J Clin Neurol.  2010 Sep;6(3):148-151. 10.3988/jcn.2010.6.3.148.

Behavioral Changes as the Earliest Clinical Manifestation of Progressive Supranuclear Palsy

• Han HJ
• Kim H
• Park JH
• Shin HW
• Kim GU
• Kim DS
• Lee EJ
• Oh HE
• Park SH
• Kim YJ

• KMID: 1462851
• DOI: http://doi.org/10.3988/jcn.2010.6.3.148

Abstract

BACKGROUND
The clinical and pathological heterogeneity of progressive supranuclear palsy (PSP) is well established. Even with a well-defined clinical phenotype and a thorough laboratory workup, PSP can be misdiagnosed, especially in its early stages.
CASE REPORT
A 52-year-old woman, who we initially diagnosed with a behavioral variant of frontotemporal dementia developed parkinsonian features, which then progressed to gait instability and gaze abnormality.
CONCLUSIONS
We report herein a pathologically confirmed case of PSP presenting with behavioral changes including agitation and irritability, which eventually led to the cardinal symptoms of progressive supranuclear palsy.

Keyword

frontotemporal dementia; parkinsonism; progressive supranuclear palsy

MeSH Terms

Dihydroergotamine
Female
Frontotemporal Dementia
Gait
Humans
Middle Aged
Parkinsonian Disorders
Phenotype
Population Characteristics
Supranuclear Palsy, Progressive
Dihydroergotamine

Figure

• 

  Fig. 1 A: Brain magnetic resonance imaging shows diffuse brain atrophy on fluid-attenuated inversion recovery images. B: Brain 18F-fluorodeoxyglucose positron emission tomography reveals subtly decreased glucose metabolism in the right prefrontal and both posterior frontal areas.

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  Fig. 2 A: Brain MRI shows marked bilateral temporal and midbrain atrophy with ventricular dilatation that is worse in the right temporal lobe than in the left temporal lobe on fluid-attenuated inversion recovery images. B: Brain 18F-fluorodeoxyglucose positron emission tomography reveals decreased glucose metabolism in the bilateral frontotemporal and parietal areas, with the decrease being in the right temporal lobe than in the left tempopral lobe.

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  Fig. 3 Brain biopsy of the temporal and frontal cortices. The immunohistochemical staining for (A) tau and (B) glial fibrillary acidic protein shows tau-positive globose tangles (×400) and reactive gliosis with tufted astrocytes (×400).

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