Place of perineal electrophysiologic testing in multiple sclerosis patients.

At present, apart from visual evoked potential testing, clinical neurophysiological testing is rarely used in multiple sclerosis (MS) patients at all. In MS patients with lower urinary tract, bowel and/or sexual dysfunction, which are highly prevalent in MS, clinical neurophysiological tests of the sacral nervous system have been introduced in the past and reported as abnormal. Their usefulness is--in selected patients--to substantiate the suspicion of the neurogenic nature of the pelvic organ dysfunction, but there is no specific recommendation for MS patients. Concentric needle EMG of the perineal muscles, bulbocavernosus reflex latency measurement and pudendal SEP may be particularly useful in those rare patients in whom MS may affect the conus medullaris, to demonstrate "directly" this lesion of the lower sacral segments. Clinical neurophysiological testing retains the potential to further clarify interesting research questions of correlation of nervous system function and pelvic organ dysfunction, particularly relevant for application of sophisticated new rehabilitative methods including electrical stimulation of the nervous system.

[Sexual disorders from the perspective of the neurologist]. / Sexuelle Störungen aus der Sicht des Neurologen.

The temporal lobes and other cortical regions are involved in the emotional, cognitive and sensorimotor aspects of central control of sexual behaviour, whereas the insula, anterior cingulate and hypothalamus coordinate the activation of the autonomic nervous system. Facilitatory and inhibitory descending pathways modify the activity of thoracolumbar sympathetic and sacral parasympathetic and somatic spinal centres which control the sexual response, i.e. arousal, orgasm and ejaculation. A normal hormonal milieu and vascular system, but also a healthy psychosocial context, are necessary for a fulfilled sexual life. Sexual dysfunction is not uncommon in the general population, particularly in the elderly; it is more common in neurological patients. It significantly lowers their quality of life. The neurologist should diagnose sexual dysfunction in his patients and provide basic management including explanation, suggestions and--if necessary--drugs.

One Europe, one neurologist?

In recent years, there has been a major shift in emphasis within neurology from being a largely diagnostic discipline to one much more actively involved in treating disease. There have been major scientific advances leading to new and effective treatments. There is also a much greater awareness of the burden of neurological disease (Olesen J, Leonardi M. European Journal of Neurology 2003; 10: 471) and informed sufferers are requesting specific intervention. There is wide variation in the delivery of neurological services throughout Europe. This is reflected in manpower levels, the place of neurology related to other medical specialties and different mixes of hospital and private office practice. These differences have been thrown into sharper focus by the recent expansion of the European Union (EU). Initial training in neurology is given to undergraduate/pre-graduate students. Post-graduate education is delivered within a residency program leading to specialist qualification and certification. We now recognize that this is only the beginning of a life long program of continuous education and development (CME/CPD). National and international exchange programs facilitate the growth of knowledge and promote professional harmony and cooperation. The free migration of medical specialists has been an aspiration but remains limited by cultural, linguistic, personal, professional, political and economic factors. Two bodies, the European Board of Neurology (EBN-UEMS) http://www.uems-neuroboard.org (Union Européenne des Médecins Spécialistes) and the European Federation of Neurological Societies (EFNS) http://www.efns.org are actively involved in harmonising and developing neurology at the European level.

Mini-Mental State Examination: standardization and validation for the elderly Slovenian population.

For more than two decades Mini-Mental State Examination (MMSE) has been adapted to the Slovenian language as 'Kratek preizkus spoznavnih sposobnosti' (KPSS). In this study, we evaluated the influences of age and education on the KPSS score, looking for the cut-off point with the optimal ratio of sensitivity (SE) and specificity (SP) to support the use of the KPSS as a screening tool. During the years 2000-03 we examined 258 Slovenian volunteers. Volunteers were divided in two groups based on clinical criteria. A total of 189 were healthy, aged from 45 to 96 years, 69 were demented patients aged from 46 to 91 years, of both sexes, all different levels of education and different degrees of dementia. Median value, SE, SP, positive predictive power and negative predictive power were calculated at cut-off points 23/24, 24/25, 25/26 and 26/27. Younger age and higher education (at least 10 years of education) were each associated with higher KPSS scores. The Slovenian modification of the MMSE demonstrates an optimal cut-off score at 25/26 points for screening dementia in the Slovenian population, due to the best SP (75%)/SE (73%) ratio. The cut-off level 26/27 is recommended for screening highly educated persons.

