We have experienced a rare case of progressive supranuclear palsy with hallucination and delusion. The common manifestations of progressive supranuclear palsy are gait disturbance, mental change and sign of vertical ophthamoplegia but no efficacious therapy has been known. Neuropsychiatric symptom clusters include cognitive impairment, affective and behavioral changes, sleep disturbance, and psychotic symptoms. Though schizophrenia-like psychosis has been reported but this is certainly rare. Addressing a case of progressive supranuclear palsy, in whom parkisonian symptoms appeared with concurrent psychotic symptoms, we emphasize accurate diagnosis.
Delusions ; Diagnosis ; Gait ; Hallucinations ; Psychotic Disorders ; Supranuclear Palsy, Progressive*

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 indicate the involvement of the oculomotor nuclear complex by the disease. We suggest that PSP may cause not only 'supranuclear' but also 'nuclear' complete ophthalmoplegia with exodeviation of the eyes.
Aged ; Exotropia/diagnosis ; Eye Movements ; Humans ; Magnetic Resonance Imaging ; Male ; Ophthalmoplegia/*diagnosis ; Supranuclear Palsy, Progressive/*diagnosis ; Time Factors ; Vision, Binocular

Neurodegenerative disorders cause a variety of dementia including Alzheimer disease, frontotemporal dementia, dementia with Lewy bodies, corticobasal degeneration, progressive supranuclear palsy, and Huntington's disease. PET scan is useful for early detection and differential diagnosis of these dementing disorders. Also, it provides valuable information about clinico-anatomical correlation, allowing better understanding of function of brain. Here we discuss recent achievements PET studies regarding these dementing disorders. Future progress in PET technology, new tracers, and image analysis will play an important role in further clarifying the disease pathophysiology and brain functions.
Alzheimer Disease* ; Brain ; Dementia* ; Diagnosis, Differential ; Frontotemporal Dementia ; Huntington Disease ; Lewy Bodies ; Neurodegenerative Diseases ; Positron-Emission Tomography ; Supranuclear Palsy, Progressive

Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2.3 years after the onset of ataxia. Mild cerebellar volume loss and midbrain atrophy were detected on brain imaging, which are supportive of a diagnosis of PSP. Videos are presented illustrating the co-existence of cerebellar signs and supranuclear gaze palsy and the disappearance of cerebellar signs with disease progression. Better recognition and the development of validated diagnostic criteria would aid in the antemortem recognition of this rare condition.
Ataxia ; Atrophy ; Cerebellar Ataxia* ; Cerebellar Diseases ; Diagnosis ; Disease Progression ; Humans ; Mesencephalon ; Neuroimaging ; Paralysis* ; Phenotype ; Retrospective Studies ; Supranuclear Palsy, Progressive

Clinical features of corticobasal degeneration (CBD) can be characterized by an asymmetric akinetic-rigid syndrome with variable combinations of other deficits (corticospinal tract signs, supranuclear gaze palsy, cerebellar ataxia, cortical sensory loss, alien limb behavior, dystonia and myoclonus). Such unique combination of clinical features of CBD have lead to the general agreement that clinical diagnosis of CBD is reliable. We describe 6 patients presenting with clinical features compatible with CBD. Three had characteristic clinical features of CBD; one showed clinical features compatible with progressive supranuclear palsy, but also had apraxia and cortical sensory disturbances; one had an early CBD. The remaining one had clinical features compatible with CBD, but brain magnetic resonance imaging study showed multiple small lesions involving periventricular white matter and basal ganglia bilaterally.
Apraxias ; Basal Ganglia ; Brain ; Cerebellar Ataxia ; Diagnosis ; Dystonia ; Emigrants and Immigrants ; Extremities ; Humans ; Magnetic Resonance Imaging ; Paralysis ; Supranuclear Palsy, Progressive

