Neurogenic Involvement Research Articles

Motor unit properties do not correlate between MUNIX and needle EMG in remote polio in the biceps brachii muscle

ObjectiveTo compare the utility of MUNIX (motor unit number index) with needle EMG in characterizing motor unit (MU) properties in the biceps brachii (BB) muscle in subjects with remote polio. MethodsThirty subjects suffering from remote polio were investigated with MUNIX and needle EMG, all with Macro EMG and 16 of these subjects with concentric needle EMG. ResultsBoth MUNIX and the needle EMG methods showed abnormal results. Fiber density (FD) was the most sensitive parameter for showing signs of reinnervation. At a group level, the methods showed neurogenic findings, but there was no correlation between the results of the MUNIX and needle EMG investigations. ConclusionsBoth MUNIX and needle EMG are valuable methods for measuring neurogenic involvement in the BB muscle. However, there was a lack of correlation between the MUNIX and needle EMG findings. The cause for this missing correlation may be multifactorial as there are several differences between the methods. SignificanceThe reason for the lack of correlation between the MUNIX and needle EMG results is discussed. By combining the needle and surface recorded methods one can obtain more information on the denervation and reinnervation process compared to using just one of the methods alone.

A study on etiology of incontinence in double knockout mouse model

Stress urinary incontinence is of concern in both pediatric and adult population. Double mutant GLI family zinc finger Gli2+/-; Gli3Δ699/+ murine model of stress incontinence has been recently developed as a reliable model which does not require surgical manipulation to create incontinence and is shown to survive to adulthood. The aim of this study was to establish the etiology of incontinence in the double mutant Gli2+/-; Gli3Δ699/+ mice. We used 13 cluster of differentiation 1 (CD-1) mice (7-9 weeks) for demonstration of histology of the bladder and urethra. There were 3 Wild Gli2+/- females, 2 Wild Gli2+/- males, 4 Gli2+/-;Gli3Δ699/+ females and 4 Gli2+/-;Gli3Δ699/+ males. The Wild Gli2+/- mice served as the control group and Gli2+/-;Gli3Δ699/+ mice served as the test group. Additionally, eight 16.5 days mice (2 each of Wild Gli2+/- females, Wild Gli2+/- males, double knockout (DKO) Gli2+/-;Gli3Δ699/+ females and Gli2+/-;Gli3Δ699/+ males) were used to assess the histology of the spinal cord. The gross appearance of bladder and urethra was studied using ink injection assays. Immunohistochemistry was done for smooth muscle actin and cytokeratin. Gross and histologic appearance confirmed the previously reported widening of bladder outlet and hypoplasia of smooth muscles in female urethra and also established them in the male urethra of Gli2+/-;Gli3Δ699/+ mice compared to Gli2+/- mice. The double knockout mice were smaller than the Gli2 mice (5.2 vs 6.1cm, p=0.002). Immunohistochemistry demonstrated epithelial hyperplasia and smooth muscle hypoplasia. Additionally, there was prostatic hypoplasia in the Gli2+/-;Gli3Δ699/+ male mice. The spinal cord length for body size appeared comparable between the Gli2+/- and Gli2+/-;Gli3Δ699/+ mice but histological evaluation revealed abnormal development of the caudal end of the vertebral body with premature termination of the spinal cord (Figure). The histological changes in the bladder neck and urethra were consistent to those previously reported. While previous report described the findings in female mice only, we confirmed that these findings are also present in males as well as prostatic hypoplasia, a possible additional factor leading to stress incontinence. The most important finding in the present study however, was the detection of premature termination of spinal cord in the DKO Gli2+/-; Gli3Δ699/+ mice which has not been reported previously and is likely a major contributor to incontinence in this model. The incontinence in male as well as female Gli2+/-; Gli3Δ699/+ mice is due to both myogenic and neurogenic involvement. These double knockout mice are a valuable model of stress incontinence related to neurogenic bladder due to low outlet resistance.

