SEMINAKS I N NEUROLOGY-VOLUME
10, N O . 3
SEPTEMBER 1990
Psychiatric and Cognitive Aspects of Multiple Sclerosis Egon Stenager, M.D., Lone Knudsen (Neuropsycholopt), and Knud Jensen, M.D., Ph.D.
PSYCHIATRIC DISORDERS FREQUENCY
Although psychiatric disorders are often seen in MS, they are certainly not present in all cases. The few investigations that deal specifically with their frequency are not comparable because of lack of standardized criteria in patient selection and inadequate methods of diagnostic classification. There are, however, some common features. In cross-sectional investigations of large MS populations in the United States, Israel, and Denmark, it has been found that 12 to 25% of the patients have been admitted to a psychiatric service or have had similar contact with a psychiatric care ~ y s t e mFur.~ thermore, 50 to 70% of MS patients consecutively admitted to a neurologic service have had mental disorder^.^-^ It may be expected, therefore, that about one quarter of MS patients will require admission to a psychiatric department at some point in their illness and that about two thirds will be found to have a psychiatric disorder, to a varying degree. In some cases, this is the first sign of MS. There is sometimes a danger that other symptoms may be overlooked when the mental disturbance is more obvious than the neurologic signs,' a situation that is more likely to occur in areas of low risk for the disease. Some MS patients have reported that the onset of the disease occurred in connection with mental or physical trauma. It has been found that, com-
Clinical Neuropsychiatric Research Unit, Odense University Hospital, Odense, Denmark Reprint requests: Dr. Stenager, Clinical Neuropsychiatric Research Unit, Odcnse University Hospital, DK 5000 Odensc C, Denmark Copyright 1990 by Thieme Medical Publishers, Inc., 381 Park Avenue South, New York, N l ' 10016. All rights reserved.
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In the more than 150 years that have passed since R. Carswell (1783-1853) and J. Cruveillier (179 1-1 874) formulated the first descriptions of a disease entity that came to be known as multiple sclerosis (MS), numerous investigations have been made of the mental disorders that are seen with the disease.' It is now known that some of Carswell's and Cruveillier's literary contemporaries had M S for example, Augustus d'Est6 ( 1774-1 848) who developed mental symptoms in the form of emotional incontinence, "minor depression," and euphoria,' and the German poet Heinrich Heine (1797-1856), who, until his death, remained intellectually and mentally intact in spite of the disease.* It is not surprising that a span from mental intactness to severe mental disturbances, associated with a disease characterized by widespread lesions in the central nervous system (CNS), has been an incitement to investigation. A historical landmark was reached with the introduction of neuropsychologic test methods in the 1950s, although their comparability was always doubtful. In recent decades, new perspectives have been opened u p with the introduction of an objective technique, magnetic resonance imaging (MRI), which permits location of the pathologic lesions in MS with much greater precision than previously. I n this article, we give an overview of the present state of knowledge concerning the psychiatric and cognitive disorders seen in MS, with particular focus on the progress that has been made since the last overview appeared in this journal."
pared with a healthy control group, MS patients which intensify with exacerbation and are less experience more stress in their lives in the 6 often seen when the physical handicap abates.I5 months preceding the onset of the disease. 'This Young people often develop MS at a stage in life finding has given rise to speculation that onset or when they are making plans for the future, and relapse is a consequence of an unstable neuroim- disability often necessitates crucial changes in their munologic system that is sensitive to such s t r e ~ s e s . ~social circumstances. Employment and education Attempts have been made in a number of plans, relationships with family and friends, stable studies to establish whether there is a premorbid partnerships, and plans for having children are afpsyche or personality that is specific to MS, as the fected. It is therefore to be expected that reactive patients often are found to have psychopathologic depressions are frequent. conditions on neurologic admission. The hypothWhen describing depressive symptoms in MS esis, however, has never been ~ e r i f i e d With .~ re- patients, some authors have, however, found a gard to the type of psychiatric disease found in MS strong correlation between MS and manic-deprespatients, only a few authors have studied neurosis, sive disease, even with the bipolar form. T h e since symptoms of affective disease and psychosis depression increases in severity with high disease have received more attention. activity.l5,I6 he risk of depression is greatest if a patient, NEUROSIS prior to onset of MS, has a history of depression In daily clinical work, MS patients in the early or has undergone treatment with prednisone or that can also precipitate mania in stages of the disease are sometimes suspected of ACTH,'"rugs MS" as well as in diseases such as rheumatoid arhaving hysteria. Surprisingly, little has been writthritis. ten about this. The first description deals characThere is incomplete agreement on the extent teristically enough with mistakenly diagnosing MS to which MS patients are more predisposed to depatients as hysterical'" and this still happem7 Howpressive disorders than healthy person^.