Tics and Tourette Syndrome
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,…
Hyperkinetic Movement Disorder: Idiopathic Torsion Dystonia
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…
Hyperkinetic Movement Disorder: Cervical Dystonia
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…
Surgical Management of Movement Disorders
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…
Parkinsonism: Hypothesized Role of Dopamine
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…
Progressive Supranuclear Palsy
CORTICOBASAL DEGENERATION (CBD) Corticobasal degeneration (CBD) is characterized by asymmetric cortical atrophy, neuronal cell loss, gliosis, and ballooned neurons in the central sulcus region (primary motor/primary sensory cortex), with achromatic…
Corticobasal Degeneration
TREATMENT Current treatment of parkinsonism centers on administration of levodopa. Despite recent advances in our understanding of the chemical and pathologic changes and development of novel therapies for PD, levodopa…
Neuropathology of Parkinson Disease
An example of a fully developed clinical syndrome due to generalized diffuse Lewy deposits is diffuse Lewy body disease/dementia complex. MULTIPLE SYSTEM ATROPHY In multiple system atrophy (MSA), the unifying…
Parkinsonism: Successive Clinical Stages
Untreated Parkinson disease may be divided into five stages. Stage 1 is characterized by mild unilateral disease. Tremor may be the only visible sign but other subtle findings, including slowness…
Schematic and Cross Section of Basal Ganglia
A biconvex structure, the subthalamic nuclei (STN) receives glutamatergic inputs from the cerebral cortex, GABA inhibition from the GPe, and provides glutamatergic innervations to the GPe, GPi, SN, and PPN….
