Cranial nerve function in workers exposed to polychlorinated dioxins and furans

OBJECTIVE: To look for possible effects of polychlorinated dioxins and furans (PCDD/F) on cranial nerve function. MATERIAL AND METHODS: Clinical and neurophysiological examinations [visual and brainstem auditory evoked potentials (VEP and BAEP), blink reflex] in 121 PCDD/F exposed workers of one pesticide producing plant. RESULTS: BAEP abnormalities were more frequent in workers with chloracne (6 of 33 workers, 18.2%) than in those without chloracne (7 of 84, 8.3%), but this was not statistically significant (chi2: 2.33). VEP abnormalities were seen in one worker with and two without chloracne. Clinically visual functions were normal except in one worker, who was amaurotic since birth. Blink reflex abnormalities without corresponding clinical findings were observed in two patients without chloracne. CONCLUSION: Severe exposure to PCDD/F is not followed by clinical signs of cranial nerve dysfunction but may create an increased risk for abnormal BAEP findings, which were more than twice as common in workers with chloracne. Although this difference did not reach statistical significance, it cannot exclude a toxic effect of PCDD/F, as statistical significance is difficult to achieve with such small numbers of workers. In none of the workers, BAEP abnormalities were accompanied by clinical signs of hearing dysfunction.     

Brain stem auditory and visual evoked potentials in multiple sclerosis

The diagnostic value of the checkerboard pattern-reversal visual evoked potential (VEP) and the random, low rate stimulated brain stem auditory evoked potential (BAEP) was compared in 99 patients with established or suspected multiple sclerosis (MS). In normal subjects examined by both techniques no abnormal recordings were found. In 49 patients with definite MS an incidence of abnormality was found in 100% of VEP and in 84% of BAEP recordings. In 50 patients with probable or possible MS an abnormal VEP was found in 70% and an abnormal BAEP in 50%. When the two examinations were combined, the diagnostic yield increased to 100 and 80%, respectively. 22 patients had only spinal symptoms; in these the VEP gave 73%, the BAEP 55% and the combination 82% abnormalities. The combination of the two techniques was found useful for demonstrating demyelinating lesions in the central nervous system, the diagnostic value being greatest when these lesions were clinically silent.     

Neurophysiologic assessment of mucopolysaccharidosis III.

OBJECTIVE: To describe finding of various neurophysiologic tests in patients with mucopolysaccharidosis III (MPS III) early in the disease course. METHODS: Patients were evaluated with flash visual evoked potentials (VEP), brainstem auditory evoked potentials (BAEP), electroencephalography (EEG), and nerve conduction studies (NCS) before they underwent hematopoietic stem cell transplantation (HSCT). RESULTS: Thirteen children underwent at least one neurophysiologic test before HSCT. The mean age at testing was 2.7 years. Ten of 11 (91%) patients had a normal flash VEP, and all 9 who had BAEP had normal central conduction. EEG was normal in 7/13 (54%), with the others showing diffuse slowing. NCS was normal in 10/11 (91%) patients. CONCLUSIONS: Despite extensive central nervous system involvement in MPS III, flash VEP and BAEP are almost always normal. EEG is often abnormal early in the disease. SIGNIFICANCE: This is the first report of neurophysiologic tests in a large series of MPS III patients.     

Electrophysiological study in patients with chronic hepatic insufficiency

Twenty patients affected by chronic hepatic insufficiency were studied by means of combined EEG, VEP and BAEP recordings. Subjects were classified, according to Parsons-Smith criteria, in grade 0 (without any clinical sign) and grade I (with minimal mental disturbances). In group 0 patients an elevated incidence of VEP alterations (40%) was observed, while EEG and BAEP recordings were less frequently (10%) abnormal. In group I patients a high degree of EEG (60%), VEP (70%) and BAEP (70%) abnormalities was found. Mean latency and interpeak interval values in group I patients were significantly prolonged if compared with the grade 0 and control groups. No correlations were found between electrophysiological data and blood biochemical parameters examined (ammoniemia, aminoacidemic pattern).     

Neurophysiologic and intellectual evaluation of beta-thalassemia patients

In order to detect involvement of the central and peripheral nervous system in beta-thalassemic patients, 32 children and young adults (mean age 14.5 +/- 6.4 years) participated in a systematic neurophysiologic and intellectual prospective study. All patients were in a regular transfusion program, receiving subcutaneous desferrioxamine chelation and maintaining a mean serum ferritin level of 2,101.56 +/- 986.32 ng/ml. Study patients underwent neurophysiologic evaluation consisting of brainstem auditory, visual and somatosensory evoked potential examination (BAEP, VEP, SEP) as well as motor and sensory nerve conduction velocity studies (MCV, SCV). Additionally, the verbal, performance and total IQ were assessed in patients under 16 years of age using the Weschler Intelligence Scale for Children (WISC-III). The incidence of abnormal BAEP, VEP, SEP and NCVs was 0, 3.12, 3.12 and 18.75%, respectively, findings comparative to or better than previously reported. On the contrary, the prevalence of abnormal total IQ score was considerably high (36.4%), not correlating, however, to any of the parameters assessed (age, sex, ferritin level, BAEP, VEP, SEP, NCV). Factors associated with chronic illness, rather than the disease per se, could play a potential role in the development of cognitive dysfunction in beta-thalassemia patients.     