^,'^ However, it has been concluded in later investigations ever, it seems that MS patients with bipolar manicthat there are no more cases of hysteria among MS depressive disease have significantly more relatives patients than among patients with other diseases.I1 who have an affective disorder. Furthermore, it It has, as mentioned, been impossible to find seems that considerably more women have depresevidence of a premorbid psyche or personality that sion, a difference that cannot be explained by the is characteristic for MS patients. At the outbreak of the disease, there is a tendency for MS patients to predominance of women as against men who have become more preoccupied with physical symptoms the disease.18 Conceding that the frequency of affective disthan healthy persons, but there is no evidence that orders among MS patients is above average, it neurosis is more frequent in MS patients.I2 Therecould be expected that this would also apply to the fore diagnosis of a neurosis at the onset of MS frequency of suicide. Surprisingly, no thorough inshould be made'with caution, although this is a vestigation of that aspect has been undertaken. It mistake frequently made when these patients try to is known, however, that suicides occur and that describe their symptoms to the physician. those carried out occur concurrently with exacerbation of MS or in connection with severe psychiDEPRESSION atric or social consequences arising from it.19 Cross-sectional investigations have demonstrated that depressive symptoms are the psychiatric symptoms most frequently present in MS.5,12,13 The life"MINOR DEPRESSION" time risk is approximately 40 to 50%.12,13 They may be present from onset, but most often they become Some authors recognize "minor depression" as more obvious in the course of the disease, with pro- a syndrome not strongly correlated to demyelinagression of the physical handicap.14 tion16or to cognitive impairment." However, noneIn recent years, research on the depressive theless, some have interpreted the depressive sympsymptoms in MS has been intensified, and our toms as being a sign of cognitive impairment, and knowledge of their character has expanded. Their emphasize that depressive symptoms first become etiology has been discussed in a number of papers significant when the cognitive impairment is probut the debate usually focuses mainly on the pos- n o ~ n c e d . It ~ ' is still controversial whether depressibility of organic etiology. sion in MS patients should be recognized as being There is general agreement that disease activ- of organic etiology, but there is no doubt that reity causes the development of depressive symptoms, active depressions are frequently present.
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PSYCHIATRIC ASPECTS OF MULTIPLE SCLEROSIS-STENAGER,KNUDSEN,JENSEK
SEMINARS I N NEUROLOGY
Euphoria in MS patients has been reported in numerous publications. It has been stated that MS patients frequently behave as if they did not realize the seriousness of their physical handicap, and their behavior was fatuous and uncritical. It has been suggested that euphoria is the result of structural brain damage, and in recent publications it has been described in connection with advanced dementia.22There is no doubt, however, that the presence of euphoria in MS patients has been exaggerated. Usually, it is not present in the early stages of the disease when most patients are faced with its implications and when depressions, as can be expected, are frequent.
neurologist. As with other diseases, some MS patients are very precise in their description of their cognitive dysfunction, whereas others are less exact or perhaps talk about them only on close questioning. Generally, both the patient and the family are aware that changes take place and draw attention to them through one or more of the following complaints: The patient tires more easily; thinks and works more slowly than earlier; has less initiative and is more difficult to motivate; is anxious and periodically sad; is more easily moved to tears; has concentration and memory problems; is uncertain of himself; has reading problems; has ceased to read newspapers, journals and books; is unable to read T V subtitles. Such complaints should always lead the clinican to consider cognitive dysfunction. NEUROPSYCHOLOGZC FINDINGS
COGNITIVE DEFICITS For 40 years, neuropsychologic investigations have been used in the assessment of MS patients. T h e first studies mainly focused on measurement of the intelligence quotient (IQ). Later on, it was found more relevant to examine specific functions, such as memory, measured by the patient's ability to process material read aloud to him and material read by him. Furthermore, the ability to learn and to store new material, concentration on a work procedure, reaction time, language, and frontal lobe functions were also tested. FREQUENCY OF COGNZTNE IMPAIRMENT
It is very difficult to measure the frequency of cognitive deficits, mainly because the various neuropsychologic schools use different tests. In some cases comparison of the investigations is impossible because the patient material is not representative. To date, only one investigation has used representative materiaL2"n spite of these reservations, major investigations agree that cognitive deficits are present in 60 to 70% of their patients."24 The functions most effectively assessed are memory and speed of information processing. Several investigations have shown that roughly one third of patients have intact cognitive function, one third have slight to moderate deficits, and one third have severe deficits. We conclude that almost all patients with definite MS have reduced speed of information proce~sing.'~ SYMPTOMS
It is surprising that no investigations have been carried out in which the patient's subjective complaints of cognitive impairment are described and analyzed, even though they are familiar to the
