Acta psychiat. scand. (1975) 51, 210-217 The York Clinic (Head: 1.J. Fleminger, M.D.)Guy’s Hospital, London, England
PRESENILE DEMENTIA: THE DIFFICULTIES OF EARLY DIAGNOSIS P.N. Nom
AND J. J. FLEMINGER
A follow-up of 35 patients first diagnosed as having presenile dementia at York Clinic is described. In only 15 cases did progressive deterioration confirm the diagnosis. A retrospective case note study of all the patients is described comparing the clinical features and results of special investigations of those patients in whom the diagnosis was confirmed in this way and those in whom it was not. The results of this study are used to illuminate some of the special difficulties in the early diagnosis of presenile dementia.
Key words. Presenile dementia
- diagnosis - clinical features -
A.E.G. - follow-up.
The early diagnosis of dementia in middle age can be a difficult problem. When this diagnosis is suspected the first priority is to try and identify an underlying cause. This is most important because many specific causes of dementia are now treatable; but also because, even if the specific cause is not treatable, its identification clarifies the prognosis and further management of the patient. In a recent retrospective case study (Marsden & Harrison (1972)), a specific cause was found in 36 of 84 patients diagnosed as having dementia in the presenium, who had been referred to the National Hospital for Nervous Diseases. This, however, still left 48 patients (57%) in whom no specific cause could be identified. In our experience it is in these cases that the early diagnosis of primary presenile dementia is particularly difficult to substantiate and at present of doubtful significance in individual cases. The present study concerns a group of patients diagnosed as having dementia in middle age but in whom no specific cause could be found. There have been many excellent retrospective studies in recent years of the primary presenile dementias. These have attempted to isolate syndromes, find hereditary factors, and relate clinical features to other findings such as biopsy or post-mortem material, ERG. changes and premorbid personality. Above all there has been a major attempt to isolate specific clinicopathological entities; to differentiate clinically between Alzheimer’s disease and Pick’s disease, and between Alzheimer’s disease and the other presenile dementias in the group (e. g. Stengel (1943), Sjogren et 41. (1953), Sim et al. (1966)). Despite these attempts it can still be very difficult, if not impossible, to make these distinctions clinically. Indeed, in practice, this remains a relatively unimportant exercise com-
211
pared with the crucial problem of differentiating between early primary dementia of any kind and disorders of a non-dementing nature, such as affective psychosis. The present study was carried out to examine the validity of the diagnosis of primary presenile dementia as made in the psychiatric unit of a general hospital. We were also interested in studying the clinical features found in these patients to see if any individual items were particularly helpful in predicting outcome. MATERIAL AND METHODS The case notes of all inpatients diagnosed as having dementia in the York Clinic, Guy’s Hospital, 1950 to 1969, were reviewed. All those fulfilling the following criteria were retained for further study: (1) an unequivocal diagnosis of dementia was made for the first time during the admission; (2) the patient was not over the age of 65 at the time of diagnosis; (3) no known cause of cerebral disease had been identified during the admission. Thus, all cases with a history or other evidence of cerebrovascular disease, hypertension, alcoholism, drug dependence, myxoedema, encephalitis, recent or serious head injury, neoplasm, or any other such types or recognised causes of cerebral disorder were excluded from the study. Between the years 1950 to 1969 there had been 50 cases which satisfied the criteria. This represents 0.7% of all admissions. Abstracts were made of the history, clinical features at the time of diagnosis and the results of special investigations in these cases. Following this an attempt was made to trace these patients. Information was obtained from G.P.s, subsequent hospital admission, 0 . P notes, coroners’ reports and post-mortem reports. In this way adequate follow-up data were obtained for 35 of the 50 cases. These 35 cases form the basis of this report.
