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On diagnosis of dementia: psychometric investigation and clinical psychiatric evaluation in relation to verified diagnosis
Arch. Gerontol. Geriatr., 4 (1985) 321-332
321
Elsevier
A G G 00126
On diagnosis of dementia: psychometric investigation and clinical psychiatric evaluation in relation to verified diagnosis * Bo H a g b e r g a n d L a r s G u s t a f s o n Departments of Psychiatry 1 and Psychogeriatrics, University Hospital, S-221 85 Lund, Sweden (Received 20 March 1985; accepted 17 September 1985)
Summary Patients with early as well as manifest dementia were studied in a longitudinal way with psychometric and psychiatric methods. Tests such as vocabulary, attention test, block design test, verbal and spatial memory tests, reaction time test and examination for aphasia were used as well as a qualitative analysis of behaviour and personality, The differential diagnosis of Alzheimer's disease. Pick's disease and other dementias with fronto-temporal degeneration and multi-infarct dementia was based on diagnostic rating scales. Differences in cognitive profiles, qualities of behaviour and scores on combinations of psychiatric rating scales were identified for pathoanatomically verified cases with Alzheimer's disease, fronto-temporal degeneration and multi-infarct dementia. psychometry; rating scales; dementia, pseudodementia; personality
Introduction In 1966, a longitudinal study was started at the University Hospital, Lund, Sweden, in order to study the debut and progression of the various dementias from a multi-disciplinary point of view. Psychiatric, psychological and neuropsychological as well as pathoanatomical variables were studied with the purpose to identify early differential signs of dementia and their relationship to progression and post-mortem pathoanatomical diagnosis. This presentation will deal with the predictive capacity of the clinical measures obtained during the patient's disease as validated against post-mortem pathoanatomical diagnosis. Results from the study have been presented successively (Ingvar and Gustafson, 1970; Gustafson and Risberg, 1974, 1979; Gustafson and Hagberg, 1975; Hagberg and Ingvar, 1976; Gustafson et al., 1976, 1981; Brun and Gustafson, 1976, 1978; Johannesson et al., 1977, 1979). * Paper presented at the International Workshop on 'Psychometry in Aging and Dementia', Montecatini Terme, June 25th, 1984, Italy.
0167-4943/85/$03.30 © 1985 Elsevier Science Publishers B.V. (Biomedical Division)
322
Material Patients with incipient, early as well as with manifest dementia were included in the longitudinal study. Patients with chronic psychosis, oligophrenia, addiction, pre-existing epilepsy, severe somatic disorders or apoplexy with gross neurological deficits were excluded. Patients with symptoms indicating a confusional reaction or a non-organic psychiatric illness were treated before final decision of inclusion into the material. All cases went through psychiatric, psychometric, neurological (including radiological and EEG) investigations and the regional cerebral blood flow (rCBF) was measured. The patients have been followed up regularly at least once a year. Autopsy including neuropathological investigation has been performed in 74% of deceased cases. The criteria for the neuropathological diagnostic classification have been presented by Dr. A. Brun earlier at this symposium. The psychometric and psychiatric diagnostic procedure was tested in 58 postmortem verified cases. Eight of these had a senile dementia of the Alzheimer type (SDAT) and a mean age of 79 years, 24 had a presenile Alzheimer disease (AD) and a mean age of 68 years, 11 had a dementia with a fronto-temporal accentuation of the cortical degeneration (DFT) and a mean age of 62 years and 15 patients had a vascular dementia due to multiple infarcts (MID) and a mean age of 71 years.
Methods
Psychometric tests The psychometric battery that we use contains verbal tests, memory tests, a test on logical-inductive reasoning and tests on intellectual and motoric speeds. When indicated, examination for aphasia according to Eisenson's model was used. See also Gustafson et al. (1978). Sometimes also a personality test was included.
