Correlation between morphological abnormalities of Chiari malformation and evoked potentials]

We studied correlation between morphological abnormalities of Chiari malformation and evoked potentials (short-latency somatosensory evoked potential [SSEP] and auditory brainstem response [ABR]). On SSEP the inter-peak latency prolongation of P3-N1 was revealed in 6 out of 8 cases with Chiari malformations. The feature of positive wave between P3 and N1 was divided into 2 groups. The tendency of the positivity between P3 and N1 was more marked in cases of prolonged P3-N1 latency and correlated with the medullary kink. On ABR the prolongation of III-V inter-peak latency was revealed in one side in 3 patients Chiari malformations with malformed pons and tegmentum.     

Multimodality evoked potentials in progression of metachromatic leukodystrophy

A 2-yr-3-mo-old girl with metachromatic leukodystrophy (MLD) was examined using serial multiple electrophysiological procedures. Sensory nerve conduction velocity was delayed earlier and more severely than motor nerve conduction velocity. Visual evoked potentials (VEPs) showed prolonged latency of wave IV. Auditory brainstem responses (ABRs) showed prolonged latency of waves I and V, and the I-V interval. As to the interpeak latency of somatosensory evoked potentials (SEPs), the P9-P14 and the P14-N20 intervals were prolonged on admission. Two months later, both intervals were more prolonged, but the prolongation of the P9-P14 interval was the most prominent. The demyelination in our case may have started in the cerebral white matter, progressed to the peripheral nerves, and at last via the spinal root reached the brainstem. An electrophysiological follow-up study may be valuable in the understanding of the progressive pathological changes and in the evaluation of therapeutic measures.     

Effects of hypothermia on short latency somatosensory evoked potentials in humans.

Short latency somatosensory evoked potentials (SSEPs) elicited by median nerve stimulation were monitored in 14 adult patients undergoing cardiac surgery under cardiopulmonary bypass and induced hypothermia. SSEPs were recorded at 1-2 degrees C steps as the body temperature was lowered from 37 degrees C to 20 degrees C to determine temperature-dependent changes. Hypothermia produced increased latencies of the peaks of N10, P14 and N19 components, the prolongation was more severe for the later components so that N10-P14 and P14-N19 interpeak latencies were also prolonged. The temperature-latency relationship had a linear correlation. The magnitude of latency prolongation (msec) with 1 degree C decline in temperature was 0.61, 1.15, 1.56 for N10, P14 and N19 components, respectively, and 0.39 and 0.68 for interpeak latencies N10-P14 and P14-N19, respectively. The rise time and duration of the 3 SSEP components increased progressively with cooling. Cortically generated component, N19, was consistently recordable at a temperature above 26 degrees C, usually disappearing between 20 degrees C and 25 degrees C. On the other hand, more peripherally generated components, N10 and P14, were more resistant to the effect of hypothermia; P14 was always elicitable at 21 degrees C or above, whereas N10 persisted even below 20 degrees C. The amplitude of SSEP components had a poor correlation with temperature; there was a slight tendency for N10 and P14 to increase and for N19 to decrease with declining temperature. Because incidental hypothermia is common in comatose and anesthetized patients, temperature-related changes must be taken into consideration during SSEP monitoring under these circumstances.     

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.     

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.     

Encephalomyelopathy demonstrated on MRI in a case of chronic toluene intoxication]

Myelopathy in chronic toluene intoxication is rare. We present obvious lesions of the spinal cord on MRI in a 30-year-old Japanese man with chronic toluene intoxication. He had abused toluene for more than 10 years, and developed visual impairment, horizontal nystagmus, pyramidal tract signs, postural tremor, Romberg's sign, and sensory disturbance below the level of Th 2 dermatome. Anti-HTLV-1 antibody titer and vitamin B12 level in the serum were within normal limits. Biochemical analysis showed no increase of very long chain fatty acids. Cerebrospinal fluid showed no abnormal findings. Auditory brainstem response showed delay of I-V interpeak latency. Somatosensory evoked potential with the median nerve stimulation showed delay of N13-N20 central conduction time, which was later followed by absence of N14-N20 components. On MRI in T2 weighted image, marked high intensity was demonstrated in the posterior limbs of the internal capsule, and in the posterior columns and lateral tracts from the cervical through the upper thoracic cord. Cerebral lesions probably reflect demyelination and axonal degeneration produced by chronic toluene abuse. Spinal cord lesions seem to be secondary to nerve fiber changes more proximal to the nerve cell bodies.     

