scholarly journals Evoked Potential Studies in Friedreich's Ataxia and Progressive Early Onset Cerebellar Ataxia

1988 ◽  
Vol 15 (3) ◽  
pp. 292-298 ◽  
Author(s):  
M. Vanasse ◽  
L. Garcia-Larrea ◽  
Ph. Neuschwander ◽  
P. Trouillas ◽  
F. Mauguière
Keyword(s):  
Cerebral Cortex ◽  
Evoked Potentials ◽  
Cerebellar Ataxia ◽  
Conduction Velocity ◽  
Early Onset ◽  
Auditory Evoked Potentials ◽  
Evoked Potential ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Potential Recording

ABSTRACT:We recorded somatosensory evoked potentials (SEP) in 15 patients affected by Friedreich's ataxia (FA) and in 9 patients with progressive early onset cerebellar ataxia (PEOCA). Brainstem auditory evoked potentials (BAEP) were also recorded in 14 FA patients and in five PEOCA patients. SEP results showed clear differences between groups of FA, evidence of peripheral involvement was seen in all patients, with absence of the N9 potential or a major reduction of its amplitude. In patients in whom central responses could be recorded, conduction velocity was normal or near normal up to the brainstem but was reduced from brainstem to cerebral cortex. Four patients with PEOCA had SEP abnormalities similar to those seen in FA. In the five other patients, the amplitude and latency of N9 were normal but conduction velocity was reduced from brainstem to cerebral cortex. In FA, BAEP were abnormal in all patients with a disease duration of four years or more but were normal in four of the five PEOCA patients. Systematic evoked potential recording is useful in the investigation of hereditary ataxias.

Limitations of Brain Stem Auditory Evoked Potentials for Intraoperative Monitoring during a Posterior Fossa Operation: Case Report and Technical Note

Neurosurgery ◽  
1985 ◽  
Vol 16 (6) ◽  
pp. 818-821 ◽  
Author(s):  
Joseph H. Piatt ◽  
Rodney A. Radtke ◽  
William C. Erwin
Keyword(s):  
Evoked Potentials ◽  
Brain Stem ◽  
Posterior Fossa ◽  
Intraoperative Monitoring ◽  
Auditory Evoked Potentials ◽  
Evoked Potential ◽  
Technical Note ◽  
Potential Recording ◽  
The Brain ◽  
Posterior Fossa Operation

Abstract We have encountered an example of the insensitivity of brain stem auditory evoked potentials (BAEPs) for monitoring the brain stem during a posterior fossa operation. the addition of somatosensory evoked potential recording to conventional BAEP protocols is readily accomplished and is likely to improve the sensitivity of intraoperative electrophysiological assessment of brain stem function.

scholarly journals The importance of central auditory evaluation in Friedreich's ataxia

2018 ◽  
Vol 76 (3) ◽  
pp. 170-176 ◽  
Author(s):  
Bianca Simone Zeigelboim ◽  
Hélio A. G. Teive ◽  
Michèlli Rodrigues da Rosa ◽  
Jéssica Spricigo Malisky ◽  
Vinicius Ribas Fonseca ◽  
...  
Keyword(s):  
Pure Tone ◽  
Evoked Potential ◽  
Pure Tone Audiometry ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Auditory Evoked Potential ◽  
Acoustic Reflex ◽  
Cross Sectional ◽  
Significant Difference ◽  
Central Auditory Function

ABSTRACT Objective To assess central auditory function in Friedreich's ataxia. Methods A cross-sectional, retrospective study was carried out. Thirty patients underwent the anamnesis, otorhinolaryngology examination, pure tone audiometry, acoustic immittance measures and brainstem auditory evoked potential (BAEP) assessments. Results The observed alterations were: 43.3% in the pure tone audiometry, bilateral in 36.7%; 56.6% in the BAEP test, bilateral in 50%; and 46.6% in the acoustic immittance test. There was a significant difference (p < 0.05) in the comparison between the tests performed. Conclusion In the audiological screening, there was a prevalence of the descending audiometric configuration at the frequency of 4kHz, and absence of the acoustic reflex at the same frequency. In the BAEP test, there was a prevalence of an increase of the latencies in waves I, III and V, and in the intervals of interpeaks I-III, I-V and III-V. In 13.3% of the patients, wave V was absent, and all waves were absent in 3.3% of patients.

