Scholars@Duke
Shih-Hsiu Wang

Shih-Hsiu Wang

Associate Professor of Pathology
Pathology
shihhsiu.wang@duke.edu
Box 3712, Durham, NC 27710
40 Duke Medicine Circle, Box 3712, Durham, NC 27710

Selected Publications

Performance of Polygenic Risk Scores for Primary Open-Angle Glaucoma in Populations of African Descent.

Journal Article JAMA Ophthalmol · November 14, 2024 IMPORTANCE: Primary open-angle glaucoma (POAG) polygenic risk scores (PRSs) continue to be evaluated in primarily European-ancestry populations despite higher prevalence and worse outcomes in African-ancestry populations. OBJECTIVE: To evaluate how establi ... Full text Link to item Cite

Star-shaped TDP-43 inclusions in the oldest-old.

Journal Article J Neuropathol Exp Neurol · November 4, 2024 Full text Link to item Cite

Very-late-onset multiple Acyl-coenzyme a dehydrogenase deficiency with elevated GDF-15 and Aldolase: a case report.

Journal Article Neuromuscular disorders : NMD · October 2024 A 72-year-old woman on sertraline and levothyroxine (Levoxyl) presented to clinic with progressive proximal > distal and left > right upper and lower extremity weakness. She had length-dependent paresthesias and sensory deficits. Aldolase was elevated but ... Full text Cite

MAPT H2 haplotype and risk of Pick's disease in the Pick's disease International Consortium: a genetic association study.

Journal Article Lancet Neurol · May 2024 BACKGROUND: Pick's disease is a rare and predominantly sporadic form of frontotemporal dementia that is classified as a primary tauopathy. Pick's disease is pathologically defined by the presence in the frontal and temporal lobes of Pick bodies, composed o ... Full text Link to item Cite

Intermediate Antiparallel β Structure in Amyloid β Plaques Revealed by Infrared Spectroscopic Imaging.

Journal Article ACS Chem Neurosci · October 18, 2023 Aggregation of amyloid β (Aβ) peptides into extracellular plaques is a hallmark of the molecular pathology of Alzheimer's disease (AD). Amyloid aggregates have been extensively studied in vitro, and it is well-known that mature amyloid fibrils contain an o ... Full text Link to item Cite

LATE-NC staging in routine neuropathologic diagnosis: an update.

Journal Article Acta neuropathologica · February 2023 An international consensus report in 2019 recommended a classification system for limbic-predominant age-related TDP-43 encephalopathy neuropathologic changes (LATE-NC). The suggested neuropathologic staging system and nomenclature have proven useful for a ... Full text Cite

Differential diagnosis of mild cognitive impairment of Alzheimer's disease by Simoa p-tau181 measurements with matching plasma and CSF.

Journal Article Front Mol Neurosci · 2023 Alzheimer's disease (AD) is characterized by a long preclinical phase. Although late-stage AD/dementia may be robustly differentiated from cognitively normal individuals by means of a clinical evaluation, PET imaging, and established biofluid biomarkers, d ... Full text Link to item Cite

GABA signaling triggered by TMC-1/Tmc delays neuronal aging by inhibiting the PKC pathway in C. elegans.

Journal Article Sci Adv · December 21, 2022 Aging causes functional decline and degeneration of neurons and is a major risk factor of neurodegenerative diseases. To investigate the molecular mechanisms underlying neuronal aging, we developed a new pipeline for neuronal proteomic profiling in young a ... Full text Link to item Cite

Site-Specific Phospho-Tau Aggregation-Based Biomarker Discovery for AD Diagnosis and Differentiation.

Journal Article ACS Chem Neurosci · December 7, 2022 Tau aggregates are present in multiple neurodegenerative diseases known as "tauopathies," including Alzheimer's disease (AD), Pick's disease (PiD), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). Such misfolded tau aggregates are ... Full text Link to item Cite

Corpora amylacea are associated with tau burden and cognitive status in Alzheimer's disease.

Journal Article Acta Neuropathol Commun · August 8, 2022 Corpora amylacea (CA) and their murine analogs, periodic acid Schiff (PAS) granules, are age-related, carbohydrate-rich structures that serve as waste repositories for aggregated proteins, damaged cellular organelles, and other cellular debris. The structu ... Full text Link to item Cite

Neuropathological associations of limbic-predominant age-related TDP-43 encephalopathy neuropathological change (LATE-NC) differ between the oldest-old and younger-old.

