Академический Документы
Профессиональный Документы
Культура Документы
, •. '"·· ,,i;n,
,i,11:~•t. l97
- PSYCKOPATHOLOOY
'
t
'
W h.en yu~l rut-:.-e completed rhis dupte1' yo.u should be able to:
1. Disrnss. the d.ifl:erent ways in which learn ing and 5. Describe ,md evaluate the main forms of
dev--elopmentJ.l disJ.btlities are t~ategor:ize.d and intervention, care and support for intt"llecru al
bbelled. disabilities.
2. Describe and compare the various types of 6 • Describe the diagnostic criteria for autistic
spedfic learning disabilities, their aetiology and spectrum disorder.
treatment- 7. Compare and contrast theories of the aetiology
3. Describe the various forms of intellectual of autistic specrtum disorder.
disability. s. Describe and evaluate the main forms of
4. Compare and c01mast genetic, biological and intervention, care and support for individuals
environmental causes of intellectual disabilities. with autistic spectnim disorder.
3../-
: -••~--•••-••••~.--•-••- ••••••••••••• ••-•••.. •••••••os••••• ,- •- •• ••• ••••••••-•••• • ••••• • • .. • •• •• • • ..•••• • •• • .. •• • •• • •• •• •••• • .. ••• • • • ••• •• •• • • •• • ;- •• • .. ••• •• .. •• • •• • • • •• •• • •••• • • • • • •• • ••• •• • • ••••••••• • • ••• •"•" ••••• ....\
Outing chikl,hood, no-one knew what/ had. Jwast:onsi.dered 'crazy' by a doctor at age 1 because I had constant tantrums, which
1 ool,y.ended. one day; when my mothertook me to the beach during a holiday. My nerves suddenly were calmed down by the sight
and the soothing soonds of the sea. I was beginning to say my first words and ~tarted to make some progress.
Desp,te the progress, I stm
had strang~ behaviours, like spinning plastic lids, jars and coins. I rejected teddy bears that other
roddlers liked, but held on to other obj ects, like dice (which had a smooth surface and were pleasant to touch). I was terrorized by
everyday noises; like planes passing by, thunder, machinery, drills, balloo,ns bursting and any sudden noise.
Being the firstborn, my mother didn't take notice of behaviours like rocking back and forth, or spending time on a rocking
horse fn the-day care centre as a toddler instead of playing with other kids.
Despite socfa/izlng difficulties, my interest fo r reading and learning the alphabet pleased my mother. Instead of pointing out
pictures in a newspaper my mother was reading, I asked her what the letters were, and that prompted her to teach me to read
before starting school.
Socialty, i had problems thatwor(iedpeop/'e. I was 11ot able to recognize people easl/y, and was not able to decode nonverbal
cues. My mother complained about always having to spell things out to me. While my younger (non-autistic) brother seemed
to know instinctively when to bring up a subject, or when to say a joke, I was a nuisance, because I couldn't tell if somebody was
angry; sad, tired, and so on, jus t by looking at him/her. I took things literally and was terrorized by my mother's 'threats: which my
younger brother did not take seriously. Sile uttered threats like 'I will send you away' when we behaved badly. My brother was able
to understand that she never meant it; however, I was terrorized by them.
One thing that discouraged socializing was that most others did not like to talk about Insects, calculators, or space al/ the
time. Other people liked my subjects once in a while' but got angry if Jwent on and on. My mother constantly reminded me not to
ralk about the s.ame things over and over. Changing subjects was hard for me. I was fixated on certain subjects like entomofogy
and arachnology. Nobody cares to hear about the chelate pecjipalps of pseudoscorpions. I
George's Story
•.._ •••• • ...• • - - •- • • • •• • • • n • • • • _ , • • • ·• • • . , -.., • • • • • • o • • • •• - • • • • .-, ••• • • ..-.~ - ,..., • •• • • • o • • • • • •• • •• • I • • o • • • • • • • ' • • • •· • • • • •• • • • • •• • • • • •• • • • • • • • , . ', , •• • • • • • • • ' • • •• O, o •• • • • • • • • • • •• • • ' • • • • • • • • • ' h • • •• • • • • 0 • • , ._ • • • • • • • • '< • • • • , -< • • • •• • • • • • • • • • • • • • • • • • o • • • • • •• ..
SECTION SUMMARY ·---·-····· ·-·-···. ····-......................······.. ' ................................' ....................' ...............' ...., .... ..... ....' .. ,..... ............... ... -~
'
• The three mam categories of learning disabilities are (1) specific learning disabilities (e.g. specific learning diso rd er),
Q) mtP.Jte aoot disabi1ities, and {3} autistk spectrum disorder (ASD).
..•··
•.,__ - - - - ~ -••--•• - -- •----- • •-u~•-•-•- •u ..O • o-•-• • ..,.• •••••• ••,-••••• ••••••• ••• ••••••• • ••••• •• ••••••• .. •• ••• •• • ••• • • • ••• • •••• • • • • • •• • •• • •• •• ••••• • •• • • • H .. I• • •
Description
Exampte symptoms
Problems with accurate or fluent word
Reading ach·ievement .1s substantially
.
recognition, poor decoding and poor " Omit, <1dd or distort the sound of
~elow th e norm for chronological age, words when reading
5pelling abilities (formerly classified
as dyslexia) intelligence and educational level • Read slowly and with poor
comprehension
Problems with written expression
Writing skills are substantially below ,. Regular errors in spelling, grammar or
th0 se expected for chronological punctuation
age, intelligence and educational
level
Difficulties mastering number sense
Mathematics ability is substantially below • Difficulty remembering arithmetic
number facts or calculation (former!~
norm for chronological age, intelligence facts (e.g. to 'carry' a number)
classified as dyscalculia)
and educational level • Failure to understand arithmetic
concepts
Difficulties with verbal and written Scores on tests of expressive language ~ Markedly limited vocabulary
expression development are substantially below • Making errors in tense
those for chronological age, intelligence • Difficulty recalling the right word
and educational level
I. Sympt()IY1j Slijft ln \',ltly tluwtoprnQnt • SQltnd prolongiltloil _of conson~nts ond voweb
• liroken words
• Olfht.ultlt'l "> dr~r nQt bHwr , o(f0Yt\lt:rl f\) 1 hy C'.C>li(JNilt
, " l or
.,,
ll~Q!J Irr < eMc11\.h) n•; 1r,dodhi~J r.creb,ol palw, ctt,~f-MM or • f illed 01 unf\ll~d p11uses in speech
othot 1m;c,ll\.t)I c.01,cilt:\Ol)S -
• Word sub~tltlltlo11 to avoid dlfftcult word!.
• Word pronw,clall9n with excE>sslva physical tension
• Mo11osyllablr whole.,worct repetitions
Childhood-onset fluency dlsotder (stuttering) •- lho disturbance cau\cs anxiety about speaking or lhnlta,
tlon In effectlvn comrnunlra tlon
'1'~11~ ls ,, probhm~ with the fluency ,,nd 1·in1c,pnt1trning
0t speech wh_k h 11w<,lvcs (1) frequunr rcperiliOJ's or pro- • Symptoms stort In early development
lo!lg;tti<ms ol St11.mds, (2) pm.1sts within words, (3) filled • Dlfficultles t1re not potter accountl!d-for by speech•mot,,r
11r t,mf\llud prnuc$ In spc!crh, (4) word sublitltutfons to or sensory deficit or other n,edlcal condition
avc\ld pronouncing problematic word,~. (~) words pro•
tlun·d with tm (rxcess nf' physicol tension, and (6) mon-
osyllabic word repetitions (1~.g. ·go-go•t~o-go out of thl:
ro<1111') (DSM., 5 Sumrnnry Tahl<: 17.4). Pcarful. unridpu- Stuttcrlng is more i.:nrnmon in niaks rhan foma lrs, with
\ion of srutterlog may ch•wlop in many sufferers and this n mak-10,fo malc t'Ul'iu of 4: l u1 adolescents and 2.3: 1
may m:\k.c s\u ttel'ing won;e in st:rc-stiful shuations, such in both y<Jungcr rhilclnm and adulrs. Of those children
as when giving u speech or diagnosed with stuttering, 12,7 per cent also have speech
1\u\terlng Ad1stu1b,mt1.1 ln thu norm,,I sound disorder, l 'i .2 per rent have another learning dis-
11u~r,~y ;ind time pntternlnq of ~pccc:h tht1t
at an interview, Sumerlng
may be acc:o mponied by ability and 5' .9 per cent have ADHI) (Blood, Ridenour,
1; mnpp1oprl.1te for th~ 1ndlvklunl•~ a9c.
