Utility of somatosensory evoked potentials in chronic acquired demyelinating neuropathy

Chronic acquired demyelinating polyneuropathy (CADP) is a heterogeneous syndrome that may be classified into a number of subtypes. Somatosensory evoked potentials (SSEPs) assess proximal segments of sensory nerves, inadequately assessed by routine nerve conduction studies (NCSs). The aim of the present study was to determine the utility of SSEPs in diagnosing and classifying different CADP subtypes. Forty-seven patients with CADP were studied and classified in five groups based on conventional NCSs and SSEPs. Some patients in Group 1 were initially misdiagnosed as having either motor neuron disease or multifocal motor neuropathy due to normal sensory NCSs, but they exhibited abnormal tibial and median nerve SSEPs, as evidenced by marked prolongation or absence of peripheral potentials (N9-median nerve, and N20-tibial nerve). These were reclassified as having chronic inflammatory demyelinating neuropathy (CIDP). In CIDP patients (Group 2), SSEPs were abnormal, thereby confirming the presence of demyelination in the proximal peripheral nerves. Patients with distal acquired demyelinating neuropathy (DADS) (Group 3), as defined by conventional NCS, exhibited abnormal SSEPs when anti-MAG antibodies were present. Anti-MAG-negative DADS patients (Group 3) had normal SSEPs. In the pure sensory ataxic group (Group 4), SSEP studies disclosed poorly formed and delayed cortical potentials with absent lumbar (N20) potentials, thereby suggesting the presence of proximal demyelination. SSEPs were normal in the pure motor CIDP and multifocal motor neuropathy patients (MMN) (Group 5), thereby differentiating asymmetric forms of CIDP from MMN. These findings suggest that SSEPs may be an important complementary investigation to conventional NCSs in the diagnosis of CADP.     

Autologous peripheral blood stem cell transplantation for chronic acquired demyelinating neuropathy

Six patients with chronic acquired demyelinating neuropathy (CADP) were treated with autologous peripheral blood stem cell transplantation (PBSCT). Two with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome improved–improvement was sustained in one but relapsed and required repeat transplant in the other. Two of the three with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and one with an IgM paraprotein and antibodies to nerve improved–of the responders, one relapsed after 18 months and the other was in remission after 6 months. Four developed neutropenic septicemia and pneumonia. The role of PBSCT in CADP refractory to other treatment deserves further investigation but the serious adverse events and lack of sustained response in some patients emphasize the need for caution.     

Somatosensory evoked potentials in chronic alcoholics with spasticity

Somatosensory evoked potentials to median and bilateral tibial nerve stimulation were investigated in eight chronic alcoholics with spasticity, 12 patients with alcoholic polyneuropathy, and 11 normal subjects. Central conduction velocities from the third lumbar vertebra to the fifth cervical vertebra and from the 12th thoracic vertebra to the fifth cervical vertebra were significantly lower in the chronic alcoholics with spasticity than in the alcoholic polyneuropathy group and in the healthy nonalcoholic group. The result indicates that chronic alcoholics with spasticity have conduction disturbance in the posterior column and/or the medial lemniscus, which is considered to be due to alcoholic myelopathy and/or a brainstem lesion.     

Somatosensory evoked potentials in abetalipoproteinemia

Visual, brain-stem auditory, and somatosensory evoked potentials (SEPs) were obtained on a patient with known abetalipoproteinemia. Only the SEP was abnormal, and it correlated with the reported neuropathology of this disease. Serial SEP studies remained stable, as had the clinical condition of this patient on vitamin E therapy. The SEP may supplement clinical examination in follow-up of patients under treatment.     

Somatosensory evoked potentials in a population with chronic lead poisoning

Eleven patients with chronic lead intoxication were submitted to somatosensory evoked potential (SEP) studies. All patients demonstrated increased lead blood levels and reduced ALA D activity in red blood cells. Three patients showed delayed spinal arrival (N13 wave), four delayed cortical arrival (N20 wave), and three prolonged central conduction time (time elapsing between N13 and N20 waves) (see table 1). No relationship was found between the abnormal findings and the levels of lead or ALA D. Time of intoxication was not related to the altered electrophysiological features either. The findings reported suggest that, beside the well known peripheral involvement in chronic lead intoxication, some patients may develop central nervous system impairment perhaps related to myelin involvement as suggested by the prolonged SEP central conduction time.     

Distal acquired demyelinating symmetric neuropathy

OBJECTIVE: To characterize an acquired, symmetric, demyelinating neuropathic variant with distal sensory or sensorimotor features. BACKGROUND: Classic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients have prominent proximal and distal weakness. However, chronic demyelinating neuropathies may present with different phenotypes. An approach that distinguishes these disorders primarily according to the pattern of weakness may be useful to the clinician. METHODS: A total of 53 patients with acquired symmetric demyelinating polyneuropathies were classified primarily according to the pattern of the neuropathy and secondarily according to the presence and type of monoclonal protein (M-protein) in this retrospective review. The authors distinguished between patients with distal sensory or sensorimotor involvement, designated as distal acquired demyelinating symmetric (DADS) neuropathy, from those with proximal and distal weakness, who were designated as CIDP. RESULTS: M-proteins were present in 22% of patients with CIDP. There were no features that distinguished clearly between CIDP patients with or without an M-protein, and nearly all of these patients responded to immunomodulating therapy. In contrast, nearly two-thirds of the patients with DADS neuropathy had immunoglobulin M (IgM) kappa monoclonal gammopathies, and this specific combination predicted a poor response to immunomodulating therapy. Antimyelin-associated glycoprotein (anti-MAG) antibodies were present in 67% of these patients. CONCLUSION: Distinguishing acquired demyelinating neuropathies by phenotype can often predict the presence of IgM kappa M-proteins, anti-MAG antibodies, and responses to immunomodulating therapy.     

