13
Meeting 10 February 1976
Psychiatric Disturbances in the Mentally Handicapped Dr Andrew H Reid (Royal Dundee Liff Hospital, Dundee, DD2 5NF)
Psychiatric disturbances are common amongst mental defectives and the investigation and treatment of these disturbances is central to the work of the consultant psychiatrist in this field. It is surprising, therefore, that there has been so little research into the problem. In a review of the literature Reid (1971) summarized the main contributions, commented on the lack of recent research and drew attention to the different diagnostic and phenomenological assumptions that had been made by various investigators. There was doubt as to whether manic-depressive and schizophrenic psychoses could occur or be diagnosed in mental defectives and no consensus of opinion about the course and responsiveness to treatment of these psychoses. The author began, therefore, by studying the applicability of the diagnostic framework of the affective and schizophrenic psychoses to mental defectives, identifying the phenomena of these psychoses and assessing their responsiveness to treatment (Reid 1971, 1972). In brief, the author came to the conclusion that manic-depressive psychosis does occur in mental defectives. quite frequently in fact, and that it can be diagnosed in mental defectives of all degrees of retardation, including occasionally the profoundly retarded. Manic-depressive psychosis seemed to respond to treatment, and recognition and diagnosis were therefore of some importance. Obviously the presence of mental deficiency modifies the clinical features and sometimes makes identification of the psychosis a diagnostic challenge. In mania, for example, the elation of mood and motor overactivity may be poorly sustained and wit and humour tend to be lacking. It may be hard to distinguish between catatonic schizophrenic excitement and chaotic manic elation. Symptoms such as flight of ideas and clang associations may be rudimentary. When depressed, patients may be sullen or irritable, or they may complain more of being 'fed up' than of being overtly depressed. Inability to rationalize and talk about their mood state may contribute to acting out in- the form of aggression or selfinjury. Anorexia, sleep disturbance, somatic, aggressive and gross hysterical symptoms are also
Section ofPsychiatry
509
common in defectives suffering from an affective psychosis. Mixed affective states in which there are dissociations between mood, motor and mental activity and resulting admixtures of depressive symptoms, such as tearfulness or lability, alongside manic symptoms, such as talkativeness, and grandiosity, are-also encountered. Delusions and hallucinations, coloured by the appropriate mood state, also occur in affective psychoses in mental defectives, just as they do in patients of normal intelligence. In defectives, however, delusions and hallucinations may sometimes have a rather naive content and be grandiosely wish fulfilling in mania or dramatically guilty in depression. Case 1 This patient with an IQ of around 55 was brain damaged following birth injury. He was referred to the out-patient clinic when he was aged 17 because he had become acutely disturbed with grandiose delusions of wealth and possessions. He was somewhat restless but his motor symptoms were relatively slight compared to his floridly deluded mental state. He was admitted to hospital where his illness remitted over a few weeks on treatment with chlorpromazine. He was discharged and followed up at the out-patient clinic, made a good recovery and returned to sheltered employment. He relapsed some 14 months later, again with florid and grandiose delusions, believing that he had become engaged to the daughter of a prominent figure in public life who was going to buy him a house, a ring and a car. He maintained that he was becoming a year older every day and he wore round his neck a key on a string symbolizing his chain of high office. He was somewhat restless and slept poorly. He was admitted to hospital where his symptoms again remitted and he was started this time on lithium. He demonstrated remarkably florid and naively wishfulfilling delusional beliefs. There were doubts about the diagnosis during his first illness because of the very obvious psychogenic factors but the occurrence of a second illness, intervening as it did without obvious precipitant, suggests that he may well be suffering from a manic-depressive psychosis.