It is important for the clinician to realize that, in spite of long experience, he can expect to observe only about 50% of the significant cognitive deficits.25The patient's family is often more capable of assessing them than the patient himself.24 Often slight to moderate cognitive deficits are overlooked; the physician must pay attention to the complaints made and use caution when interpreting them. Decline of memory and concentration is present at a very early stage of the disease, even in mild forms."','b T h e physical handicap is an important predictor of decline in memory and concentration, since deterioration increases with progression in the although it is quite possible to find a severely disabled patient who is cognitively intact.'" Sometimes MS patients with modest neurologic symptoms have severe cognitive deficits, which are often characterized by "prominent frontal release signs."28 Slowing of information-processing speed may be seen in patients with only slight physical handicap; the two may progress in parallel. Motor skills have often been used to measure concentration, since it was assumed that any deficiency was a consequence of the MS patient's motor defects. However, there is now agreement that concentration deficiency is a distinct phenomenon that can be measured without reference to motor fac~lties.~"'" Reaction time may become longer in an MS patient because of exhaustion, although there is no difference from normal in ability to solve tasks of increasing complexity.") Deterioration in memory is frequently seen." Both visual and verbal memory decline with progression of physical handicap.'Wecline in ability to learn new materia12%nd to apply material learned earlier takes place."2 It is important to remember in assessing these data that
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E UPHORZA
V O L U M E 10, NUMBER 3 SEPTEMBER 1990
PSYCHIATRIC ASPECTS OF MULTIPLE SCLEROSIS-STENAGER,KNUDSEN, JENSEN
CONSEQUENCES OF COGNITNE DEFICITS
Reliable investigations of cognitive deficits in MS were first carried out in recent years; their consequences have not attracted much attention until recently. Studies now confirm the earlier empirical experience that cognitive deficits complicate the adjustment of the MS patient to physical handicap. Intelligent and well-preserved MS patients can compensate more easily for the effect of the physical handicap on their ability to cope with daily requirement~."."~ There is the tendency for some patients, especially those with impairment of the frontal lobes, to overestimate their own strength and ability to work. Family members, nurses, and physiotherapists often report that they manage less well than they ~ l a i m . ~ " In other cases the physical handicap requires considerable adjustment because of the patient's lack of self-confidence, often resulting in less appropriate social adaptation and fewer social contacts. It is dificult to explain the precise connection between these problems and cognitive deficits, but it is important to stress that they must be taken into account in physical training and rehabilitation, so that demands made on the patient are within his competence. OTHER PSYCHIATRZC DISORDERS
In rare cases, a paranoid psychosis is seen. In an investigation of 117 MS patients, only two were
found to have paranoid delusion^.^" Strangely enough, some researchers in schizophrenia have pointed out the possibility that schizophrenia and MS may be similar disease entities because of parallels in course, age at onset, geographic spread, and immunologic changes40 Other "psychiatric" symptoms-for example, emotional incontinence and compulsive weeping and laughter-are rather common.*' Usually they occur only episodically.
NEUROPHYSIOLOGIC STUDIES MAGNETIC RESONANCE IMAGING
This new technology should, in the near future, produce information that will broaden our understanding of the mental associations of MS.42 In one of the earliest MRI investigations eight MS patients were examined. Those with mental disorders had more lesions in the temporal lobes than those In a follow-up investigation of 76 patients with isolated lesions, no correlation was found between lesions revealed by MRI and mental morbidity. On the contrary, social tensions were believed to be a more realistic explanation of the patients' mental symptoms.44 MRI scanning has proven more rewarding in the elucidation of cognitive impairment. It has been found that patients with severe cognitive deficits have more lesions than patients with slighter d e f i ~ i t s , although ~ ~ - ~ ~ there is still some disagreement.49 Some authors have found that the total number of lesions correlates with the decline in short-term memory, abstractlconceptual reasoning, and visuospatial problem ~olving.~" Understanding of the organic substrate for the mental symptoms associated with MS will soon expand greatly, but the investigations made so far must be taken as preliminary. With regard to correlation between specific cognitive functions and anatomic localization, reduction in information processing speed correlates with atrophy of corpus c a ~ ~ 0 s u m ~ ~ & 4 ~Lesions .~o on MRI correlate with anterograde memory defect and demyelination in the hippocampal region.jl There is a reported MRI correlation with I Q defect, defective auditory attention, and disease duration,41but not between white matter and cortical atrophy and dementia.4" OTHER EXAMINATIONS
Immunologic data have been correlated with mental symptoms. It was found that patients with most T 4 helperlinducer cells displayed greatest stress measured by an anxietyldepression ~ c a l e . ~ ' Cerebral blood flow studies have been carried out in an effort to improve our knowledge of cogni- 257
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methodologic problems are inherent in some of the tests used, as for example in assessment of visual memory.33 Intelligence tests may show that the patient's premorbid intelligence was normal, but in the advanced stage of MS lower I Q scores may be found."^'^ In daily clinical work the impression may often be that MS patients have impairment of the frontal lobes.'Wowever, there is no verification that such impairment is specific to MS.34 Neuropsychologic examination of MS patients is useful to assess the difference between "cortical" and "subcortical" dementia. Subcortical dementia is in particular characterized by forgetfulness, slowness, apathy, and depression, while cortical dementia is characterized by amnesia, aphasia, agnosia, and apraxia. Signs of both subcortical (as in Huntington's disease) and cortical dementia (as in Alzheimer's disease) are found in MS patient^.^' Alzheimer's dementia is more severe than MS dementia, and especially affects learning skills, memory, and verbal ability." The exact kind of dementia present is not always r e ~ o g n i z a b l e . ~ T sensitivity he of the tests used and severity of the MS should be kept in mind.
