Hiroaki Yaguchi

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Rheumatoid meningitis (RM) is one of the most severe complications of rheumatoid arthritis. The mortality rate of RM is relatively high and diagnosis can be difficult. We present an 80-year-old woman who was diagnosed with microscopic findings of RM after analysis of biopsy specimens taken from a brain lesion. MRI scanning revealed meningeal enhancement in(More)
BACKGROUND Myoclonic epilepsy with ragged-red fibres (MERRF) and mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) are established phenotypes of mitochondrial encephalomyopathy. The m.8356T>C transition in the mitochondrial tRNA(Lys) gene is a pathogenic mutations of MERRF. The m.3243A>G transition in the mitochondrial tRNA(Leu)(More)
OBJECTIVE To characterize factors that contribute to symptomatic narcolepsy and excessive daytime sleepiness in neuromyelitis optica and multiple sclerosis. SETTING Japanese university hospitals. DESIGN Case study. PATIENTS Seven Japanese patients whose initial diagnoses were multiple sclerosis and who were exhibiting excessive daytime sleepiness. (More)
Mutations in the progressive external ophthalmoplegia 1 (PEO1), adenine nucleotide translocator 1 (ANT1) and DNA polymerase gamma (POLG) genes were reported in patients with progressive external ophthalmoplegia and parkinsonism. However, the genotype–phenotype correlation and pathophysiology of these syndromes are still unknown. In order to define the(More)
OBJECTIVE Neuromyelitis optica (NMO) is an inflammatory disease that affects the optic nerve and spinal cord. Optic neuritis and longitudinally extensive myelitis associated with systemic autoimmune disease have been recently defined as NMO spectrum disorder (NMOSD). In this study, we report the efficacy of intravenous cyclophosphamide (IVCY) therapy for(More)
Title Pathology of frontotemporal dementia with limb girdle muscular dystrophy caused by a DNAJB6 mutation Author(s) Yabe, Ichiro; Tanino, Mishie; Yaguchi, Hiroaki; Takiyama, Akihiro; Cai, Huaying; Kanno, Hiromi; Takahashi, Ikuko; Hayashi, Yukiko K.; Watanabe, Masashi; Takahashi, Hidehisa; Hatakeyama, Shigetsugu; Tanak, Shinya; Sasaki, Hidenao Citation(More)
OBJECTIVE To confirm the reported findings and clarify unknown clinical features of Churg-Strauss syndrome (CSS)-associated neuropathy and design appropriate treatment. PATIENTS AND METHODS We assessed the clinical features of 6 patients with CSS-associated neuropathy. RESULTS Mononeuritis multiplex was present in 4 cases and polyneuropathy in the(More)
OBJECTIVE This study was to clarify the neuropathological findings of acute encephalomyelopathy with Sjögren's syndrome. METHODS We examined an autopsied case of acute encephalomyelopathy with Sjögren's syndrome. CASE REPORT A 40-year-old woman developed acute myelopathy and brainstem dysfunction. Magnetic resonance imaging (MRI) revealed high-intensity(More)
Relapsing polychondritis (RP) is a rare multisystem autoimmune disease. Though meningitis in RP is not common, some cases with cerebrospinal fluid (CSF) pleocytosis of the lymphocyte cells have been reported. Of the 18 previously reported cases, two cases demonstrated pleocytosis of polymorphonuclear leukocytes (PMN) in the CSF. In addition, cases with a(More)
A pregnant woman with extensive brain lesions on magnetic resonance imaging was tested positive for anti-aquaporin4 (AQP4) antibodies. An open biopsy of the left temporal lobe showed pathological changes in both the white and gray matter. Hematoxylin and eosin, Klüver-Barrera, and myelin basic protein staining results were indicative of demyelination in the(More)