Sensory and cognitive evoked potentials in a case of congenital hydrocephalus

We studied auditory and visual evoked potentials in D.W., a patient with congenital stenosis of the cerebral aqueduct. Head CT scans revealed marked hydrocephalus with expanded ventricles filling more than 80% of the cranium and compressing brain tissue to less than 1 cm in thickness. Despite the striking neuroanatomical abnormalities, however, the patient functioned well in daily life and was attending a local community college at the time of testing. Evoked potentials provided evidence of preserved sensory processing at cortical levels. Pattern reversal visual evoked potentials had normal latencies and amplitudes. Brain-stem auditory evoked potentials (BAEPs) showed normal wave V latencies. Na and Pa components of middle-latency AEP had normal amplitudes and latencies at the vertex, although amplitudes at lateral electrodes were larger than at the midline. In contrast to the normal sensory responses, long-latency auditory evoked potentials to standard and target tones showed abnormal P3 components. Standard tones (probability 85%), evoked N1 components with normal amplitudes (-3.7 microV) and latencies (103 msec), but also elicited large P3 components (17 microV, latency 305 msec) that were never observed following frequent stimuli in control subjects. Target stimuli (probability 15%) elicited P3s in D.W. and controls, but P3 amplitudes were enhanced in D.W. (to more than 40 microV) and the P3 showed an unusual, frontal distribution. The results are consistent with a subcortical source of the P300. Moreover, they suggest that the substitution of controlled for automatic processes may help high-functioning hydrocephalics compensate for abnormalities in cerebral structure.     

Prognostic value of electrically elicited blink reflex in neonates

The electrically elicited blink reflex (BR) was evaluated in 80 normal neonates and 12 neonates with neurologic abnormalities. In normal subjects, R1 and bilateral R2 responses were elicited both while awake and in a quiet sleep state. Whereas the R1 response was consistently elicited in the active sleep state, as well as while awake, the ipsilateral R2 response was markedly suppressed and the contralateral R2 response was almost absent. The BR in neonates with neurologic abnormalities showed the following variable results: normal, prolonged latency, or suppressed response at initial recording. Abnormal BRs were detected in most neonates with respiratory or sucking problems. While neonates in whom a suppressed response or prolonged latency persisted for over three months had a poor prognosis, those with normal BRs or early correction of the BR abnormality had almost normal development. The BR appears to be useful not only to evaluate brain-stem function in the neonatal period but also to predict subsequent outcome.     

Familial congenital facial diplegia: electrophysiologic and genetic studies

A 13-year-old boy with autosomal-dominant congenital facial diplegia was evaluated by electrophysiologic and genetic investigations. Thirteen members of his family were affected over 4 generations. The electrophysiologic studies revealed blink reflex abnormalities. Both R1 and R2 responses were prolonged on the left side after ipsilateral stimulation, while R2 was also delayed by contralateral stimulation. Ipsilateral R1 and R2 were of normal latencies when the right side was stimulated. A third ipsilateral response at 63 msec of latency could be obtained when stimulating the left side. These findings suggest functional damage to the brainstem. Further support for this interpretation was provided by the prolonged time between waves I and V, bilaterally, documented by study of brainstem auditory evoked potentials.     

The blink reflex in neonates with a subsequent poor outcome

We examined maturational changes in the electrical blink reflex in 11 handicapped children, i.e., 4 cases of developmental delay, 4 of cerebral palsy, 2 of congenital hydrocephalus, and 1 of congenital cytomegalovirus infection. The developmental delay and cerebral palsy cases were all born at 25–36 weeks' gestation. In all cases, prolonged latency, low amplitude, or the absence of the late component of the blink reflex was observed in the follow-up study. These results indicate that prematurity and some congenital impairment mainly influence the maturation of the long-loop reflex arc in the blink reflex.     

Acute migraine headache: possible sensitization of neurons in the spinal trigeminal nucleus?

