[Analysis of Device-Related Infection after Deep Brain Stimulation Surgery].

BACKGROUND: Device-related infection frequently becomes a serious problem after deep brain stimulation(DBS)surgery and DBS device removal is usually the only effective treatment option. In this study, we examined risk factors for infection related to DBS devices at our institution. METHODS: We retrospectively investigated 80 DBS surgeries performed between March 2009 and September 2017 at our institution. We examined the relationship between DBS device-related infection and the following items:duration of electrode placement surgery, total number of tracks of microelectrode recordings(MER), period between surgeries, highest body temperature until implantable pulse generator(IPG)implantation, and patient background characteristics. RESULTS: Four(5.0%)patients developed device-related infection after DBS surgery. Three of them required device removal, whereas one improved following antibiotic treatment alone. We did not identify any specific trend or risk factor for infection. DISCUSSION: We perform DBS surgery in two stages. Patients were implanted with an IPG 2-3 days after electrode placement until August 2016, and at 6-8 days starting in September 2016. All cases of infection developed before September 2016, and no cases of infection have occurred since September 2016. We believe that lengthy surgical electrode placement affects the general status of patients and performing surgery before stabilization might confer a risk of infection. CONCLUSION: Device-related infection after DBS surgery does not seem to be associated with any risk factors. However, a shorter period between two-staged surgeries might affect infection rates.

Lung adenocarcinoma with Lambert-Eaton myasthenic syndrome indicated by voltage-gated calcium channel: a case report.

INTRODUCTION: Lambert-Eaton myasthenic syndrome is a rare disorder and it is known as a paraneoplastic neurological syndrome. Small cell lung cancer often accompanies this syndrome. Lambert-Eaton myasthenic syndrome associated with lung adenocarcinoma is extremely rare; there are only a few reported cases worldwide. CASE PRESENTATION: A 75-year-old Japanese man with a past history of chronic rheumatoid arthritis and Sjögren syndrome was diagnosed with Lambert-Eaton myasthenic syndrome by electromyography and serum anti-P/Q-type voltage-gated calcium channel antibody level preceding the diagnosis of lung cancer. A chest computed tomography to screen for malignant lesions revealed an abnormal shadow in the lung. Although a histopathological examination by bronchoscopic study could not reveal the malignancy, lung cancer was mostly suspected after the results of a chest computed tomography and [18F]-fluorodeoxyglucose positron emission tomography. An intraoperative diagnosis based on the frozen section obtained by tumor biopsy was adenocarcinoma so the patient underwent a lobectomy of the right lower lobe and lymph node dissection with video-assisted thoracoscopic surgery. The permanent pathological examination was the same as the frozen diagnosis (pT2aN1M0: Stage IIa: TNM staging 7th edition). Immunohistochemistry revealed that most of the cancer cells were positive for P/Q-type voltage-gated calcium channel. CONCLUSIONS: Our case is a rare combination of Lambert-Eaton myasthenic syndrome associated with lung adenocarcinoma, rheumatoid arthritis and Sjögren syndrome, and to the best of our knowledge it is the first report that indicates the presence of voltage-gated calcium channel in lung adenocarcinoma by immunostaining.

[A case of cavernous angioma in the lower pons, showing subacute onset of unilateral cranial nerve palsy and segmental sensory disturbance].

We report a 54-year-old man with right abducent nerve palsy, right facial nerve palsy, and left segmental sensory disturbance, which progressed for 2 weeks. He was found to have cavernous angioma in the lower pons. When he visited our hospital, he had right facial palsy, sensory disturbance of left half of the face and left upper limb, and diplopia. He had suffered right abducent nerve palsy 5 years previously and had recently developed hypertension. Neurological examination further revealed right abducent nerve palsy, right peripheral facial nerve palsy, sensory impairment of the left half of the face, and sensory impairment on the left side from C2 to Th3. Magnetic resonance imaging of the head revealed hemorrhage with a rim at the right dorsal part of the lower pons. No abnormalities were identified on cerebral angiography. He was diagnosed as having hemorrhage originating from a cavernous angioma. We assumed that the segmental sensory disturbance was caused by medial involvement of the lateral spinothalamic tract, which is somatotopically arranged: the fibers from the sacral segments being most lateral. The ventral trigeminothalamic tract, right abducent nerve, and right facial nerve were also disturbed. Segmental sensory disturbance usually accompanies a spinal cord lesion. But several cases with similar symptoms following a brainstem lesion have been reported. Most of them had stroke, showing acute onset of illness. Our case showed subacute onset of illness; cranial nerve palsy and segmental sensory disturbance progressed for 2 weeks.

[Anti-MuSK antibody positive myasthenia gravis with HIV infection successfully treated with cyclosporin: a case report].

