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
|1.||WHO: ICD-11 for mortality and morbidity statistics (ICD-11 MMS) 2018 version. https://icd.who.int/browse11/l-m/en (last accessed on 26 February 2019).|
|2.||Statistisches Bundesamt: Statistik der schwerbehinderten Menschen, Kurzbericht 2017. www.destatis.de (last accessed on 12 August 2019).|
|3.||Zweier C: Genetische Störungen. In: Sappok T (ed.): Psychische Gesundheit bei intellektueller Entwicklungsstörung. Ein Lehrbuch für die Praxis. Stuttgart: Kohlhammer Verlag 2018; 81–7.|
|4.||Vissers LE, Gilissen C, Veltman JA: Genetic studies in intellectual disability and related disorders. Nat Rev Genet 2016; 17: 9–18 CrossRef MEDLINE|
|5.||Kochinke K, Zweier C, Nijhof B, et al.: Systematic phenomics analysis deconvolutes genes mutated in intellectual disability into biologically coherent modules. Am J Hum Genet 2016; 98: 149–64 CrossRef MEDLINE PubMed Central|
|6.||Glover G, Williams R, Heslop P, Oyinlola J, Grey J: Mortality in people with intellectual disabilities in England. J Intellect Disabil Res 2017; 61: 62–74 CrossRef MEDLINE|
|7.||O‘Leary L, Cooper SA, Hughes-McCormack L: Early death and causes of death of people with intellectual disabilities: a systematic review. J Appl Res Intellect Disabil 2018; 31: 325–42 CrossRef MEDLINE|
|8.||Ng N, Flygare Wallén E, Ahlström G: Mortality patterns and risk among older men and women with intellectual disability: a Swedish national retrospective cohort study. BMC Geriatr 2017; 17: 269 CrossRef MEDLINE PubMed Central|
|9.||Dieckmann F, Metzler H (eds.): Alter erleben. Lebensqualität und Lebenserwartung von Menschen mit geistiger Behinderung im Alter. Stuttgart: Kommunalverband für Jugend und Soziales 2013.|
|10.||Oppewal A, Schoufour JD, van der Maarl HJK, Evenhuis HM, Hilgenkamp TIM, Festen DA: Causes of mortality in older people with intellectual disability: results from the HA-ID study. Am J Intellect Dev Disabil 2018; 123: 61–71 CrossRef MEDLINE|
|11.||Schoufour JD, Oppewal A, van der Maarl HJK, et al.: Multimorbidity and polypharmacy are independently associated with mortality in older people with intellectual disabilities: a 5-year follow-up from the HA-ID study. Am J Intellect Dev Disabil 2018; 123: 72–82 CrossRef MEDLINE|
|12.||Robertson J, Chadwick D, Baines S, Emerson E, Hatton C: Prevalence of dysphagia in people with intellectual disability: a systematic review. Intellect Dev Disabil 2017; 55: 377–91 CrossRef MEDLINE|
|13.||Cooper SA, Hughes-McCormack L, Greenlaw N, et al.: Management and prevalence of long-term conditions in primary health care for adults with intellectual disabilities compared with the general population: a population-based cohort study. J Appl Res Intellect Disabil 2018; 31 (Suppl 1): 68–81 CrossRef MEDLINE|
|14.||Wallace RA: National disability insurance scheme, health, hospitals and adults with intellectual disability. Intern Med J 2018; 48: 351–9 CrossRef MEDLINE|
|15.||Sappok T, Diefenbacher A (eds.): Die 4. Dimension: Erweiterung des bio-psycho-sozialen Krankheitsmodells um die emotionale Entwicklungskomponente bei Menschen mit geistiger Behinderung. Bielefeld: Bethel Verlag 2017.|
|16.||Gardner WI, Dosen A, Griffiths DM: Practice guidelines for diagnostic, treatment and related support services for people with developmental disabilities and serious behavioral problems. New York: NADD Press 2006.|