OBJECTIVE: To investigate trigeminal sensory processing in patients with migraine using a novel "nociception-specific" blink reflex. METHODS: Seventeen patients with unilateral migraine headache were studied within 6 hours of onset. Blink reflexes were elicited with a standard stimulating electrode (standard blink reflex) and concentric stimulating electrode (nociception-specific blink reflex) during the acute migraine attack, after treatment with IV lysine acetylsalicylate (1,000 mg) or oral zolmitriptan (5 mg) and interictally. RESULTS: After standard stimulation, no differences were detected for the R1 and R2 onset latencies and areas under the curve (AUC) between the different time points and the headache and nonheadache side. Nociception-specific stimulation revealed a shortening of R2 onset latencies (44.3 +/- 5.4 ms for headache side vs 48.9 +/- 5.8 ms for nonheadache side) during the acute migraine attack compared with the headache-free interval (49.8 +/- 5.3 vs 49.8 +/- 4.5 ms). The AUC of the R2 increased on the headache side by 680% and on the nonheadache side by 230% compared with the headache-free interval. Drug treatment parallel to pain relief increased the onset latencies (zolmitriptan: 48.0 +/- 8.2 ms for headache side vs 52.3 +/- 7.6 ms for nonheadache side; lysine acetylsalicylate: 48.0 +/- 5.0 ms for headache side vs 51.2 +/- 5.6 ms for nonheadache side) and reduced the AUC of R2 (zolmitriptan by 45% and lysine acetylsalicylate by 48%). CONCLUSION: The data suggest temporary sensitization of central trigeminal neurons during acute migraine attacks.     

A new indirect method for measuring spinal conduction velocity in man

A non-invasive, indirect method for measuring spinal cord mixed afferent-efferent conduction is described. The method is based upon eliciting late reflex responses labelled R1 and R2 from voluntarily contracting thenar and tibialis anterior muscles by preferentially stimulating median and common peroneal sensory nerve fibres. The mean onset latencies of R1 measured 27.5 msec and 30.6 msec recorded from hand and leg muscles respectively. R2 mean onset latencies measured 46.0 msec and 65.1 msec respectively. R1 has characteristics similar to an H-reflex. R2 is a long-loop reflex of unknown pathway assumed to involve similar circuits and rostral turn around points when elicited by both arm and leg stimulation. Mean spinal cord conduction time between the seventh cervical and fifth lumbar spinous processes, is given by (formula; see text) It measured 7.95 msec and the calculated mean conduction velocity was 57.9 +/- 5.7 m/sec.     

Corneal reflex in normal and pathological subjects

The orbicularis oculi response can be evoked both by mechanical stimulation of the cornea (corneal reflex) and by electrical stimulation of the skin overlying the supraorbital nerve (blink reflex). Mechanical stimuli to the cornea activate A delta and C free nerve endings of the corneal mucosa. Electrical stimuli to the supraorbital nerve activate A beta, A delta and C fibers of the nerve trunk. Both reflexes present a bilateral late response, but the blink reflex shows in addition an early ipsilateral component (R1), which has never been observed with the corneal stimulation in man. We have developed a simple technique of electrical stimulation of the cornea which provides stable responses and allows precise measurements of threshold and latency of the reflex. In normal subjects, the threshold ranged from 50 to 350 microA, and the maximal stimulus that the subject could bear (tolerance level) ranged from 1000 to 2500 microA. The minimal latency to tolerance level stimuli was 39 +/- 3 msec. The latency difference between the direct responses evoked from the two opposite corneas never exceeded 8 msec and the difference between the direct and consensual responses elicited from the same cornea never exceeded 5 msec. An early ipsilateral component similar to the R1 response of the blink reflex was not observed, even with supramaximal stimulation. The electrically evoked corneal reflex was normal in 10 cases of essential trigeminal neuralgia, while the responses showed significant abnormalities in 18 subjects submitted to thermocoagulation of the Gasserian ganglion as a treatment of neuralgic pain, as well as in 2 cases of symptomatic neuralgia.(ABSTRACT TRUNCATED AT 250 WORDS)     

Blink reflex in patients with hemifacial spasm. Observations during microvascular decompression operations