physical symµton, s suc:h Qualls & Hammer, 2003) .
as eye blinks, tics, n·cmor!l, j<:·rk.ii1 g of the hcnd and
rll•nching fists. As tan be envisaged, snmedng can also
havt highly detrimti\tal effeccs on sori.'1 and ot cupn• 17,2.3 The Aetiology of
rlonal fui1ctio11lng, On~n t)f sn1rtering rypka lly ocl.'. urs Specific Learning Disabilities
betWel'n Z and 7 years of age, with a peak onset around
5 year~. The unset i~ usual'ty insidious and in.itially the In the fo llowing section we will disruss the ae tiol-
(hiki may be L1nawnrt.> or stutteriJ1g. However, as nwan·· ogy and c,rnst•s of some of the more co mmon of the
ne~s in(·rctu;~s. the child will develop compensatory specif-le lcl\rning disnbili tics we have desrribc·<l in this
stt·att~ies Jot-avoiding words nnd situations that cause settion .
~tutt~!ring. Ce>JT'tf\ll.111ity studies estim.ate prev.ilcnce
!'ates of childhood-onset
lhlldhood•OnHt fluenty c;ll!'order
litutt1rlng) A problem with tho fhJl'nry fluency disorder (stutter• Dyslexia
:ndlhnt'-Pt1t\umlng of speoch which log) for all indivldu.1ls at As mentioned earlier, dyslexia is a condition that
11 1VQlv{!-5 fr1•quMt ,~petition, 01 pmlon r1ffccts both reading and written cxpn·ssion, and is a
() ,7 per cent, rising to 1.4
1au0ns of ~ound,, f'ill,N '~ within wQrd~, per c~nt in yoi111g children pcrsistcnt, chronic con-
- llllu'I')~ In w•eQth, wc.ird sul>stl\ut lom,
V.'O rtt~mod1.1.t;E;d with i)n c-xcPs, of pt 1ysl1:ul and dmpping to 0.5 per dition in which !'t>ading dysl1ltl1 A persistent, chronic learning
ll'l'\11011, ,,r,cl monosyll11blt worri rnpotltlo,,&. cent in ,1llolcscents (Craig, ability h,A~ behind that of dls11bllity In which there 1Jre developmental
deficits lnspclllng, reading and writing
Haucock, 'lT'.an, Craig & non-impaired l.ndivi\'.iuals abllitles.
Pi:rers. 20()2). 1--k ,wcwr. the prognosis fol' tlttltterinv; i!i fo r the conrse of most of
good, with al'Ot1nd 40 per cent of sufferers ovel'coming their lifetim.e, The development of dysh!:xia can be pre•
the proble111-brfore tht>y start, school nrld super cent over- dieted by a number of risk facto rs, i_\1duding dif11culty
eo111itig- it before udolescenC'e (C<.>\!tln-e & Guirnr, 1993). recognizing rhymti•s at ~ge 4 years (Bradley & Bl'yanr,
- flS\'(:HOPATttOLOG.Y
.• • . • • .· ..1 , t f other abilities, such as
Jchot ts qum· mdepenuen · 0 .d f
1Q~1) , diffit-ub:y n,nning t>wryd3y o-bjt-.::ts ,H age 5 ye~u·s •. g vocabulary an use o
,ieneral intelligence. reasonm ·
. h
•. k il C
~. Shan ~we er. ram ,
·
"Wolf, Ually & Mnni~, 1QSt,) an.d dift1.culry karnmg ~
'-)'TltaX (Share & Stanovic · 199 ;i' .
synt..1t'tk ruks at ,1,gc .?-J ye:rrs ,sc,: rburough, 1:90). - ) 5-,1 . ;vitz & Shavw1tz (2005 ,
Katz Fowler et aL , 1995 · l.l)'V • . .
Hnwe,aer, thi• nhun c..'l.uses ot dys.le..\'1~• nuw appc.~r to ·· . .
p.1302 ) charactenze the expenenc
, · -e of the dysle.xic m
·
,. ,
bie hknu:tie,~,.1
,ts ab.norm.a l'mrs.
· m • , ,,-ti~·'-, a1·ea~
· spt:l,. • · · ot
· the
the following way:
bra.it'l :mch as ,he t~·tnpnropal'iet,tl r~gion \Shaywitz &
Shavwi~, ZOO'i \ These abnormalities m,l)' be the result
... that the affected
. .reader
_ cannot use
of itnttk fano-ts and they m:1y give ri:,e t0 the difticnl - Th e probl t>m 1s , .
. b l . her order lin~nusuc skills to access
ti~ th,ll sufferers have in de\'.oding and comprehend: I11s o r er 11g · o .
ing writlen m :\te.riaL Wt: will revie,1,: these theo~es ot t h e meamng . . the printed word
m ltt'l _ has first been
the at•tiolog:y of dy~~h by looking in turn at ev1deJ1ce decoded and identified. Suppose. tor example, an
refoted to geot:tic inheritance. 1.:ognitiYe impairments individual knows the precise meaning of the spoken
and brain abnormulitit' S. won.1.1 , appan•ri·on'·, howeYer, she will not be able to,
use her knowledge of the meaning of the word until
Genetic faciors As ea.rlv as 1950. Hallgren reported she can decode and identify the printed word on the
that rnore. th ...m 80 per ce~r of children \vith dyslexia page and it will appear that she does not know
also h,1d mher fa.rnily members with the disability, \\rith the word's meaning.
1110ft' recent studies su~>esting that between 23 and 65
per cent of children with dyslexia have a parent with
the disorder (,_Scarborough, 1990). In addition, 40 per Bra in abnormalities Associated with problems
cent of the siblings of sufferers will also exhibit symp- in relating written letters to corresponding sounds are
toms of dyslexia tPennington & Gilger. 1996). This sug- deficits in brain functioning in dyslexia - especially in
gests th«t dyslexia runs in families and so may have an the temporoparietal areas of the brain. Post-mortem
imporrnm genetic component , and evidence for this studies of the brains of dyslexia sufferers suggest abnor-
genetic component is supported by studies suggest- malities in the temporoparietal brain region (Galaburda,
ing that dyslexia c:onc.ordance rates are significantly Sherman, Rosen, Ahoitiz et al. , 1985) and the number
highe.r in MZ than in DZ twins (Stevenson. Graham. and organization of neurones in the posterior language
Fred.man & Mcloughlin, 1987). Using genetic mark- area of the cortex (Galaburda, 1993). Nevertheless, these
ers for dyslexia, linkage studies have implicated genes abnormalities found in post-mortem studies might sim-
on a number of chromosomes in the aetiology of dys- ply represent the consequences of a lifetime of poor
lexia, including loci on chromosomes 2, 3 . 6. 15 and reading rather than a causal factor in dyslexia. However,
18 (Fishel." & De-Fries. 2002). There is evidence from functional magnetic resonance imaging (fMRI) stud-
molecular genetics to suggest that many of these genes ies of the brains of young children with dyslexia indi-
participate in brain development and cause the abnor- cate that they show significantly less activation in a
malities in brain development associated with dyslexia number of left hemisphere sites when reading than do
(Galaburda, LoTurco. Ramus, Fitch & Rosen, 2.006; non-impaired children. These areas include the inferior
Sc.erri & SchJ.Ute-Koene, 2010). frontal, superior temporal, parietotemporal and middle-
temporal-middle-occipital gyri (Shaywitz, Shaywitz,
Cognitive factors Re.search on the aetiology of dys- Pugh, Mencl et al., 2002). This represents a common
lexia has recently converged on the view that reading finding from functional brain imaging studies suggest-
disabilities in dyslexia are caused primarily by difficulties ing that a failure of proper functioning in left hemi-
in di.ffere.ntiatiIJg the elements of speech (phonemes) sphere posterior brain systems is a cause of impaired
an<l associating these sounds with the letters in a reading in children \\ii.th dyslexia. Studies of lesions of
\Vrimm word (Shaywitz, 2.00-3 ) . This is known as the the temporoparietal a.reas of the brain also indicate that
phonological theory of dysle..'"{ia where, in order to learn this area may be critical for analysing the written word
to read. .t he child must and transforming the symbol into the sounds associated
phonologic-al theory The vtew that Ieam to r~cognize that with the linguistic structure of the word (Damasio &
madlng dl56h11ltres ln tlys\e;t(ii.t are t:aused letters and letter strings Damasio. .1983; M.cCandliss, Cohen & Dehaen.e, 2003 ).