|17.||NICE: Guideline: Challenging behavior and learning disabilities: prevention and interventions for people with learning disabilities whose behavior challenges 2015. www.nice.org.uk/guidance/ng11. (last accessed on 2 March 2019).|
|18.||AWMF: Leitlinie: Autismus-Spektrum-Störungen im Kindes-, Jugend- und Erwachsenenalter 2016. www.awmf.org (last accessed on 23 February 2019).|
|19.||Canadian Consensus Guidelines: Primary care of adults with developmental disabilities. Can Fam Physician 2011; 57: 541–53.|
|20.||Sappok T, Zepperitz S, Barrett BF, Došen A: Skala der emotionalen Entwicklung – Diagnostik (SEED). Bern: Hogrefe 2018.|
|21.||Sappok T, Sinzig J, Lehmkuhl G, Diefenbacher A: Kapitel 24: Intelligenzminderung. In: Berger M (ed.): Psychische Erkrankungen, Klinik und Therapie, München: Elsevier Urban & Fischer; 6th edition 2018.|
|22.||de Winter CF, Jansen AA, Evenhuis HM: Physical conditions and challenging behaviour in people with intellectual disability: a systematic review. J Intellect Disabil Res 2011; 55: 675–98 CrossRef MEDLINE|
|23.||Sappok T, Zepperitz S: Das Alter der Gefühle – über die Bedeutung der emotionalen Entwicklung bei geistiger Behinderung. Bern: Hogrefe 2nd edition 2019 CrossRef PubMed Central|
|24.||Mensch zuerst – Netzwerk People First Deutschland e. V.: Das neue Wörterbuch für Leichte Sprache. Kassel: Nordlicht Digitaldruck 2008.|
|25.||Vogel M, Feuerherd C: Die Erhebung des psychopathologischen Befundes. In: Sappok T (ed.): Psychische Gesundheit bei intellektueller Entwicklungsstörung. Ein Lehrbuch für die Praxis. Stuttgart: Kohlhammer Verlag 2018; 281–9.|
|26.||The Royal College of Psychiatrists: DC-LD: Diagnostic criteria for psychiatric disorders for use with adults with learning disabilities/mental retardation. London: Gaskell 2001.|
|27.||Bergmann T, Diefenbacher A, Heinrich M, Riedel A, Sappok T: Perspektivenverschränkung: Multiprofessionelle Autismusdiagnostik bei erwachsenen Menschen mit Intelligenzminderung. Z Psychiatr Psych Ps 2016; 64: 257–67 CrossRef|
|28.||Robertson J, Hatton C, Emerson E, Baines S: Prevalence of epilepsy among people with intellectual disabilities: a systematic review. Seizure 2015; 29: 46–62 CrossRef MEDLINE|
|29.||Walsh M, Morrison TG, McGuire BE: Chronic pain in adults with an intellectual disability: prevalence, impact, and health service use based on caregiver report. Pain 2011; 152: 1951–7 CrossRef MEDLINE|
|30.||Elstner S, Salzmann E: Angst-, Zwangstörungen, Belastungs-, dissoziative und somatoforme Störungen. In: Schanze C (ed.): Psychiatrische Diagnostik und Therapie bei Menschen mit Intelligenzminderung. Stuttgart: Schattauer Verlag 2nd edition 2014: 129–46.|
|31.||Robertson J, Baines S, Emerson E, Hatton C: Constipation management in people with intellectual disability: a systematic review. J Appl Res Intellect Disabil 2018; 31: 709–24 CrossRef MEDLINE|
|32.||de Veer AJ, Bos JT, Niezen-de Boer RC, Böhmer CJ, Francke AL: Symptoms of gastroesophageal reflux disease in severely mentally retarded people: a systematic review. BMC Gastroenterol 2008; 11: 8–23 CrossRef MEDLINE PubMed Central|
|33.||van Timmeren EA, van der Schans CP, van der Putten AA, et al.: Physical health issues in adults with severe or profound intellectual and motor disabilities: a systematic review of cross-sectional studies. J Intellect Disabil Res 2017; 61: 30–49 CrossRef MEDLINE|
|34.||Strydom A, Chan T, King M, Hassiotis A, Livingston G: Incidence of dementia in older adults with intellectual disabilities. ResDev Disabil 2013; 34: 1881–5 CrossRef MEDLINE|