Autophagic vacuolar myopathy involving the phenotype of spinocerebellar ataxia type 3.

Spinocerebellar ataxia type 3 (SCA3) is a form of autosomal dominant cerebellar ataxia with a wide range of clinical manifestations, including ataxia and pyramidal and extrapyramidal signs. A few SCA3 patients have been noticed to be predisposed to the development of inclusion body myositis. It is still unknown whether muscle can be primarily involved in the pathogenesis of SCA3. This study reported an SCA3 family in which the index patient initially presented with parkinsonism, sensory ataxia, and distal myopathy but the absence of cerebellar and pyramidal symptoms. The clinical and electrophysiological studies implied a possible combination of distal myopathy and sensory-motor neuropathy or neuronopathy. MRI muscle showed selective fat infiltration and absence of denervated edema-like changes, indicating the distal muscle weakness had a myopathic origin. Muscle pathology showed the myopathic involvement, besides neurogenic involvement, characterized by chronic myopathic changes with multiple autophagic vacuoles. Genetic screening revealed expanded CAG of 61 repeats in the ATXN3 gene, which showed co-segregation in the family. Besides the neurogenic origin, the myopathic origin may be partly attributed to the limb weakness of SCA3 patients, which expands the spectrum of the clinical manifestation of SCA3.

Man-in-the-Barrel Syndrome as an Atypical form of Amyotrophic Lateral Sclerosis

TMF, 58 years old, retired, reports that for approximately two years she has been showing amyotrophy, fasciculations and paresis in the upper limbs and pectoral girdle (Figures 1-2). Lower limbs with muscle strength grading 4, in addition to live reflexes. The electroneuromyography demonstrated a pre-ganglionic\purely motor chronic peripheral neurogenic involvement in the four limbs without conduction block. After excluding other possible diagnoses, it is an atypical case of Amyotrophic Lateral Sclerosis presenting as “Barrel Man” syndrome. Such nomenclature refers to a picture of bilateral brachial paresis that disables patients in basic and instrumental activities of daily life that requires elevation of the upper limbs. Since its description, there are some cases of patients with this syndrome, of varying etiologies (Figure 1 & 2).

A case of overlapping adult‐onset linear scleroderma and Parry‐Romberg syndrome presenting with widespread ipsilateral neurogenic involvement

Linear scleroderma is a variant of localized scleroderma. We report a 43-year-old woman who had developed left arm weakness and linear scleroderma on her back during pregnancy at 25 years of age, followed by left hemifacial atrophy and left leg weakness. She had multiple linear scleroderma lesions on her trunk and left limbs, left eyelid ptosis, impairment of vertical movement and abduction of the left eye, left hemifacial atrophy, and weakness and atrophy of the sternocleidomastoid, trapezius, and proximal limb muscles on the left side. On serology, antibodies to U1-ribonucleoprotein and Jo-1 were positive; anti-scleroderma-70 antibody was negative. Skin biopsy demonstrated increased hypertrophic collagen fibers without inflammatory infiltrates. Needle electromyography of left limb muscles revealed mild neurogenic patterns; left quadriceps muscle biopsy showed chronic neurogenic changes. Brain magnetic resonance imaging revealed mild left hemispheric atrophy. This is a rare case of linear scleroderma and Parry-Romberg syndrome presenting with widespread ipsilateral neurogenic manifestations.

