Dopa-responsive dystonia: Clinical and family study in Taiwanese

Rou Shayn Chen; Chin Chang Huang; Chin Song Lu

doi:10.1016/0303-8467(95)00081-X

Dopa-responsive dystonia: Clinical and family study in Taiwanese

Rou Shayn Chen, Chin Chang Huang^*, Chin Song Lu

^*Corresponding author for this work

Taipei Medical University

Research output: Contribution to journal › Journal Article › peer-review

5 Scopus citations

Abstract

We report the clinical features of four female patients with dopa-responsive dystonia (DRD), and the survey of the family members. The patients were 2 sisters and 2 sporadic patients from 3 families. Their age of onset ranged between 5 and 13 years. The clinical manifestation was characterized by limb dystonia which was relieved by L-dopa treatment. Diurnal fluctuation disappeared 15 years later in one patient. There was a wide spectrum of Parkinsonian features and variability of dystonia. Response of L-dopa was still excellent 20 years later. In survey of the family members, there was neither bradykinesia, rigidity, tremor nor dystonia.

Original language	English
Pages (from-to)	43-46
Number of pages	4
Journal	Clinical Neurology and Neurosurgery
Volume	98
Issue number	1
DOIs	https://doi.org/10.1016/0303-8467(95)00081-X
State	Published - 02 1996
Externally published	Yes

Keywords

Diurnal fluctuation
Dopa-responsive dystonia
Family survey
L-dopa
Parkinsonism
Segawa disease

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10.1016/0303-8467(95)00081-X

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title = "Dopa-responsive dystonia: Clinical and family study in Taiwanese",

abstract = "We report the clinical features of four female patients with dopa-responsive dystonia (DRD), and the survey of the family members. The patients were 2 sisters and 2 sporadic patients from 3 families. Their age of onset ranged between 5 and 13 years. The clinical manifestation was characterized by limb dystonia which was relieved by L-dopa treatment. Diurnal fluctuation disappeared 15 years later in one patient. There was a wide spectrum of Parkinsonian features and variability of dystonia. Response of L-dopa was still excellent 20 years later. In survey of the family members, there was neither bradykinesia, rigidity, tremor nor dystonia.",

keywords = "Diurnal fluctuation, Dopa-responsive dystonia, Family survey, L-dopa, Parkinsonism, Segawa disease",

author = "Chen, {Rou Shayn} and Huang, {Chin Chang} and Lu, {Chin Song}",

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T2 - Clinical and family study in Taiwanese

AU - Chen, Rou Shayn

AU - Huang, Chin Chang

AU - Lu, Chin Song

PY - 1996/2

Y1 - 1996/2

N2 - We report the clinical features of four female patients with dopa-responsive dystonia (DRD), and the survey of the family members. The patients were 2 sisters and 2 sporadic patients from 3 families. Their age of onset ranged between 5 and 13 years. The clinical manifestation was characterized by limb dystonia which was relieved by L-dopa treatment. Diurnal fluctuation disappeared 15 years later in one patient. There was a wide spectrum of Parkinsonian features and variability of dystonia. Response of L-dopa was still excellent 20 years later. In survey of the family members, there was neither bradykinesia, rigidity, tremor nor dystonia.

AB - We report the clinical features of four female patients with dopa-responsive dystonia (DRD), and the survey of the family members. The patients were 2 sisters and 2 sporadic patients from 3 families. Their age of onset ranged between 5 and 13 years. The clinical manifestation was characterized by limb dystonia which was relieved by L-dopa treatment. Diurnal fluctuation disappeared 15 years later in one patient. There was a wide spectrum of Parkinsonian features and variability of dystonia. Response of L-dopa was still excellent 20 years later. In survey of the family members, there was neither bradykinesia, rigidity, tremor nor dystonia.

KW - Diurnal fluctuation

KW - Dopa-responsive dystonia

KW - Family survey

KW - L-dopa

KW - Parkinsonism

KW - Segawa disease

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JO - Clinical Neurology and Neurosurgery

JF - Clinical Neurology and Neurosurgery

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Dopa-responsive dystonia: Clinical and family study in Taiwanese

Abstract

Keywords

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