We present a 77-year-old woman with levodopa-nonresponsive parkinsonism, dementia, and supranuclear gaze palsy on vertical and horizontal gaze. Laboratory findings were consistent with idiopathic hypoparathyroidism, and brain computed tomography showed extensive bilateral calcifications of the basal ganglia, centrum semiovale, dentate nuclei, and cerebellar white matter. These results illustrat...

Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease characterized by akinetic-rigid features, falls, a supranuclear gaze palsy and subcortical dementia. Pathologically, there is abnormal accumulation of tau protein. Cholinergic deficits are thought to underlie the postural instability and cognitive impairment of PSP, but trials of cholinergic agonists and cholinester...

Progressive supranuclear palsy (Steele et al.) has a characteristic pattern of dementia: (1) forgetfulness, (2) slowing of thought processes, (3) emotional or personality changes (apathy or depression with occasional outbursts of irritability), and (4) impaired ability to manipulate acquired knowledge. In many neurological disease states associated with subcortical pathology a similar pattern o...

BACKGROUND Progressive supranuclear palsy (PSP) is an akinetic-rigid syndrome of unknown aetiology which usually presents with a combination of unsteadiness, bradykinesia, and disordered eye movement. Speech often becomes dysarthric but language disorders are not well recognised. METHODS Three patients with PSP (pathologically confirmed in two) are reported in which the presenting symptoms we...

Progressive supranuclear palsy is a neurodegenerative disease which aVects the brainstem and basal ganglia. Patients present with disturbance of balance, a disorder of downward gaze and L-DOPA-unresponsive parkinsonism and usually develop progressive dysphagia and dysarthria leading to death from the complications of immobility and aspiration. Treatment remains largely supportive but, potential...

BACKGROUND The dysarthria of progressive supranuclear palsy consists of prominent hypokinetic and spastic components with less prominent ataxic components. OBJECTIVE To correlate the types of dysarthria with neuropathological changes in patients with progressive supranuclear palsy. DESIGN AND METHODS In 14 patients with progressive supranuclear palsy, we correlated the perceptual speech fin...

Clinical Features of Progressive Supranuclear Palsy Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterised by parkinsonism, supranuclear ophthalmoplegia, dysphagia and cognitive dysfunction. The National Institute of Neurological Disorders and Stroke (NINDS) PSP criteria are widely used for clinical diagnosis. However, the clinical phenotypes of pathologically confir...

mer's disease in kindreds with missense mutations in a gene on chromosome 1 related to the Alzheimer's disease type 3 gene. Estimation of the genetic contribution of presenilin-1 and-2 mutations in a population-based study of presenile Alzheimer disease. High prevalence of pathogenic mutations in patients with early-onset dementia detected by sequence analyses of four different genes. mutation ...

BACKGROUND Progressive supranuclear palsy (PSP) is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progressive apraxia of speech (AOS) and aphasia. AIM We aimed to investigate the clinical presentation of PSP, using a comprehensive multidimensional evaluation, and the disease response to va...

Progressive supranuclear palsy (PSP) is characterized by slowness, rigidity, bradykinesia, repeated falls, downgaze limitation and dementia. Midbrain atrophy on magnetic resonance imaging is highly suggestive of PSP and is described as "hummingbird sign". This sign is very helpful in differentiating PSP patients from those with Parkinson's disease.We hereby report a 72-year-old female case of P...