Diagnostic problems arise particularly amongst the more severely mentally defective patients who may have very little or no powers of verbal communication. They cannot say that they feel depressed although experienced nursing staff can certainly sense their distress. Reid contended that affective psychosis could occur in these patients and that it could be diagnosed if observation was careful and a longitudinal study was made of the patient's behaviour, accompanied by information about sleep, appetite, and other physiological functions, backed up at times by a knowledge of family histories of mental illness patterns. Case 2 This patient suffered from severe epilepsy in early infancy and was a markedly hyperkinetic child. She walked at the age of 3 but never acquired speech
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of any form and was admitted to Strathmartine Hospital when she was aged 8. Around this time epileptic activity largely ceased but at the age of 20 she was recorded as showing manneristic behaviour. These mannerisms, which include finger flicking and oscillating, swaying, rocking and endlessly chewing and extruding from her mouth a piece of thread, now occupy her almost constantly. She has some memory and shows a preference for sameness in her daily activities and environment. Her parents remain in contact and take her for a holiday every year. She was aged 32 when they visited her and commented that she was 'too quiet... just different... looked unhappy. not her usual self ... not like this before.' For domestic reasons they did not take her home and she became profoundly depressed, refusing food, losing weight and crying: she showed no interest in her usual activities, seemed apathetic and began to waken very early from sleep around midnight or before. She continued depressed for some six weeks and then her parents wrote in to say that they would shortly be coming to take her on holiday. The message was passed to her although she gave no hint of understanding. Shortly afterwards she improved, recovering to her former state and her parents were able to take her home as usual. The diagnostic problems in this patient were substantial as it was so difficult to gain access to her mental state. The phenomena were those of a depressive psychosis but they were superimposed on a preexisting illness with features of an infantile psychosis. Psychogenic factors may have been relevant but her depressive symptoms antedated the postponement of her holiday. There was nothing obviously unusual in the natural history and age at onset of affective psychosis in the majority of patients, most of whom had suffered several previous attacks in the course of their lives. We do know, however, that, particularly amongst the severely mentally defective, most of whom have become hospitalized by the time they reach adulthood, there is a high percentage with brain damage of one sort or another, and it may be that brain damage itself acts as a precipitating and perpetuating factor for affective psychosis in some cases. In other patients the illness seems to be precipitated by
14
by quite marked motor retardation amounting at times to near stupor: the other patient showed a regularly recurrent mixed affective psychosis. These mood changes were accompanied by changes in sleep, pulse rate and temperature. The cycle lengths in these 4 patients were all within the range of around 4-9 weeks and in one of them these changes were strikingly regular. Naylor et al. (1976) were able to correlate clinical state with certain biochemical variables. Naylor et al. (1974) have also studied in a double-blind controlled trial the response to the treatment with lithium of manic-depressive psychosis in mental defectives and found that lithium reduced the duration and frequency of affective disorders in mental defectives, including some who were severely retarded. They did report occasional neurotoxic side-effects at very low blood lithium levels and some patients in whom satisfactory blood levels could only be reached by increasing the dosage to a surprisingly high level, interesting findings in themselves and unexplained. Other investigators have reported further cases of successful treatment with lithium of manic-depressive psychosis in mental defectives (Adams et al. 1970, Forssman & Walinder 1969). In attempting to diagnose schizophrenia in mental defectives many of the first rank symptoms cannot be reliably identified much below an IQ of around 45. The author found it impossible to diagnose schizophrenic psychoses in mental defectives of an IQ much below this level - at least using the accepted clinical diagnostic criteria which are still the only diagnostic criteria we have (Batchelor 1964). The author found that motility disorders in mentally defective schizophrenics were similar to these phenomena in schizophrenics of normal intelligence. There was no clinical. similarity between the manneristic, stereotyped movements and finger posturing shown by some mental defectives and schizophrenic disorders of motility. There was otherwise nothing particularly unusual in the clinical features, age at onset and natural psychological stresses, infections, operations, history of schizophrenic psychoses in mental separations or bereavements. More usually there defectives although schizophrenia in defectives seemed to run a slightly more benign course with is no obvious precipitant. There is, however, one group which does show the patient remaining more accessible and showing less deterioration. These illnesses seemed to some rather unusual features and that is the short cycle patients. Hayman (1939) has drawn respond to standard physical methods of treatattention to the 'constitutional periodicity' of ment. some alternating psychoses in 'the lowest grades'. Reid & Naylor (1976) have recently studied 4 Case 3 This patient, first seen at age 49, had never patients, 3 of them severely mentally defective, been able to hold down a job and since the death of mother eight years previously had lived under who showed fairly rapidly alternating behavioural his deplorable conditions occupying a derelict room in a phases. The 3 severely defective patients showed condemned tenement building, spending his time what seemed to be a bipolar manic-depressive wandering around Dundee and collecting old railway psychosis with periods of mood elevation timetables. With the gradual demolition of this type accompanied by motor overactivity, interspersed of property he was rehoused in a new council estate with periods of apparent depression accompanied on the periphery of the town where his eccentric ..