The blink reflex cannot normally be elicited during surgical anesthesia using inhalation anesthetics. However, in patients with hemifacial spasm (HFS) the early component of the reflex response (R1) can be elicited on the affected side but not on the unaffected side during such anesthesia. The electromyographic (EMG) response from the mentalis muscle to stimulation of the supraorbital nerve was recorded during microvascular decompression (MVD) of the facial nerve to relieve HFS and compared to the response from the same muscle to stimulation of the zygomatic branch of the facial nerve in four patients. During the operation before the facial nerve was decompressed, contractions in both the orbicularis oculi and the mentalis muscles could be elicited by stimulation of the supraorbital nerve (mean latencies 12.2 +/- 1.9 and 12.9 +/- 2.0 ms, respectively). When the facial nerve had been decompressed the blink reflex could no longer be elicited, and there was no response from the mentalis muscle to stimulation of the zygomatic branch of the facial nerve. Compound action potentials (CAP) recorded from the 7th cranial nerve in response to stimulation of the supraorbital nerve had latencies of 7.5 ms +/- 1.4 ms to the negative peak.     

Clinicophysiological studies of orbicularis oculi reflex in brain stem diseases]

Orbicularis oculi reflex is consisted of two components; one is early reflex response (R1) which is 14.1 +/- 2.2 msec in latency, and the other is late reflex response (R2) that is 35.0 +/- 8.6 msec in latency (mean +/- 2SD). R1 is observed on the ipsilateral side of stimulation and R2 is visible bilaterally on ipsiand contralateral sides of stimulation. Clinically orbicularis oculi reflex can be recorded easily, however, its clinical and physiological significance has not been clearly solved. Orbicularis oculi reflex was tested on patients with the brain stem lesions of various kinds, and the factors influencing to the reflex pathways were examined, such as the site and the type of lesions, sleep, several anesthetics and the level of consciousness. Results obtained were as follows; 1) both R1 and R2 disappeared or prolonged in latencies by nuclear as well as peripheral facial nerve palsy, since the facial nerve was the final common pathway. 2) on cases of facial sensory disturbance R1 prolonged in latency with concomitant delay of R2 response. 3) both R1 and were attenuated as soon as the alpha rhythmicity of the EEG was disappeared. Administration of thiamylal also attenuated both the R1 and R2, however, ketamine (a dissociative anesthetic) enhanced the R1 in amplitude and suppressed R2. These findings indicated that the R1 and R2 were in some extent regulated from brain stem reticular formation and the orbicularis oculi reflex should be an useful diagnostic measure to detect imparied consciousness. 4) also the reflex should be used to know the extent of neoplastic process in the cerebellar hemisphere to the brain stem. 5) recovery curve of R1 from double shock stimuli (conditioned and test) indicated that this reflex was different from H wave of the spinal cord because of absent refractory period and shorter facilitatory period ranging between 40 to 60 msec.     

Voluntary eyelid contraction modifies the blink reflex recovery cycle

In order to determine the extent to which the recovery cycle of the blink reflex is modified by voluntary contraction of the eyelids we investigated the electrically elicited blink reflex with paired stimuli of identical intensity in 9 healthy subjects. We pseudorandomly administered two interstimulus intervals (150 ms and 250 ms) in three different conditions of voluntary contraction of the orbicularis oculi muscle (relaxed, mild lid closure and strong lid closure). Our results show that inhibition of the R2 response following the second stimulus is significantly reduced with voluntary contraction. In addition, we found significantly larger amplitudes and shorter latencies of R1 and R2 with voluntary contraction. We conclude that in healthy subjects, voluntary eyelid contraction causes facilitation or disinhibition not only in the nuclei of the seventh cranial nerve, but also in the polysynaptic pathway of the R2. The possible implications on the interpretation of clinical data are discussed.     