New features for scanned bioelectrical activity of motor unit in health and disease

Abstract The present study aims to find new features that support the differential diagnosis of neuromuscular diseases. Scanning EMG is an experimental method developed for understanding the motor unit organization and for observing temporal and spatial characteristics of motor unit’s electrical activity. A motor unit consists of a motor neuron and muscle fibers that are innervated by its motor neuron. Both simulation and biological data on neuromuscular diseases are considered in this study. Biological data were acquired from 3 patients with neurogenic involvement (2 with poliomyelitis sequela and 1 with spinal muscular atrophy), 2 patients with myopathy (1 with inflammatory myopathy and 1 with muscular dystrophy) and 4 healthy participants. Seven features are extracted by specifications of neuromuscular diseases and characteristics of EMG signals. These features are maximum amplitude, spike duration, the number of peaks, maximum amplitude x spike duration, number of peaks x spike duration, the ratio of the power outside the activity corridor to the power inside the activity corridor and the number of peaks outside of the activity corridor. The autocorrelation function of the sum of scanning EMG signals is effective in determining the activity corridor of these signals and the spike duration can be determined more easily by using the activity corridor. Wavelet transform based noise reduction and the windowing method are proposed for calculating the features correctly. By this method, spike duration and the number of peaks should be able to be calculated more precisely. It is confirmed that if the signals are filtered by a high pass filter with a cut off frequency of 2 KHz, the calculation of the number of peaks should be easier. While maximum amplitude and maximum amplitude times spike duration are found to be significant for diagnosing neurogenic diseases, other features are found to be significant for all groups by ANOVA test. It is determined that which features are more effective for differential diagnosis and the dataset that contains normal people and patients is classified using multi-layer perceptron (MLP), radial basis function network (RBF), support vector machines (SVM) and k nearest neighbor algorithm (k-NN). The best accuracy is obtained as 85% with MLP network.

Effects of neuregulin-1 administration on neurogenesis in the adult mouse hippocampus and characterization of immature neurons along the septotemporal axis

Adult hippocampal neurogenesis is associated with learning and affective behavioural regulation. Its diverse functionality is segregated along the septotemporal axis from the dorsal to ventral hippocampus. However, features distinguishing immature neurons in these regions have yet to be characterized. Additionally, although we have shown that administration of the neurotrophic factor neuregulin-1 (NRG1) selectively increases proliferation and overall neurogenesis in the mouse ventral dentate gyrus (DG), likely through ErbB3, NRG1’s effects on intermediate neurogenic stages in immature neurons are unknown. We examined whether NRG1 administration increases DG ErbB3 phosphorylation. We labeled adultborn cells using BrdU, then administered NRG1 to examine in vivo neurogenic effects on immature neurons with respect to cell survival, morphology, and synaptogenesis. We also characterized features of immature neurons along the septotemporal axis. We found that neurogenic effects of NRG1 are temporally and subregionally specific to proliferation in the ventral DG. Particular morphological features differentiate immature neurons in the dorsal and ventral DG, and cytogenesis differed between these regions. Finally, we identified synaptic heterogeneity surrounding the granule cell layer. These results indicate neurogenic involvement of NRG1-induced antidepressant-like behaviour is particularly associated with increased ventral DG cell proliferation, and identify novel distinctions between dorsal and ventral hippocampal neurogenic development.

The effect of recording site on extracted features of motor unit action potential

Motor unit action potential (MUAP), which consists of individual muscle fiber action potentials (MFAPs), represents the electrical activity of the motor unit. The values of the MUAP features are changed by denervation and reinnervation in neurogenic involvement as well as muscle fiber loss with increased diameter variability in myopathic diseases. The present study is designed to investigate how increased muscle fiber diameter variability affects MUAP parameters in simulated motor units. In order to detect this variation, simulated MUAPs were calculated both at the innervation zone where the MFAPs are more synchronized, and near the tendon, where they show increased temporal dispersion. Reinnervation in neurogenic state increases MUAP amplitude for the recordings at both the innervation zone and near the tendon. However, MUAP duration and the number of peaks significantly increased in a case of myopathy for recordings near the tendon. Furthermore, of the new features, “number of peaks×spike duration” was found as the strongest indicator of MFAP dispersion in myopathy. MUAPs were also recorded from healthy participants in order to investigate the biological counterpart of the simulation data. MUAPs which were recorded near to tendon revealed significantly prolonged duration and decreased amplitude. Although the number of peaks was increased by moving the needle near to tendon, this was not significant.

