Impaired conduction of Ia sensory fibers in multifocal motor neuropathy: An electrophysiological demonstration.

OBJECTIVES: To report the clinical and electrophysiological findings in two patients with multifocal motor neuropathy (MMN) and bilateral absent patellar and Achilles tendon reflexes despite normal strength of quadriceps and calf muscles. METHODS: The medical history and clinical evaluation were completed by electrophysiological tests: sensory and motor nerve conduction studies, needle electromyography, motor-evoked potentials (MEPs) after transcranial magnetic stimulation, patellar T (tendon) responses, quadriceps and soleus H (Hoffman) reflex recordings. RESULTS: In the two patients, history, clinical evaluation, nerve conduction studies, favorable response to intravenous immunoglobulins, and positive anti-GM1 antibodies fulfilled the diagnosis of MMN. The lower limbs were asymptomatic, except for a unilateral weakness of foot dorsiflexion. The patellar and Achilles tendon reflexes disappeared during the course of the disease. The sensory nerve conduction studies were normal or minimally modified, M-wave and MEP/M amplitude ratio to the quadriceps were normal, patellar T (tendon) responses were virtually absent, and H-reflex to the quadriceps and soleus muscles were absent. CONCLUSIONS: These observations, which show the interruption of the reflex afferent pathway, raise the question of Ia afferent involvement in the lower limbs of these two patients with MMN. Further investigations should determine the frequency and significance of these findings in this disorder.

Contraction response to muscle percussion: A reappraisal of the mechanism of this bedside test.

OBJECTIVE: To study whether the contraction evoked by muscle percussion stems from the excitation of the muscle or of the nerve and to discuss the changes of this response in neuromuscular disorders. METHODS: In 30 neurologically healthy patients undergoing surgery (for ear, nose, or throat problems unrelated to the study) under general anesthesia with propofol and sufentanil we measured with an electrogoniometer the maximal dorsiflexion of the ankle evoked by reflex hammer percussion of the tibialis anterior muscle before and under neuromuscular junction blockade with rocuronium bromide. In 3 additional healthy volunteers we searched for F-waves to disclose whether percussion excites axons within the muscle. RESULTS: Responses from 28 neurologically healthy patients (15 women) were analyzed after exclusion of 2 due to technical problems. Mean age (SD) was 28 (9) years. Maximal dorsiflexion of the ankle was not significantly modified by neuromuscular junction blockade (mean difference 0.01 mV [95%CI, -0.07 to 0.08], p=0.879). Muscle percussion evoked F-waves in the 3 healthy volunteers tested. CONCLUSIONS: Maximal contraction response to muscle percussion has a muscular rather than a neural origin. However, percussion also excites axons within the muscle. SIGNIFICANCE: These findings may provide clues to understand the changes observed in neuromuscular disorders.

Is hemifacial spasm accompanied by hemodynamic changes detectable by ultrasound?

BACKGROUND: Arterial tortuosity of the posterior circulation compressing the facial nerve induces the ephaptic axono-axonal cross-talk that sparks hemifacial spasm. We sought if a noninvasive method such as color duplex of these arteries might detect hemodynamical changes in this condition. METHODS: Nine patients with hemifacial spasm, successfully treated with botulinum toxin, were examined with color-coded duplex ultrasound. Mean blood flow velocities of the vertebral, basilar, posterior inferior cerebellar, and anterior inferior cerebellar arteries were measured and side-to-side comparison performed. RESULTS: In all nine patients, the mean blood flow velocity, averaging across the two arteries, was higher on the side of the hemifacial spasm (Fisher's exact p < 0.008; two-tailed). The results of the repeated measures ANOVA show that the main effect of side of flow was statistically significant, F(1,8) = 17.354, p = .0032, with higher mean blood flow velocities observed on the side of the hemifacial spasm. There was no significant association between the mean flow velocity of the vertebral artery and the side of spasm (p = 0.523). CONCLUSIONS: Hemifacial spasm also seems to relate to hemodynamic changes, which may be detectable by color duplex imaging.

