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Conduction Velocity (conduction + velocity)
Kinds of Conduction Velocity Selected AbstractsHeterogeneity of Ventricular Fibrillation Dominant Frequency During Global Ischemia in Isolated Rabbit HeartsJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 8 2007Ch.B. , JANE CALDWELL M.B. Introduction: Ventricular fibrillation (VF) studies show that ECG-dominant frequency (DF) decreases as ischemia develops. This study investigates the contribution of the principle ischemic metabolic components to this decline. Methods and Results: Rabbit hearts were Langendorff-perfused at 40 mL/min with Tyrode's solution and loaded with RH237. Epicardial optical action potentials were recorded with a photodiode array (256 sites, 15 × 15 mm). After 60 seconds of VF (induced by burst pacing), global ischemia was produced by low flow (6 mL/min), or the solution changed to impose hypoxia (95% N2/5% CO2), low pHo (6.7, 80% O2/20% CO2), or raised [K+]o (8 mM). DF of the optical signals was determined at each site. Conduction velocity (CV), action potential duration (APD90), effective refractory period (ERP), activation threshold, dV/dtmax, and membrane potential were measured in separate experiments during ventricular pacing. During VF, ischemia decreased DF in the left ventricle (LV) (to [58 ± 6]%, P < 0.001), but not the right (RV) ([93 ± 5]%). Raised [K+]o reproduced this DF pattern (LV: [67 ± 12]%, P < 0.001; RV: [95 ± 9]%). LV DF remained elevated in hypoxia or low pHo. During ventricular pacing, ischemia decreased CV in LV but not RV. Raised [K+]o did not change CV in either ventricle. Ischemia and raised [K+]o shortened APD90 without altering ERP. LV activation threshold increased in both ischemia and raised [K+]o and was associated with diastolic depolarization and decreased dV/dtmax. Conclusions: These results suggest that during VF, decreased ECG DF in global ischemia is largely due to elevated [K+]o affecting the activation thresholds in the LV rather than RV. [source] A Sonic Hedgehog (SH) Fusion Protein Corrects Multifocal Defects In Experimental Diabetic NeuropathyJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 3 2000Dr Tomlinson Diabetic neuropathy develops from defective interactions between nerve axons and other cells in the endoneurium; such interactions are influenced in development by hedgehog proteins. This study explored the possibility that this might be maintained in the adult and form a basis for therapy in diabetic neuropathies. Streptozotocin-diabetic rats were treated (final 5 weeks of 10 weeks diabetes) with a SH-IgG fusion protein (either 0.3mg/kg or 3.0mg/kg s.c. 3 times per week); control diabetic and non-diabetic rats received vehicle. Conduction velocity (MNCV, SNCV) data and sciatic nerve levels of nerve growth factor (NGF) and neuropeptide Y (NPY) are presented below. Diabetes caused significant (p < 0.05 by ANOVA with SNK tests) reductions in all variables and treatment with SH-IgG either attenuated or prevented (p < 0.05) these reductions. Since it is well-established that the conduction deficits are unrelated to neurotrophic deficits (NGF depletion) and that NPY depletion derives from a neurotrophic defect distinct from NGF, this treatment clearly acts at multiple components of the aetiology of diabetic neuropathy. [source] Conduction velocity is regulated by sodium channel inactivation in unmyelinated axons innervating the rat cranial meningesTHE JOURNAL OF PHYSIOLOGY, Issue 4 2008Roberto De Col Axonal conduction velocity varies according to the level of preceding impulse activity. In unmyelinated axons this typically results in a slowing of conduction velocity and a parallel increase in threshold. It is currently held that Na+,K+ -ATPase-dependent axonal hyperpolarization is responsible for this slowing but this has long been equivocal. We therefore examined conduction velocity changes during repetitive activation of single unmyelinated axons innervating the rat cranial meninges. In direct contradiction to the currently accepted postulate, Na+,K+ -ATPase blockade actually enhanced activity-induced conduction velocity slowing, while the degree of velocity slowing was curtailed in the presence of lidocaine (10,300 ,m) and carbamazepine (30,500 ,m) but not tetrodotoxin (TTX, 10,80 nm). This suggests that a change in the number of available sodium channels is the most prominent factor responsible for activity-induced changes in conduction velocity in unmyelinated axons. At moderate stimulus frequencies, axonal conduction velocity is determined by an interaction between residual sodium channel inactivation following each impulse and the retrieval of channels from inactivation by a concomitant Na+,K+ -ATPase-mediated hyperpolarization. Since the process is primarily dependent upon sodium channel availability, tracking conduction velocity provides a means of accessing relative changes in the excitability of nociceptive neurons. [source] The organisation of invertebrate brains: cells, synapses and circuitsACTA ZOOLOGICA, Issue 1 2010Ian A. Meinertzhagen Abstract Meinertzhagen, I.A. 2010. The organisation of invertebrate brains: cells, synapses and circuits. ,Acta Zoologica (Stockholm) 91: 64,71 Invertebrate brains are structurally diverse. Neuron numbers range from ,102 to 108 in different groups, compared with larger numbers in vertebrate brains, ,107 to 1014. The underpopulated brains of invertebrates are noted in their extreme cases for having few cells, and neurons that can be identified from animal to animal, many known in great detail. Although few in number, invertebrate neurons nevertheless comprise many classes. Correlated with the paucity of their number they are sparsely connected, many having ,50 synapses or fewer. Synaptic densities, roughly 1 per ,m3 of neuropile, differ little from those for much larger vertebrate neurons. Invertebrate neurons differ from their vertebrate counterparts in the position of their soma, generally in a cortex surrounding the neuropile that consequently occupies a relatively small volume. Their axons typically lack myelin and, supporting a range of conduction velocities, have diameters that differ over a wide range, from 103 to 10,1,m. Nerves with thousands of axons differ from neuropile fascicles, which typically have 20 or less. Unlike most vertebrate synapses, but like those of the vertebrate retina, synapses in many invertebrate groups , probably all ecdysozoans and possibly some lophotrochozoans , have synaptic contacts with multiple postsynaptic elements, dyads, triads and so on. [source] Lumbosacral spinal cord somatosensory evoked potentials for quantification of nociception in horsesEQUINE VETERINARY JOURNAL, Issue 3 