Investigation of Neurologic Complications in Closed Wedge High Tibial Valgization Osteotomy Technique by Using Electrophysiological Methods

Objective: The aim of this study is to investigate neurologic complications of closed wedge valgization osteotomy technique using electrophysiological methods. Methods: Eleven extremities of 11 patients (mean age 52.75±5.39) with medial gonarthrosis and varus deformity were included. All patients underwent closed wedge valgization osteotomy in order to correct varus deformity. Results: The post-operative amplitude of nervus peroneus superficialis significantly decreased when compared to pre-operative amplitude. The mean pre-operative N. peroneus superficialis amplitude was 12.77, whereas it reduced to 5.44 postoperatively (p<0.05). Other nerve conduction velocity investigations showed no significant differences between preand post-operative values. A Needle Electromyography (EMG) study demonstrated neurogenic involvement in 4 patients after surgery. All of these 4 patients had denervation in the m.extensor hallucis longus needle EMG study. In 2 patients, the musculus tibialis anterior needle EMG study showed neurogenic involvement. One of these had denervation. One patient showed denervation in the m.extensor digitorum brevis needle EMG study. Conclusion: Although fibula osteotomy used in the closed wedge osteotomy technique was applied from the safe zone, electrophysiological investigations suggested that this zone may, in fact, not be safe. (JAREM 2014; 1: 12-7)

Colon transit time and anorectal manometry in children and young adults with spina bifida

This study evaluates colon transit time (CTT) and anorectal manometry (ARM) in spina bifida (SB) patients in relation to the level of lesion, mobility, constipation, and continence status. SB patients between 6 and 19 years, who are not using antegrade continence enemas and followed at the SB Reference Centre UZ Ghent, were asked to participate. Medical history was retrospectively retrieved from the medical file. Stool habits were prospectively collected using standardized questionnaires. CTT was measured using the 6-day pellet abdominal X-ray method. ARM was performed in non-sedated children with a water-perfused, latex-free catheter. Forty out of 52 eligible patients consented to perform CTT, of which 19 also performed the ARM. Fifteen (37 %) SB patients were constipated despite treatment. Twenty-six (65 %) were (pseudo) continent. The total CTT was significantly prolonged in SB patients (median CTT 86.4 vs. 36 h controls). The CTT was significantly prolonged in constipated SB patients compared to non-constipated SB patients (122.4 vs. 52.8 h). Spontaneously continent patients had a normal CTT (33.6 h) as well as a significantly higher resting pressure compared to the pseudo-continent and incontinent SB patients (resting pressure 56.5 vs. 32.5 mmHg). An abnormal CTT was associated with a treatment necessity to achieve pseudo-continence (p = 0.006). CTT in SB patients was significantly prolonged, indicating a neurogenic involvement of the bowel and slow transit constipation. SB patients with a normal CTT and a normal ARM spontaneously achieved fecal continence. CTT can help tailor the continence therapy in SB patients.

Clinical profile and molecular diagnosis in patients of facioscapulohumeral dystrophy from Indian subcontinent

Facioscapulohumeral dystrophy (FSHD) is an autosomal dominant muscular dystrophy. We retrospectively studied three families (two Indian, one Nepalese) with 12 affected members (male:female-7:5). Mean age at onset of weakness was 17.63 + 5.48 years. Patients were classified according to muscle groups affected (F-face, S-scapula, H-humeral, PG-pelvic girdle, P-peroneal, A-loss of independent ambulation): FSH-A (2), four FSH (4), SH (3), FSH-PG (2) and one: F (1). Progression of weakness was classified as F>S>P>PG in eight cases, S> F>P in one, static in three. Eleven patients had electromyographic findings suggestive of myopathy and one had features of neurogenic involvement. Molecular diagnosis was done by southern blotting using probe p13E-11 after digestion of genomic DNA with EcoRI and/or EcoRI/BlnI for twelve patients and three unaffected relatives. No EcoRI fragment smaller than 35 Kb was seen in unaffected subjects. Size of EcoRI fragment varied between 17 kb to 27 kb in affected subjects and was constant for affected members of the same family. Molecular diagnosis by southern blotting has helped to provide genetic counseling for the families.