Parsonage-Turner Syndrome rather than Zoster Neuritis?

We report the case of an 86-year-old man with acute left shoulder pain, followed by left limb monoparesis and a herpetic rash on the left upper limb and thoracic region. This situation presented a diagnostic challenge because of the simultaneity of symptoms attributable to Parsonage-Turner syndrome and herpes zoster neuropathy. A detailed clinical history, physical examination and electroneuromyography were essential to distinguish the neurological structures involved and to ascertain the diagnosis.

Peripheral autoimmune neuropathy assessed using corneal in vivo confocal microscopy.

BACKGROUND: Corneal nerves can be examined using in vivo confocal microscopy (IVCM). This new technique permits sequential observation of the corneal subbasal nerve plexus and detects early signs of diabetic peripheral neuropathy. OBJECTIVE: To describe a patient with autoimmune peripheral neuropathy followed up using corneal IVCM. DESIGN: Case report. SETTING: Clinic of neurology, Geneva, Switzerland. Patient A 56-year-old man with peripheral neuropathy diagnosed as anti-myelin-associated glycoprotein neuropathy. His symptoms initially worsened despite the administration of intravenous immunoglobulins and plasma exchange. Evolution was eventually favorable after rituximab and corticosteroids were given. At 1-year follow-up, clinical recovery was almost complete, and the patient was stable according to the results of clinical and electrophysiologic assessments. Main Outcome Measure Corneal nerve measurement by IVCM. RESULTS: Examination of corneal nerves using IVCM at 2 different times during the patient's clinical evolution (peak disease and recovery phase) demonstrated histologic signs that correlated with the results of clinical and electrophysiologic assessments. CONCLUSION: This observation supports the hypothesis that corneal IVCM could also be helpful for the early detection or follow-up of autoimmune peripheral neuropathy.

Presurgical evaluation of epilepsy patients.

Epilepsy surgery has been established as an effective treatment in pharmacoresistant focal epilepsies. Most candidates for epilepsy surgery are patients with partial epilepsy syndromes refractory to medical treatment. The curative surgery procedure is resection of the epileptogenic zone; therefore, precise detection of the site responsible for seizure generation is necessary. Modern structural and functional imaging techniques have made presurgical evaluation less invasive and available for a higher number of patients. Video electroencephalography (EEG) monitoring, high-resolution structural and functional imaging techniques are used widely for presurgical evaluation. When noninvasive evaluation is not sufficient for the detection of the epileptogenic zone, invasive EEG monitoring and intracarotid amobarbital test are used. A classical example of a surgically curable epilepsy syndrome is mesial temporal lobe epilepsy with about 70-80% of patients becoming free of seizures after surgery. Results in extratemporal epilepsies are also satisfactory. Despite worldwide expansion during the recent decade, epilepsy surgery remains underutilized. Better understanding of advances in presurgical evaluation should reduce fears of epilepsy surgery and help to select patients who could achieve complete seizure control or significant amelioration after surgery.

Trial-to-trial size variability of motor-evoked potentials. A study using the triple stimulation technique.

Motor-evoked potentials (MEPs) vary in size from one stimulus to the next. The objective of this study was to determine the cause and source of trial-to-trial MEP size variability. In two experiments involving 10 and 14 subjects, the variability of MEPs to cortical stimulation (cortical-MEPs) in abductor digiti minimi (ADM) and abductor hallucis (AH) was compared to those responses obtained using the triple stimulation technique (cortical-TST). The TST eliminates the effects of motor neuron (MN) response desynchronization and of repetitive MN discharges. Submaximal stimuli were used in both techniques. In six subjects, cortical-MEP variability was compared to that of brainstem-MEP and brainstem-TST. Variability was greater for MEPs than that for TST responses, by approximately one-third. The variability was the same for cortical- and brainstem-MEPs and was similar in ADM and AH. Variability concerned at least 10-15% of the MN pool innervating the target muscle. With the stimulation parameters used, repetitive MN discharges did not influence variability. For submaximal stimuli, approximately two-third of the observed MEP size variability is caused by the variable number of recruited alpha-MNs and approximately one-third by changing synchronization of MN discharges. The source of variability is most likely localized at the spinal segmental level.