511
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Section ofPsychiatry
behaviour proved unacceptable to his neighbours. At the clinic he was very unkempt and his speech and use of language were stilted; he described what seemed to be auditory hallucinations and expressed ill-formed delusions of persecution. He was clearly mentally defective and his IQ was assessed at around 50. He was also obviously very eccentric and it seemed likely that he was suffering from schizophrenia. The author suggested that he should be offered a room in another tenement in the centre of the city where he could be in a familiar environment but this could not be arranged and he was instead rehoused in another council estate. He was probably teased by the children, became more obviously disturbed and threatening, and was charged with breach of the peace and committed to prison. In prison he developed florid paranoid symptoms believing he was accused of being a German, carrying a bomb and murdering children. He was committed to Royal Dundee Liff Hospital where his acute symptoms subsided on treatment with thioridazine. He again presented as an eccentric, defective and probably schizophrenic patient. After some months thioridazine was withdrawn as he seemed even slower than usual and within a few days he went into a state of catalepsy, maintaining an imposed posture and displaying marked negativism including retention of food in his mouth. This state gradually resolved with nursing care but a few months later he was again obviously psychotic, excited, laughing in response to hallucinatory voices and repeating odd stereotyped phrases. This relapse responded again to thioridazine and he is now back to his usual level. He illustrates a blending and intermingling of catatonic and hebephrenic symptoms, an acute paranoid state in the face of a hostile and threatening environment, and the ready resolution of these symptoms with remarkably little residual defect. Unfortunately no early IQ estimations were available.
screened all the patients over the age of 45 at Strathmartine Hospital, 155 in all, for evidence of dementia, and identified a group of II who seemed to be showing signs of a dementing process. Both authors then assessed these 11 patients. in detail and followed them up over a period of time. They found that it was possible to diagnose dementia on the usual clinical grounds in mental defectives down to an IQ of around 25. Serial EEG recordings were helpful in cases where there were diagnostic problems. To date the neuropathological -findings in the patients who have died since the survey was carried out have confirmed the clinical diagnosis. In some of the patients affective symptoms were prodromal features of the dementing process. Mongols, in particular, seemed to manifest the clinical features of a premature form of senile dementia with disproportionate frequency. Mongols are known to manifest the neuropathological changes of Alzheimer's disease with unusual frequency as they get older (Burger & Vogel 1973) and develop epilepsy with advancing age (Veall 1974). They also show premature tissue ageing in the aortic and pulmonary valves (Sylvester 1974). Ageing in mongols would therefore seem to be a pathological process and a fruitful area for intensive gerontological research.
Affective and schizophrenic psychoses were found to occur in patients whose mental retardation was of varying etiologies, including sex chromosomal and autosomal abnormalities, cerebral diplegia and brain damage, as well as in patients in whom the etiology of the mental retardation could not be specified. Blindness and deafness occurred in around 40% of the patients with paranoid syndromes. Overall in the group of patients with affective and schizophrenic psychoses the prevalence of epilepsy was about 25 %. Epilepsy is, however, common in mental defectives and this prevalence rate is almost identical to the figure reported by Penrose in the Colchester survey (1938). Reid & Aungle (1974) then proceeded to study the problem of dementia in mental defectives, in particular senile, cerebral arteriosclerotic and presenile dementia. It is now quite widely recognized that with the greater life expectancy of the mentally defective the problems of old age are assuming progressively more importance in this field. Even so there was hardly a reference in the literature to the topic. The first author therefore
Case 4 This patient was a low-grade translocation mongol with a 46 XY 21/22 chromosome abnormality. He used to be able to carry on a simple conversation, sing (tunelessly), feed and dress himself. He went blind and deaf in his late teens and developed grand mal epilepsy around the age of 40. For many years he was clean in his habits and able to find his way round the ward by touch. About three years prior to his death he became withdrawn and unsociable and underwent what appeared to be a personality change. He started to sleep poorly and began to get up at night. Soon after he became doubly incontinent,-progressively less capable of fending for himself and terminally he began to eat rubbish. He was diagnosed in life as suffering from a senile dementia of unusually early onset and he died at the age of 53. At autopsy he was found to have purulent bronchitis and bronchopneumonia. The cesophagus was obstructed by a mass of rolled up paper and there were foreign bodies in the stomach. At neuropathological examination the brain weighed 1100 g and showed severe generalized gyral atrophy with opening up of both sylvian fissures. Coronal sectioning of the cerebrum confirmed the atrophy and revealed symmetrical dilatation of the lateral and third ventricles. Changes characteristic of severe Alzheimer's disease were present in all sections of cerebral cortex examined microscopically (details on request). This patient illustrates the feasibility of diagnosing dementia in quite severely defective patients given the presence of adequate data about premorbid functioning. EEG examinations in this mongol were helpfu', showing progressively increasing amounts of slow activity.
