Objective:To improve the understanding of the clinical manifestation of immunoglobulin G 4-related disease (IgG 4-RD) with neurological involvement. Methods:Patients presenting with neurological symptoms and biopsy-confirmed IgG 4-RD were enrolled between March 2014 and March 2024 from the Department of Neurology of Xuanwu Hospital of Capital Medical University. Medical record data of all patients were retrospectively reviewed, including clinical manifestations, laboratory findings, radiological results, pathology, treatments, and outcomes. RD were enrolled between March Results:Nine patients (five males, four females; median age at onset: 63 years) were included in the study. Neurological manifestations consisted of pachymeningitis in six cases, peripheral nerve involvement in two, and brain parenchyma involvement in one. Four patients displayed isolated neurologic symptoms. The most common clinical manifestations were headache and cranial nerve palsy, each involving five cases. Magnetic resonance imaging showed thickening and enhancement of the dura mater in the six patients with pachymeningitis. Four of these cases involved the posterior cranial fossa, 3 presented with an inflammatory pseudotumor, and 2 involved the spinal cord. Four patients with pachymeningitis had normal serum IgG 4 concentrations. Eight patients exhibited elevated serum C-reactive protein or erythrocyte sedimentation rates, with some also showing decreased complement C3 and C4 levels. Cerebrospinal fluid immunoglobulin was elevated in all nine cases. In all cases, histopathologic biopsy results showed extensive infiltration of lymphocytes and plasma cells, with the latter displaying IgG 4+abnormalities. All patients received glucocorticoid therapy, with six also receiving immunosuppressants. All patients were followed up for a median of 30 months, with outcomes including two complete remissions, five partial remissions, one unchanged condition, and one death. Six patients experienced a relapse. Conclusions:Isolated hypertrophic pachymeningitis is the most common manifestation of IgG 4-RD, often occurring in the absence of elevated serum IgG 4 levels. Peripheral nerve and brain parenchyma involvement can also be seen. Clinical manifestations are non-specific, and histopathologic biopsy is frequently required for diagnosis. Although the disease responds well to hormone treatment, recurrence is common. Early combined immunosuppressive therapy can improve prognosis.

Objective:To investigate the clinical, imaging and neuropathological characteristics of neuronal intranuclear inclusion disease (NIID) with symptoms of the central nervous system, and to improve the diagnosis and treatments of NIID.Methods:The clinical data of 7 patients with NIID diagnosed by brain biopsy in Xuanwu Hospital, Capital Medical University, Beijing, China from February 2009 to December 2024 were collected. The characteristics of clinical manifestations, imaging, and histology on brain biopsy were retrospectively analyzed.Results:Among the 7 patients, 5 were male and 2 were female. Their ages ranged from 44 to 70 years, median 56 (52, 65) years. Patients were classified into three types of tumor, stroke and encephalitis according to the onset symptoms, imaging manifestations and pathological changes. The chief complaint of the 5 patients was headache, while 4 patients had paroxysmal convulsions, 3 had speech disorders, 2 had abnormal mental behaviors, 2 had memory decline, and 1 had fever accompanied by consciousness disorders. Diffusion-weighted magnetic resonance imaging of the head showed the "ribbon sign" at the junction of the cortex and medulla in 2 cases. Most of the patients had white matter lesions, gyrus swelling and cerebral atrophy. Occasionally gyrus-like enhancement was observed. Brain biopsy reveals the histological changes that matched those on images and initial symptoms. There were proliferation of oligodendrocytes and astrocytes in the white matter, leukoaraiosis and edema, cortical disintegration and lamellar necrosis, as well as infiltration of lymphocytes and microglia, etc. However, the characteristic changes were eosinophilic hyaline inclusions in the nuclei of neurons and astrocytes. Immunohistochemical staining of p62 and ubiquitin showed homogeneous staining in round or ring-shaped nuclei.Conclusions:The clinical manifestations of NIID are highly variable, and a correct diagnosis of NIID requires careful integration of clinical, imaging and histopathologic data. For patients with a high suspicion of NIID, immunohistochemical staining of p62 and ubiquitin is diagnostically valuable.

Neurodegenerative diseases are a class of nervous system disorders characterized by delayed onset and selective neuronal dysfunction, which are closely associated with aging.Given the challenges associated with early detection and the irreversible nature of the condition, the pursuit of specific biomarkers has emerged as a critical component of disease prevention strategies.Optical coherence tomography angiography (OCTA) generates images which exhibit near-histological resolution without the requirement for contrast agent injection, demonstrating superior sensitivity compared to fundus photography. OCTA has the ability to identify and evaluate neurodegenerative diseases via the quantitative analysis of retinal parameters. The OCTA of the retina and choroid has been verified as a highly sensitive instrument for detecting microvascular abnormalities in both ocular and systemic retinal disorders. This review focuses on the application of OCTA in various neurodegenerative diseases, such as Alzheimer′s disease, Parkinson disease, Huntington′s disease, amyotrophic lateral sclerosis and Wolfram syndrome, with the aim of providing a reference for the early diagnosis and prevention of neurodegenerative diseases.

