The purpose of this study was to study the role of neurofilament (NF) mRNA and calpain in NF reduction of acrylamide (ACR) neuropathy. Male Wistar adult rats were injected i.p. every other day with ACR (20 mg/kg·bW or 40 mg/kg·bW) for 8 weeks. NF mRNA expression was detected using RT-PCR and the calpain concentration was determined using western blot analysis. The NF mRNA expression significantly decreased while the level of m-calpain and μ-calpain significantly increased in two ACR-treated rats groups regardless of the ACR dose. The light NF (NF-L) protein expression was significantly correlated with NF-L mRNA expression. Combined with previous data, the concentrations of three NF subunits were negatively correlated with the calpain levels. These findings suggest that NF-L mRNA and calpain mediated the reduction in NF of ACR neuropathy.
Acrylamide ; toxicity ; Animals ; Calpain ; metabolism ; Gene Expression Regulation ; drug effects ; Intermediate Filaments ; genetics ; Male ; Peripheral Nervous System Diseases ; chemically induced ; genetics ; metabolism ; RNA, Messenger ; genetics ; metabolism ; Rats

Mitochondria contain respiratory chain enzyme complexes that carry out oxidative phosphorylation and produce a main part of cellular energy in the form of ATP. Mitochondrial disorders occur either due to sporadic or inherited mutations of the genes located in the nuclear or mitochondrial DNA or due to other exogenous factors. Although several proteins related with signalling, assembling, transporting, and enzymatic functions can be impaired in mitochondrial disorders, most frequently the activity of the respiratory chain protein complexes is primarily or secondarily affected, leading to impaired oxygen utilization and reduced energy production. Mitochondrial disorders usually show a chronic and slowly progressive course and present with multiorgan involvement with varying onsets between birth and late adulthood. They represent a diagnostic challenge because of their wide variations in the presentation and the course. The systems frequently affected in mitochondrial disorders are the peripheral nervous system, brain, endocrine system, heart, eyes, ears, guts, kidneys and bone marrow. Although there is actually no specific therapy and cure for mitochondrial disorders, the rapidly increasing understanding of the pathophysiological background of mitochondrial disorders may further facilitate the diagnostic approach and open perspectives to the future and possibly causative therapies.
Adenosine Triphosphate ; Bone Marrow ; Brain ; DNA, Mitochondrial ; Ear ; Electron Transport ; Endocrine System ; Energy Metabolism ; Heart ; Kidney ; Mitochondria ; Mitochondrial Diseases* ; Oxidative Phosphorylation ; Oxygen ; Parturition ; Peripheral Nervous System

Mitochondria contain the respiratory chain enzyme complexes that carry out oxidative phosphorylation and produce the majority of cellular energy in the form of ATP. Mitochondrial disorders are either due to sporadic or inherited mutations of genes located in nuclear or mitochondrial DNA or due to other exogenous factors. Although several proteins related with signalling, assembling, transporting, and enzymatic function can be impaired in mitochondrial disorders, most frequently however, the activity of the respiratory chain protein complexes is primarily or secondarily affected, leading to impaired oxygen utilization and reduced energy production. Mitochondrial disorders usually show a chronic slowly progressive course and present with multiorgan involvement with varying onset between birth and late adulthood. They represent a diagnostic challenge because of their wide variation in presentation and course. Systems frequently affected in mitochondrial disorders are peripheral nervous system, brain, endocrine system, heart, eyes, ears, guts, kidney and bone marrow. Although there is no specific therapy and cure for mitochondrial disorders, the rapidly increasing understanding of the pathophysiological background of the disorders may further facilitate the diagnostic approach and open perspectives to, possibly causative therapies in future.
Adenosine Triphosphate ; Bone Marrow ; Brain ; DNA, Mitochondrial ; Ear ; Electron Transport ; Endocrine System ; Energy Metabolism ; Heart ; Kidney ; Mitochondria ; Mitochondrial Diseases* ; Oxidative Phosphorylation ; Oxygen ; Parturition ; Peripheral Nervous System

OBJECTIVETo review the literature on the use of brain imaging, including functional magnetic resonance imaging (fMRI), positron emission tomography (PET), magnetic resonance spectroscopy (MRS) and voxel-based morphometry (VBM) in investigation of the activity in diverse brain regions that creates and modulates chronic neuropathic pain.