Parkinson's disease (PD) is a extrapyramidal movement disorder characterized by rigidity and bradykinesia. PD is one of the most common neurodegenerative disorders, affecting 1% of the population over the age of 60. Dementia is common and affects 40% of patients with PD during the course of the disease, the risk for the development of dementia being 6 times higher than in age-matched general population. In addition to motor abnormalities, there are several non-motor signs and symptoms that may create a considerable burden for patients and caregivers. Parkinsonism is a major feature of several dementing diseases. The parkinsonian disorders with dementia are Parkinson's disease dementia (PDD), parkinsonian-plus syndromes, sepcific heredodegenerative diseases, and secondary parkinsonisms. The parkinsonian-plus syndromes are neurodegenerative disorders charaterized by parkinsonism and at least one other nonparkinsonian neurological manfestation. This brief review concentrates on those disorders in which cognitive impairment/dementia and parkinsonism coexist: Parkinson's disease dementia, progressive supranuclear palsy, and corticobasal degeneration. The clinical and neuropsychological similarities and differences in these disorders are compared and contrasted along with Alzheimer's disease, Parkinson's disease, and dementia of Lewy bodies, highlighting the features critical for identifying the correct diagnosis.
Alzheimer Disease ; Caregivers ; Dementia* ; Diagnosis ; Humans ; Hypokinesia ; Lewy Bodies ; Movement Disorders ; Neurodegenerative Diseases ; Parkinson Disease ; Parkinsonian Disorders* ; Supranuclear Palsy, Progressive

OBJECTIVE: The provisional diagnosis of progressive supranuclear palsy (PSP) depends on a combination of typical clinical features and specific MRI findings, such as atrophy of the tegmentum in the midbrain. Atrophy of the superior cerebellar peduncle (SCP) distinguishes PSP from other types of parkinsonism. Histological factors affect the conventional fluid-attenuated inversion recovery (FLAIR) signals, such as the extent of neuronal loss and gliosis. METHODS: We investigated patients with PSP to verify the percentage of patients with various PSP phenotypes presenting a high signal intensity in the SCP. Three interviewers, who were not informed about the clinical data, visually inspected the presence or absence of a high signal intensity in the SCP on the FLAIR images. We measured the pixel value in the SCP of each patient. Clinical characteristics were evaluated using the Mann-Whitney test, followed by the χ² test. RESULTS: Ten of the 51 patients with PSP showed a high signal intensity in the SCP on FLAIR MRI. Higher pixel values were observed within the SCP of patients with a high signal intensity in the SCP than in patients without a high signal intensity (p < 0.001). The sensitivity and specificity of the high signal intensity in the SCP of patients with PSP was 19.6% and 100%, respectively. This finding was more frequently observed in patients with PSP with Richardson's syndrome (PSP-RS) (25.7%) than other phenotypes (6.2%). CONCLUSION: The high signal intensity in the SCP on FLAIR MRI might be an effective diagnostic tool for PSP-RS.
Atrophy ; Diagnosis ; Gliosis ; Humans ; Magnetic Resonance Imaging ; Mesencephalon ; Neurodegenerative Diseases ; Neurons ; Parkinsonian Disorders ; Phenotype ; Sensitivity and Specificity ; Supranuclear Palsy, Progressive