RESULTS Follow-up
Examination of the case notes of the 15 cases which could not be followed up did not reveal any specific differences which would make us think that they had differed in any special way from the 35 cases studied. Of these 35 patients, only 15 (43%) had deteriorated in the expected manner following the initial diagnosis of presenile dementia. Eighteen cases had actually improved and two cases had remained essentially unchanged. The cases therefore fell into two main groups: The 15 cases in which follow-up confirmed the diagnosis (Deteriorated or D group) and the 20 (57%) cases who did not deteriorate as expected and in whom the diagnosis was therefore not confirmed won-D group). Because our main interest rests in this, the rest of the results will be presented as a comparison between these two groups. The 15 cases in the “deteriorated @)” group consisted of seven men and eight women. The mean age at time of diagnosis was 55 years (range 35-64). At the time of follow-up ten of these patients had died, having survived on average 6 years (range 1-11 years) following diagnosis. The five remaining patients had a mean length of follow-up of 5 years (range 1-15 years). As expect-
212
ed the main cause of death for these cases was intercurrent infection, especially respiratory infection (six cases). The 20 cases in the “non-deteriorated won-D)” group consisted of 11 men and nine women. Mean age at time of diagnosis was 52 years (range 37-59). At the time of follow-up two of the three patients who had died had committed suicide. The mean length of follow-up of those surviving was 10 years (range 5-23 years). Of these 20 Non-D cases only five had remained well throughout the follow-up period. The others had persistent or recurrent signs and symptoms which were classified as follows: depression (3), anxiety state (3), somatic symptoms without organic basis (6), hysterical reaction (l), paranoid reaction associated with respiratory failure (1). One patient developed hemiplegia 20 years after the original diagnosis of presenile dementia. The overall impression was that this group consisted mainly of people with marked personality difficulties and neurotic symptoms or affective disorder. No case developed a persistant psychotic illness or showed evidence on follow-up of an organic illness which might have been present at the time of the original diagnosis and missed. Clinical features
Using the retrospective case note study, the clinical features that were recorded at the time of diagnosis of the two main groups D and Non-D were compared. Reason for referral. The source of referral was the family doctor in the majority of cases in both groups. Referrals from other hospital departments numbered seven of 20 Non-D and four of 15 D patients. The main reason for referral was for the investigation or treatment of depression (8 Non-D, 6 D). All other cases are remarkable only for the variety of reasons in both groups, e. g. shoplifting (1 Non-D), fur fetish (1 D), bizarre hypochondriasis (1 Non-D), persistent pain (1 Non-D); and perhaps remarkable for the fact that the possibility of dementia was only suggested in one case (D, referred by G.P.). Signs and symptoms at time of diagnosis. (1) Memory disturbance. All the patients were thought to have some degree of memory disturbance on examination. There were some in both groups who gave no history of memory difficulties but these were thought to show evidence of memory disorder on testing. Eleven D patients (73%) had a definite history of memory difficulties and this was a prominent feature in the history in seven cases (50%).This does not include one case in this group who had a history of two hysterical fugues. Five in this group had a history of disorientation and four of these were disorientated on examination. Confabulation was recorded in three cases. Ten (50%) in the Non-D group had a definite history of memory disturbance but in only three of these (15%) was it a prominent feature in the history. Two had a history of disorientation but only one was disorientated on examination. There was no cases of confabulation. In the D group six of the 11 (55%), and in the Non-D group three of the ten (30%), had had the memory disturbance first pointed out by a relative or colleague.
213
(2) Mood disturbance. A significant degree of mood disturbance was common in both groups. In group D 12 (80%) were thought to have a significant degree of affective disorder. Seven cases (47%) had a predominantly depressed mood with, in five of these cases, periods of apathy (3) or anxiety (2) or lability (1). Lability of mood, rapid fluctuations of mood within brief periods, was the predominant feature in five cases and only one case was thought to be predominantly apathetic. Marked irritability was noted in five cases. There was a previous history of affective disorder in two cases: atypical depression (l), mania (1). In the Non-D group 17 of the 20 ( 8 5 % ) had a significant mood disturbance and a predominantly depressed mood was noted in nine cases (45%), but in all these cases there were periods of apathy or anxiety and in one case lability. In six cases anxiety was the main feature, in two cases apathy was predominant and in no case was lability the predominant mood. Irritability was noted only in one case. In this group a history of past affective disorder was more common: at least one previous depressive illness being recorded in seven cases, one of which had also had a manic phase. (3) Dysphasia and associated disorders. With the exception of one patient who had “periodic dysarthria”, all the patients with speech disturbance were in the D group. Perseveration was recorded in one case in the D group. Nominal dysphasia was present in four cases. Of the four cases with dysphasia there was associated dysgraphia in two cases and alcalculia in one Case. One of these cases also had apraxia as did one other case which was in D group. Dysarthria was present in two of the D cases. Other features looked for because they have been thought to be useful in the diagnosis of dementia proved to be too uncommon in our groups to be useful or did not discriminate between the groups. Investigations
The psychometry reports (28 cases), E.E.G. reports (28 cases) and A.E.G. reports (25 cases) were reviewed to see if they were related to outcome. It must be emphasised here that it is the reports of a variety of specialists in different departments concerned which are reviewed and not the original material. However, in all cases these investigations were requested to support or exclude a diagnosis of dementia. The extent to which these reports did help discriminate
Degree of support for diagnosis of dementia Group
No. tested Nil
“Consistent with”
Moderate
Strong
214
our two groups can be assessed but we are not able to see if particular features of the test records discriminated when these are not mentioned in the test reports. Psychometry. As may be seen in Table 1, psychological testing was carried out on 28 patients: 18 in the Non-D group and ten in the D group. No report was
inconsistent with the diagnosis of dementia. Although 60% of the D group received moderately strong support for the diagnosis of dementia, 67% of the Non-D group had reports consistent with this diagnosis and one patient in the Non-D group had strong psychometric indications of organic impairment. E.E.G. Twenty-eight patients had electroencephalography and, as may be seen from Table 2, reports on these records failed to discriminate between the groups.
