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.     

Giant somatosensory evoked potentials in children without myoclonic epilepsy

Sixteen of 1093 children 5-14 years of age with various neurological problems were detected showing giant somatosensory evoked potentials (GSSEP). These potentials were analysed and their enlarged components described. None of the 16 children had evidence of myoclonic epileptic seizures. Nine children had epileptic seizures, but 7 did not. The characteristics of GSSEPs in patients without myoclonic seizures are described. We conclude that in patients without myoclonic seizures GSSEPs occur and bear some similarity with those elicited in patients with myoclonic seizures. They may represent a form of hyperexcitability of the CNS.     

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.     

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.     

Review of the evolution of electrodiagnostic criteria for chronic inflammatory demyelinating polyradicoloneuropathy

Chronic inflammatory demyelinating polyradicoloneuropathy (CIDP) is a treatable form of neuropathy. Efforts to devise sets of electrodiagnostic (nerve conduction) criteria to distinguish primary demyelination from primary axonal neuropathies have been elusive, and at least 16 criteria have been proposed. Modifications to criteria frequently represent minor changes based on applying a set to a small number of patients with the clinical diagnosis of CIDP, whereas others are based on physiological changes related to demyelination and other pathophysiological features. The various modifications continue to result in limited sensitivity, likely related to the wide range of nerve conduction abnormalities among CIDP patients. Although some sets are appropriate for formal clinical drug trials, their complexity makes them difficult to apply in the clinic or electromyography laboratory. This study considers the evolution of the criteria, discusses their limitations, and ends with a simplified set of guidelines that can be applied in the clinic or laboratory.     

Relevance of diagnostic investigations in chronic inflammatory demyelinating poliradiculoneuropathy: Data from the Italian CIDP database

The objective of our work was to report the clinical features and the relevance of diagnostic investigations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively reviewed data from patients with a clinical diagnosis of CIDP included in a national database. Among the 500 included patients with a clinical diagnosis of CIDP, 437 patients (87%) fulfilled the European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP (definite in 407, probable in 26, possible in four). In 352 patients (86%) motor nerve conduction abnormalities consistent with demyelination were sufficient for the diagnosis of definite CIDP. In 55 patients, this diagnosis required the addition of one or two (from probable or from possible CIDP, respectively) supportive tests, while in 20 cases they improved the diagnosis from possible to probable CIDP, seven patients did not change diagnosis. Considering these 85 patients, cerebrospinal fluid studies were performed in 79 cases (93%) upgrading the certainty of diagnosis in 59% of examined patients. Sensory nerve conduction studies (NCS) were performed in 85% of patients with an improvement of diagnosis in 32% of cases. Nerve biopsy and ultrasound and magnetic resonance imaging (US/MRI) exams resulted positive in about 40% of examined patients, but they were performed in few patients (7 patients and 16 patients, respectively). A response to the therapy was present in 84% of treated patients (n = 77), contributing to support the diagnosis in 40 patients in whom the other supportive criteria were not sufficient. In most patients with CIDP the diagnosis is possible solely with motor NCS while other investigations may help improving the diagnosis in a minority of patients.     

Chronic inflammatory lumbosacral polyradiculopathy: a regional variant of CIDP

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) affects various components and segments of the peripheral nervous system differently, and thus there can be phenotypic heterogeneity. We report a 47-year-old woman with chronic sensory disturbances and proximal weakness limited to the legs. Motor and sensory nerve conduction studies were normal. Somatosensory evoked potentials and imaging indicated a demyelinating process involving the lumbosacral roots. The patient responded favorably to IVIg. Although she did not fulfill the diagnostic criteria for CIDP we believe this patient represents a restricted regional CIDP variant.     

Multifocal sensory demyelinating neuropathy: Report of a case

Introduction: Multifocal sensory demyelinating neuropathy has not been adequately reported in the literature. Methods: A 42‐year‐old man with numbness of the left hand for 3 years and of the right hand for 6 months had a pure multifocal sensory neuropathy involving both hands, most prominently affecting 2‐point discrimination, number writing, and object recognition of the left hand. Near‐nerve needle sensory and mixed nerve conduction studies were performed on the left ulnar nerve. Results: Studies of the left ulnar nerve documented a demyelinating neuropathy characterized by temporal dispersion and marked decrease in the amplitudes of the sensory and mixed compound nerve potentials in the above‐elbow‐axilla segment. With intravenous immunoglobulin treatment, there was improvement in his neuropathic condition. Conclusion: In this study I describe a case of multifocal sensory demyelinating neuropathy as a counterpart of multifocal motor neuropathy. Muscle Nerve 56 : 825–828, 2017     

Monitoring spinal cord motor and somatosensory evoked potentials in anesthetized primates

