An Autopsy Case of Myotonic Dystrophy Type 1 With Pancreatic Intraductal Papillary Mucinous Neoplasm.

Here, we present an autopsy case of long-standing myotonic dystrophy type 1 (DM1) in a patient who developed a pancreatic intraductal papillary mucinous neoplasm (IPMN). DM1 is a progressive genetic disorder that affects multiple organs, including the respiratory muscles. Several nationwide registry-based cohort studies have suggested that patients with DM1 have an increased risk of developing pancreatic cancers such as pancreatic ductal adenocarcinoma (PDAC).

Dynamic aphasia as an early sign of corticobasal degeneration: Clinico-radio-pathological correlation.

A 72-year-old man presented with a 6-month history of decreased voluntary speech. Sparse speech and decreased word fluency were observed. Articulation, naming, comprehension, and repetition were preserved.

RETINAL MIGRATION AND SURGICAL OUTCOME AFTER HEMI-TEMPORAL INTERNAL LIMITING MEMBRANE PEELING VERSUS CONVENTIONAL PEELING FOR MACULAR HOLE: A Multicenter, Randomized, Controlled Trial.

Purpose: To investigate the anatomical changes and surgical outcomes of hemi-temporal internal limiting membrane (ILM) peeling and conventional ILM peeling for idiopathic macular hole (MH).

Methods: This randomized controlled trial was conducted at 3 centers and included 50 participants with MHs of <400 µm in minimum diameter for a duration of <6 months. All participants had undergone vitrectomy with either hemi-temporal ILM peeling (Hemi group) or 360° ILM peeling (360° group) with an injection of 5% sulfur hexafluoride gas, with or without simultaneous cataract surgery, from July 2017 to January 2021.

Clinicopathological study of dementia with grains presenting with parkinsonism compared with a typical case.

Argyrophilic grain disease (AGD) is one of the major pathological backgrounds of senile dementia. Dementia with grains refers to cases of dementia for which AGD is the sole background pathology responsible for dementia. Recent studies have suggested an association between dementia with grains and parkinsonism.

Neuropathological changes associated with aberrant cerebrospinal fluid p-tau181 and Aβ42 in Alzheimer's disease and other neurodegenerative diseases.

Recent studies suggest that increased cerebrospinal fluid (CSF) phospho-tau is associated with brain amyloid pathology rather than the tau pathology. However, confirmation using gold standard neuropathological assessments remains limited. This study aimed to determine background pathologies associated with aberrant CSF p-tau181 and amyloid-beta 1-42 (Aβ42) in Alzheimer's disease (AD) and other neurodegenerative diseases.

CSF P-Tau181 and Other Biomarkers in Patients With Neuronal Intranuclear Inclusion Disease.

Background And Objectives: CSF tau phosphorylated at threonine 181 (p-tau181) is a widely used biomarker for Alzheimer disease (AD) and has recently been regarded to reflect β-amyloid and/or p-tau deposition in the AD brain. Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease characterized by intranuclear inclusions in neurons, glial cells, and other somatic cells. Symptoms include dementia, neuropathy, and others.

Aseptic Meningitis-retention Syndrome Associated with Tocilizumab in a Patient with Idiopathic Multicentric Castleman Disease.

This is the first report of tocilizumab-associated meningitis-retention syndrome in a patient with idiopathic multicentric Castleman disease. A 57-year-old man presented with headache, nuchal rigidity, impaired consciousness, pyramidal tract signs and urinary retention. A cerebrospinal fluid examination revealed increased cell counts and protein levels.

A central nervous system metastasis of melanoma with stroke-like onset of left-lower quadrantanopsia.

"Stroke mimics" mean diseases presenting with acute neurological impairments that are taken for stroke. Discriminating them is crucial to avoid improper treatment or delayed correct treatment. We describe a 48-year-old woman presenting with a sudden onset of scintillating scotoma and left-lower quadrantanopsia.

Corticobasal degeneration with deep white matter lesion diagnosed by brain biopsy.

Corticobasal degeneration (CBD) is a rare progressive neurodegenerative disorder characterized by asymmetric presentation of cerebral cortex signs, cortical sensory disturbance and extrapyramidal signs. Herein, we report a case of a 66-year-old Japanese woman who presented with apraxia of the right hand. She subsequently developed postural instability and cognitive impairments that rapidly worsened.

Hospital-based screening to detect patients with cadmium nephropathy in cadmium-polluted areas in Japan.

Background: In health examinations for local inhabitants in cadmium-polluted areas, only healthy people are investigated, suggesting that patients with severe cadmium nephropathy or itai-itai disease may be overlooked. Therefore, we performed hospital-based screening to detect patients with cadmium nephropathy in two core medical institutes in cadmium-polluted areas in Akita prefecture, Japan.

Methods: Subjects for this screening were selected from patients aged 60 years or older with elevated serum creatinine levels and no definite renal diseases.

Genome-wide association study suggests four variants influencing outcomes with ranibizumab therapy in exudative age-related macular degeneration.

To identify factors associated with ranibizumab responses in patients with exudative age-related macular degeneration (AMD), we performed a genome-wide association study (GWAS) and a replication study using a total of 919 exudative AMD patients treated with intravitreal ranibizumab in a Japanese population. In the combined analysis of GWAS and the replication study, no loci reached genome-wide significant level; however, we found four variants showed suggestive level of associations with visual loss at month three (rs17822656, rs76150532, rs17296444, and rs75165563: P < 1.0 × 10).