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.     

Visual, early auditory and somatosensory evoked potentials in multiple sclerosis (917 cases)

Visual (VEP), brainstem auditory (BAEP) and somatosensory (SEP) evoked potentials were recorded over a 6 year period in 917 patients with or suspected of multiple sclerosis according to Mc Alpine's criteria. Evoked potentials provided information of diagnostic relevance in detecting clinically unsuspected lesions (spatial dissemination). They also gave valuable informations in patients with atypical or borderline clinical features. When abnormal, VEP indicated clinically silent lesions in 45.1 p. 100 of patients with definite MS, 66 p. 100 of those with probable MS and 78 p. 100 of the possible MS. Less than 15 p. 100 of SEP and/or BAEP abnormalities were found in 83 patients with a simple or recurring retrobulbar optic neuritis. Thirteen patients with acute transverse myelopathy and no prior history of neurological disease were studied. All had normal visual and brainstem auditory evoked potentials. Abnormal VEPs helped to the clinical assessment of 88 patients with progressive spastic paraparesis 46,6 p. 100 of whom had abnormal VEPs demonstrating disseminated lesions and 36,1 p. 100 had abnormal BAEPs. The frequency of the various types of VEP, BAEP and SEP abnormalities was studied as well as their course on repeated recordings. Results of multivariate analysis are given. It was found that the longer the time interval between the first MS relapse and the evoked potential recording, the higher the incidence of abnormalities. The incidence of evoked potentials abnormalities was lower in patients with normal CSF and higher in patients with inflammatory CSF. The abnormalities were more frequent when patients had clinical evidence of lesions of the sensory pathways explored by the tests.     

Long-term impairments of brain and auditory functions of children recovered from purulent meningitis

Sixty children who had recovered from purulent meningitis one to six years earlier were investigated for long-term impairment of brain and auditory function, using brainstem auditory evoked potentials(BAEP) and developmental screening tests. Neurological and/or audiological BAEP abnormalities were found in 23 per cent of the children: 15 per cent had mild brainstem impairment and 12 per cent had hearing dysfunction. Developmental screening tests were administered to 46 children, of whom 61 per cent had normal, 22 per cent questionable and 17 per cent abnormal results. The results of the BAEP significantly correlated with those of the developmental screening tests, suggesting that the neuropsychological development of children with BAEP abnormalities was significantly delayed compared with that of children without BAEP abnormalities. The characteristic finding in a neurologically abnormal BAEP was slightly depressed amplitude of wave V, and the authors suggest that this is the most sensitive BAEP measure for the assessment of brainstem function in children recovered from meningitis.     

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.     

Visual and auditory evoked potentials during long-term vigabatrin treatment in children and adolescents with epilepsy.

OBJECTIVES: The aim of the study was to estimate the effects of Vigabatrin (VGB) as add-on therapy on visual (VEP) and brain-stem (BAEP) evoked potentials. METHOD: The investigation covered 100 epileptic patients from 8 to 18 years of age. The treatment included therapy with carbamazepine (CBZ) or valproate acid (VPA) using slow release formulations of these AEDs. Combination therapy was administered using add-on VGB in the recommended dose 57.4+/-26.5 mg/kg body mass/day. VEP and BAEP evoked potentials were recorded by means of Multiliner (Toennies, Germany). The obtained values were compared with age matched control group. RESULTS: Compared to control groups, significant differences in epileptic groups emerged in latencies of the peak III, V along with the interpeak intervals I-III of BAEP. Also VEP studies showed the reduction of N75/P100 and P100/N145 amplitudes. CONCLUSIONS: Adding VGB did not significantly increase the percentage of pathological abnormalities observed from EPs. Our electrophysiological studies demonstrate abnormalities in EPs parameters due to subclinical toxicity induced by AEDs. Major alterations produced bitherapy of VPA-SR + VGB and minor SR formulations of CBZ or VPA.     

