Cutaneous and mixed nerve silent periods in syringomyelia.

OBJECTIVES AND METHODS: We studied cutaneous and mixed nerve silent periods (CSP, MNSP) in 4 patients with cervical syringomyelia documented by magnetic resonance imaging who on clinical examination presented with unilateral hypalgesia and hypothermesthesia. In addition, we recorded upper and lower extremity somatosensory and motor evoked potentials (SEP, MEP), and cortical silent periods. RESULTS: In all patients, CSP and the later portion of MNSP were absent or shortened on their affected side, while both were normal on their unaffected side. In all patients, SEP latencies were normal following both median and tibial nerve stimulation. In two patients, the amplitude N13 (median nerve SEP), and in one patient each the amplitudes N20 (median nerve SEP) and P37 (tibial nerve SEP) were reduced. Central motor conduction time was prolonged to abductor digiti minimi muscle in one patient on the affected side, but was normal to tibialis anterior muscle in all patients. Cortical silent periods where present bilaterally in spite of unilateral complete absence of CSP and MNSP in two subjects tested. Loss of CSP and MNSP were a sensitive parameter of spinal cord dysfunction in syringomyelia. The cervical median nerve SEP response N13 reflected gray matter involvement, while corticospinal tract dysfunction was less frequently observed. CONCLUSION: Our data suggest that CSP and later portion of MNSP are generated at the spinal level by the same small myelinated A-delta fibers, and that their central network is distinct from large diameter fiber afferents and efferents.     

SEPs and CNS magnetic stimulation in syringomyelia.

Somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) to transcranial and spinal stimulation from upper and lower limb muscles were elicited in 13 patients with syringomyelia. Seven had an associated Chiari type I anomaly. Diagnosis was confirmed by MRI. In 5 cases, SEPs and MEPs were performed before and after surgical treatment. Prolonged central motor conduction times or absent motor responses in upper or lower limbs were found in most patients. The greatest number of abnormalities was disclosed by measurement of CMCT followed by SEPs after tibial nerve stimulation. Two of 5 cases undergoing surgery improved clinically and showed reduction in CMCT after surgical treatment. Our study shows that MEPs were useful in the evaluation of neurophysiological status in syringomyelia patients, helping to estimate anterolateral spinal cord function.     

Functional organization of exteroceptive inhibition following nociceptive electrical fingertip stimulation in humans.

OBJECTIVE: To investigate the functional organization of inhibitory spinal reflexes mediated by cutaneous afferents (=cutaneous silent periods, CSPs) in order to assess differential strategies used by the human spinal cord to inhibit movement in upper limb muscles within the same myotome. METHODS: Twenty healthy subjects underwent assessment of the effect of electrical finger-tip stimulation with different intensities on voluntarily contracting hand muscles. The rectified and averaged electromyogram (EMG) was recorded with surface electrodes placed over abductor pollicis brevis (APB), abductor digiti minimi (ADM), and first dorsal interosseous (FDI) muscles following recurrent digit II and digit V stimulation. RESULTS: Finger-tip stimulation resulted in a series of inhibitory and excitatory EMG responses in all 3 hand muscles. Stimulus intensity exerted a significant influence on various CSP parameters (probability, onset and end latency, duration, index of suppression). CSPs were most pronounced in APB following digit II and, somewhat less, following digit V stimulation. FDI and ADM differed in CSP onset latency following digit II stimulation, and in the overall amount of suppression following digit V stimulation, despite being supplied by the same nerve and the same myotome. Excitatory long-loop reflexes seemed to be suppressed by increasing stimulus intensities, yet they interfered with CSPs, even when using noxious stimulation, in FDI following digit II stimulation, and in both ADM and FDI following digit V stimulation. CONCLUSIONS: The findings are consistent with the presence of an inhibitory neural circuitry within the human spinal cord which can rapidly restrain distinct muscle synergies. Neurophysiology indicates a more functional - task-related - organization of protective reflexes in the human upper extremity, rather than a merely metameric - anatomically based - order of activation of this neuronal circuitry.     