How to diagnose MSA early: the role of sphincter EMG.

Multiple system atrophy (MSA) is a degenerative disease manifesting a combination of parkinsonism, cerebellar, pyramidal, and autonomic (including urinary, sexual and anorectal) dysfunction. It is pathomorphologically defined, but lacks a definitive clinical diagnostic test. In patients with probable MSA, abnormal sphincter EMG, as compared to control subjects, has been found in the majority of patients in all the different forms of the disease in most studies, including patients who, as yet, have no urological or anorectal problems. Patients with Parkinson's disease (PD) as a rule do not show marked sphincter EMG abnormalities in the first five years of the disease. Thus, abnormal spontaneous activity or marked motor unit potential changes in sphincter muscles are helpful in distinguishing MSA from PD in the first five years after the onset of symptoms and signs, and from pure autonomic failure, as well as from cerebellar ataxias, if other causes for sphincter denervation have been ruled out. EMG does not distinguish MSA from progressive supranuclear palsy. How early in the course of MSA these abnormalities become significant enough to support diagnosis remains to be established by prospective studies.

A historical perspective on cerebral palsy as a concept and a diagnosis.

Sigmund Freud was the first to write about cerebral palsy as a nosographic category, uniting various infantile motor deficits of brain origin. He did not ascribe more than a temporary value to the term (infantile) cerebral palsy, but it has become--and still is--an indispensable part of the nosographic system. Nevertheless, it is still easier to explain what cerebral palsy is not than to define it precisely.

Sexual function in men with cauda equina lesions: a clinical and electromyographic study.

OBJECTIVE: To investigate the effects of cauda equina lesions on sexual function in men. METHODS: Sexual function was investigated in 46 men with long standing cauda equina/conus medullaris lesions. All had clinical and radiological findings supporting the diagnosis. The validated Slovene translation of the international index of erectile function (IIEF) was used. The responses were scored and sexual dysfunction categorised as absent, mild, moderate, or severe. The number of patients receiving help for sexual dysfunction was noted. Neurological examination of the trunk and lower limbs, electromyographic (EMG) evaluation of the sacral reflex, and quantitative EMG of the external anal sphincter muscles were done. RESULTS: Severe sexual dysfunction was reported by 35% of patients, moderate dysfunction by 24%, and slight dysfunction by 26%; normal sexual function was reported by 15%. Orgasmic function was slightly more impaired than erectile function, and sexual desire slightly less. The patients' age, but no findings on clinical neurological or EMG examination, correlated with sexual function. Only five men had received medical attention for sexual dysfunction. CONCLUSIONS: There is significant sexual impairment in men with lesions of the cauda equina or conus medullaris. This is poorly correlated with neurological and EMG findings and has received insufficient medical attention.

Protocol for clinical neurophysiologic examination of the pelvic floor.

