[A Case of Locally Advanced, Unresectable Pancreatic Cancer with Superior Mesenteric Artery Invasion Treated with FOLFIRINOX plus Radiation Therapy, followed by Conversion Surgery].

We report a case of a woman in her 70s who underwent conversion surgery after FOLFIRINOX, followed by radiation therapy for initially locally advanced unresectable pancreatic cancer. She visited her local doctor with a chief complaint of upper abdominal pain. Contrast-enhanced CT scan of the abdomen revealed an irregular mass invading the superior mesenteric artery, and the first and second jejunal arteries(>180°)in the pancreatic uncinate region. Based on imaging, she was diagnosed as UR-LA(sm), cT4N0M0, cStage Ⅲ pancreatic cancer, and underwent 5 courses of modified FOLFIRINOX. Radiation therapy of 50.4 Gy was added for local control, and CA19-9 decreased from 394.1 U/mL to 10.5 U/mL. The treatment effect was judged as RECIST: partial response. The tumor was considered to be potentially curative, and a subtotal stomach preserving pancreaticoduodenectomy was performed 8 months after the initial treatment. The tumor was found to be 3× 2 mm in size, pStage ⅠA, R0, and the response to preoperative chemotherapy: Evans Grade Ⅲ. The patient is alive at 5 months postoperatively without recurrence.

[A Case of Local Recurrent Liver Metastasis from Sigmoid Colon Cancer after Complete Histological Remission, Treated with Rehepatectomy].

We report a case of a patient with sigmoid colon cancer and multiple liver metastases who underwent hepatectomy after chemotherapy and pathological results showed complete remission. However, after chemotherapy was discontinued, the patient developed a local recurrence of the liver metastasis and underwent rehepatectomy. The patient came to our hospital with lower abdominal pain. Colonoscopy revealed a circumferential type Ⅱ, well-differentiated adenocarcinoma. Laparoscopic sigmoidectomy with lymph node dissection was performed. Postoperative CT scan showed multiple liver metastases at S5, S7, and S8. 11 cycles of bevacizumab plus modified FOLFOX(mFOLFOX)were subsequently performed. The liver metastases shrank at all sites, and the patient underwent right hepatectomy. The resected specimen was considered to be in complete remission, with no evidence of viable malignant cells. Postoperatively, bevacizumab plus mFOLFOX was resumed for 6 cycles and the patient remained in remission. However, 3 months after stopping chemotherapy and 1 year and 6 months after hepatectomy, a follow-up CT scan showed local recurrence of the liver edge, and a diagnosis of local recurrence of liver metastasis was made, and a partial hepatectomy was performed. The patient is recurrence-free and resuming modified FOLFOX 9 months after surgery.

A case of Vp4 hepatocellular carcinoma with tumor thrombosis extending into the confluence of the splenic/portal vein achieved a good prognosis with emergent hepatectomy and postoperative adjuvant therapy with lenvatinib.

In this report, we describe a case of highly advanced hepatocellular carcinoma with tumor thrombosis extending into the main portal vein of the pancreas that was successfully treated with adjuvant lenvatinib after right hepatic resection with thrombectomy. A 70-year-old woman was referred from the clinic because of elevated hepatobiliary enzymes. The patient was positive for the hepatitis B virus antigen at our hospital. The tumor markers were highly elevated with alpha-fetoprotein (14.5 U/mL) and protein induced by vitamin K absence (PIVKAII) (1545 ng/mL), suggesting hepatocellular carcinoma. Dynamic abdominal computed tomography showed an early enhanced tumor approximately 6 cm in size and portal vein tumor thrombosis filling the main portal vein, but not extending into the splenic or superior mesenteric vein (SMV). On magnetic resonance imaging 1 week after CT, portal vein tumor thrombosis had extended to the confluence of the splenic vein with the SMV, indicating rapid tumor growth. Thus, we performed emergent right hepatectomy with tumor thrombectomy. Postoperatively, we treated the patient with lenvatinib for a tumor reduction surgery. Fortunately, the patient was alive 2 years postoperatively without recurrence. This case report suggests that a favorable outcome may be achieved with multidisciplinary treatment including resection and postoperative treatment with lenvatinib.

