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Brainstem auditory evoked responses in progressive supranuclear palsy.

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Brainstem Auditory
Evoked
Responses
. in Progressive
Supranuclear Palsy
Eduardo S. Tolosa, MD, and James A. Zeese, M D
F i g 2.Microscopic section of cerebral cortex shows senile
plaques and neurojibrillary tangles. (Bielschowsky; x575 bejove 3 0 % reduction.)
resemblance of some cases of anatomically verified Alzlreimer disease to Jakob-Creutzfeldt disease.
,?derences
AL, Johnson PC: Rapidly evolving EEG changes in a case
of Alzheimer disease. Ann Neurol 1:593-595, 1979
Faden AL, Townsend JJ: Myoclonus in Alzheimer disease.
Arch Neurol 33:278-280, 1976
i. Jacob H: Muscular twitching in Alzheimer's disease, in
Wolstenholme GEW, O'Connor M (eds): Alzheimer's Disease
and Related Conditions. London, Churchill, 1970, pp 75-93
. Ehle
.)_
Progressive supranuclear palsy (PSP) is known to produce
widespread neuronal changes in the brainstem. Therefore,
we studied brainstem auditory evoked responses (BAER)
in 7 patients with typical PSP, 4 with moderate and 3 with
severe symptoms of brainstem dysfunction. All patients
were female; the mean age was 65 years and the age range,
50 to 74.
Clicks generated by 0.1-msec square waves with alternating polarity were delivered to each ear separately at 10
per second, 60 d b above the patient's hearing threshold.
The BAER was recorded between Cz and the ipsilateral ear
lobe, and at least two repeatable trials (less than 0.08 msec
difference in interwave latencies) were obtained. Satisfactory recordings were achieved for 12 of 14 ears. For each
patient the individual interwave latencies fell within the
normal range (99% tolerance limits) of our young normal
subjects using the same technique, and the amplitude ratio
of wave IV/V to wave I exceeded 1.0 in all repeatable trials.
The patient's mean 1-111 latency was 2.26 msec (SD 0.18)
and 111-V, 1.86 msec (SD 0.21), values not significantly
different from those reported by Rowe [ 11 for a group of
normal older subjects (mean 1-111, 2.19 msec, SD 0.27;
mean 111-Y, 1.82 msec, S D 0.21) using similar technique.
There was no correlation between interwave latencies and
the severity of the patient's brainstem symptoms.
The results indicate that BAERs can be normal in PSP
regardless of the severity of disease. This is probably because the auditory pathways are intact, or because slight
neuronal changes in the central auditory nuclei seen in
some PSP patients [2] are not reflected in an abnormality.
The frequent disorders in BAER seen in multiple sclerosis
indicate that white matter tracts are necessary for the conduction, if not the generation, of BAER waves; the relative
contribution of ceptral nuclei is less clear. O u r results indicate that mild central neuronal changes may not alter
BAER generation o r conduction.
References
1. Rowe MJ: Normal variability of the brain-stem auditory
evoked response in young and old adult subjects. Electroencephalogr Clin Neurophysiol 44:459-470, 1978
2 . Steele JC, Richardson JC, Olszewski J: Progressive supranu-
clear palsy. Arch Neurol 10:333-359, ' 9 6 4
From the Department of Neurology, University of Minnesota
Hospitals, Minneapolis, MN 55455.
Accepted for publication June 9, 1979.
Notes and Letters
369
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progressive, response, auditors, brainstem, palsy, evoked, supranuclear
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