Clinical neurophysiologic examination of the pelvic floor is performed worldwide, but there is no consensus on the choice of tests, nor on technical details of individual methods. Standardized methods are, however, necessary to obtain their valid application in different laboratories for the purpose of collection of normative data, comparison of patient data and organization of multi-center studies. It is proposed that in patients with suspected "lower motor neuron" type lesions concentric needle electromyography (CNEMG) is the most informative test to detect pelvic floor denervation/reinnervation, and the external anal sphincter (EAS) muscle is the most appropriate muscle to be examined (either in isolation-when a selective lesion is suspected-or in addition to examination of other muscles). An algorithm consisting of standardized tests including a standardized approach to CNEMG examination of the EAS is presented. The proposed electrophysiologic assessment consists of a computer-assisted analysis of denervation and reinnervation features of the CNEMG signal, a qualitative assessment of reflex and voluntary activation of EAS motor units, and of electrical (or mechanical) elicitation of the bulbocavernosus reflex in those patients in whom manual anogenital stimulation failed to elicit a robust response in the EAS. The proposed protocol could serve as a basis for further studies on validity, sensitivity and specificity of electrophysiologic assessment in patients with different types of "lower motor neuron" involvement of pelvic floor muscles and sacral dysfunction.

Interventional neurophysiology of the sacral nervous system.

Clinical neurophysiological tests have been introduced for the sacral neuromuscular system to aid with diagnosis of neurogenic conditions involving the lower urinary tract, anorectal and sexual dysfunction. The tests have, however, the potential to be of value in different interventions outside of the neurophysiological laboratory. EMG monitoring can be used for exact application of botulinum toxin by the relatively non-invasive transcutaneous approach in treatment of male detrusor sphincter dyssynergia. Checking for compound muscle action potentials of the external anal sphincter is proposed as the best method for exact placement of wire electrodes close to the 3rd sacral roots in treating lower urinary tract dysfunction by 'neuromodulation'. Presently the most established use of clinical neurophysiological techniques--outside the laboratory--as related to the sacral neuromuscular system is in the operating theatre. These tests have been introduced to identify relevant structures, for instance pudendal afferents within dorsal sacral roots, which should be spared during rhizotomy procedures for treatment of spasticity. Modified techniques are used intraoperatively to monitor the integrity of the lower sacral reflex arc (the bulbocavernosus reflex) or the lower sacral afferents throughout the spinal cord (pudendal SEP). Clinical neurophysiological tests are expected to become established in several interventions involving the sacral neuromuscular system.

Sphincter EMG and differential diagnosis of multiple system atrophy.

Multiple system atrophy (MSA) is a degenerative disease manifesting a combination of parkinsonism, cerebellar, pyramidal, and autonomic (including urinary, sexual, and anorectal) dysfunction. It is pathomorphologically defined, but lacks a definitive clinical diagnostic test. Sphincter electromyography (EMG), reflecting Onuf's nucleus degeneration, has been proposed as a helpful test; its value has been reevaluated by a critical review of the literature. In patients with probable MSA, abnormal sphincter EMG, as compared to control subjects, has been found in the majority of patients in all the different forms of the disease in most studies, including patients who, as yet, have no urological or anorectal problems. The prevalence of abnormalities in the early stages of MSA is as yet unclear. Patients with Parkinson's disease (PD) as a rule do not show severe sphincter EMG abnormalities in the early stage of the disease. Anal sphincter EMG abnormalities (abnormal spontaneous activity or motor unit potential changes three standard deviations above valid control data) distinguish MSA from PD in the first 5 years after the onset of symptoms and signs, and from pure autonomic failure, as well as from cerebellar ataxias, if other causes for sphincter denervation have been ruled out. With such criteria, the sensitivity of the method is, however, low. EMG does not distinguish MSA from progressive supranuclear palsy. Future studies should use standardized anal sphincter EMG to better compare results from different centers and precisely define the sensitivity and specificity of the method.

Standardization of anal sphincter electromyography: utility of motor unit potential parameters.