TABLE I The standard psychometrictest battery Psychologicaltests Vocabulary Synonyms Attention Test Kohs' Block Design Memory Tests: Paired Associates Object Recognition Memory for Geometrical Designs Memory for Designs(Graham-Kendall) Visual Retention (Benton) Serial Colour Word Test Visual Reaction Time (Simple and Choice) Aphasia Examination(Eisenson)
323 The standard p s y c h o m e t r i c test battery (Table I) Test specification - - Vocabulary, synonyms,
verbal tests measuring degree ot verbal understanding
(Inglis, 1958). Attention test, measuring the patient's ability to follow simple instructions and perform accordingly. - - K o h s ' B l o c k Design, spatial performance test measuring spatial ability as well as logical-inductive reasoning (Wechsler, 1958). - - Paired associates, measuring immediate recall of verbal material (Cronholm and Molander, 1954). - - Object recognition, measuring in a multiple-choice fashion the immediate recognition of concrete objects (Cronholm and Molander, 1954). - - M e m o r y f o r geometrical designs, measuring immediate recall of abstract figures. 'Abstract' in the meaning that the figures are hard to verbalize. - - M e m o r y for designs, measuring spatial perceptual ability as well as immediate memory for simple designs (Graham and Kendall, 1960). Visual retention test, a spatial memory test (Benton, 1963). Colour word test, considered measuring concentration, attention and intellectual speed in solving a task containing incongruent material of the Stroop character. - - Visual reaction time (simple and choice). - - E x a m i n a t i o n f o r aphasia (Eisenson, 1954), evaluating degree of aphasia, apraxia, agnosia, alexia, acalculia, agraphia etc. -
-
-
-
-
-
Diagnostic rating scales
The clinical differentiation was focused upon a recognition of AD, MID and D F T including Pick's disease. The clinical diagnoses were based on three diagnostic schedules containing a limited number of clinical features, which usually were noted in the case reports. The diagnosis of MID was based on the ischemic score (IS) developed by Hachinski et al. (1975). This rating scale is further discussed by Dr. Wagner at this symposium. The validity of IS has been shown in several studies. A score exceeding 7 or 8 points strongly suggests a vascular etiology of the dementia (Harrison et al., 1979; Rosen et al., 1980; Gustafson and Nilsson~ 1982). Dementias of the Alzheimer type were identified using the clinical features and scores presented in Table II. The differentiation between AD and SDAT was based on age of onset, before or after 65 years of age, respectively. The items of the rating scale for diagnosis of AD might need some clarification. 'Early' means that a symptom appears at an early or incipient stage of the disease. 'Logoclonia' is a stuttering-like phenomenon mainly affecting the terminal syllable of words. 'Epileptic seizures' are considered of late onset, when starting after several years' course of the disease, at a manifest stage o f dementia. 'Increased muscular tension' indicates an unspecific increase of muscle tone. The 'Kli~ver-Bucy syndrome' here means a combination of severe amnesia, hyperorality, bulemia and in
324 TABLE II Rating scale for diagnosis of Alzheimer's disease Clinical feature
Score
Slow progression Early loss of insight Early amnesia for remote events Early spatial disorientation Apraxia, aphasia, agnosia Logoclonia Logorrhea Progressive reduction of spontaneity of speech Epileptic seizure of late onset Increased muscular tension Myoclonic twitchings Kli~ver-Bucy syndrome
1 1 2 2 2 2 1 1 1 2 1 1
(Gustafson and Nilsson, 1982)
some cases also hypersexuality. The different scores of the item were established by item analysis. The interrater reliability of the AD scale was calculated to 0.80 (Gustafson and Nilsson, 1982). Dementia caused by cortical degeneration mainly affecting the frontal and temporal lobes was recognized by the items of Table III. Pick's disease belongs to this group of dementias. The item of 'confabulation' means a false report, which is presented spontaneously, without self-criticism. A combination of echolalia, mutism and amimia is considered pathognomonic of Pick's disease (Guiraud, 1956). The interrater reliability of the DFT scale was 0.50 (Gustafson and Nilsson, 1982). The validity of the rating scale for diagnosis of AD, DFT and MID has been tested by comparison with post-mortem diagnosis. The optimal cut-off points for
TABLE II1 Rating scale for diagnosis of dementia with fronto-temporal degeneration (DFT) Clinical feature
Score
Slow progression Early loss of insight Early signs of disinhibition Irritability, dysphoria Confabulation Logorrhea Progressive reduction of spontaneity of speech Echolalia, mutism, amimia Kli~ver-Bucy syndrome
1 2 2 1 1 1 1 2 1
(Gustafson and Nilsson, 1982)
325 respective scales have been established. An AD score greater than 5 and especially exceeding 8 points, depending on the duration of the dementia, favours an Alzheimer diagnosis. A D F T score above 5 points in combination with a moderately high AD score suggests a D F T diagnosis. In the present study a cross-validation is made to test the efficiency of the previously established cut-off points.
Results
Psychometry The results of the test examination can be grouped in the following six categories of mental functioning: verbal ability, reasoning, verbal memory, spatial memory, intellectual speed and motoric speed. The quantification is made in the ordinary stanine scale. Profile over the categories for each diagnostic group is presented. The early Alzheimer patients (Fig. 1) show an intact verbal ability, a simultaneous debut of defect reasoning ability and defect verbal and spatial memory together with some slowing-down in speed both with regard to the motoric and intellectual modalities. For those familiar with clinical testing, the cognitive pattern of the Alzheimer patient seems to be a good illustration of .the old verbal-performance discrepancy of Wechsler, which was regarded as indicative of cerebral defects (Wechsler, 1958).
STANINE 9
8
7
4
3 2 1 VERBAL ABILITY
REASONING ABILITY
VERBAL MEMORY
SPATIAL MEMORY
INTELLECT. SPEED
Fig. 1. The early psychometricsigns in patients with AIzheimertype dementia.
MOTORIC SPEED
326 STANINE 9
8
7
6
5
3 2 I VERBAL ABILITY
REASONING ABILITY
VERBAL MEMORY
SPATIAL MEMORY
MOTORIC SPEED
INTELLECT. SPEED
Fig. 2. Theearly p s y c h o m e t r i c s i g n s i n patients w i t h f r o n t o - t e m p o r a l d e g e n e r a t i o n ( D F T ) .
