Evoked potential studies in mitochondrial encephalomyopathy

Evoked potentials were studied in a patient with a mitochondrial encephalomyopathy revealing a defect of nicotinamideadenine dinucleotide dehydrogenase and cytochrome C oxidase in the mitochondria of a muscle biopsy specimen. The biopsy specimen showed myopathic changes with ragged-red fibers and markedly decreased cytochrome C oxidase in the muscle fibers. Subcortical somatosensory evoked potentials to median nerve stimulation were normal in the peak latencies of N9, N11, and N13. Cortical somatosensory evoked potentials to median nerve stimulation revealed significantly delayed peak latencies of N20, P20, P25, and N26, although N16 latency was normal. In particular, the interpeak latency between N16 and N20 was significantly delayed. In topographic maps, N20 and P20 were delayed in the peak latencies with normal scalp distributions. Dysfunction of somatosensory cortex indicated by the delay of cortical somatosensory evoked potentials may be related to a cortical mitochondrial abnormality. The absence of responses to auditory stimulation within 10 milliseconds could be related to the dysfunction of peripheral acoustic nerves.     

Multi-modality evoked potentials in hypoxaemia

The auditory nerve-brain-stem evoked response (ABR) has been shown to be insensitive to hypoxic inspiratory gas mixtures which severely depress the EEG. In order to determine the relative sensitivities of additional brain regions and pathways to hypoxaemia, anaesthetized paralysed cats were ventilated with various gas mixtures while recording the evoked responses of the auditory, somatosensory (including peripheral nerve, brain-stem and primary cortical components), visual and vestibular systems. Arterial blood pressure was maintained by dopamine infusion and pH was corrected with bicarbonate. Hypoxic gas mixtures (6-7% O2) presented for 60 min, causing severe hypoxaemia (paO2 20-30 mm Hg; O2 saturation 25-50%), were without effect on the somatosensory, vestibular and visual EPs while the auditory evoked potentials (ABR and cortical components) were depressed. However, if arterial blood pressure was allowed to fall, all of the evoked potentials became severely depressed and isoelectric. These results and others indicate that the cortical components are qualitatively similar to the more peripheral evoked potentials. The resistance of these evoked potentials to controlled hypoxaemia is probably due to their generation by oligosynaptic pathways and to a compensatory switch to anaerobic metabolism and to an elevation of cerebral blood flow.     

Event-related potentials, CNV, readiness potential, and movement accompanying potential recorded from posterior thalamus in human subjects. A SEEG study.

Intracranial recordings were obtained from three patients with intractable chronic pain who underwent analgesic electrical stimulation of the contralateral thalamus. Multilead electrode made it possible to record from several thalamic nuclei. The electrode was targeted into the ventroposterolateral (VPL) nucleus of the thalamus. During separate recording sessions, the following tests were performed: somatosensory evoked potentials (SEP) of the median or posterior tibial nerve, event-related cognitive potentials (auditory oddball P3 wave), readiness potential (RP) and contingent negative variation (CNV) using auditory warning (S1) and visual imperative (S2) stimuli. The movement accompanying potential (MAP), which was present in the VPL in all but one of the recordings, behaved as a far-field potential. Recordings obtained from the VPL confirmed its established role as a relay nucleus, processing somatosensory information to the primary somatosensory cortex. The VPL generated the 'thalamic' SEP, which was the only potential regularly recorded in this nucleus. In the recordings from one patient (No. 3), auditory and visual evoked potentials of the CNV protocol, peaking at approximately 300 ms, were obtained from the VPL and appeared to be generated in situ. Neither RP, CNV nor 'oddball' ERPs appeared in the VPL. From the pulvinar, only a visually evoked potential was recorded. Oddball P3, RP, CNV, and middle and long latency auditory and visual potentials (evoked in the CNV paradigm) appeared to be generated 'dorsally' to the VPL, probably in the nucleus posterolateralis (PL). This structure may therefore be involved in both the processing of afferent information and in cognitive operations.     

Characteristic evoked potentials in childhood-onset dentatorubral-pallidoluysian atrophy

To determine the characteristics of multimodal evoked potentials (MEPs) in childhood-onset dentatorubral-pallidoluysian atrophy (DRPLA) we studied three DRPLA patients with progressive myoclonus epilepsy. Brainstem auditory evoked potentials showed reduced or absent brainstem components as well as delayed latencies. In addition, short latency somatosensory evoked potentials (S-SEPs) had prolonged central conduction time and reduced amplitude of cortical components. Two patients with symptom onset in the first decade of life had extremely enlarged flash visual evoked potentials with shortened latency even in the absence of giant SEPs. Therefore, children with progressive myoclonus epilepsy and the above MEP findings are likely candidates for childhood-onset DRPLA and should undergo DNA analysis for DRPLA.     

