Tottori J. Clin. Res. 9(1), 38-46, 2017

Effects of short-term intensive rehabilitation admission in a PSP-PGF patientwith severe gait freezing
Makoto Sawada1) *, Yasuji Imai1), Tatsunori Murakami1), Mitsuru Doi2)
1) Department of Rehabilitation, NHO Tottori Medical Center2) Department of Neurology, NHO Tottori Medical Center*Correspondence: walkinstore43@gmail.com
Abstract
Among various types of progressive supranuclear palsy (PSP), pure akinesia with gait freezing (PAGF)(PSP-PGF, based on MDS-Clinical Diagnostic Criteria for PSP, 2016) causes freezing of gait (FOG) early after
onset, markedly reducing activities of daily living (ADL) and the quality of life (QOL). We report a PSP-PAGF
patient with severe FOG in whom short-term intensive rehabilitation admission reduced FOG.
[Case] The patient was a 67-year-old male with PSP-PAGF. In 2015, stiffness of the right hand and gait
disturbance appeared. In 2016, levodopa-/carbidopa-containing tablets and a Neupro patch™ (rotigotine) wereprescribed, but there was no efficacy. Although caregiving was not required in daily living, FOG was marked,making moving difficult. FOG appeared at the start of gait, on changing the direction, in a narrow place,
immediately before an object, and even on going straight ahead. Furthermore, FOG exacerbated in the presenceof cognitive load or mental stress. The new freezing of gait questionnaire (New FOG-Q) score was 26 points.
As a rehabilitation approach, external stimulation (vision/hearing/somatic sensation) was introduced (cueexercise). Learning of external cue methods reduced FOG, decreasing the New FOG-Q score to 22 points. Inparticular, FOG at the start of gait was relieved. However, FOG appeared in a narrow place or in the presence ofmental stress, and it was impossible to adopt external cue in some cases.
[Conclusion] Learning of external cue was effective for FOG in a patient with PSP-PAGF. However, it wasimpossible to adopt external cue under some circumstances; therefore, comprehensive approaches, such asenvironmental adjustment and mental support, may be necessary. Tottori J. Clin. Res. 9(1), 38-46, 2017
Key words: progressive supranuclear palsy (PSP), pure akinesia with gait freezing (PAGF) (PSP-PGF),
rehabilitation, cue exercise
Introduction
PSP is a neurodegenerative disordercharacterized by supranuclear vertical gaze palsy,dysphagia, speech disturbance, impaired posturalreflexes, nuchal dystonia, falling-prone features,and frontal cognitive dysfunction. In 1964, it was
first reported by Steele, Richardson, andOlszewski1). In 2005, Williams et al.2,3) reported
that pathologically diagnosed PSP could beclinicopathologicallyclassified
into
two
subtypes: typical PSP (PSP-Richardson’ssyndrome: PSP-R), which was described bySteele et al.1)), and PSP-parkinsonism (PSP-P),

which is difficult to differentiate from
Parkinson’s disease (PD). In 2007, Williams etal.4) reported the third subtype of PSP: PSP-pureakinesia with gait freezing (PAGF). In thissubtype, pure akinesia, for which L-dopa isineffective, FOG, or festinant speech appears
prior to other neurological signs, persisting for along period. Furthermore, the morbidity rate isvery low. Although pure akinesia had first beenreported by Imai and Narabayashi5) as a newsyndrome “dopa-unresponsive pure akinesia orfreezing”, which might have been a new disease
entity and been discussed as a subtype or an initi-