STANINE 9
8
7
VTA
5
v/,4
4
3 2 1
VERBAL ABILITY
REASONING ABILITY
VERBAL MEMORY
SPATIAL MEMORY
Fig. 3. Psychometric test profile in a case of cerebrovascular dementia.
INTELLECT. SPEED
MOTORIC SPEED
327
The more rare patients with non-Alzheimer fronto-temporal degeneration (Fig. 2) (DFT) show in these'same parameters a somewhat different, almost contrary pattern of deficiency. Here the verbal production is slow, reasoning and memory almost intact, but intellectual and motoric speeds slow down. In patients with cerebrovascular dementia, also called multi-infarct dementia (MID), the test pattern can show a considerable variation due to acuteness and localization of cerebral lesions (Fig. 3). Here is shown one of the most frequent patterns in this group. It is not unusual with an isolated verbal memory disturbance in cerebrovascular dementia, but, of course, also other combinations are found. In our material patients with major neurological deficits were excluded. For comparison, here the reduced cognition is shown in a group of depressed patients (Fig. 4). Prominent is the memory reduction as well as the reduced psychomotor speed. If we compare these patterns, the differences seem to be small but rather consistent. In addition, it should be emphasised that the clinical investigator also tries to establish the mechanism that brings about the deficient performance. The test profile in early Alzheimer disease seems to represent a rather pure cognitive disability. In D F T patients the observed cognitive reduction could be due to a lack of cognition, but may also be determined by defects in the cognative and emotional spheres as well as by motivational factors. Therefore, also behaviour observations
STANINE 9
8
7
6
s
;
I
4
3 2 1 VERBAL ABILITY
REASONING ABILITY
VERBAL MEMORY
SPATIAL MEMORY
INTELLECT. SPEED
Fig, 4. Psychometric test profile in patients with depressive pseudodementia.
MOTORIC SPEED
328 T A B L E IV Qualities of p e r f o r m a n c e with relevance for differential diagnosis of d e m e n t i a l. 2. 3. 4. 5. 6.
Anxiety Self-criticism N u m b e r of trials Strategy Rotations Perseverations
should be decisive for diagnosis. Finally, the depressive cognition, which sometimes mimics dementia, is distinguished by its lack of the specific qualities that can be observed in organic dementia. This brings us to the second important diagnostic variable in testing - - ' the quality of performance'.
Quality of performance Some of the relevant performance qualities in testing are shown in Table IV. It is our experience that the quantitative analysis does not give enough information when differing depressives from dementias. Therefore, a qualitative analysis of performance should be added. These qualities occur in varying frequencies in the dementias and the depressive pseudodementia (Table V). For instance, perseveration can readily be seen in the DFT group, while rotation is more frequent in the Alzheimer group and sometimes also in MID. None of these qualities are found in the depressive group. Another example, lack of systematic solution and lack of self-criticism are more frequently found in the DFT or Pick dementia group (Hagberg, 1983). In a wider personality context, differences in the coping strategies and adjustment behaviour could be found to vary. Perhaps not so much with type of dementia as with degree of dementia. And this, of course, has implications, most of all for the handling of the patient in the future.
TABLE V Differences in quality of p e r f o r m a n c e in A D , DFT, M I D and in depressive p s e u d o d e m e n t i a Test b e h a v i o u r
AD
DFT
MID
Depression
Systematic solutions Rotations Perseverations Self-criticism Anxiety
+ + + +
+ -
(+ ) + + (+ ) +
+ + (+ )
329
Personality The change in personality in the dementias can readily be understood within an ego-psychological frame of reference. Obviously the dementia affects all layers of personality but foremost the structure of the ego and its adaptive capacity (Hagberg, 1978). This adaption is supposed to take place on different qualitative levels, i.e. neurotic, primitive, regressive or disorganising, depending upon the kind and degree of dementia. Therefore it is essential to test and evaluate these personality variables, in order to plan and supervise the forthcoming treatment, habilitation and rehabilitation of the patients. It is also quite clear that the adaptive capacity of the ego function is a most vital element in the handling of the disease process by the patient. The last statement is illustrated with the following schematic representation (Fig. 5). The first three psychiatric factors describe neurotic symptoms and the last three more primitive and less adaptive forms of behaviour. In the left column we have kind and degree of cognitive reduction: isolated memory disturbances (C2), defect reasoning (C3), acalculia and anomia (C4) and finally aphasia, agnosia and apraxia
Psychiatric facton
Cogn. red.
groupa
Ixophrenia
I
Hypochondriahysterold traits
Depreuionanxiety
Paranoia
Afl'ectlve labillty
1%cI~motor ova-
Explmlvlty- activity-euphoria rmdemnes
Fig. 5. Psychiatric factors, kind and degree of cognitive reduction and functional level of the dominant hemisphere as measured by regional cerebral blood flow (rCBF). Regional differencesare indicated by circles: Sign/non sign, h i g h e r @ / @ ; sign/non sign, lower @ / @ . Mean hemispheric differences are indicated: Higher, []; lower, []. (Gustafson, Hagberg, 1975).
