Visual and auditory evoked potentials in the early diagnosis and follow-up of neurosarcoidosis

Visual and brainstem auditory evoked potentials (VEPs, BAEPs) were recorded in 23 patients with neurosarcoidosis. Eight patients (35%) had abnormal BAEPs, and 10 (43%) had abnormal VEPs. Four of the 8 patients with abnormal BAEPs had facial paresis, one had impaired memory and only 3 had symptoms and signs compatible with brainstem lesion. Seven of the patients with abnormal VEPs had no visual symptoms. These findings suggest that BAEP and VEP can reveal subclinical nervous system involvement in sarcoidosis and can also help in the early diagnosis of neurosarcoidosis. Successive recordings of 5 patients showed that BAEP and VEP were useful in the follow-up of these patients.     

Brain-stem auditory evoked potentials and blink reflex in friedreich's ataxia

Summary The brain-stem involvement in Friedreich's ataxia (FA) was studied by using brain-stem auditory evoked potentials (BAEPs) and the blink reflex. Ten out of 18 patients had abnormal BAEPs, the main abnormality being complete absence of responses and disappearance of wave V. Combined degeneration of the peripheral and central acoustic pathways probably accounts for these findings. The blink reflex was abnormal in 50% of the cases. The outstanding abnormality was bilateral delay of late responses with normal early response, which could be correlated with the known pallor of the descending trigeminal tracts. In contrast with BAEP findings, blink reflex abnormalities did not correlate with either the age of patients or the severity and duration of the disease. These data suggest a difference in susceptibility to degeneration between the auditory system and neuronal system subserving the blink reflex. We conclude that systematic BAEP and blink reflex recording is useful in the electrophysiological evaluation of FA patients.     

Evoked potentials in multiple system atrophy (MSA)

OBJECTIVES: To study the involvement of pyramidal tracts and sensory pathways in multiple system atrophy (MSA). MATERIALS AND METHODS: Evoked potential studies were performed in 45 MSA patients suffering from either MSA of cerebellar type (MSA-C) or MSA of parkinsonian type (MSA-P). RESULTS: Motor evoked potentials were normal in all MSA patients, whereas visual and somatosensory evoked potential abnormalities were found in about 40% of the MSA patients with no significant difference between the cerebellar (MSA-C) and parkinsonian (MSA-P) subgroup. Abnormal latencies of wave III in brainstem auditory evoked potentials were significantly more frequent in MSA-C. CONCLUSIONS: Abnormalities of somatosensory, visual and auditory evoked potentials are frequent findings in MSA, whereas abnormal motor evoked potentials are not a characteristic feature of the disease.     

Middle latency auditory evoked potentials improve the detection of abnormalities along auditory pathways in multiple sclerosis patients.

Brain-stem and middle latency auditory evoked potentials (BAEPs and MLAEPs) have been studied in 34 multiple sclerosis (MS) patients. We were able to detect a central nervous system auditory pathway involvement in 17 (50%) of the patients: 38% by BAEPs alone (I-V inter-peak latency) and 47% by MLAEPs alone (Na and Pa peak latency). Five patients had abnormal MLAEPs with normal BAEPs whereas the opposite was detectable in only 1 patient. In addition, most MLAEP parameters in the MS group statistically differed from those obtained in the control group. Therefore, our results demonstrated that the auditory pathway impairment could frequently be located at a rostral level along the auditory radiation. In conclusion, even if only Na and Pa components were considered, MLAEPs succeeded in improving the sensitivity of the auditory evoked potential examination without increasing the false positive rate.     

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.     

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.     

脑干听觉诱发电位及体感诱发电位在重型颅脑损伤患者预后评估中的作用

目的 分析探讨脑干听觉诱发电位(BAEP)及体感诱发电位(SEP)与重型颅脑损伤病人预后的关系.方法 对33 例重型颅脑损伤患者早期行BAEP 及SEP 测定并进行动态监测,同时行头颅CT 检查并记录GCS 评分.结果 BAEP 及SEP 预测预后的敏感性、特异性、准确性均较高.BAEP、SEP 异常程度低,则预后较好;异常程度高,则预后不良.结论 BAEP、SEP 可以比较准确地评估重型颅脑损伤患者的预后.     

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.     

