Corticobasal syndrome is a disorder of movement, cognition and behaviour with several possible underlying pathologies, including corticobasal degeneration. It presents insidiously and is slowly progressive. Clinicians should consider the diagnosis in people presenting with any combination of extrapyramidal features (with poor response to levodopa), apraxia or other parietal signs, aphasia and alien-limb phenomena. Neuroimaging showing asymmetrical perirolandic cortical changes supports the diagnosis, while advanced neuroimaging may give insight into the underlying pathology. Identifying corticobasal syndrome carries some management implications (especially if protein-based treatments arise in the future) and prognostic significance. Its treatment is largely symptomatic and is best undertaken within a multidisciplinary setting, including a neurologist, physiotherapist, occupational therapist, speech language therapist, psychiatrist and, ultimately, a palliative care clinician. Corticobasal syndrome can be a confusing entity for neurologists, not least because it has over time evolved from being considered predominantly as a movement disorder to a condition spanning a wide range of cognitive and motor manifestations. In this practical review, we attempt to disentangle this syndrome and provide clarity around diagnosis, its underlying pathological substrates, key clinical features and potential treatments.

中文翻译：

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皮质基底膜综合征：实用指南

皮质基底节综合征是一种运动、认知和行为障碍，具有多种可能的潜在病理，包括皮质基底节变性。它阴险地出现并且是缓慢进步的。临床医生应考虑对锥体外系特征（对左旋多巴反应不佳）、失用症或其他顶叶体征、失语症和异肢现象的任意组合进行诊断。神经影像学显示不对称的perirolandic 皮质变化支持诊断，而先进的神经影像学可以深入了解潜在的病理学。识别皮质基底细胞综合征具有一些管理意义（特别是如果未来出现基于蛋白质的治疗）和预后意义。它的治疗主要是对症的，最好在多学科环境中进行，包括神经科医生、物理治疗师、职业治疗师、语言治疗师、精神科医生，最后是姑息治疗临床医生。皮质基底膜综合征对于神经科医生来说可能是一个令人困惑的实体，尤其是因为随着时间的推移，它已经从主要被认为是一种运动障碍演变为一种涵盖广泛认知和运动表现的病症。在这篇实用的综述中，我们试图解开这种综合征，并围绕诊断、其潜在的病理学基础、关键的临床特征和潜在的治疗方法提供清晰的信息。尤其是因为随着时间的推移，它已经从主要被认为是一种运动障碍发展为一种涵盖广泛认知和运动表现的病症。在这篇实用的综述中，我们试图解开这种综合征，并围绕诊断、其潜在的病理学基础、关键的临床特征和潜在的治疗方法提供清晰的信息。尤其是因为随着时间的推移，它已经从主要被认为是一种运动障碍发展为一种涵盖广泛认知和运动表现的病症。在这篇实用的综述中，我们试图解开这种综合征，并围绕诊断、其潜在的病理学基础、关键的临床特征和潜在的治疗方法提供清晰的信息。