Objective Thickening of the interatrial septum (IAS) is one of red flags for cardiac amyloidosis (CA), but the available evidence concerning it is poor. We evaluated the IAS thickness (IAST) using transthoracic echocardiography to determine the criteria for thickening of the IAS for diagnosing wild-type transthyretin cardiac amyloidosis (ATTRwt-CA).

Methods We reviewed 60 patients with ATTRwt-CA (mean age: 78.7±5.6 years old, 50 men) and 56 control individuals (mean age: 74.3±6.1 years old, 29 men) with normal echocardiographic findings. We measured the end-diastolic and end-systolic (ES) IAST in the middle region of the anterior atrial septum in an apical four-chamber view and a subcostal four-chamber view.

Results The IAST was significantly greater in patients in the ATTRwt-CA group than in the control group at both sites and phases. In a receiver operating characteristic analysis to discriminate ATTRwt-CA patients from control subjects, the best cutoff value of IAST was 5.2 mm, which was measured at ES in a subcostal four-chamber view, with a sensitivity of 80.0% and specificity of 78.6%. There was no significant correlation between the IAST and disease stage of ATTRwt-CA.

Conclusion An IAST ≥5.2 mm measured in a zoomed subcostal four-chamber view at ES might be useful for detecting ATTRwt-CA, regardless of the disease stage of CA.

A 20-year-old Japanese woman moved to Manchuria and worked as a sheep farmer for 5 years. Subsequently, she returned to Japan. In her 80s, abdominal ultrasound and computed tomography revealed a large tumor that had spread to both lobes of the liver. The tumor was encapsulated, and partial calcification and multiple cysts within a large cyst (honeycomb pattern) were observed. Serological tests for echinococcosis showed that the Echinococcus-specific antigen was positive, but the antigen characteristic of E. multilocularis was negative. We herein report a case of E. granulosus infection diagnosed more than 60 years after the initial infection.

The optimal dose of chemotherapy for patients undergoing hemodialysis remains unclear. A 65-year-old man with diabetic nephropathy was treated with nivolumab plus modified FOLFOX6 therapy for recurrent gastric cancer with liver metastases. Oxaliplatin was reduced to 65 mg/m², continuous infusion of 5-fluorouracil was initiated at 2,000 mg/m², and bolus administration was discontinued. No nivolumab was administrated. Dose modification and discontinuation were performed depending on the patient's general condition and blood test during the treatment course. He received standard chemotherapy for gastric cancer; however, it was ineffective because of ascites and elevated CA125 levels.

Gastric infection by non-Helicobacter pylori Helicobacter (NHPH) species causes gastric disorders, including mucosa-associated lymphoid tissue (MALT) lymphoma. We herein report a dog lover with MALT lymphoma associated with a H. heilmannii sensu stricto infection. H. heilmannii s.s.-associated MALT lymphoma was detected one year after the eradication of H. pylori against lymphoid aggregates. Given that NHPH species colonize the stomachs of cats and dogs, gastric colonization by H. heilmannii s.s. might be involved in the development of MALT lymphoma in this dog lover. Therefore, it is crucial to consider the possibility of gastric infection with NHPH species in cat or dog lovers.

A male patient in his 60s with advanced heart failure and a HeartMate3 left ventricular assist device (LVAD) was admitted to our hospital for the sixth time in one year due to recurrent small-bowel bleeding. Nine months after admission, conservative treatment failed to achieve stable hemostasis. Gastroenterologists and surgeons performed double-balloon enteroscopy with mini-laparotomy and achieved complete hemostasis with endoscopic clipping and surgical suturing from the serosal side to multiple bleeding sites. The patient was discharged without any signs of rebleeding. In conclusion, our method represents an effective strategy for managing challenging SBB cases in patients with LVAD.

A 77-year-old man who was referred to our hospital because of dizziness and elevated troponin I level. Electrocardiography showed sinus bradycardia and a tri-fascicular block. Computed tomography revealed no coronary artery stenosis, but late gadolinium enhancement of the basal interventricular septum was detected on magnetic resonance imaging (MRI). Cardiac sarcoidosis was initially suspected, but pathological findings demonstrated extracellular amyloid deposition on the direct fast scarlet. The patient was diagnosed with cardiac amyloidosis (CA). The MRI manifestation in this case was atypical for CA; however, CA should not be overlooked.