Blink reflex alterations in diabetic patients with or without polyneuropathy

The main aim of this study is to evaluate the role of blink reflex for early diagnosis of cranial neuropathy in diabetic patients with or without polyneuropathy. Ninety-five diabetic patients were included in the present study for the evaluation of blink reflex. The diabetic patients were divided into two groups according to having diabetic neuropathy or not. Both R1, R2i and R2c latencies in all diabetic patients with or without polyneuropathy were prolonged relative to controls and the differences were statistically significant (p < .001). R1 latencies in diabetic patients with polyneuropathy were prolonged relative to diabetic patients without polyneuropathy and the differences were statistically significant (p < .001). These findings presumably reflect that facial nerve is severly involved in diabetic polyneuropathy. Finally blink reflex is of value in detection of clinically silent intraaxial brainstem functional abnormalities or extraaxial lesions in diabetic patients before peripheral neuropathy.     

Electrically and mechanically elicited blink reflexes in infants and children--maturation and recovery curves of blink reflex.

We studied the electrically and mechanically elicited blink reflexes in 2 groups of subjects, i.e., 237 newborn infants, 25-41 weeks of conceptional age, and 74 children, 1 month-12 years of age. In infants after 25 weeks of conceptional age we could usually induce the early response (R1) and ipsilateral late response (R2), while the contralateral late response (R2') of the electrical blink reflex became apparent after 33 weeks of conceptional age and the frequency of the appearance of R2' reached more than 60% after 38 weeks of conceptional age. After 7 months of age, R2' was usually observed. The R1 latency in full-term newborns was close to adult values, while the R2 and R2' latencies reached adult values at 7-12 years. After 1 year of age the latency of the R2 mechanical blink reflex had a tendency to be shorter than that of the electrical blink reflex. Under 35 weeks of conceptional age, the recovery curves of the blink reflex were considerably different from those of full-term infants, and premature infants showed little or no evidence of inhibition. These results indicate the absence of inhibitory interneurones in premature infants.     

Blink reflex in Parkinson's disease with levodopa-induced dyskinesia

We have studied the electrically evoked blink reflex (R1 and R2 components) in 40 parkinsonian patients with levodopa-induced dyskinesia (15 with facial dyskinesia, 13 with limb-truncal dyskinesia and 12 with mixed dyskinesia). R2 latencies (both ipsilateral and contralateral) were significantly prolonged in dyskinetic patients. These findings are indicative of decreased excitability of brainstem interneurones in the dyskinetic parkinsonians. We found no correlation between the neurophysiological pattern of blink reflex and the localization of dyskinesia.     

Blink reflex and stimulus detection by the facial nerve in 21 diabetics. Testing before and after precise blood sugar normalization by the artificial pancreas

Study of the blink reflex and stimulodetection of the facial nerve in 21 diabetics with chronic hyperglycemia (HbA1C: 12.17 +/- 2.34%) shows normal or slightly prolonged R1, R2 and M latent periods in most cases. Forty-eight hour metabolic correction by artificial pancreas produces an overall improvement in the latent periods of the blink reflex, while the motor latent period of the facial nerve remained unchanged. The improvement, also observed in studies of limb responses, points towards a metabolic process directly related to the hyperglycemia.     

Vestibular short latency responses to pulsed linear acceleration in unanesthetized animals.

Linear acceleration transients were used to elicit vestibular compound action potentials in non-invasively prepared, unanesthetized animals for the first time (chicks, Gallus domesticus, n = 33). Responses were composed of a series of up to 8 dominant peaks occurring within 8 msec of the stimulus. Response amplitudes for 1.0 g stimulus ranged from 1 to 10 microV. A late, slow, triphasic, anesthesia-labile component was identified as a dominant response feature in unanesthetized animals. Amplitudes increased and latencies decreased as stimulus intensity was increased (MANOVA P less than 0.05). Linear regression slope ranges were: amplitudes = 1.0-5.0 microV/g; latencies = -300 to -1100 microseconds/g. Thresholds for single polarity stimuli (0.035 +/- 0.022 g, n = 11) were significantly lower than those of alternating polarity (0.074 +/- 0.028 g, n = 18, P less than 0.001). Bilateral labyrinthectomy eliminated responses whereas bilateral extirpation of cochleae did not significantly change response thresholds. Intense acoustic masking (100/104 dB SL) produced no effect in 2 animals, but did produce small to moderate effects on response amplitudes in 7 others. Changes were attributed to effects on vestibular end organs. Results of unilateral labyrinth blockade (tetrodotoxin) suggest that P1 and N1 preferentially reflect ipsilateral eighth nerve compound action potentials whereas components beyond approximately 2 msec reflect activity from vestibular neurons that depend on both labyrinths. The results demonstrate that short latency vestibular compound action potentials can be measured in unanesthetized, non-invasively prepared animals.     