pmnarily b)l d1ffirulhes in diffeientiating
rhe elc?ment~of s~h (phonemesJ and ·
represent the sounds of lnterestingly. brain imaging studies also suggest that
associating theie ~\.Inds with the letters, in spoken language. The individuals with dysle.-tia may attempt to. compensate for
a wntti,1 WOfd. deficits in dysle.tja impair the lack of function in the temporoparie-tal areas. of the
the child\, abilitv to break brain by using other brain areas ro help the.th id~ntify
up a:spoken word into i~s baskphonological el~ments ,,'O:tds an~ -assoc.iate them Vi.11th sound.s. These (:Ompen-
and fink each letter to its corresponding sound, This satory effects involve brain sites required for physically
.11·~tuh\tm~ :1 word, ('1h1bling lh\,, in:d.iv1dt1<\I wh h dys,
lt~I~
·
t n dev1.:lnp an awa1•c.n.ess of rb,-· si ,,.
Llf .i ,vnrd
•
bv• 1-ornung
.
-t
" · '- '" ··(,urn. snucttn·c
the WQl'd wi'·t\, ti'\, · . I
CHAPTER 17 NEURODEVELOPMENTAL DISORDEJlS
(Alexander & Slinger-Constant, 2004). However, with ,1uditory feedback it gives the s1.tlfrl't'I' wht'.11 s1w:1king
older individuals suffering from reading disabilities sUl:h or through a change 111 freque ncy of the voice . Sud1
as dyslexia. a common approach in educational settings is devices appear to have success in rcdm ing the h~wl&
to provide learning materials in a form that allows them of stutte ring, hut it is still not dc~ir by whJ I' nwr ha •
to be most easily negotiated by the dyslexic student. In nism they have this effect or whether t.hry work equally
addition, to compensate for the fact that the dyslexic stu- well for everyone with a ::.tuttering problem ( Lincoln.
dent's reading is less automatic and more eftortful. extra Packman & Onslow. 2006; Lincoln, Packman , Onslow
time is given during assessments such as examinations. & Jones. 20 IO). Ano! her successful set of techniques
Treatment of communication disorders is normally u sed to address stuttering is known as prolonged speech
the domain of speech therapists and related disci- This teaches the sutlerer a set of new speet:h pattern~
plines, and there is a range of successful n·eatment pro- that result in changes in the phrasing and arriculatton of
grammes and equipment available for disabilities such speech and of the respiratory patterns produced by !>tut
as phonological disorder and stuttering (Saltuklaroglu terers while speaking (Packman, Onslow & van Doorn ,
& Kalinowski, 2005 ; Law, Garren & Nye, 2004) . For I 994). The success rates of treatments frn stuttcti ng
example, himd-held equip- are particularly high and estimated to he around 60-- 80
altered auditory feedback (AAF)
A form of treatment for stuttering in which ment can provide altered per cent, but this may at least in part be confounded
delayed auditory feedback or a change in auditory feedback (AAF) by the fact that much childhood stuttering will usually
frequency of the voice Is given to clients for the stutterer, either spontaneously remit after a few years (Saltuk.la roglu &
when they are speaking.
in terms of the delay of Kalinowski, 2005).
• What are the defining characteristics of specific learning disorder as a diagnostic category?
• What are the individual skills that may be impaired in dyscalculia?
• What are the main characteristics of language disorder, speech sound disorder and childhood-onset fluency disorder?
• What is the evidence that dyslexia is an inherited disorder?
~ Can you describe the phonological theory of dyslexia?
~ What areas of the brain appear to be most affected in dyslexia?
., What is the evidence that communication disorders might be associated with physical rath th h •
er an psyc o 1og1cal causes?
~ Can you describe treatments for stuttering such as altered auditory feedback (AAF) a d I d
· n pro onge speech?
SECTION SUMMARY
............. .. ..........................................................\
17.2 SPECIFIC LEARNING 01S·ABILITIES
1
In this section we have reviewed the characteristics, aetiology and general treat t f .
ties. These are largely disabilities associated with reading writing and ~e~ o a number of specific learning disabili-
.
. .
.
,
·
rather than cllmcal settings, they may come to the attention of clin·i I
communication generally and th
these disorders 1s the reading disorder known as dyslexia While ma·ny of th
.
d. b. . . . ' e most well-known of
ese is.a 111t1es will require tt t·
h . b
.
a en ion in educational
ca psyc o1og1sts ecause they ma O ft b .
I
ated with mental health problems and cause significant disruption to . 1f T · • Y en ecome assoc1· !
1 nd
To sum up the key points: socia' am, ia a educational functioning.
~
• Specific learning dl5order refers to a range of disabilities that affect p f ·f
mathematics or written expression. er ormance on tests of academic ability such as read_ing, '
• Dyslexia is a learning disability associated with difficulty in recognfzi
expression.
d .
ng wor s, poor spelhng and difficulty with written !
~
t
j
!
• Dysa:ilculia is a disability that . ff
• Communication disorders inti d . . . art met1c ability.
,a etts mathematical ot I h , ..
CHAPTER 17 NEUROOEVELOPMENTAL DISORDERS
-
~ e 1mpalrments in Ian . . ..
• Language disorder is ad. b'I• guage, speech and communicatIon.
Isa , ity concerned With ,
• Speech and sound d ' d . . problems in vocabulary comprehension and production.
isor er is a disability ass .
• Childhood-ons~tt fl . . . · oc,ated with difficulty in speech sound production.
uency disorder is someti k
p atterning of speech, mes nown as 'stuttering' and involves a problem with the fluency and time-
17.3 INTELLECTUAL DSM-5 SUMMARY TABLE 17.5 Criteria for intellectual disability
- PSYCHOPATHOLOGY
it difficult for them to pronounce words easily. They chemicals is impaired. There are many different type~ _of
also suffer physical disabilit)~ such as heart problems, metabolic disorders and many can affect inrellecrual ability.
and appear to age rapidly, with mortality high after 40 Such disorders are often caused by genetic factors and
years of age. Ageing is also closely associated with signs may be carried by a recessive gene. When b~th p_a re~ts
of ckmentia similar to Alzheimer's disease (see Chapter possess rhe defective recessive gene, then their oflspnng
15) and this may be a result of the causes of both disor- are in danger of developing
recessive gene A gene that must be
ders being closely located on chromosome 21 (Zigman, the metabolic disturbances pre5ent on both chromosomes in - a
Schupf, Sersen & Silverman , 1995; Selkoe , 1991). Dmvn linked to that gene. We pairto show outward signs of a certain
syndrome can be identified prenatally in high-risk par- will provide examples of characteristic.
ents by using a procedure known as amniocentesis which two such genetically deter- ..
involves extracting and ana- mined metabolic disorders that affect intellectual ability.
amniocel\tesis A procedur_e which lysing the pregnant moth- These are phenylketonuria (PKU) and Tay-Sachs_ disease.