Table 2. Electroencephalography
Group
Results
No. having E.E.G.
Non-D
16 12
D
Normal
Abnormal
6 (38%) 3 (25%)
10 (62%) 9 (75%)
A.E.G. Table 3 gives details of the 24 patients who received A.E.G.s. No patient in the D group was reported to have radiological evidence of less than moderate degree of atrophy. The only patients in whom a gross degree of atrophy was demonstrated were in the D group (three cases), and in all of these it was the widening of ventricles rather than sulci which was pronounced. Ten of the 15 Non-D patients had less than moderate degrees of sulcal or ventricular dilation. It is also noted that five (33%) of the Non-D group and six (67%) of the D group were reported to have moderate degrees of atrophy as shown by either sulcal or ventricular dilation or both.
Table 3. Airencephalography
of patients Nil
Minimal
Moderate
Gross
215
DISCUSSION Primary presenile dementia is an uncommon condition. We were able to confirm this diagnosis in only 15 cases between 1950-1969 in a busy psychiatric unit attached to a large general hospital (i.e. 0.2% of psychiatric admissions). In a psychiatric hospital setting Sjogren found 23 cases of Alzheimer’s disease or “Pick-Alzheimer syndrome” among 2,974 female patients he had seen over an 11-year period, i.e. about 0.8% of his female patients (Sj6gren et al. (1952)). Because of this relatively low incidence, the individual clinician see few new cases and most of his experience will be of patients in whom the condition is well advanced. However, even if we allow for this there seem to remain very specific difficulties at present inherent in making this diagnosis at an early stage. Other authors have of course mentioned this difficulty with respect to presenile dementias (e. g. Stengel (1943)), or when writing of the diagnosis of dementia in general (e. g. Kiloh (1961), Rorh & Myers (1969)). However, the very high frequency of misclassification in our series was quite unexpected. This led us to look at these cases specifically in order to further elucidate this. Looking at the clinical features of our two groups overall the main difficulty appears to have been differentiating between those with early presenile dementia and a small group of patients with severe neurotic or affective disorders. There was no evidence that patients suffering from schizophrenia or specific organic disease were misclassified as presenile dementia. The reasons for this become clearer if we look at individual clinical features in the two groups. There was nothing in the sources of referral or the main reasons for referral which discriminated between the two groups. The fact that dementia was only mentioned once as a possibility at the time of referral suggests that preliminary diagnoses by others did not prejudice the clinic diagnosis. What did seem a central source of error was the memory disorder found in all cases. This is mentioned as an early feature in many of the reported cases of presenile dementia. As far as our two groups are concerned all we can say in retrospect is that the more frequently prominent memory difficulties are found in the history and the more severe the memory disorder found on clinical examination the more likely the patient is to be in the dementia group. Confabulation was uncommon but did only occur in the D group. Memory difficulties, although tending to be more severe in the D group, were less often noted first by the patients in that group. But again there is a large overlap between the groups. Kiloh (1961) has emphasised that endogenous depression is the condition most likely to be misdiagnosed as dementia. Especially in the elderly, intellectual and particularly memory deterioration is common in depression. At the same time dementia not uncommonly presents with marked depressive features. Letemendiu (1953, in a retrospective study of 22 cases of Alzheimer’s disease and two cases of Pick’s disease, noted seven cases that had had depressive mood changes at the start of the illness. Sim & Sussman (1962) noted that 12 of their 22 cases of Alzheimer’s disease were severely depressed and eight showed marked agitation. In their non-Alzheimer cases depression was a common symptom, especially in the intermediate stages of the disease.