|35.||Livingstone N, Hanratty J, McShane R, Macdonald G: Pharmacological interventions for cognitive decline in people with Down syndrome. Cochrane Database Syst Rev 2015; 10: doi: 10.1002/14651858.CD011546.pub2 CrossRef MEDLINE|
|36.||Cooper SA, Smiley E, Morrison J, Williamson A, Allan L: Mental ill-health in adults with intellectual disabilities: prevalence and associated factors. Br J Psychiatry 2007; 190: 27–35 CrossRef MEDLINE|
|37.||Sheehan R, Hassiotis A, Walters K: Mental illness, challenging behaviour, and psychotropic drug prescribing in people with intellectual disability: UK population based cohort study. BMJ 2015; 351: h4326 CrossRef MEDLINE PubMed Central|
|38.||Emerson E, Bromley J: The form and functioning of challenging behavior. J Intellect Disabil Res 1995; 39: 388–98 CrossRef MEDLINE|
|39.||Rittmannsberger D, Lueger-Schuster B, Weber G: Traumafolgestörungen. In: Sappok T (ed.): Psychische Gesundheit bei intellektueller Entwicklungsstörung. Ein Lehrbuch für die Praxis. Stuttgart: Kohlhammer Verlag 2018; 331–37.|
|40.||NICE: Guideline: Mental health problems in people with learning disabilities: prevention, assessment and management. www.nice.org.uk/guidance/ng54. (last accessed on 26 February 2019).|
|e1.||Zweier C: Genetische Diagnostik. In: Sappok T (eds.): Psychische Gesundheit bei intellektueller Entwicklungsstörung. Ein Lehrbuch für die Praxis. Stuttgart: Kohlhammer Verlag 2018b: 275–80.|
|e2.||Holmes N, Shah A, Wing L: The disability assessment schedule: a brief screening device for use with mentally retarded. Psychol Med 1982; 12: 879–90 CrossRef MEDLINE|
|e3.||Meins W, Süssmann D: Evaluation of an adaptive behaviour classification for mentally retarded adults. Soc Psychiatry Psychiatr Epidemiol 1993; 28: 201−5 CrossRef MEDLINE|
|e4.||Weiß R: Grundintelligenztest Skala 2 – Revision (CFT 20-R) mit Wortschatztest und Zahlenfolgentest – Revision (WS/ZF-R). Bern: Hogrefe Verlag 2006.|
|e5.||Raven JC, Raven J, Court JH: Coloured progressive matrices (CPM). Deutsche Bearbeitung und Normierung. Frankfurt am Main: Harcourt 2006.|
|e6.||Melchers P, Preuss U: Kaufman assessment battery for children. Deutschsprachige Fassung (KABC). 8th edition. Frankfurt: Pearson Assessment 2009.|
|e7.||Kaufman AS, Kaufman NL: Kaufman assessment battery for children. 2nd edition (KABC-2). Göttingen: Hogrefe 2015 CrossRef|
|e8.||Snijders JTH, Tellegen PJ, Laros JA: Snijders-Oomen Non-verbaler Intelligenztest: SON-R 5½ – 17. 3rd edition. Göttingen: Hogrefe 2005.|
|e9.||Tellegen PJ, Laros JA, Petermann F: Snijders-Oomen non-verbaler Intelligenztest: SON-R 6–40. Göttingen: Hogrefe 1st edition 2012.|
|e10.||Petermann F: Wechsler adults intelligence scale. 2nd edition. Frankfurt: Pearson Assessment 2012.|
|e11.||Bienstein P, Werner N: Verhaltensanalyse. In: Sappok T (eds.): Psychische Gesundheit bei intellektueller Entwicklungsstörung. Ein Lehrbuch für die Praxis. Stuttgart: Kohlhammer Verlag 2018: 290–6.|
|e12.||Aman MG, Sing NN: Aberrant behavior checklist: community supplementary manual. New York: East Aurora, Slosson Educational Publications 1994.|
|e13.||Steinhausen HC, Winkler-Metzke C: Der Verhaltensfragebogen bei Entwicklungsstörungen im Erwachsenenalter (VFE-ER), Psychometrische Kennwerte und Normierung. Z Klein Psych Psychother 2011; 40: 160–71 CrossRef|