Journal Article Acta Neuropathol · July 2022 Limbic-predominant age-related TDP-43 encephalopathy neuropathological change (LATE-NC) is most often seen in the oldest-old (≥ 90 years of age) but can also be present in the younger-old (< 90 years of age). In this study, we compared the neuropathologica ... Full text Open Access Link to item Cite

Frequency of LATE neuropathologic change across the spectrum of Alzheimer's disease neuropathology: combined data from 13 community-based or population-based autopsy cohorts.

Journal Article Acta Neuropathol · July 2022 Limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC) and Alzheimer's disease neuropathologic change (ADNC) are each associated with substantial cognitive impairment in aging populations. However, the prevalence of LATE-NC a ... Full text Link to item Cite

Tuberous sclerosis complex is a novel, amyloid-independent tauopathy associated with elevated phosphorylated 3R/4R tau aggregation.

Journal Article Acta Neuropathol Commun · March 3, 2022 Tuberous sclerosis complex (TSC) is a neurodevelopmental disorder caused by mutations in the TSC1 and TSC2 genes and autosomal dominantly inherited. These mutations cause hyperactivation of the mammalian Target of Rapamycin (mTOR) pathway, leading to the d ... Full text Link to item Cite

A Case of Fragile X-Associated Tremor/Ataxia Syndrome in Association With an Unclassified Tauopathy

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2022 Cite

Selective Detection of Misfolded Tau From Postmortem Alzheimer's Disease Brains.

Journal Article Front Aging Neurosci · 2022 Tau aggregates are present in multiple neurodegenerative diseases known as "tauopathies," including Alzheimer's disease, Pick's disease, progressive supranuclear palsy, and corticobasal degeneration. Such misfolded tau aggregates are therefore potential so ... Full text Link to item Cite

Papillary Glioneuronal Tumor With a Novel GPR37L1-PRKCA Fusion.

Journal Article J Neuropathol Exp Neurol · October 26, 2021 Full text Link to item Cite

Diffuse midline glioma with H3 K27M-mutation in an 83-year-old woman.

Journal Article CNS Oncol · June 1, 2021 Diffuse midline gliomas harboring histone H3 K27M mutations are most commonly found in the brainstem of children. This mutation confers a WHO grade IV designation and is associated with a particularly poor prognosis. Although traditionally considered to be ... Full text Link to item Cite

Heterogeneity in α-synuclein fibril activity correlates to disease phenotypes in Lewy body dementia.

Journal Article Acta Neuropathol · April 2021 α-Synuclein aggregation underlies pathological changes in Lewy body dementia. Recent studies highlight structural variabilities associated with α-synuclein aggregates in patient populations. Here, we develop a quantitative real-time quaking-induced convers ... Full text Link to item Cite

A case of CNS aspergillosis in a patient with chronic lymphocytic leukemia on first-line ibrutinib therapy.

Journal Article Med Mycol Case Rep · March 2020 Ibrutinib has revolutionized the treatment of chronic lymphoid malignancies. Despite its success, ibrutinib has been linked with several reports of invasive fungal infections. We present a case of CNS aspergillosis in a CLL patient on first line ibrutinib ... Full text Link to item Cite

Association of Genetic Variants With Primary Open-Angle Glaucoma Among Individuals With African Ancestry.

Journal Article JAMA · November 5, 2019 IMPORTANCE: Primary open-angle glaucoma presents with increased prevalence and a higher degree of clinical severity in populations of African ancestry compared with European or Asian ancestry. Despite this, individuals of African ancestry remain understudi ... Full text Link to item Cite

Clinical and molecular characteristics of gliosarcoma and modern prognostic significance relative to conventional glioblastoma.

Journal Article J Neurooncol · April 2018 Gliosarcoma is a rare histopathologic variant of glioblastoma traditionally associated with a poor prognosis. While gliosarcoma may represent a distinct clinical entity given its unique histologic composition and molecular features, its relative prognostic ... Full text Link to item Cite

Invasiveness is associated with metastasis and decreased survival in hemangiopericytoma of the central nervous system.

Journal Article J Neurooncol · June 2017 Meningeal hemangiopericytoma (m-HPC) is a rare tumor of the central nervous system (CNS), which is distinguished clinically from meningioma by its tendency to recur and metastasize. The histological classification and grading scheme for m-HPC is still evol ... Full text Link to item Cite

Atypical Rapidly Enlarging Orbital Schwannoma.