irwolVi!S extracting and analysing the er's amniotic fluid. This is Phenylketonuria (PKU) is caused by a deficiency_ of
pregnant mother's amniotic fluid used
now a routine procedure the liver enzyme phenylalanine 4-hydroxylase, which
prenatally in identifying Down syndrome
in high;isk parents. fur pregnant mothers that is necessary for the effective metabolism of the amino
is carried out after week 15 acid phenylalanine. As a result of this deficit, phenylala-
of pregnancy and is recommended in the UK and US for nine and its derivative phe- phenylketonuria (PKU) A metabolic
mothers over the age of 35 years. The results of this process nylpyruvic acid build up in disorder caused by a deficiency of the
can leave prospective parents with difficult decisions about the body and irreparably liver enzyme phenylalanine 4-hydroxylase,
vvh.ether to maintain a pregnancy or not but, even so, amni- damage the brain and cen- which is necessary for the effective metab-
olism of the amino acid phenylalanine.
ocentesis will only identify between 15 and 30 per cent of tral nervous system by pre-
Down syndrome cases in pregnant mothers who are tested. venting effective myelination of neurons (myelination is
Another important chromosomal abnormality that the development of a protective sheath around the axons
causes intellectual disability is known as fragile X syndrome. of neurons that enables effective transmission between
This is where the X chromosome appears to show physi- nerve cells). This results in severe intellectual disability
cal weaknesses and may be and hyperactivity. In the UK, PKU has an incidence of
fragile X syndrome A chromosomal bent or broken, and frag- around I in 10,000 live births (NSPKU, 2004) and it is car-
abriormaUty that causes intellectual dis-
ile X syndrome occurs in ried on the phenylalanine hydroxylase gene (PAH) on
ability where the X chromosome appears
to show physical weaknesses and may be approximately 0.08--0.4 per chromosome 12 (Doss & Sethumadhavan, 2009). Several
bent or broken. cent of all births (Hagerman hundred mutations of this gene have been id.entified,
& Lampe, 1999). Individuals but just five account for approximately 60 per cent of
with fragile X syndrome possess mild to moderate levels of PKU cases in European populations. It is estimated that
intellectual disability and may also exhibit language impair- as many as 1 in 70 people may be carriers of the reces-
ment and behavioural problems such as mood irregulari- sive gene responsible for PKU. At-risk parents who may
ties (Eliez & Feinstein, 2001 ; Zigler & Hodapp, 1991). Like carry the gene are now routinely given blood tests to
individuals with Down syndrome, they also have specific determine the risk of having a child with PKU. Diet
physical characteristics, such as elongated faces and large, is also an important factor in controlling intellectual
prominent ears (see Photo 17.2). Studies suggest there may deficits in fetuses and offspring at risk of PKU. A special
be a syndrome of fragile X chromosome in which different diet low in phenylalanine is recommended for at-risk
individuals manifest rather different symptoms and degrees pregnant mothers and if children with PKU are given
of disability (Hagerman, 1995). For example, some may diets low in phenylalanine from birth to at least 6 years
have normal IQ levels but suffer specific learning disabili- of age, this can minimize neurological damage and
ties. Others may exhibit emotional !ability and symptoms intellectual deficit (Mazzoco, Nord, van Doorninck,
characteristic of autis,n, such as hand,biting, limited speech Greene et al., 1994).
and poor eye contact (Dykens, Leckman, Paul & Watson, Tay-Sachs disease is also a metabolic disorder caused
1988), and around one in three will exhibit symptoms of by a recessive gene (often found in children of Eastern
autism ~ u m disorder (Hagerman, 2006). Intellectual European Jewish ancestry). The defective gene results in
impairment will usually be greatest in males suffering frag- an absence of the enzyme hexosaminidase A in the brain
ile X synqrome because they only have one X chromosome. and central nervous system and this eventually causes
Be.cause females possess nvo X chromosomes the risk of neurones to die. The dis-
intellecrual disability is less (Sherman, 1996). order is degenerative, with T~-5.ttchs disease A metabolic disorder
infants of around 5 months caused by a l'ec;e.ssive gene which results in
Metabof h: disorders Metabolic disorders occur . · ·. an absenc~ of the enzyme hexosominidase
showmg an exaggerated A in the brain and central nervous system,
when the body's ability to produce or break down
startle response and poor eventually causing neurons to.die.
Down syndrome
· OISOROl MS
CHAPTER 17 NEUROOEVELOPMENTAL
-
. .
u)( )( I( )I !
,,
2 3 '"J - J
(( ll II Ji Ii .,
6 7 a 9 10 11
IC II ti ii
13 14 15 16 17
19 l
X V
Fragile X syndrome
.:g,
~
\
E I
]
I /
)
---
. If, tu res o c , ren
· f h •id born with Down syndrome or fragile X syndrome. lnd/v/dua/s with Down
syndrome almost always possess
PHOTO 17.2 The typica/faoa ea an extra chromoso me In pair 21' while In fragile Xsyndrome the X chromosome shows physical
Weaknesses and may be bent or broken.
involves those factors that can adversely affect the fetus ·s or severe bir th defe cts. Up tl> 20 per n: nt of b ,1hics
interuterine environment and its food supply. These born live will have CRS causing he.in db ease. d<~~fnt'~S
include factors such as maternal infections, substance and intellecnial impairme-nt. T he. incidence of CRS is
abuse or malnutrition. Disorders · acquired during pre· bet ween 70 and 170 pe.r l 00,000 live births and inciden:i.;e
natal development are known as (ongenital disorders is still relatively high in developing com1tries (Cum &
congenital dlsorders Disorders acquired because they are acquired Vynnyck:y, 1999). In contrast, maternal HIV infection
during prenatal development prior to birth prior to birth bur a re not has becom e an important maternal HIV lnfec;tlo,, The inc1dt>1Ke of
but which are not genetically inherited. genetically inherited. cause of intellectual dis· a mother havirig HIV during pregnancy,
Maternal diet is one example. For instance, if there ability. If the mother is leading to a Hkel!hood th.it the 1nfect1,i11
wlll be passed on to the fet11s.
is too little iodine in the mother's diet during pregnancy not being treated .for HIV
this can give rise to the condition known as cretinism. during pregna1icy there is a likelihood that the infection
The mother's iodine deficiency may often be caused will be passed on to the fetus . The infection can also b e
by a hormonal imbal- passed on through breastfeeding. The re is then alm,o::;r a
cretlnlsm A congenital disorder resulting d fi 50 per cent chance that the n ewborn child will de,,elop
fn slow development, intellectual disablli- ance called thyroxine e -
t ies and small stature. ciency. Children suffering moderate to severe intellectual disabilities. However, i.n
from this disorder show iitero transmission of HI V can be reduced from 2$ per
slow development, intellectual disabilities and often cent to 8 per cent if rhe mother is given an antiretrovi ·
have a small stature. Thankfully the condition is rela- ral drug such as zidovudine during pregnancy and if the
tively rare nowadays thanks to the availability of iodized newborn child then receives the drug for up to 6 weeks
table salt and the fact that most diets now contain suffi- postnatally (Belfer & Mw1ir, 1997).