Detection of brainstem lesions in multiple sclerosis: comparison of brainstem auditory evoked potentials with nuclear magnetic resonance imaging

Topographical information provided by brainstem auditory evoked potentials (BAEPs) was investigated in 43 patients by comparison with cerebral nuclear magnetic resonance imaging (NMR). Lesions in the region of the brainstem auditory pathways were demonstrated by BAEPs in 44.2%, and in 39.5% by NMR. As regards brainstem levels, in 15/21 (71.4%) with abnormal findings at least one lesion was verified by NMR-matched BAEP results. The study confirms the topographical information provided by the BAEPs on the different levels of the brainstem, but not the assumption that generation of the BAEPs is predominantly ipsilateral. BAEPs retain their importance for the detection of disseminated lesions in the diagnosis of multiple sclerosis (MS) in the era of expensive imaging methods.     

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.     

Prolonged brainstem transmission time in autism

Brainstem auditory evoked potentials (BAEPs) to clicks presented monaurally were gathered for 16 institutionalized children with a prior diagnosis of autism and with no hearing loss as tested by standard audiometry. Twenty age-matched normal children served as controls. Brainstem transmission time, defined as BAEP interpeak I-V latency, was prolonged significantly on the average in the autistic sample on both left and right sides. Individually, BAEPs for 9 of the 16 autistic children (or 56%) on whom some electrophysiological data were gathered were classified as abnormal when compared to the norms established in the control group. The most common BAEP abnormality was a prolonged interpeak III-V latency on the left side.     

The application of evoked potentials in the diagnosis and follow-up of children with intracranial tumors

Somatosensory and brainstem auditory evoked potentials (SSEPs and BAEPs) provide sensitive measures of the central conduction functions of the auditory and somatosensory input systems at different levels of the central nervous system. The contribution of SSEPs and BAEPs in the primary diagnosis and follow-up of 26 children with infra- and supratentorial tumors were reviewed. The SSEPs, especially the specific complex, showed a latency increase in patients with supratentorial and brainstem mass lesions involving directly or distantly the somatosensory tracts. The BAEPs were sensitive for supratentorial pressure effects and for local and distant posterior fossa tumor effects. In the follow-up of children, evoked potentials offer a good method of detecting tumor recurrence, whereas neuroradiological procedures may be obscured by surgical or radiation artifacts.This paper has been presented in part at the Symposium of the European Federation of Child Neurology Societies, June 1983     

Brainstem Auditory and Somatosensory Evoked Potentials in Neuro-Behqet's Syndrome

Abstract: Brainstem auditory evoked potentials (BAEPs) and somatosensory evoked potentials after median nerve stimulation (MN-SEPs) and after posterior tibial nerve stimulation (PTN-SEPs) were studied in 17 patients with neurolehget's syndrome (NB). Eleven patients (64.7%) showed an absence of wave I, III or V or a prolongation of the interpeak latency 1–111, or 111-V in BAEPs. Six patients (37.4%) showed a prolongation in the latency of cortical P37 of PTN-SEPs and/, or the interpeak latency EP-N13 or N13–N18 of MN-SEPs. The BAEP and SEP abnormalities indicated a conduction failure of the acoustic lateral lemniscus pathway and the medial lemniscus pathway in the brainstem of the patients with NB. Abnormal EPs can provide sensitive information which shows the presence of subclinical lesions in the central nervous system.     

Brain-stem involvement in multiple sclerosis: a comparison between brain-stem auditory evoked potentials and the acoustic stapedius reflex

Summary Brain-stem auditory evoked potentials (BAEPs) and the acoustic stapedius reflex (ASR) were recorded in 68 patients with definite, probable and possible multiple sclerosis (using the definitions of McAlpine). The high incidence of abnormal results, 68% and 60%, respectively, pointed to the diagnostic value of these two measures in detecting brain-stem dysfunction. Combination of the methods increased the diagnostic yield to 85%. Since in part the same brain-stem generator sites underlie BAEPs and the ASR, it was considered that a study of their correlation might serve to increase the reliability and validity of these techniques. There was 71% agreement overall between results from the two measures. Furthermore, 72% of the joint BAEP and ASR abnormalities corresponded in detection of the brain-stem lesion site. It was concluded that the combined approach may supply powerful, complementary information on brain-stem dysfunction, which may aid in establishing the diagnosis of multiple sclerosis.This study was supported by the M. Sackler Foundation for Multiple Sclerosis Research     

Neurophysiological and neuro-otological study of homozygous beta-thalassemia under long-term desferrioxamine (DFO) treatment