DATA SOURCESEnglish literatures from January 1, 2000 to July 31, 2007 that examined human brain activity in chronic neuropathic pain were accessed through MEDLINE/CD ROM, using PET, fMRI, VBM, MRS and receptor binding.

STUDY SELECTIONPublished articles about the application of fMRI, PET, VBM, MRS and chronic neuropathic pain were selected.

DATA EXTRACTIONData were mainly extracted from 40 representative articles as the research basis.

RESULTSThe PET studies suggested that spontaneous neuropathic pain is associated with changes in thalamic activity. Both PET and fMRI have been used to investigate the substrate of allodynia. The VBM demonstrated that brain structural changes are involved in chronic neuropathic pain, which is not seen in a matched control group. However, the results obtained had a large variety, which may be due to different pain etiology, pain distribution, lesion tomography, symptoms and stimulation procedures.

CONCLUSIONSApplication of the techniques of brain imaging plays a very important role in the study of structural and functional reorganization in patients with neuropathic pain. However, a unique "pain matrix" has not been defined. Future studies should be conducted using a prospective longitudinal research design, which would guarantee the control for many confounding factors.

Brain ; pathology ; physiopathology ; Humans ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Pain ; pathology ; physiopathology ; Peripheral Nervous System Diseases ; pathology ; physiopathology ; Positron-Emission Tomography ; Receptors, Dopamine ; metabolism ; Receptors, Opioid ; metabolism

Adolescent ; Adult ; CD57 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Male ; Middle Aged ; Neurilemmoma ; metabolism ; pathology ; Pelvic Neoplasms ; metabolism ; pathology ; Peripheral Nervous System Neoplasms ; metabolism ; pathology ; Retroperitoneal Neoplasms ; metabolism ; pathology ; S100 Proteins ; metabolism

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

Peripheral neuropathies occur in lymphoma patients. Causes of neuropathy include chemotherapy, opportunistic infections, and the lymphoma itself. We report a patient with lymphoma whose chief complaint was a sensory loss in the hands and feet. Electrophysiologic studies and sural nerve biopsy showed sensory polyneuropathies. We hypothesize that this neuropathy is associated with lymphoma-related ganglionopathy, and among the possible causes, we suspect that a systemic cause such as a paraneoplastic syndrome is the most likely pathogenic etiology. However, further follow-up will be necessary to see whether sensory symptoms change with lymphoma treatment.
Adult ; Biopsy ; Electrophysiology ; Hodgkin Disease/*complications/*diagnosis ; Human ; Lymphatic Metastasis ; Lymphoma/*metabolism ; Male ; Peripheral Nervous System Diseases/*complications/*pathology ; Sensation Disorders/complications/pathology

The quantitative sudomotor axon reflex testing (QSART) is a classic test of routine postganglionic sudomotor function. We investigated sudomotor function by QSART after summer (July 2012) and winter (January 2013) seasonal acclimation (SA) in the Republic of Korea. QSART with acetylcholine (ACh) iontophoresis were performed to determine directly activated (DIR) and axon reflex-mediated (AXR1, 2) sweating rate. Onset time of axon reflex, activated sweat gland density (ASGD), activated sweat gland output (ASGO), tympanic and skin temperatures (T(ty), T(sk)), basal metabolic rate (BMR), and evaporative loss volume changes were measured. Tympanic and mean body temperature (T(b); calculated from T(ty), T(sk)) were significantly lower after summer-SA than that of winter-SA. Sweat onset time was delayed during winter-SA compared to that after summer-SA. BMR, AXR(1), AXR(2), and DIR sweat rates, ASGD and ASGO, and evaporative loss volume were significantly diminished after winter-SA relative to after summer-SA. In conclusion, changes in sweating activity measured by QSART confirmed the involvement of the peripheral nervous system in variation of sudomotor activity in seasonal acclimation.
Acclimatization* ; Acetylcholine ; Axons ; Basal Metabolism ; Body Temperature ; Humans* ; Iontophoresis ; Peripheral Nervous System ; Reflex ; Republic of Korea ; Seasons* ; Skin Temperature ; Sweat ; Sweat Glands ; Sweating

OBJECTIVETo study the changes in microtubule motor protein expression in the spinal cord and sciatic nerve of rats exposed to carbon disulfide, and to investigate the possible molecular mechanism of changes in axonal transport in carbon disulfide-induced peripheral neuropathy.

