• Title/Summary/Keyword: supranuclear

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A Case of a Depressed Patient With Progressive Supranuclear Palsy (우울증으로 내원한 진행성 핵상 마비 환자 1례)

  • Seoyun Han;Jhin Goo Chang;Su Young Lee
    • Anxiety and mood
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    • v.19 no.2
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    • pp.56-60
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    • 2023
  • Progressive supranuclear palsy (PSP) is rare atypical Parkinsonism accompanied by various psycho-behavioural problems. In this case report, we describe the diagnostic and treatment progress of a 65-year-old PSP patient who visited the psychiatric clinic with a depressed mood and lumbar pain resulting in a suicide attempt. Over the course of 30 months of treatment, typical characteristics of PSP, such as postural instability, dyskinesia, cognitive dysfunction and supranuclear gaze palsy, became prominent, and magnetic resonance imaging and the F-18 FP-CIT positron emission tomography revealed midbrain atrophy and reduced dopamine uptake in the basal ganglia. When treating elderly patients with depression, parkinsonism symptoms such as gait disturbances, frequent falls, tremors, and rigidity should be closely examined.

Regional Cerebral Perfusion in Progressive Supranuclear Palsy (진행성 핵상 마비에서의 국소 뇌혈류)

  • Lee, Won-Yong;Lee, Kyung-Han;Lee, Ki-Hyeong;Yoon, Byung-Woo;Lee, Myung-Chul;Lee, Sang-Bok;Jeon, Beom-S.
    • The Korean Journal of Nuclear Medicine
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    • v.30 no.1
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    • pp.47-55
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    • 1996
  • Progressive supranuclear palsy (PSP) is a parkinson-plus syndrome characterized clinically by supranuclear ephthalmoplegia, pseudobulbar palsy, axial rigidity, bradykinesia, postural instability and dementia. Presence of dementia and lack of cortical histopathology suggest the derangement of cortical function by pathological changes in subcortical structures in PSP, which is supported by the pattern of behavioral changes and measurement of brain metabolism using positron emission tomography. This study was done to examine whether there are specific changes of regional cerebral perfusion in PSP and whether there is a correlation between severity of motor abnormality and degree of changes in cerebral perfusion. We measured regional cerebral perfusion indices in 5 cortical and 2 subcortical areas in 6 patients with a clinical diagnosis of PSP and 6 healthy age and sex matched controls using $^{99m}Tc$-HMPAO SPECT. Compared with age and sex matched controls, only superior frontal regional perfusion index was significantly decreased in PSP (p<0.05). There was no correlation between the severity of the motor abnormality and any of the regional cerebral perfusion indices (p>0.05). We affirm the previous reports that perfusion in superior frontal cortex is decreased in PSP. Based on our results that there was no correlation between severity of motor abnormality and cerebral perfusion in the superior frontal cortex, nonmotoric symptoms including dementia needs to be looked at whether there is a correlation with the perfusion abnormality in superior frontal cortex.

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A Case Report of Complex Korean Medicine Treatments of Decreased Mental Status in a Progressive Supranuclear Palsy Patient with a Cerebrovascular Accident (뇌혈관질환을 동반한 진행성 핵상마비 환자의 의식수준저하에 대한 한방복합치료 증례보고 1례)

  • Kang, Ji-hyeon;Kim, Seo-hyun;Kim, Jun-seok;Jang, Woo-seok
    • The Journal of Internal Korean Medicine
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    • v.43 no.5
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    • pp.881-890
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    • 2022
  • Objectives: This study investigated the effect of complex Korean medicine treatment on decreased mental status in a progressive supranuclear palsy patient with a cerebrovascular accident. Case presentation: The patient was treated with Korean medicine composed of herbal medicine (modified Guibi-tang), acupuncture, moxibustion, and cupping combined with Western medicine (a dopamine precursor and an anticholinergic) and physical therapy. The Glasgow Coma Scale, Mini-Mental State Examination, and Hoehn and Yahr Scale were used to evaluate the effects of the treatments. After treatment, Glasgow Coma Scores increased from 9-10 to 12-13, and Mini-Mental State Examination for dementia screen scores increased from 1 to 2. The Hoehn and Yahr score remained at 5. There were no side effects of the treatment. Conclusion: This study suggests that complex Korean medicine treatments may be effective for the decreased mental status of a progressive supranuclear palsy patient with a cerebrovascular accident, but further studies are needed.