330
(C5). In the center we have the regional distribution in cerebral functional level of the left hemisphere. Neurotic reactions are not found in patients with the most advanced cognitive deterioration (C4-C5). It could also be observed that these patients have an intact frontal functional level. On the contrary, primitive less adaptive reactions are found also in C4 and C5, where also the frontal lobe dysfunction is more marked. The inference being: the more severe the dementia, the lower the capacity for adaptive reactions. And, in addition to that statement, the worse the frontal lobe functions, the more of primitive, maladaptive behaviour is found.
Cfinical rating scales The problem of differentiation can also be handled using rating scales mapping the clinical features. In order to make this differentiation between the dementia types, the three different rating scales have been used in combination. The 24 post-mortem verified presenile A D cases had a mean A D score of 10 + 2.7, a D F T score of 3.7 _+ 2.6 and an IS of 2.8 _+ 2.3. In eight cases of senile dementia of the Alzheimer type, the mean AD score was 7.5 _+ 3, the D F T score 2.6 + 1.7 and the IS 3.6 _+ 1.9, respectively. By contrast, 11 verified D F T cases had a mean D F T score of 8 + 2, an AD score of 4 + 1.5 and an IS of I + 1.5. In 15 cases with vascular dementia due to multiple infarcts the IS was 12 + 2, the AD score 2 _+ 2 and the D F T score 1.5 _+ 1. All combinations of scores are well above and below the predicted cut-off point of the scale for respective diagnostic group. It is essential to emphasise that the diagnosis of a specific type of organic dementia should be based on positive identification of its clinical features. The best differentiation between the three types of dementia should thus be based on all three scales. For a clear diagnostic statement, however, the patient has to have a score above cutting-point on one scale and below on the other scales. The clinical relevance of the rating scales is further supported by a good correspondence between the clinical diagnosis and rCBF findings (Gustafson et al., 1984).
Conclusions The results seem to suggest first of all that it is possible to use clinical tools such as psychometric tests and clinical rating scales to identify dementia in a fairly early stage. Also, that dementia can be separated from pseudodementia-type depression. Secondly, that each type of dementia seems to have its own characteristics in the psychometric performance, in the qualitative behaviour and clinical ratings, and that these clinical classifications are in good agreement with the later on established post-mortem diagnosis. Our next step will be to optimize the clinical prediction by simultaneous use of the two fields of measurement. Furthermore, analysis of the clinical progression of the disease in the same parameters from the first examination to the last would presumably add further developmental characteristics to the various types of dementia.
331
Acknowledgements The present study was supported by the Swedish Medical Research Council (Grants No. 21X-03950 and 21P-5144) and The King Gustaf V and Queen Victoria's Foundation.
References Benton, A.L. (1963): The Revised Visual Retention Test. Clinical and Experimental Applications. State University of Iowa City, The Psychological Corporation, New York. Brun, A. and Gustafson, L. (1976): Distribution of cerebral degeneration in Alzheimer's disease. A clinico-pathological study. Arch. Psychiat. Nervenkr., 223, 15-33. Brun, A. and Gustafson, L. (1978): Limbic lobe involvement in presenile dementia. Arch. Psychiat. Nervenkr., 226, 79-93. Cronholm, B. and Molander, L. (1954): Memory disturbances after electroconvulsive therapy. Conditions 6 hours after electroshock treatment. Acta Psychiat. Neurol. Scand., 32, 280-306. Eisenson, J. (1954): Manual for the Examination of Aphasic and Related Disturbances. The Psychological Corporation, New York. Graham, F.K. and Kendall, B.S. (1960): Memory for Designs; revised general manual. Perceptual and Motor Skills, 11, 147-188. Guiraud, P. (1956): Psychiatrie Clinique. Le Francois, Paris, pp. 533-550. Gustafson, L. and Hagberg, B. (1975): Emotional behaviour, personality changes and cognitive reduction in presenile dementia: related to regional cerebral blood flow. Acta Psychiat. Scand., Suppl. 257, 39-67. Gustafson, L. and Nilsson, L. (1982): Differential diagnosis of presenile dementia on clinical grounds. Acta Psychiat. Scand., 65, 194-209. Gustafson, L. and Risberg, J. (1974): Regional cerebral blood flow related to psychiatric symptoms in dementia with onset in the presenile period. Acta Psychiat. Scand., 50, 516-538. Gustafson, L. and Risberg, J. (1979): Regional cerebral blood flow measurements by the 133Xe inhalation technique in differential diagnosis of dementia. Acta Neurol, Scand., 60, Suppl. 