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Proc. roy. Soc. Med. Volume 69 July 1976
16
So far no reference has been made to prevalence rates. Hospital-based prevalence rates can be misleading, particularly for conditions such as affective psychosis which are clinically manifest only intermittently and which form only a small part of the psychiatric pathology encountered. A high prevalence rate for affective or schizophrenic psychosis in one hospital as opposed to another may imply no more than different admission criteria or a different distribution of psychiatrically disturbed patients as between the mental illness and the mental deficiency services in a region. Moreover hospital-based prevalence rates do not apply to defectives living at home. The prevalence rates of 1.2 % for affective psychosis and 3.2 % for schizophrenic and paranoid psychoses reported by Reid (1972) have to be seen in this perspective. The prevalence rate of 7.1 % for dementia reported by Reid & Aungle (1974) refers only to the rate amongst a population in hospital aged 45 and over. These prevalence rates take no account of nonpsychotic disorders which are much more numerous, nor do they include psychotic disorders which do not fall into the recognized diagnostic categories of functional and organic psychoses. Given these limitations the rates quoted are broadly in line with those reported by Heaton-Ward (1976, personal communication). Ballinger & Reid (in preparation) have attempted to study prevalence rates for both psychotic and nonpsychotic psychiatric disorder amongst a hospital-based group of adult defectives, as compared with a group of defectives living at home and attending an adult training centre. The preliminary results of this investigation have shown surprisingly little evidence of significant psychiatric disorder in the community based group, confirming to some extent the view expressed by Oliver (1975) that the main direct treatment contribution of the consultant psychiatrist working with adult mental defectives lies with the population in hospital. This is only to be expected since most mental deficiency hospitals now admit selectively patients who are psychiatrically disturbed or physically dependent, and seek alternative placements in the community for the more numerous well-behaved adult defectives who can be assimilated into local authority social work provision. It should be noted, however, that Ballinger & Reid did not attempt in their survey to quantify the psychiatric problems within the family associated with the presence of the defective at home: such problems are frequently of considerable significance and should form a substantial part of the work load of the consultant psychiatrist outwith the hospital (Sykes 1975).
In this paper the author has considered only the applicability of the presently recognized clinical psychiatric concepts to adult mental defectives. This obviously omits substantial areas of psychiatric pathology. There are, particularly among the more severely defective, many patients in whom it is possible to agree on the presence of considerable psychiatric disorder but whose illnesses are very difficult or impossible to classify within the existing diagnostic framework. There is a need for much more research into the diagnosis and natural history of psychiatric disorder in these patients amongst whom one meets problems relating to aggressiveness, hyperkinesis, pathological social withdrawal and 'grown up' cases of infantile and childhood disintegrative psychoses to name but a few. There is also the real possibility that such research might usefully extend our understanding of the neurological, neurophysiological and neuropathological factors affecting behaviour, and our treatment options.
Acknowledgments: I am grateful to my colleagues in Dundee for their help with these investigations and also to Dr A F J Maloney, Neuropathology Laboratory, Royal Infirmary, Edinburgh, for post-mortem brain examinations. REFERENCES Adams G L, Kivowitz J & Ziskind E (1970) Archives of GeneralPsychistry 23, 305-309 Batchelor I R C (1964) Proceedings of the Royal Society ofMedicine 57, 417-419 Burger P C & Vogel F S (1973) American Journal ofPathology 73, 457-468 Forssman H & Wilinder J (1969) Acta psychiatrica Scandinavica Supplement 207, 34-40 Hayman M (1939) Journal of Mental Science 85, 1183-1193 Naylor G J, Donald J M, Le Poidevin D & Reid A H (1974) British Journal ofPsychiatry 124, 52-57 Naylor G J, Reid A H, Dick D A T & Dick E G (1976) British Journal ofPsychiatry 128, 169-180 Oliver B E (1975) Journal of Mental Subnormality 21, 115-116 Penrose L S (1938) Special Report Series of the Medical Research Council (London) No. 229 Reid A H (1971) MD Thesis, Dundee (1972) British Journal ofPsychiatry 120, 205-218 Reid A H & Aungle P G (1974) Journal ofMental Deficiency Research 18, 15-23 Reid A H &Naylor G J (1976) Journal of Mental Deficiency Research 20, 67-76 Sykes D P (1975) Journal of Mental Subnormality 21, 3-5 Sylvester P E (1974) Journal of Mental Deficiency Research 18, 367-376 Veall R M (I974) Journal of Mental Deficiency Research 18, 99-106
Dr W A Heaton-Ward (Stoke Park Hospital, Bristol) also took part in the meeting. His remarks were based on his recent Blake Marsh Lecture (see British Journal of Psychiatry, 1976, in press).