A 37-year-old Japanese female presented with stomach ache and was found to have a 21-mm adrenal incidentaloma on abdominal computed tomography (CT). Initial tests, including a 1 mg dexamethasone suppression test, showed normal cortisol levels. Over 3 years, the tumor enlarged to 32 mm, and she developed sweating and palpitations. Elevated adrenaline and noradrenaline levels suggested a pheochromocytoma, although MIBG scintigraphy showed no accumulation. Laparoscopic left adrenalectomy was performed, and the tumor was confirmed as a pheochromocytoma. Postoperatively, the patient's symptoms and catecholamine levels normalized. This case emphasizes the need for ongoing monitoring of adrenal incidentalomas.

We herein report a woman with autosomal dominant Alport syndrome (ADAS) with a family history of left ventricular noncompaction cardiomyopathy (LVNC). Exome sequencing identified a rare heterozygous variant in COL4A4, NM_000092.5: c.2510G>C (p.G837A), and a novel heterozygous variant in ACTC1, NM_005159.5: c.922T>C (p.Y308H), as causes of ADAS and LVNC, respectively. The cardiac phenotype was presumed to have been inherited paternally, whereas the renal phenotype was assumed to be maternally inherited. These two variants independently contributed to clinical phenotypes. This case highlights the clinical significance of comprehensive genetic testing for facilitating the precise diagnosis of rare and complex hereditary disorders.

An 86-year-old man developed syndrome of inappropriate antidiuresis (SIAD) following herpes zoster ophthalmicus (HZO). The patient presented with symptomatic hyponatremia and impaired consciousness. Hypertonic and isotonic saline infusions improved both parameters; however, oral sodium supplementation and fluid restriction alone failed to maintain normonatremia. Tolvaptan was added, resulting in temporary correction of sodium levels; however, SIAD relapsed after discontinuation. Ultimately, SIAD resolved with improvement in neuropathic pain. This case highlights a therapeutic strategy for symptomatic hyponatremia and the appropriate use of tolvaptan in refractory SIAD, and provides a review of HZO-induced SIAD, an uncommon but noteworthy clinical entity.

Aspiration pneumonia is one of the most severe complications associated with near-drowning and is referred to as drowning-associated pneumonia (DAP). Inhaled water may be contaminated by various pathogens, thereby facilitating DAP development. The causative organisms of DAP differ from those of community-acquired pneumonia, with Gram-negative bacilli being predominant.

We herein report an 83-year-old Japanese man with chronic kidney disease who presented to the emergency department after a near-drowning episode. His oxygen saturation level was 86% despite receiving oxygen via a 10 L/min reservoir mask. He was admitted to the intensive-care unit, where high-flow nasal cannula therapy (50 L/min with an FiO₂ of 1.0) was initiated. His respiratory condition gradually improved, and he was transferred to the general ward. However, on day 10 of hospitalization, the patient developed a fever. Chest computed tomography (CT) revealed a new cavity in the right upper lobe, which was suggestive of a lung abscess. Ceftriaxone was initiated but was ineffective. Sputum culture on day 10 revealed Pseudomonas aeruginosa, and cefepime was subsequently administered. His fever resolved, and follow-up CT on day 23 showed marked improvement in the cavity and surrounding consolidation. Due to suspected cefepime-induced acute kidney injury, the antibiotics were switched to moxifloxacin, and the patient was discharged on hospital day 28.

Following macroaspiration events, clinicians should evaluate the risk of pneumonia and likely pathogens. In patients who develop pneumonia after near-drowning, empirical coverage of Gram-negative organisms, including P. aeruginosa, should be considered.

We herein report a case of successful rechallenge with tepotinib after tepotinib-induced adverse events following first-line treatment failure with pembrolizumab in a patient with non-small cell lung carcinoma harboring mesenchymal-epithelial transition factor exon 14 skipping mutations. Initial tepotinib administration was suspended because of hepatotoxicity and hypersensitivity. However, the re-administration of tepotinib after a prolonged interval from pembrolizumab administration achieved sustained disease control. The patient continued tepotinib treatment for approximately two years after the first administration. Therefore, the treatment sequence should be considered based on comprehensive multigene testing results to achieve better therapeutic benefits.

Tumor management is the cornerstone of treatment for anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis that is refractory to immunosuppressive therapy. However, little is known about the effectiveness of chemotherapy for malignant tumors in patients with severe neurological conditions.

We herein report a 66-year-old male who fell into a coma caused by anti-NMDAR encephalitis associated with small cell lung cancer (SCLC). Corticosteroid pulse therapy failed to improve his neurological condition, but a chemotherapy regimen containing atezolizumab led to rapid improvement in his condition.

This case suggests that chemotherapy, including atezolizumab, may provide a rapid therapeutic benefit for anti-NMDAR encephalitis associated with SCLC.