|e14.||Knoedler DW: The modified overt aggression scale. Am J Psychiatry 1989; 146: 1081–2 CrossRef MEDLINE|
|e15.||Yudofsky SC, Silver JM, Jackson W, Endicott J, Williams D: The overt aggression scale for the objective rating of verbal and physical aggression. Am J Psychiatry 1986; 143: 35–9 CrossRef MEDLINE|
|e16.||Bienstein P, Nussbeck S: Inventar zur funktionellen Erfassung selbstverletzenden Verhaltens. Göttingen: Hogrefe 2010.|
|e17.||Matson JL, Gardner WI, Coe DA, Sovner R: A scale for evaluating emotional disorder in severely and profound mentally retarded persons. Development of the Diagnostic Assessment for the Severly Handicapped (DASH) scale. Br J Psychiatry 1991; 159: 404–9 CrossRef MEDLINE|
|e18.||Moss S, Prosser H, Costello H, et al.: Reliability and validity of the PAS-ADD checklist for detecting psychiatric disorders in adults with intellectual disability. J Intellect Disabil Res 1998; 42: 173–83 CrossRef MEDLINE|
|e19.||Bertelli M, Scuticchio D, Ferrandi A, et al.: Reliability and validity of the SPAID-G checklist for detecting psychiatric disorders in adults with intellectual disability. Res Dev Disabil 2012; 33: 382–90 CrossRef MEDLINE|
|e20.||Sappok T, Diefenbacher A, Bergmann T, et al.: Der Diagnostische Beobachtungsbogen für Autismus-Spektrum-Störungen – Revidiert (DiBAS-R). Ein Screening-Instrument für Erwachsene mit Intelligenzminderung. Bern: Huber 2015 CrossRef MEDLINE|
|e21.||Belot M: Der Ausdruck von Schmerz bei mehrfachbehinderten Personen: Evaluation der Schmerzzeichen bei Jugendlichen und Erwachsenen mit Mehrfachbehinderung. In: Maier-Michalitsch NJ (eds.): Leben pur – Schmerz bei Menschen mit schweren und mehrfachen Behinderungen. Düsseldorf: verlag selbstbestimmtes leben 2009.|
|e22.||Schanze C: Checkliste zur Erfassung von demenziellen Entwicklungen bei Menschen mit Intelligenzminderung 2009. www.demenz-service-westliches-ruhrgebiet.de/tl_files/westliches_ruhrgebiet/Meldungen/edbMed0211_SA_SchanzeChecklisten.pdf (last accessed on 2 March 2019).|
|e23.||Müller SV, Kuske B: Demenztest für Menschen mit Intelligenzminderung. Bern: Hogrefe Verlag 2019; in press.|
|e24.||Duis J, van Wattum PJ, Scheimann A, et al.: A multidisciplinary approach to the clinical management of Prader-Willi syndrome. Mol Genet Genomic Med 2019; 7(3): e514 CrossRef MEDLINE PubMed Central|
|e25.||McDonald-McGinn DM, Sullivan KE, Marino B, et al.: 22q11.2 deletion syndrome. Nat Rev Dis Primers 2015; 19;1: 15071 CrossRef MEDLINE PubMed Central|
|e26.||Capone GT, Chicoine B, Bulova P, et al.: Down Syndrome Medical Interest Group DSMIG-USA Adult Health Care Workgroup: Co-occurring medical conditions in adults with Down syndrome: a systematic review toward the development of health care guidelines. Am J Med Genet A 2018; 176: 116–33 CrossRef MEDLINE|
|e27.||Yates JR, Maclean C, Higgins JN, et al.: The Tuberous Sclerosis 2000 Study: presentation, initial assessments and implications for diagnosis and management. Arch Dis Child 2011; 96; 1020–5 CrossRef MEDLINE|
|e28.||Erickson CA, Kaufmann WE, Budimirovic DB et al.: Best practices in fragile X syndrome treatment development. Brain Sci 2018; 15: pii: E224 CrossRef MEDLINE PubMed Central|
|e29.||Spiesshoefer J, Runte M, Heidbreder A, et al.: Sleep-disordered breathing and effects of non-invasive ventilation on objective sleep and nocturnal respiration in patients with myotonic dystrophy type I. Neuromuscul Disord 2019; 29: 302–9 CrossRef MEDLINE|