216
There is no doubt that this was a source of some difficulty in our series. Post (1965) suggests that when dealing with depressive symptoms in the elderly the difference between organic and functional is not of practical importance, since both groups respond well, as far as depressive symptoms are concerned, to antidepressant treatment. It may well be that more vigorous antidepressant measures in some of the nine Non-D patients who were found to be depressed would have clarified the issue. In fact, four patients did receive such treatment, but in spite of this a diagnosis of presenile dementia was made. Other features of mood did seem to discriminate to some extent between the groups. Lability of mood and marked imtability were not common in the series as a whole, but lability in particular only occurred in the D group. The neurological features noted under speech disorder again were uncommon but when they did occur did so only in the D group. The main finding under this heading is therefore that very few clinical features are specific for presenile dementia and when these occur they do so only in a minority of cases, leaving a considerable number of cases in which the diagnosis cannot be made with certainty on clinical features alone. The help provided by ancillary investigations in this situation was limited. Psychometry tended to confirm intellectual deterioration or difficulty in all cases without being of clear discriminating value in the individual case. The E.E.G. similarly was only of qualified support in diagnosing dementia since a quarter of our deteriorated group had normal E.E.G.s and abnormal E.E.G.s were only slightly more frequent in the D group. In both cases we could not say whether specific psychological tests or specific E.E.G. abnormalities would have discriminated between the groups. It may be that the only way of markedly improving this situation is by repeated testing over a period of time, using senat E.E.G. (Gordon (1968)) or repeated psychometry (Peurce-Miller (1973)). Of the ancillary investigations the A.E.G. appeared to be the most useful in discriminating between our two groups. Our finding that gross widening of the ventricle only occurred in the D group is in keeping with Mann’s findings (Munn (1973)) that substantial widening of the lateral ventricle was associated with dementia. Munn found that widening of the sulci to any degree was not significant in this respect. Also in line with his findings is our own finding that eight of 15 of our Non-D patients (47%) were reported as having minimal sulcal or ventricular enlargement, whereas none of the patients in D group received similar reports. In view of these findings it is encouraging to learn that the recently introduced technique of computerised transverse axial scanning (Guwler et al. (1974), Hounsfield (1973)) may be useful in demonstrating cerebral atrophy. It is hoped that this less disturbing procedure, which does not require the introduction of contrast media, may turn out to be useful in the situation we have been discussing. At present the diagnosis of presenile dementia in its early stages remains a difficult exercise. The few specific clinical features are rare and only gross radiological changes can be relied on with confidence. In this study we have tried to highlight some of the difficulties confronting the clinician trying to make this diagnosis.
2’17 REFERENCES Gawler, J., G . H . du Boulay, J . W . D . Bull & J . Marshall (1974): Computer-assisted tomography (EM1 Scanner). Lancet ii, 419-423. Gordon, E . B. (1968): Serial E.E.G. studies in presenile dementia. Brit. I. Psychiat. 114, 779-780. Hounsfield, G . N . (1973): Computerised transverse axial scanning (tomography). Brit. J. Radiol. 46, 1016. Kiloh, L . G. (1961): Pseudodementia. Acta psychiat. scand. 37, 336-351. Letemendia, F. (1955): Early symptoms - signs in presenile dementia. Postgraduate Thesis. University of London. Mann, A. H . (1973): Cortical atrophy and air encephalography: a clinical and radiological study. Psychol. Med. 3, 374-378. Marsden, C. D., & M . J . G . Harrison (1972): Outcome of investigation of patients with presenile dementia. Brit. med. J. 2, 249-252. Pearce, J., & E. Miller (1973): Clinical aspects of dementia. Baillikre and Tindall, London. Post, F. (1965): The clinical psychiatry of late life. Pergamon Press, Oxford. Rorlt, M., & D . H . Myers (1969): The diagnosis of dementia. Brit. J. hosp. Med. 2, 705-717. Sim, M., & I . Swsman (1962): Alzheimer’s disease: itt natural history and differential diagnosis. J. nerv. ment. Dis. 135, 489-499. Sim, M., E. Turner & W . T . Smith (1966): Cerebral biopsy in the investigation of presenile dementia. Brit. J. Psychiat. 112, 119-125, 127-133. Sjogren, T., H . Sjogren & A . Lindgren (1952): Morbus Alzheimer and Morbus Pick. Acta psychiat. scand., Suppl. 82. Stengel, E. (1943): A study on the symptomatology and differential diagnosis of Alzheimer’s disease and Pick’s disease. J. ment. Sci. 89, 1-20. Received October 14, 1974
P. N . Nott, M.D. University Department of Psychiatry The Warneford Hospital Oxford, OX3 7JX England J . J . Fleminger, M.D. York Clinic Guy’s Hospital 117 Borough High Street London S.E.l England
PRESENILE DEMENTIA: THE DIFFICULTIES OF EARLY DIAGNOSIS P.N. Nom
AND J. J. FLEMINGER
A follow-up of 35 patients first diagnosed as having presenile dementia at York Clinic is described. In only 15 cases did progressive deterioration confirm the diagnosis. A retrospective case note study of all the patients is described comparing the clinical features and results of special investigations of those patients in whom the diagnosis was confirmed in this way and those in whom it was not. The results of this study are used to illuminate some of the special difficulties in the early diagnosis of presenile dementia.
