Clinical features of dystonia in atypical parkinsonism

Abstract

-Background:The association between Dystonia and Parkinson's disease (PD) has been well described especially for foot and hand dystonia. There is however few data on dystonic postures in patients with atypical parkinsonism. Objective: To evaluate the frequency and pattern of dystonia in a group of patients with atypical parkinsonism (multiple system atrophy -MSA, progressive supranuclear palsy -PSP, and corticobasal degeneration -CBD) and to investigate whether dystonia could be the first presenting symptom at disease onset in those patients. Method: A total of 38 medical charts were reviewed (n=23/MSA group; n=7/CBD group; n=8/PSP group) and data values were described as means/standard deviations. The variables evaluated were sex, age at onset, disease duration, first symptom, clinical features of dystonia and other neurological signs, response to levodopatherapy, Hoehn&Yahr scale >3 after three years of disease, and magnetic resonance imaging findings. Results: The overall frequency of dystonia in our sample was 50% with 30.4% (n=7) in the MSA group, 62.5% (n=5) in the PSP group, and 100% (n=8) in the CBD group. In none of these patients, dystonia was the first complaint. Several types of dystonia were found: camptocormia, retrocollis, anterocollis, blepharoespasm, oromandibular, and foot/hand dystonia. Conclusion: In our series, dystonia was a common feature in atypical parkinsonism (overall frequency of 50%) and it was part of the natural history although not the first symptom at disease onset. Neuroimaging abnormalities are not necessarily related to focal dystonia, and levodopa therapy did not influence the pattern of dystonia in our group of patients.KeY worDS: dystonia, atypical parkinsonism, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, Parkinson's disease. Características clínicas da distonia no parkinsonismo atípicoResumo -Introdução: A associação de distonia e doença de Parkinson (DP) já foi bem estabelecida, principalmente para distonia focal em pé ou mão. entretanto, há poucos dados quanto a distonia em pacientes com parkinsonismo atípico. Objetivo: Avaliar a freqüência e o padrão da distonia em um grupo de pacientes com parkisnonismo atípico (atrofia de múltiplos sistemas -AMS; paralisia supranuclear progressiva -PSP; degeneração corticobasal -DCB) e investigar se a distonia pode ser a manifestação inicial neste grupo. Método: Um total de 38 prontuários médicos foi revisado (n=23/grupo AMS; n=8/grupo PSP; n=7/grupo PSP) e os dados foram apresentados em médias/desvios padrões. As variaveis avaliadas foram: sexo, idade de início, duração da doença, primeiro sintoma, características clínicas da distonia e outros sinais neurológicos, resposta ao tratamento com levodopa, escala de Hoehn & Yahr >3 em 3 anos de doença, e achados de ressonância magnética. Resultados: A frequência total de distonia em nosso grupo foi 50%, sendo 30,4% (n=7) no grupo AMS, 62.5% (n=5) no grupo PSP e 100% (n=8) no grupo DCB. em nenhum dos pacientes, distonia foi o primeiro sintoma...

Corticobasal Degeneration

“…1 Other studies, however, report a higher frequency of dystonia, probably due to a combination of referral bias and mixed populations of clinically and pathologically proven cases. 57,58 Dystonia in CBS typically occurs within 2 years from disease onset, affecting most commonly the upper limb, and then may evolve to hemidystonia or also affect the other side, but rarely starting on the leg. 56 The classic distribution of dystonia includes adduction and flexion of the arm, forearm, wrist, and metacarpophalangeal joints with extension of interphalangeal joints, whereas in the leg, the hip can be flexed and internally rotated, associated with flexion of the knee and foot inversion.…”

Oromandibular dystonia in encephalitis

“…OMD may be due to medications (neuroleptics, prokinetic, anticancer), neurodegenerative and neurodevelopmental disorders (neuroacanthocytosis, Hallervorden-Spatz disease, Wilson disease, neuroferritinopathy, progressive supranuclear palsy, Rett syndrome, Down's syndrome, Joubert syndrome, Angelman syndrome), metabolic disorders (LeschNyhan syndrome, Gaucher disease), infections (varicella), stroke, status epilepticus, post-anoxic, post-traumatic, primary generalized dystonia, episodic focal lingual dystonic spasms and idiopathic [1][2][3][4][5][6][7][8][9]. Japanese encephalitis (JE) is the most important endemic encephalitis in South East Asia.…”

Progressive supranuclear palsy: new concepts

“…According to our previous published experience the overall frequency of dystonia in Brazilian patients with atypical parkinsonian syndromes is of 50% 46 . Therefore, botulinum toxin may be helpful in PSP for treating dystonia, such as retrocollis (Fig 1), and apraxia of eyelid opening, reducing disability provoked by these symptoms 36 .…”