2010J. P. A. M. Van LOON Summary Reasons for performing study: There is a need for objective evaluation and quantification of the efficacy of analgesic drugs and analgesic techniques in horses. Objectives: To determine whether lumbosacral spinal cord somatosensory evoked potentials (SSEP) can be a useful and reliable tool to assess nociception in equines. Methods: SSEPs and electromyograms (EMG) from the epaxial muscles were recorded simultaneously, following electrical stimulation applied to the distal hindlimb in lightly anaesthetised Shetland ponies (n = 7). In order to validate the model, the effect of increasing stimulus intensity was documented and the conduction velocities (CV) of the stimulated nerves were calculated. The effect of epidurally applied methadone (0.4 mg/kg bwt) in a randomised, crossover design was investigated. Results: Two distinct complexes (N1P1 and N2P2) were identified in the SSEP waveform. Based on their latency and conduction velocity and the depressant effect of epidurally applied methadone, the SSEP N2P2 was ascribed to nociceptive A,-afferent stimulation. The SSEP N1P1 originated from non-nociceptive A,-afferent stimulation and was not influenced by epidurally applied methadone. Conclusions and potential relevance: The nociceptive A, component of the SSEP, the N2P2 complex, is presented as a valid and quantitative parameter of spinal nociceptive processing in the horse. Validation of the equine SSEP model enables the analgesic effects of new analgesics/analgesic techniques to be quantified and analgesia protocols for caudal epidural analgesia in equidae improved. [source] Electrophysiological sensory demyelination in typical chronic inflammatory demyelinating polyneuropathyEUROPEAN JOURNAL OF NEUROLOGY, Issue 7 2010Y. A. Rajabally Background:, The presence of electrophysiological demyelination of sensory nerves is not routinely assessed in the evaluation of suspected chronic inflammatory demyelinating polyneuropathy (CIDP). Whether this can be useful is unknown. Methods:, We compared, using surface recording techniques, in 19 patients with typical CIDP and 26 controls with distal large fibre sensory axonal neuropathy, the forearm median sensory conductions, sensory nerve action potential (SNAP) amplitudes and durations and sensory nerve conduction velocities (SNCVs) of median, radial and sural nerves. Results:, Median nerve sensory conduction block (SCB) across the forearm was greater in CIDP patients than in controls (P = 0.005). SNAP durations were longer in CIDP patients for median (P = 0.001) and sural nerves (P = 0.004). Receiver operating characteristic (ROC) curves provided sensitive (>40%) and specific (>95%) cut-offs for median nerve SCB as well as median and sural SNAP durations. SNCVs were significantly slower for median and sural nerves in CIDP patients, but ROC curves did not demonstrate cut-offs with useful sensitivities/specificities. Median SCB or prolonged median SNAP duration or prolonged sural SNAP duration offered a sensitivity of 73.7% for CIDP and specificity of 96.2%. Used as additional parameters, they improved diagnostic sensitivity of the American Academy of Neurology (AAN) criteria for CIDP of 1991, from 42.1% to 78.9% in this population, with preserved specificity of 100%. Discussion:, Sensory electrophysiological demyelination is present and may be diagnostically useful in typical CIDP. SCB detection and SNAP duration prolongation appear to represent more useful markers of demyelination than SNCV reduction. [source] Ulnar neuropathy at the elbow due to unusual sleep positionEUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2000J. Finsterer Abnormal strain of the ulnar nerve over the sulcus due to an unusual sleep position is a rare cause of ulnar neuropathy at the elbow. A 57-year-old patient with Mandelung's deformity developed progressive weakness in the flexion of fingers 4 and 5 and in finger straddling on the left side. Additionally, there was slight wasting of the left hypothenar and the left interossei muscles. Motor and sensory nerve conduction studies of the left ulnar nerve showed delayed conduction velocities over the left ulnar sulcus. He preferred to sleep in a left lateral position with his head lying on a headrest roll, his left forearm being flexed at 110° and his hand lying either under his cheek or placed on the roll. Only three weeks after the patient had been advised to change his sleep position and to sleep without the headrest roll, weakness markedly improved. This case shows that sleeping in a lateral position with the head on a headrest roll and the hand placed on the roll or under the cheek may cause ulnar neuropathy at the elbow. Change of such a habitual sleep position promptly resolves the symptoms. [source] Phenotype and Function of Somatic Primary Afferent Nociceptive Neurones with C-, A,- or A,/,-FibresEXPERIMENTAL PHYSIOLOGY, Issue 2 2002S. N. Lawson Nociceptive dorsal root ganglion (DRG) neurones have fibres that conduct in the C, A, and A,/, conduction velocity range. The properties of nociceptive compared with non-nociceptive somatic afferent dorsal root ganglion neurones appear to fall into two patterns, A and B. Pattern A properties of nociceptive neurones, the more common type, include longer action potential duration and slower maximum rate of fibre firing, as well as a greater expression of substance P and calcitonin gene-related peptide immunoreactivity. The values of pattern A properties appear to be graded according to the conduction velocity group (C, A, or A,/,) of the fibres. The most pronounced forms of A-type properties are expressed by nociceptive neurones with C-fibres, and these become less pronounced in nociceptive neurones with A,-fibres and least pronounced in those with A,/, fibres (C > A, > A,/,). Some of these properties are also expressed in a less extreme but similarly graded manner through C, A, and A,/, groups of non-nociceptive low threshold mechanoreceptive (LTM) neurone. The less common pattern B properties of nociceptive neurones have similar values in C-, A,- and A,/,-fibre nociceptive neurones but these clearly differ from LTM units with C-, A,- and A,/,-fibre conduction velocities. These features of nociceptive neurones include consistently larger action potential overshoots and longer after-hyperpolarisation durations in nociceptive than in LTM neurones. [source] Effect of pneumatic power tool use on nerve conduction velocity across the wristHUMAN FACTORS AND ERGONOMICS IN MANUFACTURING & SERVICE INDUSTRIES, Issue 4 2005John Rosecrance The purpose of this study was to determine if the use of pneumatic power tools altered electrophysiologic properties of the median and ulnar nerves at the wrist during the work