Key words. Presenile dementia
- diagnosis - clinical features -
A.E.G. - follow-up.
The early diagnosis of dementia in middle age can be a difficult problem. When this diagnosis is suspected the first priority is to try and identify an underlying cause. This is most important because many specific causes of dementia are now treatable; but also because, even if the specific cause is not treatable, its identification clarifies the prognosis and further management of the patient. In a recent retrospective case study (Marsden & Harrison (1972)), a specific cause was found in 36 of 84 patients diagnosed as having dementia in the presenium, who had been referred to the National Hospital for Nervous Diseases. This, however, still left 48 patients (57%) in whom no specific cause could be identified. In our experience it is in these cases that the early diagnosis of primary presenile dementia is particularly difficult to substantiate and at present of doubtful significance in individual cases. The present study concerns a group of patients diagnosed as having dementia in middle age but in whom no specific cause could be found. There have been many excellent retrospective studies in recent years of the primary presenile dementias. These have attempted to isolate syndromes, find hereditary factors, and relate clinical features to other findings such as biopsy or post-mortem material, ERG. changes and premorbid personality. Above all there has been a major attempt to isolate specific clinicopathological entities; to differentiate clinically between Alzheimer’s disease and Pick’s disease, and between Alzheimer’s disease and the other presenile dementias in the group (e. g. Stengel (1943), Sjogren et 41. (1953), Sim et al. (1966)). Despite these attempts it can still be very difficult, if not impossible, to make these distinctions clinically. Indeed, in practice, this remains a relatively unimportant exercise com-
211
pared with the crucial problem of differentiating between early primary dementia of any kind and disorders of a non-dementing nature, such as affective psychosis. The present study was carried out to examine the validity of the diagnosis of primary presenile dementia as made in the psychiatric unit of a general hospital. We were also interested in studying the clinical features found in these patients to see if any individual items were particularly helpful in predicting outcome. MATERIAL AND METHODS The case notes of all inpatients diagnosed as having dementia in the York Clinic, Guy’s Hospital, 1950 to 1969, were reviewed. All those fulfilling the following criteria were retained for further study: (1) an unequivocal diagnosis of dementia was made for the first time during the admission; (2) the patient was not over the age of 65 at the time of diagnosis; (3) no known cause of cerebral disease had been identified during the admission. Thus, all cases with a history or other evidence of cerebrovascular disease, hypertension, alcoholism, drug dependence, myxoedema, encephalitis, recent or serious head injury, neoplasm, or any other such types or recognised causes of cerebral disorder were excluded from the study. Between the years 1950 to 1969 there had been 50 cases which satisfied the criteria. This represents 0.7% of all admissions. Abstracts were made of the history, clinical features at the time of diagnosis and the results of special investigations in these cases. Following this an attempt was made to trace these patients. Information was obtained from G.P.s, subsequent hospital admission, 0 . P notes, coroners’ reports and post-mortem reports. In this way adequate follow-up data were obtained for 35 of the 50 cases. These 35 cases form the basis of this report.
