Case Of Progressive Supranuclear Palsy Research Articles

Isolated Jaw and Tongue Tremor in a Definite Case of Progressive Supranuclear Palsy.

Movement Disorders Clinical PracticeVolume 10, Issue 2 p. 348-349 LETTERS: PUBLISHED ARTICLES Isolated Jaw and Tongue Tremor in a Definite Case of Progressive Supranuclear Palsy Nahid Olfati MD, Nahid Olfati MD orcid.org/0000-0003-4621-2490 Department of Neurology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USASearch for more papers by this authorDennis W. Dickson MD, Dennis W. Dickson MD Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USASearch for more papers by this authorAli Shoeibi MD, Ali Shoeibi MD Department of Neurology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, IranSearch for more papers by this authorDietrich Haubenberger MD, Dietrich Haubenberger MD UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USASearch for more papers by this authorIrene Litvan MD, Corresponding Author Irene Litvan MD [email protected] UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USA Correspondence to: Dr. Irene Litvan, UC San Diego Department of Neurosciences, Altman Clinical Translational Research Institute, Parkinson & Other Movement Disorders Center, 9452 Medical Center Drive, 2W114, La Jolla, California, USA; E-mail: [email protected]Search for more papers by this author Nahid Olfati MD, Nahid Olfati MD orcid.org/0000-0003-4621-2490 Department of Neurology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USASearch for more papers by this authorDennis W. Dickson MD, Dennis W. Dickson MD Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USASearch for more papers by this authorAli Shoeibi MD, Ali Shoeibi MD Department of Neurology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, IranSearch for more papers by this authorDietrich Haubenberger MD, Dietrich Haubenberger MD UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USASearch for more papers by this authorIrene Litvan MD, Corresponding Author Irene Litvan MD [email protected] UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USA Correspondence to: Dr. Irene Litvan, UC San Diego Department of Neurosciences, Altman Clinical Translational Research Institute, Parkinson & Other Movement Disorders Center, 9452 Medical Center Drive, 2W114, La Jolla, California, USA; E-mail: [email protected]Search for more papers by this author First published: 11 December 2022 https://doi.org/10.1002/mdc3.13640Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume10, Issue2February 2023Pages 348-349 RelatedInformation

A Case Report on Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a quite rare neurodegenerative disease that affects mainly the brain stem, basal ganglia, and cerebellum in the central nervous system. PSP is classified in the rubric of parkinsonism plus syndromes, which causes progressive degeneration in the midbrain areas those controls eye movements. PSP often goes under diagnosed, hence making it essential for physicians to be well-known of this disorder. We present a case of PSP of a 65- year-old female, which came primarily with reiterative falls due to postural instability and speech disturbances. Radiodiagnosis with magnetic resonance imaging (MRI) of brain diagnosed a classical sign of PSP.

Hyperparathyroidism: A rare cause of worsening of parkinsonism

Parkinsonism secondary to hypoparathyroidism is well described. The association of hyperparathyroidism with parkinsonism is rare. We describe here a case of progressive supranuclear palsy (PSP) associated with hyperparathyroidism secondary to a parathyroid adenoma who improved significantly with surgical excision of the parathyroid adenoma.

Case of Progressive Supranuclear Palsy Showing Improved Balance Ability after Use of the Balance Evaluation Systems Test for Clinical Decision Making

〔目的〕進行性核上性麻痺に対し，バランス制御システムを特異的に評価するBalance Evaluation Systems Test（BESTest）を用いて目標設定と介入を実施した．バランス介入の臨床意思決定における示唆を得ることを目的とした．〔対象と方法〕進行性核上性麻痺により易転倒性を認めていた78歳，女性を対象とした．下肢筋力の低下，予測的姿勢制御と反応的姿勢制御というバランス制御システムの低下が活動量と歩行能力低下の原因と考えられ，動作の自立度に応じて課題難易度を調節した介入を実施した．〔結果〕約3週間の介入後，歩行能力の向上を認め自宅復帰に至った．フォローアップでは転倒回数の減少を認めた．〔結語〕臨床意思決定にBESTestを用いることはバランス障害に対する介入を効果的にする可能性が示唆された．