Clinical characteristics of long-term survival with noninvasive ventilation and factors affecting the transition to invasive ventilation in amyotrophic lateral sclerosis.

INTRODUCTION: We evaluated post-noninvasive ventilation survival and factors for the transition to tracheostomy in amyotrophic lateral sclerosis (ALS). METHODS: We analyzed 197 patients using a prospectively collected database with 114 patients since 2000. RESULTS: Among 114 patients, 59 patients underwent noninvasive ventilation (NIV), which prolonged the total median survival time to 43 months compared with 32 months without treatment. The best post-NIV survival was associated with a lack of bulbar symptoms, higher measured pulmonary function, and a slower rate of progression at diagnosis. The transition rate from NIV to tracheostomy gradually decreased over the years. Patients using NIV for more than 6 months were more likely to refuse tracheostomy and to be women. DISCUSSION: This study confirmed a positive survival effect with NIV, which was less effective in patients with bulbar dysfunction. Additional studies are required to determine the best timing for using NIV with ALS in patients with bulbar dysfunction. Muscle Nerve 58:770-776 2018.

Anti-MOG antibody-positive ADEM following infectious mononucleosis due to a primary EBV infection: a case report.

BACKGROUND: Anti-Myelin oligodendrocyte glycoprotein (MOG) antibodies are detected in various demyelinating diseases, such as pediatric acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, and aquaporin-4 antibody-seronegative neuromyelitis optica spectrum disorder. We present a patient who developed anti-MOG antibody-positive ADEM following infectious mononucleosis (IM) due to Epstein-Barr virus (EBV) infection. CASE PRESENTATION: A 36-year-old healthy man developed paresthesia of bilateral lower extremities and urinary retention 8 days after the onset of IM due to primary EBV infection. The MRI revealed the lesions in the cervical spinal cord, the conus medullaris, and the internal capsule. An examination of the cerebrospinal fluid revealed pleocytosis. Cell-based immunoassays revealed positivity for anti-MOG antibody with a titer of 1:1024 and negativity for anti-aquaporin-4 antibody. His symptoms quickly improved after steroid pulse therapy followed by oral betamethasone. Anti-MOG antibody titer at the 6-month follow-up was negative. CONCLUSIONS: This case suggests that primary EBV infection would trigger anti-MOG antibody-positive ADEM. Adult ADEM patients can be positive for anti-MOG antibody, the titers of which correlate well with the neurological symptoms.

Renal dysfunction caused by severe hypothyroidism diagnosed by renal biopsy: a case report.

There is a close relationship between thyroid dysfunction and renal dysfunction. However, thyroid dysfunction can unfortunately result in inaccurate measurements of serum creatinine and cystatin C levels. The chronic decrease in cardiac output due to hypothyroidism can reduce renal plasma flow (RPF) resulting in renal dysfunction. We report the case of a 36-year-old male in whom renal dysfunction detected during a company health check-up was found to be caused by severe hypothyroidism. His serum creatinine levels showed poor results, but serum cystatin C levels were within the normal range. The physician thus prioritized serum cystatin C for assessing the patient's renal function, and concluded that his renal function was normal. He subsequently visited our hospital, aged 36 years, for a comprehensive examination. His serum creatinine level was 1.88 mg/dL and his serum cystatin C level was 0.75 mg/dL, indicating an unusual discrepancy between the two measurements. The patient also presented with fatigue, suggesting hypothyroidism, and we therefore evaluated his thyroid function. His free thyroxine level was below the sensitivity of the assay, while his thyroid-stimulating hormone level was > 100 µIU/mL. A renal biopsy was performed to further explore the underlying cause of his renal dysfunction, which suggested that reduced RPF could be the leading cause of his renal ischemia, with no indications of chronic glomerulonephritis or other abnormalities. His hypothyroidism and renal function improved after thyroid hormone replacement therapy. Given the limited reports of renal biopsy tissue examination during the acute phase of hypothyroidism, the current case provides important information regarding the diagnosis of renal dysfunction in patients with hypothyroidism.

