Multimodality evoked potentials in motor neuron disease

We performed median and tibial nerve somatosensory evoked potentials (SEPs), pattern-shift visual evoked potentials (PSVEPs), and brain-stem auditory evoked potentials (BAEPs) on 27 patients with motor neuron disease (MND). Median and tibial nerve SEPs were abnormal in 8 (30%) of 27 and 3 (14%) of 21 patients tested, respectively. Central and peripheral abnormalities were recorded in the absence of spondylosis. As a group, patients with MND and no evidence of cervical spondylosis had normal conduction to Erb's point following median nerve stimulation, but conduction times beyond this point were prolonged. The PSVEPs and BAEPs were within normal limits in all patients, excluding abnormalities attributable to other disease, but the group P100 latency was significantly prolonged in the group with MND. The BAEPs were normal in the group with MND. This study provides neurophysiological evidence of sensory system involvement in MND.     

Brain function in patients with cerebral fat embolism evaluated using somatosensory and brain-stem auditory evoked potentials

Summary In two victims of traffic accidents with broken bones and fat embolism, serial recordings of somatosensory evoked potentials (SEPs) and brain-stem auditory evoked potentials (BAEPs) were examined to assess brain function. Initial SEPs and BAEPs revealed normal subcortical components, while the late cortical components of SEPs were abolished, findings indicative of diffuse dysfunction of grey rather than of white matter. As the neurological functions became normal, the late components appeared. It is concluded that while absent late components of SEPs do reflect cortical dysfunction, they are not necessarily associated with a poor prognosis. Repetitive recordings of SEPs appear to be a useful tool for assessing the neurological condition and the prognosis of patients with cerebral fat embolism.     

Serial evoked potentials and outcome in cerebrovascular critical care patients.

Median-nerve evoked somatosensory evoked potentials (SEPs) and brainstem auditory evoked potentials (BAEPs), examined early in the course of patients suffering from cerebrovascular disease, correlate statistically significantly with outcome. Little is known about the changes of evoked potentials in the course of disease and their correlation to outcome. In a series of 215 patients (75 supratentorial infarctions, 36 infratentorial infarctions, 58 supratentorial hemorrhages, 18 infratentorial hemorrhages, and 28 aneurysmatic subarachnoid hemorrhages) requiring neurologic intensive care treatment, we prospectively examined the correlation between the findings of serial SEPs and BAEPs and outcome at 4 weeks. Evoked potentials were examined after admission, after 1 week, and after 2 weeks. The findings were classified in 4 categories (normal, unilateral or bilateral pathologic findings, unilaterally attenuated, and bilaterally attenuated). Clinical outcome was determined by classification according to the Glasgow Outcome Scale (death, persistent vegetative state, severely incapacitated, mildly incapacitated, and recovery). Statistical evaluation was performed using Fisher's exact test for all variables. In all subgroups, SEPs correlated statistically significantly with outcome at all three examinations. No correlation was found for BAEPs at first examination in infratentorial disease, nor at second examination in subarachnoid hemorrhages. In all other cases, SEPs and BAEPs were correlated statistically significantly with outcome at all three examination timepoints.     

Brainstem auditory evoked potentials in Wilson's disease

Twelve patients with Wilson's disease, aged 11-25 years, underwent brainstem auditory evoked potential (BAEP) study. The results were correlated to clinical, neuroradiological and laboratory data. Ten had prominent to severe neurological manifestations, suggestive of involvement of one or several CNS structures, and 2 were neurologically free. All had evidence of abnormal copper metabolism, and 8 had CT scan evidence of brain atrophy, or hypodense areas in basal ganglia, or both. The 2 patients without neurological manifestations as well as one with neurological signs had normal BAEP. One patient with neurological signs had increased N1 latency due to cochlear hearing loss, but normal interpeak intervals, while 8 of 10 patients with prominent neurological symptoms and signs had abnormal BAEPs (prolongation of NIII-NV interpeak interval). The value of NIII-NV interpeak interval correlated with the number of different neurological signs (neurological score) attributable to involvement of different CNS structures (r = 0.64 at P = 0.001). Abnormal BAEPs do not seem to be an early finding in Wilson's disease.     