shift. Sensory nerve conduction velocities were measured in hands of workers before work and then at 2-hour intervals during the workday. Ten workers exposed to pneumatic power tool use and 10 workers not exposed to intensive hand activity were evaluated. The conduction velocities slowed significantly across the wrist in the median and ulnar nerves among workers using pneumatic tools but not among control workers. This investigation demonstrated that short-term exposure to highly intensive hand tasks causes significant slowing in nerve conduction velocity across the wrist. © 2005 Wiley Periodicals, Inc. Hum Factors Man 15: 339,352, 2005. [source] The Relationship Between Endocardial Voltage and Regional Volume in Electroanatomical Remodeled Left Atria in Patients with Atrial Fibrillation: Comparison of Three-Dimensional Computed Tomographic Images and Voltage MappingJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 12 2009JAE HYUNG PARK B.Sc. Background: Long-standing atrial fibrillation (AF) changes left atrial (LA) morphology, and the LA size is related to recurrence after radiofrequency catheter ablation (RFCA). We hypothesize that LA morphology, based on embryological origin, affects the outcome of RFCA. Methods: We analyzed 3D computed tomographic (CT) images of LA in 70 patients with AF (54 males, 55.6 ± 10.5 years old, paroxysmal AF (PAF):persistent AF (PeAF) = 32:38) who underwent RFCA. Each LA image was divided into venous atrium (VA), anterior LA (ALA), LA appendage (LAA), and both antrum. Absolute and relative volumes were calculated, and the lengths of linear ablation sites were measured. Results: (1) In patients with the mean LA voltage , 2.0 mV, LA volume, especially ALA, was larger (P < 0.01) compared to those with LA voltage > 2.0 mV. (2) The total LA volume was significantly larger (P < 0.01) and LAA voltages (P < 0.05) and conduction velocities (P < 0.05) were lower in patients with PeAF than in those with PAF. (3) In patients with recurrence, LA volume was generally larger (P < 0.01) than in those without recurrence. In PAF patients with recurrence, the relative volume of ALA was significantly larger (P < 0.01) than those without recurrence. Conclusions Morphologically remodeled LA has low endocardial voltage, and enlargement of ALA is more significant in electroanatomically remodeled LA. The disproportional enlargement of ALA was observed more often in PAF patients with recurrence after ablation than those without recurrence. [source] Diabetes mellitus exacerbates motor and sensory impairment in CMT1AJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 4 2008Soham Sheth Abstract Charcot-Marie-Tooth disease type 1A (CMT1A) is caused by a duplication of PMP22 on chromosome 17 and is the most commonly inherited demyelinating neuropathy. Diabetes frequently causes predominantly sensory neuropathy. Whether diabetes exacerbates CMT1A is unknown. We identified 10 patients with CMT1A and diabetes and compared their impairment with 48 age-matched control patients with CMT1A alone. Comparisons were made with the Charcot-Marie-Tooth disease (CMT) neuropathy score (CMTNS) and by electrophysiology. The CMTNS was significantly higher in patients with diabetes (20.25 ± 2.35) compared with controls (15.19 ± 0.69; p = 0.01). Values were particularly higher for motor signs and symptoms. Seven of the 10 diabetic patients had CMTNS >20 (severe CMT), while only 7 of the 48 age-matched controls had scores >20. There was a trend for CMT1A patients with diabetes to have low compound muscle action potentials and sensory nerve action potentials, although nerve conduction velocities were not slower in diabetic patients compared with controls. Diabetes was associated with more severe motor and sensory impairment in patients with CMT1A. [source] Electrophysiological features in the distinction between hereditary demyelinating and chronic acquired demyelinating neuropathiesJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2004F Poglio We carried out an electrophysiological retrospective study in 55 patients with chronic demyelinating acquired and hereditary neuropathies. Alterations of motor nerve conduction velocities (MNCV), distal motor latencies (DML), conduction blocks (CB) and compound muscle action potential (CMAP) were compared, considering the whole number of nerves for each disease. MNCV, DML, CB and CMAP were considered suggestive of demyelination when meeting the American Academy of Neurology (AAN) criteria. Abnormally slow MNCV was found respectively in the 46% of all the CMTX female nerves studied, in the 56.5% of CMTX males, 84% of CMT1A, 74% CIDP and 70% of MAG-PNP. Prolonged DML was observed in the 25% of the CMTX female nerves studied, in the 49.5% of CMTX males, 81% of CMT1A, 63% of CIDP and 71% of MAG-PNP. Moreover, CB were quite often evidenced in CIDP and MAG-PNP nerves (respectively in 48% and 29%) and rarely in hereditary neuropathies. Finally, we observed CMAP reduction in the 45% of all the CMTX female nerves studied, in the 50% of CMTX males, 63% of CMT1A, 49% of CIDP and 60% of MAG-PNP. A well-characterized pattern generally allows an electrophysiological distinction between CMT1A, CMTX males, MAG-PNP on one side and CIDP and CMTX females on the other side. A clear electrodiagnostic distinction result is often hard between CMTX males and MAG-PNP and between CIDP and CMTX females. [source] Unravelling the molecular basis of CMT4B pathologyJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2004A Bolis Charcot-Marie-Tooth type 4B (CMT4B) disease is a severe autosomal recessive peripheral neuropathy with childhood onset, characterised by progressive muscular atrophy and weakness in the distal extremities, sensory loss, severely decreased nerve conduction velocities, and demyelination with myelin outfoldings in the peripheral nerve. We demonstrated that CMT4B is caused by loss of function mutations in the Myotubularin-related 2 gene, MTMR2, on chromosome 11q22 (Bolino et al., Nat Genet 25:17,19, 2000). MTMR2 belongs to the myotubularin family of protein phosphatases, of which, myotubularin (MTM), mutated in the X-linked myotubular myopathy (XLMTM) is the founder member. MTMR2 shows specific activity towards phosphatidylinositol 3-phosphate and 3,5-biphosphate, PI(3)P and PI(3,5)P2, respectively. However, how abrogation of this lipid phosphatase activity is leading to the specific disease phenotype has not yet been demonstrated. To elucidate the biological role of MTMR2 in the nerve, we performed an extensive expression analysis of this protein in the peripheral nervous system. Since MTMR2 was demonstrated to be ubiquitously expressed also within the nerve, we sought nerve-specific interactors using the yeast