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Forced vital capacity (FVC) is recommended as a respiratory function test in patients with amyotrophic lateral sclerosis (ALS). However, in ALS associated with orofacial palsy, FVC may be an unreliable test. Slow vital capacity (SVC) is an easier and more reliable test even in cases with bulbar symptoms. However, it remains unclear whether respiratory function tests using SVC and FVC are associated with prognosis after percutaneous endoscopic gastrostomy (PEG) placement. This study aimed to confirm whether both SVC and FVC are related to prognosis after PEG placement in patients with ALS.We conducted this retrospective observational cohort study of 69 consecutive patients diagnosed with sporadic ALS who underwent PEG placement between July 2007 and February 2020. We analyzed the association with mortality 6 months after PEG placement and evaluated long-term prognosis.Forty-four patients met the inclusion criteria. In cases with decreased SVC (p .01) and FVC (p .01), a significant difference was observed in mortality 6 months after PEG placement, with an optimal cut-off of SVC ≤57.4% (sensitivity, 0.828; specificity, 0.867) and FVC ≤57.3% (sensitivity, 0.828; specificity, 0.867). Multivariate analysis showed that onset age ≥ 65 years (p .05), SVC ≤57.4% (p .01), and FVC ≤57.3% (p .01) were associated with survival after PEG placement.SVC, like FVC, is an important prognostic factor after PEG placement in patients with ALS, and there is a possibility that evaluation using SVC can complement respiratory function testing even in cases where the evaluation of FVC is limited.

Mild palmar digital neuropathy may be underestimated because selective nerve conduction studies (NCS) of the palmar digital nerve are challenging. We herein report two cases of palmar digital neuropathy. We performed sensory NCS in each finger using the standard approach. Both cases showed a decrease in the amplitude of sensory nerve action potential (SNAP) in the localized finger. Furthermore, the sensory nerve inching test identified the lesion site. When performing NCS in patients with finger sensory impairment, we recommend recording the SNAP in each finger using standard NCS at the wrist, as well as sensory nerve inching testing.

Non-motor symptoms (NMSs) negatively impact the health-related quality of life (HrQOL) of patients with Parkinson’s disease (PD). The Movement Disorder Society-Sponsored Revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) is a comprehensive scale for evaluating PD. It remains unclear whether the NMSs evaluated with MDS-UPDRS are predictive of HrQOL. This study aimed to investigate whether NMSs, as evaluated with the MDS-UPDRS, could predict the HrQOL of patients with PD. We conducted a 2-year retrospective observational cohort study assessing 108 patients with PD who were recruited from a single tertiary center between January 2015 and December 2017. MDS-UPDRS was used to assess NMSs and motor symptoms and Parkinson’s Disease Questionnaire-39 (PDQ-39) to measure patients’ HrQOL. The median age of patients was 69 years, and 65.7% were female. The median MDS-UPDRS part I, part II, part III, and PDQ-39-summary index scores were 8, 10, 22, and 25, respectively. The final stepwise multiple linear regression model showed that female sex (standard partial regression coefficient β = 0.131, P

Biomarkers of tumour response to radiotherapy may help optimise cancer treatment. The aim of the present study was to identify changes in extracellular microRNAs (miRNAs) as a biomarker of radiation-induced damage to human colorectal cancer cells. HCT116 cells were exposed to increasing doses of X-rays, and extracellular miRNAs were analysed by microarray. The results were correlated with the frequency of micronuclei. A total of 59 miRNAs with a positive correlation and 4 with a negative correlation between dose (up to 6 Gy) and extracellular miRNA expression were identified. In addition, for doses between 0 and 10 Gy, 12 miRNAs among those 59 miRNAs with a positive correlation were identified; for these extracellular miRNAs, a significantly positive correlation was observed between their expression and the frequency of micronuclei for doses up to 10 Gy. These results suggest that specific miRNAs may be considered as cell damage markers and may serve as secreted radiotherapy response biomarkers for colorectal cancer; however, the results must be further validated in serum samples collected from patients undergoing radiotherapy.