Objective:The development and application of end-of-life patient decision aids for elderly were systematically reviewed and analyzed in order to supply guidance for the design of localized patient decision aids in China.Methods:Using the Scoping Review Guidelines as a methodological framework, a systematic search was conducted across databases including China National Knowledge Infrastructure, Wanfang, China Biology Medicine, PubMed, Web of Science, Embase, Cochrane Library, CINAHL, and BIOSIS. The search period covered from the inception of these databases to October 31, 2023. Two professionally trained researchers were responsible for screening, extracting, and organizing the retrieved literature.Results:A total of 5 studies on decision aids were included. These primarily employed paper graphics, interactive web pages, or multimedia slides as formats. The content encompassed various aspects such as palliative care options along with their pros and cons, assisting patients in clarifying their personal values, encouraging patients to document their decision-making preferences, and initiating end-of-life conversations with family members. Age-friendly design strategies were adopted in 4 studies; efficacy was validated in 3 studies, and in two of these studies, significant improvements were observed in engagement of patients (or substitute decision-makers) in ACP and the consistency of decision preferences (both P< 0.05). Conclusions:End-of-life decision aids for elderly patients can improve decision-making outcomes to some extent, however, the form and quality standards of them need to be improved, and their long-term effect needs further research and verification. It is recommended that in the future, the needs for end-of-life decision-making should be properly assessed based on the health literacy level, and high-quality localized end-of-life decision aids should be developed and popularized.

Neurodegenerative diseases are a class of nervous system disorders characterized by delayed onset and selective neuronal dysfunction, which are closely associated with aging.Given the challenges associated with early detection and the irreversible nature of the condition, the pursuit of specific biomarkers has emerged as a critical component of disease prevention strategies.Optical coherence tomography angiography (OCTA) generates images which exhibit near-histological resolution without the requirement for contrast agent injection, demonstrating superior sensitivity compared to fundus photography. OCTA has the ability to identify and evaluate neurodegenerative diseases via the quantitative analysis of retinal parameters. The OCTA of the retina and choroid has been verified as a highly sensitive instrument for detecting microvascular abnormalities in both ocular and systemic retinal disorders. This review focuses on the application of OCTA in various neurodegenerative diseases, such as Alzheimer′s disease, Parkinson disease, Huntington′s disease, amyotrophic lateral sclerosis and Wolfram syndrome, with the aim of providing a reference for the early diagnosis and prevention of neurodegenerative diseases.

Objective:To explore the role adaptation experience of informal caregivers of elderly patients with swallowing disorders, providing reference for developing caregiver support programs.Methods:This study was a qualitative study. Purposive sampling method was used to select informal caregivers of elderly patients with swallowing disorders admitted to West China Hospital of Sichuan University and Chengdu Second People's Hospital from July to October 2023 for semi-structured interviews. Analyze the data and extract themes using Colaizzi's 7-step analysis method.Results:From the caregiving experience, two main themes and nine subthemes were identified: poor adaptation to multiple roles (insufficient role adaptation resources, disrupted physiological rhythms, prominent negative emotions, limited social participation, heavy economic burden) ; role activation (emotion and responsibility, external support system assistance, internal self-efficacy enhancement, self-healing) .Conclusions:Medical staff should optimize training for informal caregivers of elderly patients with swallowing disorders, enhancing their knowledge and skills to alleviate poor role adaptation experiences and improve the quality of care.

Objective:The development and application of end-of-life patient decision aids for elderly were systematically reviewed and analyzed in order to supply guidance for the design of localized patient decision aids in China.Methods:Using the Scoping Review Guidelines as a methodological framework, a systematic search was conducted across databases including China National Knowledge Infrastructure, Wanfang, China Biology Medicine, PubMed, Web of Science, Embase, Cochrane Library, CINAHL, and BIOSIS. The search period covered from the inception of these databases to October 31, 2023. Two professionally trained researchers were responsible for screening, extracting, and organizing the retrieved literature.Results:A total of 5 studies on decision aids were included. These primarily employed paper graphics, interactive web pages, or multimedia slides as formats. The content encompassed various aspects such as palliative care options along with their pros and cons, assisting patients in clarifying their personal values, encouraging patients to document their decision-making preferences, and initiating end-of-life conversations with family members. Age-friendly design strategies were adopted in 4 studies; efficacy was validated in 3 studies, and in two of these studies, significant improvements were observed in engagement of patients (or substitute decision-makers) in ACP and the consistency of decision preferences (both P< 0.05). Conclusions:End-of-life decision aids for elderly patients can improve decision-making outcomes to some extent, however, the form and quality standards of them need to be improved, and their long-term effect needs further research and verification. It is recommended that in the future, the needs for end-of-life decision-making should be properly assessed based on the health literacy level, and high-quality localized end-of-life decision aids should be developed and popularized.