72, 546-547. Gustafson, L., Brun, A. and Ingvar, D.H. (1977): Presenile dementia: clinical symptoms, pathoanatomical findings and cerebral blood flow. In: Cerebral Vascular Disease, pp. 5-9. Editors: J.S. Meyer, H. Lechner and M. Reivich. Excerpta Medica, Amsterdam. Gustafson, L., Hagberg, B. and Ingvar, D.H. (1978): Speech disturbances in presenile dementia related to local cerebral blood flow abnormalities in the dominant hemisphere. Brain Lang., 5, 103-118. Gustafson, L., Risberg, J. and Silfverskii31d, P. (1981): Regional cerebral blood flow in organic dementia and affective disorders. In: Advances in Biological Psychiatry, Vol. 6, 109-116. Editors: J. Mendlewicz and H.M. van Praag. Karger, Basel. Gustafson, L., Risberg, J., Johanson, M. and Brun, A. (1984): Evaluation of organic dementia by regional cerebral blood flow measurements and clinical and psychometric methods. In: Monographs in Neural Sciences, Vol. 11, pp. 111-117. Editor: M.M. Cohen. Karger, Basel. Hachinski, V.C., iliff, L.D., Zihlka, E., et al. (1975): Cerebral blood flow in dementia. Arch. Neurol., 32, 632-637. Hagberg, B. (1978): Cognitive Impairment and Personality Change in Relation to Brain Dysfunction. Studia Psychol. et Paedag. Series Altera XXXXIV, Gleerup, Lund. Hagberg, B. (1983): Cognitive impairment and test taking attitudes in organic dementia. A comparison between diffuse pre- and postcentral lesions. Psychol. Res. Bull., 23, 2. Hagberg, B. and Ingvar, D.H. (1976): Cognitive reduction in presenile dementia related to regional abnormalities of the cerebral blood flow. Br. J. Psychiat., 128, 209-222. Harrison, M.J.G., Thomas, D.J., du Boulay, G.H. and Marshall, J. (1979): Multi-infarct dementia. J. Neurol. Sci., 40, 97-103.
332 Inglis, J. (1958): Psychological investigations of cognitive deficit in elderly psychiatric patients. Psychol. Bull., 55, 197-214. Ingvar, D.H. and Gustafson, L. (1970): Regional cerebral blood flow in organic dementia with early onset. Acta Neurol. Scand., 46, Suppl. 43, 42-73. Johannesson, G., Brun, A., Gustafson, L. and Ingvar, D.H. (1977): EEG in presenile dementia related to cerebral blood flow and autopsy findings. Acta Neurol. Scand., 56, 89-103. Johannesson, G., Hagberg, B., Gustafson, L. and lngvar, D.H. (1979): EEG and cognitive impairment in presenile dementia. Acta Neurol. Scand., 59, 225-240. Rosen, W.G., Terry, R.D., Fuld, P.A. Katzman, R. and Peck, A. (1980): Pathological verification of ischemic score in differentiation of dementias. Ann. Neurol., 7, 486-488. Wechsler, D. (1958): The Measurement and Appraisal of Adult Intelligence. Baltimore.
321
Elsevier
A G G 00126
On diagnosis of dementia: psychometric investigation and clinical psychiatric evaluation in relation to verified diagnosis * Bo H a g b e r g a n d L a r s G u s t a f s o n Departments of Psychiatry 1 and Psychogeriatrics, University Hospital, S-221 85 Lund, Sweden (Received 20 March 1985; accepted 17 September 1985)
Summary Patients with early as well as manifest dementia were studied in a longitudinal way with psychometric and psychiatric methods. Tests such as vocabulary, attention test, block design test, verbal and spatial memory tests, reaction time test and examination for aphasia were used as well as a qualitative analysis of behaviour and personality, The differential diagnosis of Alzheimer's disease. Pick's disease and other dementias with fronto-temporal degeneration and multi-infarct dementia was based on diagnostic rating scales. Differences in cognitive profiles, qualities of behaviour and scores on combinations of psychiatric rating scales were identified for pathoanatomically verified cases with Alzheimer's disease, fronto-temporal degeneration and multi-infarct dementia. psychometry; rating scales; dementia, pseudodementia; personality
Introduction In 1966, a longitudinal study was started at the University Hospital, Lund, Sweden, in order to study the debut and progression of the various dementias from a multi-disciplinary point of view. Psychiatric, psychological and neuropsychological as well as pathoanatomical variables were studied with the purpose to identify early differential signs of dementia and their relationship to progression and post-mortem pathoanatomical diagnosis. This presentation will deal with the predictive capacity of the clinical measures obtained during the patient's disease as validated against post-mortem pathoanatomical diagnosis. Results from the study have been presented successively (Ingvar and Gustafson, 1970; Gustafson and Risberg, 1974, 1979; Gustafson and Hagberg, 1975; Hagberg and Ingvar, 1976; Gustafson et al., 1976, 1981; Brun and Gustafson, 1976, 1978; Johannesson et al., 1977, 1979). * Paper presented at the International Workshop on 'Psychometry in Aging and Dementia', Montecatini Terme, June 25th, 1984, Italy.