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.     

Hearing impairment in two boys with I-cell disease.

Auditory brainstem responses (ABR) were analyzed in 2 boys with I-cell disease. Both patients showed a prolonged latency of wave I, a normal I-V interpeak latency and an elevated threshold of wave V. The intensity-latency curve had a steep slope. These abnormal ABR findings reflect the presence of conductive hearing impairment associated with the cochlear component in I-cell disease.     

Neurophysiological mechanisms of conduction impairment of the auditory nerve during cerebellopontine angle surgery

ObjectiveIntraoperative auditory brainstem response (ABR)-monitoring is useful for hearing preservation in patients undergoing cerebellopontine angle surgery. Prolongation of the latency of wave V, for example, is observed under surgical stress such as cerebellar retraction. We analyzed intraoperative ABR findings to study the neurophysiological mechanism(s) underlying latency prolongation.MethodsThe ABR recorded during microvascular decompression surgery was studied in 18 patients with hemifacial spasm. We measured each trace of the ABR records, both the latency of each wave and some interpeak latencies. We also analyzed their waveforms especially in the early component, to assess changes during surgery.ResultsThe latency of wave V varied with cerebellar retraction. The delayed latency of wave V was correlated with the prolonged interpeak latency of waves I–III. An additional wave (designated wave I′) between waves I and II was appeared; it was accompanied by a prolongation in the latency of wave V. Wave I′ contributed to prolongation of the interpeak latency of waves I–III, resulting in a delay in the latency of wave V. Chronological analysis revealed that the minimum latency of wave I′ was the same as wave IN, suggesting that wave I′ arose near the porus acusticus internus (PAI).ConclusionOur study showed that cerebellar retraction may result in conduction impairment of the auditory nerve near the PAI, suggesting that the Obersteiner-Redlich zone is an electrophysiologically vulnerable site and wave I′ is derived from the change in the vector of wave IN.SignificanceOur findings may provide neurophysiological evidence to support the theoretical model of ABR generators by Scherg and von Cramon.     

Short-latency somatosensory and brainstem auditory evoked potentials in patients with Parkinson's disease

Short-latency somatosensory evoked potentials (SSEPs) and brainstem auditory evoked potentials (BAEPs) were recorded in 44 patients with Parkinson's disease (mean age 67.3 years) and 23 normal subjects (mean age 69.3 years). Patients with Parkinson's disease and normal subjects did not show any significant difference with regard to the interpeak latencies between N13 and N20 central conduction time (CCTs). Likewise, there were no significant differences in CCTs between patients with and without dementia. The interpeak latencies between waves I and V (I-V IPLs) in patients with Parkinson's disease were significantly longer than those of the normal subjects (p less than 0.05). In particular, patients with dementia revealed significant prolongation of I-V IPLs compared to patients without dementia and normal subjects (p less than 0.01, p less than 0.001) although no significant differences were observed between patients without dementia and normal subjects. These results show that auditory brainstem pathways are involved in Parkinson's disease patients with dementia.     

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.     

Multimodality evoked potentials in amyotrophic lateral sclerosis

Visual, brainstem auditory and somatosensory evoked potentials to medial nerve stimulation were recorded in 27 patients affected by amyotrophic lateral sclerosis. VEP N75, P100, N140, N75-P100 latencies and P100 amplitude, BAEP I-III, III-V and I-V interpeak-latencies were within normal limits in all ALS patients. Somatosensory evoked potentials were abnormally delayed in 8 patients: in 3 arms because of a delayed N9-N13 latency, in 9 arms because of a delayed N13-N19 latency.     

