The Medical Care of People With Intellectual Disability
Background: An estimated 1.5 million persons in Germany are intellectually disabled. Persons with intellectual disability (ID) are especially vulnerable to somatic and mental illnesses.
Methods: This review is based on pertinent literature retrieved by selective searches in PubMed and the Cochrane Library.
Results: Genetic abnormalities are a frequent cause of diseases that affect multiple organs and need interdisciplinary treatment. A number of somatic diseases are more common in persons with ID than in the general population, including epilepsy (30–50% in persons with severe or very severe ID, vs. 0.5% in the general population) and dementia (five times more common than in the general population). Patients with Down syndrome are 20 times more likely than the general population to develop acute lymphoblastic leukemia. Some mental illnesses, too, are more common in persons with ID, e.g., autism spectrum disorders (7.5–15% vs. 1% in the general population). The history and the findings of the physical and psychiatric examination are assessed in accordance with the biopsychosocial model of disease, and in the light of the patient’s mental developmental age. Structured instruments for behavioral evaluation and diagnosis are an important additional component of the diagnostic assessment. A holistic approach is required that takes multiple life areas into account and involves the patient’s familial and social environment, while obeying the rules of simple language. Psychotherapeutic and psychosocial measures must be adapted to the patient’s cognitive abilities and mental developmental age.
Conclusion: Intellectually disabled persons can be treated in a multimodal, multiprofessional approach. As of early 2019, there were 38 medical centers for adults with intellectual disability or severe multiple disabilities in Germany (Medizinische Behandlungszentren für Erwachsene mit geistiger Behinderung oder schweren Mehrfachbehinderungen, MZEB), where they can be cared for with due attention to their special needs.
Intellectual disability (ID) is defined as the result of a disturbance of intellectual development (1) and, therefore, must begin before the patient becomes an adult. Common synonyms for ID include cognitive impairment, mental impairment, intellectual disability. These persons’ IQ is significantly below the mean in the general population (more than 2 standard deviations below the norm, 1–2% of the population, ca. 1.5 million affected persons); they thus have difficulty in learning new and complex information or skills and in living independently (eTable 1).
The German Federal Statistical Office reported that, in 2017, 9.4% of the population (7.8 million persons) suffered from a severe disability of some kind, i.e., a degree of disability above 50%. In approximately one million persons, a disturbance of intellectual development or organic brain dysfunction was the sole or main type of disability present (2).
This review is based on pertinent publications retrieved by selective searches in the PubMed and Cochrane Library databases. The following search terms were used, among others: “intellectual (developmental) disability,” “somatic disorder,” “mental disorder,” “therapy,” and “diagnostics.”
Common genetic causes
Impaired intellectual development may be due to exogenous factors such as alcohol consumption (fetal alcohol spectrum disorder) or malnutrition during gestation, intrauterine or perinatal infection, obstetric complications, or metabolic disease, e.g., hypothyroidism. Learning impairment (IQ: 70–84) can also be caused, in part, by a lack of educational opportunities or attention from the individual’s social environment.
Chromosomal anomalies (mainly, new microdeletions) are found in some 20% of cases. Alongside monogenetic disturbances of dominant or recessive inheritance (ca. 30–40%), polygenetic disturbances have been described as well (3, 4). There are now 1222 known primary genes for ID that have been definitely clinically associated with a developmental disorder, and a further 1127 candidate genes with a possible association (5).
Increased morbidity and mortality
Mortality is three to four times higher than in the general population (6–8). In a study in Germany, ID was found to lower life expectancy by 6 to 12 years compared to the general population: 65 to 71 vs. 77 years for men, 70–73 vs. 82.5 years for women (9). The most common causes of death are respiratory diseases (especially pneumonia), cardiovascular diseases, and cancer (7, 8, 10). Aside from unpreventable conditions such as multimorbidity or neurodegenerative diseases, the preventable contributory causes of death include aspiration pneumonia (in persons with unrecognized dysphagia), avoidable falls and injuries, insufficient vaccination, lack of preventive care, and polypharmacy (6, 11–14).
The mental developmental age must be considered within the biopsychosocial model of disease
The history and the findings of the physical and psychiatric examination are supplemented with historical information from the main persons that interact with the patient in the major areas of his or her life. The basis of the evaluation is the biopsychosocial model of disease (Figure), extended by consideration of the patient’s emotional development. This model provides insight into the individual’s mental developmental age, which may be very different from his or her biological age, and which can be determined using a scale of emotional development (15–20). In the light of the individual’s emotional and cognitive stage of development, modes of behavior become intelligible and diagnostic systems in common international use can be applied (eFigure). For example, in a person with a mental reference age of about three years, oppositional defiant behavior may be developmentally appropriate and of no pathological significance.
