Factor Stewart A, Jennings Danna L, Molho Eric S, Marek Kenneth L
Parkinson's Disease and Movement Disorder Center of Albany Medical Center, 215 Washington Ave Extension, Albany, NY 12203, USA.
Arch Neurol. 2002 Nov;59(11):1778-83. doi: 10.1001/archneur.59.11.1778.
Primary progressive freezing gait disorder is considered to be a distinct clinical entity that manifests predominantly as a progressive freezing gait disorder without accompanying abnormalities. However, confusion remains about its clinical presentation, natural history, and classification.
To examine the natural history, clinical and brain imaging characteristics, and response to dopaminergic medications of primary progressive freezing gait (PPFG) disorder.
DESIGN/METHODS: Review of medical records, videotape examinations, and computed tomographic and magnetic resonance imaging of the brain and results of neurological evaluations, including the Unified Parkinson's Disease Rating Scale, in patients with PPFG.
Thirty patients (16 male) were diagnosed as having PPFG (mean age at onset, 72.2 years; mean duration of disease, 5 years). Gait disorder was the initial complaint in 27 patients. Freezing gait was the initial manifestation in 18 and was present within the first year in 27. Natural history included 25 patients falling within 3 years of onset, 20 experiencing retropulsion within 4 years, and 16 requiring wheelchairs by 5 years. On neurological examination, bradykinesia was present in 29 patients, muscle rigidity in 15, and postural tremor in 11. Other features included speech abnormalities in 10, hyperreflexia without clonus in 17, and dementia in 8. Extraocular movement abnormalities and dysphagia were rare. All 30 patients were treated with levodopa with minimal effect. Eighteen were treated with a dopamine agonist with no notable effect. Of the 23 patients with magnetic resonance imaging scans, results were normal in 9 and included minor nonspecific changes in 14. The computed tomographic scans obtained in 12 patients showed similar results. One patient underwent fluorine F ((18)F) labeled deoxyglucose positron emission tomography, which showed mild reduction in medial frontal glucose metabolism.
Primary progressive freezing gait appears to be a clinically distinct progressive neurological disorder that primarily affects gait, initially resulting in freezing and later in postural instability. A wheelchair-bound state often develops within 5 years. It is accompanied by other parkinsonian features, particularly bradykinesia, but is unresponsive to dopaminergic medications. It progresses in a fairly stereotyped manner. Primary progressive freezing gait disorder should be a unifying term for this disorder that has gone by many names in the literature and should be classified as a Parkinson-plus disorder.
原发性进行性冻结步态障碍被认为是一种独特的临床实体，主要表现为进行性冻结步态障碍，无伴随异常。然而，关于其临床表现、自然史和分类仍存在混淆。
研究原发性进行性冻结步态（PPFG）障碍的自然史、临床和脑影像学特征以及对多巴胺能药物的反应。
设计/方法：回顾PPFG患者的病历、录像检查、脑部计算机断层扫描和磁共振成像以及神经学评估结果，包括统一帕金森病评定量表。
30例患者（16例男性）被诊断为患有PPFG（平均发病年龄72.2岁；平均病程5年）。27例患者以步态障碍为首发症状。18例以冻结步态为首发表现，27例在发病第一年内出现冻结步态。自然史包括25例患者在发病3年内跌倒，20例在4年内出现后冲现象，16例在5年内需要轮椅。神经学检查显示，29例患者有运动迟缓，15例有肌肉僵硬，11例有姿势性震颤。其他特征包括10例言语异常，17例无阵挛的反射亢进，8例痴呆。眼球运动异常和吞咽困难少见。所有30例患者均接受左旋多巴治疗，效果甚微。18例接受多巴胺激动剂治疗，无明显效果。23例进行磁共振成像扫描的患者中，9例结果正常，14例有轻微非特异性改变。12例患者的计算机断层扫描结果相似。1例患者接受氟F（（18）F）标记的脱氧葡萄糖正电子发射断层扫描，显示内侧额叶葡萄糖代谢轻度降低。
原发性进行性冻结步态似乎是一种临床上独特的进行性神经疾病，主要影响步态，最初导致冻结，随后导致姿势不稳。通常在5年内发展为依赖轮椅状态。它伴有其他帕金森病特征，尤其是运动迟缓，但对多巴胺能药物无反应。它以相当固定的方式进展。原发性进行性冻结步态障碍应该是该疾病的一个统一术语，该疾病在文献中有许多名称，应归类为帕金森叠加综合征。