two-hybrid approach. The neurofilament light chain protein, NF-L, mutated in various CMTs including axonal type CMT2E, and demyelinating Dejerine-Sottas syndrome, was found to interact with MTMR2 in Schwann cells as well as in neurons. Since NF-L is specifically expressed in the nervous system, the interaction between MTMR2 and NF-L would explain why loss of a ubiquitously expressed phosphatase affects specifically the nerve (Previtali and Bolino, Hum Mol Genet 12:1713,1723, 2003). To model the CMT4B pathology, we generated a general knock-out mouse arising from inactivation f Mtmr2 in all cells. The characterisation of this animal model is underway. Overall, Mtmr2 null mice display a milder phenotype with respect to the human disorder. The morphological analysis of the peripheral nerve of this mouse line performed at P28 revealed the presence of myelin outfoldings, which are the hallmark of CMT4B pathology. [source] Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 76JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2003D Pareyson The X-linked form of Charcot-Marie-Tooth disease (CMTX) is associated with mutations in the Connexin 32 gene (Cx32) and is the second most common CMT subtype after CMT1A, in which the 17p11.2 duplication is the underlying molecular defect. CMTX is characterized by no male-to-male transmission, intermediate motor conduction velocities (MCV), and more severe disease in males. In our series of CMT patients, we found 9 different Cx32 mutations in 11 families. Overall there were 26 patients, 13 males and 13 females, aged 11,76 yrs. Age at onset ranged considerably (1,60 yrs), but symptoms began earlier in males (mean 15.4 yrs, 77% within age 20) than in females (mean 25 yrs). All patients were autonomous, but disease severity was greater in males, while 4 female carriers were asymptomatic. Pain and tremor were frequent complaints. Two patients had Babinski sign and one had rest tremor. Nerve conduction studies were performed in 23 patients (13 males, 10 females). Upper limb motor conduction velocities (MCV) ranged between 25 and 57 m/s, and were slower in males (25,48 m/s) than in females (34,57 m/s). MCV were in the upper range of CMT1 (25,38 m/s) in 10/13 males but only in 3/10 females. In some cases, nerve conduction slowing was non-uniform within single nerves, and one female patient had a previous diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy. There was considerable asymmetry of involvement between different nerves. The median nerve was often more severely affected than the ulnar nerve, and not only in females, as previously reported, but also in males. Therefore, it appears unlikely that this asymmetry is accounted for by a Lyonization phenomenon. Subclinical abnormalities of central nervous system as revealed by multimodal evoked potential studies were found in 8/10 patients. Expression of Cx32 in the brain is the likely explanation of this finding that confirms previous non-systematic observations. We found seven missense and two nonsense mutations (one novel mutation). Two families presented distinct mutations at the same codon (Arg164), while the Arg22Stop and Arg220Stop mutations were each found in two unrelated cases. Partially supported by a grant from the Italian Ministry of Health to F.T and D.P. (Progetto Ricerca Finalizzata ICS 030.3/RF00.174). [source] Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 57JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2003D Cocito BACKGROUND: Since 1991, five sets of electrophysiological criteria for CIDP have been reported. However, until now, receiver operator characteristic (ROC), such as sensitivity and specificity, of only AAN criteria were investigated, showing a high specificity, but intermediate sensitivity. The application of these criteria may be useful in clinical trials, but is inadequate in clinical practice, since they preclude immunomodulating treatment in patients who do not meet them. OBJECTIVE: 1) to evaluate the ROC and predictive value of five different electrophysiological criteria for CIDP (AAN, INCAT, Rotta et al, Nicholas et al. and Saperstein et al.); 2) to identify the most informative electrophysiological features indicative of demyelination by mean of the likelihood ratio; 3) to determine, in our series of cases, a set of minimal electrophysiological criteria (albeit aspecific) enough sensitive for CIDP diagnosis. PATIENTS AND METHODS: 20 patients with sensorimotor polyneuropathy, progressive for at least 2 months, with weakness in least two limbs and documented improvement in strength in response to immunotherapy. Other potential causes were excluded, including diabetes and IgM paraproteinemia with or without anti-MAG reactivity. Twelve patients with axonal polyneuropathy associated with diabetes and 12 patients with amyotrophic lateral sclerosis were included as controls. Nerve conduction studies (NCS) were evaluated according to all five sets of NCS criteria and ROC was calculated. Likelihood ratio for CIDP was evaluated for each set of NCS criteria and for presence of each of the following features in different number of nerves: motor conduction velocities (MCV), conduction blocks/temporal dispersion, distal motor (DML) and F-wave latencies (FWL). MCV, DML and FWL were considered indicative of demyelination if decreased or increased as suggested by AAN criteria. Results will be discussed. [source] AUTOMIC FAILURE AND NORMAL PRESSURE HYDROCEPHALUS IN A PATIENT WITH CHRONIC DEMYELINATING INFLAMMATORY NEUROPATHYJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2002M. Laurą A 75-year-old man with HCV hepatitis developed at the age of 70 presented with rest and action tremor localized at both hands and progressive cognitive impairment with memory loss. Four years later he begun to complain of progressive fatigue, occasional falls, numbness at the extremities and orthostatic hypotension. One month after admission, he rapidly worsened with inability to walk, mainly because of autonomic failure. Neurological examination revealed gait disturbances, including a wide base of support and short stride, slurred speech, reduction of upward gaze, rest and action tremor at both hands, intrinsic hand muscle and anterior tibialis muscle wasting and weakness on both sides, absent deep tendon reflexes, loss of vibration sense at lower limbs, and bilateral pes cavus. Routine laboratory studies, autoantibodies, thyroid function, neoplastic markers and immunoelectrophoresis were normal. Cryoglobulins were absent, whereas CSF protein content was increased (142 mg/dl). Autonomic nervous system investigation detected severe orthostatic hypotension. Nerve conduction studies showed absent sensory potentials and a marked reduction of