Chronic acquired demyelinating motor neuropathy

A patient with chronic, acquired, demyelinating, pure or predominantly motor asymmetric neuropathy is described. Electrophysiological tests showed multifocal conduction block in motor nerves. The sensory system was intact and the first signs of slight trival involvement appeared after 4 years of disease duration. The antiganglioside antibodies were present in serum and the patient responded to immunosuppressive therapy (azathioprine). Distinction of such cases from motor neuron disease is critical since motor demyelinating neuropathy is treatable in most cases.     

Somatosensory evoked potentials in intraspinal tumors

SEPs have been recorded in 15 patients with spinal cord tumors (mostly ependymomas) and in 5 patients with cord compression due to meningiomas (3 cas), neuromas (1 case) or neurosarcoma (1 cas). All the patients have been operated on so that precise informations were available on the histology and the location of the tumor. SEPs were monitored during surgery in 8 patients. The main conclusions of this study are as follows: There was a good correlation between the somatosensory troubles for touch, vibration sense and joint position sense, and SEPs abnormalities, however, SEPs may be clearly abnormal in the absence of any somatosensory deficit when the dorsal columns are compressed and not infiltrated by the tumor. In the latter situation the reverse dissociation (i.e., normal SEPs with somatosensory troubles) may be observed. The possibility of a dissociation between normal N11 and N13 cervical components and absent P14 far field components (non-cephalic reference) has been confirmed in 6 patients with cervicomedullary tumors. In 3 of them, this dissociation was found to be reversible and early SEPs returned to normal after surgery. Peroperative monitoring of scalp SEPs recorded with a non-cephalic reference electrode allowed the detection of transient SEP abnormalities related with a traction on the dorsal columns of the cord. SEP monitoring is recommended for any surgical removal of tumor that needs a posterior myelotomy.     

Somatosensory evoked potentials in Arnold-Chiari malformation

Nearly all patients with repaired myelomeningoceles have an Arnold-Chiari (AC) malformation and about 20% of these patients develop clinical signs of brainstem dysfunction. The management of symptomatic AC malformation is still controversial and techniques are needed to provide an objective assessment of brainstem function. We recorded somatosensory evoked potentials (SEPs) in 52 patients aged between 8 months and 20 years (median 7.3 years) with AC malformation, to determine whether the SEPs discriminate patients with symptomatic AC malformation from those without symptoms. The subcortical far-field components P13, P14 and N18, which are generated within the brainstem, were recorded with non-cephalic reference electrodes and the cortical N20 with a frontal reference. Fourteen patients (27%) had signs and symptoms of brainstem dysfunction, which were related to the AC malformation. Abnormal SEPs were mainly recorded in symptomatic patients (sensitivity 0.7, specificity 0.9). The SEPs were particularly useful in patients from 4 years of age (sensitivity 0.9, specificity 0.9), but not in the younger age group. Abnormal somatosensory conduction reflects dysfunction of the brainstem or the upper cervical cord and may be clinically useful to assess patients with late onset symptomatic AC malformation.     

Somatosensory evoked potentials in Huntington's disease

Scalp recorded somatosensory evoked potentials (SEPs) elicited by left and right median nerve stimulation were obtained in 21 patients with Huntington's disease (HD), 14 individuals at risk (AR) for HD, and 21 non-patient controls matched for age and sex. Although SEP abnormalities were not uniform in the HD group, no HD patient had SEPs that conformed fully to the normal configuration with respect to peak latencies, presence of all components and spatial distribution. The most common abnormality was non-specific in nature, consisting of amplitude reduction or virtual abscence of components after 100 msec. More specific deviations were noted in the early SEP events. In half of the HD patients, peak P30 seemed to occur at approximately 45 msec poststimulus; this peak could have been taken as the normal P45 had it not reversed in phase between the central and frontal leads. In these cases peak P45 prepared to be missing. Peak N20 latency values were longer in the HD group than in the non-patient controls, whereas the P15 latencies did not differ significantly. The conduction time between P15 and N20 was significantly longer in HD patients than the non-patient controls. SEPs of the majority of the ARs were similar to those of the non-patients controls in terms of overall configuration, although mean amplitudes were generally lower for ARs than non-patient controls and 4 ARs exhibited prolonged P15-N20 latency differences.     