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tive deficits. Reduced cerebral blood flow has been connections with family and friends, stable partfound, but the conclusion is that the phenome- nerships, marriage plans, and having children. It non is probably secondary to cerebral dysfunction is important to support the patient and his family, caused by MS and that changes in blood flow first and talk with them about all the problems that occur when cognitive impairment is p r o n ~ u n c e d . ~ "must be tackled. Counsel on partnerships and marUsing the electroencephalogram, attempts have riage and the implications of the disease must be been made to determine the effect of MS on the given and help provided in fairly assessing the electrophysiologic environment, but results are as depth of the patient's own feelings. Is he or she yet preliminary" and have no practical significance strong and sincere enough to embark on marriage in clinical work. However, it is very likely that cor- and perhaps to start a family? Aspects that require relation of neurophysiologic examinations with careful counseling follow. positron emission tomography (PET) and singleSexual dysfunction. Sexual problems are frephoton emission computed tomography (SPECT) quent for MS patients of' both sexes and help and scanning will increase our knowledge about the advice should be given when at all possible. For pathologic lesions in the brain and their relation to men, practical information concerning papaverine injections and the possibility of surgical intervenmental symptoms and cognitive deficits. tion in the form of a penile prosthesis" can prove helpful in cases of erectile dysfunction. Pregnancy. When the onset of the disease is at a young age, advice on the prospects involved in TREATMENT OF MENTAL DISORDERS marriage, starting a family, and on having children OF MS is essential. Our investigations indicate that women All treatment offered the MS patient today is with MS who become pregnant and give birth besymptomatic and is concentrated largely on the come more physically handicapped than those who neurologic ~ ~ m p t o m s . ~ Most ~ - ~literature ' makes do not." On the other hand, MS women with chilno reference to treatment of the psychiatric o r cog- dren seem to be less prone to cognitive deficiency nitive diseases seen in connection with the disease. than those without children." It could be that the demands of having children constitute a safeguard against cognitive impairment, as is sometimes seen PSYCHIATRIC DISEASES in elderly couples when one dies and the surviving Depression in patients with MS should be partner soon after becomes clearly demented. treated as in other cases of depression. The MS pa- However, it is important that a young woman with tient with manic-depressive disease has the same MS who desires to have children is prepared for benefit from antidepressants as other patients, and the risks involved, and that her partner is made lithium is not contraindicated. MS patients with re- aware of the risk of deterioration in the patient's active depression should also be treated in the condition and of the extra burden it would mean same way as others with the disorder, utilizing a for him.6n combination of psychotherapy and psychotropic When should patients with M S be told their diagmedications. nosis? The diagnosis should surely be told and careIt should be stressed that prophylaxis of reactive fully explained to the patient as soon as the neudepression is very important and with better coun- rologist knows it, but at a point when he is seling of the MS patient, many of these depressions prepared to take the time needed to advise the pacan be avoided. They are provoked by the drastic tient on its consequences while extending his supchanges that inevitably take place in the life of the port. There is a tremendous need for support in MS patient and therefore frequently occur in the the early stages, when the patient feels helpless and early stages of the disease at the first sign of phys- dreads a long life in a wheelchair.'" ical handicap. MS is often contracted at an early Many aspects must be handled with empathy, age, and these patients face tremendous problems particularly when the family or marriage is inas they see all their plans and dreams for the future volved, and those concerned must be drawn into fall apart. talks about the future. Counseling is the only form of prophylaxis that can be practiced, and it is esCOUNSELING sential that it be available if depression is to be avoided and the risk of' suicide diminished. That Social Problems risk must be kept in mind, especially at times of The MS patient faces many difficulties when exacerbation of the disease, in conjunction with plans for the future must be revised. This applies which increased psychosocial problems are apt to not only to education and employment, but also to arise or increase.Ig
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SEMINARS I N NEUROLOGY
PSYCHIATRIC ASPECTS O F MULTIPLE SCLEROSIS-STENA~ER,KNUDSEN, JENSEN
Patients showing signs of cognitive impairment have the same need to communicate their fear and receive supportive advice. When the initial signs are observed, the patient must be helped to see the impairment in the context of disease and social situation, so that both the patient and his family realize the implications. In addition, the patient must be assisted in carrying out a reasonable life plan, reasonable dispositions, for example, with regard to work,h' education, pastimes, and future, all of which are aspects of life that often preoccupy the MS patients and their families when acknowledgment has been reached of the considerable limitations of the treatment that can be offered. This takes time, and many consultations are necessary. Having recognized the implication of cognitive impairment, the patient can be helped with practical information. 'I'hose with memory impairment with reduced visual learning often have difficulty in remembering directions and complex tasks. It is therefore important to use oral instructions and checklists instead of practical demonstrations. On the other hand, patients who show impaired verbal learning with memory impairment can best be helped by demonstrations, whereas checklists are not of much use.
Education and Rehabilitation Depending on the severity of cognitive impairment, the MS patient can be trained to learn new material, but learning speed is slower than prior to the illness. Training should be adjusted to the ability of the individual and focus only on subjects of interest to the patient. Counseling is also essential in such cases, and in particular when considering change of workplace or work process. Families can, with advantage, be involved in considerations concerning such changes, since MS patients at times overestimate their own potential. As already mentioned, MS affects not only the patient but also family life. T h e family should therefore always be involved in decision-making processes. If there is to be a good relationship with the patient, the family must know as much as possible about the disease and the chances of remission or exacerbation. It is important that neither patient nor family is left with unanswered questions as far as possible. The patient's social interaction and quality of life depend to a great extent on how appropriate the counseling given in the early stages of the disease is and how well it is followed up. Treatment of the
MS patient has for the most part concentrated on symptomatic treatment of the various neurologic signs and symptoms, without sufficient appreciation of the necessity for the patient and the family to have information that is relevant to their situation and that would help them to avoid crises. Knowledge of the disease and proper counseling is necessary, if these patients are to achieve an optimal quality of life.