Peroxisomal bifunctional enzyme deficiency: serial neurophysiological examinations of a case

We report on a case of 21-month-old girl with peroxisomal bifunctional enzyme deficiency, which was diagnosed by means of complementation analysis. Serial neurophysiological examinations were also carried out. The motor and sensory nerve conduction velocities of the median nerve showed lower borderline values at 3 months of age and were within normal range at 11 months of age. Later, those velocities had gradually decreased. The electrically elicited blink reflex at 3 months of age showed the prolongation of latencies of R1, R2 and R2′ and the interpeak latencies of R1, R2 and R1 -R2′. Furthermore, RI, R2 and R2′ showed prolonged latencies at 11 months of age and were absent at 15 months of age. The auditory brainstem response (ABR) showed, bilaterally, normal latency of wave I, prolonged interpeak latencies of waves I–V. At 11 months of age, waves III and IV–V of ABR were detected, but their amplitude was very low. At the age of 15 months ABR was absent. These results and the following report are valuable for understanding the pathogenesis of neurological symptoms.     

Portal-systemic encephalopathy: alterations in somatosensory and brainstem auditory evoked potentials

Median somatosensory and brainstem auditory evoked potentials (SEP and BAEP) were studied in 40 patients with liver cirrhosis consequent to chronic viral hepatitis. The patients were divided into 4 groups: group 1 with liver cirrhosis only, group 2 with hepatic failure (HF), group 3 with grade 1 or 2 hepatic encephalopathy (HE), and group 4 with grade 3 or 4 HE. The control group consisted of 10 age-matched normal subjects. The major changes occurred in the median cortical SEP late components (peaks after N20 and P25). From group 1 to group 4, there were progressive prolongation and sequential disappearance of the late components. Those changes in the cortical SEPs were reversible. The subcortical somatosensory and brainstem auditory conductions (SEP N13-N20 and BAEP I-V interpeak latencies) were slightly prolonged in all groups of patients. The present data indicate that SEP may be useful in detecting subclinical HE and in monitoring the clinical course of HE. The present data further indicate that chronic portal-systemic shunting in liver cirrhosis may result in a minimal impairment of cerebral function and sensory conduction in the CNS.     

Somatosensory evoked potentials and auditory brain-stem responses in congenital hypothyroidism. I. A longitudinal study before and after treatment in six infants detected in the neonatal period

We report the results of a longitudinal study of auditory brain-stem responses (ABRs) and somatosensory evoked potentials (SEPs) performed in 6 children with congenital hypothyroidism. These infants were detected by the Quebec Network for Genetic Medicine and treated early. ABRs and SEPs were recorded both before and 2 weeks after the initiation of therapy and at 6 months of age. Before treatment, for SEP, we found increased wave N19, P22 latencies and N13-N19, N19-P22 interpeak latencies (IPLs) in congenital hypothyroid (CH) children. For ABR, there were increased wave I latencies with normal I-V IPLs. Substitutive therapy improved these abnormalities although this improvement was more evident after a shorter period of time for ABRs than for SEPs. Even at 6 months, 2 CH children still showed increased N13-N19 IPLs. Both had very low serum T4 levels at the time of diagnosis and one had also a very small knee surface area, both criteria indicating a severe hypothyroidism. It will be interesting to verify if initial and persisting increase of N13-N19 IPL is associated with later neuropsychological problems.     

Multimodal evoked potentials in HIV-1-seropositive patients: relationship between the immune impairment and the neurophysiological function

Multimodal evoked potentials (PRVEP, BAEP, mSEP) were recorded in 56 HIV-1 seropositive outpatients free from opportunistic CNS pathologies and/or overt HIV-1 encephalopathy. EPs were altered in 17 of 39 (43.6%) seropositive subjects without AIDS (group A) and in 13 of 17 (76.5%) patients with AIDS (group B). A high incidence of subclinical alterations (30.8%) were found in group A patients. Significant BAEP (I-III, III V, I-V) interpeak latency and mSEP (N9-N13, N9-N20) conduction time prolongations were found in group A and B patients. PRVEP PI00 was significantly prolonged only in group B. An inverse relationship between BAEP interpeak latencies and CD4 count was found. Our findings support the hypothesis of an important role of immunodepression in the development of neurophysiologic abnormalities, together with a preferential involvement of acoustic pathways, in the course of HIV-1 infection.     

Sensory evoked potentials in herpes simplex encephalitis.