A case of progressive supranuclear palsy confused with alcohol-related dementia

A case of progressive supranuclear palsy confused with alcohol-related dementia

123I-FP-CIT and 99mTc-HMPAO in Pathologically Confirmed Progressive Supranuclear Palsy

Molecular brain imaging I-FP-CIT SPECT is an important tool in evaluation of patients with parkinsonism. However, various neurodegenerative etiologies cannot be differentiated by I-FP-CIT SPECT alone. We present a case of progressive supranuclear palsy with abnormal I-FP-CIT SPECT and abnormal Tc-HMPAO SPECT depicted by quantitative analyses but unremarkable MRI 16 months after the onset of symptoms. Brain autopsy demonstrated presence of neuronal and glial tau pathology in both cortical and subcortical regions confirming the diagnosis of progressive supranuclear palsy. This case illustrates potential values of multimodal molecular brain imaging in conjunction with quantitative analysis in the evaluation of movement disorders.

Diversified psychiatric presentation in a case of progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is an unusual neurodegenerative disorder with variant clinical phenotypes. Accurate diagnosis is challenging in the early stage, especially in psychiatric clinics, where misdiagnoses with psychiatric illness are common. A case of PSP was difficult to differentiate from other Parkinsonian syndromes initially, and the patient's affective symptoms predated the onset of other symptoms. Gaze abnormality and frontal lobe syndromes emerged and she was diagnosed with PSP 5 months after the first psychiatric visit. Heightened awareness of PSP and its diagnosis are important, not only because of prognostic implications, but also because of appropriate interventions and focused therapeutic targets.

Challenges with the differential diagnosis of progressive supranuclear palsy and Parkinson’s disease

Progressive supranuclear palsy (PSP) is a severe old-age neurodegenerative disease that is characterized by pronounced clinical polymorphism and has different phenotypes. Practical differential diagnosis of PSP and Parkinson’s disease (PD) presents significant challenge, especially in onset of the disease. We report an atypical case of PSP characterized by symmetric levodopa-resistant parkinsonian syndrome, early postural instability, supranuclear oculomotor disorders (vertical gaze palsy), MR signs of midbrain atrophy, and a number of overlapping clinical symptoms similar with the manifestations of PD. A detailed algorithm of differential diagnosis of PSP and PD as well as a more adequate diagnostic formulation are proposed for this and similar cases of neurodegenerative pathology.

The "hummingbird" lady: A case report on progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease, which tends to be progressive, causing weakness of the structures in the brain stem that controls eye movements. PSP falls under the rubric of Parkinsonism plus syndromes that are a group of heterogeneous degenerative neurological disorders that differ from the classical idiopathic Parkinson's disease. PSP is often underreported, making it important for clinicians to be aware of this disorder. Here we report a case of PSP, which presented primarily with speech disturbances and recurrent falls due to postural instability. On investigating, the magnetic resonance imaging (MRI) revealed a classical sign diagnostic of PSP.

Zolpidem in Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disorder, characterized by motor symptoms, postural instability, personality changes, and cognitive impairment. There is no effective treatment for this disorder. Reduced neurotransmission of GABA in the striatum and globus pallidus may contribute to the symptoms of motor and cognitive symptoms seen in PSP. Zolpidem is a GABA agonist of the benzodiazepine subreceptor BZ1. Here a nondiabetic, normotensive case of PSP is (Progressive Supranuclear Palsy) described, which showed improvement in swallowing, speech, and gaze paresis after zolpidem therapy and possible mechanism of actions are discussed. However, more trials are needed with large number of patients to confirm the effectiveness of zolpidem in progressive supranuclear palsy.

Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia

We report an autopsy case of progressive supranuclear palsy (PSP) that clinically showed only slowly progressive and symmetric upper motor neuron syndrome over a disease course of 12years. A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs. However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. She was clinically diagnosed as having motor neuron disease consistent with so-called primary lateral sclerosis. Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a lesser degree, in the basal ganglia and brain stem nuclei. In addition, severe fibrillary gliosis was noted in the precentral gyrus and corticospinal tract, being consistent with upper motor neuron syndrome observed in this case. No TAR-DNA binding protein 43-positive lesion, FUS pathology, Bunina body, or Lewy body-like hyaline inclusion was noted in the motor cortex or lower motor neurons. These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis.

