Muscle spasm induced sympathetic reflex bursts on microneurography in a case with pontine demyelination

Microneurography was performed in a 39-year-old woman with demyelination of the pontine white matter associated with muscle spasms in the lower extremities. Single bursts on the microneurogram were observed immediately after cessation of the spasm with no systemic changes in the blood pressure or heart rate. Voluntary tonic flexion of the lower extremities induced similar bursts with small amplitudes. These reflex bursts possessed a characteristic of muscle sympathetic nerve activity, because the latency between the peak of each burst and the prior R-wave on the electrocardiograph was constant. The occurrence of these bursts suggests that a segmental compensatory mechanism in the spinal cord may stabilize the muscle blood flow influenced by muscle contraction.     

Expression of calmin,a novel developmentally regulated brain protein with calponin-homology domains

We examined the expression in the mouse brain of a recently isolated protein named calmin that has two calponin-homology domains in tandem at the N-terminus and a transmembrane domain at the C-terminus. Calmin mRNA and protein were detected in neurons of the hippocampus, cerebral cortex, and thalamus, Purkinje cells, and also in the choroid plexus and ependymal cells. The protein is present predominantly in dendrites and cell bodies of the neurons, but not in axons. Furthermore, the amounts of calmin mRNA and protein increase during the period of maturation of the mouse brain after birth, in a manner similar to that of PSD95 and synaptophysin. These results indicate that calmin may be involved in the development and/or maintenance of neuronal functions.     

Effect of the free radical scavenger edaravone on peripheral nerve ischemia–reperfusion injury

We investigated the effects of edaravone, a free radical scavenger, on peripheral nerve ischemia–reperfusion injury caused by ligation of vessels supplying the sciatic and tibial nerves in rats. The control group was administered a placebo, the standard‐dose group was given 3 mg/kg of edaravone intraperitoneally every 24 hours, and the low‐dose group was given 1 mg/kg of edaravone. At 7 days after reperfusion, neurological and electrophysiological parameters were improved in the standard‐dose group as compared with the control group. After 14 days, however, these differences were no longer observed. After 21 days, persistent edema and nerve fiber degeneration were noted in the standard‐dose group, but not in the control or low‐dose groups. Edaravone was effective during the early reperfusion period, but chronic inhibition of reactive oxygen species may be detrimental for nerve regeneration after ischemia–reperfusion injury. Further studies are necessary to confirm the long‐term influence of edaravone. Muscle Nerve, 2009     

Analysis of the relationship between muscle sympathetic nerve activity and cardiac 123I-metaiodobenzylguanidine uptake in patients with Parkinson's disease.

To analyze the correlation between muscle sympathetic nerve activity (MSNA) and cardiac (123)I-metaiodobenzylguanidine (MIBG) uptake in patients with Parkinson's disease (PD), we measured both parameters in 14 PD patients who were 51 to 82 years of age (mean, 63.1 +/- 8.7 years). The duration of PD was 2 to 26 years, and the disability level (modified Hoehn and Yahr stage) ranged from 2.0 to 4.0 (mean, 3.2 +/- 0.5). MSNA was recorded from the peroneal nerve fascicles using microneurographic methods, and then cardiac MIBG scintigraphy was performed within 1 month. We analyzed the correlation between the standardized MSNA, expressed as a percentage of the predicted value based on control subject data, and the heart-to-mediastinum ratio (H/M) or washout ratio (WR) from early and delayed MIBG images. The relationships between disease duration or disability and MSNA, the H/M ratio, or the WR were also analyzed. No significant correlations were found between MSNA and H/M ratio or WR. Although MSNA was inversely correlated with disease duration and with disability level, neither the H/M ratio nor the WR showed a significant correlation with disease duration or disability level. Because MSNA and MIBG abnormalities were not related, functional changes in addition to organic changes in cardiac sympathetic nerve endings may result in abnormal uptake of MIBG in Parkinson's disease. .     

The follow up study of intra-arterial infusion chemotherapy with local vein blocking as a surgical neo-adjuvant treatment for locally advanced breast cancer

Preoperative intra-arterial infusion neo-adjuvant chemotherapy, in combination with local vein blocking, was administered to thirtyone patients with locally advanced stage III breast cancer. The anti-cancer drugs and dosages used were 500 mg of 5-Fluorouracil (5FU), which was infused daily for 7–14 days, and 20 mg of Adriamycin (ADM), which was administered as a bolus dose twice into the subclavian and internal mammary arteries. The response rate of this method on the primary tumor was 48.4 per cent, and, histologically it was found to be as high as 90.3 per cent. The response rate of the clinical effects on the regional lymph nodes was 50.0 per cent, however, histologically, it was found to be lower than that of the primary tumor. In the long-term follow up study the 5-year survival rate was 72.2 per cent. Thus, this method seems to be effective as a combined modality in cases of locally advanced stage III breast cancer.     