COVID-19-associated pulmonary aspergillosis in hemodialysis patients.

Background: Coronavirus disease 2019 (COVID-19)-associated pulmonary aspergillosis (CAPA) is a fatal complication in the general population. However, there are few reports on CAPA in patients undergoing hemodialysis (HD). Methods: This retrospective observational cohort study was conducted at a single center between December 2020 and June 2021. We enrolled 21 HD patients with COVID-19 undergoing treatment and divided them into two groups, CAPA and non-CAPA (COVID-19 with and without pulmonary aspergillosis), and evaluated their characteristics, clinical outcomes and comorbidities. Results: The log-rank test revealed that the 90-day survival rate after the initiation of treatment for COVID-19 was significantly lower in the CAPA (n = 6) than in the non-CAPA group (n = 15) (P = 0.0002), and the 90-day mortality rates were 66.6% and 0% in the CAPA and non-CAPA groups, respectively. In the CAPA group, four patients died due to respiratory failure (on Days 6 and 20), gastrointestinal bleeding (Day 8) and sepsis (Day 33); the reverse transcription-polymerase chain reaction (RT-PCR) for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) remained positive when they died. The remaining two patients survived and the negative conversion of RT-PCR for SARS-CoV-2 was confirmed on Days 10 and 15. The negative conversion of serum (1, 3)-ß-d-glucan (BDG) was confirmed on Day 15 in one patient; the BDG remained positive on Day 64 in the other. Conclusions: CAPA is a fatal complication in HD patients and the general population. Therefore, clinicians should consider the possibility of testing for CAPA in patients undergoing HD. Mycological workups may be helpful for the early detection of CAPA.

Reduced port laparoscopic surgery using umbilical zigzag incision for Meckel's diverticulitis.

INTRODUCTION: Reduced port laparoscopic surgery using an umbilical zigzag incision is comparable to conventional multiport laparoscopic surgery. This method is associated with improved cosmesis and decreased wound pain. PRESENTATION OF CASE: A 67-year-old man visited our hospital. He presented emergency room with a chief complaint of right lower abdominal pain by walking. The patient was diagnosed Meckel's diverticulitis by computed tomography (CT). At first, antibiotics therapy and fasting were performed. Three months later, the patient underwent resection of Meckel' diverticulum with zigzag transumbilical laparoscopic surgery. The patient's postoperative course was good, and he rarely felt wound pain. The patient started oral intake three days after surgery, and was discharged 10 days after surgery. DISCUSSION: Zigzag transumbilical laparoscopic surgery is very useful for resection of the small intestine. This method is associated with improved cosmesis and decreased wound pain. In the case of Meckel's diverticulitis, we suggest that interval resection of Meckel's diverticulum was very useful as it was an operation that could be performed easily. CONCLUSION: Reduced port laparoscopic surgery using an umbilical zigzag incision is considered to be an excellent technique in terms of operability and aesthetic outcomes.

Acute tubulointerstitial nephritis and IgM deposits on glomerular capillary walls after immunotherapy with nivolumab for metastatic renal cell carcinoma.

