cases are lacking. We report the clinical characteristics and course of four patients with botulinum toxin (BoNT)-associated prolonged remission of idiopathic cervical dystonia. Mean age at onset was 40 years. All had a relatively short duration of symptoms (mean 10.3 months), and with remission occurring after ≤ 3 treatments with BoNT. At last examination, the remission duration was 2–5 years. In the two cases that subsequently relapsed after 4–5 years, there was an altered phenomenology and worsened severity than at the onset. Recognizing this rare phenomenon has valuable clinical implications.

Functional neurological disorder (FND) is a complex neuropsychiatric syndrome with many phenotypes that are commonly encountered in clinical practice. Despite the heterogeneity of FND, the rate of misidentification is consistently low. For the more common motor subtypes, there are clear positive clinical, electrophysiological, and rarely imaging criteria that can establish the diagnosis in the traditional sense. For nonmotor subtypes, the characterization may be less clear. Here, we argue that the current diagnostic criteria are not reflective of the current shared neuropsychiatric understanding of FND, and, as a result, provide an incomplete picture of the diagnosis. We propose a three-step diagnostic triad for FND, in which the traditional neurological diagnosis is only the first element. Other steps include psychiatric/psychological formulation, integration, and follow-up. We advocate that this diagnostic approach should be the shared responsibility of neurology and mental health professionals. Finally, a research agenda is proposed to address the missing factors in the field.

Because individuals develop dementia as a manifestation of neurodegenerative or neurovascular disorder, there is a need to develop reliable approaches to their identification. We are undertaking an observational study (Ontario Neurodegenerative Disease Research Initiative [ONDRI]) that includes genomics, neuroimaging, and assessments of cognition as well as language, speech, gait, retinal imaging, and eye tracking. Disorders studied include Alzheimer’s disease, amyotrophic lateral sclerosis, frontotemporal dementia, Parkinson’s disease, and vascular cognitive impairment. Data from ONDRI will be collected into the Brain-CODE database to facilitate correlative analysis. ONDRI will provide a repertoire of endophenotyped individuals that will be a unique, publicly available resource.

Since the initiation of bromocriptine therapy for Parkinson's disease several newer dopamine agonists have been developed. Pergolide has reached the stage of Phase 3 clinical trials and will probably be available for general use sometime in the foreseeable future. Lisuride shows most promise in its parenteral form for infusion therapy of patients with severe fluctuations. Mesulergine, another ergot-derivative and ciladopa, a new non-ergot agonist, have been withdrawn from further clinical use due to tumorogenesis in rats. It is questionable how applicable these findings are to the use of the drugs in elderly humans with parkinsonism. Recently a small number of drugs have been found to have postsynaptic dopamine agonist properties only in the setting of denervated supersensitive dopamine receptors. These agents may be particularly effective in the early treatment of patients with Parkinson's disease. This paper will review a number of the dopamine agonists which have been developed since the introduction of bromocriptine therapy. Several of these have shown beneficial effects in early clinical trials while others show promise in preclinical studies of animal models of parkinsonism.

The stiff-person syndrome is a disorder of persistent, painful muscle contractions predominately affecting the axial musculature. We describe a patient with this disorder and review its pathophysiology. Molecular biologic and immunologic techniques have recently added to the understanding of the mechanism of this disorder. Association with diseases such as diabetes, vitiligo and hypothyroidism have strengthened the auto-immune nature of this syndrome. Auto-antibodies against glutamic acid decarboxylase (GAD), an intraneuronal enzyme, have been implicated in the etiology of this unique disease. Therapeutic intervention with agents such as benzodiazepines that modify central GABAergic activity have demonstrated significant benefit in patients with stiff-person syndrome.

Although pain is not generally recognized as a symptom of tic disorders, we have seen a number of patients in whom this was a prominent feature, at times even the symptom of greatest concern. The commonest pain complaints are those arising from the actual performance of a tic. Most often this follows directly from the discomfort produced by sudden or repeated extreme exertion. Here the origin of the pain is usually musculoskeletal, though rare examples of neuropathic pain may occur. Pain also may arise from striking or being struck by a moving body part involved in large amplitude tics. Other related painful acts include deliberate self-injury and pain inflicted upon others. A second major category is represented by a smaller number of patients who complain of pain during voluntary efforts to suppress their tics. Finally, there are patients who obtain relief from tics while experiencing pain, to such an extent that they will deliberately provoke pain to obtain its benefit. We feel that pain should be recognized as a common complaint, and occasionally a source of significant disability, in patients with tics.

Anticholinergics are one of the most common treatments used in adult-onset focal dystonias although their general efficacy has not been proven. We have investigated the effects of intravenous administration of atropine, benztropine and chlorpheniramine, in comparison to normal saline, in 20 patients with adult-onset focal dystonias (spasmodic torticollis (9), cranial dystonia (6), writer’s cramp (5)), and have retrospectively reviewed the notes of 78 patients (spasmodic torticollis (38), cranial dystonia (25), writer’s cramp (15)), who had received chronic oral anticholinergic therapy in varying doses at some time in the past. Patients with spasmodic torticollis and writer’s cramp showed no consistent change with any of the intravenous drugs, while the few with cranial dystonia who improved usually did so at the expense of drug-induced sedation. The natural variability of these disorders was evident in the response to normal saline, which caused changes in the scores for severity of the involuntary movements of 20% or more in some patients. Only 8 of the patients who had received chronic oral therapy obtained more than mild benefit, and in 3 of them this improvement was transient. We conclude that cholinergic mechanisms are not of general or prime importance in the pathogenesis of adult-onset focal dystonias. However, each new patient warrants a trial of chronic oral anticholinergic therapy in view of the occasional useful responses obtained with these drugs.

Two patients are reported with hemiballismus due to lesions in the striatum as documented on CT scan. The involuntary movements persisted for one and five years respectively. A third patient with more typical self-limited hemiballismus had no evidence of a causative lesion on CT scan. It is proposed that lesions outside the subthalamic nucleus of Luys may interfere with the compensatory mechanisms which can result in the spontaneous resolution of hemiballistic movements. None of the patients benefited from the use of sodium valproate therapy, however, pimozide reduced the severity of the dyskinesia in the two with longstanding hemiballismus.