Adrenoleukodystrophy is an inborn error of metabolism characterized by adrenal insufficiency and progressive demyelmation of brain white matter and peripheral nerves. Authors experienced three cases of adrenoleukodystrophy in a 7 year old boy, a 17 and a 210 year old males that were diagnosed by increased plasma content of very long chain fatty acid(VLCFA). The clinical symptoms include seizure, visual impairment, and hemiparesis. Two cases showed typical radiological findings and sural nerve biopsy was performed in one.
Adrenal Insufficiency ; Adrenoleukodystrophy* ; Biopsy ; Brain ; Child ; Humans ; Male ; Metabolism ; Paresis ; Peripheral Nerves ; Plasma ; Seizures ; Sural Nerve

Pancreatic ductal adenocarcinoma (PDAC) is a highly aggressive lethal malignancy, characterized by late diagnosis, aggressive growth, and therapy resistance, leading to a poor overall prognosis. Emerging evidence shows that the peripheral nerve is an important non-tumor component in the tumor microenvironment that regulates tumor growth and immune escape. The crosstalk between the neuronal system and PDAC has become a hot research topic that may provide novel mechanisms underlying tumor progression and further uncover promising therapeutic targets. In this review, we highlight the mechanisms of perineural invasion and the role of various types of tumor innervation in the progression of PDAC, summarize the potential signaling pathways modulating the neuronal-cancer interaction, and discuss the current and future therapeutic possibilities for this condition.
Humans ; Carcinoma, Pancreatic Ductal/pathology* ; Pancreatic Neoplasms/therapy* ; Signal Transduction ; Peripheral Nerves/metabolism* ; Tumor Microenvironment

OBJECTIVETo investigate the therapeutic effect of nerve growth factor (NGF) on changes of myelin basic protein (MBP) and functional repair of sensory and motor nerve following sciatic nerve injury.

METHODSThe sciatic nerves of rats were injured by sectioning with shaver,and divided into 3 groups: NGF group (Group A), group of normal saline solution (Group B), untreated group (Group C). The time point of observation was at the 4th week after operation. Sensory evoked potential (SEP) and motor evoked potential (MEP) were detected by Model WD-4000 nerve potential working diagnosis system. Immunohistochemical analysis was used for identification of MBP.

RESULTSThe latency of SEP in the Group A at the 4th week after operation was shorter than that in the Group B (P<0.05). The MEP was elicited in 76% of the Group A and was higher than that in the Group B. Results of immunohistochemistry showed that there were less MBP-positive cells in the Group A than in the Group B in one and four weeks respectively.

CONCLUSIONSNGF can improve the conductive function of injured peripheral nerve and facilitate regeneration of nerve.

Animals ; Evoked Potentials ; Female ; Immunohistochemistry ; Myelin Basic Protein ; metabolism ; Nerve Growth Factor ; pharmacology ; Peripheral Nerve Injuries ; Peripheral Nerves ; metabolism ; Rats ; Rats, Wistar ; Sciatic Nerve ; injuries ; metabolism

BACKGROUND: Methcobalamin was known to act as a methyl donor in DNA metabolism and increase Protein synthesis for nerve regeneration. To determine whether methylcobalamin affects capsaicin induced polyneuropathy in rat sural nerves. METHODS: On their second day of life, 18 newborn rats were injected subcutaneously with capsaicin(50mg/kg). Ten rats were treated with methylcobalamin(500microgram/kg) and eight rats with normal saline 5 days a week for 12weeks after capsaicin injection. Using morphometry and computer digitization, the number and sloe distribution of myelinated and unmyelinated fibers were evaluated in sural nerves at 13 weeks. RESULTS: There was significant difference in the number of unmyelinated fibers between two groups(control group 33,321 +/- 15,539/mm2; methylcobalamin group 99,614+/-20,581/mm2. p<0.0001). Methylcobalamin did not change the number and size distribution of'myelinated fibers. The size distribution of'unmyelinated fibers was approximately the same. CONCLUSIONS: This study suggested that methylcobalamln could increase regeneration of peripheral nerve morphometrically. It appears that methylcobalamin might be one of the useful therapeutic agents in various polyneuropathies.
Animals ; Capsaicin* ; DNA ; Humans ; Infant, Newborn ; Metabolism ; Myelin Sheath ; Nerve Regeneration ; Peripheral Nerves* ; Polyneuropathies ; Rats* ; Regeneration ; Sural Nerve ; Tissue Donors

OBJECTIVETo evaluate the different effect on the expression of Calcitonin gene related peptide (CGRP)and neuropeptide Y (NPY) between tissue engineered bone with vascular bundle graft in vivo and that with sensory nerve tract graft in vivo.