Pulmonary pleomorphic carcinoma, a rare chemoresistant sarcomatoid lung cancer, responds to immune checkpoint inhibitors (ICIs). ICIs are effective when programmed death-ligand 1 expression is high. We treated a 61-year-old man with pembrolizumab whose spindle cell carcinoma regressed. However, salvage resection of a slowly enlarging residual nodule revealed adenocarcinoma, prompting a reconsideration of the entire clinical course. The lesion was ultimately considered suggestive of pulmonary pleomorphic carcinoma, underscoring the pronounced intratumoral heterogeneity with variable ICI sensitivity. When durable disease control is achieved with ICIs in pulmonary pleomorphic carcinoma, this heterogeneity should prompt consideration of local therapies, particularly surgical excision, for recurrent foci.

We herein report a 46-year-old patient with primary ciliary dyskinesia (PCD) caused by a large deletion spanning exons 5-11 of DNAAF11 accompanied by a hemizygous nonsense variant. The PICADAR score was 14, which was based on the presence of heterotaxy, congenital heart disease, history of neonatal respiratory distress, history of neonatal intensive care unit admission, chronic sinusitis, and otitis media. In addition, the patient's nasal nitric oxide level was markedly low at 30.7 nL/min. These findings suggest that the clinical features of DNAAF11-related PCD are typical of PCD.

We herein report two cases of immunotactoid glomerulopathy (ITG) associated with multiple myeloma treated with daratumumab-based regimens. The first patient was an 81-year-old woman with severe renal insufficiency and IgAκ multiple myeloma (MM) that progressed to end-stage renal disease despite administering daratumumab-based therapy. The second patient, a 69-year-old man with smoldering MM, showed a favorable response to daratumumab-based treatment, with a resolution of nephrotic proteinuria. These cases underscore the association between hematological malignancies and ITG, while also emphasizing the need for clone-directed therapy. Although further accumulation of cases is needed, myeloma regimens, including daratumumab-based regimens, may be a beneficial treatment for ITG accompanied by plasma cell dyscrasia.

We present the case of a 78-year-old patient with B-cell acute lymphoblastic leukemia (B-ALL), characterized by the presence of pseudo-Chédiak-Higashi (PCH) granules in lymphoblasts. A morphological analysis using transmission electron microscopy revealed heterogeneous blasts with irregular and bilobed nuclei containing PCH granules, identified as autophagosomes. A genomic analysis revealed mutations in IKZF1 and KRAS. The patient's condition was refractory to induction chemotherapy. The morphological heterogeneity of B-ALL with PCH granules poses a diagnostic challenge. Few reports have so far described cases of B-ALL with PCH granules refractory to induction therapy, thus highlighting the need for further investigation to better understand its clinical and pathological features.

A 41-year-old man with high-grade B-cell lymphoma developed neurolymphomatosis (NL) after frontline chemotherapy. He received CD19 chimeric antigen receptor (CAR) T-cell therapy (lisocabtagene maraleucel) and subsequently developed tumor inflammation-associated neurotoxicity (TIAN) type 2, a localized neurological event distinct from immune effector cell-associated neurotoxicity syndrome. Conservative treatment resulted in a neurological recovery and complete remission. This case suggests that CD19 CAR T-cell therapy may be effective for NL, and it highlights the importance of recognizing TIAN in patients with central nervous system involvement. As CAR T-cell use has increased in Japan, awareness of such complications is crucial for performing safe and effective treatment.

Pleural effusion following chimeric antigen receptor (CAR) T-cell therapy is typically transient, bilateral, and associated with cytokine release syndrome. We herein report persistent unilateral massive pleural effusion with CAR T-cell infiltration in a 34-year-old man with refractory primary mediastinal large B-cell lymphoma involving pleural lesions treated with axicabtagene ciloleucel. Flow cytometry confirmed CAR T-cell infiltration into the pleural fluid. Although temporarily improved by corticosteroids, the effusion persisted for several months but eventually resolved spontaneously. Physicians should consider CAR T-cell infiltration in persistent unilateral pleural effusion after CAR T-cell therapy, emphasizing the importance of flow cytometry for the diagnosis and potential spontaneous resolution without corticosteroids.

