The “Journal of Clinical Neurology” is published in Seoul by the Korean Neurological Association. Last month a case report was published about a 52-year-old woman with behavioral changes. She was thought to have the behavioral variant of frontotemporal dementia (bvFTD). Some of her symptoms were restlessness, insomnia, agitation, inappropriate behavior (meddling in other people’s affairs), and severe anxiety. Later, she developed a gait disturbance. She had no eye movement problems. The working diagnosis changed to FTD with parkinsonism. The clinicians “requested a genetic study for FTD and parkinsonism linked to chromosome 17 (FTDP-17), but this did not reveal a genetic mutation of microtubule associated protein tau.” Eventually vertical gaze problems appeared, and the woman was diagnosed with PSP.
A brain biopsy was done; the diagnosis was PSP. I’ve heard about brain biopsies being done in very rare instances. I was talking years ago to someone about this and they said: “you’re not going to remove any part of my loved one’s brain while he is still alive!”
The authors note that previous investigations of behavioral symptoms in PSP reveal “that apathy was the most frequent behavioral abnormality; …the presence of high apathy and low agitation scale scores correctly identified the patients with PSP. In contrast, in our case, agitation and disinhibition were the earliest and most prominent behavioral symptoms. … To the best of our knowledge, agitation or disinhibition as an initial symptom of PSP is very rare… Based on previous reports and our case, gait instability and gaze abnormality are not absolute requirements as presenting symptoms for a diagnosis of probable PSP, and the initial clinical diagnosis criteria are not mandatory.”
The article is available at no charge online.
Journal of Clinical Neurology. 2010 Sep;6(3):148-51. Epub 2010 Sep 30.
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.
Department of Neurology, Myongji Hospital, College of Medicine, Kwandong University, Goyang, Korea.
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.
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