RESULTS Follow-up
Examination of the case notes of the 15 cases which could not be followed up did not reveal any specific differences which would make us think that they had differed in any special way from the 35 cases studied. Of these 35 patients, only 15 (43%) had deteriorated in the expected manner following the initial diagnosis of presenile dementia. Eighteen cases had actually improved and two cases had remained essentially unchanged. The cases therefore fell into two main groups: The 15 cases in which follow-up confirmed the diagnosis (Deteriorated or D group) and the 20 (57%) cases who did not deteriorate as expected and in whom the diagnosis was therefore not confirmed won-D group). Because our main interest rests in this, the rest of the results will be presented as a comparison between these two groups. The 15 cases in the “deteriorated @)” group consisted of seven men and eight women. The mean age at time of diagnosis was 55 years (range 35-64). At the time of follow-up ten of these patients had died, having survived on average 6 years (range 1-11 years) following diagnosis. The five remaining patients had a mean length of follow-up of 5 years (range 1-15 years). As expect-
212
ed the main cause of death for these cases was intercurrent infection, especially respiratory infection (six cases). The 20 cases in the “non-deteriorated won-D)” group consisted of 11 men and nine women. Mean age at time of diagnosis was 52 years (range 37-59). At the time of follow-up two of the three patients who had died had committed suicide. The mean length of follow-up of those surviving was 10 years (range 5-23 years). Of these 20 Non-D cases only five had remained well throughout the follow-up period. The others had persistent or recurrent signs and symptoms which were classified as follows: depression (3), anxiety state (3), somatic symptoms without organic basis (6), hysterical reaction (l), paranoid reaction associated with respiratory failure (1). One patient developed hemiplegia 20 years after the original diagnosis of presenile dementia. The overall impression was that this group consisted mainly of people with marked personality difficulties and neurotic symptoms or affective disorder. No case developed a persistant psychotic illness or showed evidence on follow-up of an organic illness which might have been present at the time of the original diagnosis and missed. Clinical features
Using the retrospective case note study, the clinical features that were recorded at the time of diagnosis of the two main groups D and Non-D were compared. Reason for referral. The source of referral was the family doctor in the majority of cases in both groups. Referrals from other hospital departments numbered seven of 20 Non-D and four of 15 D patients. The main reason for referral was for the investigation or treatment of depression (8 Non-D, 6 D). All other cases are remarkable only for the variety of reasons in both groups, e. g. shoplifting (1 Non-D), fur fetish (1 D), bizarre hypochondriasis (1 Non-D), persistent pain (1 Non-D); and perhaps remarkable for the fact that the possibility of dementia was only suggested in one case (D, referred by G.P.). Signs and symptoms at time of diagnosis. (1) Memory disturbance. All the patients were thought to have some degree of memory disturbance on examination. There were some in both groups who gave no history of memory difficulties but these were thought to show evidence of memory disorder on testing. Eleven D patients (73%) had a definite history of memory difficulties and this was a prominent feature in the history in seven cases (50%).This does not include one case in this group who had a history of two hysterical fugues. Five in this group had a history of disorientation and four of these were disorientated on examination. Confabulation was recorded in three cases. Ten (50%) in the Non-D group had a definite history of memory disturbance but in only three of these (15%) was it a prominent feature in the history. Two had a history of disorientation but only one was disorientated on examination. There was no cases of confabulation. In the D group six of the 11 (55%), and in the Non-D group three of the ten (30%), had had the memory disturbance first pointed out by a relative or colleague.
213
(2) Mood disturbance. A significant degree of mood disturbance was common in both groups. In group D 12 (80%) were thought to have a significant degree of affective disorder. Seven cases (47%) had a predominantly depressed mood with, in five of these cases, periods of apathy (3) or anxiety (2) or lability (1). Lability of mood, rapid fluctuations of mood within brief periods, was the predominant feature in five cases and only one case was thought to be predominantly apathetic. Marked irritability was noted in five cases. There was a previous history of affective disorder in two cases: atypical depression (l), mania (1). In the Non-D group 17 of the 20 ( 8 5 % ) had a significant mood disturbance and a predominantly depressed mood was noted in nine cases (45%), but in all these cases there were periods of apathy or anxiety and in one case lability. In six cases anxiety was the main feature, in two cases apathy was predominant and in no case was lability the predominant mood. Irritability was noted only in one case. In this group a history of past affective disorder was more common: at least one previous depressive illness being recorded in seven cases, one of which had also had a manic phase. (3) Dysphasia and associated disorders. With the exception of one patient who had “periodic dysarthria”, all the patients with speech disturbance were in the D group. Perseveration was recorded in one case in the D group. Nominal dysphasia was present in four cases. Of the four cases with dysphasia there was associated dysgraphia in two cases and alcalculia in one Case. One of these cases also had apraxia as did one other case which was in D group. Dysarthria was present in two of the D cases. Other features looked for because they have been thought to be useful in the diagnosis of dementia proved to be too uncommon in our groups to be useful or did not discriminate between the groups. Investigations
The psychometry reports (28 cases), E.E.G. reports (28 cases) and A.E.G. reports (25 cases) were reviewed to see if they were related to outcome. It must be emphasised here that it is the reports of a variety of specialists in different departments concerned which are reviewed and not the original material. However, in all cases these investigations were requested to support or exclude a diagnosis of dementia. The extent to which these reports did help discriminate
Degree of support for diagnosis of dementia Group
No. tested Nil
“Consistent with”
Moderate
Strong
214
our two groups can be assessed but we are not able to see if particular features of the test records discriminated when these are not mentioned in the test reports. Psychometry. As may be seen in Table 1, psychological testing was carried out on 28 patients: 18 in the Non-D group and ten in the D group. No report was
inconsistent with the diagnosis of dementia. Although 60% of the D group received moderately strong support for the diagnosis of dementia, 67% of the Non-D group had reports consistent with this diagnosis and one patient in the Non-D group had strong psychometric indications of organic impairment. E.E.G. Twenty-eight patients had electroencephalography and, as may be seen from Table 2, reports on these records failed to discriminate between the groups.