Preservation of brainstem neurophysiological function in hydranencephaly

The preservation of central neurophysiological function was assessed in a 32-year-old woman with hydranencephaly using brainstem auditory evoked responses (BAER), auditory middle latency responses (MLR), cortical auditory evoked responses (CER), strobe electroretinograms (ERG), strobe-flash visual evoked responses (VER) and median and tibial nerve somatosensory evoked responses (SER). The BAER to the right ear stimulation revealed wave peaks I through VII with normal thresholds, morphology and latencies, while the BAER in the left ear was abnormal. The auditory MLR and CER were absent. Grossly normal strobe ERGs were acquired bilaterally with peak waves at 20 and 50 ms. Strobe VERs were poorly defined and abnormal bilaterally. Left and right median nerve SER revealed significant conduction defects in the large fiber sensory system caudal to the thalamus, above the lower pontine level. Bilateral tibial nerve stimulation revealed normal knee popliteal fossa potentials, but distinct conduction defects in the large fiber sensory system rostral to the lower spinal cord. Brainstem electrophysiological measures revealed functional auditory afferent tracts and nuclei, in the absence of cortical influence, suggesting intact unilateral auditory function, which would support clinical observations of behavioral auditory responses in hydranencephaly.     

Cortical deafferentation in cat focal ischemia: disturbance and recovery of sensory functions in cortical areas with different degrees of cerebral blood flow reduction

During and after 15-min occlusion of the middle cerebral artery (MCA) in cats, local CBF and neuronal activity were measured in cortical areas varying in the degree of CBF reduction. In an area within the ischemic center (primary auditory cortex, middle ectosylvian gyrus), CBF was severely suppressed. Click-induced auditory evoked potentials and evoked as well as spontaneous single-unit activity ceased within 1 min after occlusion. Recirculation resulted in a recovery of the different neurophysiological parameters with a time delay ranging from several minutes to 2 h. In two areas surrounding the ischemic focus (a visual area in the marginal gyrus and the forelimb representation area in the primary somatosensory cortex), CBF was reduced but remained above 30 ml/100 g/min during MCA occlusion. Visual flash-induced evoked potentials and somatosensory evoked potentials induced by median nerve electrical stimulation ceased in the corresponding areas with a somewhat slower time course as compared to the auditory responses and they recovered faster after recirculation. In another somatosensory area (hindlimb projection area in the primary somatosensory cortex), CBF stayed nearly at control levels during occlusion. Evoked potentials and single-unit activity induced by tibial nerve electrical stimulation decreased approximately 5 min after occlusion and were abolished approximately 5 min later. At that time, single-unit activity had changed to a nonresponsive pattern but persisted. However, potentials evoked transcallosally by electrical stimulation of the contralateral hemisphere were still recorded. After reopening the MCA, the recovery of neuronal functions was usually complete and occurred within approximately 5 min.(ABSTRACT TRUNCATED AT 250 WORDS)     

Brainstem auditory and somatosensory evoked potentials in neuro-Beh?et's syndrome

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 neuro-Beh?et's syndrome (NB). Eleven patients (64.7%) showed an absence of wave I, III or V or a prolongation of the interpeak latency I-III, or III-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.     

Short latency somatosensory evoked potentials in patients with neurological lesions

Short latency somatosensory potentials following median nerve stimulation were recorded in patients grouped according to anatomic location of neurological lesion. Patients with cerebral lesions causing severe sensory deficit lacked a major positive wave of cortical origin that in normal subjects peaked at a mean latency of 20.5 ms. Patients with severe cervical spinal cord disease lacked all of the normal somatosensory response except for the earliest component attributed to peripheral nerve activity. Patients with brain-stem lesions showed delayed latencies of later waves and prolonged interwave latencies. However, auditory evoked potentials measured in the group with brain-stem lesions were more helpful in localization. Analysis of short latency somatosensory potentials can discriminate between peripheral nerve, spinal cord, brain-stem, and cerebral lesions. Further experience and refinement of technique of measurement should increase the value of this procedure.     

Electrophysiologic recordings in a patient with a discrete unilateral thalamic infarction

The electroencephalogram, and somatosensory and auditory evoked potentials were recorded from a patient, who, at necropsy, showed a restricted unilateral thalmic infarct involving predominantly the anterior and lateral thalamus. The electroencephalogram showed distinct monomorphic delta activity and a suppression of the alpha rhythm over the side of the lesion. Short latency somatosensory evoked potentials were present bilaterally; mid-latency somatosensory evoked potentials were absent ipsilateral to the lesion. Both mid-latency and long latency auditory evoked potentials were normal.     

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.     