METHODSHealthy adult male Wistar rats were randomly divided into one control group and three experimental groups (10 rats per group). The rats in experimental groups were intoxicated by gavage of carbon disulfide at a dose of 200, 400, or 600 mg/kg 6 times a week for 6 consecutive weeks, while the rats in control group were given the same volume of corn oil by gavage. Animals were sacrificed after exposure, with nerve tissue separated. The levels of dynein, dynactin, and kinesin in the spinal cord and sciatic nerve were determined by Western blot.

RESULTSThe content of dynein, dynactin, and kinesin in the sciatic nerve decreased significantly under exposure to carbon disulfide. The levels of dynein in the sciatic nerve were reduced by 23.47% and 33.34% at exposure doses of 400 and 600 mg/kg, respectively. The levels of dynactin in the sciatic nerve of the three experimental groups were reduced by 19.91%, 24.23%, and 41.30%, respectively. The level of kinesin was reduced by 25.98%under exposure to 600 mg/kg carbon disulfide. All the differences were statistically significant (P < 0.01). As compared with the control group, the 600 mg/kg group experienced a 28.24% decrease in level of dynactin in the spinal cord (P < 0.01), but no significant change was observed in the level of dynein or kinesin.

CONCLUSIONCarbon disulfide has an impact on microtubule motor protein expression in nerve tissues, which might be involved in the development of carbon disulfide-induced peripheral neuropathy.

Animals ; Axonal Transport ; drug effects ; physiology ; Carbon Disulfide ; toxicity ; Dynactin Complex ; Male ; Microtubule-Associated Proteins ; metabolism ; Nerve Tissue ; metabolism ; Peripheral Nervous System Diseases ; chemically induced ; metabolism ; Rats, Wistar ; Sciatic Nerve ; metabolism ; Spinal Cord ; metabolism

OBJECTIVETo study clinicopathologic features, immunohistochemical profile, diagnosis and differential diagnosis of childhood central nervous system primitive neuroectodermal tumors (CNS PNETs) with the features of ependymoblastoma and neuroblastoma.

METHODSThe clinical data, morphologic and immunohistochemical features were analyzed in 4 cases of pediatric CNS PNETs with features of ependymoblastoma and neuroblastoma. EnVision method immunohistochemistry was applied.

RESULTSFour patients including three boys and one girl presented at the age from 12 month to 4 years and three tumors located in cerebrum, one in brain stem. All tumors showed typical combined histological patterns of ependymoblastoma and neuroblastoma, demonstrating zones of true rosettes, occasional pseudovascular rosettes, and undifferentiated neuroepithelial cells in a prominent background of mature neuropils. There was focal expression of glial fibrillary acidic protein (GFAP) consistent with glial differentiation and epithelial membrane antigen (EMA) consistent with ependymal differentiation. Necrosis was seen in three cases and calcification was present in one case. Immunohistochemically, the rosettes and undifferentiated neuroepithelial cells were positive for vimentin, partially positive for GFAP and EMA but negative for synaptophysin. The tumor cells were also positive for synaptophysin in neuropils. The Ki-67 label index ranged from 20% to 60%.

CONCLUSIONSCNS PNETs with the features of ependymoblastoma and neuroblastoma is a rare tumor with poor prognosis. The tumor primarily occurs in childhood, especially infant and belongs to the family of embryonal tumors of the CNS. The morphologic, immunohistochemical and genetic features are important in differential diagnosis from other tumors of the CNS.

Antigens, Neoplasm ; metabolism ; Central Nervous System ; pathology ; Child ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Immunohistochemistry ; Infant ; Male ; Mucin-1 ; metabolism ; Neuroblastoma ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; pathology ; Synaptophysin ; metabolism ; Vimentin ; metabolism