Brain-stem auditory evoked potentials in multiple sclerosis: the relation to VEP,SEP and CSF immunoglobulins

Summary One hundred patients with multiple sclerosis (MS) were analysed retrospectively with respect to investigations of brain-stem auditory evoked potentials (BAEP), pattern reversal visual evoked potentials (VEP), somatosensory evoked potentials (SEP), and cerebrospinal fluid immunoglobulins (CSF-IG). BAEP were abnormal in 42% of those with normal VEP and SEP examinations, and in 38% of patients with normal CSF-IG. The chance of obtaining at least one abnormal EP was lower in patients with normal CSF-IG than in patients with abnormal CSF. When a dispersion ratio was included in the criteria for BAEP abnormality, the sensitivity increased compared with conventional BAEP criteria. We recommend that BAEP should still be included in the EP test battery for patients with suspected MS.     

Relation of optic myelitis (OM) and multiple sclerosis (MS)

Brainstem auditory evoked potentials (BAEP) were studied on 38 optic myelitis (OM) and 16 of myelopathy with definite abnormal VEP. Forty-two normal subjects were studied for comparison. The results showed that BAEP were abnormal in 42% of the OM and 38% of the latter myelopathy groups. Among the abnormal BAEP 82% showed V wave abnormality, 32% prolongation in I-III or III-V interpeak latency or absence of III wave, and 59% with unilateral lesions. These findings would imply that the lesions were mostly on the white matter and small and localized in character which was compatible with the pathology of MS. 41% of BAEP abnormality in these two groups showed a lesion would be in the brainstem in almost half of these two types of disorders. OM would be most likely a clinical variant type of MS and not a unique disease.     

Electrophysiological (EEG, BAEP, VEP) study in patients with beta-thalassemia major

Twenty-four patients affected by beta-thalassemia major were studied by means of combined EEG, VEP and BAEP recordings. All the subjects were treated with regular blood transfusions and chelating therapy (DFO). An elevated incidence of EEG abnormalities (70.8%) consisting of diffused slow waves and/or diffused small sharp spikes was seen. VEP P100 latency was abnormally prolonged in eight patients (33.3%). Furthermore, a voltage increase of N75-P100 (29%) and P100-N145 (33.3%) VEP components was observed. Mean latency and voltage values were significantly increased when compared with those of a control group. No BAEP alterations were observed. No correlations were found between electrophysiological data, serum ferritin levels and transfusional treatment duration. The possible mechanisms involved in provoking such electrophysiological abnormalities are discussed.     

Evoked potential abnormalities in the various inherited ataxias

Visual (VEP), brainstem auditory (BAEP), and somatosensory (SEP) evoked potential tests were performed in 45 patients representing ten types of inherited disorders in which ataxia was the most prominent symptom. Comparable VEP abnormalities were present among all types of patients. Normal BAEP tests were recorded in most patients except those with olivopontocerebellar atrophy. SEP results were often more severely abnormal in patients with Friedreich's ataxia. The observations emphasize the similarity in expression of different metabolic-degenerative disorders. When these tests are used clinically, certain features of evoked potentials (especially left-right symmetry) are typical of the inherited ataxias as a group. Few distinguishing features differentiate the individual disorders.     

多发性硬化患者的MRI及多种诱发电位研究

目的探讨磁共振成像(MRI)和诱发电位(EPs)在诊断多发性硬化中的价值。方法对68例多发性硬化患者的头颅MRI、脑干听觉诱发电位、视觉诱发电位以及体感诱发电位等指标进行回顾性分析和比较。结果多发性硬化患者的头颅MRI、脑干听觉诱发电位、视觉诱发电位以及体感诱发电位的异常率分别为91.2%(62/68)、80.9%(55/68)、82.4%(56/68)和77.9%(53/68),且均发现多发性硬化的亚临床病灶;两项或多项联合检查的异常率较单项检查的异常率增高,差异有统计学意义(P<0.01)。结论头颅MRI和诱发电位检查有助于临床早期确诊多发性硬化,联合应用可使其敏感性提高。     

Electroencephalogram and brainstem auditory evoked potential in 539 patients with central coordination disorder

BACKGROUND： Electroencephalogram （EEG） and brainstem auditory evoked potential （BAEP） are objective non-invasive means of measuring brain electrophysiology. OBJECTIVE： To analyze the value of EEG and BAEP in early diagnosis, treatment and prognostic evaluation of central coordination disorder. DESIGN, TIME AND SETTING： This case analysis study was performed at the Rehabilitation Center of Hunan Children＇s Hospital from January 2002 to January 2006. PARTICIPANTS： A total of 593 patients with severe central coordination disorder, comprising 455 boys and 138 girls, aged 1-6 months were enrolled for this study. METHODS： EEG was monitored using electroencephalography. BAEP was recorded using a Keypoint electromyogram device. Intelligence was tested by professionals using the Gesell scale. MAIN OUTCOME MEASURES： （1） The rate of abnormal EEG and BAEP, （2） correlation of abnormalities of EEG and BAEP with associated injuries, （3） correlation of abnormalities of EEG and BAEP with high risk factors. RESULTS： The rate of abnormal EEG was 68.6% （407/593 patients）, and was increased in patients who also had mental retardation （P 〈 0.05）. The rate of abnormal BAEP was 21.4% （127/593 patients）. These 127 patients included 67 patients （52.8%） with peripheral auditory damage and 60 patients （47.2%） with central and mixed auditory damage. The rate of abnormal BAEP was significantly increased in patients who also had mental retardation （P 〈 0.01 ）. Logistic regression analysis showed that asphyxia （P 〈 0.05）, jaundice, preterm delivery, low birth weight and the umbilical cord around the neck were closely correlated with abnormal EEG in patients with central coordination disorder, lntracranial hemorrhage, jaundice （P 〈 0.05）, low birth weight and intrauterine infection （P 〈 0.05） were closely correlated with abnormal BAEP in patients with central coordination disorder. CONCLUSION： Central coordination disorder is often associated with abnormal EEG an     