The positivity of specific IgE to salmon roe and cod roe in outpatients

PURPOSE: To clarify the positivity of specific IgE to salmon and cod roe in outpatients. METHODS: Specific IgE were assayed using CAP RAST system in 91 pediatric outpatients. They were 47 males and 44 females, including 22 allergic, 29 infectious, 10 neurological, 8 gastrointestinal, 7 urological, 6 hematologic, 3 metabolic disease and 6 other disorders. For control, 30 sera from healthy normal adult volunteers were assayed. Additionally, sera from 653 allergic patients were also collected in our laboratory. Specific IgEs against salmon and cod meat were also assayed simultaneously. RESULTS: In 91 pediatric patients, two children were salmon roe positive and one child was cod roe positive. Three children scored class 1, borderline positive in salmon roe, and one child scored class 1 in cod roe. Other children were negative in all allergens. No positive sera were found in normal adult volunteers. Among 653 specimens in our laboratory, the positivity of specific IgE to salmon and cod roe were 25%, and 9%, respectively. Infants younger than two years old had higher ratio than older children. There was a significant correlation (r = 0.676) between the titers of IgE to salmon and cod roe. On the other hand, the titers of IgE to their meats correlated less than those to their roes. Our results support previous reports that fish roe from different species have common antigen apart from those of fish meat. CONCLUSION: Positive ratio of salmon and cod roe specific IgE were 2.2 and 1.1% in pediatric outpatients.     

Advanced Glycation End Products in Alzheimer’s Disease and Other Neurodegenerative Diseases

Advanced glycation end products (AGEs) have been implicated in the chronic complications of diabetes mellitus and have been reported to play an important role in the pathogenesis of Alzheimer’s disease. In this study, we examined the immunohistochemical localization of AGEs, amyloid β protein (Aβ), apolipoprotein E (ApoE), and tau protein in senile plaques, neurofibrillary tangles (NFTs), and cerebral amyloid angiopathy (CAA) in Alzheimer’s disease and other neurodegenerative diseases (progressive supranuclear palsy, Pick’s disease, and Guamanian amyotrophic lateral sclerosis/Parkinsonism-dementia complex). In most senile plaques (including diffuse plaques) and CAA from Alzheimer’s brains, AGE and ApoE were observed together. However, approximately 5% of plaques were AGE positive but Aβ negative, and the vessels without CAA often showed AGE immunoreactivity. In Alzheimer’s disease, AGEs were mainly present in intracellular NFTs, whereas ApoE was mainly present in extracellular NFTs. Pick’s bodies in Pick’s disease and granulovacuolar degeneration in various neurodegenerative diseases were also AGE positive. In non-Alzheimer neurodegenerative diseases, senile plaques and NFTs showed similar findings to those in Alzheimer’s disease. These results suggest that AGE may contribute to eventual neuronal dysfunction and death as an important factor in the progression of various neurodegenerative diseases, including Alzheimer’s disease.     

Clinical and histopathological features of progressive-type familial amyloidotic polyneuropathy with TTR Lys54

The purpose of this study was to evaluate the clinical and pathological features in patients with progressive-type familial amyloidotic polyneuropathy (FAP) using autopsy and biopsy specimens. A proband is a 33-year-old man with FAP type I who developed motor, sensory and autonomic impairments with neuropathy, heart failure, and anorexia. Genetic findings of transthyretin (TTR) revealed G to A transition in codon 54 causing a rare mutation of TTR Lys54. He died of pneumonia and severe cardiac failure 4 years after onset. Autopsy showed heavy amyloid deposition in the heart, peripheral nerves, thyroid, skin, fat tissue, prostate and testis, moderate in the sympathetic nerve trunk, vagal nerve, celiac plexus, pelvic plexus, bladder, gastrointestinal tract, tongue, pancreas, lung, pituitary, blood vessel, gall bladder, adrenals and muscles, and free in the central nervous system, liver, kidney and spleen. Sural nerve biopsy in a sibling confirmed TTR amyloidosis immunohistochemically. Electronmicroscopic findings of amyloid fibrils were similar to that of FAP Met30. Immunoelectronmicroscopic findings indicated the relationship between amyloid fibrils or non-fibrillar structure and collagen fibers. The distribution of amyloid deposition, heavy in the heart and lacking in the kidney, is a characteristic feature and reflected severity of FAP with TTR Lys54.