Adaptation of the diagnostic assessment
Patients with ID can often give no more than a limited account of their own symptoms and may express their distress or pain in unusual ways (Box 1) ( 21). In general, any sudden change in the pattern of behavior should prompt suspicion of, and diagnostic evaluation for, an underlying somatic disease (Table 1) (22). Somatic distress, and pain in particular, often leads to aggression against others, or against the patient him- or herself (22); this can complicate the clinical assessment. In such cases, an imaging study under analgesia/sedation, or even under general anesthesia, may be needed and, if so, must be undertaken despite the additional cost and labor involved. Knowledge of the patient’s emotional reference age can be helpful in the interpretation and diagnostic classification of observed behavior patterns (23). Communication between the physician and the patient should obey the rules of simple language (24), and the physician should give the patient sufficient time to answer questions (the “6-second rule”) (Box 2) (25).
The operationalized diagnostic systems (DSM-5, ICD-10) are often of limited utility for persons with ID, because the disease manifestations must be interpreted against the background of their emotional and intellectual stage of development (Figure). The Royal College of Psychiatrists has adapted the ICD-10 criteria in its Diagnostic Criteria for Learning Disabilities (26). An overview of standardized examining techniques is given in eTable 2. These diagnostic instruments can be used to screen for certain suspected diagnoses and modes of behavior (e1–e23). Definitively establishing a diagnosis is the physician’s task; in complex situations involving, e.g., autism or dementia, the diagnosis may need to be determined in a multiprofessional case conference (27).
Common somatic diseases
Many of the genetically based disorders are mutiorgan diseases requiring treatment by specialists from multiple medical disciplines (Table 2). Pediatricians and pediatric neurologists have broad training, but adult patients generally need to be treated by multiple specialists, with the primary care physician coordinating the specialized consultations. For this purpose, specialized outpatient clinics are well-established in academic hospitals. In Germany, these patients can also be cared for in the newly founded medical centers for adults with disabilities (Medizinische Zentren für Erwachsene mit Behinderung, MZEB). Care requirements are highly disease-specific (e24, e25): adult Down syndrome patients suffer mainly from neurologic disturbances (e26), while those with tuberous sclerosis have neoplasias and epilepsy (e27), those with fragile X syndrome have mental abnormalities, and those with Curschmann–Steinert syndrome have sociomedical difficulties, respiratory disturbances, and cardiological problems (e28, e29). In general, the more severe the intellectual disability, the more intense the patient’s medical care needs to be.
The prevalence of epilepsy is 0.5% in the general population, 15% in persons with mild intellectual disability, and 30–50% in persons with severe to very severe intellectual disability (28). Mortality is elevated because of sudden death in epilepsy (SUDEP), seizure-related injuries, and an increased tendency to fall in persons taking antiepileptic drugs (28).
Persons with ID often suffer from pain of various causes that may go unrecognized, or is recognized only after a delay, because of the unusual presentation. Pain adversely affects sleep and quality of life in general (29, e30, e31). Table 1 contains information on a number of characteristic pain-related behavior patterns and their association with organ diseases (21, 30).
Chronic constipation is common in persons with ID and can take a severe course, e.g., with overflow coprostasis and pseudodiarrhea, potentially even resulting in death (31, e33). Meticulous rectal digital examination and laxative treatment can spare the patient an unnecessary diagnostic evaluation (31).
Gastro-esophageal reflux is more common with more severe disability (>50% in persons with IQ <35); risk factors include cerebral palsy, scoliosis, and anticonvulsive drugs. Sleep impairment, depressive mood disturbance, rumination, inappetence, and agitation are potential manifestations (32).
Cardiovascular disease is no more common among persons with ID than in the general population but is less commonly diagnosed and treated. The cardiovascular risk is elevated by the increased prevalence of obesity and diabetes mellitus (13, e34).
Cancerous and non-cancerous tumors
Persons with ID have a different profile of cancers than the general population (7). Some cancers are more common in certain genetic syndromes, e.g., acute lymphoblastic leukemia is 20 times more common in persons with Down syndrome, because of a CRLF2 rearrangement (Table 2) (e35). Increased mortality from certain cancers, e.g. colorectal cancer, and shortened median survival times are attributed to delayed diagnosis, relative lack of preventive care, and poor treatment adherence (6, 14). Patients with tuberous sclerosis, the most common phakomatosis associated with ID, can develop giant-cell astrocytoma, possibly leading to hydrocephalus, as well as renal angiomyolipoma (Table 2) (e27).
The syndrome called cerebral palsy is the most common sequela of brain damage in early childhood (e32); there are also dystonic, athetoid, and ballistic syndromes. These patients need lifelong interdisciplinary neuro-orthopedic and neurological care, with individually tailored operations, physical aids, and drugs to improve their motor function (33).