Evoked potentials in the Rett syndrome

In order to have an electrophysiological approach to the pathogenesis of the Rett syndrome (RS), EEG testing together with evoked potential studies were performed in five children with RS, observed in our hospital during the last three years. All of the patients, aged from 18 months up to 4.5 years, had abnormal EEG: normal background activity with paroxysmal, epileptiform discharges was seen. In contrast with the abnormal EEGs, the evoked potentials were normal in each patient: brainstem auditory evoked potentials (BAEP), visual evoked potentials (VEP), and central conduction time (CCT) of somatosensory evoked potentials (SSEP). These findings together with the few previous ones suggest a predominantly gray matter pathophysiology in the early stages of RS.     

Brain-stem auditory evoked potentials in children with brain-stem or cerebellar dysfunction

Brain-stem auditory evoked potentials (BAEPs) were recorded in 23 children who had signs of brain-stem or cerebellar dysfunction. In patients with brain-stem gliomas, BAEPs were abnormal in all except one, in whom involvement of the brain-stem auditory pathway was limited to the midbrain tectum. The BAEPs were normal in neuronal ceroid lipofuscinosis, but abnormal bilaterally in inheritable leukoencephalopathies. All patients with Leigh's encephalopathy had BAEP abnormalities; in two, abnormalities occurred before the appearance of lesions on computed tomographic scan. Patients with Friedreich's ataxia and giant axonal dystrophy had abnormal BAEPs, but the test was normal in a child with similar neurologic findings with vitamin E deficiency. Patients with diffuse metabolic encephalopathies had variable findings. Thus, BAEP abnormalities are nonspecific for various disease processes but are frequently seen in neoplastic and neurodegenerative diseases, with primary white matter or extensive brain-stem involvement.     

Diagnostic value of electroencephalography and brainstem auditory evoked potentials in dizziness

Electroencephalogram (EEG) and brainstem auditory evoked potentials (BAEP) were recorded from 142 dizzy patients. The results were compared with those obtained by other neurological examinations of the same patients. Thirty-three percent (N = 44) of the patients had an abnormal EEG: 21% (28) showed focal abnormality, 12% (16) irritative features, and 10% (13) generalized disturbance. There were no differences in the distribution of abnormal EEGs in different diagnostic categories of dizziness, but irritative findings were not found in the cases of peripheral vestibular or psychogenic disturbances. EEG findings in 3 patients suggested temporal epilepsy, correlating with the clinical picture. BAEPs were abnormal in 18% (N = 21) of the recordings. There were 3 cases of MS and 5 ischaemic lesions in the vertebro-basilar region. Twelve of the 13 other patients with abnormal BAEP showed evidence of CNS pathology with other methods. EEG abnormalities were mostly non-specific; however, the irritative findings suggested cerebral pathology, such as epilepsy. An abnormal BAEP offers reliable evidence for brainstem lesion and is thus a useful examination in dizziness.     

Visual evoked potentials in high-risk infants

Flash VEPs were recorded in 109 high-risk infants, and the result were compared with the clinical outcome of the infants at the age of one year. 87 of the infants (80%) had a normal outcome and also seemed to have normal VEP maturation. This material was used as a reference for infants with abnormal outcome. Altogether, 20 infants (18%) had abnormal VEPs. In most of these repeated VEPs were recorded. In 70 cases the first VEP was recorded at an age of less than three months. Among these 57 children had normal outcome, with abnormal VEPs in 8 cases (14%). 13 infants who had an abnormal outcome had abnormal VEPs in 7 cases (54%). 7 infants of them had poor outcome, and they had abnormal VEPs in 6 cases (86%). The difference between normal and abnormal outcome was statistically significant. The present results indicate that it is possible to predict the poor outcome but not the moderate abnormality by VEP. The absence of VEP or its abnormal wave form were the most important parameters to predict the prognosis. Our present opinion is that VEPs should be recorded selectively, e.g. according to the findings in ultrasound examination, at least twice, the first time as soon as possible after birth and the second time at the age of two months.