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Diagnostic value of periodic complexes in CreutzfeldtЦJakob disease.

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Diagnostic Value of Periodic Complexes in
Creutzfeldt–Jakob Disease
Bernhard J. Steinhoff, MD,1,2 Inga Zerr, MD,2 Maya Glatting, MD,2 Walter Schulz-Schaeffer, MD,3
Sigrid Poser, MD,2 and Hans A. Kretzschmar, MD3,4
In 1996, our group published objective electroencephalogram (EEG) criteria to define periodic sharp-wave complexes
(PSWCs) suggestive for Creutzfeldt–Jakob disease (CJD). These criteria have since then been strictly applied in all cases
reported to us as possible CJD in the course of the German CJD surveillance study. Furthermore, EEG analysis of the
records was performed without any additional information on complementary clinical and laboratory data. In this study,
we investigated sensitivity, specificity, and the predictive values of these EEG criteria exclusively in cases in which
autopsy confirmed (n ⴝ 150) or excluded (n ⴝ 56) CJD. EEG criteria were positive in 64% (n ⴝ 96) of the CJD cases
and falsely positive in 9% (n ⴝ 5) of other dementias. The resulting figures for sensitivity, specificity, and positive and
negative predictive values were 64%, 91%, 95%, and 49%, respectively. In the falsely positive cases, Alzheimer’s disease
(n ⴝ 4) and vascular dementia (n ⴝ 1) were the underlying diseases. However, only in one of these five cases both
clinical and EEG data would have led to the false-positive result to diagnose probable CJD. These data prove the high
diagnostic value of our objective EEG criteria in CJD.
Ann Neurol 2004;56:702–708
Sporadic Creutzfeldt–Jakob disease (sCJD) is the most
frequent human prion disease.1–3 Because the diagnosis
is proved only post mortem and sCJD always has a
fatal course, it is essential to establish diagnostic intra
vitam criteria as sensitive and especially as specific as
possible. For decades, along with the widely accepted
clinical criteria,4 periodic sharp-wave complexes
(PSWCs) in the electroencephalogram (EEG) were reported to represent the most typical finding in the
course of sCJD5–9 and therefore were included in the
World Health Organization classification criteria of
Because PSWCs in sCJD were too poorly defined
for objective diagnostic use, especially in epidemiological or multicenter studies, we proposed objective criteria.8 In this previous study, we found an excellent
interobserver reliability along with a sensitivity of 67%
and a specificity of 86%, respectively.8 The drawback
of this study was the relatively small group of patients,
with 15 patients in the CJD and 14 in the non-CJD
group. In addition, all non-CJD cases were scored according to their clinical course and not confirmed by
autopsy. Since then, we have applied our objective
EEG criteria as follows. One board-certified electroen-
cephalographer (B.J.S.) scored all EEG records of clinically suspected CJD patients reported to the German
Surveillance Study Group without any information on
clinical and additional neuroradiological or biochemical
data. We thus were able to collect a large series of patients, comprising 150 autoptically verified versus 56
autoptically excluded cases. This article addresses the
diagnostic value of our objective EEG criteria in this
From the 1Epilepsy Center Kork, Kehl-Kork; Departments of 2Neurology and 3Neuropathology, Georg-August University of Göttingen; and 4Department of Neuropathology, Ludwig-Maximilians
University of Munich, Munich, Germany.
Address correspondence to Dr Steinhoff, Epilepsy Center Kork,
Landstrasse 1, 77694 Kehl-Kork, Germany.
Patients and Methods
EEG records of all patients reported to us as suspected CJD
were collected and scored by one of us (B.J.S.) who was not
aware of the original EEG report results or any additional
diagnostic data of the patients. Based only on the objective
EEG criteria previously published8 and shown in Table 1,
classification of EEG recordings was as typical or not typical.
We analyzed only the data of patients who had undergone
autopsy to confirm or rule out CJD. From January of 1996
until August of 2000, it was possible to obtain a definite
neuropathological postmortem CJD diagnosis in 330 of
1001 patients reported to us as suspected CJD cases. In 56
cases, CJD was autoptically excluded. Demographic and clinical characteristics of the autopsied CJD patients were not
different from the patients not undergoing autopsy. The 56
Received Mar 18, 2004, and in revised form Jul 19. Accepted for
publication Jul 19, 2004.