Abstract

Monitoring Motor Evoked Potential (MEP) to Transcranial Stimulation (TMS) monitoring (MEP) is a growing technique to assess motor function under anesthesia. The following primate study was conducted to analyze the non-myogenic spinal motor and sensory volleys and to examine their reproducibility under nitrous oxide-methohexidone anesthesia. The traveling periodic spinal descending MEP to TMS and ascending somatosensory (SEP) to posterior tibial nerve stimulation across the thoracic cord were recorded in 12 cynomolgus monkeys. Through a small Tn~Tu laminotomy, an insulated stainless steel electrode was inserted into the epidural thoracic space. The potentials were analyzed under 50 vol% NO in 02 with methohexital (0.1-0.2 mg kg~1 min~1). A well-defined periodic TMS-MEPs and PTN-SEPs were recorded with high reproducibility and consistency in repeated trials under N20-methohexital anesthesia. MEP tracing consisted of an initial peak (direct (D) wave), occurring at 2.43 (±0.28) msec followed by subsequent five positive (indirect (I) waves). Spinal SEPs-MEPs were clearly defined, morphologically stable, and consistent over time under N20-methohexitone anesthesia. The present primate study may set a model to monitor both modalities in anesthetized neurosurgical patients.[Neurol Res 1999; 21: 359-367]     

Abnormal somatosensory evoked potentials in two patients with conversion disorder

On clinical grounds, somatosensory evoked potentials (SEP) and motor evoked potentials (MEP) are currently used to discriminate between hysterical and neurological conditions. The present paper reports on two patients with severe gait disturbance who had the near-total absence of SEP responses on the scalp during the symptomatic period, which normalized after recovery. These findings, along with others, may shed light on the brain correlates of conversion phenomena.     

Considerations after intraoperative monitoring of somatosensory evoked potentials during carotid endarterectomy

Somatosensory evoked potentials (SEPs) following median nerve stimulation were used to monitor cerebral function during 26 carotid endarterectomies. The patients with minor SEP variations had no neurological deficits on regaining consciousness while the one with more serious SEP variations had a transient deficit. The method thus seems useful in the early detection of ischemic brain impairment.

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Sommario I Potenziali Evocati Somatosensoriali (PES) da stimolazione del nervo mediano sono stati usati come metodo per monitorare la funzione cerebrale durante endoarteriectomia carotidea. Sono stati eseguiti 26 interventi di endoarteriectomia carotidea in 23 pazienti. Durante il clampaggio carotideo, più frequentemente si è osservato un aumento della latenza della P25, che in nessun caso è risultato superiore ai 2 msec. In un solo paziente si è registrato un appiattimento monolaterale della traccia con scomparsa delle componenti N20 e P25; tale paziente era l'unico a presentare un deficit neurologico al risveglio. I nostri dati sembrano suggerire l'utilità del monitoraggio intraoperatorio del PES del mediano, al fine di svelare una sofferenza ischemica cerebrale.

     

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.     

Auditory and somatosensory evoked potentials (AEPs and SEPs) and ballistic movements in parkinson disease

In five patients with initial idiopathic Parkinson disease AEPs (early and late components of auditory evoked potentials), SEPs (somatosensory evoked potentials) and arm ballistic movements (abduction of the humerus) were studied. Experimental sessions were conducted before starting treatment (L-Dopa plus Carbidopa) and at two and six month intervals. Before treatment evoked potential abnormalities were found in four out of five patients; EMG patterns underlying ballistic arm abduction movements were altered in all patients; corresponding prolonged duration of initial movements and low mean velocities were found. After treatment AEP and SEP showed a reduction of previously observed abnormalities and both EMG patterns and kinematic variables consistently improved. It is suggested that the electrophysiological investigations employed in this preliminary study may be a useful tool in clinical and pharmacological researches on Parkinson disease.

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Sommario Sono stati studiati in 5 pazienti affetti da sindrome di Parkinson idiopatica iniziale gli AEPs (componenti precoci e tardive), i SEPs ed i movimenti balistici (abduzione dell'omero). Lo studio è stato condotto prima dell'inizio della terapia (L-Dopa+Carbidopa), e due e sei mesi dopo l'inizio della terapia. Anomalie dei potenziali evocati sono state riscontrate prima dell'inizio della terapia in 4 pazienti. I dati elettromiografici relativi ai movimenti balistici di abduzione del braccio erano alterati in tutti i pazienti ed erano caratterizzati da un ritardo nell'inizio del movimento e da una diminuizione della velocità media.Dopo la terapia si è rivelata una riduzione delle anomalie precedentemente riscontrate per gli AEPs ed i SEPs ed anche i patterns EMG e le variabili kinematiche sono apparse notevolmente migliorati. Dall'analisi di questi risulati sembra emergere la possibilità che queste metodiche neurofisiologiche possano essere utilizzate quale mezzo per controllare la progressione della sindrome e l'efficacia di eventuali trials farmacologici.