cient iodine . Similarly, mineral and vitamin deficiencies A further significant cause of intellectual disability
caused by maternal malnutrition during pregnancy can is maternal drug use during pregn ancy. In many cases
also result in intellectual the drugs responsible for offspring intellectual disabil-
maternal rnalnutritiQn Mineral and
disability and significantly ity may be ones taken for m edicinal purposes (such as
vitamin deficiencies during pregnancy that
can re.suit in intellectual disabilities in the affect the child's physical drugs taken during cancer chemotherapy treatment)
c!iild. and behavioural develop· but most other cases occur where tbe mother is a sub-
ment (Barrett & Frank, stance abuser. For instan ce, US studies indicate that
1987). However, the adverse effects of maternal mal- 18 per cent of pregnant women smoke tobacco, 9. 8
nutrition can often. be partially rectified by providing per cenr drink alcohol and 4 per cent use illegal drugs
new-born infants with intellectually supportive environ- (Jones, 2006). Feral alcohol syndrome ( FAS) is one such
ments and appropriate food supplements (Zeskind & example of maternal cl.rug abuse causing childhood
Ramsay, 1981; Super, Herrera & Mora, 1990). In most intel1ectua1 disabilities. Whenever a pregnant m o rher
Westernized societies maternal malnutrition is rela- drinks alcohol, it will enter the fetus 's bloodstream,
tively rare -but when it does occur it probably occurs in slow down its metabolism and aftect development. If
conjunction with other factors likely to harm the child's this occui:s on a regular basis, then development of
intellectual and physical development, such as mater- the fetus will be severely impaired . Children suffer-
nal drug or alcohol addiction, low socio-economic ing FAS will usually have lower birth weight, low~r lQ
status and possibly maternal HIV or syphilis infection (between 40 and 80) and suffer motor impalrmcnti: and
(see below). deficits in attention and working memory (Niccols,
Maternal infectious diseases during pregnancy are 1994; Burden, Jacobson, Sokol & Jacobson, 2005). ihey
another: potential cause of intellectual disability in the will also freq uently exhibit distinctive facial character•
offspring. Such diseases are most damaging during isrics including slit eyes, short noses, drooping eyelids
the first trimester of pregnancy when the fetus has lit- and thin upper lips. ln the 'UK around one in every
+le or no immunological protection. Common maternal six to seven thousand babies born have PAS (National
· diseases that can cause intellectual impairment in the Organization on Fetal Alcohol Syndrome, 2012).
offspring include rubella (German measles). syphilis and Recently attention has also been focused on the tntel-
HIV amongst others. If a mother contracts rubella dur- Iectual and developmental eflects on d1Hdr~n of illegal
ing the first 10 weeks of drugs use by pregnant moth ers.: Use of both cocaine.
congenitaf rubella syndrome (CRS) The pregnancy, there is altnost and crack cocaine (see Chapter 9 ) by a pregnant
constellation of abnormalit ies caused a 90 per cent chan.ce that mother can lead to babies being physically addicted ro
by infectfr,)l'l with the rubella (German the baby will develop con- · the. drug .before pirth (knm\in a.s 'crack babies'). 11,ere
measles) virus before birth, The syndrome .
genital rubella syndrome is. some. evidence that .this can adver~tc~ly affect physi-
is characteri~ed by mult iple .congenital
mafformatio ns (birth defects) and Int el , .(CRS) resulting in abor- cal development and brajn development in pnt•tkular
fectu al disability. tion, miscarriage, stillbirth (Hade.ed & Siegel. 1989) and re~ult in slow la11gi.tage
CHAPTER 17 NEURODEVELOPM£HTAL l)ISORDERS -
~ cas. ~re o fte n unmarried, live in poverty as a resulr Whitman, Passino. Rellinger et al.. 1992 ; Broman ,
o t their premature motherhood, and have a signifi- Nichols. Shaughnessy, Kennedy et al. , 1987). As we said
cantly lower than average IQ themselves (Carnegie earlier, it is difficult to estimate solely how much this is
Corporation, 1994; Borkowski, Whitmqn, Passino, due to the teenage mother's age and her parenting prac-
Relhnger et al., 1992.). Studies-have shown that teenage tices, because the child of a teenage mother is signifi-
mothers are significantly more likely to punish their chil- cantly more likely to be raised in the kinds of deprived
dren than praise them and are significantly less sensitive environments that contain many other risk factors for
tO their children's needs than older mothers (Borkowski, intellectualdisabilitv.
Whitman, Passino, Rellinger et al. , 1992; Brooks-Gunn & Finally, one imp,ortant feature of deprived environ-
Chase-Lansdale. 1995). As a result. children born to teen- ments is that they will usually provide significantly
age mothers are at increased riskof problematic parent- decreased levels of stimulation for young children,
child interactions, (Leadbeater, Bishop & Raver, 1996), including lower rates of sensory and educational stimu-
behavioural difficulties (Fergusson & Lynskey, 1993), and lation, lack. of one-to-one child-parent experiences and
cognitive disadvantage and educational underachieve- poverty of verbal communication - all factors that are
ment (Fergusson & Woodward, 1999; Brooks-Gunn, thought to be associated with poor intellectual develop-
Guo & Fustenberg, 1993). Consequently, mild intellec- ment. There are some claims that Jack of stimulation can
tual disability is reckoned to occur three times more fre- have a direct effect on the early physical development of
quently in the children of teenage mothers (Borkowski, the brain and so result in permanent impairments to
7
I
TEENAGE MOTHERS AND THE CYCLE OF UNDERACHIEVEMENT
The UK has the highest teenage birth rate in Western • Teenage mothers are more likely to smoke dur-
Europe (Avery & Lazdane, 2008). In 2006, in England ing pregnancy and are less likely to breastfeed,
39,000 girls under 18 years of age became pregnant both of which have negative consequences for
(Department for Education and Skills, 2006). Although the child.
around half lead to an abortion, the remainder become • Teenage mothers have three times the rate of '
teenage methers. They are mothers who are likely to postnatal depression of older mothers and a
have lived in deprived areas prior to giving birth, they are higher risk of poor mental health for 3 years after
often unmarr-ied, live in poverty as a result of their pre- the birth.
mature motherhood, and are likeJy to have a significantly • Children of teenage mothers are generally at
lower than average JQ· (Borkowski, Whitman, Passino, increased risk of poverty, low educational attain-
Rellinger et al, 1992). When teenage girls become moth- ment, poor housing and poor health, and have
ers in deprived areas, this sets up a cycle of deprivation, lower rates of economic activity in adult life.
poverty anti intellectual underachievement (Wildsmith, • Rates of teenage pregnancy are highest among
Manlove, J,ekielek,- Anderson Moore & Mincieli, 2012). deprived communities, so the negative conse-
a
As result of their relatively poor parenting skills and quences of teenage pregnancy are dispropor-
the stress that accrues from living in deprived areas, the tionately concentrated among those who are
chrldren of teenage mothers are significantly more likely already disadvantaged.
than the children of older mothers to have behavioural
difficulties (Fergusson & Lynskey, 1993), and suffer cog- As we can see from reading section 17.3.4 many 1
nitive impairments and educational underachievement of these conditions represent risk factors for intellec-
(Fergusson & Woodward, 1999; Brooks-Gunn, Guo & tual disability and underachievement for the teen-
Fustenberg, 1993). The UK Department for Education age mother's offspring. These include poor parenting
and Skills (2006) provided the following stark facts: skills, maternal mental health problems,, being r?ised
in unstimulating environments abundant in potentia'I
Teenage mothers are less likely to finish their stressors, a high likelihood of maternal drug or alcohol
education and are more llkely to bring up abuse during pregnancy, and increased risk of physical
their children alone rn poverty. abuse or accidents (Moffitt & the E-Risk Te.am, 2002). At
• The infant mortaftty rate for babies born to teen- age 5, the children of teenage mothers already have a
ag.e mothers is 60 per ct>nt higher than for babies significantly lower IQ than the children of older moth-
born fo older mothers.. ers (Lubinski, 2000).
.
··. .
._...... .-.__:. -,---~·--•--- -.....---•·--- ....~ ~----- ·----'----,---- _,. ___ __ ----~--------~ .. ..__..,_. __ _..,
.., ,
1
CHAP'fl!.R 11 NtiUftOtliVELOPM£NTAL OlSOROERS -
br;1ln f\J1,n11_ ,nn1~. llrir .h rnt:.ln,•., , ..
..... . ,,t:' lll a 1 t.lt~\.·r l 1
'
rlw bn 1m t 1H' lll'fi. mnr-t
.
ti.x t~i·,,~i. ,. 1 ..
· '-" n l' Y Hill1l"l!1l lJ , j · h •,,
° 1111 (.•n1 o. 1· n 1!9tti,lil'll l' Ht'<' :He tHJW gom:.• uo(l ptuvu:inn for $UCh pt.o·
vc.i r· Jtti~t· h1n.h (Kl->lh. Wt;tv) .1.. 1 .