A Case Report of a Progressive Supranuclear Palsy Patient with Gait Disturbance and Dizziness (보행장애 및 현훈을 호소하는 진행성 핵상마비 환자 치험 1례)

  • Cho, Jae Hyun;Park, Hye Lim;Hong, Minna;Lee, Hye Yoon;Lee, Ji Hye;Kim, So Yeon;Choi, Jun Yong;Han, Chang Woo;Yun, Young Ju;Park, Seong Ha;Hong, Jin Woo;Lee, In;Kwon, Jung Nam
    • The Journal of the Society of Stroke on Korean Medicine
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    • v.16 no.1
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    • pp.25-34
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    • 2015
  • ■ Objectives The purpose of this case report is to show the effect of korean medicine on a progressive supranuclear palsy patient with gait disturbance and dizziness. ■ Methods A patient with gait disturbance and dizziness diagnosed as progressive supranuclear palsy was treated with herbal medicine, acupuncture, electro-acupuncture, moxibustion. The period of admission is 16 days and we evauated the improvements of symptoms by the Unified Parkinson's Disease Rating Scale(UPDRS), Numeric Rating Scale(NRS) and Global Assessment(G/A). ■ Results After taking Modified Bosimhwan andbeing treated by acupuncture therapy, improvements of gait disturbance and dizziness are observed. ■ Conclusion This case report proved the effect of Korean medicine on progressive supranuclear palsy.

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PET studies in Alzheimer Disease and Other Degenerative Dementias (알쯔하이머병과 다른 퇴행성 치매에서의 양전자방출단층촬영)

  • Jeong, Yong;Na, Duk-L.
    • The Korean Journal of Nuclear Medicine
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    • v.37 no.1
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    • pp.13-23
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    • 2003
  • 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.

Interspecies comparative morphological evaluation of the corneal epithelial stem cell niche: a pilot observational study

  • Popova, Petya;Malalana, Fernando;Biddolph, Simon;Ramos, Tiago;Parekh, Mohit;Chantrey, Julian;Ahmad, Sajjad
    • Journal of Veterinary Science
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    • v.23 no.4
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    • pp.62.1-62.10
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    • 2022
  • Background: The corneal and limbal morphology relevant to corneal epithelial maintenance in ten different species was examined using histological methods. Objectives: The presence of a Bowman's layer, limbal epithelial cell, and superficial stromal morphology was examined in the following species to evaluate the differences in corneal thickness and epithelium: Java sparrows, frogs, macaws, spoonbills, red pandas, penguins, horses, Dobermans, orangutans, and humans. Methods: Corneal sections (4 ㎛) were obtained from ten ocular globes from three different animal classes: Aves, Amphibia, and Mammalia. All sections were stained with hematoxylin and eosin and periodic acid-Schiff reaction. After microscopy, all stained slides were photographed and analyzed. Results: Significant morphological differences in the corneal and limbal epithelia and their underlying stroma between species were observed. The number of corneal epithelial cell layers and the overall corneal epithelial thickness varied significantly among the species. The presence of a Bowman's layer was only observed in primates (orangutans and humans). Presumed supranuclear melanin caps were noted in four species (orangutans, macaws, red pandas, and horses) in the limbal basal epithelial layer (putative site of corneal epithelial stem cells). The melanin granules covered the apex of the cell nucleus. Conclusions: Supranuclear melanin capping has been described as a process within the epidermis to reduce the concentration of ultraviolet-induced DNA photoproducts. Similarly, there may be a relationship between limbal stem cell melanin capping as a protective mechanism against ultra-violet radiation.