Advanced electromyography systems offer quantitative analysis of a number of motor unit potential (MUP) parameters. However, only limited data are available on the diagnostic usefulness of these parameters. In the present study, we compared the sensitivities of MUP parameters in revealing "neuropathic" changes in the external anal sphincter (EAS) muscles in 56 patients examined 5-240 months after damage to the cauda equina or conus medullaris. Using multi-MUP analysis, 20 MUPs were obtained from patients' EAS muscles. Their MUP parameters were compared with normative data from 64 controls. The diagnostic sensitivities of mean values/"outliers" of MUP parameters for detecting neuropathic EAS muscles were calculated (area 25%/30%; number of turns 18%/29%; size index 13%/24%; thickness 18%/18%; amplitude 17%/17%; spike duration 20%/9%; duration 15%/12%; number of phases 15%/11%; and their combination 51%/52%). Altogether, the cumulative sensitivity of multi-MUP analysis using both mean values and "outliers" was 62%. The combination of MUP parameters improves the diagnostic yield of MUP analysis, but the influence on specificity remains unknown.

Pelvic muscle activity in nulliparous volunteers.

The aim of this study was to describe normal characteristics of spontaneous and voluntary pelvic muscle function in nulliparous healthy continent women and to assess the reaction of the pelvic floor to stress and fatigue. Ten nulliparous volunteers were recruited. Pelvic muscle strength was evaluated by palpation and perineal ultrasound. Kinesiological EMG and perineal ultrasound were performed to test for possible fatigue and to assess bladder neck mobility during coughing with a pre-contraction of the pelvic floor muscles. Bladder neck mobility did not increase after attempts to fatigue the pelvic floor muscles. Bladder neck descent was significantly less when the women were instructed to contract the pelvic floor muscles before coughing. The contraction of the pelvic floor muscles stabilizes the vesical neck in nulliparous women.

Intraoperative monitoring of the bulbocavernosus reflex: the method and its problems.

OBJECTIVES: This study evaluates the method of intraoperative neurophysiological monitoring of the bulbocavemosus reflex (BCR) with reference to stimulation and detection. METHODS: The study was performed on a group of 65 patients, 53 men and 12 women, who underwent surgery for lower thoracic and lumbar spinal trauma, with no neurological deficit prior to or following surgery. Stimulating electrodes were placed on the dorsum of the penis or the clitoris. Single and double stimuli were used, as well as trains of 3, 4 and 5 stimuli. Detecting wire electrodes were introduced into the perianal region. RESULTS: A single stimulus elicited the BCR in 50%, pairs in 75%, trains of 3 in 95%, and trains of 4 and 5 in 100% of patients. With placement of the detecting electrodes 2.5 cm deep to the skin, the BCR was detected in 13% of hemisphincters in women, and in 81% of hemisphincters in men. With controlled intramuscular placement of detecting electrodes, the BCR was detected in 97% of hemisphincters in men. With bifocal detection the BCR amplitudes were 30-312 mV (median 90), and interside amplitude ratio was 0.05-1 (median 0.66); with monofocal detection, amplitudes and interside amplitude ratio were 30-560 mV (median 200) and 0.15-1 (median 0.86), respectively. CONCLUSIONS: A train of 4 electrical stimuli is optimal in eliciting the BCR in anesthetized patients. The low rate of elicitability in women was most probably due to inefficient stimulation. Detection was improved by controlled intramuscular placement of electrodes. Monofocal detection yielded higher BCR amplitudes. Interside difference and interindividual variability of the BCR amplitude were considerable.

Bladder neck mobility in continent nulliparous women.

OBJECTIVE: To evaluate the mobility of the vesical neck during coughing and valsalva in healthy nulliparous volunteers and to test the reliability of the technique applied. DESIGN: Clinical observational study. SETTING: Department of Obstetrics and Gynaecology, Cantonal Hospital Lucerne, Switzerland. POPULATION: Thirty-nine nulliparous volunteers. METHODS: Vesical neck motion was assessed with perineal ultrasound. Intra-abdominal pressure was controlled for with an intrarectal probe. Intra-rater reliability was evaluated. RESULTS: Vesical neck mobility was significantly lower during coughing (8 mm, SD 4 mm) than during valsalva (15 mm, SD 10 mm) (P < 0.005). Between individuals mobility varied from 4 mm to 32 mm during coughing and from 2 mm to 31 mm during valsalva. Test-retest-studies showed a maximum difference between to tests during coughing of 4 mm and during valsalva of 5 mm. CONCLUSION: The bladder neck is mobile in normal continent women and bladder neck mobility is lower during coughing than during Valsalva.

