Strategy for lung parenchyma-sparing bronchial resection: a case series report

Lung parenchyma-sparing bronchial resection is uncommon, and the operative procedure depends on the cause and location of the stenosis. We present 6 cases and discuss the different surgical strategies for sleeve resection of the central airway without lung resection. Bronchoplasty for the main bronchus and truncus intermedius was performed with a posterolateral approach. We resected the right main bronchus including the right lateral wall of the lower trachea and half of the carina obliquely and performed an anastomosis. The tumour in the left lobar bronchus was exposed and removed by transient division of the accompanying pulmonary artery. Although post-transplant stenosis and malacia can pose a challenge, bronchoplasty can be used as a definitive treatment in experienced centres

Occupational risk factors for Parkinson's disease: a case-control study in Japan

<p>Abstract</p> <p>Background</p> <p>The evidence for associations between occupational factors and the risk of Parkinson's disease (PD) is inconsistent. We assessed the risk of PD associated with various occupational factors in Japan.</p> <p>Methods</p> <p>We examined 249 cases within 6 years of onset of PD. Control subjects were 369 inpatients and outpatients without neurodegenerative disease. Information on occupational factors was obtained from a self-administered questionnaire. Relative risks of PD were estimated using odds ratios (ORs) and 95% confidence intervals (CIs) based on logistic regression. Adjustments were made for gender, age, region of residence, educational level, and pack-years of smoking.</p> <p>Results</p> <p>Working in a professional or technical occupation tended to be inversely related to the risk of PD: adjusted OR was 0.59 (95% CI: 0.32-1.06, <it>P </it>= 0.08). According to a stratified analysis by gender, the decreased risk of PD for persons in professional or technical occupations was statistically significant only for men. Adjusted ORs for a professional or technical occupation among men and women were 0.22 (95% CI: 0.06-0.67) and 0.99 (0.47-2.07), respectively, and significant interaction was observed (<it>P </it>= 0.048 for homogeneity of OR). In contrast, risk estimates for protective service occupations and transport or communications were increased, although the results were not statistically significant: adjusted ORs were 2.73 (95% CI: 0.56-14.86) and 1.74 (95% CI: 0.65-4.74), respectively. No statistical significance was seen in data concerning exposure to occupational agents and the risk of PD, although roughly a 2-fold increase in OR was observed for workers exposed to stone or sand.</p> <p>Conclusion</p> <p>The results of our study suggest that occupational factors do not play a substantial etiologic role in this population. However, among men, professional or technical occupations may decrease the risk of PD.</p

Alcohol drinking and risk of Parkinson's disease: a case-control study in Japan

<p>Abstract</p> <p>Background</p> <p>Although some epidemiologic studies found inverse associations between alcohol drinking and Parkinson's disease (PD), the majority of studies found no such significant associations. Additionally, there is only limited research into the possible interactions of alcohol intake with aldehyde dehydrogenase (ALDH) 2 activity with respect to PD risk. We examined the relationship between alcohol intake and PD among Japanese subjects using data from a case-control study.</p> <p>Methods</p> <p>From 214 cases within 6 years of PD onset and 327 controls without neurodegenerative disease, we collected information on "peak", as opposed to average, alcohol drinking frequency and peak drinking amounts during a subject's lifetime. Alcohol flushing status was evaluated via questions, as a means of detecting inactive ALHD2. The multivariate model included adjustments for sex, age, region of residence, smoking, years of education, body mass index, alcohol flushing status, presence of selected medication histories, and several dietary factors.</p> <p>Results</p> <p>Alcohol intake during peak drinking periods, regardless of frequency or amount, was not associated with PD. However, when we assessed daily ethanol intake separately for each type of alcohol, only Japanese sake (rice wine) was significantly associated with PD (adjusted odds ratio of ≥66.0 g ethanol per day: 3.39, 95% confidence interval: 1.10-11.0, <it>P </it>for trend = 0.001). There was no significant interaction of alcohol intake with flushing status in relation to PD risk.</p> <p>Conclusions</p> <p>We did not find significant associations between alcohol intake and PD, except for the daily amount of Japanese sake. Effect modifications by alcohol flushing status were not observed.</p

The carboxy-terminal fragment of α1A calcium channel preferentially aggregates in the cytoplasm of human spinocerebellar ataxia type 6 Purkinje cells

Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant neurodegenerative disease caused by a small polyglutamine (polyQ) expansion (control: 4–20Q; SCA6: 20–33Q) in the carboxyl(C)-terminal cytoplasmic domain of the α1A voltage-dependent calcium channel (Cav2.1). Although a 75–85-kDa Cav2.1 C-terminal fragment (CTF) is toxic in cultured cells, its existence in human brains and its role in SCA6 pathogenesis remains unknown. Here, we investigated whether the small polyQ expansion alters the expression pattern and intracellular distribution of Cav2.1 in human SCA6 brains. New antibodies against the Cav2.1 C-terminus were used in immunoblotting and immunohistochemistry. In the cerebella of six control individuals, the CTF was detected in sucrose- and SDS-soluble cytosolic fractions; in the cerebella of two SCA6 patients, it was additionally detected in SDS-insoluble cytosolic and sucrose-soluble nuclear fractions. In contrast, however, the CTF was not detected either in the nuclear fraction or in the SDS-insoluble cytosolic fraction of SCA6 extracerebellar tissues, indicating that the CTF being insoluble in the cytoplasm or mislocalized to the nucleus only in the SCA6 cerebellum. Immunohistochemistry revealed abundant aggregates in cell bodies and dendrites of SCA6 Purkinje cells (seven patients) but not in controls (n = 6). Recombinant CTF with a small polyQ expansion (rCTF-Q28) aggregated in cultured PC12 cells, but neither rCTF-Q13 (normal-length polyQ) nor full-length Cav2.1 with Q28 did. We conclude that SCA6 pathogenesis may be associated with the CTF, normally found in the cytoplasm, being aggregated in the cytoplasm and additionally distributed in the nucleus

Investigating the role of genetic counseling in neuromuscular disease considering life events

Genetic diagnoses are becoming a routine in the medical practice of neuromuscular diseases. Many diagnoses, however, can have an influence on relatives and family members and thus must be handled carefully by genetic counseling (GC). Here, we aimed to assess the purpose of undergoing GC to verify the utility of collaborations between clinical and genetic divisions. We investigated consecutive GC cases of neuromuscular disease and examined the role of GC. Our study included 102 cases who underwent GC in our hospital from July 2005 to March 2018: 86.3% were women and 45.1% were in their 30’s. Disease explanation was the most common reason for attending GC (29.4%), followed by prenatal diagnosis (25.5%), pre-symptomatic diagnosis (17.6%), and carrier diagnosis (14.7%). Clients typically visited the hospital for GC when some kind of life event occurred, such as marriage, had a desire to bear a child, or a change in the condition of the proband. Clinicians should be conscious of such life events from the perspective of both the client and their relatives, and guide the GC at an appropriate time. Overall, the degree of recognition of genetic risk by clients differed; thus, it is important for GC to determine the status of each unique situation and respond individually

FDG-PET SUV can distinguish between spinal sarcoidosis and myelopathy with canal stenosis

Spinal cord sarcoidosis is a rare manifestation of sarcoidosis. Magnetic resonance imaging (MRI) of spinal cord sarcoidosis sometimes resembles that of the non-inflammatory spinal cord lesion. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) is an effective method to detect both systemic and central nervous system lesions in sarcoidosis. This study compared the standard uptake value (SUV) of FDG-PET between spinal cord sarcoidosis and non-inflammatory spinal cord lesions. We retrospectively reviewed the records of patients who underwent both spinal MRI and FDG-PET scans. We used SUV to evaluate the FDG-PET uptake of the lesion. The region of interest was the center of high-intensity areas on T2-weighted MR images. We included three patients with spinal cord sarcoidosis, five with myelomalacia caused by cervical spondylosis or ossification of the posterior longitudinal ligament, one with spinal cord edema associated with cervical spondylosis, and one with spinal cord edema associated with dural arteriovenous fistula. The spinal cord sarcoidosis group had a significantly higher SUV (mean = 4.38, range 3.30-4.93) than patients with the other diseases (mean = 1.87, range 1.42-2.74). The SUV of FDG-PET thus may be able to distinguish spinal cord sarcoidosis from other non-inflammatory lesions. FDG-PET can play an important role in the diagnosis of spinal cord sarcoidosis because the gadolinium enhancement in MRI is sometimes seen in spondylotic myelopathy or vascular malformation. FDG-PET is informative for the accurate diagnosis of spinal cord sarcoidosis and may enable clinicians to start treatment at an earlier stage