Normal conduction in pathways traversing an asymptomatic multiple sclerosis plaque.

A 31-year-old woman developed right facial myokymia as the initial manifestation of multiple sclerosis (MS). An MRI scan revealed a focal signal abnormality confined to the left dorsolateral pontomedullary region. Brain-stem auditory evoked potentials (BAEPs), somatosensory evoked potentials (SEPs), and blink reflex (BR) failed to show a conduction abnormality through the left brain-stem lesion. Instead, BAEP and BR indicated a conduction defect in the right pons and EMG showed myokymic discharges in right facial muscles. Our findings provide rare documentation of normal conduction through a presumably asymptomatic MS plaque. The abnormal MRI signal likely represents tissue edema, rather than demyelination. This case demonstrates that physical findings in MS patients may correlate better with electrophysiological abnormalities than with MRI abnormalities.     

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.     

Middle latency auditory evoked potentials (MLAEPs) in (MS)

Middle latency auditory evoked potentials (MLAEPs) were studied in 30 definite multiple sclerosis (MS) patients in addition to brain-stem auditory evoked potentials (BAEPs). BAEP abnormalities were detected in 18 (60%) patients. MLAEPs were abnormal in 22 (73%) of them. In 15 patients BAEPs and MLAEPs were both abnormal. MLAEPs were found abnormal in 7 of the 12 patients with normal BAEPs. In 18 patients with abnormal BAEPs only 3 had normal MLAEPs. MLAEPs abnormalities are consistent with a rostral auditory pathway involvement. Therefore, they can be used in combination with BAEPs to examine the whole auditory system to improve the sensitivity.     

Evoked potentials and EEG in multiple sclerosis

Thirteen patients with clinically definite multiple sclerosis (MS) were studied with electroencephalogram (EEG), magnetic resonance imaging (MRI), evoked potentials and cerebrospinal fluid (CSF) analysis. We attempted to correlate the findings with physical disability as defined by Kurtzke score and presence of dementia or seizures. More severe plaque disease on MRI and increased physical disability correlated significantly with abnormality on brain-stem auditory evoked potentials (BAEPs) while visual evoked potential (VEP) abnormality correlated only with MRI findings. No such correlation was found with the EEG. The close relationship between BAEP and MRI abnormalities probably reflects frequent involvement of brain-stem corticospinal pathways.     

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     

Brain-stem compression in vertebrobasilar dolichoectasia. A multimodal electrophysiological study.

OBJECTIVE: To evaluate the effects of mechanical compression of the brain-stem in patients with vertebrobasilar dolichoectasia (VBD). METHODS: In the framework of a prospective, observational study that collected clinical and laboratory data in patients with VBD, we studied 20 patients with compression of the brain-stem due to ectatic, tortuous basilar or vertebral arteries. Patients with cerebral lesions other than small lacunae in the white matter of the cerebral hemispheres were excluded from the study. Patients underwent vestibular and auditory function testing, including brain-stem auditory evoked potentials (BAEPs), blink reflex (BR), somatosensory evoked potentials (SEPs), and motor evoked potentials (MEPs). RESULTS: Almost all of the patients complained of auditory or vestibular symptoms and none had symptoms or signs of impairment of long tracts or the facial and trigeminal nerves. The most consistent findings were BR abnormalities with prolongation of ipsilateral R1 latency in cases of compression of the pons (10/16) and prolongation of the R2 and R2c latencies with compression of the medulla oblongata (5/15). Subclinical impairment of corticospinal pathways was found in 13 out of 25 instances of compression, and this was more frequent with compression of the pons. Abnormal BAEPs or SEPs were less frequently encountered, and only in cases with compression of the pons. CONCLUSIONS: Neurovascular compression of the brain-stem, even with severe distortion, is seldom associated with overt clinical signs, whereas subclinical dysfunctions are relatively frequent. The central pathways of the BR and the corticospinal pathways are more susceptible to compression than acoustic and sensory pathways. BR, MEP and BAEP data provide a functional evaluation of the brain-stem and some cranial nerves, which is lacking in imaging studies. Functional investigations may be useful in the long-term management of these patients, since VBD may be progressive and surgical correction may be required at some stage.     