進行性核上性麻ひの１例:CT, SPECT, MRI所見の検討

進行性核上性麻ひの１例:CT, SPECT, MRI所見の検討

An autopsied case of progressive supranuclear palsy, initially diagnosed as spinocerebellar degeneration with severe olivopontocerebellar involvement

A Japanese female patient presented with dysarthria and gait disturbance with ataxia at the age of 63. She was initially diagnosed with spinocerebellar degeneration because a head CT showed atrophy of the cerebellum and brainstem, and dilatation of the fourth ventricle. Symptoms including abnormal behavior, dementia, vertical gaze palsy, dysphagia, retrocollis, axial rigidity, grasp reflex and positive Babinski's sign were gradually observed. Tegmental atrophy of the brainstem and dilatation of the third ventricle were apparent on images. The diagnosis was modified to progressive supranuclear palsy (PSP), 6 years after the onset of symptoms. Gastrostomy and tracheotomy were performed 7 and 8 years after onset, respectively, and the patient died one year later. At autopsy the brain weighed 1,030g and showed atrophy of the frontal lobe and cerebellum. The brainstem was also atrophic, particularly in the tegmentum and pontine base. Neurofibrillary tangles, mainly globose-type, were widespread in the subcortical structure, particularly in the globus pallidus, subthalamic nucleus, nucleus of the oculomotor nerve, substantia nigra, locus ceruleus, pontine nucleus, and the inferior olivary nucleus. Numerous glial fibrillary tangles and argyrophilic threads were also observed particularly in the frontal lobe, basal ganglia, brainstem and cerebellar white matter. The cerebellar dentate nucleus showed neuron loss with grumose degeneration and the Purkinje neuron layer showed neuron loss and Bergmann's gliosis with torpedoes. Tau positive inclusions in the Purkinje neurons, Bergmann glias and dentate nucleus neurons were observed. Pathological findings were consistent with the diagnosis of PSP but the olivopontocerebellar involvement and the quantity of Gallyas-positive/tau-positive structures were generally more severe than in typical PSP cases. According to these clinicopathologic findings and a review of the literature, we speculate on the existence of a PSP subtype with severe olivopontocerebellar involvement that tends to be clinically misdiagnosed as spinocerebellar degeneration.

Coexistence of PSP and MSA: a case report and review of the literature

Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy characterized by Parkinsonism, vertical gaze palsy, and early falls. The neuropathology is characterized by neurofibrillary tangles, tufted astrocytes, and coiled bodies, but some brains show other pathologic processes. To investigate the frequency of alpha-synuclein pathology in PSP with immunohistochemistry and to report the clinical and pathological features of a case of PSP with concomitant Multiple system atrophy (MSA) (PSP/MSA), 290 cases of PSP were screened for alpha-synuclein pathology with immunohistochemistry. Double-labeling immunohistochemistry was performed on a case of PSP/MSA. Among the PSP cases screened for alpha-synuclein pathology, a single case of PSP/MSA was detected. The patient was an 86-year-old woman with clinical features consistent with PSP. She had no documented dysautonomia or cerebellar signs, and imaging studies were not diagnostic of MSA. Pathological examination showed tau-immunoreactive neuronal and glial lesions consistent with PSP as well as alpha-synuclein immunoreactive glial cytoplasmic inclusions diagnostic of MSA. Double-immunolabeling studies showed no co-localization of alpha-synuclein and tau in most neuronal and glial lesions. Based upon the findings in this case, the neuropathologic changes of PSP and MSA are distinct and independent processes, but they can occasionally coexist.