Nivolumab is an anti-programmed cell death-1 antibody that is utilized as an immune checkpoint inhibitor for several malignancies. However, this agent is associated with immune-related adverse events (irAEs), mainly in the spectrum of autoimmune disease including interstitial pneumonia, colitis, type 1 diabetes, and renal impairment. We herein present the case of a 59-year-old man with renal cell carcinoma who developed worsening renal function approximately 4 months after initiation of nivolumab. Urinalysis showed proteinuria and microscopic hematuria along with increase levels of N-acetyl-ß-D-glucosaminidase. Renal biopsy revealed acute tubulointerstitial nephritis and thickening of the glomerular basement membranes. Immunofluorescence showed granular IgM deposits in capillary loops. We initiated high-dose prednisolone therapy with nivolumab, which improved renal function and achieved complete remission of proteinuria. Although renal irAEs are considered to be rare and glomerulonephropathy is not typical presentation, physicians need the close monitoring of renal function and urinalysis in patients under immunotherapy with this agents. In addition, our case provides a possible link between nivolumab and immune-mediated glomerulonephropathy.

Up-regulated expression of ADAM17 in gastrointestinal stromal tumors: coexpression with EGFR and EGFR ligands.

Metalloproteinase activities of a disintegrin and metalloproteinases (ADAMs), matrix metalloproteinases (MMPs), and membrane type (MT-)MMPs are involved in many aspects of tumor biology. ADAMs are transmembrane proteins that cleave membrane-anchored proteins to release soluble factors, and thereby mediate important biological phenomena in tumors. The aim of this study was to analyze histopathology, expression and roles of metalloproteinases, especially ADAMs, in gastric gastrointestinal stromal tumor (GIST). Histopathology and immunohistochemical expression of ADAMs were examined in 89 gastric GISTs. In 11 GISTs, ADAM expression was examined at mRNA and protein levels by reverse transcription-polymerase chain reaction (RT-PCR) and immunoblotting, respectively. RT-PCR analysis showed frequent expression of ADAM9 (91%), ADAM10 (64%), ADAM17 (82%), MMP-2 (82%), and MT1-MMP (73%). However, ADAM17 and MMP-2 were the only metalloproteinases that were up-regulated in GISTs at the protein level compared with non-neoplastic gastric tissues. ADAM17 was immunohistochemically expressed in 93% of GIST versus 16% of normal gastric tissues. Furthermore, CD117-positive interstitial cells of Cajal in normal gastric tissues were all negative for ADAM17 with double immunostaining. Expressions of epidermal growth factor receptor (EGFR) and several EGFR ligands such as amphiregulin, heparin-binding epidermal growth factor (HB-EGF), betacellulin, and epiregulin were also demonstrated in GIST by RT-PCR. Protein expression of EGFR, phosphorylated EGFR, amphiregulin, and HB-EGF, both of which can be shed by ADAM17, was confirmed in tumors coexpressing ADAM17 by immunoblotting. Moreover, proteolytically cleaved soluble forms of amphiregulin were identified in tumor extracts. Considered together, the results suggest that ADAM17 may contribute to the progression and growth of GIST through shedding of EGFR ligands and consequent EGFR stimulation. ADAM17, as a major sheddase in GIST, could be potentially a suitable target in anticancer treatment of imatinib-resistant GISTs.

Occurrence of cerebral small vessel disease at diagnosis of MPO-ANCA-associated vasculitis.

BACKGROUND: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often causes peripheral nervous system impairments. However, little is known about subclinical involvements of the central nervous system in AAV. We investigated the frequency and progression of cerebral small vessel disease (SVD) in patients with AAV. METHODS: This single-center, case-control study comprised 56 patients with myeloperoxidase (MPO)-ANCA-positive AAV. Cerebral SVD presenting periventricular and deep white matter hyperintensities was assessed using brain magnetic resonance imaging (MRI). Seventy-five patients with non-stroke-associated neurological diseases were employed as controls. RESULTS: At clinical diagnosis of MPO-ANCA-positive AAV, the frequency of periventricular hyperintensities in the AAV group was significantly higher than that in the control group (P = 0.014). Shinohara and Fazekas grades of periventricular hyperintensities in the AAV group were significantly higher than those in the control group (P = 0.019 and 0.020, respectively). In the AAV group, atherosclerosis-related factors, such as age and hypertension, were not associated with the Shinohara grades of periventricular hyperintensities, whereas serum CRP levels were significantly associated (odds ratio = 6.000, 95% confidence interval 1.648-21.840, P = 0.004). MRI changes were followed in 23 patients with AAV until 2 years after 6 months of diagnosis. Six of these patients worsened the grades of periventricular hyperintensities, while two of 27 in the control group worsened the grades (P = 0.013). CONCLUSION: Inflammatory events are associated with the occurrence of cerebral SVD before clinical diagnosis of MPO-ANCA-positive AAV. The patients may be continuously exposed to the risk of cerebral SVD after immunosuppressive therapy.