compound motor action potential amplitudes and of motor conduction velocities. A sural nerve biopsy revealed remarkable onion bulb-like changes, endoneurial and perivascular infiltrations of inflammatory cells. Psychometric tests showed mild cognitive impairment. Brain MRI was consistent with normotensive hydrocephalus. The findings indicated the presence of chronic inflammatory demyelinating polyneuropathy, autonomic nervous system involvement and normal pressure hydrocephalus. A condition of multiple system atrophy (MSA) might be taken into account, even if somatic peripheral nerve involvement may rarely occur in MSA. Moreover the normal pressure hydrocephalus could be due to the high protein content in CSF (Fukatsu R et al., 1997). [source] Novel MPZ Mutation In A Sporadic CMT PatientJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2001E Bellone Mutations in the gene for the major structural protein component of peripheral nerve myelin, myelin protein zero (MPZ), are associated with some forms of hereditary neuropathies such as Charcot-Marie-Tooth disease type 1B (CMT1B), Dejerine-Sottas syndrome (DSS) and congenital hypomyelinating neuropathy (CHN). The common pathological characteristics of these allelic disorders are severe demyelination and remyelination of peripheral nerves. Recently, MPZ mutations were also found in patients with the axonal form of CMT neuropathy (CMT2). We studied a patient with negative familiar history and clinical and electrophysiological features of Charcot-Marie-Tooth disease: distal muscle weakness and atrophy, foot deformities (pes cavus), and severely reduced nerve conduction velocities in the motor and sensory nerves. The sural nerve biopsy showed marked loss of myelinated fibers, few onion bulbs, and a high percentage of fibers showing excessive myelin outfoldings. DNA analysis excluded CMT1A duplication by Southern blot and by pulsed field gel electrophoresis methods. SSCP analysis of all six exons of MPZ revealed a shift band in exon 2 in the patient's DNA. No such difference was detected in normal controls. Direct sequencing disclosed a G , A transition at nucleotide position 181. This base substitution predicts the replacement of aspartic acid with asparagine at codon 61. A mutation at the same codon (but different amino acid replacement) was recently identified in a family with the axonal type of CMT, in which the disease was autosomal dominantly inherited. This finding provides further confirmation of the role of MPZ gene in peripheral neuropathies and suggests that MPZ coding region mutations may account for a considerable number of CMT cases which do not involve DNA duplication on 17p11.2-p12. This research was partially supported by a MURST and an Ateneo grant to FA, by a Ministero della Sanitą grant to PM. Our laboratory is a member of the European Charcot-Marie-Tooth Consortium co-ordinated by Prof. Christine Van Broeckhoven. [source] Reduced Nerve Blood Flow In Diabetic Rats Is A Reflection Of Hindlimb Muscle WastingJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 3 2000Dr Tomlinson We examined the influence of muscle wasting, as a result of streptozotocin-induced diabetes, on sciatic nerve laser Doppler flux (SNLDF), as an index of nerve blood flow, and conduction velocity (NCV). We compared dietary-restricted weight-reduced non-diabetic rats with controls and with diabetic rats and we studied the effects of clenbuterol, an anabolic ,-adrenoceptor agonist, in control and diabetic rats. Dietary restriction reduced the weights of hindlimb muscles,extensor digitorum longus, soleus and gastrocnemius,half as much as did streptozotocin-diabetes and clenbuterol increased muscle weights in control and diabetic rats. This gave a hierarchy of muscle weights in the order,clenbuterol-controls, untreated controls, weight-reduced non-diabetics, clenbuterol-diabetics and untreated diabetics. Diabetes without treatment reduced SNLDF by 51% (p < 0.01); dietary restriction by 25% (p < 0.01) and there were proportional increases associated with clenbuterol treatment. Combined muscle weights regressed closely with SNLDF (r2=0.69; p < 0.001) and, when the latter was expressed relative to muscle weights, a similar value was obtained for all five groups,there were no significant differences. Thus, sciatic nerve blood flow is closely related to hindlimb muscle weight and the effect of diabetes on nerve blood flow may be secondary to muscle wasting. Sciatic/tibialis motor and sensory conduction velocities were also reduced by muscle wasting in the dietary restricted group of non-diabetic rats, but, unlike nerve Doppler flux, it was unaffected by clenbuterol. [source] POSTIRRADIATION LUMBOSACRAL RADICULOPLEXOPATHY: IMPROVEMENT AFTER IMMUNE THERAPYJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2000A. Bersano A delayed progressive impairment of peripheral nervous system including brachial and lumbosacral radiculoplexopathy is a well-known complication of local radiotherapy. No treatment for this infrequent complication is currently available. Recently, improvement after treatment with high dose immunoglobulin (IVIg) has been reported in some patients, suggesting either an immune-mediated inflammatory nerve damage induced by irradiation or a dysimmune neuropathy (CIDP-like) misdiagnosed as a postirradiation disease. We report on two patients who developed motor lumbosacral radiculoplexopathy several years after local radiotherapy. The first patient (ZA) is a 49 y.o. man developing a progressive proximal>distal weakness and hypotrophy of lower limbs, 20 years after radiotherapy of lumbosacral region for seminoma. Electrophysiological studies showed markedly reduced motor conduction velocities (CV) and prolonged F-wave latencies in lower limb nerves. The second patient (BF), is a 52 y.o. woman who developed progressive left brachial plexopathy and distal>proximal weakness and hypotrophy of lower limbs 12 years after a first course of toracoascellar and lumbar irradiation for Hodgkin lymphoma followed by a second course of cervicoclavicular irradiation for tumor recurrence 7 years later. Electrophysiological studies showed markedly reduced CMAP amplitudes and proportionally reduced CV in motor nerves. No sensory impairment was detected in both patients. CSF protein was elevated in both patients while cells were normal. On the assumption of a possible dysimmune origin of the disease, patient ZA underwent high dose intravenous steroid treatment, while patient BF, who had previously deteriorated after steroids, was treated with IVIg. After treatment, patient ZA became able to walk with less waddling, to rise from the floor and climb stairs without support, and to run. Improvement was less consistent in patient