A-58-year old man presented with fluctuating ptosis and dysphagia. When he was 53 years old, he developed oral candidiasis and serum human immunodeficiency virus (HIV) RNA was detected. After starting highly active antiretroviral therapy, serum HIV RNA became undetectable. Neurological examination revealed ptosis and bulbar symptoms. Myasthenia gravis was comfirmed by a positive edrophonium test, showing 20% decrement of the compound muscle action potential on repetitive stimulation. Anti-acetylcholine receptor antibodies were negative and anti-muscle specific tyrosine kinase (MuSK) antibodies were positive. The chest CT scan was normal. He experienced transient clinical remission with pyridostigmine bromide and prednisolone. However relapse occurred after he returned to work. Persistent clinical remission was first observed after cyclosporin administration. There are eleven reports in which patients had concomitant myasthenia gravis and HIV infection. Most of those cases were benign in clinical course and required only anticholinesterase therapy. In our case, however, anti-MuSK antibodies were positive, and symptoms of myasthenia gravis remained despite prednisolone administration. Cyclosporin is directly active against HIV, and thus, cyclosporine therapy may be helpful in patients with concomitant myasthenia gravis and HIV infection.

Three-repeat Tau 69 is a major tau isoform in laser-microdissected Pick bodies.

By utilizing a novel combinatorial method of a Laser Microdissection System and Western blot analysis, we demonstrate that a distinct isoform of abnormally phosphorylated tau (69 kDa, Tau 69) predominantly aggregated in laser-microdissected Pick bodies (PBs) in sporadic Pick's disease. By contrast, tau migrated as two major bands of 60 and 64 kDa (Tau 60 and 64) in total brain homogenates as previously reported. Comparative immunohistochemical analysis with anti-4-repeat antibody revealed that a major component of the abnormally phosphorylated tau in these PBs was 3-repeat tau (3R-tau). Whether 29 amino acid repeat encoded by exons 2 and 3 in the Tau 69 might accelerate the formation of PBs remains to be further investigated. Such a combination of morphological and biochemical techniques significantly complements the existing histopathological methods.

Association study of the chemokine, CXC motif, ligand 1 (CXCL1) gene with sporadic Alzheimer's disease in a Japanese population.

Inflammation is profoundly involved in the development of Alzheimer's disease (AD) and other neurodegenerative diseases. Chemokine, CXC motif, ligand 1 (CXCL1; or GRO1) is an inflammatory cytokine and appears to be implicated in the pathogenesis of AD. It is of interest and importance to see if the CXCL1 gene, mapped on chromosome 4q12-q13, has potential for conferring the predisposition to AD. Here we report on an association study of the CXCL1 gene with sporadic AD patients in a Japanese population; three single nucleotide polymorphisms (SNPs) in the CXCL1 locus were investigated in 103 AD patients and 130 healthy individuals. The results indicate that neither genotype frequencies nor allele frequencies of the examined SNPs attained statistical significance even after being stratified by the presence or absence of the Apolipoprotein E epsilon4 allele. Therefore, the data presented here suggests that the CXCL1 gene could not be associated with the susceptibility to AD in a Japanese population.

Non-glycosylphosphatidylinositol (GPI)-anchored recombinant prion protein with dominant-negative mutation inhibits PrPSc replication in vitro.

Dominant-negative mouse prion protein (PrP) with a lysine mutation at codon 218 (Q218K) is known to inhibit prion replication. In order to gain further mechanistic insight into such dominant negative inhibition, non-glycosylphosphatidylinositol (GPI)-anchored recombinant PrP with Q218K (rPrP-Q218K) was investigated. When applied into scrapie-infected mouse neuroblastoma (ScN2a) cells, rPrP-Q218K but not wild-type rPrP (rPrP-WT) exclusively inhibited abnormal protease-resistant pathogenic isoform (PrPSc) replication without reducing the viability of the cells. It was even more efficient than quinacrine, which has already been prescribed for sporadic Creutzfeldt-Jakob disease (CJD) patients; 50% effective concentration (EC50) = 0.20 microM, 99% effective concentration (EC99) = 0.86 microM vs. EC50 = 0.45 microM, EC99 = 1.5 microM. Besides, no apparent cell damage was observed at the concentration of up to 4.3 microM (100 micrograms/ml). In combination treatment with 0.43 microM (10 micrograms/ml) of rPrP-Q218K, EC99 of quinacrine was decreased from 1.5 microM to 0.5 microM, and the cell viability was recovered from 50% to over 90% as inversely proportional to the concentration of quinacrine. Such combination could alleviate the side effects of quinacrine by reducing its effective concentration without changing or even acceleration the inhibition efficacy. Since homogeneous, high-quality rPrPs could be easily prepared from Escherichia coli in large quantities, rPrP-Q218K is a good candidate for a prion replication antagonist.

Lack of association between TrkA single nucleotide polymorphisms and sporadic Alzheimer's disease in a Japanese population.

Nerve growth factor and its receptor with tyrosine kinase activity (TrkA) have been implicated in the development of Alzheimer's disease (AD). Entire coding regions of TrkA gene harboring exons 1 through 17 were sequenced in 114 patients with sporadic AD and 112 control subjects in a Japanese population, and six known and two novel single nucleotide polymorphisms were identified, but no mutation associated with sporadic AD was identified. Concurrently, case-control analysis of TrkA gene A1674G polymorphism in 534 patients with sporadic AD and 454 control subjects has revealed no significant differences in TrkA genotype or allele frequencies even after stratification for Apolipoprotein E epsilon4 carrier statuses. Thus, the TrkA genotype does not appear to influence the risk of developing sporadic AD in a Japanese population.