Motor and somatosensory evoked potentials in Autosomal Dominant Hereditary Spastic Paraparesis (ADHSP) linked to chromosome 2p, SPG4

The aim of our study was to evaluate Motor Evoked Potentials (MEPs) and cortical excitability, using Transcranial Magnetic Stimulation (TMS) as well as short latency Somatosensory Evoked Potentials (SEPs) in Autosomal Dominant Hereditary Spastic Paraparesis (ADHSP) patients. MEPs were recorded from upper and lower limb muscles in 12 patients (7 m and 5f) affected by ADHSP with spastin mutation (SPG4). We measured: (i) motor threshold (MTh); (ii) total motor conduction time (TMCT); (iii) direct and indirect central motor conduction time (d-CMCT and i-CMCT) calculated by subtracting from the cortical latency those obtained on magnetic spinal stimulation (d-PMCT) and via the F-wave method (i-PMCT); (iv) MEP amplitude (MEP/Mmax ratio%) and (v) duration of the cortical silent period (CSP). Latency, amplitude and persistence of the F-wave obtained with electrical nerve stimulation were also considered; H reflex was also tested from lower extremities. SEPs were recorded from spine and scalp sites following median and posterior tibial nerve stimulation; conventional latency and amplitude measurements were performed. In a comparison with the control group, the MTh recording from lower limbs was significantly higher (67.5 +/- 7.7% versus 52.5 +/- 6.9%), MEPs were absent in one case and showed reduced amplitude in the remainders (22.9 +/- 12.6% versus 66.3 +/- 25.9% of M wave); TMCT resulted to be abnormal (36.5 +/- 3.9 ms versus 27.1 +/- 1.4 ms) and d-CMCT as well as i-CMCT were significantly prolonged (23.1 +/- 3.5 ms versus 13.8 +/- 1.3 ms; and 20.1 +/- 3.4 ms versus 10.6 +/- 1.3 ms, respectively). The CSP, which was normal from the hands, was significantly shortened from the legs and correlated with spasticity scoring (Ashworth scale). Cortical SEPs from lower limbs were abnormal in all cases, whereas SEPs by stimulation of median nerves were normal; F-wave parameters from upper limbs showed no abnormalities, whereas an increased persistence was detected from lower limbs; H reflex amplitudes resulted larger compared with controls. Moreover, shortening of the CSP, being correlated with the Ashworth scale, can be considered an electrophysiological marker of spasticity that seems to arise from impairment of the supraspinal or intracortical inhibitory pathways with an additional contribution of increased segmental motor neuron excitability. These data prove the existence of comparable neurophysiological abnormalities in ADHSP with spastin mutation (SPG4) when long ascending and descending pathways are involved.     

Cortical motor neuron excitability during cutaneous silent period

Objective: To investigate cortical motor neuron excitability during cutaneous silent period (CSP), motor evoked potentials (MEPs) from abductor pollicis brevis following transcranial magnetic stimulation (TCM) were recorded with and without a conditioning of ipsilateral painful digital nerve electric stimulation.Methods: MEPs following TCM were recorded with and without a conditioning stimulation at an interstimulus interval (ISI) from 0 ms to 100ms in 6 controls and four patients who had reduced pain sensation in unilateral upper limbs associated with cervical syringomyelia. In addition MEPs and evoked spinal cord potentials (ESCPs) from cervical epidural space following TCM with and without a conditioning stimulation were recorded in four patients with thoracic myelopathy.Results: MEP amplitude was clearly attenuated by a conditioning stimulation at an ISI from 40 ms to 80 ms in controls (statistically significant at 60 ms). In patients with cervical syringomyelia, MEP amplitude was attenuated by a conditioning stimulation in asymptomatic hands similarly in controls but that was unchanged by a conditioning stimulation in the symptomatic hand with reduced pain sensation. In patients with thoracic myelopathy MEP amplitude was attenuated by conditioning stimulation similarly in controls, but ESCP amplitude was unchanged.Conclusions: We demonstrated that noxious cutaneous nerve stimulation suppressed spinal motor neurons but cortical motor neuron excitability was unchanged during CSP. In clinical practice, measurement of MEP suppression after noxious cutaneous nerve stimulation may provide useful information in patients with damaged pain related nerve fibers.     