Journal Article Ophthalmic Plast Reconstr Surg · 2017 Orbital schwannomas are typically slow-growing benign tumors that can cause gradual loss of vision, proptosis, and limitation of ocular motility. The authors present an atypical case of a rapidly growing orbital apex schwannoma in a patient with preexistin ... Full text Link to item Cite

Atypical Rapidly Enlarging Orbital Schwannoma

Journal Article OPHTHALMIC PLASTIC AND RECONSTRUCTIVE SURGERY · 2017 Cite

Widely metastatic atypical pituitary adenoma with mTOR pathway STK11(F298L) mutation treated with everolimus therapy.

Journal Article CNS Oncol · October 2016 Pituitary adenomas are the commonest intracranial tumor, but metastases are rare (0.2% yearly incidence) and portend poor prognosis. CAPecitabine and TEMozolomide improved outcomes for neuroendocrine tumors. However, no chemotherapy is approved for refract ... Full text Link to item Cite

Staged laser interstitial thermal therapy and topectomy for complete obliteration of complex focal cortical dysplasias.

Journal Article J Clin Neurosci · September 2016 Anatomically complex focal cortical dysplasias may present significant challenges to safe and complete surgical resection via standard operative corridors. Laser interstitial thermal therapy (LITT) is an emerging minimally invasive technique that may addre ... Full text Link to item Cite

Ryk controls remapping of motor cortex during functional recovery after spinal cord injury.

Journal Article Nat Neurosci · May 2016 Limited functional recovery can be achieved through rehabilitation after incomplete spinal cord injury. Eliminating the function of a repulsive Wnt receptor, Ryk, in mice and rats by either conditional knockout in the motor cortex or monoclonal antibody in ... Full text Link to item Cite

Native valve emphysematous endocarditis caused by finegoldia magna in a novel pathogenic role

Journal Article Infectious Diseases in Clinical Practice · January 1, 2016 Endocarditis presenting with invasion of the myocardium by gas-forming organisms is rare, previously limited to Escherichia coli and Clostridia species. We describe a case of native valve emphysematous endocarditis presenting with stroke and ultimately lea ... Full text Cite

The use of fluorescein sodium in the biopsy and gross-total resection of a tectal plate glioma.

Journal Article J Neurosurg Pediatr · December 2015 Intravenous administration of fluorescein sodium fluoresces glioma burden tissue and can be visualized using the surgical microscope with a specialized filter. Intraoperative guidance afforded through the use of fluorescein may enhance the fidelity of tiss ... Full text Link to item Cite

Spindle cell sarcoma of the vulva with myofibroblastic differentiation.

Journal Article J Low Genit Tract Dis · April 2015 OBJECTIVE: Primary vulvar sarcomas are rare lesions of the lower genital tract. We report the case of a patient with a spindle cell sarcoma of the vulva. MATERIALS AND METHODS: A 44-year-old woman presented with a painless vulvar mass. Vulvar biopsy demons ... Full text Link to item Cite

NHERF1/EBP50 is an organizer of polarity structures and a diagnostic marker in ependymoma.

Journal Article Acta Neuropathol Commun · March 8, 2015 NHERF1/EBP50, an adaptor protein required for epithelial morphogenesis, has been implicated in the progression of various human malignancies. NHERF1-deficient mice have intestinal brush border structural defects and we report here that they also have disor ... Full text Link to item Cite

Semaphorin 5A inhibits synaptogenesis in early postnatal- and adult-born hippocampal dentate granule cells.

Journal Article Elife · October 14, 2014 Human SEMAPHORIN 5A (SEMA5A) is an autism susceptibility gene; however, its function in brain development is unknown. In this study, we show that mouse Sema5A negatively regulates synaptogenesis in early, developmentally born, hippocampal dentate granule c ... Full text Link to item Cite

Dlg5 regulates dendritic spine formation and synaptogenesis by controlling subcellular N-cadherin localization.

Journal Article J Neurosci · September 17, 2014 Most excitatory synapses in the mammalian brain are formed on dendritic spines, and spine density has a profound impact on synaptic transmission, integration, and plasticity. Membrane-associated guanylate kinase (MAGUK) proteins are intracellular scaffoldi ... Full text Link to item Cite