Cerebral cortical contributions to sensory evoked potentials: hydranencephaly

The contribution of the cerebral cortex to the generation of sensory evoked potentials was studied in an infant with hydranencephaly. On CT scan no tissue above the thalamus was noted. Long-latency potentials to auditory stimuli were absent whereas the short-latency or brain-stem auditory evoked potentials and some of the components of the middle latency auditory evoked potentials (No and Po) were present. To visual stimulation only the electroretinogram was detected. To somatosensory stimulation only the spinal cord potentials could be detected. The absence of long-latency components in each of the sensory modalities supports the concept that these potentials require intact cerebral hemispheres in man.     

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.     

Familial paroxysmal exercise-induced dyskinesia and benign epilepsy: a clinical and neurophysiological study of an uncommon disorder

We report a family with 6 members affected by a long-lasting paroxysmal exertion-induced dyskinesia. Fasting and stress were precipitating factors. All the patients of this family had also epileptic seizures mainly of generalised type with a favourable outcome. All patients were submitted to a neurophysiological study which included somatosensory evoked potentials by median nerve stimulation (MN-SEPs), somatosensory evoked potentials by posterior tibial nerve stimulation (PTN-SEPs), brainstem auditory evoked potentials (BAEPs), visual evoked potentials (VEPs), motor evoked potentials (MEPs) by magnetic transcranial cortical stimulation (TCS) and electromyography (EMG). The neurophysiological findings suggest a hyperexcitability at the muscular and brain membrane levels, probably due to an ion channel disorder. Received: 2 February 2000 / Accepted in revised form: 16 June 2000     

The diagnostic value of sensory evoked potentials in pediatric Wilson disease

We studied the sensory evoked potentials in pediatric Wilson disease to verify their subclinical neurologic involvement and to elucidate the role of cirrhosis in abnormal evoked potentials in non-neurologic Wilson disease. Thirty children (17 male, 13 female), diagnosed with Wilson disease before 18 years, were enrolled. The mean age during studies was 15.8 +/- 6.3 years, and disease duration since diagnosis was 3.0 +/- 3.3 years. In 12 neurologic Wilson disease cases, there were prolonged interpeak latencies of brainstem auditory evoked potentials III-V, I-V, somatosensory evoked potentials N13-N20 (P < 0.01 vs controls and non-neurologic cases), and P100 latency (P < 0.01 vs controls). All 12 patients had at least one abnormal evoked potential, including 91.7% brainstem auditory, 58.3% somatosensory, and 25% visual evoked potentials. In 18 non-neurologic Wilson disease cases, there were still prolonged interpeak latencies for brainstem auditory evoked potentials I-V and somatosensory evoked potentials N13-N20 (P < 0.05 vs controls), with 27.8% of them having at least one abnormal evoked potential, including 16.6% brainstem auditory, 5.6% somatosensory, and 11.1% visual evoked potentials. In those with non-neurologic Wilson disease, there were no significant differences in all the evoked potential parameters between the cirrhotic and non-cirrhotic patients.     

Evaluation of central neuropathy in type II diabetes mellitus by multimodal evoked potentials

The electrophysiological results in 51 patients with diabetes mellitus type II were compared with those in 30 age and sex matched healthy control subjects. Peripheral and cortical latencies of median and tibial somatosensory evoked potentials (SEP), bilateral I-III and I-V interpeak latencies (IPL) of brainstem auditory evoked potentials (BAEP), bilateral P100 latency of visual evoked potentials (VEP) and bilateral cortical latency and central motor conduction time of motor evoked potentials (MEP) were evaluated. We observed prolonged latencies suggestive of central neuropathy in DM type II. It has been shown that most of the electrophysiological parameters in patients with DM type II correlate with the duration of the disease, some of them with the age of the patient, and few of them with the onset of the disease. To our knowledge, there is no correlation between the electrophysiological parameters and the level of glycemia or the degree of metabolic control. We conclude that central and peripheral neuropathies in DM are related to the duration of the disease and not to the degree of hyperglycemia and metabolic control.     

Generalisability of sensory gating during passive movement of the legs

Movement-related gating of somatosensory evoked potentials in the upper limb is restricted mainly to nerve stimulation supplying the moved limb segment. In the lower limb, this principle may not be followed. Tibial nerve (stimulation at the knee) somatosensory evoked potentials (SEPs) and soleus H reflexes exhibit quite similar patterns of modulation during movement. We hypothesised that movement-related gating of initial SEPs in the leg would be generalised from ipsilateral to contralateral leg movement and that such sensory gating would not be generalised to modalities with no functional relevance to the movement. Somatosensory, visual, and auditory evoked potentials (SEPs, VEPs, and AEPs) were recorded from scalp electrodes during unilateral passive movement. Short-latency tibial nerve SEPs, representing the first cortical components, and soleus H reflexes in both the moved leg and the stationary leg were attenuated compared to non-movement controls (p<0.05). Neither VEPs nor middle latency AEPs were modulated (p>0.05). We conclude that sensory gating occurs during contralateral movement. This gating is absent in other sensory modalities with no apparent functional relationship to the imposed movement.     