0167-4943/85/$03.30 © 1985 Elsevier Science Publishers B.V. (Biomedical Division)
322
Material Patients with incipient, early as well as with manifest dementia were included in the longitudinal study. Patients with chronic psychosis, oligophrenia, addiction, pre-existing epilepsy, severe somatic disorders or apoplexy with gross neurological deficits were excluded. Patients with symptoms indicating a confusional reaction or a non-organic psychiatric illness were treated before final decision of inclusion into the material. All cases went through psychiatric, psychometric, neurological (including radiological and EEG) investigations and the regional cerebral blood flow (rCBF) was measured. The patients have been followed up regularly at least once a year. Autopsy including neuropathological investigation has been performed in 74% of deceased cases. The criteria for the neuropathological diagnostic classification have been presented by Dr. A. Brun earlier at this symposium. The psychometric and psychiatric diagnostic procedure was tested in 58 postmortem verified cases. Eight of these had a senile dementia of the Alzheimer type (SDAT) and a mean age of 79 years, 24 had a presenile Alzheimer disease (AD) and a mean age of 68 years, 11 had a dementia with a fronto-temporal accentuation of the cortical degeneration (DFT) and a mean age of 62 years and 15 patients had a vascular dementia due to multiple infarcts (MID) and a mean age of 71 years.
Methods
Psychometric tests The psychometric battery that we use contains verbal tests, memory tests, a test on logical-inductive reasoning and tests on intellectual and motoric speeds. When indicated, examination for aphasia according to Eisenson's model was used. See also Gustafson et al. (1978). Sometimes also a personality test was included.
TABLE I The standard psychometrictest battery Psychologicaltests Vocabulary Synonyms Attention Test Kohs' Block Design Memory Tests: Paired Associates Object Recognition Memory for Geometrical Designs Memory for Designs(Graham-Kendall) Visual Retention (Benton) Serial Colour Word Test Visual Reaction Time (Simple and Choice) Aphasia Examination(Eisenson)
323 The standard p s y c h o m e t r i c test battery (Table I) Test specification - - Vocabulary, synonyms,
verbal tests measuring degree ot verbal understanding
(Inglis, 1958). Attention test, measuring the patient's ability to follow simple instructions and perform accordingly. - - K o h s ' B l o c k Design, spatial performance test measuring spatial ability as well as logical-inductive reasoning (Wechsler, 1958). - - Paired associates, measuring immediate recall of verbal material (Cronholm and Molander, 1954). - - Object recognition, measuring in a multiple-choice fashion the immediate recognition of concrete objects (Cronholm and Molander, 1954). - - M e m o r y f o r geometrical designs, measuring immediate recall of abstract figures. 'Abstract' in the meaning that the figures are hard to verbalize. - - M e m o r y for designs, measuring spatial perceptual ability as well as immediate memory for simple designs (Graham and Kendall, 1960). Visual retention test, a spatial memory test (Benton, 1963). Colour word test, considered measuring concentration, attention and intellectual speed in solving a task containing incongruent material of the Stroop character. - - Visual reaction time (simple and choice). - - E x a m i n a t i o n f o r aphasia (Eisenson, 1954), evaluating degree of aphasia, apraxia, agnosia, alexia, acalculia, agraphia etc. -
-
-
-
-
-
Diagnostic rating scales
The clinical differentiation was focused upon a recognition of AD, MID and D F T including Pick's disease. The clinical diagnoses were based on three diagnostic schedules containing a limited number of clinical features, which usually were noted in the case reports. The diagnosis of MID was based on the ischemic score (IS) developed by Hachinski et al. (1975). This rating scale is further discussed by Dr. Wagner at this symposium. The validity of IS has been shown in several studies. A score exceeding 7 or 8 points strongly suggests a vascular etiology of the dementia (Harrison et al., 1979; Rosen et al., 1980; Gustafson and Nilsson~ 1982). Dementias of the Alzheimer type were identified using the clinical features and scores presented in Table II. The differentiation between AD and SDAT was based on age of onset, before or after 65 years of age, respectively. The items of the rating scale for diagnosis of AD might need some clarification. 'Early' means that a symptom appears at an early or incipient stage of the disease. 'Logoclonia' is a stuttering-like phenomenon mainly affecting the terminal syllable of words. 'Epileptic seizures' are considered of late onset, when starting after several years' course of the disease, at a manifest stage o f dementia. 'Increased muscular tension' indicates an unspecific increase of muscle tone. The 'Kli~ver-Bucy syndrome' here means a combination of severe amnesia, hyperorality, bulemia and in
324 TABLE II Rating scale for diagnosis of Alzheimer's disease Clinical feature
Score
Slow progression Early loss of insight Early amnesia for remote events Early spatial disorientation Apraxia, aphasia, agnosia Logoclonia Logorrhea Progressive reduction of spontaneity of speech Epileptic seizure of late onset Increased muscular tension Myoclonic twitchings Kli~ver-Bucy syndrome
1 1 2 2 2 2 1 1 1 2 1 1
(Gustafson and Nilsson, 1982)
some cases also hypersexuality. The different scores of the item were established by item analysis. The interrater reliability of the AD scale was calculated to 0.80 (Gustafson and Nilsson, 1982). Dementia caused by cortical degeneration mainly affecting the frontal and temporal lobes was recognized by the items of Table III. Pick's disease belongs to this group of dementias. The item of 'confabulation' means a false report, which is presented spontaneously, without self-criticism. A combination of echolalia, mutism and amimia is considered pathognomonic of Pick's disease (Guiraud, 1956). The interrater reliability of the DFT scale was 0.50 (Gustafson and Nilsson, 1982). The validity of the rating scale for diagnosis of AD, DFT and MID has been tested by comparison with post-mortem diagnosis. The optimal cut-off points for
TABLE II1 Rating scale for diagnosis of dementia with fronto-temporal degeneration (DFT) Clinical feature
Score
Slow progression Early loss of insight Early signs of disinhibition Irritability, dysphoria Confabulation Logorrhea Progressive reduction of spontaneity of speech Echolalia, mutism, amimia Kli~ver-Bucy syndrome
1 2 2 1 1 1 1 2 1
(Gustafson and Nilsson, 1982)
325 respective scales have been established. An AD score greater than 5 and especially exceeding 8 points, depending on the duration of the dementia, favours an Alzheimer diagnosis. A D F T score above 5 points in combination with a moderately high AD score suggests a D F T diagnosis. In the present study a cross-validation is made to test the efficiency of the previously established cut-off points.