, · . . . .
l ' · ' Y u i; c hl'.$ t pit nut 011ly "' tt rn pts tO 1icl.dress. theit· tl(;:.t ds btit ,dso tee•
· •, ,t. i). t 1t , 11t tn t J i
t(.1rnY1cnt 1~ tlftt"m,m·y For furl j ,. . • . • .. ·1 : ,ihtlMe1wl, ognizcs cht>i1' fn11Llanlem.i.l 1'ights 1\s hW'l'l:m beings imd
. • tit:'. Vt! 1npnwnt· of t] · l ,
\ l1'll <-'l ,m· rN<'\l~m1 & lki~(J1w t, 2000). ./\It •, . 'le i 1·n1n s d tlztns to a11 iJ.1du:liVc liC<-styk . Thus. interventions for
1-1.m,tln_·u,it_alinN, stt't'S~ful cuvil'onn••· i ,, u l,,_~tlvdy. ,m i1.1tcllet•t.u.1I disnhillt:ies have a nurnher o f diverse alms. At
. , . • . . . ,,;. ,.: C~ll'\ urtu,uly ri . · , .
\IW )il 'f'J'r tlw1 t.,t hrm11t,,1.. ~ tl· 'lt 1· ' •.
.• .,, . H
r~ I 1--1ggt
,1 ~Vl' !1t l' ·rt ('tt ·•o
I 1-hl' pt·lt11,try level the re· a re tho~c interven.rton.s .ii111ecl at
,trvdf1r,mcnt (Ch,lnliiW 1,JqK) . : • 111 • vc 1,lin 11revl.' nllng irHclk c1ual disability in the first pfoc-e by edu-
~ · · .1 ~rucly ct11·1t•, · l't , ·1 11 rc1tlng pntc11tictl p.u·cnts about the risk factots fm i ntel·
d!'t'll hrnug.hl up In ct_ t'f1riwd lttnt r ··It·\/ ..• . l ,1 ,1g t- l
.. .,,1 d . 1 I . , c 1 ,n c.u1wM1a 1,rotm lel.·tttal di!iahHtt y (r .g. educating parents about the effects
pwvfo l.' w,11 guod nut 1·1\ i<Jt1 an -I . ts .1 . " ~
. ·•, . l 11 , ". I:\ s i111t 1 I.I ting env lton nf mat<:t·n~l alnihnl i:md drng ahllst dur'ing pregnancy).
ruc-nl, c ,mp H~ & lt"m~cy ( 1Ol)-4) fie,• •d h· . .
, ' j 1 . • , . · ' · , It 1 · I. t\ t 1 ) Y I l Yeut1, /\ tiCt'nnd bro.id ail'l! (if interventions is to make training
of aw· r ).~ l,.l tl f'l'lV,.HlOl'l l'XJ'crit·n tt? I by t·h. f' . .·.· . "
· 'f' ~ C Urt\1(:: J' g t'(llll1 prognmnws ~wnilnble th,H will provide th<' suffeter wirh
hacl ~ ~JJ~nl }c-.un IW~;HiVt' dlert. 'ltl ""
l')I"rl i·n. J"I Ut'J.(ti<.ming,
. . ·t
etmugh hnsk skills to ropt' wi.th tnany nf the challenges
ur evet'yday life (t. g. :-;elf-help skills. communication
Summary of the a~tlology of skills) Thirdly. appru.irh~s to hclpb'.1g thost with intel-
Intellectual dlsobllltles lectual dis,1bilities Me based on the principle of inclusion
From tlw lll,tf:1.•rhll
, cuv(•1·rd
· in •'·),1"~ ,~i·•c.: ti on,
. you can in an altPti.1pr ro help strch lntluslon Str,Hegle s intf.'!nded to te.ich
5t l."
rhal the l' i.lU fltS \ii l11tdknt1al disablHLy :u e divt:rsC'. A~ we individuals achieve their high functioning Individuals self-help
1T1cnt
•
ltmcd rJ d tt't,
, . , • ., , ,
v_ttry .~,ft~11
. ., , :
ii ·is ,in\tto~"'t
•. , , • " ·
potcnrial. Por cxam1,k. in strategl~s. social ond living skills, ond ~elf-
'lJlc· 1.,. [ 1· •
.v 111potnt
tlw UK, sthools are now n1t1nogemcnt that are dE:signed to help the
t.ht ~pt ~iJH,_ t •~wa: _ot m~indw1dui1l s 1nt.elkrtu::.1I dl:.nbllity, lndlvldUill fun c.tlon more effectively
but 1ntcllect1.ml d1sabH1ty t.n1:wd by dm..n1iosoitrnl diso r rt•quired tu draw up acces In 5odety.
dt!t'S ymch .is Dc)Wll ~yudrciml~ ut1d fragile x syitdl'onie) slbllity sl'. rategies to allow
M1cl mhcr1tei.J ntt'.tabolic <.Usordcri, tll't somt of 1hl: rnort· pup.ii:- with iritcllet tuul disabilirks to engage in the edu-
easil y ,~fontifi~cl.' Individuals ar~ most at rlsk c_if develop, catiom1l process without being disadvantaged. Similarly.
ing pcnnan cm 11\tt lk c' t tt.,l disabilitks during early dr vcl sodul inclusion is also ~11eolu·aged to providt' chose with
opmc~l of their ttntn tl 1i C.l'Vli \.1:. systc111, whlrh is why i11tcllC'Ctual disabilities the opportunity for pcrso11.al,
rnndlt1ons in tht ut ~rns and in the lt111ne<llatt po:muual ~m•ial. cmurkmnl and sex1.:1al dcvd()pmcnt. We will now
pc1fod arc ,·ri1 ka·1 for t1(H'n1(1 l drwlopm rnt . RJsk fac tors discLtss each of these three types of approach to i.ntet'-
rlrnr can di:wupt'. nornut l prc11,1tal dewlopme1\l cJf the vcnrfon it, more detail.
brain Mld rt•ntr.tl nl'.'rvous sy:.tr m include rnatt•rnal infcc•
tfonii. t1kohollsm, ~lrug .lhUs<' and rrrnh1utritlon . tfa rly Prevention strategies
diildliood f'acwrs th~1t nm affect normal neurologkal 'l';thlc 17 .2 lists rmmy of the nn.1ses a nd risk factors for
dcvcfopmem in.dude ucdc.knts, physical abuse, ex.po· ir1tellcnual disability and you c.::an probably glean from
sure tn 1·oxil1s, in foc tlo11s illnesses -1nd an early rhlklhoud this list that mnny of these en uses are pott:-ntially prevent-
spent In. deprivation und povt:i:-ty, As we hnve mentioned able. This is particularly the c:as~ with many perinatal
many tiines in th.ls sec:tion, 1nany of th(•St risk factc1:>rs causes, and espedally those itw<>lving m:.1tcmal factors
may operate wncurrcntJy to dcn:rmine lewi s of intcl during prebll"lancy, For e,.-xample, furn} alcohol syndrome
l~ctu.:i l c..li:wbllhy. (RA~) ls a signific1mt cause of intellectual disubility and
pre\: ntln? program.mes nim at identityl.ng those won:1cn
'.lt .,.risk _of ak:ohol abuse during pregnancy n.nd provid-
1t1g inter~c~mons such ,\s akohol•reduction counselling
17.3.S Interventions for (Pluy_d, 0 ~ otmo1~ Bert1~.tt'Ld & Sokol. .2006), Recognizing
thos~ at t'ISk e:.m. b~· achieved by u sing established din.g-
Intellectual Disabilities no:mc ~nd scrcc11mg qnestionnrurcs (lsrnail, Budcll..-y,
Most: forms of l11tclle.(~rtrnl db1ahlliry impose HtnirntfotlS Uuuadu. Jnbbar &. Gallicnno, 2010) t1nd interv1;:ntfons
on tht' i.uflh·cr'R ability w ftirtetion foUy and act'iv~ly it~ ~nclude ~rnvidlng fet.>dbark on l'ates of drinking beh:w-
aoctct.y. Thi~ means that' - depc11.cli.ng on the seve:11cy of lOllt durmg pt'cgnarn.:y, discussing strategi~s for ~wo.i ding
the di.sabiliry ,. the irtdi\lic!~al will m:r:d.suppcw~ to ,~opf akohi:>1 cravings a11.d bJnge drinking se~lons and, rnor't~
wlih nw 1y of lht> rigour.!I r,f c·veryday living. A~ a ~t:sL~lt recently, . we~~bas.ed. illte1·ve,n tigns .(Tel'lkku. Mengel.