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10, N O . 3
SEPTEMBER 1990
Psychiatric and Cognitive Aspects of Multiple Sclerosis Egon Stenager, M.D., Lone Knudsen (Neuropsycholopt), and Knud Jensen, M.D., Ph.D.
PSYCHIATRIC DISORDERS FREQUENCY
Although psychiatric disorders are often seen in MS, they are certainly not present in all cases. The few investigations that deal specifically with their frequency are not comparable because of lack of standardized criteria in patient selection and inadequate methods of diagnostic classification. There are, however, some common features. In cross-sectional investigations of large MS populations in the United States, Israel, and Denmark, it has been found that 12 to 25% of the patients have been admitted to a psychiatric service or have had similar contact with a psychiatric care ~ y s t e mFur.~ thermore, 50 to 70% of MS patients consecutively admitted to a neurologic service have had mental disorder^.^-^ It may be expected, therefore, that about one quarter of MS patients will require admission to a psychiatric department at some point in their illness and that about two thirds will be found to have a psychiatric disorder, to a varying degree. In some cases, this is the first sign of MS. There is sometimes a danger that other symptoms may be overlooked when the mental disturbance is more obvious than the neurologic signs,' a situation that is more likely to occur in areas of low risk for the disease. Some MS patients have reported that the onset of the disease occurred in connection with mental or physical trauma. It has been found that, com-
Clinical Neuropsychiatric Research Unit, Odense University Hospital, Odense, Denmark Reprint requests: Dr. Stenager, Clinical Neuropsychiatric Research Unit, Odcnse University Hospital, DK 5000 Odensc C, Denmark Copyright 1990 by Thieme Medical Publishers, Inc., 381 Park Avenue South, New York, N l ' 10016. All rights reserved.
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In the more than 150 years that have passed since R. Carswell (1783-1853) and J. Cruveillier (179 1-1 874) formulated the first descriptions of a disease entity that came to be known as multiple sclerosis (MS), numerous investigations have been made of the mental disorders that are seen with the disease.' It is now known that some of Carswell's and Cruveillier's literary contemporaries had M S for example, Augustus d'Est6 ( 1774-1 848) who developed mental symptoms in the form of emotional incontinence, "minor depression," and euphoria,' and the German poet Heinrich Heine (1797-1856), who, until his death, remained intellectually and mentally intact in spite of the disease.* It is not surprising that a span from mental intactness to severe mental disturbances, associated with a disease characterized by widespread lesions in the central nervous system (CNS), has been an incitement to investigation. A historical landmark was reached with the introduction of neuropsychologic test methods in the 1950s, although their comparability was always doubtful. In recent decades, new perspectives have been opened u p with the introduction of an objective technique, magnetic resonance imaging (MRI), which permits location of the pathologic lesions in MS with much greater precision than previously. I n this article, we give an overview of the present state of knowledge concerning the psychiatric and cognitive disorders seen in MS, with particular focus on the progress that has been made since the last overview appeared in this journal."
pared with a healthy control group, MS patients which intensify with exacerbation and are less experience more stress in their lives in the 6 often seen when the physical handicap abates.I5 months preceding the onset of the disease. 'This Young people often develop MS at a stage in life finding has given rise to speculation that onset or when they are making plans for the future, and relapse is a consequence of an unstable neuroim- disability often necessitates crucial changes in their munologic system that is sensitive to such s t r e ~ s e s . ~social circumstances. Employment and education Attempts have been made in a number of plans, relationships with family and friends, stable studies to establish whether there is a premorbid partnerships, and plans for having children are afpsyche or personality that is specific to MS, as the fected. It is therefore to be expected that reactive patients often are found to have psychopathologic depressions are frequent. conditions on neurologic admission. The hypothWhen describing depressive symptoms in MS esis, however, has never been ~ e r i f i e d With .~ re- patients, some authors have, however, found a gard to the type of psychiatric disease found in MS strong correlation between MS and manic-deprespatients, only a few authors have studied neurosis, sive disease, even with the bipolar form. T h e since symptoms of affective disease and psychosis depression increases in severity with high disease have received more attention. activity.l5,I6 he risk of depression is greatest if a patient, NEUROSIS prior to onset of MS, has a history of depression In daily clinical work, MS patients in the early or has undergone treatment with prednisone or that can also precipitate mania in stages of the disease are sometimes suspected of ACTH,'"rugs MS" as well as in diseases such as rheumatoid arhaving hysteria. Surprisingly, little has been writthritis. ten about this. The first description deals characThere is incomplete agreement on the extent teristically enough with mistakenly diagnosing MS to which MS patients are more predisposed to depatients as hysterical'" and this still happem7 Howpressive disorders than healthy person^.