This report presents data on visual evoked potentials (VEPs) and brainstem auditory evoked potentials (BAEPs), as well as neurologic, ophthalmologic and otologic assessments performed on 120 patients with beta-thalassemia major undergoing long-term DFO treatment. A total of 32 patients showed abnormal VEPs and 14 abnormal BAEPs; seven had both VEP and BAEP abnormalities; 12 had sensorineural hearing loss (SNHL); 18 had conductive hearing loss, while 14 showed a combination of SNHL and conductive hearing loss. After DFO administration was modified (taking in consideration the serum ferritin levels) patients with abnormal findings were retested. The values of 15 patients of 23 who underwent VEP examinations had been normalized. Eleven of 15 who repeated the BAEP test had also gained normal values. The audiogram had not returned to normal in any patient with SNHL. In a second repetition of the examinations, no change was observed. It is concluded that in a great percentage of thalassemics at least one of the above examinations shows abnormal values. These abnormalities are mostly reversible, and probably reflect a dysfunction of the visual or auditory system, due either to DFO neurotoxicity or to iron overload or both.     

Magnetic resonance imaging and brain-stem auditory evoked potentials in neuro-Behcet's disease

We studied central nervous system lesions in patients with neuro-Behcet's disease using magnetic resonance imaging (MRI) of the brain and recording of brain-stem auditory evoked potentials (BAEPs). MRI revealed abnormal findings in seven of eight patients. MRI studies demonstrated extensive regions with high intensity signal in the brain stem and/or basal ganglia on T2-weighted images obtained during the acute stage of the disease in three patients. One of these patients had a strongly gadolinum-enhanced round lesion in the lower pons. In four of the other five patients with chronic disease, brain-stem atrophy was observed on T1-weighted images. Atrophic changes were more severe in the brain stem than in the cerebellum. Abnormal BAEPs were observed in three patients and consisted of prolongation of interpeak latency of waves III-V and defects of wave III or V. Abnormal BAEPs were recorded in patients with severe inflammatory changes or progression of atrophic changes in the brain stem. Our findings show that MRI and BAEPs are useful in detecting the presence and assessing the degree of neurological involvement in patients with neuro-Behcet's disease.     

Evoked potential abnormalities in progressive spastic paraparesis associated to HTLV-1

The electrophysiological features of progressive spastic paraparesis (PSP) associated with HTLV-1 in Chile, a non-tropical country, are presented. Twenty-two of 45 PSP subjects were positive for HTLV-1 antibodies. Trimodal evoked potentials were all normal in only 2 of these cases (9.1%). Somatosensory evoked potentials (SSEPs) were abnormal in 19 patients (86.3%) with a mean amplitude of 1.31 uV SD 0.92. Visual evoked potentials (VEPs) and brainstem auditory evoked potentials (BAEPs) were normal in all patients except four. Peripheral nerve conduction was normal in all but one who showed discrete slowness of the motor conduction velocity in the peroneal nerves. EMG was normal in 15 cases in whom it was performed. SSEP's were abnormal in 2 (8.7%) of 23 HTLV-1 negative cases with a mean amplitude of 2.4 uV SD 1.5, which is statistically different with respect to the positive cases (p less than 0.001). These results support an involvement of the spinal cord not restricted to the pyramidal tracts but also including subclinical damage of the posterior columns in PSP associated to HTLV-1.     

实验性胸部挤压伤的脑诱发电位研究

本文探索胸部挤压伤后的体感诱发电位(SEPs)和脑干听觉诱发电位(BAEPs)改变。实验以16只健康杂种犬为对象,通过制成挤压伤模型,在各时间点记录上述两种诱发电位。结果:发现挤压伤初期BAEPsⅠ波不受影响,Ⅲ、Ⅴ波延长;后期三波均受累。三波消失揭示脑功能衰竭;SEPs早期5个波潜伏期延长,晚期均消失。将两种检测方法结合对判断伤情、估计预后更有帮助。     

Effects of carbamazepine and valproate on brainstem auditory evoked potentials in epileptic children

Brainstem auditory evoked potentials (BAEPs) were recorded in 18 epileptic children receiving carbamazepine and 10 epileptic children receiving valproate. BAEPs were recorded before the administration of antiepileptic drugs (AEDs) and 13 months later during which the children received AEDs. Statistical analysis of peak latencies and interpeak intervals of waves I–III–V were made. Carbamazepine treatment resulted in prolongation of peak latencies of waves I–III–V and interpeak intervals I–III and I–V. Valproate monotherapy, on the other hand, caused no consistent changes on BAEP. On the basis of these results we suggest that chronic carbamazepine therapy exerts a suppressive influence on the auditory pathways, both peripherally at the level of the cochlea and/or auditory nerve, and centrally at the brainstem.     

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.