Cerebrotendinous xanthomatosis: 11-year treatment with chenodeoxycholic acid in five patients. An electrophysiological study

We report the electrophysiological follow-up of five cerebrotendinous xanthomatosis patients treated for 11 years with chenodeoxycholic acid (CDCA). Nerve conduction velocity (NCV) was reduced in three cases. P100 latency of visual evoked potentials was delayed in four cases, interpeaks I–III and I–V of brainstem auditory evoked potentials (BAEPs) was increased in two and interpeak N13–20 of upper limb somatosensory evoked potentials (SEPs) was slowed in one. After 4 months of therapy with CDCA, NCV was normal and did not show any significant change during the 11 years of observation. Central motor conduction time of motor evoked potentials (MEPs) and N24–P40 interpeak latency of lower limb SEPs were increased in five and four cases, respectively, in spite of 2/3-year treatment with CDCA. Improvement of evoked potentials, especially of MEPs and SEPs, was slower and continued over the whole 11-year period. The size of xanthomas slightly decreased in some patients during treatment and the clinical manifestations stabilized, avoiding progressive worsening, but there was no significant improvement in neurological deficit. Two sisters of patients who never took CDCA showed progressive worsening of clinical manifestations, upper limb SEPs and BAEPs.     

Clinical, CT and evoked potential manifestations in Wilson's disease with cerebral white matter involvement

Four patients with Wilson's disease had CT manifestations of cerebral white matter lesions involving frontal lobes in two patients but extending to other cerebral areas in the remaining two. Two patients with extensive white matter involvement were bed-ridden with generalized flexion rigidity and spasticity, but the other 2 patients led an independent life with minimal extrapyramidal symptoms. Three patients developed tonic focal seizures with or without secondary generalization. Scalp-recorded somatosensory evoked potentials (SEPs) over the affected hemispheres were absent but the spinal SEPs and brain stem auditory evoked potentials were preserved. The evoked potential data support the CT findings that the white matter involvement in Wilson's disease was primarily confined to the cerebral hemispheres.     

Electrophysiological findings in neurofibromatosis type 1

Neurofibromatosis type 1 (NF1) is a common, autosomal dominant neurocutaneous disorder in which any organ system, including the skin, skeleton and nervous system can be affected. In this study, we compared the electrophysiological and magnetic resonance imaging (MRI) findings in patients with NF1. Thirty-nine adolescent and adult patients (23 women and 16 men) diagnosed with NF1 with a mean age of 25.8±10 years (10-56) were included in this study. We collected data in the form of the results of neurological examinations, multimodal evoked potentials (EPs; brainstem auditory evoked potentials, BAEPs; somatosensory evoked potentials, SEPs; and visual evoked potentials, VEPs), cerebral/orbital/spinal MRIs, and electroneuromyography (ENMG). Twenty (51.3%) patients showed abnormal VEPs, 14 (35.9%) showed abnormal SEPs, and six (15.4%) showed abnormal BAEPs. All evoked potentials were abnormal in four (10.3%) cases. These electrophysiological findings occurred primarily in the absence of any clinical sign related to the affected system. MRI revealed pathologic findings in 26 of 39 patients, and these were not always correlated with visual, auditory, or somatosensory pathway abnormalities. ENMG showed polyneuropathy in two of 33 patients who underwent ENMG. Our study showed that MRI and electrophysiological abnormalities may be found in most patients with NF1, even in the absence of associated clinical symptoms or signs. Electrophysiological testing is helpful for monitoring the subclinical involvement of the central and peripheral nervous systems in patients with NF1.     

The assessment of severe head injury by short-latency somatosensory and brain-stem auditory evoked potentials

The relative prognostic value of short-latency somatosensory evoked potentials (SEPs) and brain-stem auditory evoked potentials (BAEPs) was assessed in 35 patients with post-traumatic coma. Analysis of the evoked potentials was restricted to those recorded within the first 4 days following head injury. Abnormal SEPs were defined as an increase in central somatosensory conduction time or an absence of the initial cortical potential following stimulation of either median nerve. Abnormal BAEPs were classified as an increase in the wave I-V interval or the loss of any or all of its 3 most stable components (waves I, III and V) following stimulation of either ear. SEPs reliably predicted both good and bad outcomes. All 17 patients in whom SEPs were graded as normal had a favourable outcome and 15 of 18 patients in whom SEPs were abnormal had an unfavourable outcome. Although abnormal BAEPs were associated with an unfavourable outcome in almost all patients (6 of 7), only 19 of 28 patients with normal BAEPs had a favourable outcome. The finding of normal BAEPs was therefore of little prognostic significance. These results confirm the superiority and greater sensitivity of the SEP in detecting abnormalities of brain function shortly after severe head trauma.     