BF, whose right leg strength improved even if she still needed bilateral support to walk. The improvement observed in both patients supports the hypothesis that, at least in some patients, an immune-mediated mechanism may underlie postirradiation radiculoplexopathy. [source] ROSS SYNDROME: CLINICAL AND LABORATORY EVALUATION OF TWO CASESJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2000G. De Joanna We describe two males, aged 41 and 55, come to our observation complaining of heat intolerance, abnormal increase in body temperature with minimal exercising, reduced sweating and, generalized fatigability; one of them had distal paresthesias. Neurologic evaluation showed bilateral Adie's tonic pupil and an absence of deep-tendon jerks. A diagnosis of Ross' Syndrome was advanced. Autonomic tests, nerve conduction study, H-reflex, computerized termoregulatory and pain thresholds, laser CO2 cortical evoked potentials, and skin biopsy were performed. One of them performed a histamine test and hand photopletismography resulted positive for sympathetic impairment, and pilocarpine pupil test that showed a parasympathetic denervation hypersensitivity. The following tests gave the same results in both patients: parasympathetic and most sympathetic tests were normal. Sympathetic skin response was absent and Minor test showed an almost complete absence of sweating. Sweating was possible only in two or three small areas. Positive pilocarpine test suggested a postganglionic involvement of sympathetic nervous system. Sensitive and motor nerve conduction velocities were normal, while H-reflex was not detectable. Termoregulatory and pain thresholds were abnormal. Laser CO2 cortical evoked potentials showed the absence of C fibre potentials, whereas A-, fibres response was abnormal in one of them. Hairy skin biopsy showed a definite reduction of sweat glands and of small vessel innervation; glabrous skin biopsy performed in one of them showed a reduced number of Meissner corpuscles. These findings suggest that in Ross' Syndrome the degenerative process can involve, besides the autonomic fibres, myelinated somatosensory fibres also. [source] SUB-CLINICAL PERIPHERAL NERVE INVOLVEMENT IN PSORIATIC ARTHRITISJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2000C. Di Girolamo Immunological studies document the role of HLA in psoriasis and the correlation between neuropeptides, psoriasis, and related arthritis. Some anecdotal case reports, moreover, describe a noncasual association between peripheral neuropathy and psoriatic manifestations. To verify a possible subclinical peripheral nerve involvement in this disimmune pathology, we started a pilot study in twenty patients with psoriatic arthritis and in whom other common causes of peripheral neuropathies had been ruled out. We performed a complete clinical neurological examination and a neurophysiological examination (orthodromic sensory and motor nerve conduction velocity in median and tibial nerves; antidromic sensory nerve conduction velocity in sural nerve). In 40% of the patients there was a mild but definite "glove-stocking" hypoesthesia, while hypopallesthesia was detected in only 20%. Electrophysiologic examinations were less informative borderline distal conduction velocities in 30% of patients. These preliminary data suggest a peripheral nerve involvement in this pathology, mainly affecting the small nerve fibres. [source] CLINICAL AND IMMUNOLOGICAL FEATURES AND RESPONSE TO IVIg IN PATIENTS WITH CLINICALLY TYPICAL MULTIFOCAL MOTOR NEUROPATHY BUT NO OVERT CONDUCTION BLOCKJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2000E. Nobile-Orazio Multifocal motor neuropathy (MMN) is characterized by progressive asymmetric limb weakness usually predominant in the upper limbs associated with conduction block (CB) in motor but not sensory nerves. There are, however, occasional patients with clinically typical MMN in whom no CB can be detected. Whether these patients differ from patients with MMN and CB remains unclear. Since 1991, we have observed 24 patients with the typical clinical features of MMN. In 20 of them (14 men and 6 women), electrophysiological studies disclosed the presence of CB in at least one motor nerve. In four (all women), no evidence of CB could be detected in examined nerves even if three had some features of demyelination, including asymmetric reduction of motor conduction velocities (1 patient) or prolonged or absent F wave latencies (3 patients). Three of them had markedly reduced or absent proximal and distal CMAP amplitudes in some nerves. The mean age of onset of MMN was similar in patients with (41.5 years, range 21,70) and without CB (41.5 years, range 24,57). The mean duration of the disease at the time of our first visit was longer in patients without CB (18.5 years, range 13,25) than in those with CB (6.3 years, 3 months,25 years); only 3 patients with CB had a duration of the disease longer than 10 years. All patients without CB had a predominant or exclusive impairment of upper limbs compared with 18 (90%) of those with CB. The mean Rankin score before therapy was slightly worse in patients without (2.5) than with (2.2) CB. Anti-ganglioside antibodies were found in 1 patient without CB (25%) and in 8 (40%) with CB. All but 2 patients with CB (90%) consistently improved with IVIg. All patients without CB also improved with IVIg, but only one did so consistently. In conclusion, patients with the typical clinical presentation of MMN but no overt CB are clinically and immunologically indistinguishable from those with MMN and CB. The longer duration of the disease and frequent axonal impairment in patients without CB may explain the lower efficacy of IVIg in these patients than in those with CB. [source] Resolution of alcoholic neuropathy following liver transplantationLIVER TRANSPLANTATION, Issue 12 2004Edward Gane Between 10 and 20% of adult liver transplants are performed for end-stage alcoholic liver disease. Severe extrahepatic end-organ damage from alcoholism (cardiomyopathy, pancreatitis, central nervous system injury, and neuropathy) is widely regarded as an absolute contraindication to liver transplantation, despite a lack of data on the effect of transplantation on these complications. We describe such a patient who presented with decompensated alcoholic liver disease and moderately severe peripheral neuropathy. Both his liver failure and neuropathy progressed despite 9 months abstinence and intensive nutritional support. By 12 months post-transplant, however, this patient had regained almost normal muscle strength, with associated recovery in sensory and motor conduction velocities. Direct alcohol toxicity, nutritional and vitamin deficiencies, and liver failure