Are evoked potentials clinically useful in the study of patients with Chiari malformation Type 1?

OBJECTIVE In this study, the authors describe the brainstem auditory evoked potential (BAEP) and somatosensory evoked potential (SSEP) alterations found in a large cohort of patients with Chiari malformation Type 1 (CM-1), the relationship between the BAEPs/SSEPs and the clinical findings, the abnormalities in patients with associated syringomyelia, and the clinical and neuroradiological risk factors that are associated with abnormal evoked potentials (EPs). METHODS A prospectively collected database containing 545 patients with CM-1 was queried to search for patients satisfying the following criteria: 1) an age of at least 14 years, 2) neuroradiological criteria of CM-1, 3) no prior Chiari-related surgeries, and 4) preoperative EP studies conducted at the authors' institution. The 200 patients included in this cohort were classified into CM-0, CM-1, and CM-1.5 subtypes. Linear, planimetric, and angular measurements of the posterior fossa were conducted, as well as syringomyelia measurements. Two separate multiple logistic regression models were used, one to predict the covariates associated with abnormal BAEPs, and a second model to explore the variables associated with an abnormal SSEP. In these models, the BAEPs and SSEPs were dichotomized as being normal or abnormal. RESULTS Headaches were the main symptom in 70.5% of the patients, and Valsalva-induced headaches were most frequent in patients with CM-1 and CM-1.5 compared with patients with CM-0 (p = 0.031). BAEPs were abnormal in 38.5% of patients, and abnormal SSEPs were found in 43.5% of the entire cohort. Syringomyelia was most frequent in patients with CM-0 (64.3%) and CM-1 (51.1%) compared with those with CM-1.5 (34.7%; p = 0.03). Age (OR 1.03, 95% CI 1.00-1.06), the degree of tonsillar herniation (OR 1.08, 95% CI 1.01-1.16), and lower cranial nerve dysfunction (OR 3.99, 95% CI 1.29-14.01) had a statistically significant correlation with abnormal BAEPs. Only age (OR 1.07, 95% CI 1.04-1.10) and the degree of tonsillar herniation (OR 1.11, 95% CI 1.04-1.19) had a statistically significant correlation with abnormal SSEPs. CONCLUSIONS A high percentage of patients with CM-1 exhibited EP alterations regardless of their clinical or radiological findings. These findings suggest that EPs do not add any clinically relevant information nor are they helpful in establishing which symptomatic patients with CM should undergo surgical treatment. However, BAEP and SSEP studies clearly play an important role in incidentally detected patients with CM and may help to establish objective evidence of subclinical dysfunctions. In addition, neurophysiological studies may help to define subgroups of patients who require further testing and follow-up to personalize strategies for the management of incidental and oligosymptomatic patients.

Interside Latency Differences in Brainstem Auditory and Somatosensory Evoked Potentials. Defining Upper Limits to Determine Asymmetry.

PURPOSE: Limits of the interside differences are invaluable when interpreting asymmetry in brainstem auditory evoked potentials and somatosensory evoked potentials (SEP) recordings. The aim of this study was to analyze the normal upper limits of interside latency differences of brainstem auditory evoked potentials and SEP from the posterior tibial nerve and median nerve to determine asymmetry. METHODS: The authors performed a prospective study in 56 healthy subjects aged 15 to 64 years with no neurological or hearing disorders. They analyzed (1) the latencies of I, III, and V waves and I-III, III-V, and I-V intervals and the amplitude ratios V/I and IV/I for brainstem auditory evoked potentials bilaterally; (2) the latencies of N8, N22, N28, and P37 waves and the interval N22-P37 and the amplitude P37 for posterior tibial nerve SEP bilaterally; and (3) the latencies and amplitudes of N9, N13, and N20 waves and N9-N13 and N13-N20 intervals for median nerve SEP bilaterally. The interside differences for these parameters were calculated and analyzed. RESULTS: The authors obtained an upper limit for the interside latency differences from brainstem auditory evoked potentials that was significantly lower than the previously published data. However, the upper limits of interside latency differences for SEP were similar to those previously reported. CONCLUSIONS: The findings of this study should be considered when laboratories analyze asymmetry using the normative data published by another center, however temporarily, in organizing new laboratories.

Brainstem auditory and somatosensory evoked potentials in relation to clinical and neuroimaging findings in Chiari type 1 malformation.