Published online Sep 24, 2004, in Wiley InterScience
( DOI: 10.1002/ana.20261
© 2004 American Neurological Association
Published by Wiley-Liss, Inc., through Wiley Subscription Services
Table 1. Objective Diagnostic Criteria of Periodic SharpWave Complexes Typical for Sporadic Creutzfeldt–Jakob
● Strictly periodic cerebral potentials, the majority with a
duration between 100 and 600 milliseconds and an intercomplex interval between 500 and 2,000 milliseconds
● Generalized and lateralized complexes accepted
● At least five repetitive intervals with a duration difference
of less than 500 milliseconds to rule out semiperiodic
From Steinhoff and colleagues.8
non-CJD cases were completely included in our study,
whereas a sample of 150 cases among the neuropathologically verified CJD cases appeared to be sufficient for the intention of this study. These 150 patients did not differ in
any aspect from the remaining patients and were randomly
selected from our charts. In addition to the EEG and neuropathological data, we considered the clinical data (onset
and duration of disease, age at death, and clinical symptoms
at onset and during the course of the disease).
Sensitivity, specificity, and the (positive and negative) predictive values of the intra vitam EEG diagnosis “typical” or
“not typical” of the clinical data and of both criteria were
assessed. Statistical tests comprised the ␹2 test or the Fisher
exact tests on a 0.05 level, if applicable.
Clinical Data
Table 2 shows the clinical data of the CJD and the
non-CJD group. The mean duration of the disease and
the 6- and 12-month survivor rate was considerably
higher in the non-CJD group, whereas the mean age
did not differ markedly. However, the higher standard
deviation for age at onset and mean duration of the
disease indicates the less homogenous clinical characteristics in the non-CJD group.
Clinical Symptoms in the Autoptically Verified
Creutzfeldt–Jakob Disease Cases
The clinical symptomatology could be completely verified in 131 of the 150 CJD cases. In the remaining
cases, only single clinical symptoms could not be
judged sufficiently to be included in the data analysis.
The detailed figures are shown in Table 3.
The onset of the disease was defined as the time
point when first unequivocal neurological, psychiatric,
or other clinical symptoms became apparent and were
documented. At the beginning of the disease, clinical
symptoms were similarly distributed with the exception
of visual or oculomotor symptoms which occurred significantly more often in CJD. Conversely, myoclonic
jerks appeared significantly less frequently in the CJD
group. Distinct clinical differences became more evident in the course of the disease, because, beyond visual or oculomotor symptoms, rapidly progressive dementia, cerebellar symptoms, and akinetic mutism
were diagnosed significantly more frequently in CJD
cases. The incidence of myoclonic jerks did not differ
anymore between the two patient groups.
The most frequent neuropathological diagnosis in
the non-CJD group was Alzheimer’s disease. The detailed figures are shown in Table 4.
Sensitivity, Specificity, and Predictive Values of the
Clinical Data
Clinical symptoms alone had been sufficient for the
correct diagnosis of CJD in 139 cases (93%). In the
non-CJD cases, clinical criteria suggested the falsely
positive diagnosis of CJD in 34 of 56 cases (61%).
Thus, clinical criteria achieved a sensitivity of 93% and
a specificity of 39%. The positive predictive value
(PPV) was 80% (139/173), and the negative predictive
value (NPV) was 67% (22/33).
Electroencephalogram Recordings
A total
of 443 EEG recordings could be assessed. Typical EEG
findings were apparent in 96 cases (64%). In this
group, a mean of 3.0 ⫾ 1.5 recordings were performed
(range, 1–9). The first typical EEG was recorded 3.7 ⫾
3.1 months after the onset of the disease (0.2–19.2
months); the latest typical EEG was recorded 2.3 ⫾
3.4 months (range day of death, 17.1 months) before
death. Typical EEG findings were always apparent dur-
Table 2. Clinical Data
Female (n)
Male (n)
Female/male ratio
Mean age at onset, range (yr)
Mean duration of disease, range (mo)
Survivors 6 months after onset of disease (%)
Survivors 12 months after onset of disease (%)
Total (n)
CJD Group
Non-CJD Group
92 (61%)
58 (39%)
66 ⫾ 9 (24–85)
7 ⫾ 5.7 (1–25)
31 (55%)
25 (45%)
64 ⫾ 16 (26–85)
22 ⫾ 29.4 (1–156)
CJD ⫽ Creutzfeldt–Jakob disease.