An Autopsy Case of PSP with Astrocytic Inclusions

Argyrophilic glial cytoplasmic inclusions were observed in astrocytes (Astrocytic Inclusions) in a case of progressive supranuclear palsy (PSP). The inclusions were predominantly distributed in the lenticular nucleus and midbrain, but not in the cerebral cortex, white matter nor in the thalamus. When examined by double staining with Gallyas silver and GFAP, they resembled the neurofibrillary tangles (NFT) and were tau positive. The distribution of these inclusions was not related to that of NFT. These inclusions indicate that astrocytes were involved in the same pathological changes as NFT. Argyrophilic inclusions were also observed in oligodendroglias on Gallyas silver staining. They appeared coiled and located mainly in the cerebral and cerebellar white matter.

A case of progressive supranuclear palsy with widespread senile plaques

A case of progressive supranuclear palsy (PSP) with frontal lobe atrophy is reported, in which many senile plaques were widely distributed in the neocortex, the entorhinal cortex, the amygdala, and, to a lesser extent, the cerebellar cortex, but not in the hippocampus. Most of the plaques were of the diffuse and primitive types. They were well visualized by beta-protein immunostaining, modified Bielschowsky staining and methenamine silver staining, but were not seen by Bodian staining. The widespread distribution of senile plaques in the cerebral and cerebellar cortices was far beyond that seen in normal aging, and was reminiscent of concomitant Alzheimer's disease (AD). Unlike AD, however, this case had neither senile changes in the hippocampus nor neurofibrillary tangles in the amygdala and entorhinal cortex. It seems that many senile plaques may appear widely in the cerebral cortex and even, to a lesser extent, in the cerebellar cortex of some patients with PSP. Additional case studies using sensitive silver and amyloid antibody preparations are required to elucidate the presence of senile plaques in the cerebral cortex of PSP.

Ｃｅｎｔｒａｌ ｐｏｎｔｉｎｅ ｍｙｅｌｉｎｏｌｙｓｉｓを合併した進行性核上性麻ひの一剖検例

An autopsy case of progressive supranuclear palsy (PSP) associated with central pontine myelinolysis (CPM) is reported. A 73-year-old male patient suffered from gait disturbance for about 5 years. The clinical features were characterized by gradual development of supranuclear ophthalmoplegia, tremor, bradykinesia, rigidity, neck dystonia, dementia and pseudobulbar palsy at the advanced stage of his illness. Treatment with levodopa did not improve his neurological signs and symptoms. PSP or multiple system atrophy was considered as a clinical diagnosis of the patient. He died of pneumonia, acute pancreatitis and liver dysfunction in November 1985. The main neuropathological findings were neuronal loss and gliosis with neurofibrillary tangles of globose type in the globus pallidus, subthalamic nucleus, substantia nigra and dentate nucleus, and at the base of the pons, bilateral and symmetrical demyelination was found. In addition, myelin staining revealed circumscribed pallor in the cerebral white matter. The histologic diagnosis was PSP associated with CPM. An association of PSP with CPM is rare in the elderly and possible etiologic factors of both diseases were discussed.

A Case of Progressive Supranuclear Palsy with Partial Improvement by Bromocriptine

A Case of Progressive Supranuclear Palsy with Partial Improvement by Bromocriptine

Progressive supranuclear palsy: A cause of subcortical dementia

AbstractA case of progressive supranuclear palsy in a 69‐year‐old female is reported. Initially the patient presented to the pscyhiatric services with prominent paranoid delusions. Later, when dementia became more evident, neurological examination revealed typical features of progressive supranuclear palsy, including complete failure of upward gaze, facial bradykinesia, slow gait and bilateral cogwheel rigidity. During hospitalization, the patient's condition improved and she was discharged to an ordinary residential home for the elderly.This unusual presentation of dementia demonstrates, once again, the importance of a full neurological examination in all cases of dementia.

Reappraisal of the fine structure of Alzheimer's neurofibrillary tangles

Alzheimer's neurofibrillary tangles were studied by electron microscopy. The study includes four cases of Alzheimer's disease, two cases of atypical senile dementia, and one case of progressive supranuclear palsy. In Alzheimer's disease the tangles were composed of either straight filaments or paired helical filaments. In progressive supranuclear palsy the tangles were composed of 15 nm straight filaments or helical filaments. A few straight filaments were mixed with paired helical filaments. In atypical senile dementia, both straight and paired helical filaments comprised the tangles and one type of filaments appeared to intermingle with the other in the same neurons.