Persons with ID, independently of their degree of cognitive impairment, develop dementia five times more often than the general population; the frequency of dementia is particularly elevated in persons with Down syndrome (34, e36). Characteristic features include memory impairment, loss of practical abilities in everyday life, and the early appearance of behavioral abnormalities (e37–e39). The two-step diagnostic assessment consists of neuropsychological evaluation and history-taking from persons in the patient’s near social environment (eTable 2) (e22, e23). Laboratory and imaging studies are essential to rule out treatable causes, such as hypothyroidism (e26). Dementia is treated with acetylcholinesterase (AChE) inhibitors in accordance with current guidelines; patients with ID, however, are more vulnerable to side effects (e.g., epileptic seizures), and thus non-pharmacological measures, e.g., adapting the patient’s physical environment to dementia, should receive due attention (35, e40).
Common mental illnesses
According to population-based studies from the United Kingdom, the point prevalence of mental illness in persons with ID is approximately 20% (36, 37). In addition to mental illness in the narrow sense, some 25% of persons with ID have severe behavioral disturbances that are not attributable to a somatic or mental illness (36–38). Behavioral disturbances have been defined as culturally inappropriate behavior of such intensity, frequency, and duration that it either threatens the physical integrity of the patient or others or else renders the patient’s use of public facilities difficult or impossible (38).
Persons with mild ID have similar living situations to the general population and also have a similar point prevalence of substance dependence (1.8%). On the other hand, persons with severe or very severe ID suffer less commonly from substance dependence (0.5%); the cases that do arise are often iatrogenic (36). Substance dependence in persons with ID is treated with a combination of withdrawal, dishabituation, psychotherapy, and environmental adaptation.
The point prevalence of schizophrenic psychosis is elevated in persons with mild ID (6%) and lower in persons with more severe ID (36). In circumscribed syndromes, such as velocardiofacial syndrome (Table 2, the prevalence is increased (persistent in about 30%) (e41). Schizophrenia may go unrecognized in persons with ID because of “diagnostic overshadowing,” i.e., misinterpretation of schizophrenic manifestations as part of the disability (e.g., movement disturbances, negative manifestations). Hallucinations may not be reported by the patient, and thought disorders may not be interpretable. The following aspects may point to the presence of schizophrenia:
- Age of onset (young adulthood)
- Positive family history
- Talking with persons who are not there; covering or hitting the ears
- Paroxysmal aggression and anxiety.
The prevalence of affective disorders is the same across all degrees of severity of ID. The point prevalence is 6–7 %, similar to that in the general population (9.3%) (36, e42). The risk of depression is elevated in certain syndromes, such as fetal alcohol spectrum disorder (FASD), Prader–Willi syndrome, phenylketonuria, and Down syndrome, while bipolar affective disorder is more common in velocardiofacial syndrome, fragile X syndrome, Klinefelter syndrome, Rubenstein–Taybi syndrome, and FASD (e43). Depressed mood is often less obvious in intellectually disabled persons. It may manifest itself as:
- Self-injury and aggression
- Psychomotor agitation, screaming
- Regressive behavior
- Sleeping and eating disturbances.
Antidepressants and mood stabilizers are prescribed; tricyclic antidepressants should be avoided, because persons with ID are more vulnerable to their side effects (e44). Adapted psychotherapeutic methods can also be used, particularly cognitive behavioral therapy (e45).
Anxiety disorders can be seen in patients with a mental developmental age of at least six months (“first socialization” on the German emotional development scale, Skala der emotionalen Entwicklung—Diagnostik [SEED]-2). Social phobia and generalized anxiety disorder can only arise in the presence of mentalization ability and are thus seen only in persons with an emotional reference age of at least four years. The exact prevalence of anxiety disorders in unclear; their point prevalence is inversely related to the severity of ID (International Statistical Classification of Diseases and Related Health Problems [ICD] F70, ca. 6%; F71–73, ca. 2.4%) (36). Specific phobias, e.g., of dogs or elevators, are often seen. Exposure therapy and other treatments are often effective, but the procedure must be adapted to the intellectual and emotional developmental age of the patient (e46).
Disorders due to emotional trauma
Disabled persons often experience violence (39). In a meta-analysis, the risk of experiencing violence among disabled adults was found to be 6%, corresponding to an odds ratio [OR] of 1.5 compared to the general population (e47). Disabled children, in fact, reportedly have even higher prevalences of combined violent experiences (27%, OR: 3.68), physical violence (20%; OR: 3.56), and sexual violence (14%; OR: 2.88) (e48). Institutionalization, dependence on others, lessened coping capacity, and lack of social support make the intellectually disabled more vulnerable to the experience of an emotionally traumatizing event, and about 10% of those affected develop post-traumatic stress disorder (e49). The diagnostic assessment can be performed with trauma-biographical scales as well as structured instruments for the evaluation of disorders due to emotional trauma (e49). As for treatment, there have been individual case series on the efficacy of Eye Movement Desensitization and Reprocessing (EMDR) (39, e50).