The clinical diagnostic utility of transcranial magnetic stimulation: report of an IFCN committee.

The review focuses on the clinical diagnostic utility of transcranial magnetic stimulation (TMS). The central motor conduction time (CMCT) is a sensitive method to detect myelopathy and abnormalities may be detected in the absence of radiological changes. CMCT may also detect upper motor neuron involvement in amyotrophic lateral sclerosis. The diagnostic sensitivity may be increased by using the triple stimulation technique (TST), by combining several parameters such as CMCT, motor threshold and silent period, or by studying multiple muscles. In peripheral facial nerve palsies, TMS may be used to localize the site of nerve dysfunction and clarify the etiology. TMS measures also have high sensitivity in detecting lesions in multiple sclerosis and abnormalities in CMCT or TST may correlate with motor impairment and disability. Cerebellar stimulation may detect lesions in the cerebellum or the cerebellar output pathway. TMS may detect upper motor neuron involvement in patients with atypical parkinsonism and equivocal signs. The ipsilateral silent period that measures transcallosal inhibition is a potential method to distinguish between different parkinsonian syndromes. Short latency afferent inhibition (SAI), which is related to central cholinergic transmission, is reduced in Alzheimer's disease. Changes in SAI following administration of cholinesterase inhibitor may be related to the long-term efficacy of this treatment. The results of MEP measurement in the first week after stroke correlate with functional outcome. We conclude that TMS measures have demonstrated diagnostic utility in myelopathy, amyotrophic lateral sclerosis and multiple sclerosis. TMS measures have potential clinical utility in cerebellar disease, dementia, facial nerve disorders, movement disorders, stroke, epilepsy, migraine and chronic pain.

A clinical study of corticospinal and peripheral conductions to proximal lower limb muscles.

OBJECTIVE: To investigate the clinical utility of the newly developed "quadriceps combined technique" (QCT), which provides a global evaluation of the central and peripheral conduction to the proximal muscles of lower limbs, in a variety of central and peripheral neurological disorders. METHODS: Using surface recordings from the vastus medialis of the quadriceps muscle, we analyzed amplitudes and latencies of M response, patellar T reflex and motor evoked potentials (MEPs) after transcranial magnetic stimulation. We studied 180 patients with disorders impairing proximal strength of one or both lower limbs and compared them with 100 controls reported previously. RESULTS: The best parameters to detect central motor disorders were the central motor conduction time, MEP/M amplitude, T/MEP amplitude and latency ratios, whereas peripheral motor conduction time (PMCT) was best to assess peripheral disorders. The best parameter to identify proximal peripheral disorder was PMCTprox, whereas for distal peripheral disorders M amplitude and T/MEP amplitude ratio were most discriminative. CONCLUSIONS: We report a simple, rapidly performed and well-tolerated method that improves proximal lower limbs evaluation, helps distinguishing pathologic from physiological brisk reflexes and provides clues for etiologic diagnosis. SIGNIFICANCE: The QCT is a sensitive and specific tool to investigate central and peripheral neurological disorders.

[Sensory neuropathies]. / Neuropathies sensitives.

Peripheral sensory neuropathies are rare. Their clinical and electrophysiological pictures vary mainly with the course of the disorder (acute, subacute or chronic), with the size of the nerve fibres (large or small diameter) and the nervous structure involved (sensory axon or dorsal root ganglion). We discuss the characteristics and aetiologies of the various sensory neuropathies. Clinical case reports underline the practical aspects of these disorders.

Electrophysiological evaluation of motor pathways to proximal lower limb muscles: a combined method and reference values.

