Wilson Disease
Although a neurologic disorder, it affects multiple organs, with the liver being the most common and earliest affected. Approximately 40% of newly diagnosed cases have hepatic involvement. Neurologic manifestations include…
Although a neurologic disorder, it affects multiple organs, with the liver being the most common and earliest affected. Approximately 40% of newly diagnosed cases have hepatic involvement. Neurologic manifestations include…
Certain features may suggest a nonorganic basis for the movement disorder. Features in the history that raise this possibility are the abrupt onset of symptoms and their marked variability in…
The spectrum of tics includes transient tics of childhood when present for less than 1 year, chronic motor or vocal tics when tics are present for more than 12 months,…
There are many causes of chorea, such as pregnancy (chorea gravidarum), Huntington disease, benign hereditary chorea, neuroacanthocytosis, Sydenham chorea, systemic lupus erythematosus, focal vascular lesions, medications (particularly the chronic use…
Dystonia-plus syndromes include dystonia accompanied by other neurologic findings on examination. Dopa-responsive dystonia (DRD), an autosomal dominant condition with incomplete penetrance, is due to a defect in chromosome 14 encoding…
Other genetic mutations causing childhood-onset generalized dystonia have been described more recently. Next to DYT1, perhaps the most important is DYT6, which is associated with a mutation in the THAP1…
Surgery By the middle of the 20th century, a handful of surgical procedures had been developed for the treatment of Parkinson disease and other movement disorders (see Plate 7-10). Cortical…
DBS does not improve symptoms that are resistant to levodopa and, consequently, careful documentation of an adequate response to levodopa is important in surgical candidates. A positive symptomatic benefit from…