A 72-year-old man presented with continuous hyperCKemia and intermittent claudication. He exhibited no calf muscle hypertrophy at that time or afterward. Other than an increased creatine kinase (CK) level (1,525 U/l), none of the laboratory tests was abnormal, including that for myositis-related autoantibodies. Electromyography showed neurogenic changes in the left gastrocnemius. Lumbar magnetic resonance imaging revealed spinal canal stenosis (L3/4, L4/5), left L4 radiculopathy, and bilateral S1 radiculopathy. T2-weighted and short tau inversion recovery images showed high signal intensity in the bilateral biceps femoris and gastrocnemius. Histopathological evaluation of a specimen obtained from the right gastrocnemius muscle revealed neurogenic changes. The patient was diagnosed with S1 radiculopathy caused by lumbar spinal canal stenosis with hyperCKemia. Although S1 radiculopathy with hyperCKemia is usually associated with calf muscle hypertrophy, we should consider S1 radiculopathy in patients with intermittent claudication and hyperCKemia even in the absence of calf muscle hypertrophy.

Background Although several risk scoring systems that predict postoperative complication incidence are available, the optimal scoring tool for elderly colorectal cancer patients remains unknown. Material and Methods Records of 659 patients underwent surgery for colorectal cancer were retrospectively reviewed, and 130 patients aged >80 years were divided into two groups according to postoperative complications (Clavien-Dindo classification ≥ grade II) as Complication group and Non-complication group.Scoring systems such as the Surgical Apgar score (SAS), and the Prognostic nutritional index (PNI), and sections of the Physiological and operative severity score for the enumeration of mortality and morbidity (POSSUM; physiological score (PS-P), and operative severity score (OS-P)), the Colorectal POSSUM (CR-POSSUM; physiological score (PS-CP) and operative severity score (OS-CP)), and Estimating the physiologic ability and surgical stress score (E-PASS; preoperative risk score (PRS), surgical stress score (SSS) and comprehensive risk score (CRS)) were analyzed. Results The PS-P, PRS, and CRS were significantly different between the two groups in univariate analysis. Area under the Receiver Operating Characteristic Curve of PRS was the highest among the scoring systems. Multivariate analysis also showed PRS was a useful risk scoring tool. Conclusions PRS may be useful for predicting the occurrence of complications for colorectal cancer in elderly patients.

Autoantibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been detected in inflammatory demyelinating central nervous system diseases. A 30-year-old woman had blurred vision, marked optic nerve disc swelling, serous retinal detachment at the macular on optic coherence tomography, and MOG-IgG seropositivity. The patient was thought to have optic papillitis associated with MOG-IgG. Her symptoms rapidly improved after high-dose methylprednisolone therapy. We hypothesize that serous retinal detachment was secondary, arising from optic papillitis. This is the first report of the concurrence of optic papillitis with MOG-IgG and serous retinal detachment. MOG-IgG should be tested in patients with marked optic disc swelling.

Background Paraneoplastic neurological syndromes (PNS) are rare disorders associated with cancer and are believed to be immune mediated. Patients with autonomic PNS suffer from variable combinations of parasympathetic and sympathetic failure. Autonomic PNS are usually associated with other PNS, such as encephalomyelitis and sensory neuropathy; however, autonomic symptoms may rarely manifest as PNS symptoms. Autonomic symptoms, therefore, may be overlooked in patients with cancer. Case presentation We described a 65-year-old Japanese man who was diagnosed with autonomic PNS due to small-cell lung carcinoma (SCLC) with Eastern Cooperative Oncology Group (ECOG) performance status 3, who suffered from orthostatic hypotension, and urinary retention needing a urethral balloon. Laboratory studies showed decreased levels of noradrenaline, and were positive for anti-ganglionic acetylcholine receptor antibody, type 1 antineuronal nuclear antibody, and sry-like high mobility group box 1 antibody. Nerve conduction evaluations and 123I-metaiodobenzylguanidine myocardial scintigraphy showed no abnormalities. Abdominal contrast-enhanced computed tomography revealed marked colonic distention. The patient’s autonomic symptoms resolved following integrated treatment (symptomatic treatment, immunotherapy, and additional chemotherapy) enabling the patient to walk, remove the urethral balloon, and endure further chemotherapy. ECOG performance status remained at 1, 10 months after admission. Conclusions Integrated treatment for autonomic PNS may improve autonomic symptoms and ECOG performance status of patients with cancer.