Objective:To explore the role adaptation experience of informal caregivers of elderly patients with swallowing disorders, providing reference for developing caregiver support programs.Methods:This study was a qualitative study. Purposive sampling method was used to select informal caregivers of elderly patients with swallowing disorders admitted to West China Hospital of Sichuan University and Chengdu Second People's Hospital from July to October 2023 for semi-structured interviews. Analyze the data and extract themes using Colaizzi's 7-step analysis method.Results:From the caregiving experience, two main themes and nine subthemes were identified: poor adaptation to multiple roles (insufficient role adaptation resources, disrupted physiological rhythms, prominent negative emotions, limited social participation, heavy economic burden) ; role activation (emotion and responsibility, external support system assistance, internal self-efficacy enhancement, self-healing) .Conclusions:Medical staff should optimize training for informal caregivers of elderly patients with swallowing disorders, enhancing their knowledge and skills to alleviate poor role adaptation experiences and improve the quality of care.

Objective:To improve the understanding of the clinical manifestation of immunoglobulin G 4-related disease (IgG 4-RD) with neurological involvement. Methods:Patients presenting with neurological symptoms and biopsy-confirmed IgG 4-RD were enrolled between March 2014 and March 2024 from the Department of Neurology of Xuanwu Hospital of Capital Medical University. Medical record data of all patients were retrospectively reviewed, including clinical manifestations, laboratory findings, radiological results, pathology, treatments, and outcomes. RD were enrolled between March Results:Nine patients (five males, four females; median age at onset: 63 years) were included in the study. Neurological manifestations consisted of pachymeningitis in six cases, peripheral nerve involvement in two, and brain parenchyma involvement in one. Four patients displayed isolated neurologic symptoms. The most common clinical manifestations were headache and cranial nerve palsy, each involving five cases. Magnetic resonance imaging showed thickening and enhancement of the dura mater in the six patients with pachymeningitis. Four of these cases involved the posterior cranial fossa, 3 presented with an inflammatory pseudotumor, and 2 involved the spinal cord. Four patients with pachymeningitis had normal serum IgG 4 concentrations. Eight patients exhibited elevated serum C-reactive protein or erythrocyte sedimentation rates, with some also showing decreased complement C3 and C4 levels. Cerebrospinal fluid immunoglobulin was elevated in all nine cases. In all cases, histopathologic biopsy results showed extensive infiltration of lymphocytes and plasma cells, with the latter displaying IgG 4+abnormalities. All patients received glucocorticoid therapy, with six also receiving immunosuppressants. All patients were followed up for a median of 30 months, with outcomes including two complete remissions, five partial remissions, one unchanged condition, and one death. Six patients experienced a relapse. Conclusions:Isolated hypertrophic pachymeningitis is the most common manifestation of IgG 4-RD, often occurring in the absence of elevated serum IgG 4 levels. Peripheral nerve and brain parenchyma involvement can also be seen. Clinical manifestations are non-specific, and histopathologic biopsy is frequently required for diagnosis. Although the disease responds well to hormone treatment, recurrence is common. Early combined immunosuppressive therapy can improve prognosis.

Objective:To investigate the clinical, imaging and neuropathological characteristics of neuronal intranuclear inclusion disease (NIID) with symptoms of the central nervous system, and to improve the diagnosis and treatments of NIID.Methods:The clinical data of 7 patients with NIID diagnosed by brain biopsy in Xuanwu Hospital, Capital Medical University, Beijing, China from February 2009 to December 2024 were collected. The characteristics of clinical manifestations, imaging, and histology on brain biopsy were retrospectively analyzed.Results:Among the 7 patients, 5 were male and 2 were female. Their ages ranged from 44 to 70 years, median 56 (52, 65) years. Patients were classified into three types of tumor, stroke and encephalitis according to the onset symptoms, imaging manifestations and pathological changes. The chief complaint of the 5 patients was headache, while 4 patients had paroxysmal convulsions, 3 had speech disorders, 2 had abnormal mental behaviors, 2 had memory decline, and 1 had fever accompanied by consciousness disorders. Diffusion-weighted magnetic resonance imaging of the head showed the "ribbon sign" at the junction of the cortex and medulla in 2 cases. Most of the patients had white matter lesions, gyrus swelling and cerebral atrophy. Occasionally gyrus-like enhancement was observed. Brain biopsy reveals the histological changes that matched those on images and initial symptoms. There were proliferation of oligodendrocytes and astrocytes in the white matter, leukoaraiosis and edema, cortical disintegration and lamellar necrosis, as well as infiltration of lymphocytes and microglia, etc. However, the characteristic changes were eosinophilic hyaline inclusions in the nuclei of neurons and astrocytes. Immunohistochemical staining of p62 and ubiquitin showed homogeneous staining in round or ring-shaped nuclei.Conclusions:The clinical manifestations of NIID are highly variable, and a correct diagnosis of NIID requires careful integration of clinical, imaging and histopathologic data. For patients with a high suspicion of NIID, immunohistochemical staining of p62 and ubiquitin is diagnostically valuable.