Meeting 10 February 1976
Psychiatric Disturbances in the Mentally Handicapped Dr Andrew H Reid (Royal Dundee Liff Hospital, Dundee, DD2 5NF)
Psychiatric disturbances are common amongst mental defectives and the investigation and treatment of these disturbances is central to the work of the consultant psychiatrist in this field. It is surprising, therefore, that there has been so little research into the problem. In a review of the literature Reid (1971) summarized the main contributions, commented on the lack of recent research and drew attention to the different diagnostic and phenomenological assumptions that had been made by various investigators. There was doubt as to whether manic-depressive and schizophrenic psychoses could occur or be diagnosed in mental defectives and no consensus of opinion about the course and responsiveness to treatment of these psychoses. The author began, therefore, by studying the applicability of the diagnostic framework of the affective and schizophrenic psychoses to mental defectives, identifying the phenomena of these psychoses and assessing their responsiveness to treatment (Reid 1971, 1972). In brief, the author came to the conclusion that manic-depressive psychosis does occur in mental defectives. quite frequently in fact, and that it can be diagnosed in mental defectives of all degrees of retardation, including occasionally the profoundly retarded. Manic-depressive psychosis seemed to respond to treatment, and recognition and diagnosis were therefore of some importance. Obviously the presence of mental deficiency modifies the clinical features and sometimes makes identification of the psychosis a diagnostic challenge. In mania, for example, the elation of mood and motor overactivity may be poorly sustained and wit and humour tend to be lacking. It may be hard to distinguish between catatonic schizophrenic excitement and chaotic manic elation. Symptoms such as flight of ideas and clang associations may be rudimentary. When depressed, patients may be sullen or irritable, or they may complain more of being 'fed up' than of being overtly depressed. Inability to rationalize and talk about their mood state may contribute to acting out in- the form of aggression or selfinjury. Anorexia, sleep disturbance, somatic, aggressive and gross hysterical symptoms are also
Section ofPsychiatry
509
common in defectives suffering from an affective psychosis. Mixed affective states in which there are dissociations between mood, motor and mental activity and resulting admixtures of depressive symptoms, such as tearfulness or lability, alongside manic symptoms, such as talkativeness, and grandiosity, are-also encountered. Delusions and hallucinations, coloured by the appropriate mood state, also occur in affective psychoses in mental defectives, just as they do in patients of normal intelligence. In defectives, however, delusions and hallucinations may sometimes have a rather naive content and be grandiosely wish fulfilling in mania or dramatically guilty in depression. Case 1 This patient with an IQ of around 55 was brain damaged following birth injury. He was referred to the out-patient clinic when he was aged 17 because he had become acutely disturbed with grandiose delusions of wealth and possessions. He was somewhat restless but his motor symptoms were relatively slight compared to his floridly deluded mental state. He was admitted to hospital where his illness remitted over a few weeks on treatment with chlorpromazine. He was discharged and followed up at the out-patient clinic, made a good recovery and returned to sheltered employment. He relapsed some 14 months later, again with florid and grandiose delusions, believing that he had become engaged to the daughter of a prominent figure in public life who was going to buy him a house, a ring and a car. He maintained that he was becoming a year older every day and he wore round his neck a key on a string symbolizing his chain of high office. He was somewhat restless and slept poorly. He was admitted to hospital where his symptoms again remitted and he was started this time on lithium. He demonstrated remarkably florid and naively wishfulfilling delusional beliefs. There were doubts about the diagnosis during his first illness because of the very obvious psychogenic factors but the occurrence of a second illness, intervening as it did without obvious precipitant, suggests that he may well be suffering from a manic-depressive psychosis.