uf dw c.!isah!Uty. i-uffcret'!'l .1re at rl~k of u.n~t..,·ach1evmg m ~icholsm;1, H1le etaL1 2011) . Comrolled-<:.~mpari.son i ttld-
rno1r1y iu·ra:- of thdr lifo, indtidi.ng rducauor.rn.11~, 01::1.1pa 1es suggest tha,t i.uch screening a-rtd itttervention met.hods
t!c.mally, ~C()Momi('aUy and ~udally. rn rn~s.r soe1ftcl~~\ ~~e. -s.~gt~ifkant.ly •·~,t~ce the ~k for al~ohol-ex:posed pregnat'i~
day~ wht;-t\ pc.opk with inrt 11e~t.,lal .<l~sab1!t-ti~s ~.ve~e smi- des conip.lr~d ~th non•m.tctvcm,on control p,n-ricipam~
(Lngersr)ll, Cepern:h. Nt!ttlen:1an, K.a.r:,ndn ct a1., ZOOS) .
p1y ln~t.Udonalizcd. and provided wLth little 1note th:m
- - PSYCHOPAllfOl.OOY
Pien~nnon. cm al:ro be ~~ in a number of ocher Behavioural techniques that adopt basic principles of
'""'~~ for aample, gen-erx ar.a.lyS'is and counselling ena- operant and classical conditioning are used extensively
bt5 ~ parems at risk of abnormal births to be :idemi-- in these contexts, and the application of learning theory
ficd. ifefonned of the r<Sk and counselled. about how to to training in these areas
,pplied .b ehJviour .inalysis Applying
proceed. Blood rests and tests of amniotic fluid such as is also known as applied
the principles of learning. theory (par-
~ r e s i s enabte. parents tO be informed of risks for behaviour analysis (Davey, ticularry operant conditioning) to the
a range of diwr-d.ers I:ru-luding Down syndrome. Tay- 1998). Basic techniques that aS5essment and treatment of individuals
~~•. phenJlk.etonuria and intellectual disability are used include operant suffering psychopathology.
~ b_r rongenir.tl rtilietla syndrome 1CRS). in -addi- reinforcement ( rewarding
tion, dlo:se_ dtsabilitks related to dietary irregularities cancorrect responses - for example. 'INith attention or praise).
~ be ide:rumed and treated, and me.Se include provid- response shaping (breaking down complex behaviours
ing aNWc pregnant mothers Ywith iodine supplements into small achievable steps and ·then rewarding each step
to pr~! cretinism and providing diets low in phen~·- successively), errorless learning /breaking down a behav-
f ~ ror pregnant mothers and offspring at ris:k of iour to be learnt into simple components that can be learnt
p,.,.1ienrkewnum, without making errors - errorless learning is stronger and
Finally, ai dis:cussed earlier_. conditions associated more durable than learning with errors), imitation learn-
,1,-nh ~-e~- and social depri,ari-on also put children ing (where the trainer demonstrates a response for the
:.n. mk of educatimr.J underachievement, tower than client to unitate), chaining (training the individual on
a.-a:age IQ and mild intellecrual disabiliC:4 and support the final components of a task first and then working
~ogv.mnru:'S in the USA and Europe have been devel- backwards co learn the earlier steps) and self.instruc-
oped ro try to counrerai...--r this risk factor. For example, tional training (teaching the client to guide themsel ves
funn_ty ruppa:-t programmes in the USA have indicated through a task by verbally instructing themselves what
mar mothers of fow .socio-economic status participat- to do at each step). Very often, inappropriate, life-threat-
mg in s-..:ch schemes are tnore affectionate and positive ening or challenging behaviours may inadvertently be
wid1 th-eu children and provide more stimulating env1 • maintained by reinforcement from others in the environ•
r<-W..ments ilian mot:heTs \!;'IJO are not in .such schemes menr f e.g. self-muu1ating behaviour may be maintained
•Jona-~ Wafker. & Rodiiguez. 1996). Being a teenage by the attention it anracts from family or care staff). In
motl:-..er ~ also a risk facror for drildren of lower IQ than these cases, a functional analysis can be carried out to help
:n'erage i ,ree fi::;.cus, Poim 17,I ; and this can be tackled identify the factors maintaming the behaviour, and this is
ma rnunher of wa;,s, in.dud.mg pr0viding teenage girls done by keeping a record of the frequency of the behav-
,;;;iili a:hrkr on md access to contracepoon, improving iours and noting the antecedents and consequences
ieen.-ge mothers' access ro educanon (Department for of rhe behaviour (see Treatment m Practice Box 17. l ).
Chlkiren. Schoo-is & Families. 2010), impr~ing housing Once 1t is known what consequences might be maintain-
quality ,1nd ~ me preserr.e of a co-residenrial mg the behaviour, these can be addressed to prevent the
p.a~ radtu Ehao rasng a child alone (Berrington, behaviour being reinforced (Mazaleski, Iwata, Vollmer,
Diair..ond, Jngbam, Stevenson et aL, 1004J Zarcone & Smith, I 993 ; Wacker, Steege , Northrup,
Sasso etaL, 1990).
Tn,lnlng procfllures
The quahty of life of people ""irh inrcl1ecrual disabili- Inclusion strat~les
ties cm be impr-m-ed s.1gnificandy ~ith the hdp of basic Policy on the development and educ.arion of individu-
~ g p rocedures mar will equip them with a range als with imellccrual disabilities has changed significantly
of skilli dtpendmg on their·!~'el of dis.ability, Types of over the past 3.J years. Prior to inclusion policies being
skills mw!d- .sdf-hefp and adaptive skills (such as- toilet- introduced. even individuals with mild intellectual dis-
ing. feed>.ng and ttrc"fflllg,. languagc, and cornrouruation abilities were ofi:en deprived of any effective participa-
s&;iB.s •incl!.~ .g ~ . comprehension, sign lang..1age\ tion in the society in which they liv.ed~ and more often
ici~ and reaeational skills (such as play,i.'lg g~mes,. than not they would be institutionalized or educated
~ s b T u;,. basiic:da-iiy lit.ing skills ~tb-fug.a telephone. separately. However. many countri~s have intro4uced
handling m<mey1 ati-d ftonttoffing: anger outbursts and. accessibility strateg:ies that extend the rights of individu-
~gress.ivie and cbafknging behaviour ~reducing the: ter-1- als with intellectual dis.abilities. to be educated ac-co-rd-
df;n,cy ro communicate through aggressive or cha.Heng- ing to their needs in w.;ainstream schools. This approach
mg beh2~~ sfil"h .as pri,...shmg or shouting_,. Trnnmg evahl;,tes the inditidual's specific. needs and then s:ug-
merbock can :Jw be used in more ~1::re cases to rnn- ge~ straregies, senices and supports that will optimize
troi hf.e-dJreatening behaviours such a! .self-mutilation err the tunctioning of these individuals v.i.thin society: In the
t't'~ .1-N~, UK. the government's straregy for individuals with
CHAPTER 17 NEURODEVELOPMEMTAL DISORDERS 581
A fUNCTIONAL ANALYstS CASE HISTORY in the lounge when there were a number of people in
the room and t he tetevislon was on. The consequence
Andy i& a middle- aged man with severe intellectual of Andy starting to rock c1nd slap himself was that staff
disability. He has re.cently moved Into a group home would remove him from the room and ta~ him into the
which he share$ with seven other people. Since he kitchen where it was empty and qui.e t.
moved in, stQff ha:ve observed several Incidents o f It was hypothesized that the function ofthe behaviour
self-injury. They report that Andy starts to rock back• for Andy was to escape from a noisy and crowded situa-
wards and forw.a:rds in hls chair, and this then escalates tion. Staff decided to respond by watching Andy for early
into him slapping his face repeatedly. Staff have asked s1gns he may be feeling overstimulated and to ask hhn if
he wanted to leave the roor:n.