Continuous intrajejunal infusion of levodopa-carbidopa intestinal gel (LCIG) is an established device-aided therapy for advanced Parkinson's disease (PD). Phytobezoar associated with LCIG is a rare device-related complication and presents with exacerbations of gastrointestinal and PD symptoms. We herein report the case of a phytobezoar that was formed at a knot on the pigtail-shaped J-tube and developed only in association with postprandial abdominal pain, similar to a feeling of a tube being pulled in without an exacerbation of PD symptoms. Such abdominal pain may be a warning sign of phytobezoar in LCIG-treated patients. Despite device-related complications, high-pressure alarms are not always present, and PD symptoms are not always exacerbated.

Background The risk of venous thromboembolism (VTE) after surgery for malignancy in Japanese patients is unclear; hence, standard prevention protocols have not been established, especially for minimally invasive surgeries. We aimed to investigate the additional effect of low-molecular-weight heparin (LMWH) on the prevention of VTE after laparoscopic surgery for gastrointestinal malignancy. Study Design From February 2013 to January 2017, Four hundred patients scheduled for laparoscopic surgery were included. Cases were randomly allocated to the physical therapy group (Control group; 201 patients) or to the combination-therapy group (LMWH group; 199 patients) in which enoxaparin sodium (20 mg twice a day) was administered for 1 week postoperatively in addition to the physical therapy. A diagnosis of VTE was made by contrast-enhanced computed tomography or ultrasonography when symptomatic or D-dimer was ≥10 μg/ml. Results VTE was observed in 1.2% and 4.0% patients of the LMWH and Control groups, respectively (odds ratio [OR]=0.3, 95% confidence interval [CI]: 0.03–1.53). Pulmonary embolism was confirmed only in the Control group (1.7%). No major bleeding occurred in either group. Logistic multiple regression analysis revealed that surgical time extension (OR=1.02, 95%CI: 1.00–1.04) was a risk factor of VTE, while administration of LMWH (OR=0.21, 95%CI: 0.03–0.99), male sex (OR=0.12, 95%CI: 0.01–0.60), and early cancer (OR=0.17, 95%CI: 0.02–0.82) reduced the risk of VTE. Conclusions Postoperative LMWH administration is safe. The additional effect of the LMWH administration to the physical therapy was not statistically proved in this study. However, it could be useful for the patients with risk factors such as female, long operation time, and higher cancer stage.

Background Hyperthermia in patients with acute ischemic stroke is associated with poor outcome. Although previous studies have shown a negative effect on functional outcome, even in patients treated with intravenous recombinant tissue plasminogen activator (rt-PA), the effect on survival remains unclear. Aims of the study The aim of this study was to evaluate the association between the functional and survival prognosis and hyperthermia in patients with acute ischemic stroke treated with rt-PA. Methods We studied 120 patients treated with rt-PA from 2306 consecutive Japanese patients with acute cerebral infarction at Aomori Prefectural Central Hospital between December 2009 and March 2017. We defined hyperthermia as ≥38°C within 72 hours after rt-PA administration. Propensity score matching was used to compare 34 non-hyperthermia and hyperthermia patient pairs. Results Final modified Rankin Scale scores were higher in the hyperthermia group than in the non-hyperthermia group. In addition, the Kaplan-Meier model showed that the non-hyperthermia group had significantly better survival rates than the hyperthermia group (hazard ratio, 5.3; 95% confidence intervals, 1.2-24.8). Conclusions Hyperthermia within 3 days after rt-PA is associated with poor functional prognosis and survival outcome in patients with acute cerebral infarction.

Miller Fisher syndrome (MFS) can be difficult to diagnose, particularly in mild cases where some of the standard triad of symptoms (external ophthalmoplegia, ataxia, and loss of deep tendon reflex) are absent. We herein report a case of the incomplete form of MFS diagnosed in a 54-year-old Japanese man who presented only with ataxia symptoms and was positive for the anti-GQ1b antibody. However, the patient also suffered from dysgeusia, a significant impairment of taste perception. We propose that dysgeusia in acute-onset ataxia cases may constitute an important clinical feature to aid in the diagnosis of the incomplete form of MFS.