PURPOSE: The aim of this study was to describe the abnormalities found in the recordings of evoked potentials (EPs), in particular those of brainstem auditory evoked potentials and somatosensory evoked potentials, in a homogeneous series of patients with Chiari type 1 malformation (CM-1) and study their relationship with clinical symptoms and malformation severity. CM-1 is characterized by cerebellar tonsils that descend below the foramen magnum and may be associated with EP alterations. However, only a small number of authors have described these tests in CM-1, and the patient groups studied to date have been small and heterogeneous. METHODS: The clinical findings, neuroimages, and EP findings were retrospectively studied in a cohort of 50 patients with CM-1. RESULTS: Seventy percent of patients had EP abnormalities (brainstem auditory evoked potential: 52%, posterior tibial nerve somatosensory evoked potential: 42%, and median nerve somatosensory evoked potential: 34%). The most frequent alteration was an increased central conduction time. Morphometric measurements differed between the normal and pathological groups, although no statistical significance was found when comparing these groups. CONCLUSIONS: A high percentage of patients with CM-1 show EP alterations regardless of their clinical or radiological findings, thus highlighting the necessity of performing these tests, especially in patients with few or no symptoms.

[Brainstem auditory evoked potentials and somatosensory evoked potentials in Chiari malformation]. / Potenciales evocados auditivos del tronco cerebral y somatosensoriales en los pacientes con malformacion de Chiari.

INTRODUCTION: Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with compression in the brainstem, upper spinal cord, and cranial nerves, consequently altering the responses of brainstem auditory evoked potentials (BAEP) and somatosensory evoked potentials (SSEP). However, only a small number of authors have described the indications of these tests in CM, and study groups to date have been small and heterogeneous. AIM: To review the results of BAEPs and SSEPs in published studies of patients with Chiari type 1 malformation (CM-1) or Chiari type 2 malformation (CM-2) as well as the indications of both tests in the diagnosis, treatment, and monitoring of both diseases, especially CM-1. DEVELOPMENT: We present a review article analyzing data from all published studies in Medline starting in 1966, located through PubMed, using combinations of the following keywords: 'Chiari malformation', 'Arnold-Chiari malformation', 'Chiari type 1 malformation', 'Arnold-Chiari type 1 malformation', 'evoked potentials', 'brainstem auditory evoked potentials' and 'somatosensory evoked potentials' as well as records of patients with CM-1 from the neurosurgery and neurophysiology departments at the Hospital Universitari Vall d'Hebron. CONCLUSIONS: Common findings of SSEP are a reduction in cortical amplitude from the posterior tibial nerve, a reduction or absence of cervical median nerve potential, and an increased N13-N20 interval. In BAEP, the most frequent findings are an increased I-V interval and a peripheral or cochlear auditory disturbance.

TITLE: Potenciales evocados auditivos del tronco cerebral y somatosensoriales en los pacientes con malformacion de Chiari.Introduccion. La malformacion de Chiari (MC) incluye una serie de anomalias congenitas que tienen como comun denominador la ectopia de las amigdalas del cerebelo por debajo del foramen magno, lo que puede condicionar fenomenos compresivos del troncoencefalo, la medula espinal alta y los nervios craneales, alterando las respuestas de los potenciales evocados auditivos del tronco cerebral (PEATC) y de los potenciales evocados somatosensoriales (PESS). Sin embargo, las indicaciones de ambas exploraciones en las MC han sido motivo de estudio en un numero limitado de publicaciones, centradas en series cortas y heterogeneas de pacientes. Objetivo. Revisar los hallazgos de los PEATC y los PESS en los estudios publicados en pacientes con MC tipo 1 (MC-1) o tipo 2 (MC-2), y su indicacion en el diagnostico, tratamiento y seguimiento, especialmente en la MC-1. Desarrollo. Es un estudio de revision realizado mediante analisis de los estudios publicados en Medline desde 1966, localizados mediante PubMed, utilizando combinaciones de las palabras clave 'Chiari malformation', 'Arnold-Chiari malformation', 'Chiari type 1 malformation', 'Arnold-Chiari type 1 malformation', 'evoked potentials', 'brainstem auditory evoked potentials' y 'somatosensory evoked potentials', asi como informacion de pacientes con MC-1 valorados en los servicios de neurocirugia y neurofisiologia clinica del Hospital Universitari Vall d'Hebron. Conclusiones. Los hallazgos mas comunes de los PESS son la reduccion en la amplitud cortical para el nervio tibial posterior, la reduccion o ausencia del potencial cervical del nervio mediano y el aumento del intervalo N13-N20. En el caso de los PEATC, los hallazgos mas frecuentes descritos son el aumento del intervalo I-V y la alteracion periferica o coclear.