Neurophysiological and radiological findings in myotonic dystrophy patients

Somatosensory evoked potentials (SEPs) and brainstem auditory evoked potentials (BAEPs) were recorded in 10 patients with myotonic dystrophy and in 20 sex and age-matched healthy controls. In all patients a brain MRI examination was also performed. In our results, the significantly longer absolute peak latencies of the SEPs and the abnormal increasing of the later components of the BAEPs suggest an involvement of the afferent sensory and central auditory pathways. Brain MRI showed white matter hyperintense lesions (WMHL) in eight patients (80%). No correlations were found between individual abnormal electrophysiological parameters or severity of WMHL and age, age at onset, disease duration or muscular impairment. The total number (SEP + BAEP) of electrophysiological abnormalities significantly correlated with muscular impairment ( p < 0.05) and MRI changes ( p < 0.05), suggesting a strict pathogenetic linkage between muscular and nervous system alterations in this disease.     

Multimodal evoked potentials in myotonic dystrophy (MyD).

By means of multimodal evoked potentials (EPs), we evaluated the central nervous system (CNS) involvement in 25 subjects suffering from myotonic dystrophy: brainstem auditory evoked potentials (BAEPs), middle-latency auditory evoked potentials (MLAEPs) and somatosensory evoked potentials (SEPs) from the upper limb were performed on all subjects, whereas only the 19 patients, whose clinical ocular abnormalities were slight, underwent pattern-electretinograms (PERGs) and pattern visual-evoked potentials (VEPs) in order to identify the site of lesion among visual pathways (retinal and/or retroretinal). PERGs were abnormal in 8/19 subjects, VEPs in 8/19 subjects (the two techniques were simultaneously abnormal in 8 eyes), BAEPs in 7/25 subjects, MLAEPs in 4/25 subjects (in one subject both BAEps and MLAEPs were abnormal) and SEPs were abnormal in 1/25 subjects. 13/25 of our subjects showed at least one EP that revealed a CNS involvement. The electrophysiological alterations were not correlated either with subject age or with disease duration. Multimodal EPs enabled us to demonstrate that CNS involvement in myotonic dystrophy is important and mainly affects the visual and auditory system.     

Central somatosensory conduction time and acoustic brainstem transmission time in post-traumatic coma

Short-latency evoked potentials (SEPs) of the scalp and neck after median nerve stimulation and acoustic brainstem evoked potentials (BAEPs) were recorded in 85 patients in post-traumatic coma with clinical signs of brainstem impairment between days 2 and 6 after trauma. The central somatosensory conduction time (CCT), the amplitude ratio (AR) N20:N13, the interpeak latencies (IPL) I-III, III-V, I-V, and the ARs between waves I and V (I:V) and between wave I and the wave IV/V complex (I:IV/V) were calculated and related to the outcome of the patients. In cases of coma due to supratentorial lesions, CCT and ARs of SEPs were close to normal in patients with good outcome: CCT increased and ARs decreased with worsening of outcome. In cases of primary brainstem injury, a significant prolongation of CCT was also seen in patients with good recovery, whereas normal CCTs could be found in patients with severe disability and death outcome. In this case, unilateral absent scalp SEPs were frequently found. The IPLs I-III, III-V, I-V, and the ARs of BAEPs increased with worsening of outcome. Significant differences of IPL I-V and III-V (brainstem transmission time) were seen between patients with good recovery or moderate disability outcome and the patients with severe disability or death outcome. There was no difference in BAEPs between patients with primary brainstem lesion and patients with secondary brainstem lesion. Patients with bilateral absent SEPs and bilateral absent BAEPs not related to traumatic or preexisting hearing disorders died or survived severely disabled. Unilateral absence of scalp SEPs and unilateral absence of BAEPs were frequently found in patients who died or who had severe disability. Asymmetries in scalp SEPs appeared to be distributed equally to all outcome categories, but asymmetries in BAEPs increased with worsening of outcome too. In most of the patients who died or survived disabled, both SEPs and BAEPs were abnormal.     