OBJECTIVE: In contrast with their important functional and clinical role, motor pathways to proximal muscles of lower limbs are rarely investigated in clinical neurophysiology. We describe a method to evaluate central and peripheral pathways to these muscles and report reference values. METHODS: Recording of both quadriceps was performed in 100 subjects. We analyzed the maximal M response after electrical stimulation of the femoral nerve, the patellar T reflex and the motor evoked potential (MEP) after transcranial magnetic stimulation. We defined the central motor conduction time as the difference between the MEP latency and the peripheral motor conduction time, estimated as the half of the T latency minus 0.5ms. RESULTS: The mean MEP latency is 20.6ms (SD 1.99ms), central motor conduction time 10.1ms (SD 1.29ms), MEP/M amplitude ratio 60.0% (SD 15.75%). Normal limits according to height and age are provided for each parameter and for interside asymmetry. CONCLUSIONS: This method to investigate the central and peripheral motor pathways supplying the L2-L4 myotomes is simple, painless and rapidly performed. The T reflex provides additional information on the proximal sensory pathways. SIGNIFICANCE: This method will be useful in many clinical conditions.

Electrophysiology of small peripheral nerve fibers in man. A study using the cutaneous silent period.

UNLABELLED: Methods for assessing small peripheral nerve fiber function objectively are limited. The cutaneous silent period (CuSP), a transient suppression of electromyographic voluntary activity that follows painful stimuli, could serve as an objective functional measure of the A delta fibers. OBJECTIVES: To establish normal values of CuSP, to compare these values to those in the literature and to discuss the yield of the CuSP in pathological conditions. MATERIAL AND METHODS: We investigated the CuSP of the upper and lower limbs of 40 normal subjects. RESULTS: We observed that the spinal circuitry mediating the CuSP is mainly unilateral and restricted to one limb, and that CuSP latency decreases and duration increases with increasing stimulus intensity, then CuSP stabilizes with strong stimuli. In an additional study, we observed that painful cutaneous stimuli are either inhibitory, causing a pause in an ongoing contraction (CuSP), or excitatory, inducing a contraction of a muscle at rest (RIII). Inhibition and excitation have similar timings. CONCLUSION: The method for studying the CuSP is simple and well tolerated; it is useful to study A delta fibers in peripheral neuropathies and the central circuitry of this cutaneous nociceptive response in conditions affecting the spinal cord. Comparison of CuSP studies suggests the need for a standardization of the method.

Cutaneous silent period in carpal tunnel syndrome.

The cutaneous silent period (CuSP), a transient suppression of electromyographic activity that follows painful stimuli, allows an indirect study of the small-diameter A-delta fibers. To assess the function of these fibers in peripheral nerve disorders, we compared the CuSP of 40 controls to that of 40 patients with carpal tunnel syndrome (CTS) and one patient with a traumatic transection of the median nerve. Patients with CTS were divided into three severity groups, based on electrophysiological data. In CTS, digit 2 evoked CuSP onset latency was increased in all groups, and CuSP duration from abductor digiti minimi was reduced in all groups. In our series, although some parameters of the CuSP were altered, only transection of the nerve abolished it. A-delta fibers are robust, probably due to their less vulnerable small diameter. This characteristic may be useful to study various conditions and essential for patients to retain some sensation within the median nerve territory.

Transcranial magnetic stimulation in clinical practice.

Transcranial magnetic stimulation allows a non-invasive and painless stimulation of the human brain and cranial nerves. The method is in use since 1985. Transcranial magnetic stimulation can use single stimuli, pairs of stimuli separated by different intervals (to the same or to several brain areas), or trains of repetitive stimuli at various frequencies. Single stimuli give rise to motor evoked potentials that have clinical use and serve diagnostic and prognostic purposes. Repetitive transcranial magnetic stimulation can modify excitability of cerebral cortex. Repetitive transcranial magnetic stimulation has opened a new field of investigation of the neural circuitry, and is developing into a therapeutic tool. This general review considers basic principles of transcranial magnetic stimulation, discusses methodological aspects and techniques, and analyses their utility in clinical practice.