BACKGROUND: Reports evaluating the efficacy of transcranial sonography (TCS) for the differential diagnosis of Parkinson disease (PD) and other movement disorders in China are scarce. Therefore, this study aimed to assess the application of TCS for the differential diagnosis of PD, multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and essential tremor (ET) in Chinese individuals. METHODS: From 2017 to 2019, 500 inpatients treated at the Department of Dyskinesia, Beijing Tiantan Hospital, Capital Medical University underwent routine transcranial ultrasound examination. The cross-sections at the midbrain and thalamus levels were scanned, and the incidence rates of substantia nigra (SN) positivity and the incidence rates of lenticular hyperechoic area were recorded. The echo of the SN was manually measured. RESULTS: Of the 500 patients, 125 were excluded due to poor signal in temporal window sound transmission. Among the 375 individuals with good temporal window sound transmission, 200 were diagnosed with PD, 90 with ET, 50 with MSA, and 35 with PSP. The incidence rates of SN positivity differed significantly among the four patient groups (χ2 = 121.061, P < 0.001). Between-group comparisons were performed, and the PD group showed a higher SN positivity rate than the ET (χ2 = 94.898, P < 0.017), MSA (χ2 = 57.619, P < 0.017), and PSP (χ2 = 37.687, P < 0.017) groups. SN positivity showed a good diagnostic value for differentiating PD from the other three movement diseases, collectively or individually. The incidences of lenticular hyperechoic area significantly differed among the four patient groups (χ2 = 38.904, P < 0.001). Next, between-group comparisons were performed. The lenticular hyperechoic area was higher in the PD group than in the ET (χ2 = 6.714, P < 0.017) and MSA (χ2 = 18.680, P < 0.017) groups but lower than that in the PSP group (χ2 = 0.679, P > 0.017). CONCLUSION SN positivity could effectively differentiate PD from ET, PSP, and MSA in a Chinese population.
Diagnosis, Differential ; Humans ; Multiple System Atrophy/diagnostic imaging* ; Parkinson Disease/diagnostic imaging* ; Substantia Nigra/diagnostic imaging* ; Supranuclear Palsy, Progressive

Imaging of the dopamine transporter (DAT) provides a marker for the integrity of presynaptic nigrostriatal dopaminergic system. DAT density is reduced in Parkinson disease, multiple system atrophy, and progressive supranuclear palsy. In patients with suspicious parkinsonism, normal DAT imaging suggests an alternative diagnosis such as essential tremor, vascular parkinsonism, or drug-induced parkinsonism. DAT imaging is a useful tool to aid clinician's differential diagnosis in parkinsonism.
Diagnosis ; Diagnosis, Differential ; Dopamine Plasma Membrane Transport Proteins* ; Dopamine* ; Essential Tremor ; Humans ; Multiple System Atrophy ; Parkinson Disease ; Parkinsonian Disorders ; Supranuclear Palsy, Progressive

BACKGROUND AND PURPOSE: Various magnetic resonance (MR) measurements have been proposed to aid in differentiating between progressive supranuclear palsy (PSP) and idiopathic Parkinson's disease (IPD); however, these methods have not been compared directly. The aim of this study was to determine which measurement method exhibits the highest power to differentiate between PSP and IPD. METHODS: Brain MR images from 82 IPD and 29 PSP patients were analyzed retrospectively. T1-weighted 3D volumetric axial images, or sagittal images reconstructed from those axial images were examined. MR measurements included the length from the interpeduncular fossa to the center of the cerebral aqueduct at the mid-mammillary-body level, adjusted according to the anterior commissure-posterior commissure length (MB(Tegm)), the ratio of the midbrain area to the pons area (M/P ratio) as measured by both Oba's method (Oba M/P) and Cosottini's method (Cosottini M/P), and a modified MR parkinsonism index (mMRPI). RESULTS: Receiver operating characteristic (ROC) analysis indicated that the areas under the ROC curves (AUCs) exceeded 0.70, with a high intrarater reliability for all MR measurement methods. ROC analyses of four MR measurements yielded AUCs of 0.69-0.76. At the cutoff value with the highest Youden index, mMRPI had the highest sensitivity, while Oba M/P offered the highest specificity. A comparison of the ROC analyses revealed that MB(Tegm) was superior to mMRPI in differentiating PSP from IPD (p=0.049). There was no difference in discriminating power among Oba M/P, Cosottini M/P, and MB(Tegm). CONCLUSIONS: Simple measurements of MB(Tegm) on axial MR images at the mid-mammillary-body level are comparable to measurements of the M/P ratio with regard to their ability to discriminate PSP from IPD.
Area Under Curve ; Brain ; Cerebral Aqueduct ; Diagnosis, Differential* ; Humans ; Magnetic Resonance Imaging ; Mesencephalon* ; Neuroimaging ; Parkinson Disease* ; Parkinsonian Disorders ; Pons ; Retrospective Studies ; ROC Curve ; Sensitivity and Specificity ; Supranuclear Palsy, Progressive*