Une amylose finlandaise chez une patiente française

Finnish amyloid variety is a rare familial amiloidosis polyneuropathy essentially observed in Finland. It concerns about six hundred people in the world in which five hundred reside in Finland. We report a case of a 58-year-old French woman with a 10-year history of lattice cornea dystrophy. She consulted in January 2004 for impaired swallowing, facial paralysis principally of the right superior territory and symptoms of arthritis which had developed a few months earlier. Observation revealed facial cutis laxa, tongue amyotrophy and some fasciculation. Electroneuromyography showed chronic neurogenic involvement of the facial muscles. Limbs and the sympathetic neuronal system were free of involvement. Pathological examination revealed areas of peri vascular amiloid deposits. Molecular biology confirmed the diagnosis of Finnish amiloidosis: substitution of aspartic acid by tyrosine in the 187 codon in the 9th chromosome (gelsoline gene). This mutation has been previously found in Denmark and the Czech Republic. Finnish amiloidosis is a familial polyneuropathy characterized by an association of cornea lattice dystrophy, cutis laxa and a chronic neurogenic involvement of the cranial nerves. Two mutations are known. Life expectancy is not affected, but quality of life is altered.

Topical capsaicin application and axon reflex vasodilatation of the tongue: A videocapillaroscopic study

The aim of this study was to evaluate the effects of topical capsaicin application on human lingual mucosa and to assess if neurogenic inflammation might have a role in the pathogenesis of lingual diseases. Thirty patients (16 males and 14 females; mean age: 41.46 +/- 11.8 years; range: 23-60) were examined in our laboratory. The neurogenic inflammation was experimentally induced in the lingual mucosa close to 1) the left margin of the tongue and 2) the right margin of the tongue after ipsilateral nerve trunk anesthesia. The characteristics of lingual microcirculation were observed using computerized videocapillaroscopic techniques. The vasodilatation was observed close to the left margin of the tongue, and an important correlation was observed between capsaicin application and capillary tortuosity. No significant modifications in vascular diameter and tortuosity were observed after capsaicin application close to the right margin of the tongue. The results of the present study are of interest, in particular, in regard to recent studies pointing to neurogenic involvement in oral diseases such as burning mouth syndrome.

An uncommon presentation of hexosaminidase deficiency

Focal muscular atrophy (FMA) can occur due to several causes. We report three cases of FMA associated with deficiency of hexosaminidase A. The serum level of hexosaminidase A was assayed in seven patients with FMA without any definite aetiology identified over a period of two years. Three cases of FMA showed deficiency of hexosaminidase A. All these patients had clinical features of isolated lower motor neurone involvement in one limb without any evidence of involvement of the rest of the neuraxis. Detailed laboratory tests were negative. Electromyography confirmed neurogenic involvement without any evidence of radiculopathy or neuropathy. Hexosaminidase deficiency as a possible association for FMA is highlighted.

Variation in the Expression of Inflammatory Markers and Neuroreceptors in Human Conjunctival Epithelial Cells