scholarly journals Molecular Analysis of Friedreich's Ataxia in Macedonian Patients

2008 ◽  
Vol 11 (1) ◽  
pp. 61-64 ◽  
Author(s):  
S Kocheva ◽  
S Trivodalieva ◽  
S Vlaski-Jekic ◽  
M Kuturec ◽  
G Efremov
Keyword(s):  
Molecular Analysis ◽  
Early Onset ◽  
Trinucleotide Repeat ◽  
Neurodegenerative Disorder ◽  
Autosomal Recessive Inheritance ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
The Republic ◽  
Gaa Repeats ◽  
Frataxin Gene

Molecular Analysis of Friedreich's Ataxia in Macedonian PatientsFriedreich's ataxia (FRDA) is rare a progressive neurodegenerative disorder of autosomal recessive inheritance, which is associated with an unstable expansion of a GAA trinucleotide repeat in the first intron of the frataxin gene on chromosome 9q13. We have performed molecular analyses of the frataxin gene of 40 patients with spinocerebellar ataxia from the Republic of Macedonia. Fifteen had early onset of progressive ataxia (before the age of 25), while the remainder were over 25 years old at the time of diagnosis. Only 14 patients had a mutation in the frataxin gene and all of these had early onset ataxia. The number of GAA repeats was in the normal range in 50 healthy individuals.

Perturbation of precision grip in Friedreich's ataxia and late-onset cerebellar ataxia

1994 ◽  
Vol 9 (6) ◽  
pp. 650-654 ◽  
Author(s):  
Joachim Hermsdöourfer ◽  
Karl Wessel ◽  
Norbert Mai ◽  
Christian Marquardt
Keyword(s):  
Cerebellar Ataxia ◽  
Late Onset ◽  
Precision Grip ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia

scholarly journals A wireless marker system to enable evoked potential recordings using a wireless EEG system (EPOC) and a portable computer

2013 ◽  
Author(s):  
Johnson Thie
Keyword(s):  
Evoked Potentials ◽  
Evoked Potential ◽  
Low Cost ◽  
Setup Time ◽  
Checkerboard Pattern ◽  
Marker System ◽  
Potential Recording ◽  
Receiver Unit ◽  
Small Form Factor ◽  
Wireless Eeg

As wireless EEG devices have become affordable at low cost, have a small form factor and quick setup time, they can be deployed at universities and schools for teaching purposes. However they have not been applied for evoked potential recording since they lack an option to receive stimulus markers. Meanwhile evoked potential recording is required for functional assessment of the sensory systems such as auditory and visual. This paper describes a wireless system that embeds information about the stimulus in the EEG channels. The transmitter unit is connected to the stimulus device to detect the stimulus and transmit the stimulus information to the receiver unit. The receiver unit attached to two of the EEG electrodes decodes the information and generates a pulse across the electrodes. The pulse width conveys the information about the stimulus. Hence the stimuli are synchronised with the EEG data allowing users to evaluate the evoked potentials in the offline processing. The wireless marker system was verified with audio stimuli consisting of 1000Hz and 1200Hz tones and reliably generated pulses with 100ms and 200ms width respectively. The delay between the onset of the tone and the onset of the pulse was 19.3 +/- 0.1ms. Since the variability of the delay was under 1ms and so negligible, the evoked potentials could be evaluated reliably. The evoked potential could be shifted back by 19.3ms to compensate for the delay. The system was also verified with a black-and-white checkerboard pattern stimuli and reliably generated pulses with 100ms width when the pattern reversed. The delay between the onset of the reversal and the onset of the pulse was 6.4ms. Similarly the variability of the delay was negligible.

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