Bone marrow stroma cells are susceptible to prion infection.

Abnormal protease-resistant prion protein (PrP-res) is the only surrogate biochemical marker for prion diseases, and a sensitive technique to detect PrP-res in blood or tissues is urgently needed. Primary cultured bone marrow stromal cells (MSCs) expressed PrP and were capable of supporting stable human prion infection. Using a mouse-adapted BSE strain, we demonstrated that PrP-res can be detected in expanded MSCs.

Hot spots in prion protein for pathogenic conversion.

Prion proteins are key molecules in transmissible spongiform encephalopathies (TSEs), but the precise mechanism of the conversion from the cellular form (PrP(C)) to the scrapie form (PrP(Sc)) is still unknown. Here we discovered a chemical chaperone to stabilize the PrP(C) conformation and identified the hot spots to stop the pathogenic conversion. We conducted in silico screening to find compounds that fitted into a "pocket" created by residues undergoing the conformational rearrangements between the native and the sparsely populated high-energy states (PrP*) and that directly bind to those residues.

[A case of brachial amyotrophic diplegia accompanied with Sjögren's syndrome presenting good response to immunotherapies in the early course of the disease].

A 74-year-old woman suffered from progressive muscle atrophy and weakness of her arms since she was seventy two years old. Before referral to our department, she was diagnosed as having cervical spondylotic myeloradiculopathy and received spinal fusion. Though spinal decompression was successful, muscle weakness of her upper limbs were progressive even after the surgery.

[Neurosyphilis presenting the left total ophthalmoplegia: a case report].

We report a 73-year-old woman with meningitis-type neurosyphilis presenting the main symptom of the left total ophthalmoplegia. Three months after the appearance of the deviation of the eyeball to the inside and ptosis of the left eyelid, the left eyelid was completely closed. On admission, about four months after the appearance of neurosyphilis, she showed paralysis of the left oculomotor nerve, trochlearis nerve and abducens nerve, and the right mydriasis and absent light reflex.

[A case of Crow-Fukase syndrome with extramedullary plasmacytoma: marked clinical deterioration following a biopsy to plasmacytoma].

A 66-year-old man developed paresthesia of the distal parts of the bilateral lower limbs a week after his upper respiratory infection, followed by the weakness with the legs and paresthesia with the lip area, tongue and finger tips. Those symptoms gradually became worse to the point that he was unable to walk 10 days later. Although skin pigmentation, edema, and lymph node swelling were not found, we made a diagnosis of Crow-Fukase syndrome (CFS) because of clinical features of polyneuropathy, IgG-lambda type M proteinemia, endocrinological abnormality, elevated plasma level of vascular endothelial growth factor (VEGF) and extramedullary plasmacytoma in his abdomen.

[A case of Charcot-Marie-Tooth disease (CMT) type 1 complicated by diabetes mellitus (DM) showing bilateral phrenic nerve palsy].

We here report a 44-year-old woman with Charcot-Marie-Tooth disease (CMT) type 1 who showed severe bilateral phrenic nerve palsy (PNP). She had chronic progressive distal dominant muscle weakness and atrophy since early in her second decade and had been unable to walk by herself due to weakness of the legs since she was 40-years old. At that time, she was diagnosed with diabetes mellitus (DM).