Diagnostic problems arise particularly amongst the more severely mentally defective patients who may have very little or no powers of verbal communication. They cannot say that they feel depressed although experienced nursing staff can certainly sense their distress. Reid contended that affective psychosis could occur in these patients and that it could be diagnosed if observation was careful and a longitudinal study was made of the patient's behaviour, accompanied by information about sleep, appetite, and other physiological functions, backed up at times by a knowledge of family histories of mental illness patterns. Case 2 This patient suffered from severe epilepsy in early infancy and was a markedly hyperkinetic child. She walked at the age of 3 but never acquired speech
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Proc. roy. Soc. Med. Volume 69 July 1976
of any form and was admitted to Strathmartine Hospital when she was aged 8. Around this time epileptic activity largely ceased but at the age of 20 she was recorded as showing manneristic behaviour. These mannerisms, which include finger flicking and oscillating, swaying, rocking and endlessly chewing and extruding from her mouth a piece of thread, now occupy her almost constantly. She has some memory and shows a preference for sameness in her daily activities and environment. Her parents remain in contact and take her for a holiday every year. She was aged 32 when they visited her and commented that she was 'too quiet... just different... looked unhappy. not her usual self ... not like this before.' For domestic reasons they did not take her home and she became profoundly depressed, refusing food, losing weight and crying: she showed no interest in her usual activities, seemed apathetic and began to waken very early from sleep around midnight or before. She continued depressed for some six weeks and then her parents wrote in to say that they would shortly be coming to take her on holiday. The message was passed to her although she gave no hint of understanding. Shortly afterwards she improved, recovering to her former state and her parents were able to take her home as usual. The diagnostic problems in this patient were substantial as it was so difficult to gain access to her mental state. The phenomena were those of a depressive psychosis but they were superimposed on a preexisting illness with features of an infantile psychosis. Psychogenic factors may have been relevant but her depressive symptoms antedated the postponement of her holiday. There was nothing obviously unusual in the natural history and age at onset of affective psychosis in the majority of patients, most of whom had suffered several previous attacks in the course of their lives. We do know, however, that, particularly amongst the severely mentally defective, most of whom have become hospitalized by the time they reach adulthood, there is a high percentage with brain damage of one sort or another, and it may be that brain damage itself acts as a precipitating and perpetuating factor for affective psychosis in some cases. In other patients the illness seems to be precipitated by
14
by quite marked motor retardation amounting at times to near stupor: the other patient showed a regularly recurrent mixed affective psychosis. These mood changes were accompanied by changes in sleep, pulse rate and temperature. The cycle lengths in these 4 patients were all within the range of around 4-9 weeks and in one of them these changes were strikingly regular. Naylor et al. (1976) were able to correlate clinical state with certain biochemical variables. Naylor et al. (1974) have also studied in a double-blind controlled trial the response to the treatment with lithium of manic-depressive psychosis in mental defectives and found that lithium reduced the duration and frequency of affective disorders in mental defectives, including some who were severely retarded. They did report occasional neurotoxic side-effects at very low blood lithium levels and some patients in whom satisfactory blood levels could only be reached by increasing the dosage to a surprisingly high level, interesting findings in themselves and unexplained. Other investigators have reported further cases of successful treatment with lithium of manic-depressive psychosis in mental defectives (Adams et al. 1970, Forssman & Walinder 1969). In attempting to diagnose schizophrenia in mental defectives many of the first rank symptoms cannot be reliably identified much below an IQ of around 45. The author found it impossible to diagnose schizophrenic psychoses in mental defectives of an IQ much below this level - at least using the accepted clinical diagnostic criteria which are still the only diagnostic criteria we have (Batchelor 1964). The author found that motility disorders in mentally defective schizophrenics were similar to these phenomena in schizophrenics of normal intelligence. There was no clinical. similarity between the manneristic, stereotyped movements and finger posturing shown by some mental defectives and schizophrenic disorders of motility. There was otherwise nothing particularly unusual in the clinical features, age at onset and natural psychological stresses, infections, operations, history of schizophrenic psychoses in mental separations or bereavements. More usually there defectives although schizophrenia in defectives seemed to run a slightly more benign course with is no obvious precipitant. There is, however, one group which does show the patient remaining more accessible and showing less deterioration. These illnesses seemed to some rather unusual features and that is the short cycle patients. Hayman (1939) has drawn respond to standard physical methods of treatattention to the 'constitutional periodicity' of ment. some alternating psychoses in 'the lowest grades'. Reid & Naylor (1976) have recently studied 4 Case 3 This patient, first seen at age 49, had never patients, 3 of them severely mentally defective, been able to hold down a job and since the death of mother eight years previously had lived under who showed fairly rapidly alternating behavioural his deplorable conditions occupying a derelict room in a phases. The 3 severely defective patients showed condemned tenement building, spending his time what seemed to be a bipolar manic-depressive wandering around Dundee and collecting old railway psychosis with periods of mood elevation timetables. With the gradual demolition of this type accompanied by motor overactivity, interspersed of property he was rehoused in a new council estate with periods of apparent depression accompanied on the periphery of the town where his eccentric ..
