a 67-year - old man was admitted to our hospital presenting gait disturbance and facial dystonia . his wife noted that he showed nonfluent speech having difficulty in naming objects and abnormal behavior such as hyperphagia for the first time 6 years previously .
thereafter , tremor in the lower limbs , more dominantly in the left leg , gait disturbance and postural instability insidiously progressed for 4 years . since the age of 65
he had been known to be a very gentle and quiet person , but now often showed temperament .
one year previously , he began to develop stooped posture and left shuffled gait which gradually worsened .
by then , his movements slowed considerably , his gait became unsteady , and he fell frequently . a trial of levodopa ( 200 mg/ day ) was prescribed dose subsequently increased to 600 mg per day over one year .
however , no objective signs of improvement showed despite increased dose of levodopa , and involuntary facial dystonia began to develop . neurological examination taken after the levodopa medication revealed severe facial dystonia .
involuntary facial grimacing usually sustained around both eyes accompanied with hypertonicity in facial muscles and deep facial folds .
body bradykinesia , axial rigidity and rest tremor in both limbs , and rest tremor in both arms and legs , which was more severe on the left limbs , were shown .
the motor score of the unified parkinson s disease rating scale ( updrs ) scored 59 . despite no definite supranuclear palsy , optokinetic nystagmus was not found .
after the discontinuation of levodopa , motor score of updrs dropped to 38 points and facial dystonia completely disappeared .
when answering questions or having conversation , he showed groping speeches , intermittent palilalia and severe anomia .
a month later , levodopa administration restarted and doses were gradually increased to 150 mg per day .
results of routine laboratory investigations for dementia with parkinsonism and gene ( mapt and progranulin ) study for ftd were normal .
fourteen months later , at the age of 68 years , he became completely unable to walk even with assistance .
results of neuropsychologic assessment performed 3 years previously showed naming difficulty ( 12.1 percentile ) and severely impaired frontal executive function ( 2.68 percentile ) .
follow - up neuropsychologic assessment in our laboratory also showed a more significant decline in boston naming test result ( 4.85 percentile ) and frontal executive function ( 0.02 percentile ) than the past examination .
in addition , the performance of visuospatial function evaluated with the rey complex copy test and memory ability evaluated with auditory verbal learning test marked several standard deviations below those of normal control subjects . because of facial dystonia , he seldom spoke during hospitalization .
he underwent the korean version of the western aphasia battery ( kwab ) after the discontinuation of levodopa .
magnetic resonance images of the brain showed markedly bilateral frontotemporal lobe , midbrain and cereballar atrophy ( figure 1 ) .
f - fluorodeoxyglucose positron emission tomography showed hypometabolism definitely in the bilateral frontotemporal area ( figure 2 ) .
i - metaiodobenzylguanidine scintigraphy revealed normal early and delayed h / m ratios ( early h / m ratio : 1.91 , delayed h / m ratio : 2.38 ) .
a 67-year - old man was admitted to our hospital presenting gait disturbance and facial dystonia . his wife noted that he showed nonfluent speech having difficulty in naming objects and abnormal behavior such as hyperphagia for the first time 6 years previously .
thereafter , tremor in the lower limbs , more dominantly in the left leg , gait disturbance and postural instability insidiously progressed for 4 years . since the age of 65
he had been known to be a very gentle and quiet person , but now often showed temperament .
one year previously , he began to develop stooped posture and left shuffled gait which gradually worsened .
by then , his movements slowed considerably , his gait became unsteady , and he fell frequently . a trial of levodopa ( 200 mg/ day ) was prescribed dose subsequently increased to 600 mg per day over one year .
however , no objective signs of improvement showed despite increased dose of levodopa , and involuntary facial dystonia began to develop . neurological examination taken after the levodopa medication revealed severe facial dystonia .
involuntary facial grimacing usually sustained around both eyes accompanied with hypertonicity in facial muscles and deep facial folds .
