Nonprimary dystonias

Abstract

Dystonias can be classified as primary or secondary, as dystonia-plus syndromes, and as heredodegenerative dystonias. Their prevalence is difficult to determine. In our experience 80-90% of all dystonias are primary. About 20-30% of those have a genetic background; 10-20% are secondary, with tardive dystonia and dystonia in cerebral palsy being the most common forms. If dystonia in spastic conditions is accepted as secondary dystonia, this is the most common form of all dystonia. In primary dystonias, the dystonic movements are the only symptoms. In secondary dystonias, dystonic movements result from exogenous processes directly or indirectly affecting brain parenchyma. They may be caused by focal and diffuse brain damage, drugs, chemical agents, physical interactions with the central nervous system, and indirect central nervous system effects. Dystonia-plus syndromes describe brain parenchyma processes producing predominantly dystonia together with other movement disorders. They include dopa-responsive dystonia and myoclonus-dystonia. Heredodegenerative dystonias are dystonic movements occurring in the context of other heredodegenerative disorders. They may be caused by impaired energy metabolism, impaired systemic metabolism, storage of noxious substances, oligonucleotid repeats and other processes. Pseudodystonias mimic dystonia and include psychogenic dystonia and various orthopedic, ophthalmologic, vestibular, and traumatic conditions. Unusual manifestations, unusual age of onset, suspect family history, suspect medical history, and additional signs may indicate nonprimary dystonia. If they are suspected, etiological clarification becomes necessary. Unfortunately, potential etiologies are legion. Diagnostic algorithms can be helpful. Treatment of nonprimary dystonias, with few exceptions, does not differ from treatment of primary dystonias. The most effective treatment for focal and segmental dystonias is local botulinum toxin injections. Deep brain stimulation of the globus pallidus internus is effective for generalized dystonia. Antidystonic drugs, including anticholinergics, tetrabenazine, clozapine, and gamma-aminobutyric acid receptor agonists, are less effective and often produce adverse effects. Dopamine is extremely effective in dopa-responsive dystonia. The Bertrand procedure can be effective in cervical dystonia. Other peripheral surgery, including myotomy, myectomy, neurotomy, rhizotomy, ramizectomy, and accessory nerve neurolysis, has largely been abandoned. Central surgery other than deep brain stimulation is obsolete. Adjuvant therapies, including orthoses, physiotherapy, ergotherapy, behavioral therapy, social support, and support groups, may be helpful. Analgesics should also be considered where appropriate.