METHODThirty-six healthy New Zealand rabbits were divided into 3 groups randomly and equally: vascular bundle group (A), sensory nerve tract group (B), tissue-engineering group (C). Group A segmental bone defect of 1.5 cm long was made at the right femur in each animal. After plate fixation, the defects were implanted respectively with the engineered bone prepared in the above-mentioned 3 methods. At 3, 6 and 12 months post-operatively, the distribution of CGRP and NPY in the new bone were detected by immunohistochemistry and analyzed semi-quantitatively by image analysis software.

RESULTSCGRP and NPY immuno-histochemical results indicated their contents increased significantly in all 3 groups as time passed (P = 0.000). Compared with group B, the contents of CGRP and NPY in group A significantly increased at 3 months (P = 0.000), but there was no statistic difference between them at 6 or 12 months (P > 0.05). The expression of CGRP and NPY in both group A and B were significantly more than that in group C at 3, 6 or 12 months (P = 0.000).

CONCLUSIONImplantation of vascular bundle into tissue-engineered bone can significantly improve the CGRP and NPY contents at early 3 months comparing with Implantation of sensory tract into tissue-engineered bone, but the changes are not significant at 6 or 12 months post-operatively.

Animals ; Blood Vessels ; transplantation ; Bone Substitutes ; Calcitonin Gene-Related Peptide ; metabolism ; Disease Models, Animal ; Femur ; injuries ; Male ; Neuropeptide Y ; metabolism ; Peripheral Nerves ; transplantation ; Rabbits ; Random Allocation ; Tissue Engineering

Diseases of the peripheral nervous system are the most prevalent in patients with end-stage renal disease (ESRD). Although increased blood levels of lead in ESRD have been reported, the role of lead remains to be elucidated. The purpose of this study was to determine the connection of blood lead concentration with peripheral nerve conduction velocity. One hundred ninety-eight healthy subjects (control group) and 68 patients with ESRD undergoing hemodialysis (ESRD group) were enrolled. Nerve conduction was measured within two hours after hemodialysis. Orthodromic sensory nerve action potentials and compound muscle action potentials were recorded on the median, ulnar, and radial nerves. Hemoglobin-corrected blood lead was significantly higher in ESRD patients than in controls (9.1+/-2.8 microgram/dL vs. 5.9+/-2.3 microgram/dL, p<0.001). 32.4% of 68 ESRD patients with diabetes mellitus were significantly related to poorer motor and sensory nerve conduction velocity (p<0.001). However, blood lead was not a significant predictor of the nerve conduction velocity (p>0.05). Our result suggested that even though the blood lead levels were high in ESRD, they were not associated with the decline of peripheral nerve function. Diabetes mellitus is a primary independent risk of neuropathy in ESRD patients.
Peripheral Nervous System Diseases/blood/etiology/physiopathology ; Peripheral Nerves/physiopathology ; Neural Conduction/*physiology ; Middle Aged ; Male ; Lead/*blood/metabolism ; Kidney Failure, Chronic/*blood/complications/*physiopathology ; Humans ; Female ; Diabetic Neuropathies/blood/physiopathology ; Case-Control Studies ; Bone and Bones/metabolism ; Body Burden ; Adult

OBJECTIVETo study the role of CYP2E1 in the protective effects and mechanism of garlic oil (GO) on the peripheral nerve injuries induced by n-hexane.

METHODSFifty male Wistar rats were randomly divided into five groups (n = 10): the control, the GO (80 mg/kg) control, the n-hexane (2000 mg/kg) model, the low dose GO (40 mg/kg) plus n-hexane, and the high dose GO (80 mg/kg) plus n-hexane groups. All rats were treated by intragastric administration 6 times a week for 10 weeks. The gait scores were determined every two weeks for monitoring the peripheral neurotrosis. All rats were sacrificed in 10 weeks, the activities and expression levels of hepatic CYP2E1 and 2, 5-HD in serum were examined.

RESULTSAs compared with control group, the content and activity of hepatic CYP2E1 in GO control group reduced by 83.1% and 48.3% respectively (P < 0.01), the content and activity of hepatic CYP2E1 in model group increased by 112.5% and 72.2% respectively (P < 0.01). As compared with model group, the contents of hepatic CYP2E1 in low dose and high dose GO groups reduced by 32.9% and 39.1% respectively, the activities of hepatic CYP2E1 in low dose and high dose GO groups reduced by 27.4% and 44.5% respectively (P < 0.01); the contents of serum 2,5-HD in low dose and high dose GO groups reduced by 47.7% and 78.7% respectively (P < 0.01). The gait scores in model, low dose and high dose GO groups were significantly lower than that in control group, but the gait scores in low dose and high dose GO groups were significantly lower than that in model group (P < 0.05).

CONCLUSIONGarlic oil can effectively reduce the peripheral neurotrosis induced by n-hexane due to the decreased content and activity of hepatic CYP2E1, resulting in the reduced formation of 2, 5-HD from n-hexane.