511
15
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behaviour proved unacceptable to his neighbours. At the clinic he was very unkempt and his speech and use of language were stilted; he described what seemed to be auditory hallucinations and expressed ill-formed delusions of persecution. He was clearly mentally defective and his IQ was assessed at around 50. He was also obviously very eccentric and it seemed likely that he was suffering from schizophrenia. The author suggested that he should be offered a room in another tenement in the centre of the city where he could be in a familiar environment but this could not be arranged and he was instead rehoused in another council estate. He was probably teased by the children, became more obviously disturbed and threatening, and was charged with breach of the peace and committed to prison. In prison he developed florid paranoid symptoms believing he was accused of being a German, carrying a bomb and murdering children. He was committed to Royal Dundee Liff Hospital where his acute symptoms subsided on treatment with thioridazine. He again presented as an eccentric, defective and probably schizophrenic patient. After some months thioridazine was withdrawn as he seemed even slower than usual and within a few days he went into a state of catalepsy, maintaining an imposed posture and displaying marked negativism including retention of food in his mouth. This state gradually resolved with nursing care but a few months later he was again obviously psychotic, excited, laughing in response to hallucinatory voices and repeating odd stereotyped phrases. This relapse responded again to thioridazine and he is now back to his usual level. He illustrates a blending and intermingling of catatonic and hebephrenic symptoms, an acute paranoid state in the face of a hostile and threatening environment, and the ready resolution of these symptoms with remarkably little residual defect. Unfortunately no early IQ estimations were available.
screened all the patients over the age of 45 at Strathmartine Hospital, 155 in all, for evidence of dementia, and identified a group of II who seemed to be showing signs of a dementing process. Both authors then assessed these 11 patients. in detail and followed them up over a period of time. They found that it was possible to diagnose dementia on the usual clinical grounds in mental defectives down to an IQ of around 25. Serial EEG recordings were helpful in cases where there were diagnostic problems. To date the neuropathological -findings in the patients who have died since the survey was carried out have confirmed the clinical diagnosis. In some of the patients affective symptoms were prodromal features of the dementing process. Mongols, in particular, seemed to manifest the clinical features of a premature form of senile dementia with disproportionate frequency. Mongols are known to manifest the neuropathological changes of Alzheimer's disease with unusual frequency as they get older (Burger & Vogel 1973) and develop epilepsy with advancing age (Veall 1974). They also show premature tissue ageing in the aortic and pulmonary valves (Sylvester 1974). Ageing in mongols would therefore seem to be a pathological process and a fruitful area for intensive gerontological research.
Affective and schizophrenic psychoses were found to occur in patients whose mental retardation was of varying etiologies, including sex chromosomal and autosomal abnormalities, cerebral diplegia and brain damage, as well as in patients in whom the etiology of the mental retardation could not be specified. Blindness and deafness occurred in around 40% of the patients with paranoid syndromes. Overall in the group of patients with affective and schizophrenic psychoses the prevalence of epilepsy was about 25 %. Epilepsy is, however, common in mental defectives and this prevalence rate is almost identical to the figure reported by Penrose in the Colchester survey (1938). Reid & Aungle (1974) then proceeded to study the problem of dementia in mental defectives, in particular senile, cerebral arteriosclerotic and presenile dementia. It is now quite widely recognized that with the greater life expectancy of the mentally defective the problems of old age are assuming progressively more importance in this field. Even so there was hardly a reference in the literature to the topic. The first author therefore
Case 4 This patient was a low-grade translocation mongol with a 46 XY 21/22 chromosome abnormality. He used to be able to carry on a simple conversation, sing (tunelessly), feed and dress himself. He went blind and deaf in his late teens and developed grand mal epilepsy around the age of 40. For many years he was clean in his habits and able to find his way round the ward by touch. About three years prior to his death he became withdrawn and unsociable and underwent what appeared to be a personality change. He started to sleep poorly and began to get up at night. Soon after he became doubly incontinent,-progressively less capable of fending for himself and terminally he began to eat rubbish. He was diagnosed in life as suffering from a senile dementia of unusually early onset and he died at the age of 53. At autopsy he was found to have purulent bronchitis and bronchopneumonia. The cesophagus was obstructed by a mass of rolled up paper and there were foreign bodies in the stomach. At neuropathological examination the brain weighed 1100 g and showed severe generalized gyral atrophy with opening up of both sylvian fissures. Coronal sectioning of the cerebrum confirmed the atrophy and revealed symmetrical dilatation of the lateral and third ventricles. Changes characteristic of severe Alzheimer's disease were present in all sections of cerebral cortex examined microscopically (details on request). This patient illustrates the feasibility of diagnosing dementia in quite severely defective patients given the presence of adequate data about premorbid functioning. EEG examinations in this mongol were helpfu', showing progressively increasing amounts of slow activity.
