Neurology Department, Hospital Pedro Hispano/Unidade Local de Saúde de Matosinhos, Matosinhos, Portugal.
Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
Mov Disord. 2021 Sep;36(9):2017-2025. doi: 10.1002/mds.28663. Epub 2021 May 31.
The landscape of genetic forms of Parkinson's diseases (PD) has grown exponentially in recent years. Today, around 10% of PD cases are estimated to be of genetic etiology. However, the link between parkinsonism or tremor and chromosome disorders, both numerical and structural, has been neglected. We reviewed the occurrence and characteristics of parkinsonism and tremor syndromes in patients with chromosomic disorders. We searched PubMed for articles published until December 2018, using the non-MESH terms "Chromosomopathy," "karyotype," "chromosome," "aneuploidy," "deletion," "inversion," "insertion," "duplication," and "Parkinson," "Parkinsonism," "Tremor," and "Parkinsonian disorder." We restricted the search to human studies and selected articles for further analysis after abstract review. Tremor syndromes in which patients had another possible clinical reason for syndromes were excluded, as well as tremor syndromes associated with point mutations, imprinting syndromes, and patients presenting with other hyperkinetic disorders. Fifty-four articles were reviewed. Aneuploidies of sex chromosomes were the most common chromosomopathy. These patients more commonly exhibited postural and kinetic tremor, often meeting the description of essential tremor. In structural chromosomopathies, the most frequent association was PD and 22q11.2 deletion syndrome, but we found case reports and case series of several additional deletion and duplication syndromes. © 2021 International Parkinson and Movement Disorder Society.
近年来,帕金森病(PD)的遗传形式的研究迅速发展。目前,约有 10%的 PD 病例被认为具有遗传病因。然而,染色体疾病,无论是数目异常还是结构异常,与帕金森综合征或震颤之间的联系一直被忽视。我们回顾了染色体疾病患者中帕金森综合征和震颤综合征的发生和特征。我们在 PubMed 中使用非 MeSH 术语“染色体病”、“核型”、“染色体”、“非整倍体”、“缺失”、“倒位”、“插入”、“重复”和“帕金森病”、“帕金森综合征”、“震颤”以及“帕金森病障碍”进行了文献检索。我们将搜索范围限制为人类研究,并在摘要审查后选择进一步分析的文章。排除了震颤综合征患者有其他可能的临床原因,以及与点突变、印迹综合征和其他多动障碍相关的震颤综合征。共回顾了 54 篇文章。性染色体非整倍体是最常见的染色体病。这些患者更常表现出姿势性和运动性震颤,通常符合特发性震颤的描述。在结构性染色体病中,最常见的关联是 PD 和 22q11.2 缺失综合征,但我们发现了几种额外的缺失和重复综合征的病例报告和病例系列。 © 2021 国际帕金森病和运动障碍学会。