Evaluation of afferent pain pathways in adrenomyeloneuropathic patients

Objective

Patients with adrenomyeloneuropathy may have dysfunctions of visual, auditory, motor and somatosensory pathways. We thought on examining the nociceptive pathways by means of laser evoked potentials (LEPs), to obtain additional information on the pathophysiology of this condition.

Somatosensory and brainstem auditory evoked potentials in neurodegenerative system disorders

Brainstem auditory (BAEP) and somatosensory (SEP) evoked potentials to median and peroneal nerve stimulation were investigated in 25 patients with neurodegenerative system disorders: 9 Friedreich's ataxia, 7 hereditary motor sensory neuropathies, 3 familial spastic paraplegia, 3 olivopontocerebellar atrophy, 1 ataxia telangiectasia and 1 abetalipoproteinemia. BAEPs were abnormal in 39%, SEPs to both upper- and lower-limb stimulation were abnormal in 63%. Serial evoked potential testing paralleled the clinical progression. SEPs were more frequently and severely altered than BAEPs suggesting that SEP testing may be a more sensitive indicator of early involvement of afferent pathways in these disorders.     

Evoked potentials in basilar artery thrombosis: correlation with clinical and angiographic findings

In 28 patients with vertebro-basilar or basilar artery thrombosis brain-stem auditory evoked potentials (BAEPs) and somatosensory evoked potentials (SEPs) have been recorded. Visual evoked potentials (VEPs) were recorded in 7 of these 28 patients. In 24 patients the diagnosis was angiographically proven and in 4 patients Doppler sonography and computerized tomography suggested this diagnosis. The BAEP and SEP findings were correlated to clinical and angiographical signs. BAEPs could be classified into 6 different patterns. In more than half of the patients different BAEP patterns from the two ears could be found. A pathological IV/V complex was most often found in comatose patients and in patients with a basilar artery occlusion distal to the anterior inferior cerebellar artery. Prolonged interpeak latency of I-III was mainly found in alert or drowsy patients with caudal occlusions. The frequent occurrence of a BAEP with only wave I preserved, or with no waves preserved, in patients with brain-stem functions suggests that BAEPs are not useful in the diagnosis of brain death when basilar artery thrombosis is suspected. SEPs were either absent bilaterally or else severely altered on one side in all comatose patients. In alert patients, including those with 'locked-in' syndrome, SEPs were never absent bilaterally. Increased N13-N20 interpeak latency was an uncommon finding in this series. There was no correlation between the SEP and the angiographically proven location of the occlusion. In the 'locked-in' syndrome both SEP and BAEP findings were non-uniform. Normal SEPs were sometimes found in combination with severely altered BAEPs, suggesting partial deafferentation. Since basilar artery thrombosis is now a treatable condition, early diagnosis and documentation of functional deficits moves into a more important clinical area than heretofore.     

Evoked potentials in HIV infection]

The study of the literature data on the multimodal evoked potentials in HIV infected patients shows many abnormalities as well in asymptomatic subjects without AIDS as in AIDS subjects with or without neurological signs. Visual evoked potentials (VEPs) reveal prolonged P100 wave latency in 22% of HIV asymptomatic subjects and in 26% of HIV symptomatic subjects; brainstem auditory evoked potentials (BAEPs) reveal an increase of the interpeak latency I-V in 16% of asymptomatic subjects and in 32% of symptomatic subjects; somatosensory evoked potentials (SEPs) by median nerve stimulation reveal prolonged central conduction time in 6% of asymptomatic subjects and in 11% of symptomatic subjects; somatosensory evoked potentials (SEPs) by tibial nerve stimulation reveal prolonged central conduction time in 4% of asymptomatic subjects and in 45% of symptomatic subjects; motor evoked potentials (MEPs) by magnetic stimulation reveal prolonged central motor conduction time in 46% of asymptomatic subjects.     