Autism spectrum disorders
In persons with ID, the prevalence of autism spectrum disorders is 7.5–15%, much higher than the estimated 1% in the general population (36, 37, e51, e52). Suspected autism should be evaluated in a multistep procedure; various screening scales and structured behavioral observations can be used to support the diagnostic process (eTable 2) (e20). Even if autism cannot now be cured, special needs education in qualified hands can improve both the level of social functioning (effect size [ES]: 0.65) and maladaptive behavior (ES: –0.92) (e53). Patients with severe behavioral abnormalities can be treated, for a limited time, with low doses of risperidone or aripiprazole (evidence level Ia) (e54, e55). Methylphenidate, atomoxetine, and alpha-2 agonists are effective in attention-deficit/hyperactivity disorder (ADHD) (e56), and melatonin is beneficial in sleep disorders (e57). There is no evidence for the efficacy of serotoninergic drugs or antiepileptic drugs in the treatment of autism (e58, e59).
Somatic and mental illnesses are treated by the same principles as in non-disabled persons. The need-based, goal-oriented overall treatment plan should take all levels of disturbance (bio-psycho-socio-emotional) and all persons in the patient’s near environment into account. Psychoactive drugs should be used only for strict indications, and as monotherapy wherever possible (16–19, 40), in patient-centered fashion, with regular reassessment of the indication, drug holidays, and drug monitoring as matters of routine. Psychoactive drugs for the treatment of behavioral problems should be avoided if possible and given only in rare cases, transiently, and at low doses. All drugs should be started at a low dose, with slow escalation up to the lowest possbile target dose (“start low, go slow”). Polypharmacy and off-label use should be avoided, and reserve drugs should be used only in clearly defined situations (11, 16–19, 40). Non-drug treatments are usually the treatment of first choice in patients with mild ID; simple language is used, learning speed is adapted to the patient’s situation, and more experiential and body-oriented exercises are done (24). In severe or very severe ID, special needs education and developmentally based approaches are the main elements of treatment (23).
The medical care of persons with ID is complex, requiring thinking across disciplinary boundaries and, sometimes, special knowledge and skills. In order to teach the latter, specialty societies such as the German Society for Persons with Intellectual and Multiple Disability (Deutsche Gesellschaft für Medizin für Menschen mit geistiger und mehrfacher Behinderung, DGMGB; www.dgmgb.de) and the German Society for Mental Health in Persons with Intellectual Disability (Deutsche Gesellschaft für seelische Gesundheit bei Menschen mit geistiger Behinderung, DGSGB; www.dgsgb.de) host regular contining medical education events and issue specialized publications, such as the journal “Inklusive Medizin” or the DGSGB materials collection. A number of providers of continuing medical education offer a certificate of training in medicine for persons with intellectual or multiple disability that is recognized by the German Medical Association. The organizational challenges of this compex work were taken into account in the founding, in 2016, of medical treatment centers for adults with intellectual disability or severe multiple disabilities (Medizinische Behandlungszentren für Erwachsene mit geistiger Behinderung oder schweren Mehrfachbehinderungen, (MZEB; www.bagmzeb.de ) of which there have been 38 in Germany since early 2019.
Conflict of interest statement
Dr. Sappok has received royalities for authoring or co-authoring publications on the topic of this article. She has received third-party funding for an SEO project from the Bodelschwinghsche Stiftungen Bethel.
Dr. Winterholler serves on the board of the BAG-MZEB. He has received lecture honoraria from Merz Pharma, Ipsen Pharma, Allergan Deutschland, and UCB Pharma.
Prof. Diefenbacher has received lecture honoraria and reimbursement of travel expenses from FomF GmbH.
Manuscript submitted on 22 May 2019, revised version accepted on 13 September 2019.
Translated from the original German by Ethan Taub, M.D.
PD Dr. med. Tanja Sappok
Behandlungszentrum für psychische Gesundheit
Evangelisches Krankenhaus Königin Elisabeth Herzberge
Herzbergstr. 79, 10365 Berlin,Germany
Cite this as:
Sappok T, Diefenbacher A, Winterholler M: The medical care of people with intellectual disability. Dtsch Arztebl Int 2019; 116: 809–16. DOI: 10.3238/arztebl.2019.0809
For eReferences please refer to:
Department of Psychiatry, Psychotherapy and Psychosomaticsk, Evangelisches Krankenhaus Königin Elisabeth Herzberge, Berlin: Prof. Dr. med. Albert Diefenbacher
Department of Neurology, Krankenhaus Rummelsberg: PD Dr. med. Martin Winterholler
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