Abnormalities of somatosensory and motor evoked potentials in adrenomyeloneuropathy: Comparison with magnetic resonance imaging and clinical findings

We studied 6 patients with adrenomyeloneuropathy (AMN) showing mild signs of central nervous system involvement. All patients underwent brain and spinal magnetic resonance imaging (MRI) and somatosensory (SEP) and motor (MEP) evoked potential study. Whereas SEPs and MEPs were abnormal in all patients, only 1 patient showed brain MRI abnormalities; spinal MRI showed hypotrophy without focal abnormalities in 4 of 6 patients. Median nerve SEPs, which were recorded with noncephalic reference montage, revealed delayed or absent scalp P14 far-field potential in all patients and abnormal spinal N13 in 2. Moreover, tibial nerve SEPs revealed abnormalities of the subcortical P30 response in all 4 patients in whom scalp-to-ear recording was employed. These findings strongly suggest that in the early stages of disease neurological dysfunction is localized in the spinal cord, where it is difficult to assess using MRI. However, SEPs and MEPs, which show a typical pattern of abnormality in these patients, could be useful in disclosing signs of long tract involvement and in monitoring treatment. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 1249–1257, 1997     

Subcortical somatosensory evoked potentials after median nerve and posterior tibial nerve stimulation in high cervical cord compression of achondroplasia

Children with achondroplasia may have high cervical myelopathy from stenosis of the cranio-cervical junction resulting in neurological disability and an increased rate of sudden death. To detect myelopathy we recorded somatosensory evoked potentials after median nerve (MN) and posterior tibial nerve (PTN) stimulation in 77 patients with achondroplasia aged 0.3–17.8 years (mean 2.7 years). In addition to the conventional technique of recording the cortical components and the central conduction time (CCT) we employed non-cephalic and mastoid reference electrodes to record the subcortical waveforms N13b and P13 (MN-SEP) as well as P30 (PTN-SEP), respectively, which are generated near the cranio-cervical junction. The findings were related to the MRI results. Thirty-four patients had abnormal MRI findings including spinal cord compression (n = 28) and/or myelomalacia (n = 24) at or below the cranio-cervical junction. The sensitivity of the MN-SEPs was 0.74 including all abnormal upper cervical cord MRI findings (specificity 0.98), and the sensitivity was 0.79 (specificity 0.92) for cervical cord compression, respectively. The sensitivity of the PTN-SEPs was 0.52 (specificity 0.93) for all abnormal MRI findings and 0.59 (specificity 0.92) for cervical cord compression. The subcortical SEPs N13b and P13 as well as P30 were more sensitive than the conventional recordings. The MN-SEPs, notably the subcortical tracings, are useful for the detection of cervical myelopathy in children with achondroplasia. The PTN-SEPs are less sensitive. However, the tibial nerve SEPs might contribute additional information from the lumbar or thoracic spinal cord, which was, however, not tested in this study.     

Neurophysiological evaluation of sensorimotor functions of the leg: comparison of evoked cortical potentials following electrical and mechanical stimulation,long-latency muscle responses,and transcranial magnetic stimulation

Summary Twenty-two patients with localized lesions of the central nervous system (unilateral cerebral ischaemia, cervical myelopathy, spinal tumour, familial spastic paraplegia) underwent neurophysiological evaluation of sensorimotor deficits of the leg. Functional methods using muscle stretch as stimulus, i.e. long-latency muscle responses and cortical potentials evoked by dorsiflection of the foot, were compared with transcranial magnetic stimulation and somatosensory evoked cortical potentials following electrical stimulation of the posterior tibial nerve. The functional neurophysiological methods yielded no diagnostic superiority with respect to the procedures using artificial (i.e. magnetic and electrical) stimulation. However, in most cases of missing compound motor action potentials following transcranial magnetic stimulation or missing electrically evoked cortical potentials, the long-latency muscle responses still allowed quantitative assessment of sensorimotor function.     

Long-term nervous system damage from radiation of the spinal cord: An electrophysiological study

Summary A group of 13 patients suffering from Hodgkin's disease who had undergone chemotherapy and radiotherapy (above and below the diaphragm) approximately 10 years earlier was studied. The total chemotherapeutic dose was similar for all patients; the radiotherapy dose, however, was standard for 7 patients, while the other 6 received much higher dosages over limited regions of the spinal cord. Although most of these patients appeared normal both clinically and on magnetic resonance imaging, a neurophysiological study was performed to determine whether there was any involvement of the central or peripheral nervous system. Motor conduction velocity and sensory conduction velocity were measured in the lower limbs as well as spinal- and scalprecorded somatosensory evoked potentials (SEPs) in response to stimulation of the posterior tibial and sural nerves at the ankle. In addition, motor evoked potentials were recorded from the upper and lower limbs during cortical stimulation. All neurophysiological data were normal in patients who had received a standard radiation dose, while most of those who had been exposed to higher doses showed altered cortical SEPs and a slowing of central conduction time (D10-P1). Thus even though they were asymptomatic, these patients appeared to have sustained CNS damage, mainly at the level of the spinal cord.     