Herpes simplex encephalitis without cerebrospinal fluid pleocytosis in a patient with bullous pemphigoid: a case report.

A 78-year-old woman was diagnosed with bullous pemphigoid 2 months ago, and she had been treated with steroid and plasmapheresis. She developed sudden fever, vomiting, disorientation, and abnormal behavior. Diffusion weighted images and fluid-attenuated inversion recovery (FLAIR) magnetic resonance (MR) images showed high-intensity signals in the right temporal lobe hippocampus and right insular cortex. Cerebrospinal fluid (CSF) examination showed normal cell count (4/mm(3)), but was positive for HSV1-DNA by PCR. She was diagnosed with herpes simplex encephalitis (HSE), and acyclovir was started on the first day of admission. She had complete recovery, and was discharged. She didn't show CSF pleocytosis throughout her course of HSE. No CSF pleocytosis could be due probably to her immunosuppressed state under the steroid therapy for bullous pemphigoid. Because the morbidity and mortality of HSE is drastically reduced by early antiviral treatment, it is important to accelerate the diagnosis and treatment of HSE, especially in immunosuppressed or immunocompromised hosts.

Intraductal tubular adenoma of the pancreas, pyloric gland type: a clinicopathologic and immunohistochemical study of 6 cases.

The intraductal tubular adenoma (ITA), pyloric gland type, of the pancreas is an uncommon benign tumor, akin to the pyloric gland type adenoma of the gallbladder. We report 6 cases of ITA of the pancreas: 3 male and 3 female aged 50 to 79 years (mean, 63.5 years; median, 65 years); all were examined clinicopathologically. Four patients showed no symptoms, but appetite loss and/or general fatigue presented in two. Grossly, all tumors formed a localized polypoid mass protruding into the lumen of the dilated pancreatic duct. Five of the six tumors were found within the main duct, and the other arose within the branch duct of the pancreas. Microscopically, the tumors were composed of closely packed tubular glands resembling pyloric type glands. They were lined by columnar or cuboidal epithelial cells with foci of mild to moderate dysplastic change. In 2 cases, the adjacent pancreas showed foci of intraductal papillary-mucinous adenoma. Histochemically, the tumors largely showed neutral mucin with a lesser amount of acidic mucin made up mainly of sialomucin. Endocrine cells were found in five tumors. Immunohistochemically, all tumors were labeled with M-GGMC-1 and MUC6, whereas MUC1 and MUC2 stains were negative. Pepsinogen II was positive in 5 tumors; thus, the results displayed a pattern of differentiation similar to those of ordinary gastric pyloric or metaplastic pyloric glands. DPC4 expression was maintained in all tumors and p53-positive nuclei were hardly encountered. All patients are alive with no evidence of disease 3 to 10.5 years after surgical resection.

[Recurrence of esophageal cancer treated by combination TS-1/CDDP therapy].

