Dystonia in corticobasal degeneration: A review of the literature on 404 pathologically proven cases

Abstract

A B S T R A C T :Dystonia is considered one of the classical features of corticobasal degeneration and is reported in up to 83% in clinical, not pathologically confirmed, series. Here, we aimed to establish the frequency and the clinical characteristics of dystonia in CBD by reviewing the literature on 404 pathologically proven cases. Further, we aimed to identify the frequency and characteristics of dystonia in all described phenotypes with CBD pathology. Dystonia was present in only 37.5% of the 296 cases with adequate information. The majority of the cases with dystonia presented with a corticobasal syndrome, and dystonia occurred in the first 2 years from disease onset, affecting the upper limb. In cases with dystonia that presented with a ''dementia'' phenotype, dystonia tended to appear later in the disease course and to more affect the cervical region and the face. With regard to the distribution of the phenotypes, fifty-four percent of 374 cases presented as corticobasal syndrome, 15% as frontotemporal dementia, and 10.7% as progressive supranuclear palsy. Dystonia and myoclonus were present in about half of all cases with corticobasal syndrome, implying that these features may not be as frequent in corticobasal syndrome as are akinetic-rigid syndrome and apraxia (100% and 86.3%, respectively). Dystonia and myoclonus almost co-occurred in our analysis, suggesting a possible association. In conclusion, despite dystonia being an inclusion criterion in all sets of clinical criteria for corticobasal degeneration, this was present in only one third of the pathologically proven cases presented here. More accurate characterization of dystonia in corticobasal degeneration would be of importance for clinical diagnosis and development of treatment strategies. V C 2012 Movement Disorder Society

Parkinsonian Syndromes

“…Dystonia and myoclonus are less frequent than the akinetic-rigid syndrome and apraxia. 43 Alien-limb phenomenon is seen in some patients and identified by involuntary grasping, purposeless movements, or levitation in an apraxic limb. When CBS affects the right extremities, it is more likely to be associated with a nonfluent aphasia, whereas, when affecting left extremities, it may associate at onset with visuospatial and visuoconstructive deficits.…”

Atypical Parkinsonism

“…[3][4][5][6][7][8] Moreover, the early differential diagnosis is complicated by patients with pathologically proven PSP, CBD, or MSA that may present clinically with phenotypes other than the classic ones. [9][10][11][12] Conversely, patients with the classic AP phenotypes may turn out to have other pathologic abnormalities. [9][10][11][12] Here, a guide on how to diagnose these conditions is provided and the limited available treatment options are discussed.…”

“…[9][10][11][12] Conversely, patients with the classic AP phenotypes may turn out to have other pathologic abnormalities. [9][10][11][12] Here, a guide on how to diagnose these conditions is provided and the limited available treatment options are discussed.…”

Possible Treatments of Atypical Parkinsonism

“…Although not sufficiently studied, botulinum toxin injections can be helpful to relieve dystonic spasms of the hand or to treat levator inhibition [30]. Early clinical trials in PSP employed drug treatment to correct alterations in the dopaminergic, cholinergic, and gamma-amino butyric acid (GABA)ergic pathways and produced limited evidence of benefit.…”