were all likely etiologic factors in this patient's neuropathy. In conclusion, this case suggests that peripheral neuropathy in a patient with alcoholic cirrhosis may resolve following liver transplantation and should not constitute a contraindication to transplantation, even when it is disabling. (Liver Transpl 2004;10:1545,1548.) [source] Peripheral synapses and giant neurons in whip spidersMICROSCOPY RESEARCH AND TECHNIQUE, Issue 4 2002Rainer Foelix Among invertebrates the synapses between neurons are generally restricted to ganglia, i.e., to the central nervous system (CNS). As an exception, synapses occur in the sensory nerves of arachnid legs, indicating that some nervous integration is already taking place far out in the periphery. In the antenniform legs of whip spiders (Amblypygi), a very special synaptic circuit is present. These highly modified legs contain several large interneurons (giant neurons) that receive mechanosensory input from 700,1,500 tarsal bristles. Some of the sensory cell axons contact a giant neuron at its short, branched dendrite, a few at the soma, but most synapse onto the long giant axon. The fine structure of these synapses resembles that of typical chemical synapses in other arthropods. Although thousands of sensory fibers converge on a single giant neuron, there is no reduction in the actual number of sensory fibers, because these afferent fibers continue their course to the CNS after having made several en passant synapses onto the giant neuron. Touching a single tarsal bristle is sufficient to elicit action potentials in a giant neuron. Owing to the large diameter of the giant axon (10,20 ,m), the action potentials reach the CNS within 55 ms, at conduction velocities of up to 7 m/s. However, mechanical stimulation of the tarsal bristles does not elicit a fast escape response, in contrast to giant fiber systems in earthworms, certain insects, and crayfishes. A quick escape is observed in whip spiders, but only after stimulation of the filiform hairs (trichobothria) on the regular walking legs. Although the giant fiber system in the antenniform legs undoubtedly provides a fast sensory pathway, its biological significance is not clearly understood at the moment. Microsc. Res. Tech. 58:272,282, 2002. © 2002 Wiley-Liss, Inc. [source] Electrophysiological studies in a mouse model of Schwartz,Jampel syndrome demonstrate muscle fiber hyperactivity of peripheral nerve originMUSCLE AND NERVE, Issue 1 2009Andoni Echaniz-Laguna MD Abstract Schwartz,Jampel syndrome (SJS) is an autosomal-recessive condition characterized by muscle stiffness and chondrodysplasia. It is due to loss-of-function hypomorphic mutations in the HSPG2 gene that encodes for perlecan, a proteoglycan secreted into the basement membrane. The origin of muscle stiffness in SJS is debated. To resolve this issue, we performed an electrophysiological investigation of an SJS mouse model with a missense mutation in the HSPG2 gene. Compound muscle action potential amplitudes, distal motor latencies, repetitive nerve stimulation tests, and sensory nerve conduction velocities of SJS mice were normal. On electromyography (EMG), neuromyotonic discharges, that is, bursts of motor unit action potentials firing at high rates (120,300 HZ), were constantly observed in SJS mice in all muscles, except in the diaphragm. Neuromyotonic discharges were not influenced by general anesthesia and disappeared with curare administration. They persisted after complete motor nerve section, terminating only with Wallerian degeneration. These results demonstrate that perlecan deficiency in SJS provokes a neuromyotonic syndrome. The findings further suggest a distal axonal localization of the generator of neuromyotonic discharges. SJS should now be considered as an inherited disorder with peripheral nerve hyperexcitability. Muscle Nerve, 2009 [source] Distal sensory nerve conduction of the superficial peroneal nerve: New method and its clinical applicationMUSCLE AND NERVE, Issue 5 2001Shin J. Oh MD Abstract The superficial peroneal nerve subserves sensation on the entire surface of the dorsum of the foot, except in small areas. All previously reported techniques for evaluating nerve conduction along this nerve tested a proximal portion of the nerve. We report a new method for evaluating sensory nerve conduction of the four branches of the distal superficial peroneal nerve. Two branches to the second and third toes of the medial dorsal cutaneous nerve and two branches to the fourth and fifth toes of the intermediate dorsal cutaneous nerve were studied orthodromically and antidromically in 37 feet of 21 normal volunteers using surface stimulating and recording electrodes and with a distance of 10 cm between the stimulating and recording electrodes. Maximum nerve conduction velocities (NCV) ranged from 41.8 to 46.9 m/s, and mean response amplitude ranged from 6.5 to 7.6 ,V with the orthodromic technique. Values for NCV were almost identical when elicited by antidromic and orthodromic techniques, but response amplitudes were higher with the antidromic technique. Mean amplitudes of the distal superficial peroneal nerve were about 50% of the proximal superficial peroneal, and the conduction velocity in the distal superficial peroneal was slower than that in the proximal superficial peroneal nerve, by 8,14 m/s. In seven cases, distal superficial peroneal neuropathy was confirmed with this technique: two with proper digital neuropathy, two with medial dorsal cutaneous neuropathy, and three with intermediate dorsal cutaneous neuropathy. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 689,694, 2001 [source] Origin and propagation of spontaneous excitation in smooth muscle of the guinea-pig urinary bladderTHE JOURNAL OF PHYSIOLOGY, Issue 2 2001Hikaru Hashitani 1The origin and propagation of waves of spontaneous excitation in bundles of smooth muscle of the guinea-pig bladder were examined using intracellular recording techniques and visualization of the changes in the intracellular calcium concentration ([Ca2+]i). 2Bladder smooth muscle cells exhibited spontaneous transient increases in [Ca2+]i which originated along a boundary of each smooth muscle bundle and then spread to the other boundary with a conduction velocity of 2.0 mm s,1. 3Spontaneous increases in [Ca2+]i were always preceded by action potentials. Nifedipine (10 ,M) abolished increases in both [Ca2+]i and action potentials. Caffeine (10 mM), ryanodine (50 ,M) and cyclopiazonic acid (10 ,M) reduced the amplitude of the associated increases in [Ca2+]i without preventing the generation of action potentials. 