Results
Psychometry The results of the test examination can be grouped in the following six categories of mental functioning: verbal ability, reasoning, verbal memory, spatial memory, intellectual speed and motoric speed. The quantification is made in the ordinary stanine scale. Profile over the categories for each diagnostic group is presented. The early Alzheimer patients (Fig. 1) show an intact verbal ability, a simultaneous debut of defect reasoning ability and defect verbal and spatial memory together with some slowing-down in speed both with regard to the motoric and intellectual modalities. For those familiar with clinical testing, the cognitive pattern of the Alzheimer patient seems to be a good illustration of .the old verbal-performance discrepancy of Wechsler, which was regarded as indicative of cerebral defects (Wechsler, 1958).
STANINE 9
8
7
4
3 2 1 VERBAL ABILITY
REASONING ABILITY
VERBAL MEMORY
SPATIAL MEMORY
INTELLECT. SPEED
Fig. 1. The early psychometricsigns in patients with AIzheimertype dementia.
MOTORIC SPEED
326 STANINE 9
8
7
6
5
3 2 I VERBAL ABILITY
REASONING ABILITY
VERBAL MEMORY
SPATIAL MEMORY
MOTORIC SPEED
INTELLECT. SPEED
Fig. 2. Theearly p s y c h o m e t r i c s i g n s i n patients w i t h f r o n t o - t e m p o r a l d e g e n e r a t i o n ( D F T ) .
STANINE 9
8
7
VTA
5
v/,4
4
3 2 1
VERBAL ABILITY
REASONING ABILITY
VERBAL MEMORY
SPATIAL MEMORY
Fig. 3. Psychometric test profile in a case of cerebrovascular dementia.
INTELLECT. SPEED
MOTORIC SPEED
327
The more rare patients with non-Alzheimer fronto-temporal degeneration (Fig. 2) (DFT) show in these'same parameters a somewhat different, almost contrary pattern of deficiency. Here the verbal production is slow, reasoning and memory almost intact, but intellectual and motoric speeds slow down. In patients with cerebrovascular dementia, also called multi-infarct dementia (MID), the test pattern can show a considerable variation due to acuteness and localization of cerebral lesions (Fig. 3). Here is shown one of the most frequent patterns in this group. It is not unusual with an isolated verbal memory disturbance in cerebrovascular dementia, but, of course, also other combinations are found. In our material patients with major neurological deficits were excluded. For comparison, here the reduced cognition is shown in a group of depressed patients (Fig. 4). Prominent is the memory reduction as well as the reduced psychomotor speed. If we compare these patterns, the differences seem to be small but rather consistent. In addition, it should be emphasised that the clinical investigator also tries to establish the mechanism that brings about the deficient performance. The test profile in early Alzheimer disease seems to represent a rather pure cognitive disability. In D F T patients the observed cognitive reduction could be due to a lack of cognition, but may also be determined by defects in the cognative and emotional spheres as well as by motivational factors. Therefore, also behaviour observations
STANINE 9
8
7
6
s
;
I
4
3 2 1 VERBAL ABILITY
REASONING ABILITY
VERBAL MEMORY
SPATIAL MEMORY
INTELLECT. SPEED
Fig, 4. Psychometric test profile in patients with depressive pseudodementia.
MOTORIC SPEED
328 T A B L E IV Qualities of p e r f o r m a n c e with relevance for differential diagnosis of d e m e n t i a l. 2. 3. 4. 5. 6.
Anxiety Self-criticism N u m b e r of trials Strategy Rotations Perseverations
should be decisive for diagnosis. Finally, the depressive cognition, which sometimes mimics dementia, is distinguished by its lack of the specific qualities that can be observed in organic dementia. This brings us to the second important diagnostic variable in testing - - ' the quality of performance'.
