Corticobasal degeneration

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  • In writing An Atlas of Parkinson’s Disease and Related Disorders, I have been conscious of the need to find an appropriate match between the text and the illustrative material. The text is designed to provide a basic overview of the conditions discussed, inevitably concentrating on those areas which lend themselves best to photographic illustration. Some movement disorders, by their very nature, do not lend themselves to still photography whereas others, characterized by sustained postures, are ideally suited to the technique.

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  • The field of movement disorders is relatively broad, encompassing disorders of increased movement, such as tremors, dystonia, and tics, to disorders characterized by a paucity of movement, such as Parkinson’s disease. Our understanding of the pathogenic mechanisms and our treatment options are expanding at a rapid pace. This expansion ranges from the medical and surgical advances in treating Parkinson’s disease to the flood of genetic abnormalities that have now been found to cause various movement disorders.

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  • The neurons of the corpus striatum receive an excitatory input from the cerebral cortex and the thalamus. The major outputs project to the globus pallidus and the substantia nigra pars reticula (SNr), and use gamma-aminobutyric acid (GABA) as a transmitter. Major efferent pathways from the globus pallidus interna and the SNr project to the thalamus. Feedback to the striatum is through the dopaminergic striatonigral pathway originating in the substantia nigra pars compacta (SNc; Figure 1). These separate pathways utilize different neuropeptides and dopamine receptors.

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  • Parkinsonism and Freezing Gait Parkinson's disease (Chap. 366) is common, affecting 1% of the population 55. The stooped posture and shuffling gait are characteristic and distinctive features. Patients sometimes accelerate (festinate) with walking or display retropulsion. There may be difficulty with gait initiation (freezing) and a tendency to turn en bloc. Imbalance and falls may develop as the disease progresses over years.

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