Maturation of blink reflex in children

The blink reflex was examined in 57 subjects aged from neonate to adult in the alert state. The ipsilateral late response (R2) was elicited in all subjects and considered most suitable to evaluate maturational changes of the blink reflex. In a few subjects older than 3 years and of adults, the ipsilateral early response (R1) was difficult to observe. The contralateral late response (R2') could not be obtained in 32% of neonates and infants. From the observation about developmental change of an interference pattern, a latency shortening of R2 and a latency difference between R2' and R2, the blink reflex in children may be considered as mature at no later than 5 years of age. In addition, the R2 latency tended to increase temporarily through 1 or 2 years from late infancy. The reflex circuit evaluated by the blink reflex in children may partially change its makeup after the early infantile period and is almost fully mature at no later than 5 years.     

Developmental changes in electrically elicited blink reflex in infancy and childhood

Developmental changes in electrically elicited blink reflex (BR) in 118 normal subjects, aged from 32 weeks of conceptional age to 14 years of age. During the waking state, R1 and bilateral R2 responses were always elicited. The latency of R1 shortened rapidly during the neonatal period, reaching the adult value at 3 months of age. The latency of R2 and contralateral R2 (CR2), which were elicited synchronously after 3 years of age, shortened slowly during childhood and reached the adult value at 6 years of age. The conduction indexes of R1, R2 and CR2, which were calculated as head circumference/latency (m/sec), increased rapidly during the neonatal period and reached the adult values at 6 years of age. The influence of NREM sleep on BR in neonates was different from those in infants and children. While BR of neonates in NREM sleep showed similar to that seen in wakefulness, BR of infants and children after one month of age was suppressed during NREM sleep. BR pattern in NREM sleep in infants and children after 6 months of age was similar to that seen in adults.     

The effect of paired stimuli on blink reflex latencies in normal subjects

Introduction: In this study we assessed the effect of paired stimuli on the latencies and amplitudes of the blink reflex. Methods: Blink reflexes were performed with single and paired (5‐ms interstimulus interval) stimuli in 47 patients. The changes in latencies between paired and single stimuli were calculated. Results: Paired stimulation produced two types of R1 waveform morphologies: single‐ and double‐peaked waveforms. Increases in R1 and contralateral R2 latencies with paired stimulation were significantly higher in those with single‐peaked R1 responses compared to those with double‐peaked R1 responses. Conclusions: Interpreting the blink reflex latencies using paired stimulation requires visualization of the R1 waveform morphology. A double‐peaked R1 response requires no change in normal latency values, but the latency of a single‐peaked R1 should be interpreted from the second shock artifact. The effect on the R2 latency is variable. Muscle Nerve, 2011     

A study of blink reflex in person with severely handicapped]

1) We evaluated blink reflex from 50 cases of severe handicapped. 7 cases (14%) had normal blink reflex. Abnormality of prolonged latency or no response of blink reflex was much more easily seen on R2 and R2' than R1, and dysfunction of spinal trigeminal complex or bulbar reticular formation might be existed in those cases. 2) We found abnormal blink reflex had some relationship with mental disturbance or bulbar function. Result of ABR and head CT also suggested that some kinds of cerebral factors might influenced to blink reflex of severe handicapped cases. 3) Blink reflex was one of the useful records for severe handicapped patients to evaluated underline pathogenesis of brain stem function.     

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.