The Natural History of the Syndrome of Primary Progressive Freezing Gait

Abstract

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.

Primary Progressive Freezing: does it stand on its own?

“…Several studies suggest that PPF is part of the spectrum disease of progressive supranuclear palsy (PSP) [1,2,7,8,12], particularly the Pure-akinesia subtype [1,12]. In these patients, although freezing of gait may be the presenting feature, they latter show micrography, abnormalities of speech, axial rigidity, vertical supranuclear gaze palsy and postural instability [13].…”

“…Primary progressive freezing (PPF) is a gait disorder characterized by freezing of gait (FOG) [1,2]. It is found during gait initiation, turning and overcoming obstacles [3,4], resulting in falls [1].…”

“…Disturbances in frontal cortical regions, the basal ganglia, and the midbrain locomotor region have been implicated [5]. The nigrostriatal dopaminergic system is thought not to be involved, and the affected patients do not respond to dopaminergic medications [1].…”

“…Primary progressive freezing was first described in 1993 [6] and various names were used to describe it [1,7]. Furthermore, distinct criteria were used (some studies consider parkinsonian features as part of the clinical syndrome [1,2] while others consider only isolated freezing of gait [7]).…”

“…Furthermore, distinct criteria were used (some studies consider parkinsonian features as part of the clinical syndrome [1,2] while others consider only isolated freezing of gait [7]). This entity was considered part of the Parkinson-plus syndromes [1,2] but recent papers [2,3,8] suggested that it is caused by other neurodegenerative diseases, such as progressive supranuclear palsy, corticobasal degeneration, dementia with Lewy bodies, pallidonigroluysian degeneration [7], primary lateral sclerosis [9].…”