The purpose of this research was to investigate the role of neurogenic involvement in the etiopathogenesis of ocular surface inflammation, with the final goal of identifying new potential anti-inflammatory agents. We describe the presence of two “classic” markers of inflammation (HLA-DR and ICAM-1) and some neuroreceptors in cultured human conjunctival epithelial cells under basal and pro-inflammatory conditions. Two markers of inflammation (HLA-DR, ICAM-1) and several neuroreceptor subtypes (M1-, M2-, and M3-muscarinic; α1A-, α1B-, α1D-, α2A-, α2B-, α2C-, β1-, β2-, and β3-adrenergic) were analyzed in a normal human conjunctival epithelial cell line (IOBA-NHC). These markers were studied in basal conditions and under the influence of two pro-inflammatory cytokines: tumor necrosis factor alpha (TNF-α) and/or interferon gamma (IFN-γ). Immuno-fluorescence (confocal microscopy), western blotting, or flow cytometry techniques were used. In basal conditions, epithelial cells expressed all inflammatory markers except HLA-DR. The addition of IFN-γ enhanced expression of HLA-DR, ICAM-1, and M2-muscarinic receptor. TNF-α up-regulated the expression of ICAM-1. When epithelial cells were incubated in the presence of both cytokines together, the cell surface expression of HLA-DR, ICAM-1, α1B-, and α2B-adrenergic receptors was increased. Cultured human conjunctival epithelial cells have been shown to be susceptible to up-regulation of the expression of inflammatory markers and cell membrane expression of some neuroreceptors under pro-inflammatory conditions. Consequently, pharmacologic neuromodulation could have a role in the comprehensive management of ocular surface inflammation.

Shoulder function after accessory nerve-sparing neck dissections.

This study was designed to observe the effect of preserving the spinal accessory nerve (SAN) during neck dissection (ND) and adjuvant radiotherapy (ART) after ND on shoulder function. Fifty-seven patients with head and neck cancer who had undergone primary tumor resection and various types of NDs were enrolled in this prospective study. Postoperative shoulder joint range of motion was evaluated by goniometry, and muscle strength was measured manually. SAN function was evaluated with electromyography (EMG) with respect to percentage of denervation and presence of neurogenic involvement. Patients were grouped by treatment as follows: radical ND (RND) versus modified radical ND (MRND)/selective ND (SND) and ART versus no ART. Shoulder joint range of motion and shoulder muscle strength were significantly better in the MRND/SND group than in the RND group. However, EMG findings were similar in the RND and MRND/SND groups. When all patients who underwent ND, RND, or MRND/SND were compared with the control group, statistically significant changes in shoulder joint range of motion and shoulder muscle strength were found. Also, denervation and neurogenic involvement of the SAN were significantly higher after all NDs than in the control group. ART did not affect range of motion of the shoulder joint, shoulder muscle strength, or the degree of denervation and neurogenic involvement in any of the ND groups. ART does not have a negative effect on shoulder function after ND. SAN is always functionally impaired even if we preserve it macroscopically during ND.

Effectiveness of Neural Mobilization in the Treatment of a Patient with Lower Extremity Neurogenic Pain: A Single-Case Design

It is postulated that neural tissue mechanosensitivity contributes to symptoms associated with peripheral neurogenic pain disorders. However, there is a paucity of literature regarding the most effective clinical practices for managing pain of peripheral neurogenic origin. As clinical use of neural mobilization continues to flourish in the management of these pain syndromes, it is imperative to document outcomes associated with these techniques. The purpose of this single-case A1-B1-A2-B2 design was to investigate the effectiveness of neural mobilization in the management of a 29-year-old female patient with symptoms suggestive of peripheral neurogenic involvement. The intervention phases (B1 and B2) consisted of neural mobilizations specifically directed at the sciatic continuum. Outcome measures (degrees of hip flexion during the straight-leg-raise and pain) demonstrated both visual improvement and statistically significant improvements following implementation of the neural mobilization techniques. This single-case design provides a measure of scientific support for the use of neural mobilizations with patients presenting with lower extremity neurogenic pain disorders. However, generalizability is poor, and further methodologically sound clinical trials are necessary to investigate the effectiveness of neural mobilization in a larger patient population.