body bradykinesia , axial rigidity and rest tremor in both limbs , and rest tremor in both arms and legs , which was more severe on the left limbs , were shown .
the motor score of the unified parkinson s disease rating scale ( updrs ) scored 59 . despite no definite supranuclear palsy , optokinetic nystagmus was not found .
after the discontinuation of levodopa , motor score of updrs dropped to 38 points and facial dystonia completely disappeared .
when answering questions or having conversation , he showed groping speeches , intermittent palilalia and severe anomia .
a month later , levodopa administration restarted and doses were gradually increased to 150 mg per day .
results of routine laboratory investigations for dementia with parkinsonism and gene ( mapt and progranulin ) study for ftd were normal .
fourteen months later , at the age of 68 years , he became completely unable to walk even with assistance .
results of neuropsychologic assessment performed 3 years previously showed naming difficulty ( 12.1 percentile ) and severely impaired frontal executive function ( 2.68 percentile ) . verbal and visual memory , attention , and visuospatial function were still preserved .
follow - up neuropsychologic assessment in our laboratory also showed a more significant decline in boston naming test result ( 4.85 percentile ) and frontal executive function ( 0.02 percentile ) than the past examination .
in addition , the performance of visuospatial function evaluated with the rey complex copy test and memory ability evaluated with auditory verbal learning test marked several standard deviations below those of normal control subjects . because of facial dystonia , he seldom spoke during hospitalization .
he underwent the korean version of the western aphasia battery ( kwab ) after the discontinuation of levodopa .
magnetic resonance images of the brain showed markedly bilateral frontotemporal lobe , midbrain and cereballar atrophy ( figure 1 ) .
f - fluorodeoxyglucose positron emission tomography showed hypometabolism definitely in the bilateral frontotemporal area ( figure 2 ) .
i - metaiodobenzylguanidine scintigraphy revealed normal early and delayed h / m ratios ( early h / m ratio : 1.91 , delayed h / m ratio : 2.38 ) .
in addition , anomia and impaired repetition , which were documented by kwab , were supportive of the diagnostic features of pnfa.2 therefore , this initially led us to clinically suspect his condition as ftd . on the other hand , the mapt and progranulin ,
the most popular genes in the ftd,13 were not found . despite the lack of typical clinical symptoms of psp in the early stage ,
this patient showed some positive features that conform to the criteria,1 which are symmetric proximal parkinsonism , retrocolic posture , poor response of levodopa , and early onset cognitive impairment including frontal dysfunction . over the six years from the symptom onset , gait disturbance and falling gradually became more severe and extraocular movement impairment began to show ( from initial negative optokinetic nystagmus to supranuclear palsy eventually ) .
facial dystonia in this patient was thought to be a motor complication from levodopa therapy and could be considered as a form of levodopa - induced dyskinesia ( lid ) .
the development of lid in parkinson s disease usually depends on neuroplastic changes in the basal ganglia circuitry caused by degeneration of nigrostriatal dopaminergic neurons after the prolonged levodopa treatment.14 in contrast , the rarity of dyskinesias in psp is associated with multilevel damage in the nigrostriatal and striatopallidal systems.15 abnormal movements of this patient included facial dystonia , which occurred when the patient was on levodopa medication . in conclusion
although lifd was rarely reported in psp , our case suggested that lifd could be considered as an atypical feature of psp .
 progressive supranuclear palsy ( psp ) is frequently misdiagnosed as other parkinsonism because of clinical heterogeneity of psp . 
 we present here a case of a 67-year - old male patient with frontotemporal dementia - like cognitive impairment including language difficulties and abnormal behaviors . 
 he showed severe facial dystonia after the levodopa treatment . 
 herein , we describe an unusual case of a patient presenting with psp which , we believe could contribute to our knowledge about atypical leveodopa - induced facial dystonia in psp . 
Case
Clinical course
Neuropsychologic evaluation
Neuroimaging studies
Discussion