A 68-year-old man underwent subtotal esophagectomy with two fields lymphadenectomy and postoperative chemotherapy so called low dose FP therapy for advanced esophageal cancer (Stage IIIa, pT 3, pN 1, M 0) in October 1999. As he was diagnosed with a recurrence of esophageal cancer as metastatic lymph node tumors which were placed in the right anterocervical and supraclavicular region in March 2001, he underwent enucleation of metastatic lymph node tumors and postoperative chemoradiation therapy, so-called low-dose FP-R therapy. Recently, since other metastatic lymph node tumors in the neck appeared again in August 2001, he underwent radical neck lymph node dissection and postoperative chemoradiation treatment, so-called FAP-R therapy. In October 2003, a chest CT showed multiple lung tumors. He was diagnosed with multiple metastatic lung tumors originating from esophageal cancer. Then, two courses of a combined chemotherapy consisting of TS-1 and CDDP were administered at an interval of one month. We judged the effect of this chemotherapy to be a partial response (PR), because the largest metastatic lung tumor 18 mm in diameter showed a reduction rate of 81.9%, and other tumors had almost disappeared in the chest CT after the combined therapy. No severe adverse effects of more than grade 3 were observed during this combined therapy. This combined chemotherapy consisting of TS-1 and CDDP may prove effective for treating recurrent cases of esophageal cancer.

Reduced expression of heterogeneous nuclear ribonucleoprotein B1 in adult T-cell lymphoma/leukemia.

It is considered that hnRNP B1 expresses similarly in the various types of tumor cells. Recently, we demonstrated high B1 expression in B-cell lymphoma and carcinoma. To evaluate the difference of B1 expression between B and T-cell lymphoma, we immunologically studied the B1 expression in 22 cases with nodal T-cell lymphoma, comprising adult T-cell leukemia/lymphoma (ATLL; n=15) and angioimmunoblastic T-cell lymphoma (AILD; n=7), using an anti-hnRNP B1 monoclonal antibody, 2B2. In ATLL cases, scattered large transformed lymphoma cells demonstrated strong B1 expression, while the medium-sized lymphoma cells were negative. On the one hand, lymphoma cells in AILD diffusely expressed B1. The mean B1 expression rate in ATLL was 22%, which was significantly lower than that in AILDs (45%), B-cell lymphomas (44%), and metastatic carcinomas (53%) (p<0.01). Our result might suggest that process of hnRNP B1 expression in ATLL differs from those in other lymphoid neoplasms and carcinoma.

Overexpression of heterogeneous nuclear ribonucleoprotein B1 in lymphoproliferative disorders: high expression in cells of follicular center origin.

It is reported that overexpression of hnRNP A2 and B1 proteins is useful for detecting early cancers, and that B1, a splicing minor isoform of A2, is more specific than A2. The B1 expression is still undetermined in human lymphoid tissues. We quantitatively studied the B1 expression in 85 lymph node specimens, comprising reactive lymphoid hyperplasia (RLH; n=8), B-cell lymphoma (n=23), T-cell lymphoma (n=22), and metastatic carcinoma (n=32). Immunostaining and immunoblotting analyses with an anti-B1 monoclonal antibody, 2B2 were performed, and the two sets of results correlated with each other (p<0.05). In RLH specimens, B1 expression rate was significantly higher in follicular centers (FC; 44%) than in mantle zone (MZ; 15%) and paracortex (16%) (p<0.01). B1 expression was statistically higher in B-cell lymphoma than in T-cell lymphoma (p<0.01). In B-cell lymphomas, B1 expression rates were 51% in diffuse large B-cell lymphoma (DLBL; n=5) and 45% in follicular lymphoma (FL; n=16), and they were almost the same as that of the FC. Especially in DLBLs, CD10+ FC-origin lymphomas expressed greater amount of B1 than CD10- non-FC-origin lymphomas. B1 expression rate was low in mantle cell lymphoma (MCL; n=2) and similar to that of MZ in RLH. These results suggest that B1 expression is associated with differentiation in lymphoid tissue rather than transformation. B1 expression increases during the process of B-cell differentiation in the FC, and that high B1 expression is maintained in B-cell lymphomagenesis, especially in cells of FC-origin DLBL.

Chronic herpes simplex virus type 2 encephalitis associated with posterior uveitis.