Multimodal evoked potentials in the early period after cranio-cerebral trauma]

In 83 patients aged 17-68 years somatosensory evoked potentials by median nerve stimulation, and visual and auditory evoked potentials were studied 5-28 days after craniocerebral trauma. Brain concussion was diagnoses in 43 cases on the basis of neurological examination, CT and duration of unconsciousness. In the remaining 40 cases brain contusion was diagnosed. In SSEP the latency was calculated of waves N9, N13, P16, N20, P22, N35 and P40: in the visual evoked potentials the latency of the P100 component, and in auditory evoked potential the latency of waves I, III and V, and interpeak latency I-III, III-V and I-V SSEP changes were found in 39% of cases of brain concussion and 52.9% of brain contusion cases. The abnormalities in both groups involved mainly the component of latency and deviation P100 of visual evoked potential P40 and N35. Prolongation of the latency of P100 of the visual evoked potential was recorded in 20% of patients with brain concussion and 16.7% with brain contusion. Auditory evoked potentials were abnormal in 10.3% of brain concussion and 26.5% of brain contusion cases. In 64 cases all three types of evoked potentials were studied and pathological changes in at least one of these types were found in 56.4% of brain concussion and 72% of brain contusion cases. The results show that as least in a part of cases diagnosed as brain concussion according to generally accepted criteria, central nervous system injury is present.     

Electrophysiologic study of Fisher syndrome

Electrophysiologic studies were performed on a 6-year-old girl with Fisher syndrome. We recorded several evoked potentials in this patient: visual evoked potentials, auditory brainstem responses, auditory evoked potentials, short-latency somatosensory evoked potentials, blink reflex elicited by photic stimuli (photo-evoked eyelid microvibration), blink reflex elicited by auditory stimuli (auditory evoked eyelid microvibration), and motor nerve conduction velocity. In our study, photo-evoked eyelid microvibration response was not obtainable; laterality was indicated in visual evoked potential and electroencephalographic studies, and the remaining evoked potentials demonstrated normal responses. The results obtained from the brainstem reflex (photo-evoked eyelid microvibration) suggest that the pathologic focus of Fisher syndrome is located in the midbrain, particularly in the pretectum. It is expected that the combined use of these electrophysiologic techniques may facilitate differentiation between Fisher and Guillain-Barré syndromes.     

Electrophysiologic study of 10 cases of Miller Fisher syndrome

The association of ophthalmoplegia, ataxia and areflexia was described by Miller Fisher in 1956. It is postulated as a variant of the Guillain Barré syndrome. We report 10 Miller Fisher syndrome patients admitted in an intensive care unit between June 1990 and February 1999 who were selected according to clinical criteria of Ropper and Wijdicks. All patients had motor and sensory nerve conduction studies and electromyography, nine had visual and brainstem auditory evoked potentials and two had short latency somatosensory evoked potentials. Peripheral neuropathy was found in all patients. All had sensory nerve changes and some were severe. Motor nerve conduction abnormalities were observed in 7 only cases with moderate increase of F latency in 3 cases and compound muscle action potential reduction in 3 other cases. In the last case, motor conduction abnormalities was more severe, characterized by conduction velocity slowing in both distal and proximal sites and by temporal dispersion of action potentials. All brainstem auditory evoked studies were normal. In 4 patients, MRI studies were normal. These data support that brainstem is preserved in MFS. Only one patient had visual evoked potential abnormalities. Optic neuropathy is debated in Miller Fisher and in Guillain Barré syndrome. As a conclusion, in MFS peripheral neuropathy is always present with severe sensitive changes and moderate motor changes (This is different as compared to Guillain Barré syndrome according to electrophysiological data). We did not find involvement of brainstem in our patients with Miller Fisher syndrome.     

Brain stem auditory, visual and somatosensory evoked potentials in leukodystrophies

Brain stem auditory (BAERs), visual (VEPs) and somatosensory evoked responses (SEPs) were recorded in 12 patients with Pelizaeus-Merzbacher leukodystrophy (PMD), three with adrenoleukodystrophy (ALD) and three with metachromatic leukodystrophy (MLD). All the 3 evoked responses were abnormal in all patients except normal VEPs and SEPs in a patient with early ALD. In most patients wave I with and without wave II were the only components of the BAERs that remained, subsequent components (waves III-VII) were absent. VEPs were severely altered; either no identifiable response to flash or pattern reversal stimuli could be recorded or the major components were significantly delayed in latency. Short latency SEPs following median nerve stimulation usually showed a normally recorded Erb's potential (N10), but an absence or marked attenuation of cervical (N14) and early scalp components (N19 and P22) or the occurrence of the scalp components with a significant delay. Multimodality evoked responses provide more information regarding the functional integrity of several afferent systems in patients with white matter disorders.