Steinhoff et al: EEG in CJD
Table 3. Clinical Symptoms in the CJD and Non-CJD Group
CJD (n ⫽ 150), N (%)
Non-CJD (n ⫽ 56), N (%)
87/150 (58)
72/147 (49)
51/146 (35)
10/148 (7)
16/148 (11)
12/148 (8)
0/149 (0)
31/55 (56)
19/53 (36)
9/55 (16)
8/55 (15)
10/53 (19)
11/54 (20)
0/55 (0)
144/146 (99)
124/144 (86)
82/146 (56)
92/147 (63)
104/146 (71)
119/148 (80)
65/147 (44)
40/55 (73)
32/51 (63)
17/54 (31)
30/54 (56)
31/52 (60)
39/55 (71)
15/55 (27)
Onset of disease
Rapidly progressive dementia
Cerebellar symptoms
Visual/oculomotor symptoms
Pyramidal signs
Extrapyramidal signs
Myoclonic jerks
Akinetic mutism
In the course of the disease
Rapidly progressive dementia
Cerebellar symptoms
Visual/oculomotor symptoms
Pyramidal signs
Extrapyramidal signs
Myoclonic jerks
Akinetic mutism
Clinical symptoms at onset (time point when first neurological, psychiatric, or other clinical symptoms became apparent and documented) and
during the course of the disease in the CJD and the non-CJD group. Statistical significance was assumed on a 0.05 level, ␹2-test. The clinical
symptomatology could be completely verified in 131 of the 150 CJD cases.
CJD ⫽ Creutzfeldt–Jakob disease; n.s. ⫽ not significant.
ing one of the first five recordings. Before the recording of PSWCs, the mean EEG number was 0.9 ⫾ 1.2
(range, 0 –5).
In 54 CJD cases, typical EEG findings, according to
our criteria, were not apparent (36%, mean number of
recordings 2.8 ⫾ 1.5; range, 1–9). The first recordings
Table 4. Neuropathological Diagnosis in the Non–
Creutzfeldt–Jakob Disease Group
Alzheimer’s disease
B-cell lymphoma
Gliomatosis cerebri
Paraneoplastic encephalitis
Vascular encephalopathies
Vascular dementia
Multiple cerebral infarctions
Inflammatory diseases
Cerebral vasculitis
Meningitis (⫹ hypoxic cerebral lesion)
Progressive encephalomyelitis
Neurodegenerative diseases
Amyotrophic lateral sclerosis
Lewy body dementia
Parkinson’s disease
Other disease
Niemann-Pick’s disease (type C)
Congophil angiopathy
Toxic alcohol encephalopathy
November 2004
Annals of Neurology
Vol 56
No 5
were performed 4.9 ⫾ 4.4 months (range, 0.8 –23.9)
after the onset of the disease; the latest ones were performed 2.5 ⫾ 3.0 months (range, 3 days to 15.4
months) before death. In most cases (n ⫽ 52), one to
five recordings were performed. The distribution of the
number of EEG recordings thus was almost identical as
in the CJD group with typical EEG findings.
total of 230 EEG recordings (mean, 4.1 ⫾ 2.9; range,
1–17) could be analyzed. In the non-CJD group without typical PSWCs (n ⫽ 51, 91%), a mean of 4.2 ⫾
2.9 (range, 1–17) recordings were performed. The first
recordings took place 15.4 ⫾ 25.1 months (range,
0.3–141.5) after the onset of the disease, the latest one
3.5 ⫾ 6.6 months (range day of death, 38.9 months)
before death.
An EEG typical for sCJD was found in five patients
(9%); four of them suffered from Alzheimer’s disease.
The first clinical signs of dementia appeared in Patient
1 at age 61 years. Three EEGs were recorded, 5, 6, and
9 months after the onset of the disease. The third one
showed typical PSWCs. The patient died 3 months
later. In Patient 2, two EEGs 4 months after the beginning of the disease had been negative, whereas five
further recordings 1 month later contained typical
PSWCs. He died another 2 months later. The only
EEG in Patient 3 was recorded 1 month after onset
and 4 months before death. Figure 1 shows an example
of this EEG recording.