Table 2. Electroencephalography
Group
Results
No. having E.E.G.
Non-D
16 12
D
Normal
Abnormal
6 (38%) 3 (25%)
10 (62%) 9 (75%)
A.E.G. Table 3 gives details of the 24 patients who received A.E.G.s. No patient in the D group was reported to have radiological evidence of less than moderate degree of atrophy. The only patients in whom a gross degree of atrophy was demonstrated were in the D group (three cases), and in all of these it was the widening of ventricles rather than sulci which was pronounced. Ten of the 15 Non-D patients had less than moderate degrees of sulcal or ventricular dilation. It is also noted that five (33%) of the Non-D group and six (67%) of the D group were reported to have moderate degrees of atrophy as shown by either sulcal or ventricular dilation or both.
Table 3. Airencephalography
of patients Nil
Minimal
Moderate
Gross
215
DISCUSSION Primary presenile dementia is an uncommon condition. We were able to confirm this diagnosis in only 15 cases between 1950-1969 in a busy psychiatric unit attached to a large general hospital (i.e. 0.2% of psychiatric admissions). In a psychiatric hospital setting Sjogren found 23 cases of Alzheimer’s disease or “Pick-Alzheimer syndrome” among 2,974 female patients he had seen over an 11-year period, i.e. about 0.8% of his female patients (Sj6gren et al. (1952)). Because of this relatively low incidence, the individual clinician see few new cases and most of his experience will be of patients in whom the condition is well advanced. However, even if we allow for this there seem to remain very specific difficulties at present inherent in making this diagnosis at an early stage. Other authors have of course mentioned this difficulty with respect to presenile dementias (e. g. Stengel (1943)), or when writing of the diagnosis of dementia in general (e. g. Kiloh (1961), Rorh & Myers (1969)). However, the very high frequency of misclassification in our series was quite unexpected. This led us to look at these cases specifically in order to further elucidate this. Looking at the clinical features of our two groups overall the main difficulty appears to have been differentiating between those with early presenile dementia and a small group of patients with severe neurotic or affective disorders. There was no evidence that patients suffering from schizophrenia or specific organic disease were misclassified as presenile dementia. The reasons for this become clearer if we look at individual clinical features in the two groups. There was nothing in the sources of referral or the main reasons for referral which discriminated between the two groups. The fact that dementia was only mentioned once as a possibility at the time of referral suggests that preliminary diagnoses by others did not prejudice the clinic diagnosis. What did seem a central source of error was the memory disorder found in all cases. This is mentioned as an early feature in many of the reported cases of presenile dementia. As far as our two groups are concerned all we can say in retrospect is that the more frequently prominent memory difficulties are found in the history and the more severe the memory disorder found on clinical examination the more likely the patient is to be in the dementia group. Confabulation was uncommon but did only occur in the D group. Memory difficulties, although tending to be more severe in the D group, were less often noted first by the patients in that group. But again there is a large overlap between the groups. Kiloh (1961) has emphasised that endogenous depression is the condition most likely to be misdiagnosed as dementia. Especially in the elderly, intellectual and particularly memory deterioration is common in depression. At the same time dementia not uncommonly presents with marked depressive features. Letemendiu (1953, in a retrospective study of 22 cases of Alzheimer’s disease and two cases of Pick’s disease, noted seven cases that had had depressive mood changes at the start of the illness. Sim & Sussman (1962) noted that 12 of their 22 cases of Alzheimer’s disease were severely depressed and eight showed marked agitation. In their non-Alzheimer cases depression was a common symptom, especially in the intermediate stages of the disease.