Pontine lesions mimicking acute peripheral vestibulopathy

OBJECTIVES: Clinical signs of acute peripheral vestibulopathy (APV) were repeatedly reported with pontine lesions. The clinical relevance of such a mechanism is not known, as most studies were biased by patients with additional clinical signs ofbrainstem dysfunction. METHODS: Masseter reflex (MassR), blink reflex (BlinkR), brainstem auditory evoked potentials (BAEPs), and DC electro-oculography (EOG) were tested in 232 consecutive patients with clinical signs of unilateral APV. RESULTS: Forty five of the 232 patients (19.4%) had at least one electrophysiological abnormality suggesting pontine dysfunction mainly due to possible vertebrobasilar ischaemia (22 patients) and multiple sclerosis (eight patients). MassR abnormalities were seen in 24 patients, and EOG abnormalities of saccades and following eye movements occurred in 22 patients. Three patients had BlinkR-R1 abnormalities, and one had delayed BAEP waves IV and V. Clinical improvement was almost always (32 of 34 re-examined patients) associated with improvement or normalisation of at least one electrophysiological abnormality. Brain MRI was done in 25 of the 44 patients and confirmed pontine lesions in six (two infarcts, three inflammations, one tumour). CONCLUSIONS: Pontine dysfunction was suggested in 45 of 232 consecutive patients with clinical signs of APV on the basis of abnormal electrophysiological findings, and was mainly attributed to brainstem ischaemia and multiple sclerosis. The frequency of pontine lesions mimicking APV is underestimated if based on MRI established lesions only.     

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.     

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.     

Variations of brain stem auditory evoked potentials correlated to duration and severity of multiple sclerosis

A total of 158 recordings of brain stem auditory evoked potentials (BAEPs) were obtained in 121 patients with multiple sclerosis. A statistically significant increased incidence of abnormality and diffusely abnormal recordings was found with increasing duration and severity of symptoms. Alterations in follow-up recordings might reflect changes in the clinical state, but also occurred in clinically stable patients. These findings suggested that transition from normality of BAEPs might occur either by successive disappearance of the later waves or by uniformly decreasing amplitudes of all the waves to diffusely abnormal recordings. It was concluded that the main value of BAEP recording in patients with multiple sclerosis was to indicate clinically silent lesions, and its value in monitoring the clinical condition of the individual patient was dubious.     

The anatomic and physiologic bases of brain stem auditory evoked potentials

The generators of human BAEPs, as summarized in Table 1 and Figure 12, are more complex than has often been presumed. Most peaks reflect activity in several structures, though patterns of BAEP abnormality can be correlated with abnormalities in general areas of the brain stem. The phasic peaks originate in action potentials, whereas the SN reflects postsynaptic potentials within brain-stem auditory nuclei.     

Influence of GAA expansion size and disease duration on central nervous system impairment in Friedreich's ataxia: contribution to the understanding of the pathophysiology of the disease.

OBJECTIVE: To verify if GAA expansion size could account for the severity of the central nervous system involvement in Friedreich's ataxia (FA). METHODS: Retrospective study of 52 FA patients (mean age 26.9+/-12.1 years; mean disease duration 10.6+/-7.6 years) homozygous for GAA expansion. Median nerve somatosensory evoked potentials (SSEPs) were available in 36 FA patients, upper limb motor evoked potentials (MEPs) to transcranial magnetic stimulation in 32, brainstem auditory evoked potentials (BAEPs) in 24, and visual evoked potentials (VEPs) in 34. N20, P100, MEP amplitude, SSEP and MEP central conduction time (CCT and CMCT), P100 latency and I-III and I-V interpeak latency, and a BAEP abnormality score were correlated with disease duration and GAA expansion size on the shorter (GAA1) and larger (GAA2) allele in each pair. RESULTS: The GAA1 size inversely correlated with the N20 amplitude (r = -0.49; P<0. 01). Disease duration directly correlated with CMCT (r = 0.57; P<0.01) and BAEP score (r = 0.61; P<0.01) and inversely with MEP (r = -0.40; P<0.05) and P100 amplitude (r = -0.39; P<0.05). CONCLUSIONS: Our data suggest that central somatosensory pathway involvement in FA is mainly determined by GAA1 expansion size. Vice versa, degeneration of pyramidal tracts, auditory and visual pathways seems to be a continuing process during the life of FA patients.     

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.     

Clinical correlates of brain-stem auditory evoked potential variables in multiple sclerosis. Relation to click polarity.