Somatosensory evoked potentials in lacunar syndromes

Summary Parietal and prerolandic somatosensory evoked potentials (SEPs) to median nerve stimulation were recorded from 40 patients with lacunar syndromes due to CT-verified lacunar infarcts. The control group consisted of 30 age-matched normal controls. Nineteen patients showed SEP abnormalities, mainly an increase of height-covariated latency of cortical components and/or of the central conduction time. Such changes occurred independently of the clinical features of lacunar syndromes, being related more to the lesion location than to its size. SEP studies may be a useful adjunct to the clinical diagnosis of lacunar infarct, possibly also when the CT scans are normal.     

Somatosensory evoked potentials in syringomyelia.

The two types of upper limb somatosensory evoked potential abnormality observed in nine patients with syringomyelia were reduced amplitude or absent cervical potentials and an abnormal central conduction time. Although this pattern of abnormalities resembles that observed in other intrinsic spinal cord lesions, it differs from peripheral nerve diseases and cervical radiculopathy in which the central conduction time is normal.     

Somatosensory evoked potentials in Huntington's chorea

Somatosensory evoked potentials were measured in 21 patients with Huntington's chorea and 12 controls. Central brain conduction time was normal. Early cortical component amplitudes were reduced in the patient group, latencies were normal. These abnormalities probably can be attributed to cortical dysfunction in Huntington's chorea. No indication of brain-stem dysfunction was found.     

Somatosensory evoked potentials in Charcot-Marie-Tooth disease

SEPs by median nerve stimulation have been performed in 18 adult patients (12 males and 6 females) affected by CMTD (type I, 13 patients; type II, 5 patients). All patients underwent MCV studies (median, ulnar, peroneal nerve), SCV studies (median and sural nerve), VEP, BAEP. N9 and N13 peaks were not detectable in 7/13 and 5/13 cases (HMSN type I) while cortical N19 were always recorded. Latency values of all responses were moderately or markedly delayed in all cases with HMSN type I, but proved normal or slightly delayed in HMSN type II cases. The prolonged latencies were mainly related to slowing of peripheral conduction. N9-N13 inter-peak was abnormally prolonged in 2 cases and N13-N19 in 2 other cases; both were prolonged in another case. In another 3 cases an abnormal BAEP was recorded. The few patients with abnormal CCT and BAEP probably belong to a borderline form between HMSN and hereditary ataxias.     

Somatosensory evoked potentials of the trigeminal nerve in chronic trichloroethylene poisoning

Industrial intoxication by trichloroethylene can produce neurological disturbance of the trigeminal nerve. Therefore, among 105 workers exposed to this toxin, we recorded cortical responses after stimulation of the trigeminal nerve (TSEP) and we compared our results with a control group of 52 subjects. We considered the physical examination exploring, the facial sensitivity and reflexes, the levels of urinary metabolites of trichloroethylene and TSEP. We can describe 4 groups of workers: group A, 13 subjects with perturbations of clinical and evoked responses; group B, 27 subjects with normal physical examination though with disturbed TSEP; group C, 6 subjects had an abnormal examination though evoked responses were normal; group D, 59 subjects had normal clinical examination and TSEP. Disturbed TSEP (particularly delayed responses) are found among the older workers with the longest duration of exposure and higher level of exposure. The alteration of TSEP may appear before clinical disturbance. TSEP could represent a useful test for the supervision of the exposed workers and the clinical diagnosis of the trigeminal impairment.     

Visual evoked potentials study in chronic idiopathic inflammatory demyelinating polyneuropathy.

BACKGROUND: The frequency of the association between chronic demyelinating inflammatory polyneuropathy (CIDP) and central nervous system (CNS) demyelinating lesions is probably underestimated. OBJECTIVE: To investigate the occurrence of combined central and peripheral demyelination in CIDP patients and to correlate visual evoked potential (VEP) abnormalities with CNS demyelinating lesions, observed on brain magnetic resonance imaging, and antibodies against glycolipids. METHODS: Nerve conduction studies, brain MRI and antibodies against glycolipids were prospectively studied in 17 patients who fulfilled the diagnostic criteria proposed for CIDP (Cornblath DR, Asbury AK, Albers JW, Feasby TE, Hahn AF, McLeod JG, Mendell JR, Parry GJ, Pollard JD, Thomas PK. Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy. Neurology, 1991;41:617-618). VEPs were performed in each case before and after 6 months treatment with either intravenous immunoglobulins (IVIG) or steroids. RESULTS: Eight patients (47%) had increased latencies in at least one eye or showed increased interocular latency difference. Four patients (23%) presented a significant high signal intensity on T2-weighted brain MRI images. Of these 4 patients, 3 had prolonged VEP latency. Two patients with delayed VEP latency had antibodies against GM1, and SGLPG and anti-sulfatides, respectively. One patient with normal VEPs also had antibodies to GM1. VEP results were not significantly modified after treatment, either with steroids or IVIG. CONCLUSION: This study confirmed the high frequency of abnormal VEPs in CIDP patients, and found that they are poorly correlated with CNS demyelinating lesions and antibodies against glycolipids. The VEP abnormalities of these patients may be explained by the susceptibility to immune-mediated damage of both the peripheral nervous system and the optic nerve.