Neurophysiological testing in anorectal disorders

The neurophysiological techniques currently available to evaluate anorectal disorders include concentric needle electromyography (EMG) of the external anal sphincter, anal nerve terminal motor latency (TML) measurement in response to transrectal electrical stimulation or sacral magnetic stimulation, motor evoked potentials (MEPs) of the anal sphincter to transcranial magnetic cortical stimulation, cortical recording of somatosensory evoked potentials (SEPs) to anal nerve stimulation, quantification of electrical or thermal sensory thresholds (QSTs) within the anal canal, sacral anal reflex (SAR) latency measurement in response to pudendal nerve or perianal stimulation, and perianal recording of sympathetic skin responses (SSRs). In most cases, a comprehensive approach using several tests is helpful for diagnosis: needle EMG signs of sphincter denervation or prolonged TML give evidence for anal motor nerve lesion; SEP/QST or SSR abnormalities can suggest sensory or autonomic neuropathy; and in the absence of peripheral nerve disorder, MEPs, SEPs, SSRs, and SARs can assist in demonstrating and localizing spinal or supraspinal disease. Such techniques are complementary to other methods of investigation, such as pelvic floor imaging and anorectal manometry, to establish the diagnosis and guide therapeutic management of neurogenic anorectal disorders.     

Transcranial magnetic stimulation in patients with cervical spondylotic myelopathy: Clinical and radiological correlations

Motor evoked potentials (MEPs) were studied in 28 patients with cervical spondylotic myelopathy. MEPs after cortical stimulation were abnormal in 27 patients, the responses in the leg muscles being affected the most often. Clinically asymptomatic motor lesions were detected in 7 patients (25%). The central motor conduction time (CMCT) for the abductor digiti minimi muscles correlated significantly with the clinical disability, whereas the radiological findings did not correlate with the clinical and neurophysiological parameters. In 9 patients MEPs were also recorded in the biceps muscles. The 7 patients with an abnormal CMCT for the biceps muscles had the most severe stenosis at the C-4–C-5 level or higher. The 2 patients with normal MEPs of the biceps muscles both had a stenosis at the C-5–C-6 level. The results of this study suggest that MEPs are useful for detecting spinal cord dysfunction and for localizing the level of the lesion. Some recommendations regarding the possible use of MEPs in the clinical evaluation of patients with cervical spondylotic myelopathy are given. © 1994 John Wiley & Sons, Inc.     

Analysis of somatosensory evoked potentials in peroneal nerve palsy

The peroneal nerve SEPs over the CZ' of the scalp were studied in patients with peroneal nerve palsy. The initial positive peak latencies of P27 (to popliteal fossa stimulation), P30 (to fibular neck stimulation) and P37 (to dorsum of the foot stimulation) were measured. The latency difference P30-P27 was prolonged in all patients with the fibular head lesions. In patients with the superficial peroneal nerve lesions at the foreleg, P37-P27 was prolonged whereas P30-P27 was normal. Clinical application of peroneal nerve SEPs was useful in deciding the site of the lesion causing the peroneal nerve palsy.     

Dissociation between cutaneous silent period and laser evoked potentials in assessing neuropathic pain

In this study we investigate whether the cutaneous silent period (CSP)—an inhibitory response evoked in hand muscles by painful digital nerve stimulation—is useful for assessing nociceptive pathway function in patients with neuropathic pain. In 40 patients with peripheral neuropathy (21 without and 19 with neuropathic pain) we recorded the CSP in the abductor digiti minimi after fifth digit stimulation and also recorded laser evoked potentials (LEPs) after stimulation applied to the ulnar territory of the hand. Although the LEP amplitude was significantly lower in patients with pain than in those without (P < 0.005), the CSP duration did not differ between groups (P > 0.50). Pain intensity correlated significantly with LEP amplitudes (P < 0.005) but not with CSP duration (P > 0.5). Our findings indicate that the CSP is not useful for assessing nociceptive pathway function in patients with neuropathic pain. Muscle Nerve, 2008     

Magnetically evoked motor potentials in demyelinating and axonal polyneuropathy: a comparative study

