Noteworthy Cardiovascular Involvement with Sporadic Late-onset Nemaline Myopathy.

Cardiac involvement has recently been the focus of sporadic late-onset nemaline myopathy (SLONM). However, right ventricular failure and pulmonary hypertension, in addition to repetitive cardiac arrest, are noteworthy characteristics of SLONM. We herein report a 66-year-old woman with SLONM whose main symptoms were cardiac arrest, right ventricular failure, and pulmonary hypertension. Despite permanent pacemaker replacement, cardiac arrest occurred repetitively, and even with continuous positive airway pressure, right ventricular failure and pulmonary hypertension persisted. The patient was finally diagnosed with SLONM by a muscle biopsy. Our case suggests the possibility of cardiovascular involvement in SLONM, especially right ventricular failure and pulmonary hypertension.

Intrapulmonary solitary fibrous tumour: a case report.

Solitary fibrous tumours (SFTs) mainly originate from the visceral pleura and may protrude to the thoracic cavity, but intrapulmonary SFTs are extremely rare. We describe a rare case of SFT arising in the right lung of an 83-year-old man who underwent surgical excision. Chest computed tomography (CT) revealed a 10-mm tumour in the lower lobe of the right lung. The size of tumour gradually increased and reached 17 mm 2 years after the first radiologic examination. Considering the possibility of malignancy, wedge resection of the right lower lobe was performed via video-assisted thoracic surgery. Microscopically, the tumour consisted mainly of spindle-shaped cells. Immunohistochemical staining indicated the tumour was positive for CD34, STAT6, vimentin and bcl-2, but negative for cytokeratins, D2-40 and S-100. Based on the histological findings, the tumour was diagnosed as SFT. The patient has been in good health for 6 months since the surgery.

Relapsing polychondritis coupling with cerebral amyloid deposit inducing cerebral amyloid angiopathy-related inflammation.

Cerebral amyloid angiopathy-related inflammation is a syndrome of reversible encephalopathy with cerebral amyloid angiopathy, however the pathology is not well understood. We clear a part of the pathology through the first case of an 80-year-old man with cerebral amyloid angiopathy-related inflammation induced by relapsing polychondritis (RP) analysis. An 80-year-old man was diagnosed with RP by auricular cartilage biopsy. Almost no abnormality including intracranial microbleeding was detected by cranial magnetic resonance image (MRI) at diagnosis. However, he developed a headache and hallucination after five months. Seven-month cranial MRI showed novel, multiple, intracranial microbleeding, especially in the bilateral but asymmetry posterior, temporal, and parietal lobes. 123I-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography showed increased cerebral blood flow in the bilateral posterior lobes. After treatment, both of his neurological symptoms and increased cerebral blood flow improved to mild. Photon emission computed tomography using Pittsburgh compound B (PiB) for evaluation of brain amyloidosis at 12 months after onset showed an amyloid deposit in the bilateral frontal lobes, but a lack of uptake corresponded to the RP lesions. Our case suggests that inflammation coupled with an amyloid deposit, induced the multiple intracranial bleeding, and resulted in the lack of PiB uptake. Findings from our case show that inflammation including excess blood flow coupled with an amyloid deposit synergistically facilitate intracranial bleeding.