216
There is no doubt that this was a source of some difficulty in our series. Post (1965) suggests that when dealing with depressive symptoms in the elderly the difference between organic and functional is not of practical importance, since both groups respond well, as far as depressive symptoms are concerned, to antidepressant treatment. It may well be that more vigorous antidepressant measures in some of the nine Non-D patients who were found to be depressed would have clarified the issue. In fact, four patients did receive such treatment, but in spite of this a diagnosis of presenile dementia was made. Other features of mood did seem to discriminate to some extent between the groups. Lability of mood and marked imtability were not common in the series as a whole, but lability in particular only occurred in the D group. The neurological features noted under speech disorder again were uncommon but when they did occur did so only in the D group. The main finding under this heading is therefore that very few clinical features are specific for presenile dementia and when these occur they do so only in a minority of cases, leaving a considerable number of cases in which the diagnosis cannot be made with certainty on clinical features alone. The help provided by ancillary investigations in this situation was limited. Psychometry tended to confirm intellectual deterioration or difficulty in all cases without being of clear discriminating value in the individual case. The E.E.G. similarly was only of qualified support in diagnosing dementia since a quarter of our deteriorated group had normal E.E.G.s and abnormal E.E.G.s were only slightly more frequent in the D group. In both cases we could not say whether specific psychological tests or specific E.E.G. abnormalities would have discriminated between the groups. It may be that the only way of markedly improving this situation is by repeated testing over a period of time, using senat E.E.G. (Gordon (1968)) or repeated psychometry (Peurce-Miller (1973)). Of the ancillary investigations the A.E.G. appeared to be the most useful in discriminating between our two groups. Our finding that gross widening of the ventricle only occurred in the D group is in keeping with Mann’s findings (Munn (1973)) that substantial widening of the lateral ventricle was associated with dementia. Munn found that widening of the sulci to any degree was not significant in this respect. Also in line with his findings is our own finding that eight of 15 of our Non-D patients (47%) were reported as having minimal sulcal or ventricular enlargement, whereas none of the patients in D group received similar reports. In view of these findings it is encouraging to learn that the recently introduced technique of computerised transverse axial scanning (Guwler et al. (1974), Hounsfield (1973)) may be useful in demonstrating cerebral atrophy. It is hoped that this less disturbing procedure, which does not require the introduction of contrast media, may turn out to be useful in the situation we have been discussing. At present the diagnosis of presenile dementia in its early stages remains a difficult exercise. The few specific clinical features are rare and only gross radiological changes can be relied on with confidence. In this study we have tried to highlight some of the difficulties confronting the clinician trying to make this diagnosis.
2’17 REFERENCES Gawler, J., G . H . du Boulay, J . W . D . Bull & J . Marshall (1974): Computer-assisted tomography (EM1 Scanner). Lancet ii, 419-423. Gordon, E . B. (1968): Serial E.E.G. studies in presenile dementia. Brit. I. Psychiat. 114, 779-780. Hounsfield, G . N . (1973): Computerised transverse axial scanning (tomography). Brit. J. Radiol. 46, 1016. Kiloh, L . G. (1961): Pseudodementia. Acta psychiat. scand. 37, 336-351. Letemendia, F. (1955): Early symptoms - signs in presenile dementia. Postgraduate Thesis. University of London. Mann, A. H . (1973): Cortical atrophy and air encephalography: a clinical and radiological study. Psychol. Med. 3, 374-378. Marsden, C. D., & M . J . G . Harrison (1972): Outcome of investigation of patients with presenile dementia. Brit. med. J. 2, 249-252. Pearce, J., & E. Miller (1973): Clinical aspects of dementia. Baillikre and Tindall, London. Post, F. (1965): The clinical psychiatry of late life. Pergamon Press, Oxford. Rorlt, M., & D . H . Myers (1969): The diagnosis of dementia. Brit. J. hosp. Med. 2, 705-717. Sim, M., & I . Swsman (1962): Alzheimer’s disease: itt natural history and differential diagnosis. J. nerv. ment. Dis. 135, 489-499. Sim, M., E. Turner & W . T . Smith (1966): Cerebral biopsy in the investigation of presenile dementia. Brit. J. Psychiat. 112, 119-125, 127-133. Sjogren, T., H . Sjogren & A . Lindgren (1952): Morbus Alzheimer and Morbus Pick. Acta psychiat. scand., Suppl. 82. Stengel, E. (1943): A study on the symptomatology and differential diagnosis of Alzheimer’s disease and Pick’s disease. J. ment. Sci. 89, 1-20. Received October 14, 1974
P. N . Nott, M.D. University Department of Psychiatry The Warneford Hospital Oxford, OX3 7JX England J . J . Fleminger, M.D. York Clinic Guy’s Hospital 117 Borough High Street London S.E.l England