We investigated the value of magnetically evoked motor potentials (MEPs) for the differentiation of demyelinating and axonal polyneuropathies. The study population comprised 107 patients, with polyneuropathy verified by electromyography (EMG) and nerve conduction study (NCS), who had also been examined by means of MEP. MEPs were evoked by magnetic stimulation of the cortex and the spinal roots and were recorded from three upper limb muscles and two lower limb muscles bilaterally. From the EMG/NCS results 53 patients were characterized as having primary demyelination (demyelinating patients) and 54 as having axonal involvement (axonal patients). Demyelinating patients were classified as acute (acute inflammatory demyelinating polyradiculoneuropathy: AIDP) or chronic (chronic inflammatory demyelinating polyradiculoneuropathy: CIDP) according to the duration of illness. A series of indices were calculated from MEP results. One demyelinating patient and two axonal patients had normal MEPs. The MEPs of the demyelinating patients showed significantly longer peripheral conduction times, larger interside differences and lower amplitudes than the axonal patients. The central conduction index and the amplitudes upon cortical stimulation were significantly higher in patients with CIDP than in those with AIDP. Peripheral conduction time prolonged by more than 85% in at least one of the 10 muscles studied or a peripheral conduction index of above 9.4 were pathognomonic for demyelination. By combining all criteria 75% of the patients could be categorized as CIDP vs. AIDP in accordance with the EMG/NCS diagnosis. Likewise, 83% were categorized correctly as demyelinating versus axonal according to the EMG/NCS data.     

Direct cortical stimulation with cylindrical depth electrodes in the interhemispheric fissure for leg motor evoked potential monitoring

ObjectiveTo evaluate cylindrical depth electrodes in the interhemispheric fissure as an alternative to subdural strip electrodes for direct cortical stimulation (DCS) leg motor evoked potential (MEP) monitoring.MethodsA cylindrical depth electrode was positioned in the interhemispheric fissure of 37 patients who underwent supratentorial brain surgery. Leg sensory and motor cortices were localized by highest tibial nerve somatosensory evoked potential amplitude and lowest DCS leg MEP threshold; the lowest-threshold electrode was then used for DCS leg MEP monitoring.ResultsIntraoperative leg MEPs were obtained from all the patients in the series. The mean intensity applied for leg MEP monitoring with the cylindrical depth electrode was 15.2 ± 4.0 mA. No complications secondary to neurophysiological monitoring were detected.ConclusionsLower extremity MEPs were consistently recorded using a multi-contact cylindrical depth electrode in the interhemispheric fissure by DCS.SignificanceCylindrical depth electrodes may be a safe and effective alternative for DCS in the interhemispheric fissure, where subdural strips are difficult to place.     

Serial changes in motor and somatosensory evoked potentials in putaminal haemorrhage

Little is known about evoked potential changes in putaminal haemorrhage. In this study, somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) have been serially evaluated and their role in the prognosis of putaminal haemorrhage is now reported. Nineteen patients with CT- or MRI-proven putaminal haemorrhage were examined after a mean duration of 13 days (range 2–30); there were 4 females and 9 males, ranging in age between 25 and 70 years. The haematomas were of medium size in 13 and large or small in 3 patients each. The changes in the clinical picture and the SEPs and MEPs were evaluated on admission, and after 30 and 90 days. Central motor conduction time (CMCT) could not be recorded in 13, but was prolonged in 2 and normal in 4 patients. Median SEPs revealed the absence of near field potentials in 11 and prolongation of N9–N20 conduction time in 1 patient. In the follow-up period MEP and SEP abnormalities only changed in 5 patients; MEPs changed in 4 and SEPs in 2. The period of normalisation of MEPs ranged between 1 and 6 months. CMCT correlated with motor and N9–N20 conduction time with sensory impairment. Eight patients had poor, 6 partial and 5 complete recovery. Power, sensation, CMCT, and size and location of haematoma made positive contributions to recovery.     

Giant somatosensory evoked potentials in a patient with the anterior spinal artery syndrome

We studied a previously healthy 25-year-old woman with the anterior spinal artery syndrome, a rare thoracocervical myelopathy with multiple potential etiologies. Quantitative and clinical sensory examination showed dissociated loss of pin-prick and temperature discrimination below the level of the lesion, with normal light touch, vibratory, and position sense. Magnetic resonance imaging was consistent with cervical spinal cord infarction. Median SEPs showed normal Erb's potential with absent spinal N—₁₃ and normal scalp N—₂₀ latency. Tibial SEPs showed normal lumbosacral responses and normal scalp P—₃₀ latency. Both median and tibial nerve stimulation produced cortical responses of unusually large amplitude (median 38 m?V, tibial 17 m?V). We hypothesize that large SEP amplitudes in this patient resulted from loss of anterolateral inhibitory influences on the dorsal column–medial lemniscal system. © 1993 John Wiley & Soncs, Inc.     