A 34-year-old Japanese man developed bilateral periorbital pain and cranial autonomic symptoms that mimicked cluster headache after carotid artery stenting. Imaging revealed hemorrhagic infarction and multifocal vasoconstriction, confirming reversible cerebral vasoconstriction syndrome (RCVS). He had a history of radiotherapy for nasopharyngeal carcinoma, which likely contributed to the delayed vascular injury, including carotid stenosis and vertebral artery dissection. Combined with procedural stress from stenting, these factors may have triggered the RCVS. This case highlights the importance of considering RCVS in patients presenting with atypical headaches after endovascular intervention, particularly in those with prior radiation exposure. Repeat neuroimaging is crucial for a timely diagnosis.

Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic disorder characterized by either an arterial rupture or organ perforation, which is challenging to diagnose prior to the onset of major complications. A 46-year-old male presented with sudden severe headache and left hemiplegia. Magnetic resonance imaging (MRI) revealed simultaneous cerebral infarction and subarachnoid hemorrhage (SAH) due to middle cerebral artery (MCA) dissection. Genetic testing confirmed vEDS through identification of a pathogenic COL3A1 variant (c.2438G>A). Clinicians should consider vEDS in young patients demonstrating stroke with arterial dissection, especially in the presence of distinctive facial features or a relevant family history, even if the diagnostic criteria are not fully met.

We herein report a case of chronic inflammatory demyelinating polyneuropathy (CIDP) that was ultimately identified as hereditary neuropathy with liability to pressure palsies (HNPP) based on PMP22 gene testing. The patient exhibited gradually progressive relapsing-remitting sensorimotor symptoms without obvious nerve compression episodes and received immunotherapy for CIDP for one year without improvement. This case highlights the clinical and electrophysiological challenges of differentiating long-standing HNPP from CIDP. In refractory CIDP cases with bilateral foot drop and immunotherapy resistance, HNPP should be considered and genetic testing performed, even in the absence of a family history.

We herein report a rare case of progressive supranuclear palsy (PSP) that initially presented as progressive fluent aphasia and echolalia. A 75-year-old woman exhibited significant language impairments for 2 years before developing motor symptoms, including vertical gaze palsy and frequent falls. Neuroimaging revealed asymmetric frontal and midbrain atrophy, with reduced perfusion in the left frontal and parietal lobes. This case highlights the importance of recognizing atypical PSP presentations, particularly language-dominant variants that may mimic other neurodegenerative disorders. Early identification using detailed neuropsychological assessments and imaging may facilitate a timely diagnosis and intervention in non-classical PSP cases.

Anti-signal recognition particle (SRP) myopathy has recently been regarded as immune-mediated necrotizing myopathy (IMNM). Anti-SRP myopathy primarily presents with muscle symptoms, but it can also involve extramuscular symptoms, among which cardiac disease can be fatal. Despite its potentially severe course, anti-SRP myopathy often resists conventional immunosuppressive therapy. A 72-year-old woman diagnosed with anti-SRP myopathy developed cardiomyopathy and pericardial effusion. The disease could not be controlled with prednisolone (PSL) and tacrolimus (TAC), but an improvement was achieved with intensified therapy using intravenous immunoglobulin (IVIG). Early intensification of IVIG may therefore improve the patient prognosis.

A 40-year-old man with acquired immunodeficiency syndrome (AIDS) presented with severe thrombocytopenia and disseminated Kaposi's sarcoma (KS). Initially diagnosed with immune thrombocytopenic purpura (ITP), he was treated with corticosteroids and antiretroviral therapy; however, his condition worsened. He met the criteria for Kaposi's sarcoma-associated inflammatory cytokine syndrome (KICS) and received liposomal doxorubicin, showing temporary improvement. KS progression was observed after immune recovery, suggesting KS-associated immune reconstitution inflammatory syndrome. Rituximab was administered, but the patient deteriorated and died. This case highlights the difficulty in distinguishing KICS from ITP, and the importance of an early diagnosis and multidisciplinary care in advanced AIDS.

Rapid molecular and immunological assays for tuberculosis, including loop-mediated isothermal amplification (LAMP) and T-SPOT.TB assays, may yield false-negative results. We herein report a 62-year-old immunocompetent man with a productive cough, elevated C-reactive protein (22.8 mg/dL) and procalcitonin (2.7 ng/mL) levels, and computed tomography findings suggestive of nontuberculous mycobacterial infection. Despite the acid-fast bacilli-positive smears, two separate Mycobacterium tuberculosis LAMP assays and a T-SPOT.TB assay were negative. Empirical treatment with ampicillin/sulbactam and erythromycin improved the patient's condition. Four-week cultures confirmed pulmonary tuberculosis. This case underscores the importance of interpreting rapid assays within the clinical context and supports the use of reflex polymerase chain reaction for a definitive diagnosis.