Reinnervation of anal sphincter muscle by low and high-threshold motor neurones.

The external anal sphincter (EAS) has continuously active low-threshold and recruitable high-threshold motor units (MUs), the latter being 'larger'. On performing concentric needle electromyography (EMG) of the EAS, the high-threshold MUs seemed to reveal more neuropathic changes than the low-threshold MUs. To verify this hypothesis, low- and high-threshold motor unit potentials (MUPs) were compared in patients with neuropathic EAS and controls. Fifteen subjects without pelvic disorders and 29 patients with sequela after cauda equina lesions were studied. In patients, only muscles ipsilateral to severe perianal sensory loss were included. MUPs were sampled using multi-MUP analysis during relaxation ('low-threshold'), and on activation ('high-threshold' MUs). MUP parameters of low- and high-threshold MUs from controls and patients were compared, as was the sensitivity and specificity with which MUPs were classified as normal or pathological (using discriminant analysis). MUP changes due to reinnervation, and the sensitivity and specificity in classifying MUPs as normal or pathological were not significantly different between the low- and high-threshold MUPs. Stronger activation of EAS does not improve discrimination between neuropathic and normal MUPs. New EMG techniques for sampling sphincter MUPs at higher activation levels would seem not to yield additional information.

Standardization of anal sphincter electromyography: normative data.

OBJECTIVES: Electromyography (EMG) of the external anal sphincter (EAS) is important in the evaluation of conus/cauda lesions, the differential diagnosis of parkinsonism and anal incontinence. The aim of our study was to establish normative data in a sufficiently large group of healthy subjects, using a rigorously standardized examination technique. METHODS: Sixty-four subjects (aged 19-83 years) without pelvic or neurological disorders were included. Motor unit potentials (MUPs)/interference pattern (IP) samples were obtained from the EAS using multi-MUP and turn/amplitude analyses, respectively. The effect of age, gender, parity, and constipation on MUP/IP parameters was studied. For MUP parameters the lower/upper limits for mean values, and 'outlier' limits, and for IP parameters normal 'clouds' were calculated. RESULTS: From 112 muscles 15-30 MUPs were sampled. As no effect of evaluated factors on mean values could be demonstrated, common reference values were calculated. Lower/higher limits for mean values were: amplitude 148/661 microV, duration 3.2/7.8 ms, area 87/625 microVms, and number of phases 2. 3/3.7. 'Outlier' limits for individual MUPs were: amplitude 84/1315 microV, duration 1.6/13.8 ms, area 46/1222 microVms, number of phases 2/6. From 95 muscles 2706 IP samples were obtained. CONCLUSIONS: The presented normative data should allow valid quantitative EMG of the EAS muscle in patients.

Standardization of anal sphincter electromyography: effect of chronic constipation.

Severe chronic constipation has been implicated as a cause of damage to the pelvic floor innervation. The aim of the present study was to examine the role of mild to moderate chronic constipation, a condition more relevant for clinical electromyographers, because this complaint is common in patients sent for evaluation of possible neurogenic dysfunction of lower sacral myotomes. A group of 59 subjects without major uroneurological dysfunction, proctological disorders, or neurological abnormalities participated in the study, which involved concentric needle electromyography of the external anal sphincter (EAS). Motor unit potentials (MUPs; sampled using multi-MUP analysis) and interference pattern (IP, sampled using turn/amplitude analysis) of chronically constipated and control subjects were compared. No effect of chronic constipation on MUP/IP parameters compatible with neurogenic injury was found. Our results suggest that mild chronic constipation does not cause damage to the EAS innervation, and that no separate reference values are needed for this group of subjects.