Somatosensory evoked potentials after multisegmental lower limb stimulation in focal lesions of the lumbosacral spinal cord

OBJECTIVES: Recording techniques permit the separate analysis of the response from cauda equina roots and the spinal potential that is probably generated by the activation of dorsal horn cells. To improve the functional assessment of focal lesions of the lumbosacral cord, lower limb somatosensory evoked potentials (SEPs) were measured by multisegmental stimulation. METHODS: Common peroneal and tibial nerves SEPs were recorded in 14 patients in whom MRI demonstrated compressive cord damage ranging from T9 to L1 levels. SEPs were recorded in each patient at the lumbar level (cauda equina response), lower thoracic level (spinal response), and from the scalp (cortical response). RESULTS: Abnormalities in spinal response occurred in 50% and 70% of tibial and common peroneal nerve SEPs respectively; these findings were well explained by the radiological compression level, involving in most of the patients lumbar rather than sacral myelomeres. The SEPs were often more effective than the clinical examination in showing the actual extension of damage. CONCLUSIONS: The recording of spinal SEPs after multisegmental lower limb stimulation proved useful in assessing cord dysfunction and determining the cord levels mainly involved by the compression.     

Conditioning effect on the long latency potentials in the lower limb to transcranial magnetic stimulation

Objectives – We used an electrical conditioning stimulation followed by transcranial magnetic stimulation (TMS) to facilitate the occurrence of long latency potentials (LLPs) in order to study the relationship between primary motor evoked potentials (MEPs) and LLPs in the lower limbs. Materials and methods – The study group included 6 healthy subjects, 1 patient with right thalamic infarction, and 3 patients with spinal cord injuries. The subjects were subjected to electrical conditioning (C) stimulation delivered to the left big toe at 250 Hz in a train of pulses of 20 ms duration prior to TMS (T) from 0 to 150 ms at an increment of 10 ms. The surface electromyographic signals were recorded at the tibialis anterior and gastrocnemius medialis for 400 ms. Results – The C-T test facilitated both primary MEPs and LLPs with a pattern similar to the primary MEPs of its antagonist. There was no facilitation of the primary MEPs or LLPs in the affected limb of patients with thalamic or spinal cord lesions. Conclusion – At appropriate C-T interval, LLPs could be consistently provoked by TMS. The LLPs were absent in the patients with thalamic infarction and spinal cord injuries. It suggests that LLPs might be provoked through a supraspinal control.     

Neurophysiological evaluation of the central nervous impulse propagation in patients with sensorimotor disturbances

Motor evoked potentials (MEPs) to transcranial stimulation (TCS) and somatosensory evoked potentials to median nerve stimulation (MN-SEPs) were examined in 74 patients affected by multiple sclerosis (MS = 49 cases), amyotrophic lateral sclerosis (ALS = 9 cases), cervical cord lesions (7 cases), Parkinson's disease (PD = 5 cases), Huntington's chorea (HC = 2 cases), Wilson's disease (WD = 1 case), subacute combined degeneration (SCD = 1 case). MN-SEPs were altered in 38% of arms in MS with a higher incidence in clinically affected than in clinically 'silent' arms (= 77.8% vs. 27.5%). MEP alterations were found in 54% of examined arms, mostly because of a prolongation of the motor CCT. This index was invariably altered in the affected arms, whilst it was involved in 40% of the 'silent' ones. Twelve out of 18 arms displayed abnormal MEPs in ALS. These were mainly due to an absent response, even if moderate motor CCT prolongation and 'giant' MEPs were also encountered. MN-SEPs were altered in 3/18 arms. By recording MEPs from proximal and distal upper limb muscles, cues on the level of abnormal propagation were obtained in patients suffering from 'focal' lesions of the spinal cord. Combining SEP records enhanced the diagnostic yield in this field. Both MEPs and SEPs were normal in patients with PD and HC, whilst abnormally prolonged CCTs were found in the case with WD. MEP and SEP recording revealed central propagation abnormalities coupled to a severe clinical picture of the peripheral nerve involvement (as in the case of SCD).