An 83-year-old Japanese man was admitted with dysarthria and right hemiparesis. He had had a large intracranial aneurysm on the left internal carotid artery 5 years before admission and had been followed up under conservative treatment. On admission, diffusion-weighted imaging revealed a hyperintense signal on the left anterior choroidal artery territory. Time-of-flight magnetic resonance angiography demonstrated poor visibility of the middle and anterior cerebral arteries and the inferior giant aneurysm, suggesting distal emboli from aneurysm thrombosis or a reduction of blood outflow due to aneurysm thrombosis. Arterial spin labeling (ASL) signal increased in the giant aneurysm, suggesting blood stagnation within the aneurysmal sac, and decreased in the left hemisphere. We diagnosed cerebral infarction due to aneurysm thrombosis, and started antithrombotic therapy. On day 2, he suddenly died of subarachnoid hemorrhage due to rupturing of the giant aneurysm. When thrombosis occurs in a giant aneurysm, increasing ASL signal within the aneurysm and decreasing ASL signal with poor visibility on magnetic resonance angiography in the same arterial territory may indicate the danger of impending rupture of the giant aneurysm.

The mechanisms of dopamine dysregulation syndrome (DDS) in Parkinson's disease (PD) remain unclear, although it is known that the nucleus accumbens (NAc) plays a role in its development. Based on the hypothesis that DDS and levodopa-induced dyskinesia (LID) share a pathophysiological basis, we investigated dendritic spine morphology of medium spiny neurons (MSNs) in the NAc of a rat model of LID, because spine enlargement in MSNs of the caudate/putamen has been proposed to be a morphological hallmark of LID. Spines of NAc MSNs also became enlarged in the LID model. This result suggests that excitatory supersensitivity of MSNs in the NAc is involved in the development of DDS, similar to what occurs in the caudate/putamen in LID.

• Serotonin and norepinephrine transporters are capable of dopamine reuptake in the parkinsonian striatum.

Long-term administration of levodopa for Parkinson's disease is associated with various motor and non-motor complications. We examined the dendritic spine morphology of pyramidal tract-type neurons in the prefrontal cortex in a rat model of Parkinson's disease chronically treated with levodopa. Dendritic spines showed decreased density and increased average volume after dopamine denervation and levodopa treatment. These morphologic alterations suggest that the prefrontal neurons may maladaptively respond to excitatory input, which might be one of the mechanisms underlying various levodopa-induced complications in patients with Parkinson's disease.

Unilateral oculomotor nerve palsy can result from various neurological disorders. We herein report the case of a 68-year-old man with complete unilateral oculomotor nerve palsy following campylobacter infection. Based on the antecedent infection and the patient's decreased tendon reflexes, incomplete Miller Fisher syndrome (MFS) without ataxia was suspected. His serum tested positive for anti-GQ1b antibodies. He recovered over a period of 87 days without immunotherapy. We conclude that incomplete MFS following campylobacter infection can cause unilateral oculomotor nerve palsy without ataxia. Mild MFS should be considered in patients presenting with unilateral isolated ophthalmoplegia and decreased tendon reflexes.

A 41-year-old Korean man was admitted to our hospital with a 1-day history of headache and abnormal left visual field. Neurological examination revealed left homonymous hemianopia and difficulty in fine movement of the left finger. Laboratory studies indicated hyperlipidemia and hyperhomocysteinemia. Diffusion-weighted imaging showed a hyperintense signal on the right posterior cerebral-middle cerebral artery watershed area. Computed tomography angiography and carotid angiography revealed the bottle neck sign on the right internal carotid artery (ICA) with occlusion of the distal right ICA, but not the left ICA. Carotid angiography showed moyamoya vessels as well as occlusion of the distal right ICA. The right carotid canal was hypoplasic. We diagnosed the patient with unilateral moyamoya disease and carotid canal hypoplasia. This case demonstrates that unilateral carotid canal hypoplasia may co-exist with ipsilateral moyamoya disease. A unilateral bottle neck sign may indicate the presence of carotid canal hypoplasia. Evaluation of the bony carotid canal may be useful when it is necessary to distinguish between secondary vascular stenosis due to arteriosclerosis and MMD. Keywords: Moyamoya disease, Carotid canal, Bottle neck sign