Quality of performance Some of the relevant performance qualities in testing are shown in Table IV. It is our experience that the quantitative analysis does not give enough information when differing depressives from dementias. Therefore, a qualitative analysis of performance should be added. These qualities occur in varying frequencies in the dementias and the depressive pseudodementia (Table V). For instance, perseveration can readily be seen in the DFT group, while rotation is more frequent in the Alzheimer group and sometimes also in MID. None of these qualities are found in the depressive group. Another example, lack of systematic solution and lack of self-criticism are more frequently found in the DFT or Pick dementia group (Hagberg, 1983). In a wider personality context, differences in the coping strategies and adjustment behaviour could be found to vary. Perhaps not so much with type of dementia as with degree of dementia. And this, of course, has implications, most of all for the handling of the patient in the future.
TABLE V Differences in quality of p e r f o r m a n c e in A D , DFT, M I D and in depressive p s e u d o d e m e n t i a Test b e h a v i o u r
AD
DFT
MID
Depression
Systematic solutions Rotations Perseverations Self-criticism Anxiety
+ + + +
+ -
(+ ) + + (+ ) +
+ + (+ )
329
Personality The change in personality in the dementias can readily be understood within an ego-psychological frame of reference. Obviously the dementia affects all layers of personality but foremost the structure of the ego and its adaptive capacity (Hagberg, 1978). This adaption is supposed to take place on different qualitative levels, i.e. neurotic, primitive, regressive or disorganising, depending upon the kind and degree of dementia. Therefore it is essential to test and evaluate these personality variables, in order to plan and supervise the forthcoming treatment, habilitation and rehabilitation of the patients. It is also quite clear that the adaptive capacity of the ego function is a most vital element in the handling of the disease process by the patient. The last statement is illustrated with the following schematic representation (Fig. 5). The first three psychiatric factors describe neurotic symptoms and the last three more primitive and less adaptive forms of behaviour. In the left column we have kind and degree of cognitive reduction: isolated memory disturbances (C2), defect reasoning (C3), acalculia and anomia (C4) and finally aphasia, agnosia and apraxia
Psychiatric facton
Cogn. red.
groupa
Ixophrenia
I
Hypochondriahysterold traits
Depreuionanxiety
Paranoia
Afl'ectlve labillty
1%cI~motor ova-
Explmlvlty- activity-euphoria rmdemnes
Fig. 5. Psychiatric factors, kind and degree of cognitive reduction and functional level of the dominant hemisphere as measured by regional cerebral blood flow (rCBF). Regional differencesare indicated by circles: Sign/non sign, h i g h e r @ / @ ; sign/non sign, lower @ / @ . Mean hemispheric differences are indicated: Higher, []; lower, []. (Gustafson, Hagberg, 1975).
330
(C5). In the center we have the regional distribution in cerebral functional level of the left hemisphere. Neurotic reactions are not found in patients with the most advanced cognitive deterioration (C4-C5). It could also be observed that these patients have an intact frontal functional level. On the contrary, primitive less adaptive reactions are found also in C4 and C5, where also the frontal lobe dysfunction is more marked. The inference being: the more severe the dementia, the lower the capacity for adaptive reactions. And, in addition to that statement, the worse the frontal lobe functions, the more of primitive, maladaptive behaviour is found.
Cfinical rating scales The problem of differentiation can also be handled using rating scales mapping the clinical features. In order to make this differentiation between the dementia types, the three different rating scales have been used in combination. The 24 post-mortem verified presenile A D cases had a mean A D score of 10 + 2.7, a D F T score of 3.7 _+ 2.6 and an IS of 2.8 _+ 2.3. In eight cases of senile dementia of the Alzheimer type, the mean AD score was 7.5 _+ 3, the D F T score 2.6 + 1.7 and the IS 3.6 _+ 1.9, respectively. By contrast, 11 verified D F T cases had a mean D F T score of 8 + 2, an AD score of 4 + 1.5 and an IS of I + 1.5. In 15 cases with vascular dementia due to multiple infarcts the IS was 12 + 2, the AD score 2 _+ 2 and the D F T score 1.5 _+ 1. All combinations of scores are well above and below the predicted cut-off point of the scale for respective diagnostic group. It is essential to emphasise that the diagnosis of a specific type of organic dementia should be based on positive identification of its clinical features. The best differentiation between the three types of dementia should thus be based on all three scales. For a clear diagnostic statement, however, the patient has to have a score above cutting-point on one scale and below on the other scales. The clinical relevance of the rating scales is further supported by a good correspondence between the clinical diagnosis and rCBF findings (Gustafson et al., 1984).
Conclusions The results seem to suggest first of all that it is possible to use clinical tools such as psychometric tests and clinical rating scales to identify dementia in a fairly early stage. Also, that dementia can be separated from pseudodementia-type depression. Secondly, that each type of dementia seems to have its own characteristics in the psychometric performance, in the qualitative behaviour and clinical ratings, and that these clinical classifications are in good agreement with the later on established post-mortem diagnosis. Our next step will be to optimize the clinical prediction by simultaneous use of the two fields of measurement. Furthermore, analysis of the clinical progression of the disease in the same parameters from the first examination to the last would presumably add further developmental characteristics to the various types of dementia.
331
Acknowledgements The present study was supported by the Swedish Medical Research Council (Grants No. 21X-03950 and 21P-5144) and The King Gustaf V and Queen Victoria's Foundation.
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