A patient with diffuse Lewy body disease presented with supranuclear vertical and horizontal ophthalmoplegia, dementia, axial rigidity and falls, bradykinesia and pyramidal signs. This broadens the clinical presentation of this pathological diagnosis and re-emphasises the heterogeneity of patients diagnosed clinically as progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).

Conventional single tensor diffusion analysis models have provided mixed findings in the substantia nigra of Parkinson's disease, but recent work using a bi-tensor analysis model has shown more promising results. Using a bi-tensor model, free-water values were found to be increased in the posterior substantia nigra of Parkinson's disease compared with controls at a single site and in a multi-si...

Patients with corticobasal degeneration can present with several different clinical syndromes, making ante-mortem diagnosis a challenge. Corticobasal syndrome is the clinical phenotype originally described for corticobasal degeneration, characterized by asymmetric rigidity and apraxia, cortical sensory deficits, dystonia and myoclonus. Some patients do not develop these features, but instead ha...

概 要PSP排 泄 試験に含 まれ る腎血流 以外 の副次 的な情報を解 析 し,PSPの 腎排 泄に お よぼす各 種 因子 の影響 を定量 的に把握 す るため,PSP排 泄 のkineticsの模擬 を試 みた.ま ず動 物実 験 の結 果, PSPは 体内で二 相分布を とる ことが判 明 した ので,こ れに基づ き数学 モデルを作成 し,電 子計算機 に よ りsimulationを 行 なつた ところ,そ の応答曲 線 は動物実験 に よる実 測値 とよ く一 致 し,モ デル の 妥 当性を裏づ け る成績を得 た.そ こでモデル の医学 的意義 を検 討 した結果,PSP分 布 の第1相 は循 環血漿,第2相 は肝腸系 と推定 され,ま た移行係 数k1, k2, k3は それぞれ腎 か らの排泄 能,肝 の異物 排 泄 能,腸 管吸 収を意味す るもの と考 え ...

A 70-year-old woman presented with a 2-year history of progressive difficulty in walking with frequent falls. Neurologic examination showed postural instability with backward falls, vertical supranuclear gaze palsy with normal vestibular-ocular reflex, rigidity, and pyramidal signs in the right limbs. There was no clinical response to levodopa. Laboratory serologic tests had normal results. MRI...

We compared image restoration methods [Richardson-Lucy (RL), Wiener, and Next-image] with measured "scatter" point-spread-functions, for removing subsurface fluorescence from section-and-image cryo-image volumes. All methods removed haze, delineated single cells from clusters, and improved visualization, but RL best represented structures. Contrast-to-noise and contrast-to-background improvemen...

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...

We report a patient with progressive supranuclear palsy (PSP) with his serial photographs before the onset of ocular symptoms and after the onset with two year intervals. These photographs show his progressive eyeball deviations toward complete exotropia. There were no effective voluntary eyeball movements, Bell's phenomenon, doll's eye movements, and vestibulo-ocular reflexes. These signs indi...

Multiple system atrophy is a progressive neurodegenerative disorder that is characterized by autonomic failure, cerebellar ataxia and parkinsonism syndrome in various combinations. In spite of the presence of well-established clinical criteria for multiple system atrophy, ante-mortem diagnosis is difficult. In our case report, we present a 78-year-old female patient who presented with early pro...

Three patients with progressive supranuclear palsy (PSP) with different symptoms at onset presented intense elementary motor perseveration before the appearance of the distinctive features of the illness. In elementary motor perseveration once an element of a movement has begun it is no longer inhibited at the right time and continues unchecked. Perseverations were observed in these patients du...