^,'^ However, it has been concluded in later investigations ever, it seems that MS patients with bipolar manicthat there are no more cases of hysteria among MS depressive disease have significantly more relatives patients than among patients with other diseases.I1 who have an affective disorder. Furthermore, it It has, as mentioned, been impossible to find seems that considerably more women have depresevidence of a premorbid psyche or personality that sion, a difference that cannot be explained by the is characteristic for MS patients. At the outbreak of the disease, there is a tendency for MS patients to predominance of women as against men who have become more preoccupied with physical symptoms the disease.18 Conceding that the frequency of affective disthan healthy persons, but there is no evidence that orders among MS patients is above average, it neurosis is more frequent in MS patients.I2 Therecould be expected that this would also apply to the fore diagnosis of a neurosis at the onset of MS frequency of suicide. Surprisingly, no thorough inshould be made'with caution, although this is a vestigation of that aspect has been undertaken. It mistake frequently made when these patients try to is known, however, that suicides occur and that describe their symptoms to the physician. those carried out occur concurrently with exacerbation of MS or in connection with severe psychiDEPRESSION atric or social consequences arising from it.19 Cross-sectional investigations have demonstrated that depressive symptoms are the psychiatric symptoms most frequently present in MS.5,12,13 The life"MINOR DEPRESSION" time risk is approximately 40 to 50%.12,13 They may be present from onset, but most often they become Some authors recognize "minor depression" as more obvious in the course of the disease, with pro- a syndrome not strongly correlated to demyelinagression of the physical handicap.14 tion16or to cognitive impairment." However, noneIn recent years, research on the depressive theless, some have interpreted the depressive sympsymptoms in MS has been intensified, and our toms as being a sign of cognitive impairment, and knowledge of their character has expanded. Their emphasize that depressive symptoms first become etiology has been discussed in a number of papers significant when the cognitive impairment is probut the debate usually focuses mainly on the pos- n o ~ n c e d . It ~ ' is still controversial whether depressibility of organic etiology. sion in MS patients should be recognized as being There is general agreement that disease activ- of organic etiology, but there is no doubt that reity causes the development of depressive symptoms, active depressions are frequently present.
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PSYCHIATRIC ASPECTS OF MULTIPLE SCLEROSIS-STENAGER,KNUDSEN,JENSEK
SEMINARS I N NEUROLOGY
Euphoria in MS patients has been reported in numerous publications. It has been stated that MS patients frequently behave as if they did not realize the seriousness of their physical handicap, and their behavior was fatuous and uncritical. It has been suggested that euphoria is the result of structural brain damage, and in recent publications it has been described in connection with advanced dementia.22There is no doubt, however, that the presence of euphoria in MS patients has been exaggerated. Usually, it is not present in the early stages of the disease when most patients are faced with its implications and when depressions, as can be expected, are frequent.
neurologist. As with other diseases, some MS patients are very precise in their description of their cognitive dysfunction, whereas others are less exact or perhaps talk about them only on close questioning. Generally, both the patient and the family are aware that changes take place and draw attention to them through one or more of the following complaints: The patient tires more easily; thinks and works more slowly than earlier; has less initiative and is more difficult to motivate; is anxious and periodically sad; is more easily moved to tears; has concentration and memory problems; is uncertain of himself; has reading problems; has ceased to read newspapers, journals and books; is unable to read T V subtitles. Such complaints should always lead the clinican to consider cognitive dysfunction. NEUROPSYCHOLOGZC FINDINGS
COGNITIVE DEFICITS For 40 years, neuropsychologic investigations have been used in the assessment of MS patients. T h e first studies mainly focused on measurement of the intelligence quotient (IQ). Later on, it was found more relevant to examine specific functions, such as memory, measured by the patient's ability to process material read aloud to him and material read by him. Furthermore, the ability to learn and to store new material, concentration on a work procedure, reaction time, language, and frontal lobe functions were also tested. FREQUENCY OF COGNZTNE IMPAIRMENT
It is very difficult to measure the frequency of cognitive deficits, mainly because the various neuropsychologic schools use different tests. In some cases comparison of the investigations is impossible because the patient material is not representative. To date, only one investigation has used representative materiaL2"n spite of these reservations, major investigations agree that cognitive deficits are present in 60 to 70% of their patients."24 The functions most effectively assessed are memory and speed of information processing. Several investigations have shown that roughly one third of patients have intact cognitive function, one third have slight to moderate deficits, and one third have severe deficits. We conclude that almost all patients with definite MS have reduced speed of information proce~sing.'~ SYMPTOMS
It is surprising that no investigations have been carried out in which the patient's subjective complaints of cognitive impairment are described and analyzed, even though they are familiar to the