|e30.||Martin P: Pain in Rett syndrome: peculiarities in pain processing and expression, liability to pain causing disorders and diseases, and specific aspects of pain assessment. Adv Autism 2017; 3: 163–82 CrossRef|
|e31.||Walter-Fränkel S: Schmerzdiagnostik. In: Sappok T (ed.): Psychische Gesundheit bei intellektueller Entwicklungsstörung. Ein Lehrbuch für die Praxis. Stuttgart: Kohlhammer Verlag 2018; 265–74.|
|e32.||Schwartz L, Engel JM, Jensen MP: Pain in persons with cerebral palsy. Arch Phys Med Rehabil 1999; 80: 1243–6 CrossRef|
|e33.||Andresen V, Enck P, Frieling T, et al.: S2 Leitlinie chronische Obstipation. www.awmf.org/uploads/tx_szleitlinien/021–019l_S2k_Chronische_Obstipation_2013–06-abgelaufen.pdf (last accessed on 12 August 2019).|
|e34.||Franke ML, Heinrich M, Adam M, Sünkel U, Diefenbacher A, Sappok T: Körpergewicht und psychische Erkrankungen – Ergebnisse einer klinisch-psychiatrischen Querschnittsanalyse bei Menschen mit Intelligenzminderung. Nervenarzt 2017; 89: 552–8 CrossRef MEDLINE|
|e35.||Brown AL, de Smith AJ, Gant VU, et al.: Inherited genetic susceptibility of acute lymphoblastic leukemia in Down syndrome. Blood 2019 26. pii: blood.2018890764.|
|e36.||Holland AJ, Hon J, Huppert FA, Stevens F, Watson P: Population-based study of the prevalence and presentation of dementia in adults with Down‘s syndrome. Br J Psychiatry 1998; 172: 493–8 CrossRef MEDLINE|
|e37.||Dekker AD, Sacco S, Carfi A, et al.: The behavioral and psychological symptoms of dementia in Down Syndrome (BPSD-DS) Scale: comprehensive assessment of psychopathology in down syndrome. J Alzheimers Dis 2018; 63: 797–819 CrossRef MEDLINE PubMed Central|
|e38.||Devshi R, Shaw S, Elliott-King J, et al.: Prevalence of behavioural and psychological symptoms of dementia in individuals with learning Disabilities. Diagnostics (Basel) 2015; 5: 564–76 CrossRef MEDLINE PubMed Central|
|e39.||Axmon A, Björne P, Nylander L, Ahlström G: Psychiatric diagnoses in relation to severity of intellectual disability and challenging behaviors: a register study among older people. Aging Ment Health 2018; 22: 1344–50 CrossRef MEDLINE|
|e40.||Fonseca LM, Navatta AC, Bottino CM, Miotto EC: Cognitive rehabilitation of dementia in adults with Down Syndrome: a review of non-pharmacological interventions. Dement Geriatr Cogn Dis Extra 2015; 5: 330–40 CrossRef MEDLINE PubMed Central|
|e41.||Tang SX, Moore TM, Calkins ME, et al.: Emergent, remitted and persistent psychosis-spectrum symptoms in 22q11.2 deletion syndrome. Transl Psychiatry 2017; 25: 7: e1180 CrossRef MEDLINE PubMed Central|
|e42.||Jacobi F, Höfler M, Strehle J et al.: Psychische Störungen in der Allgemeinbevölkerung. Nervenarzt 2014; 85: 77–87 CrossRef|
|e43.||Fletcher R, Barnhill J, Cooper SA (eds.): Diagnostic manual-intellectual disability: a textbook of diagnosis of mental disorders in persons with intellectual disability. Kingston: NADD Press/ National Association for the Dually Diagnosed 2nd edition 2016.|
|e44.||Hiremath A, Gangavati S, Gumber R, Barrett M: Depression. In: Bhaumik S, Branford D, Barret M, Gangadharan SK (eds.): The frith prescribing guidelines for people with intellectual disability. 3rd edition. Chichester: John Wiley & Sons 2015; 169–78 CrossRef|
|e45.||Hamers PCM, Festen DAM, Hermans H: Non-pharmacological interventions for adults with intellectual disabilities and depression: a systematic review. J Intellect Disabil Res 2018; 62: 684–700 CrossRef MEDLINE|
