ABSTRACT
This chapter will review the pathophysiology of primary dystonia. Primary generalized dystonia of children may differ from the focal dystonias of adults, and it is important to keep in mind that most physiology has been done with focal dystonia. Anatomical pathology is usually described as normal, but in fact there is very little information. Recently, signs of neural degeneration have been found in the brainstem in patients with primary generalized dystonia (1). Anatomical speculation largely comes from secondary dystonia, and many abnormalities have been found in these pathways.Wewill focus mainly on neurophysiological investigation with electromyography (EMG), electroencephalography (EEG), and transcranial magnetic stimulation (TMS). Another chapter will cover neuroimaging findings of dystonia.