Objectives: Physician-staffed helicopter transport is faster than ground transport and allows for prompt medical care of patients in rural areas. In this study, we evaluated the relationship between helicopter transport and the prognosis of patients with acute cerebral infarction in rural Japan. Methods: This retrospective, observational study included 546 patients with acute cerebral infarction attending Aomori Prefectural Central Hospital, which serves a rural region of Japan. Patients were separated into 2 transport groups: physician-staffed helicopter emergency medical services and ground emergency medical services. Patients were assessed for stroke severity, treatment, and prognosis. Results: Of the 546 patients, 11.2% were transported by physician-staffed helicopter emergency medical services and 88.8% by ground emergency medical services. Although the distance transported was significantly longer in the physician-staffed helicopter emergency medical services group, the time from onset to reaching our hospital was similar between the groups. National Institutes of Health Stroke Scale on admission and final prognosis were significantly worse with physician-staffed helicopter emergency medical services than with ground emergency medical services. Multivariate analysis showed no association between transport system and prognosis. Conclusions: In this study, patients transported by physician-staffed helicopter emergency medical services had more severe stroke symptoms and poorer functional outcomes than those transported by ground emergency medical services. However, the transport time was shorter for physician-staffed helicopter emergency medical services; thus, physician-staffed helicopter emergency medical services may be useful for reducing transport time for patients in rural Japan.

Subacute sensory ataxic neuronopathy is a well-known form of paraneoplastic syndrome. Most sensory neuronopathies are associated with small cell lung cancer and anti-Hu antibodies, and usually show only slight improvement with immunotherapy. To date, there have been few reports of neuropathy associated with thymoma and no treatment strategy has been established for thymoma-related neuropathy. Here, we provide the first report of a case of sensory ataxic neuronopathy with thymoma that showed marked improvement after steroid therapy, even though preceding intravenous immunoglobulin treatments and tumor resection were less effective. A 57-year-old Japanese man was referred to our hospital with a 6-week history of distal paresthesia in his four limbs and an unsteady gait. He presented with left-dominant ataxia in his four limbs due to reduced sensation in his extremities. He also complained of constipation, difficulty urinating, and erectile dysfunction. Upon investigation, including electrodiagnostic studies, the patient was diagnosed as having sensory ataxic neuronopathy with invasive thymoma. A first round of intravenous immunoglobulin therapy, a following thymectomy, and a second round of intravenous immunoglobulin therapy after the surgery were not effective in treating his neurological symptoms. Subsequently, oral steroid therapy was started, which brought about a remarkable improvement; 6 weeks after the beginning of the steroid therapy, his neurological symptoms were resolved, except for slight distal paresthesia in his feet. Although rarely reported, thymoma can underlie sensory neuronopathy, and the response of thymoma-associated sensory neuronopathy to immunotherapy might be better than that of anti-Hu antibody-related neuropathies. Even if the first immunotherapy is not effective in treating neuropathy with thymoma, further immunomodulatory treatment should be tried after treating the tumor.

A 66-year-old man presented with a 6-day history of progressive posterior cervical pain that included lower back pain and fever. Neurological examinations revealed neck stiffness. Computed tomography demonstrated convexity subarachnoid hemorrhage. A spinal T2-weighted image revealed a hypointense signal lesion with contrast enhancement of the intradural extramedullary space at Th12–L1. Digital subtraction angiography showed a fusiform aneurysm with a 10 mm diameter in the artery of Adamkiewicz. We diagnosed the patient's condition as a ruptured aneurysm of the artery of Adamkiewicz. By day 41 the aneurysm had disappeared following conservative treatment. Aneurysms arising from the artery of Adamkiewicz are extremely rare and can cause both convexity and spinal subarachnoid hemorrhages. Clinicians should therefore look for spinal lesions if patients with convexity subarachnoid hemorrhage of an unknown origin have lower back pain as their initial symptom.