Superficial siderosis associated with abundant   and  -synuclein accumulation

Takao, Masaki; Murayama, Shigeo; Yoshida, Youji; Mihara, Ban

doi:10.1136/bcr.10.2011.4925

Cited by 5 publications

(10 citation statements)

References 32 publications

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“…Cerebrospinal fluid tau protein is also elevated in superficial siderosis . Takao et al reported unique neuropathological findings of superficial siderosis at autopsy . Abundant tau deposits were notable in their case.…”

Section: Discussionsupporting

confidence: 78%

“…Abundant tau deposits were notable in their case. The distribution of NFTs and neuritic threads was consistent with stage III of Braak's classification . In addition, AT8‐immunoreactive pretangles and NFTs were observed in the cerebellum and locus ceruleus .…”

Section: Discussionsupporting

confidence: 78%

“…The distribution of NFTs and neuritic threads was consistent with stage III of Braak's classification . In addition, AT8‐immunoreactive pretangles and NFTs were observed in the cerebellum and locus ceruleus . In the cerebellar cortex, AT8‐immunoreactive deposits were observed in Bergmann glia .…”

Section: Discussionmentioning

confidence: 97%

“…The patient first experienced an intracerebral hemorrhage (ICH) at the age of 32 years, and had nine ICHs and/or intraventricular hemorrhages (IVHs) during the following 40 years. Recently, Takao et al reported similar tauopathy at autopsy in a patient with superficial siderosis . We also suspect repeated ICH and/or IVH over a long time may accelerate phosphorylated tau accumulation in the brainstem.…”

Section: Introductionmentioning

confidence: 99%

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Adult moyamoya disease associated with abundant phosphorylated tau accumulation in the brainstem: Report of a case with autopsy findings

Okamoto¹,

Naito²,

Fukuda³

et al. 2017

Neuropathology

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We report the case of a 72-year-old Japanese woman with moyamoya disease (MMD). She experienced her first intracerebral hemorrhage (ICH) at the age of 32 years, and had nine ICHs and/or intraventricular hemorrhages during the following 40 years. Cerebral angiograms and vascular pathologies at autopsy confirmed that the patient suffered from MMD. Macroscopically, there were brown-colored changes in the subarachnoid space, mainly at the base of the brain and around the cerebellar hemispheres. Microscopically, hemosiderin deposits were observed mainly in the old hemorrhagic lesions and on the surface of the brainstem and cerebellum. Many AT8-immunoreactive neurons and neurites were observed in the pons and midbrain, mainly in the locus ceruleus and reticular formation in the midbrain. Several AT8-immunoreactive neurons and neurites were positive for Gallyas silver staining. A few tiny and short AT8-immunoreactive processes were observed in the molecular, Purkinje cell and granular layers of the cerebellum. There were a few phosphorylated tau accumulations in the cerebrum without senile plaques. Lewy pathologies and transactive response DNA-binding protein 43 kDa proteinopathy were not detected. We suspect that oxidative stress after repeated bleedings with long-term courses in the ventricles and subarachnoid space may accelerate phosphorylated tau accumulation in the brainstem. To our knowledge, this is the first report of MMD with tauopathy in the brainstem.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Adult moyamoya disease associated with abundant phosphorylated tau accumulation in the brainstem: Report of a case with autopsy findings

Okamoto¹,

Naito²,

Fukuda³

et al. 2017

Neuropathology

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“…In comparison with previous neuropathological reports of infratentorial SS, gliosis and the neuronal loss in our case appeared to be milder.…”

Section: Cognitive Impairments and Cssmentioning

confidence: 70%

An autopsy case of cortical superficial siderosis with persistent abnormal behavior

et al. 2016

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In recent years, MRI has revealed cortical superficial siderosis (cSS), which exhibits hemosiderin deposition in only the cortical surface. However, the associations between the histological findings and clinical symptoms of cSS remain unclear. We herein report an autopsy case of a 75-year-old Japanese man with cSS with persistent abnormal behavior according to cognitive impairment, hallucination and delusion. At 73 years of age, the patient presented with unusual behavior that indicated auditory hallucination and delusion. One year later, he was admitted to the hospital for malignant lymphoma. On admission, cognitive impairment was detected by a screening test. Soon after hospitalization, he presented with active delirium including visual hallucination and delusion. The patient's excited behavior was improved by the administration of a major tranquilizer. However, the abnormal behavior and cognitive impairment persisted. At 75 years of age, he died of heart failure. A neuropathological investigation revealed hemosiderin depositions in the superficial layer of the cortex in the medial and lateral frontal lobe, the lateral temporal lobe, the parietal lobe, and the medial and lateral occipital lobe. Neuritic plaques and diffuse plaques were extensively observed, which corresponded to Braak stage C and CERAD B, although NFTs were observed that corresponded to Braak stage II. Cortical amyloid angiopathy was not observed in any regions. Ischemic change of brain was also mild. Our report suggests that localized deposition of hemosiderin in the cortex might affect the manifestation of cognitive impairments and hallucination. Further clinicopathological studies are needed to clarify the clinical manifestations of patients with cSS.

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Neuropathology of Vascular Brain Health: Insights From Ex Vivo Magnetic Resonance Imaging–Histopathology Studies in Cerebral Small Vessel Disease

Veluw

Arfanakis

Schneider

2022

Stroke

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Sporadic cerebral small vessel disease (SVD) is a major contributor to vascular cognitive impairment and dementia in the aging human brain. On neuropathology, sporadic SVD is characterized by abnormalities to the small vessels of the brain predominantly in the form of cerebral amyloid angiopathy and arteriolosclerosis. These pathologies frequently coexist with Alzheimer disease changes, such as plaques and tangles, in a single brain. Conversely, during life, magnetic resonance imaging (MRI) only captures the larger manifestations of SVD in the form of parenchymal brain abnormalities. There appears to be a major knowledge gap regarding the underlying neuropathology of individual MRI-detectable SVD abnormalities. Ex vivo MRI in postmortem human brain tissue is a powerful tool to bridge this gap. This review summarizes current insights into the histopathologic correlations of MRI manifestations of SVD, their underlying cause, presumed pathophysiology, and associated secondary tissue injury. Moreover, we discuss the advantages and limitations of ex vivo MRI-guided histopathologic investigations and make recommendations for future studies.

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Superficial siderosis associated with abundant and -synuclein accumulation

Cited by 5 publications

References 32 publications

Adult moyamoya disease associated with abundant phosphorylated tau accumulation in the brainstem: Report of a case with autopsy findings

Adult moyamoya disease associated with abundant phosphorylated tau accumulation in the brainstem: Report of a case with autopsy findings

An autopsy case of cortical superficial siderosis with persistent abnormal behavior

Neuropathology of Vascular Brain Health: Insights From Ex Vivo Magnetic Resonance Imaging–Histopathology Studies in Cerebral Small Vessel Disease

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