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Amongst all regions of the body, the craniocervical region is the one most frequently affected by dystonia. Whilst blepharospasm--involuntary bilateral eye closure--is produced by spasmodic contractions of the orbicularis oculi muscles, oromandibular dystonia may cause jaw closure with trismus and bruxism, or involuntary jaw opening or deviation,(More)
OBJECTIVES To develop a hypothetical scheme to account for clinical disorders of vertical gaze based on recent insights gained from experimental studies. METHODS The authors critically reviewed reports of anatomy, physiology, and effects of pharmacologic inactivation of midbrain nuclei. RESULTS Vertical saccades are generated by burst neurons lying in(More)
OBJECTIVES To investigate the relative roles of burst neurons (which generate the saccadic command) and omnipause neurons (which gate the activity of burst neurons) in the pathogenesis of slow saccades in progressive supranuclear palsy (PSP). BACKGROUND Experimental inactivation of mesencephalic burst neurons impairs vertical but not horizontal saccades.(More)
OBJECTIVE Although gait ataxia is usually associated with cerebellar lesions, we review a less familiar cause. We present three patients with dorsal midbrain lesions and correlate these presentations with recent findings in the functional anatomy of the midbrain. CONCLUSION We suggest that these lesions involve a well-studied but generally unfamiliar area(More)
BACKGROUND Friedreich ataxia (FA), the most common hereditary ataxia, is caused by pathological expansion of GAA repeats in the first intron of the X25 gene on chromosome 9. Since the discovery of the gene, atypical features are increasingly recognized in individuals with FA, and up to 25% of patients with recessive or sporadic ataxia do not fulfill the(More)
The formation of neutralizing antibodies (NAbs) directed specifically against the active neurotoxin part of the botulinum neurotoxin (BoNT) complex is often cited as a major cause of secondary non-responsiveness (SnR) to treatment. This systematic and meta-analytic review evaluates the frequency of NAbs among patients treated with BoNT therapy for any(More)
Deep brain stimulation (DBS) is used increasingly in the field of movement disorders. The implanted electrodes create not only a prior risk to patient safety during MRI, but also a unique opportunity in the collection of functional MRI data conditioned by direct neural stimulation. We evaluated MRI-related heating for bilateral neurostimulation systems used(More)