Neurokinin 1-receptors and sensory neuropeptides in tendon insertions at the medial and lateral epicondyles of the humerus: Studies on tennis elbow and medial epicondylalgia

There is no information on the sensory innervation at the flexor muscle origin at the medial epicondyle of the humerus and it is not known if substance P receptors (Neurokinin 1-receptors, NK1-R) are present in tendon insertions in general. In the present investigation, we have studied the muscle origin in patients suffering from medial epicondylalgia and tennis elbow. Immunohistochemistry and antibodies to substance P (SP) and CGRP as well as the general nerve marker PGP 9.5 were used. Specific immunoreactions were observed in nerve bundles and as free nerve fibers. The immunoreactive structures were partly seen in association with some of the blood vessels. The observations constitute a morphological correlate for the occurrence of nerve mediated effects in this region. By using immunohistochemistry and antibodies to NK1-R, the distribution of this receptor was studied at the insertion of the proximal tendon of the extensor carpi radialis brevis muscle at the lateral epicondyle. Specific immunoreactions were seen as varicose fibers occurring as single fibers or grouped into bundles, indicating that SP has effects in the nerves in this region. The results give further evidence for a possible neurogenic involvement in the pathophysiology of tennis elbow and in medial epicondylalgia.

Neuromuscular dysfunction in adult growth hormone deficiency.

Adult growth hormone deficiency (AGHD) is associated with fatigue, tiredness and myalgias, which improve after initiating recombinant human GH (rhGH) therapy. To conduct an extensive neuromuscular investigation of patients with AGHD in an attempt to explain their neuromuscular symptoms. Twenty adult patients (11 males) with untreated GHD of whom 10 were childhood-onset (CO) underwent a prospective neurological protocol, including physical examination and a neurophysiological study that comprised sensory and motor neurography, repetitive stimulation, electromyogram (EMG) and interference pattern analysis (IPA). In the first seven patients (four CO), a biceps muscle biopsy was also performed for histochemical analysis and Western blot, and investigation of signal transducers and activators of transcription (STATs)-1 and -3 and the two isoforms STAT-5a and -5b. Neuromuscular examination, sensory and motor neurography and repetitive stimulation were normal in 20/20 patients. Fourteen [seven CO and seven adult-onset (AO)] of the 20 patients had abnormal EMG and/or IPA suggestive of a neurogenic involvement. In those seven patients with initially abnormal results, who also remained on regular rhGH for at least 1 year, repeated IPA was normal in six and improved in the remaining patient (P=0.004). The biceps muscle biopsy disclosed abnormal groupings in the seven cases tested, indicative of a neurogenic pattern. No changes in skeletal muscle STAT-1 and -3 were seen compared to controls, but a marked increase in both STAT-5 isoforms was observed in all seven patients. Skeletal muscle of patients with both adult-onset and childhood-onset adult GH deficiency shows a neuromuscular dysfunction, indicated by the muscle biopsy and the neurophysiological study, which in the subgroup of treated patients responds positively to rhGH therapy. The results obtained suggest that the STAT-5 signal transduction pathway in muscle is abnormal in adult GH deficiency.

Micturition and the Sacral Reflex Arc: Lessons from Electrophysiological Techniques

Electrophysiological techniques applied either for diagnostic or therapeutic reasons have contributed to elucidation of the neurocontrol of micturition. But the ability of electrophysiological diagnostic tests to reveal neurogenic involvement in patients suffering from lower urinary tract dysfunction is limited, and has been overestimated. Furthermore, the correlation between a proven neurogenic deficit and the consecutive dysfunction is complex. The electrophysiological tests, however, may also be applied for testing the central nervous system integrative function, such as in testing reflex thresholds, excitability levels of motor nuclei, etc. The use of electrical stimulation for therapeutic purposes has instigated much physiological research. Insights related to micturition neurocontrol, as for instance that afferent inputs from lumbosacral and vesical sensory fibres modulate the micturition threshold, are being therapeutically exploited. It is expected that further use of electrophysiological techniques, being complementary to functional neuroimaging, will clarify particularly the temporal aspects of neurocontrol mechanisms involved in micturition.