A Clinical Report on One Case of Nuclear Facial Nerve Paralysis (핵성(核性) 안면신경마비(顔面神經麻痺) 환자 증례 1례(例))

  • Kim Bong-Suk;Lim Hee-Yong;Kim Seong-Mo;Park Jae-Hyun;Lee Tae-Hyun
    • The Journal of Internal Korean Medicine
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    • v.24 no.2
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    • pp.395-401
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    • 2003
  • Facial nerver paralysis is classified as Supranuclear, Peripheral and Nuclear. It is mostly Spontaneous Peripheral Facial Paralysis(Bell's palsy) or Supranuclear Paralysis by C.V.A, but Nuclear Facial Nerve Paralysis is rarely reported. We treated a 64-years-old female patient who had 7-years history of C.V.A with hypertension and heart disease, and complained of these symptoms; left facial palsy, ocular dysmetria, diplopia, and right extremity weakness. We diagnosed as direct attack from the wind pathogen(風邪入中) with deficiency of both Gi(Qi, vital energy) and blood(氣血雨虛), and employed Oriental medical treatments; herb-medication, acupuncture and moxa therapy. The result was relatively acceptable. So We report this case with a brief review of related literatures.

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Clinical and Pathological Characteristics of Frontotemporal Lobar Degeneration(FTLD) and Molecular Genetics of Tau Protein (Frontotemporal Lobar Degeneration(FTLD)의 임상적, 병리적 특징과 타우 단백질의 분자 유전학)

  • Woo, Sung-Il
    • Korean Journal of Biological Psychiatry
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    • v.10 no.2
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    • pp.97-106
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    • 2003
  • Criticisms about amyloid cascade hypothesis of Alzheimer's disease(AD) are based on the findings, first, that the degree of dementia does not correlate with the number of plaques, and second, that the neurofibrillary tangle formation seems to predate plaque formation. In addition, neurofibrillary tangle counts correlate well with the degree of cognitive impairment. These findings suggest the independent importance of tau abnormality in AD research which is involved in the neurofibrillary tangle formation. Recently, tau pathology without amyloid deposits and mutations in tau protein gene were reported to be the major pathogenic mechanism in Pick's disease, progressive supranuclear palsy, corticobasal degeneration and FTDP-17(frontotemporal dementia and parkinsonism linked with chromosome 17). These data suggest that understanding the causes and consequences of tau dysfunction might give new clinical and therapeutic solutions to many known tauopathies.

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Clinical Usefulness of Dopamine Transporter Imaging (도파민운반체 영상의 임상적 유용성)

  • Kim, Jong-Min;Kim, Yu-Kyeong;Kim, Sang-Eun;Jeon, Beom-S.
    • Nuclear Medicine and Molecular Imaging
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    • v.41 no.2
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    • pp.152-157
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    • 2007
  • 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.

A Case of an 18-month-old Boy with Type 3 Gaucher Disease Presenting with Hepatosplenomegaly and Growth Retardation: The Clinical Course after Enzyme Replacement Therapy (18개월 남아에서 간비장비대, 성장 부진을 동반한 3형 고셔병 증례: 효소 대체 요법 후 임상 경과)

  • Lim, Young Shin;Hwang, Jeongyun;Kim, Jinsup;Yang, Aram;Park, Hyung Doo;Jeon, Tae Yeon;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.17 no.2
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    • pp.55-62
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    • 2017
  • Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by beta-glucosidase deficiency. An 18 month-old male with hepatosplenomegaly, anemia, thrombocytopenia, and growth retardation referred to our hospital. The patient showed neurological symptoms, such as supranuclear gaze palsy and developmental delay. Bone marrow biopsy performed to rule out malignancy and the results revealed no malignant cell; however, abnormal histiocytes suggesting storage disease was noted. Based on hepatosplenomegaly, bicytopenia and unexplained neurologic manifestations, enzyme activity and genetic analysis were conducted emergently with a strong suspicion of GD. Beta-glucosidase activity in leukocyte was decreased. GBA sequencing to confirm the diagnosis revealed compound heterozygous pathogenic variants (i.e., c.754T>A, c.887G>A), both previously reported as the cause of neuronopathic GD. Under the diagnosis of type 3 GD, the patient immediately received enzyme replacement therapy (ERT). After 17 months of ERT, the size of spleen decreased, and hemoglobin and platelet count returned to normal. In addition, the activity of chitotriosidase and angiotensin converting enzyme decreased. However, myoclonic movement and generalized seizure occurred at the age of 19 months and antiepileptic drug was started. Other neurological deterioration including supranuclear gaze palsy and developmental delay also persisted. A new therapy to overcome neurologic problems should be developed for patients with type 3 GD.

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