[How should a muscular disease be studied?]. / Comment investiguer une maladie musculaire?

Muscle diseases are an expanding field, mainly due to the progress in genetics and biochemistry. Evaluation starts with a thorough history of the patient's symptoms and signs. The leading clinical manifestations are weakness, atrophy, myalgia, fatigue, more rarely myotonia and in the child hypotonia or walking difficulty. A detailed family history might give clues to an underlying genetic etiology. Diagnostic workup begins with the measurement of serum creatine kinase. Electroneuromyography is an important investigation procedure which includes motor and sensory nerve conduction studies and concentric needle electromyography. Muscle biopsy is performed in all patients with clinical evidence of myopathy. A fine-needle technique is generally used, more often than a surgical biopsy. Molecular analysis of candidate genes is becoming a major diagnostic tool in many muscle disorders. Muscle imaging, in particular MR, provides diagnostic and follow-up information, especially in dystrophic, metabolic and inflammatory myopathies. Exercise testing can be useful in some metabolic myopathies. There is no standard protocol for the choice and course of investigations which must always be based on a detailed clinical evaluation. It is important to establish a precise diagnosis in order to inform the patient about the nature and the evolution of the disease, the therapeutic options and to propose, when indicated, genetic counseling.

[Contribution of electrophysiology to the study of a paresis of central origin]. / Apport de l'électrophysiologie à l'étude d'une parésie d'origine centrale.

Motor evoked potentials (MEPs) to magnetic transcranial stimulation allow a functional study of the central motor pathways (from cortex to muscle). The method--noninvasive, painless and hazard-free--is readily accepted by the patients. Two main parameters are measured, namely the central motor conduction time (CMCT) and the size of the MEP. The CMCT provides information on the conduction velocity along the corticospinal pathway, it is particularly prolonged in myelin disorders. The size of MEPs correlates with the proportion of functioning motor neurons. A particular technique allows a precise quantification of conduction defects due to conduction blocks or neuronal lesions that are responsible for the paresis. Clinical applications of the method are numerous; mainly, to detect, measure and localize a dysfunction of central motor conduction of any origin. The method serves the diagnosis and on some occasions the prognosis; it allows the follow up of central motor paresis and the effect of treatments.

Effect of discharge desynchronization on the size of motor evoked potentials: an analysis.

OBJECTIVE: Motor evoked potentials (MEPs) after transcranial magnetic brain stimulation (TMS) are smaller than CMAPs after peripheral nerve stimulation, because desynchronization of the TMS-induced motor neurone discharges occurs (i.e. MEP desynchronization). This desynchronization effect can be eliminated by use of the triple stimulation technique (TST; Brain 121 (1998) 437). The objective of this paper is to study the effect of discharge desynchronization on MEPs by comparing the size of MEP and TST responses. METHODS: MEP and TST responses were obtained in 10 healthy subjects during isometric contractions of the abductor digiti minimi, during voluntary background contractions between 0% and 20% of maximal force, and using 3 different stimulus intensities. Additional data from other normals and from multiple sclerosis (MS) patients were obtained from previous studies. RESULTS: MEPs were smaller than TST responses in all subjects and under all stimulating conditions, confirming the marked influence of desynchronization on MEPs. There was a linear relation between the amplitudes of MEPs vs. TST responses, independent of the degree of voluntary contraction and stimulus intensity. The slope of the regression equation was 0.66 on average, indicating that desynchronization reduced the MEP amplitude on average by one third, with marked inter-individual variations. A similar average proportion was found in MS patients. CONCLUSIONS: The MEP size reduction induced by desynchronization is not influenced by the intensity of TMS and by the level of facilitatory voluntary background contractions. It is similar in healthy subjects and in MS patients, in whom increased desynchronization of central conduction was previously suggested to occur. Thus, the MEP size reduction observed may not parallel the actual amount of desynchronization.