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Proc. roy. Soc. Med. Volume 69 July 1976
16
So far no reference has been made to prevalence rates. Hospital-based prevalence rates can be misleading, particularly for conditions such as affective psychosis which are clinically manifest only intermittently and which form only a small part of the psychiatric pathology encountered. A high prevalence rate for affective or schizophrenic psychosis in one hospital as opposed to another may imply no more than different admission criteria or a different distribution of psychiatrically disturbed patients as between the mental illness and the mental deficiency services in a region. Moreover hospital-based prevalence rates do not apply to defectives living at home. The prevalence rates of 1.2 % for affective psychosis and 3.2 % for schizophrenic and paranoid psychoses reported by Reid (1972) have to be seen in this perspective. The prevalence rate of 7.1 % for dementia reported by Reid & Aungle (1974) refers only to the rate amongst a population in hospital aged 45 and over. These prevalence rates take no account of nonpsychotic disorders which are much more numerous, nor do they include psychotic disorders which do not fall into the recognized diagnostic categories of functional and organic psychoses. Given these limitations the rates quoted are broadly in line with those reported by Heaton-Ward (1976, personal communication). Ballinger & Reid (in preparation) have attempted to study prevalence rates for both psychotic and nonpsychotic psychiatric disorder amongst a hospital-based group of adult defectives, as compared with a group of defectives living at home and attending an adult training centre. The preliminary results of this investigation have shown surprisingly little evidence of significant psychiatric disorder in the community based group, confirming to some extent the view expressed by Oliver (1975) that the main direct treatment contribution of the consultant psychiatrist working with adult mental defectives lies with the population in hospital. This is only to be expected since most mental deficiency hospitals now admit selectively patients who are psychiatrically disturbed or physically dependent, and seek alternative placements in the community for the more numerous well-behaved adult defectives who can be assimilated into local authority social work provision. It should be noted, however, that Ballinger & Reid did not attempt in their survey to quantify the psychiatric problems within the family associated with the presence of the defective at home: such problems are frequently of considerable significance and should form a substantial part of the work load of the consultant psychiatrist outwith the hospital (Sykes 1975).
In this paper the author has considered only the applicability of the presently recognized clinical psychiatric concepts to adult mental defectives. This obviously omits substantial areas of psychiatric pathology. There are, particularly among the more severely defective, many patients in whom it is possible to agree on the presence of considerable psychiatric disorder but whose illnesses are very difficult or impossible to classify within the existing diagnostic framework. There is a need for much more research into the diagnosis and natural history of psychiatric disorder in these patients amongst whom one meets problems relating to aggressiveness, hyperkinesis, pathological social withdrawal and 'grown up' cases of infantile and childhood disintegrative psychoses to name but a few. There is also the real possibility that such research might usefully extend our understanding of the neurological, neurophysiological and neuropathological factors affecting behaviour, and our treatment options.
Acknowledgments: I am grateful to my colleagues in Dundee for their help with these investigations and also to Dr A F J Maloney, Neuropathology Laboratory, Royal Infirmary, Edinburgh, for post-mortem brain examinations. REFERENCES Adams G L, Kivowitz J & Ziskind E (1970) Archives of GeneralPsychistry 23, 305-309 Batchelor I R C (1964) Proceedings of the Royal Society ofMedicine 57, 417-419 Burger P C & Vogel F S (1973) American Journal ofPathology 73, 457-468 Forssman H & Wilinder J (1969) Acta psychiatrica Scandinavica Supplement 207, 34-40 Hayman M (1939) Journal of Mental Science 85, 1183-1193 Naylor G J, Donald J M, Le Poidevin D & Reid A H (1974) British Journal ofPsychiatry 124, 52-57 Naylor G J, Reid A H, Dick D A T & Dick E G (1976) British Journal ofPsychiatry 128, 169-180 Oliver B E (1975) Journal of Mental Subnormality 21, 115-116 Penrose L S (1938) Special Report Series of the Medical Research Council (London) No. 229 Reid A H (1971) MD Thesis, Dundee (1972) British Journal ofPsychiatry 120, 205-218 Reid A H & Aungle P G (1974) Journal ofMental Deficiency Research 18, 15-23 Reid A H &Naylor G J (1976) Journal of Mental Deficiency Research 20, 67-76 Sykes D P (1975) Journal of Mental Subnormality 21, 3-5 Sylvester P E (1974) Journal of Mental Deficiency Research 18, 367-376 Veall R M (I974) Journal of Mental Deficiency Research 18, 99-106
Dr W A Heaton-Ward (Stoke Park Hospital, Bristol) also took part in the meeting. His remarks were based on his recent Blake Marsh Lecture (see British Journal of Psychiatry, 1976, in press).
