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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)
Accumulation of alpha-synuclein is a pathological feature in several neurological diseases. Its characterization has allowed for a re-grouping of diseases according to the expected pathology. The clinical syndrome of PD can now be classified into forms with and without alpha-synuclein pathology. DLB and PDD are synucleinopathies, and MSA shows(More)
Tremor is one of the most common involuntary movement disorders seen in clinical practice. In addition to the detailed history, the differential diagnosis is mainly clinical based on the distinction at rest, postural and intention, activation condition, frequency, and topographical distribution. The causes of tremor are heterogeneous and it can present(More)
BACKGROUND Dystonia refers to a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures. Although age at onset, anatomic distribution, and family history are essential elements in the evaluation of dystonia, new classification increasingly relies on etiologic and genetic data. In recent years,(More)
Botulinum toxin type A (BTX-A) is best known to neurologists as a treatment for neuromuscular conditions such as dystonias and spasticity and has recently been publicized for the management of facial wrinkles. The property that makes botulinum toxin type A useful for these various conditions is the inhibition of acetylcholine release at the neuromuscular(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)
Idiopathic cervical dystonia (ICD) is the most common form of focal dystonia. A characteristic and unique diagnostic feature is the presence of "sensory tricks", for example, a finger placed on the chin to neutralize the spasm. Although approximately 70% of patients with ICD experience effective sensory tricks, the exact mechanism of these tricks is still(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)