Somatosensory evoked potentials: monitoring cerebral functions following liver transplantation

Serial somatosensory evoked potentials (SEPs) were obtained from a patient with Wilson's disease who had had liver transplantation. Correlating with recovery of hepatic encephalopathy and liver functions following the surgery, SEPs showed progressive normalization of peak latencies and waveforms. The data indicate that SEP may be a useful objective method in assessing recovery of cerebral functions following liver transplantation.     

Brain-stem auditory evoked potentials in different types of hepatic diseases

Brain-stem auditory evoked potentials (BAEPs) were studied in 69 patients with different types of hepatic diseases. BAEPs were normal in viral hepatitis, but the peak V and interpeak I-V latencies were prolonged in liver cirrhosis. Prolongation in peaks III, IV and V and interpeaks I-III, III-V and I-V were observed in both alcoholic liver disease and Wilson's disease with the latter more severely affected. The present data indicate that BAEPs may be used to study the effect of various hepatic diseases on the CNS.     

Brain-stem auditory evoked potentials in children with perinatal encephalopathies.

OBJECTIVE: We sought to describe if neurological damage, in terms of brain lesions, syndrome and syndrome severity led to abnormalities in the brain-stem auditory evoked potentials (BAEPs) in order to provide a profile of children that could be used as an indicator of subsequent neurological sequelae. We analyzed the BAEPs from a group of children having prior evidence of neurological damage and determined the presence of neurological sequelae when the subjects were 3 years old. METHODS: Brain-stem auditory evoked potentials (BAEPs) were carried out in a group of 154 children with perinatal neurological damage. The children were classified with neurofunctional (clinical and EEG alterations) or organic and neurofunctional brain disease (clinical, EEG and image alteration) and were all followed from the first month of life and serially for 3 years. We used principal component analysis (PCA), clustered analysis and linear correlation to determine association between BAEPs, risk factors and future sequelae. RESULTS: Latencies of BAEPs decreased significantly with age, and the time of conduction was modified by the presence of neurological damage. All statistical analyses suggested positive and significant associations between risk factors (trophism and condition at birth), and the latencies of waves I, III and V as well as with IPL III-V (interpeak latency) and I-V. PCA showed that IPL I-III was also positively associated with condition at birth, severity of the neurological syndrome and encephalopathy. In addition, we found that the presence and type of sequela reflected changes in the latencies of the waves, as well as IPLs, primarily those of IPL I-III. CONCLUSIONS: Our results suggest that statistical methods are often needed to analyze neurological damage. The relation between BAEPs, risk factors and neurological sequelae allowed us to obtain a profile of children, which can be then used as an aid in the prognosis of children having a risk of developing neurological sequelae.     

EVALUATION OF BRAINSTEM AUDITORY POTENTIAL IN BRUCELLOSIS PATIENTS WITH AND WITHOUT NEUROLOGICAL INVOLVEMENT

This study investigated the changes in brainstem auditory evoked potentials (BAEPs) in acute phase of brucellosis. Twenty-two patients with brucellosis without neurologic involvement and seven patients with neurobrucellosis were included. BAEPs were evaluated before treatment. No patient had a symptom of hearing loss. Patients who had neurologic involvement did not show any abnormalities in their BAEPs recording. This study suggests that patients with brucellosis who are in the acute phase of the disease with or without neurological involvement may have normal BAEPs. BAEPs do not seem to be a sensitive method for central nervous system involvement of brucella patients in the acute phase of the disease.     

Evaluation of brainstem auditory potential in brucellosis patients with and without neurological involvement

This study investigated the changes in brainstem auditory evoked potentials (BAEPs) in acute phase of brucellosis. Twenty-two patients with brucellosis without neurologic involvement and seven patients with neurobrucellosis were included. BAEPs were evaluated before treatment. No patient had a symptom of hearing loss. Patients who had neurologic involvement did not show any abnormalities in their BAEPs recording. This study suggests that patients with brucellosis who are in the acute phase of the disease with or without neurological involvement may have normal BAEPs. BAEPs do not seem to be a sensitive method for central nervous system involvement of brucella patients in the acute phase of the disease.