Animals ; Cytochrome P-450 CYP2E1 ; metabolism ; Garlic ; Hexanes ; toxicity ; Liver ; drug effects ; enzymology ; Male ; Peripheral Nerves ; drug effects ; pathology ; Plant Oils ; pharmacology ; Rats ; Rats, Wistar

OBJECTIVETo investigate an effective treatment of peripheral nerve injuries by means of gene transference.

METHODS48 adult Wister rats were divided evenly into 3 groups. A 10 mm sciatic nerve gap was created and bridged with a silicone chamber. The silicone chamber was filled with glial cell-line derived neurotrophic factor(GDNF) gene modified Schwann cells(SCs) (group 1), the normal SCs(group 2) and nothing(the control). At 4, 8, 12, and 16 weeks after the operation, the general and histological observations, the electromyographic and immunohistochemical examinations were performed to the regenerated nerves.

RESULTSThe GDNF-SCs group was significantly better than the SCs and the control groups in nerve conduction velocity, the number and density of reinnervation, the area of regenerated nerve and the thickness of myelin sheath of the regenerated nerves.

CONCLUSIONGDNF gene modified SCs secrete higher levels of neurotrophic factors for a prolonged time, which are more effective in peripheral nerve repair than the normal SCs.

Animals ; Female ; Glial Cell Line-Derived Neurotrophic Factor ; Immunohistochemistry ; Male ; Nerve Growth Factors ; analysis ; genetics ; physiology ; Nerve Regeneration ; physiology ; Peripheral Nerves ; chemistry ; physiopathology ; Peripheral Nervous System Diseases ; surgery ; Rats ; Rats, Wistar ; Schwann Cells ; metabolism ; transplantation

OBJECTIVETo investigate whether cannabinoid receptor 1 (CB1R) is involved in nerve growth in endometriosis-associated ectopic cyst.

METHODSThe effect of CB1R agonist and antagonist on the expression of pan-neuronal marker protein gene product (PGP) 9.5 in ectopic cyst was examined by immunofluorescence and Western blot in endometriosis model of 18 rats.

RESULTSImmunofluorescence revealed that PGP 9.5 was expressed in the nerve fibers and was mainly distributed in the cyst hilum. Western blot revealed that the protein density of either PGP 9.5 (2 week: 0.38 ± 0.05; 4 week: 0.63 ± 0.03; 8 week: 0.80 ± 0.07, P < 0.01) or CB1R (2 week: 0.48 ± 0.04; 4 week: 0.68 ± 0.01; 8 week: 0.80 ± 0.03, P < 0.01) in the ectopic cyst increased with cyst size. In addition, compared to control group (0.75 ± 0.01), PGP 9.5 expression in the ectopic cyst was promoted by CB1R agonist ACPA (0.81 ± 0.01, P < 0.05), and inhibited by CB1R antagonist AM251 (0.67 ± 0.03, P < 0.01).

CONCLUSIONSCB1R was involved in the nerve growth of ectopic cyst associated with endometriosis.

Animals ; Blotting, Western ; Cysts ; metabolism ; Disease Models, Animal ; Endometriosis ; metabolism ; Female ; Peripheral Nerves ; growth & development ; metabolism ; Piperidines ; pharmacology ; Pyrazoles ; pharmacology ; Rats ; Receptor, Cannabinoid, CB1 ; antagonists & inhibitors ; physiology ; Ubiquitin Thiolesterase ; metabolism

OBJECTIVETo investigate the association of nerve growth factor (NGF) expression with perineural invasion and pain in pancreatic cancer.

METHODSNGF expression was detected by Northern blotting and immunohistochemistry in 28 pancreatic cancer and 20 normal pancreatic tissue samples. Correlation analysis of the results with the extent of perineural invasion, pain and histopathologic characteristics of the tumor was performed.

RESULTSNorthern blot analysis revealed that NGF levels in pancreatic cancer tissues increased by 3.1 folds in comparison with normal pancreas tissue (P<0.05), and immunohistochemistry detected the presence of obvious NGF expression in the cytoplasm of pancreatic cancer cells. Tumors with high NGF expression were associated with more frequent perineural invasion (P<0.01), and increased NGF expression was related to more intense pain (P<0.01).

CONCLUSIONIncreased NGF expression may contribute to perineural invasion and pain in pancreatic cancer.

Adult ; Aged ; Blotting, Northern ; Carcinoma, Pancreatic Ductal ; genetics ; metabolism ; pathology ; Female ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Neoplasm Invasiveness ; Nerve Growth Factor ; biosynthesis ; genetics ; Neuralgia ; genetics ; metabolism ; pathology ; Pancreatic Neoplasms ; genetics ; metabolism ; pathology ; Peripheral Nerves ; metabolism ; pathology ; RNA, Messenger ; biosynthesis ; genetics