It is important for the clinician to realize that, in spite of long experience, he can expect to observe only about 50% of the significant cognitive deficits.25The patient's family is often more capable of assessing them than the patient himself.24 Often slight to moderate cognitive deficits are overlooked; the physician must pay attention to the complaints made and use caution when interpreting them. Decline of memory and concentration is present at a very early stage of the disease, even in mild forms."','b T h e physical handicap is an important predictor of decline in memory and concentration, since deterioration increases with progression in the although it is quite possible to find a severely disabled patient who is cognitively intact.'" Sometimes MS patients with modest neurologic symptoms have severe cognitive deficits, which are often characterized by "prominent frontal release signs."28 Slowing of information-processing speed may be seen in patients with only slight physical handicap; the two may progress in parallel. Motor skills have often been used to measure concentration, since it was assumed that any deficiency was a consequence of the MS patient's motor defects. However, there is now agreement that concentration deficiency is a distinct phenomenon that can be measured without reference to motor fac~lties.~"'" Reaction time may become longer in an MS patient because of exhaustion, although there is no difference from normal in ability to solve tasks of increasing complexity.") Deterioration in memory is frequently seen." Both visual and verbal memory decline with progression of physical handicap.'Wecline in ability to learn new materia12%nd to apply material learned earlier takes place."2 It is important to remember in assessing these data that
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E UPHORZA
V O L U M E 10, NUMBER 3 SEPTEMBER 1990
PSYCHIATRIC ASPECTS OF MULTIPLE SCLEROSIS-STENAGER,KNUDSEN, JENSEN
CONSEQUENCES OF COGNITNE DEFICITS
Reliable investigations of cognitive deficits in MS were first carried out in recent years; their consequences have not attracted much attention until recently. Studies now confirm the earlier empirical experience that cognitive deficits complicate the adjustment of the MS patient to physical handicap. Intelligent and well-preserved MS patients can compensate more easily for the effect of the physical handicap on their ability to cope with daily requirement~."."~ There is the tendency for some patients, especially those with impairment of the frontal lobes, to overestimate their own strength and ability to work. Family members, nurses, and physiotherapists often report that they manage less well than they ~ l a i m . ~ " In other cases the physical handicap requires considerable adjustment because of the patient's lack of self-confidence, often resulting in less appropriate social adaptation and fewer social contacts. It is dificult to explain the precise connection between these problems and cognitive deficits, but it is important to stress that they must be taken into account in physical training and rehabilitation, so that demands made on the patient are within his competence. OTHER PSYCHIATRZC DISORDERS
In rare cases, a paranoid psychosis is seen. In an investigation of 117 MS patients, only two were
found to have paranoid delusion^.^" Strangely enough, some researchers in schizophrenia have pointed out the possibility that schizophrenia and MS may be similar disease entities because of parallels in course, age at onset, geographic spread, and immunologic changes40 Other "psychiatric" symptoms-for example, emotional incontinence and compulsive weeping and laughter-are rather common.*' Usually they occur only episodically.
NEUROPHYSIOLOGIC STUDIES MAGNETIC RESONANCE IMAGING
This new technology should, in the near future, produce information that will broaden our understanding of the mental associations of MS.42 In one of the earliest MRI investigations eight MS patients were examined. Those with mental disorders had more lesions in the temporal lobes than those In a follow-up investigation of 76 patients with isolated lesions, no correlation was found between lesions revealed by MRI and mental morbidity. On the contrary, social tensions were believed to be a more realistic explanation of the patients' mental symptoms.44 MRI scanning has proven more rewarding in the elucidation of cognitive impairment. It has been found that patients with severe cognitive deficits have more lesions than patients with slighter d e f i ~ i t s , although ~ ~ - ~ ~ there is still some disagreement.49 Some authors have found that the total number of lesions correlates with the decline in short-term memory, abstractlconceptual reasoning, and visuospatial problem ~olving.~" Understanding of the organic substrate for the mental symptoms associated with MS will soon expand greatly, but the investigations made so far must be taken as preliminary. With regard to correlation between specific cognitive functions and anatomic localization, reduction in information processing speed correlates with atrophy of corpus c a ~ ~ 0 s u m ~ ~ & 4 ~Lesions .~o on MRI correlate with anterograde memory defect and demyelination in the hippocampal region.jl There is a reported MRI correlation with I Q defect, defective auditory attention, and disease duration,41but not between white matter and cortical atrophy and dementia.4" OTHER EXAMINATIONS
Immunologic data have been correlated with mental symptoms. It was found that patients with most T 4 helperlinducer cells displayed greatest stress measured by an anxietyldepression ~ c a l e . ~ ' Cerebral blood flow studies have been carried out in an effort to improve our knowledge of cogni- 257
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methodologic problems are inherent in some of the tests used, as for example in assessment of visual memory.33 Intelligence tests may show that the patient's premorbid intelligence was normal, but in the advanced stage of MS lower I Q scores may be found."^'^ In daily clinical work the impression may often be that MS patients have impairment of the frontal lobes.'Wowever, there is no verification that such impairment is specific to MS.34 Neuropsychologic examination of MS patients is useful to assess the difference between "cortical" and "subcortical" dementia. Subcortical dementia is in particular characterized by forgetfulness, slowness, apathy, and depression, while cortical dementia is characterized by amnesia, aphasia, agnosia, and apraxia. Signs of both subcortical (as in Huntington's disease) and cortical dementia (as in Alzheimer's disease) are found in MS patient^.^' Alzheimer's dementia is more severe than MS dementia, and especially affects learning skills, memory, and verbal ability." The exact kind of dementia present is not always r e ~ o g n i z a b l e . ~ T sensitivity he of the tests used and severity of the MS should be kept in mind.
