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Clinical similarity of Alzheimer and Creutzfeldt-Jakob disease.

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The myelographic findings in almost all previously
reported cases demonstrated variable intradural filling defects 121, most of them characteristic of diffuse
arachnoiditis. We know of only 1 report of an isolated cyst
without surrounding arachnoiditis 141. If one recognizes
the uncommon phenomenon of puncturing an intraspinal
cyst at myelography, the correct diagnosis may be suggested, especially if the patient has been in an endemic area
such as Mexico or Eastern Europe. The realization that a
cyst has been punctured should begin when one encounters
the cerebrospinal fluid dynamics of unusually rapid egress
of fluid without subsequent flow. Sequential roentgenograms after introduction of contrast material may suggest
the interior of a cyst.
R efirences
I . Dixon HBF, Lipscomb FM: Cysticercosis: an analysis and followup of 450 cases, series 299 (Medical Research Council Special Report). London, H e r Majesty's Stationery Office, 1961
2. Dorfsman J: Radiological aspects of spinal cysticercosis. Acta
Radio1 [Diagnl (Stockh) 5:1003-1006, 1966
3. Firemark H . Spinal cysticercosis. Arch Neurol 35:25O-25 1,
1978
4. Santin G, Vargas J: Roentgen study of cysticercosis of central
nervous system. Radiology 86:520-527, 1966
Clinical Similarity
of Alzheimer and
Creutzfeldt-Jakob Disease
C. P. Watson, MD
Ehle and Johnson 1 1J pointed out that the clinical and electroencephalographic distinction between Alzheimer disease and Jakob-Creutzfeldt disease may be difficult, and
sometimes requires pathological examination. The following case illustrates that rapidly progressing dementia,
myoclonus, and periodic complexes on the electroencephalogram may all be features of Alzheimer disease.
A 5 5-year-old woman developed dementia and myoclonic
jerking of the upper extremities ten months before death.
An EEG showrd diffuse slow activity in the 3 to 4 cps
range. A tentative diagnosis of Jakob-Creutzfeldt disease
was made. We examined t h e p a t i e n t three months prior to
death. H e r dementia had progressed. She had pseudobulbar palsy and bilateral hyperreflexia of the limbs with upgoing toes and sustained clonus. Symmetrical myoclonic
jerking ot all four extremities occurred every few minutes
and was exaggerated by intentional movements An EEG
Accepted tor publication May 17, 1979.
Address reprint
Dr Watson, 3025 ~~~~~~~i~ St, Suite
5 0 4 , Mississauga, Ont, Canada L5A 2H1.
368 Annals of Neurology Vol 6 No 4 October 1079
showed diffuse slowing of background activity with intermittent projected slow activity, suggesting a widespread
disturbance of function. Periodic low-amplitude, sharp
transients and medium-amplitude sharp waves ant1 spikes
were seen, diffusely and synchronously distributed to all
head regions and separated by 4 t o 8 seconds o f lowamplitude activity. Myoclonic jerks had n o electrophysiological accompaniment except for movement artifact. Jakob-Creutzfeldt disease was helieved t o be the
most likely diagnosis. The patient died three months later.
At autopsy the main finding was hronchopneuriionia
bilaterally. The brain weighed 1,100 gm and showed moderate ventricular dilatation with no cortical atrophy o n sectioning (Fig 1). Microscopic examination showed generalized neuronal degeneration in the cerebral hemispheres
with senile plaques and neurofibrillary tangle fornm;xion
(Fig 2). Granulovacuolar degeneration was prominent in
the hippocampus. No morphological evidence of JakobCreutzfeldt disease was present. The pathological diagnosis
was Alzheimer disease.
Ehle and Johnson 5 patient had subacute dementia without myoclonus, with EEG changes rc-sembling chow of
Jakob-Creutzfeldt disease. One previous case of quasiperiodic EEG abnormalities in Alztieinier disease was
reported by Jacob 131. Faden and Townsencl 121 have reported 2 cases of myoclonus associatecl with Alzheimer disease. The findings i n our patient reemphasize the clinical
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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similarity, clinical, jakos, disease, alzheimers, creutzfeldt
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