In Patient 4, autopsy revealed multiple cerebral infarctions. Three EEGs were recorded 28, 24, and 13 days
Fig. Electroencephalogram example of Case 3, suffering from neuropathologically verified Alzheimer’s disease. Only one EEG was
recorded 4 months before the death of the patient. Note the periodic sharp-wave complexes which had been scored as typical for
sporadic Creutzfeldt–Jakob disease cases.
before he died. The second recording showed typical
PSWCs. Whereas EEG recordings 28, 24, and 23 days
before death were negative, a recording 3 days after the
first EEG was positive in Patient 5. In only one of the
five patients with typical EEG findings, the clinical data
also suggested CJD. In the other four cases, because of
the atypical clinical data, a misdiagnosis of probable
CJD was avoided. Table 5 summarizes the data.
Sensitivity, Specificity, and Predictive Values of the
Electroencephalogram Data
We found, based on the correctly diagnosed CJD cases
using only our EEG criteria and the false-positive cases
in the non-CJD group, that sensitivity and specificity
were 64 and 91%, respectively. The PPV was 95%
(96/101), and the NPV was 49% (51/105).
Table 5. Non-CJD Patients with EEG Recordings Typical for Sporadic CJD
Age at Onset
EEG Recordings
Typical EEG Recordings
Alzheimer’s disease
Alzheimer’s disease
Alzheimer’s disease
Multiple cerebral infarctions
Alzheimer’s disease
CJD ⫽ Creutzfeldt–Jakob disease; EEG ⫽ electroencephalogram.
Steinhoff et al: EEG in CJD
Sensitivity, Specificity, and Predictive Values of the
Combination of Clinical and Electroencephalogram
Ninety-five of 206 patients were classified as probable
(typical clinical findings and typical EEG) and 78 as
possible CJD (typical clinical findings but atypical
EEG). The remaining 33 patients were excluded because neither the clinical nor the EEG data suggested
CJD. In the group of 150 autoptically verified CJD
cases, 94 had been classified as probable because of
corresponding clinical and EEG data, if both criteria
were applied. Sensitivity therefore was 63%. As mentioned earlier, only 1 patient of the 56 autoptically
excluded cases fulfilled both the clinical and EEG criteria to be classified as probable CJD, resulting in a
specificity of 98%. PPV and NPV were 99% and
49%, respectively.
In a previous study, our EEG criteria were associated
with a high sensitivity and specificity and an excellent
interobserver reliability documented by a k value of
0.958 that strongly suggested the applicability in multicenter series with various electroencephalographers.
Beyond the small groups of patients, the drawback of
this investigation was the problem that the control
group consisted of patients who had been referred as
suspected CJD cases but for whom clinical criteria had
been used to rule out CJD. It may have happened that
CJD patients with an unusual clinical course were included in this control group, thus causing misleading
results. Therefore, in this study we included only cases
with neuropathologically verified diagnosis and increased the number of cases considerably.
Our data show the applicability of our objective
EEG criteria, especially for specificity, which in our
opinion is probably more important in the diagnosis of
a disease that still has such a devastating prognosis as
CJD. Clinical data alone were not sufficient enough to
meet this objective. Objective EEG criteria alone were
associated with a PPV of 95% and, when added to the
clinical criteria, improved the PPV value of the clinical
symptoms by remarkable 19% to a value of 99%.