4Spontaneous action potentials had conduction velocities of 40 mm s,1 in the axial direction and 1.3 mm s,1 in the transverse direction. The electrical length constants of the bundles of muscle were 425 ,m in the axial direction and 12.5 ,m in the transverse direction. 5Neurobiotin, injected into an impaled smooth muscle cell, spread more readily to neighbouring cells located in the axial direction than those located in the transverse direction. The spread of neurobiotin was inhibited by 18,-glycyrrhetinic acid (18,-GA, 40 ,M), a gap junction blocker. 6Immunohistochemistry for Connexin 43 showed abundant punctate staining on the smooth muscle cell membranes. 7These results suggested that spontaneous action potentials and associated calcium waves occur almost simultaneously along the boundary of bladder smooth muscle bundles and then propagate to the other boundary probably through gap junctions. [source] Promotion of central nervous system remyelination by induced differentiation of oligodendrocyte precursor cells,ANNALS OF NEUROLOGY, Issue 3 2009Sha Mi PhD Objective Repair of demyelinated axons in diseases such as multiple sclerosis requires activation of the myelination program in existing or newly recruited oligodendrocyte precursor cells (OPCs). The control of OPC differentiation and initiation of myelination during repair is poorly understood. In this study, we test the ability of anti,LINGO-1 reagents to promote myelination in vitro and remyelination in the rodent adult central nervous system in vivo. Methods The effects of LINGO-1 antagonists on the differentiation of OPCs and the promotion of myelination has been assayed using a combination of coculture and slice culture preparations. Using three different animal models of demyelination and remyelination, we morphologically and functionally assessed the effects of LINGO-1 antagonists on OPC differentiation and myelin repair. Results The data indicate that in vitro treatment with antagonists of LINGO-1 promote OPC differentiation and myelination, whereas in vivo remyelination is accelerated in lysophosphatidylcholine- or cuprizone-induced demyelination. This remyelination is associated with enhanced OPC differentiation and functional recovery of conduction velocities in demyelinated axons. Interpretation Our studies demonstrate that LINGO-1 antagonism promotes OPC differentiation and remyelination, and suggest LINGO-1 functions as an inhibitor of OPC differentiation to retard central nervous system remyelination. Ann Neurol 2009;65:304,315 [source] Polyneuropathy associated with IgM vs IgG monoclonal gammopathy: comparison between clinical and electrophysiological findingsACTA NEUROLOGICA SCANDINAVICA, Issue 1 2010M. Vrethem Vrethem M, Reiser N, Lauermann C, Svanborg E. Polyneuropathy associated with IgM vs IgG monoclonal gammopathy: comparison between clinical and electrophysiological findings. Acta Neurol Scand: 2010: 122: 52,57. © 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objective,,, The neuropathy associated with IgM monoclonal gammopathy (IgM-MG) is regarded as a sensorimotor, mainly demyelinating neuropathy. It is not fully known whether the neuropathy in IgG-MG is caused by the same mechanisms and shows the same electrophysiological characteristics. We aimed at making a comparison between clinical and neurophysiological findings in these two conditions. Patients and methods,,, Twenty-seven patients with IgM-associated neuropathy [18 with anti-myelin-associated glycoprotein (anti-MAG) antibodies] were compared with 15 age-matched patients with IgG-associated neuropathy. Results,,, Patients with IgM-associated neuropathy (especially those with anti-MAG antibodies) had significantly clinically more severe disabilities with involvement of both motor and sensory functions compared with patients with IgG-associated neuropathy in whom clinical sensory disturbances were more prominent than motor dysfunction. Motor and sensory conduction velocities were significantly lower and distal latencies significantly longer in the IgM group than in the IgG group concerning the median, ulnar and peroneal nerves. Fifty-four per cent of the patients in the IgM group did not present a sensory response of the median nerve vs 13% in the IgG group. There was also a significant difference concerning absent responses from the peroneal and sural nerves in the IgM vs IgG group (peroneal: 48% vs 13%, sural: 88% vs 27%). Conclusion,,, Polyneuropathy associated with IgM-MG, especially when associated with anti-MAG antibodies, appears to have more of a demyelinating involvement that meets the criteria for demyelination. This was not as clear in those associated with IgG. The IgG neuropathy showed less and milder deficit in the electrophysiological studies. [source] C-peptide: new findings and therapeutic implications in diabetesCLINICAL PHYSIOLOGY AND FUNCTIONAL IMAGING, Issue 4 2004John Wahren Summary In contrast to earlier views, new data indicate that proinsulin C-peptide exerts important physiological effects and shows the characteristics of an endogenous peptide hormone. C-peptide in nanomolar concentrations binds specifically to cell membranes, probably to a G-protein coupled receptor. Ca2+ - and MAP-kinase dependent signalling pathways are activated, resulting in stimulation of Na+, K+ -ATPase and endothelial nitric oxide (NO) synthase, two enzyme systems known to be deficient in diabetes. C-peptide may also interact synergistically with insulin signal transduction. Studies in intact animals and in patients with type 1 diabetes have demonstrated multifaceted effects. Thus, C-peptide administration in streptozotocin-diabetic animals results in normalization of diabetes-induced glomerular hyperfiltration, reduction of urinary albumin excretion and diminished glomerular expansion. The former two effects have also been observed in type 1 diabetes patients given C-peptide in replacement dose for up to 3 months. Peripheral nerve function and structure are likewise influenced by C-peptide administration; sensory and motor nerve conduction velocities increase and nerve structural changes are diminished or reversed in diabetic rats. In patients with type 1 diabetes, beneficial effects have been demonstrated on sensory nerve conduction velocity, vibration perception and autonomic nerve function. C-peptide also augments blood flow in several tissues in type 1 diabetes via its stimulation of endothelial NO release, emphasizing a role for C-peptide in maintaining vascular homeostasis. Continued research is needed to establish whether, among the hormones from the islets of Langerhans, C-peptide is the ugly duckling that , nearly 40 years after its discovery , may prove to be an endogenous peptide hormone of importance in the treatment of diabetic long-term complications. [source] | |||||||||||||||||||||||||||||||||||||