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tive deficits. Reduced cerebral blood flow has been connections with family and friends, stable partfound, but the conclusion is that the phenome- nerships, marriage plans, and having children. It non is probably secondary to cerebral dysfunction is important to support the patient and his family, caused by MS and that changes in blood flow first and talk with them about all the problems that occur when cognitive impairment is p r o n ~ u n c e d . ~ "must be tackled. Counsel on partnerships and marUsing the electroencephalogram, attempts have riage and the implications of the disease must be been made to determine the effect of MS on the given and help provided in fairly assessing the electrophysiologic environment, but results are as depth of the patient's own feelings. Is he or she yet preliminary" and have no practical significance strong and sincere enough to embark on marriage in clinical work. However, it is very likely that cor- and perhaps to start a family? Aspects that require relation of neurophysiologic examinations with careful counseling follow. positron emission tomography (PET) and singleSexual dysfunction. Sexual problems are frephoton emission computed tomography (SPECT) quent for MS patients of' both sexes and help and scanning will increase our knowledge about the advice should be given when at all possible. For pathologic lesions in the brain and their relation to men, practical information concerning papaverine injections and the possibility of surgical intervenmental symptoms and cognitive deficits. tion in the form of a penile prosthesis" can prove helpful in cases of erectile dysfunction. Pregnancy. When the onset of the disease is at a young age, advice on the prospects involved in TREATMENT OF MENTAL DISORDERS marriage, starting a family, and on having children OF MS is essential. Our investigations indicate that women All treatment offered the MS patient today is with MS who become pregnant and give birth besymptomatic and is concentrated largely on the come more physically handicapped than those who neurologic ~ ~ m p t o m s . ~ Most ~ - ~literature ' makes do not." On the other hand, MS women with chilno reference to treatment of the psychiatric o r cog- dren seem to be less prone to cognitive deficiency nitive diseases seen in connection with the disease. than those without children." It could be that the demands of having children constitute a safeguard against cognitive impairment, as is sometimes seen PSYCHIATRIC DISEASES in elderly couples when one dies and the surviving Depression in patients with MS should be partner soon after becomes clearly demented. treated as in other cases of depression. The MS pa- However, it is important that a young woman with tient with manic-depressive disease has the same MS who desires to have children is prepared for benefit from antidepressants as other patients, and the risks involved, and that her partner is made lithium is not contraindicated. MS patients with re- aware of the risk of deterioration in the patient's active depression should also be treated in the condition and of the extra burden it would mean same way as others with the disorder, utilizing a for him.6n combination of psychotherapy and psychotropic When should patients with M S be told their diagmedications. nosis? The diagnosis should surely be told and careIt should be stressed that prophylaxis of reactive fully explained to the patient as soon as the neudepression is very important and with better coun- rologist knows it, but at a point when he is seling of the MS patient, many of these depressions prepared to take the time needed to advise the pacan be avoided. They are provoked by the drastic tient on its consequences while extending his supchanges that inevitably take place in the life of the port. There is a tremendous need for support in MS patient and therefore frequently occur in the the early stages, when the patient feels helpless and early stages of the disease at the first sign of phys- dreads a long life in a wheelchair.'" ical handicap. MS is often contracted at an early Many aspects must be handled with empathy, age, and these patients face tremendous problems particularly when the family or marriage is inas they see all their plans and dreams for the future volved, and those concerned must be drawn into fall apart. talks about the future. Counseling is the only form of prophylaxis that can be practiced, and it is esCOUNSELING sential that it be available if depression is to be avoided and the risk of' suicide diminished. That Social Problems risk must be kept in mind, especially at times of The MS patient faces many difficulties when exacerbation of the disease, in conjunction with plans for the future must be revised. This applies which increased psychosocial problems are apt to not only to education and employment, but also to arise or increase.Ig
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SEMINARS I N NEUROLOGY
PSYCHIATRIC ASPECTS O F MULTIPLE SCLEROSIS-STENA~ER,KNUDSEN, JENSEN
Patients showing signs of cognitive impairment have the same need to communicate their fear and receive supportive advice. When the initial signs are observed, the patient must be helped to see the impairment in the context of disease and social situation, so that both the patient and his family realize the implications. In addition, the patient must be assisted in carrying out a reasonable life plan, reasonable dispositions, for example, with regard to work,h' education, pastimes, and future, all of which are aspects of life that often preoccupy the MS patients and their families when acknowledgment has been reached of the considerable limitations of the treatment that can be offered. This takes time, and many consultations are necessary. Having recognized the implication of cognitive impairment, the patient can be helped with practical information. 'I'hose with memory impairment with reduced visual learning often have difficulty in remembering directions and complex tasks. It is therefore important to use oral instructions and checklists instead of practical demonstrations. On the other hand, patients who show impaired verbal learning with memory impairment can best be helped by demonstrations, whereas checklists are not of much use.
Education and Rehabilitation Depending on the severity of cognitive impairment, the MS patient can be trained to learn new material, but learning speed is slower than prior to the illness. Training should be adjusted to the ability of the individual and focus only on subjects of interest to the patient. Counseling is also essential in such cases, and in particular when considering change of workplace or work process. Families can, with advantage, be involved in considerations concerning such changes, since MS patients at times overestimate their own potential. As already mentioned, MS affects not only the patient but also family life. T h e family should therefore always be involved in decision-making processes. If there is to be a good relationship with the patient, the family must know as much as possible about the disease and the chances of remission or exacerbation. It is important that neither patient nor family is left with unanswered questions as far as possible. The patient's social interaction and quality of life depend to a great extent on how appropriate the counseling given in the early stages of the disease is and how well it is followed up. Treatment of the
MS patient has for the most part concentrated on symptomatic treatment of the various neurologic signs and symptoms, without sufficient appreciation of the necessity for the patient and the family to have information that is relevant to their situation and that would help them to avoid crises. Knowledge of the disease and proper counseling is necessary, if these patients are to achieve an optimal quality of life.
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