We report on a patient with chronic herpes simplex virus-2 encephalitis who was characteristic for concomitantly having chronic or recurrent posterior uveitis. A 66-year-old immunocompetent man suffering from a 6-month refractory posterior uveitis developed a 1-month history of impaired short-term memory and orientation. Brain MRI demonstrated hyperintense lesions in the right parietal lobe in diffusion and fluid attenuated inversion recovery (FLAIR) sequences. Cerebrospinal fluid (CSF) examination showed mild pleocytosis and increased protein concentration. Quantitative PCR for HSV-2 DNA was positive in CSF. Treated with acyclovir, his cognitive functions gradually improved and the posterior uveitis was cured. Clinicians must be aware that HSV-2 should be considered in the aetiological investigation of chronic encephalitis in an immunocompetent patient. HSV-2 is well known for its ability to cause unilateral chronic or recurrent posterior uveitis. Therefore, posterior uveitis should be considered as an associated feature of HSV-2 encephalitis.

[Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with swelling in the brainstem: a case report].

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare central nervous system inflammatory disease characterized by the punctate gadolinium enhancement peppering the pons and the cerebellar peduncles as neuroimaging. We report the case of a 66-year-old woman who presented with CLIPPERS associated with swelling in the brainstem. She was hospitalized because of gait ataxia and consciousness disturbance. MRI of the brain showed FLAIR hyperintense lesions in the pons, cerebellar peduncles, cerebellum and the subcortical white matter lesion in the right occipital lobe with significant swelling in the brainstem. Diffusion-weighted MRI did not show an abnormal signal, indicating vasogenic edema. Post-contrast T1-weighted MRI showed enhanced area in the right occipital lobe and panctate gadolinium enhancement peppering brainstem. Treatment with steroids led to rapid improvement. However, she showed exacerbation of clinical and radiological findings during the tapering schedule of steroid. The biopsy from the occipital lobe revealed intense perivascular and parenchymal lymphocytic infiltrates composed of primarily T cells, B cells and macrophages. The patient was diagnosed with CLIPPERS, and treatment with increased dose of corticosteroid induced a clinical improvement. Previous reports well described a characteristic MRI finding of punctate enhancement peppering the pons. In addition, the pons and cerebellar peduncles swelling can occur in this disorder.

Tracheostomy and invasive ventilation in Japanese ALS patients: decision-making and survival analysis: 1990-2010.

OBJECTIVE: To evaluate the factors related to the choice of a tracheostomy and invasive ventilation in amyotrophic lateral sclerosis patients and to determine survival time after a tracheostomy at a single institute in Japan between 1990 and 2010. METHODS: Data for survival time until death or tracheostomy were obtained from 160 patients. Fifty-two patients (33%) underwent tracheostomy/mechanical ventilation. RESULTS: Tracheostomy and invasive ventilation prolonged median survival time (74 months), as did non-invasive ventilation (48 months) when compared to a non-ventilation-supported control group (32 months; p<0.001 each). The ratio of tracheostomy/mechanical ventilation in patients >65 years old significantly increased after 1999 (27%) compared to earlier years (10%, p=0.002). Cox proportional modeling confirmed an age of ≤65 years as advantageous for long-term survival after a tracheostomy. In univariate logistic regression analysis, factors related to the decision to perform a tracheostomy included an age of ≤65 years, greater use of non-invasive ventilation, the presence of a spouse, interval and speed from disease onset to diagnosis/tracheostomy and preservation of motor function. In multivariate logistic regression analysis, age, shorter duration from disease onset until tracheostomy and the presence of a spouse were independently associated with the decision to perform a tracheostomy. Kaplan-Meier plots revealed longer survival times in patients who resided at home after a tracheostomy compared to patients who stayed at a hospital (p=0.007). CONCLUSIONS: Tracheostomy and invasive ventilation are frequently used in Japan. Various factors impact patients' decisions to have these procedures. This study identified factors related to the decision-making process and post-tracheostomy survival.