PSWCs are not only a sensitive and specific diagnostic indicator in sCJD but also appropriate to differentiate probable sCJD from other prion diseases. With
the exception of patients with the codon 200 mutation, they occur only occasionally in familial CJD10
and are not recorded in patients with kuru,11
Gerstmann-Sträussler-Scheinker syndrome,12 fatal familial insomnia,13 and the new variant of CJD.14
Despite the high specificity of our objective EEG
criteria, there are still rare cases with rapidly progressive
dementia other than sCJD with PSWCs. In our first
series8 as well as in this and other studies,15 Alzheimer’s disease has been the most frequent differential
Annals of Neurology
Vol 56
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November 2004
diagnosis to rule out if PSWCs are recorded. Other
infrequent possibilities may be dementia with Lewy
bodies15 or vascular dementias as in one patient in this
series. It is somewhat satisfying that in this study the
combined specificity of the established clinical and our
EEG criteria resulted in a remarkable figure of 97%. In
the five falsely positive cases based on EEG, only once
was probable CJD still diagnosed after consideration of
the clinical data. The inclusion of other methods such
as the detection of 14-3-3 protein in the cerebrospinal
fluid16 –18 and MRI studies should allow an almost safe
intra vitam diagnosis of sCJD.19,20 Although still a
matter of debate, the 14-3-3 protein has been incorporated in the World Health Organization classification
criteria,4 whereas MRI criteria of sCJD have been proposed19 but have not yet been officially recommended.4 MRI reached a sensitivity of 67% and a specificity
of 93% in a recently published study.20 It would have
been tempting to include it in our analysis. However,
although MRI was performed in almost all cases, we
did not feel safe enough to include it, because the MRI
data were not analyzed by one investigator or at least a
homogenous investigator’s group.19
It is interesting to speculate why PSWCs are not
found in some sCJD cases. It has been discussed that
an imbalance in the subcortical, most probably thalamic, pacemaker systems, may be the underlying condition of PSWCs in CJD.21,22 The crucial role of the
ascending reticulothalamocortical system for the generation of PSWCs becomes more evident, if one considers that because of the cortical degeneration in CJD
patients the physiological sleep architecture is replaced
by a “tracé-alternante”–like pattern. In preterm newborns, this EEG finding of rapidly alternating sleepEEG and PSWCs reflects the still-immature cortical
electrophysiological activity.22–24 In adult patients with
CJD, the very similar EEG findings result from a dramatic degeneration of the formerly intact cerebral network.
PSWCs in CJD may be unilateral.6,24 –28 This may
reflect a state of the disease when the commissural
progress, which has been shown in experiments,29 has
not yet led to the diffuse cortical disease.27,28 Unequivocally, the probability of PSWCs corresponds to the
amount of neuronal cell loss.24 The tendency of unilateral PSWC activity to a bilateral spread during the
course of CJD has been reported25 and has been seen
by us in several instances. The dominating bilateral distribution of PSWCs suggests an underlying midline
pacemaker.30 Traub und Pedley31 emphasized the important role of the corpus callosum for widespread
EEG activity in CJD and suggested that they originate
from a pathological electrophysiological interneuronal
coupling based on diffuse neuronal cell destruction
during the course of the disease. The short intervals of
PSWCs in CJD suggest that the subcortical-cortical
electrophysiological axis is still intact but pathologically
simplified, whereas underlying cortical deafferentiation
typically would result in markedly longer intercomplex
intervals.32–34 Consequently, diseases with dominating
destruction of the white matter are associated with periodic EEG activity with longer intervals than in
CJD.35–38 Finally, the fact that prefinal EEG recordings in sCJD are no longer characterized by PSWCs,
but by low-voltage EEG followed by electrocerebral inactivity,39 strongly supports the hypothesis of a still
partially functioning subcortical-cortical network as a
prerequisite of PSWCs. Moreover, depth electrode
EEG of the basal ganglia further support this assumption, because they showed PSWCs in CJD, which were
inconstantly apparent on simultaneous surface
Recent investigations by our group41 support this hypothesis. It could be demonstrated that parvalbuminimmunoreactive (PV⫹) neurons resembling inhibitory
neuronal function are markedly reduced in several thalamic regions in sCJD and that in CJD patients with
typical PSWCs, a predominant loss of PV⫹ cells was
apparent in the reticular thalamic nucleus. In addition,
marked PV⫹ cell loss was observed in the brain of patients with homozygosity for methionine at codon 129,
namely, in those patients with the highest incidence of
PSWCs of 87%.18 Thus, the involvement of the thalamus may be the key to understanding why PSWCs are
such a typical finding in sCJD, whereas they are hardly
ever found in other human prion diseases.
This work was supported by the Deutsche Forschungsgemeinschaft
(grant BMG 325-4471-02/15, S.P., H.A.K.).
This study was presented in part at the Annual Meeting of the European Neurological Society (ENS), Paris, France, 2001.
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