Flash visual potentials (FEPs), somatosensory evoked potentials (SEPs) and auditory brainstem responses (ABR) were recorded in a 66-year-old patient presenting with clinical, EEG and CT brain scan features of herpes simplex encephalitis (HSE). At the time of evoked potential study (10 days after onset of the disease) the patient was treated with iv barbiturate on controlled respiration (lidocaine and phenytoin were not utilized); core temperature was 37 degrees C and pupils were dilated and nonreactive. Cortical FEPs were not recognizable on 02 lead, whereas they were clearly evident on 01 with normal latency of early N1, P1, N2 waves and delayed P2 component. SEPs showed normal peripheral and central conduction times, but N20 peak was bilaterally absent with unrecognizable (on P3) or delayed (on P4) N33 wave. No ABR (including wave I) were found on stimulation of the right ear, whereas delayed wave V with prolonged interpeak I-V latency was found on stimulation of the left ear. In conclusion, changes in sensory evoked potentials in HSE seem to be caused either by necrotic-hemorrhagic damage (with the disappearance of some cortical responses), by coma (with alterations in middle-latency cortical responses) and by increased intracranial pressure (with subsequent ABR abnormalities).     

Effects of stimulus intensity on latency and conduction time of short-latency somatosensory evoked potentials.

We studied the effect of stimulus intensity on latencies of short-latency somatosensory evoked potentials (SSEP) by measuring both onset and peak latencies individually. The latencies of N9, N13, N20 and N9-N13 peripheral conduction time (PCT) of median nerve (MN) SSEP, and N8, N23, P37 and N8-N23 PCT of tibial nerve (TN) and sural nerve (SN) SSEP significantly shortened with increasing stimulus intensity by onset latency measurement. However, those latencies by peak latency measurement were less significantly shortened or had only a trend of latency shortening without statistical significance. In contrast to PCT, N13-N20 central conduction time (CCT) of MN-SSEP and N23-P37 CCT of TN- or SN-SSEP showed no latency changes with the increased stimulus intensity by both onset and peak latencies measurement. As peak latencies had greater interindividual variability than onset latencies shown by larger standard deviation, shortening of onset latencies were more consistent than that of peak latencies. We think shortening of onset latencies indicates the recruitment of faster conduction fiber along with increased stimulus intensity. As the degree of latency shortening was less if stimulus intensity was above 2.5 times sensory threshold, the stimulus intensity greater than 2.5 times the sensory threshold should be used for clinical application.     

Median nerve somatosensory evoked potentials: studies on latency variability as a function of subject height, limb length and nerve conduction velocity.

Report on the results of regression analysis studies concerning median nerve somatosensory evoked potentials (SEPs) latencies, as dependent variables, and subject height, limb length and nerve conduction velocity (NCV), as independent variables. The tests were performed on 23 normal volunteers. Absolute SEP latencies could be predicted by a linear regression model when the independent variable was arm length; when it was subject height, however, both exponential and polynomial models proved better, the latter showing the best coefficients of determination, R 2. Multiple linear regression with two independent variables (arm length and NCV) was found to be better than simple linear regression for predicting P/N13 latency. The regression line for EP-P/N13 latency on height was found to be a polynomial curve; although the regression was found to be significant by the "F" test (alpha = 1%), the model had a low R 2 value (0.41). The same applies to the P/N13-N19 interpeak latency regression curve, but the regression was significant for alpha = 5% in that case. Although interwave latencies are the most useful parameters for clinical interpretation of median SEPs, absolute latencies may occasionally be important, and should be corrected for body size. In unusually tall subjects, it might be useful to double-check EP-P/N13 interwave latency prolongation by estimating the maximum expected P/N13 latency, using a model that takes into account both limb length and NCV.     

Auditory brainstem response (ABR) in congenital central alveolar hypoventilation

Auditory brainstem responses (ABRs) were studied in a child with congenital central alveolar hypoventilation showing marked depression of respiratory drive during sleep. During wakefulness and normoventilation no ABR abnormalities were found, either at the age of 14 months or five years. ABR recordings during sleep at 14 months of age showed marked wave V latency and wave I to wave V interpeak latency prolongation of about 0.4 ms both for periods of hypoventilation and normoxic hypercapnia. ABR findings of this and other studies carried out in sleep apneas are discussed with respect to brainstem dysfunction associated with varied sleep apnea syndromes.