|e46.||Sappok T, Voß T, Millauer E, Schade C, Diefenbacher A: Psychotherapie bei Menschen mit IM: Theoretischer Hintergrund und praktische Umsetzung. Der Nervenarzt 2010; 81: 827–36 CrossRef MEDLINE|
|e47.||Hughes K, Bellis MA, Jones L, et al.: Prevalence and risk of violence against adults with disabilities: a systematic review and meta-analysis of observational studies. The Lancet 2012; 379: 1621–9 CrossRef|
|e48.||Jones L, Bellis MA, Wood S, et al.: Prevalence and risk of violence against children with disabilities: A systematic review and meta-analysis of observational studies. Lancet 2012; 380: 899–907 CrossRef|
|e49.||Daveneyn J, Hassiotis A, Katona C, Matcham F, Sen P: Ascertainment and prevalence of post-traumatic stress disorder (PTSD) in people with intellectual disabilities. J Ment Health Res Intellect Disabil 2019; DOI: 10.1080/19315864.2019.1637979 CrossRef|
|e50.||Gilderthorp RC: Is EMDR an effective treatment for people diagnosed with both intellectual disability and post-traumatic stress disorder? J Intellect Disabil 2015;19: 58–68 CrossRef MEDLINE|
|e51.||Baio J, Wiggins L, Christensen DL, et al.: Prevalence of autism spectrum disorder among children aged 8 years—autism and developmental disabilities monitoring network,11 Sites, United States, 2014. MMWR Surveill Summ 2018; 67: 1–23 CrossRef MEDLINE PubMed Central|
|e52.||Brugha TS, Spiers N, Bankart J, et al. Epidemiology of autism in adults across age groups and ability levels. Brit J Psychiat 2016; 209: 498–503 CrossRef MEDLINE|
|e53.||Virues-Ortega J, Julio FM, Pastor-Barriuso R: The TEACCH program for children and adults with autism: a meta-analysis of intervention studies. Clin Psychol Rev 2013; 33: 940–53. CrossRefMEDLINE|
|e54.||Jesner OS, Aref-Adib M, Coren E: Risperidone for autism spectrum disorder. Cochrane Database Syst Rev 2007; 24:CD005040 CrossRef MEDLINE|
|e55.||Hirsch LE, Pringsheim T: Aripiprazole for autism spectrum disorders (ASD). Cochrane Database Syst Rev 2016; 26:CD009043 CrossRef MEDLINE|
|e56.||Reichow B, Volkmar FM, Bloch MH: Systematic review and meta-analysis of pharmacological treatment of the symptoms of attention-deficit/hyperactivity disorder in children with pervasive developmental disorders. J Autism Dev Disord 2013; 43: 2435–41 CrossRef MEDLINE PubMed Central|
|e57.||Cuomo BM, Vaz S, Lee EAL, Thompson C, Rogerson JM, Falkmer T: Effectiveness of sleep-based: interventions for children with autism spectrum disorder: a meta-synthesis. Pharmacotherapy 2017; 37: 555–78 CrossRef MEDLINE|
|e58.||Williams K, Brignell A, Randall M, Silove N, Hazell P: Selective serotonin reuptake inhibitors (SSRIs) for autism spectrum disorders (ASD). Cochrane Database Syst Rev 2013; (8):CD004677 CrossRef|
|e59.||Hirota T, Veenstra-Vanderweele J, Hollander E, Kishi T: Antiepileptic medications in autism spectrum disorder: a systematic review and meta-analysis. J Autism Dev Disord 2014; 44: 948–57 CrossRef MEDLINE|
|e60.||Sappok T: Psychische Gesundheit bei intellektueller Entwicklungsstörung. Ein Lehrbuch für die Praxis. Stuttgart: Kohlhammer Verlag 2018.|
‘I stayed here just for my son:’ the experience of parents of children with intellectual disabilities who migrated from Turkey to GermanyEuropean Journal of Special Needs Education, 202110.1080/08856257.2021.1961196
Deutsches Aerzteblatt Online, 201910.3238/arztebl.2019.0807
Der Pneumologe, 202110.1007/s10405-021-00378-1