Efficacy of intravitreal aflibercept injections for Japanese patients with polypoidal choroidal vasculopathy.

Purpose: To evaluate the efficacy of intravitreal aflibercept therapy in treatment-naïve Japanese patients with polypoidal choroidal vasculopathy (PCV) using Early Treatment Diabetic Retinopathy Study (ETDRS) letter scores.

Subjects And Methods: This study was a prospective, nonrandomized, interventional exploratory clinical trial performed in an institutional setting. Patients with PCV were treated with intravitreal aflibercept 2 mg/0.

Clinical features of a toddler with bilateral bullous retinoschisis with a novel mutation.

Purpose: To report the clinical and genetic findings of a male toddler who presented bilateral bullous retinoschisis with a novel mutation.

Observations: This is an observational case report of a patient referred to our hospital with esotropia. A comprehensive ophthalmic examination was performed with the boy (age, 1 year 4 months) under general anesthesia that included fundus examinations, fluorescein angiography (FA), swept-source optical coherence tomography (SS-OCT), and full-field electroretinography (FF-ERG).

Comparison of Time to Retreatment and Visual Function Between Ranibizumab and Aflibercept in Age-Related Macular Degeneration.

Purpose: To compare time to retreatment and visual function between patients with treatment-naïve neovascular age-related macular degeneration (AMD) treated with either intravitreal ranibizumab (IVR) or intravitreal aflibercept (IVA) in routine clinical practice.

Design: Retrospective, interventional comparative case series.

Participants: A total of 200 eyes of 197 patients with neovascular AMD.

AFLIBERCEPT FOR POLYPOIDAL CHOROIDAL VASCULOPATHY: As Needed Versus Fixed Interval Dosing.

Purpose: To evaluate the functional and morphologic outcomes of patients with polypoidal choroidal vasculopathy undergoing intravitreal aflibercept (IVA) treatment using every 2-month injections compared with a pro re nata (PRN) regimen after 3 initial monthly doses.

Methods: The authors prospectively studied all the treatment-naive patients with polypoidal choroidal vasculopathy who were scheduled to undergo IVA using every 2-month injections or PRN after induction treatment between March 2013 and October 2013. All patients who had a follow-up period of 1 year or longer were included in the study.

Outcomes of microincision vitrectomy surgery with internal limiting membrane peeling for macular edema secondary to branch retinal vein occlusion.

Purpose: To evaluate the anatomic and functional effect of microincision vitrectomy surgery (MIVS) with internal limiting membrane (ILM) peeling for macular edema secondary to branch retinal vein occlusion (BRVO).

Methods: The medical records of 101 eyes of 101 patients who had undergone MIVS with ILM peeling for macular edema secondary to BRVO were studied. Patients were classified into ischemic and non-ischemic BRVO based on angiograph.

Long-term outcome of intravitreal pegaptanib sodium as maintenance therapy in Japanese patients with neovascular age-related macular degeneration.

Purpose: To evaluate the results of a 3-year follow-up of intravitreal pegaptanib sodium injection as maintenance therapy for the treatment of neovascular age-related macular degeneration (AMD) in Japanese patients.

Methods: In this prospective, uncontrolled interventional study, 20 eyes of 19 patients with treatment-naïve AMD who had received 3 consecutive monthly injections of 0.5 mg/0.

Long-term Outcomes After Preoperative Intravitreal Injection of Bevacizumab Before Trabeculectomy for Neovascular Glaucoma.

Purpose: To evaluate the long-term (>3 y) outcomes of patients given preoperative intravitreal bevacizumab (IVB) injection before trabeculectomy for neovascular glaucoma (NVG).

Method: We performed a retrospective study of a consecutive series of 12 eyes of 11 patients who underwent trabeculectomy with mitomycin C combined with preoperative IVB for NVG at our institution between April 2009 and April 2010. The mean follow-up period was 43.

Intravitreal injection of ranibizumab using a pro re nata regimen for age-related macular degeneration and vision-related quality of life.

Background: The purpose of this study was to assess visual function and vision-related quality of life after intravitreal injection of ranibizumab (IVR) using a pro re nata regimen for the treatment of age-related macular degeneration.

Methods: A prospective study of 54 eyes in 54 patients scheduled to undergo IVR for the treatment of exudative age-related macular degeneration was performed. A self-administered, 25-item National Eye Institute Visual Function Questionnaire (NEI VFQ-25) was completed before and 3 and 12 months after the initial IVR treatment.

Displacement of submacular hemorrhages in age-related macular degeneration with subretinal tissue plasminogen activator and air.

Objective: To study the anatomic and visual outcomes of a surgical procedure in which tissue plasminogen activator and air are injected subretinally to displace massive submacular hemorrhages secondary to age-related macular degeneration.

Design: Prospective, consecutive, interventional case series.

Participants: Thirteen consecutive patients (13 eyes) with massive submacular hemorrhages secondary to age-related macular degeneration.

Short-term efficacy of intravitreal aflibercept in treatment-naive patients with polypoidal choroidal vasculopathy.

Purpose: To evaluate the functional and morphologic outcomes of patients with polypoidal choroidal vasculopathy who underwent intravitreal aflibercept treatment.

Methods: We prospectively studied all the treatment-naive patients with polypoidal choroidal vasculopathy who were scheduled to undergo intravitreal aflibercept between February 2013 and July 2013. The best-corrected visual acuity was compared before treatment and at 6 months after the initial treatment.