The correlations between clinical signs and BAEP latency, amplitude and dispersion variables were investigated in 98 multiple sclerosis patients. A new dispersion variable, the wave IV-V "shape ratio" (SR IV-V), correlated most strongly with brain-stem signs (i.e., nystagmus). Severely reduced wave IV-V amplitude was frequently found in patients with vertical nystagmus or internuclear ophthalmoplegia, and interpeak latency (IPL) III-V correlated most strongly with cerebellar dysfunction (i.e., ataxia). The results may reflect different localizing ability among the various BAEP variables. The association between ataxia and increased IPL III-V was significantly stronger for BAEP to C clicks than to R clicks. Patients with abnormal BAEPs to one polarity (C or R) but not to the other, had significantly more clinical dysfunction than patients with normal BAEPs to both C and R clicks. Hence, C vs. R discordance may be interpreted to indicate possible brain-stem dysfunction.     

Auditory evoked potentials in anxiety disorder

The pathophysiology of anxiety has received much recent attention. EEG findings in anxiety are nonspecific, and some changes in psychophysiological measures have been reported. We recorded short-latency brainstem auditory evoked potentials (BAEPs) and long-latency auditory event-related potentials (AEPs) in 12 patients with generalized anxiety disorder. All 12 patients had BAEP latencies within clinical norms, but I-V interpeak latencies were significantly longer in patients with anxiety than controls. N1, N2, P2, and P3 AEP components were within normal limits; N1 and P2 were reduced in amplitude in anxiety patients, but differences from controls were not significant. The BAEP findings may suggest altered brain-stem function in anxiety, which has been implied by biochemical studies of anxiety and depression. AEP differences may be related to difficulties in concentration and attention direction reported by anxious patients.     

The blink reflex and the diagnosis of multiple sclerosis. Clinical correlations in 66 patients

The blink reflex obtained from 66 patients with multiple sclerosis (MS) shows 73% pathologic responses with MS considered as definite, 50% with MS considered as probable and only 18% with possible MS. These findings are of great diagnostical help, particularly in the absence of clinical evidence of involvement of the brain-stem. Among 8 electrological syndromes of alteration of the blink reflex, those due to large lesions of the brain-stem are often associated with definite MS, but small alterations of the blink reflex are more valuable for multifocal characterisation of the illness.     

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.     

Measurements of brain-stem auditory evoked potentials in infancy,childhood, and adolescence

Brain-stem auditory evoked potentials (BAEPs) were measured in 38 patients between 3 months and 18 years of age. In patients with blunt head injuries (N=28; 73.7%) no significant correlation between BAEP changes and final outcome could be established until day 36 with the exception of wave III changes in 3 children. Because various BAEP abnormalities occurred in all outcome groups and the nature of the changes was transient, we conclude that within this age group there must be a higher resistance to various lesions until irreversible impairment of brain-stem function results. In spite of this result, continuous BAEP recording in this age group appears useful, since this noninvasive investigation represents an objective assessment of topical brain-stem function.     

Predictive value of brain-stem auditory evoked potentials in children with post-traumatic coma produced by diffuse brain injury

In the present study, brain-stem auditory evoked potentials (BAEPs) in 31 children in post-traumatic coma with diffuse brain injury were examined. The BAEPs were recorded in the first 72 h after hospital admission and the findings of 29 patients related to the level of neurological recovery at 6 months after head injury. On the basis of the results, children were divided into three groups: the first consisted of children with bilateral and normal BAEPs (n=19); the second of those with asymmetrical BAEPs (n=6); and the third of those in whom BAEPs had disappeared or in whom only responses of the seventh cranial nerve and cochlear nucleus were recorded (n=4). All the patients in the first group presented a good clinical outcome, with excellent recovery in 80%. In the second group three children (42.8%) had a good recovery, two (28.6%) were moderately disabled and one (14.3%) died of extraneurological causes. All the patients in the third group died. Abnormal BAEPs showed a significant correlation with absence of pupillary and/or corneal reflex, but not with the Glasgow Coma Score and anisocoria. Good statistical correlation was observed between normal BAEPs and visualization of basal cisterns on computed tomographic scan. The incidence of increased intracranial pressure was higher in patients with abnormal BAEPs, but the differences were not significant (P=0.06). Our study confirms the predictive value of BAEPs in children's post-traumatic coma due to diffuse brain injury.     

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.