for hefp. This led to a reductJon of self·lnj1,1ry and later·It was
The staff team· work,nQ with Andy were asked to com-
noted that Andy would ·now·try to attract staf:f atteotfon
plete ABC charts for 4 weeks. These were then analysed
and it was found that the behaviour tended to ow.1r when ht wanted to leave.
living t <) living in the community. Most studies reported have opportunities ro pursue social, educational and
r10 improvement in three areas: (I) overall improvements occupational goals and pursue their own personal dev~l-
in coping with day-to-day living and increases in self- opment. Por example, individuals with intellecrual dis-
csteem. (2) a decrease in the frequency of maladaptive abilities now have the right to pursue their own sexual
hl:'haviours, such as aggression o~ anger outbursts, and and emotional development - usually with the support
(3) aJ1 improvement in self.care behaviours and social of their family. Whereas in the past involuntary sre_rili-
skills. Case History I 7.1 recounts the story of Thomas, a zation was common for such individuals, appropnate
0(>Wn syndrome sufferer, who lives with his family and training and counselling now means that most of them
has benefited in a variety of ways from participating in a can be taught about sexual behaviour to a level appropri-
range of community activities. ate for theit functioning. This often means that they can
fnclusinn policies have resulted in significant improve- learn to use contraceptives, employ responsible family
me11ts to the quality of life experienced by individuals planning, get married and - in many cases - successfully
with intellectual disabilities, and such individuals now rear a family, either on their own or with the help of
THOMAS'S STORY
Thomas is 23 and lives with his mum and dad. His brother now lives away but sees him quite regularly.
Thomas has Down's syndrqme and needs a great deal of support. He goes to college four days a week and, on
Fridays, attends a project where he is learning living skills and enjoying cooking. Thomas has a supported work
placement for two hours a week in a riding stable. He has a hectic social lrfe with weekly activities including rid-
ing, sports, going to the gym, trampolining and football. He goes to monthly discos with a group of young people
with learning disabilities and is regularly to be found in the local pub playing pool with his friends.
We were transporting him to and from these activities and were concerned that he should be. able to mix
more with his own age group. We arranged for 15½ hours' worth of direct payments for Thomas to choose some-
one in his peer group to help him access these activities.There was a great deal of interest in the advert we placed
at the local university for a student to help with this and we have had several different students helping over the
past year, who have become firm friends. Thomas's current helper, Laura, accompanies him on his outings and
Thomas has oow become a part of a wider social circle, going to the pub, out for meals and watching videos at
Laura's 'hollse with her friends, which he greatly enjoys.
Thomas's moods, self-esteem and well-being are greatly improved by the stimulation and social nature of all .
that he does, as well as the routine and structure it brings to his life.
Thomas and his friends have gained enormous confidence from attending several drama ·courses and
the group has enjoyed the feeling of empowerment and also the opportunity to show their feelings. La5t year,
a group of 11 young adolescents, including Thomas, attended a week-long outward bound course, run by the
Calvert Trust, without their families. Afterwards, the group made a presentation to about 80 people who had
been involved in organiZing or fundraising their trip, with a very professional PowerPoint presentation and
question-and-answer session. They were all keen to contribute, wanted to find other groups to make t heir
presentation to and gained lots of confidence from this. It makes a change from the usual painting eggs and
bingo offered by local services, which are just not appropriate for a 22-year-old.
Source: From evidence from a family carer given to the Foundat1on for People with Learning Disabillties' tnquiry into Meeting
theMenMI Health Needs ofYoung People with Learning Disabilities - Count Us In.
Clinical CQmm~ntary
Thomas rs an example of how individuals with intellectual disabilities can bene.fit significantly from acces-
sibility and inclusion strategies. He has work in a supported employment setting and has a full social life
in which he can mix with people of his own age. This approach has the bf n,_efit of building confidence and
self-esteem, as well as pmviding the individual with a real sense of empowerment.
CHAPTER 17 NEURODEVELOPMENTAL DISORDERS
Jsf.iF. r&sT-Ques.noNs· -; :
'\..:., . ~ ., '
• Canyou descn·be•bo th the trad. itional and more recent alternative approaches to defining intellectual disability?
• What are the different levels of intellectual disability defined in DSM-5?
· ch romosomal disorders that cause intellectual disability?
• What are t he mam
• Can you descn""e -,1.. at Ieas t t wo metabolic disorders that give rise to intellectual disability?
SECTION SUMMARY
17 .3 INTELlECl'UAL DIS"81~;.~~-- - · - ··- - - - -·- ' . " . ' .• - " - .. - ·1
· ,. · · ''. · . . . he DSM-5 diagnostic criteria for intellectual disability and di~cu.ssinQ some alternative \
We began this section by desc:nbmg th t focus on identifying. needs and facilitating adaptive functioning. The aetiology Qf . i::',
types -of defipition and terminology t_ a ith no identifiable ca~ being found for a large proportion of those with-intel~
· · · tw:i.·<;. w. i .arJly-responsible
. intellectual di_sorder:s ··JS .a ·dive{se · ·for those aet10=11ll:?
· t,..,.,• t'-.. at~an-~ ·,.., ·fieu,
'-- 1uentj ,4
and· th.ese m,
· Iud e
· t 1.i;_._.. "'"'logical
.ec-rua. Idisab"f't'=
1 0 "' causes are pr m .. . '
- PSYCHOPATHOLOGY
chromqsomal disorders, recessive gene disorders and perinatal factors. Childhood problems can also contribute to intellec-
tual disability and we discussed the role of accidents, abuse, infectious diseases and the nonspecific detrimental effect that
social deprivation and poverty can have on intellectual development. In the final section we covered interventions for the
prevention of intellectual disability and programmes for the care, development and support of individuals with intellectual
disabilities.
To summarize the key points:
• Intellectual disability is a term that covers impairments in both intellectual and adaptive functioning.
• Intellectual disability involves significantly below average intellectual functioning, usually defined by a score on a standard-
ized IQ test of below 70.
• Modern approaches to defining intellectual disabilities attempt to highlight those factors that might be required to facili-
tate more adaptive functioning, and to draw up accessibility strategies to ensure that such individuals are not excluded or
disadvanta~ed in their education.
• Chromosomal disorders such as Down syndrome and fragile X syndrome account for around 25-30 per cent of all diagnosed
cases of intellectual disability.
• Metabolic disorders that cause intellectual disability are usually carried by a recessive gene and include phenylketonuria
(PKU) and Tay-Sachs disease.
• Congenital disorders are those that are acquired prior to birth but are not genetically inherited. Congenital causes of intel-
lectual disability include maternal malnutrition, congenital rubella syndrome (CRS), maternal HIV infection and fetal alcohol
syndrome (FAS) .
• Childhood environmental causes of intellectual disability include childhood accidents (including intentional physical abuse
by others), exposure to toxins (such as lead), childhood infections and poverty and deprivation.
• Prevention strategies for intellectual disability include prevention campaigns and screening for such factors as maternal
alcohol abuse and genetic risk factors.
• Behavioural training procedures can equip sufferers with a range of self-help and adaptive skills, and the application of
learning theory in these areas is known as applied behaviour analysis.
• Inclusion strategies provide those with intellectual disabilities with access to mainstream educational and occupational l
· ·· ···~: ~~~.~.'.~:.~~.·................. -.........................................................................................................................................................................................•)
Clinical Commentary
. ly age Adam exhibited symptoms of the triad of impairments typical of autistic spectrum dis•
From a very ear , . nJ . . • • • • ( k f·
h .. (1 ) no sign of engaging m ore oying rec1,proca1socw1interactions e.g. the 1ac o interest
0rdflJ, _He -~ ows_th thers at his birthday party), (2) a significant delay in the development of spoken speech
In soc1allzmg w1
.
° I
ed b his (allure to form word sour,ds, lobe objects or _
h. . ·
ex_press . 1s wants and _n~eds), ~nd_(3) a lack of
st
(,Hu : t0
Y d fl 'b/lity of thought (as demonstrated by his mablltty to use toys m 1magmat1ve play and
imagination an . ex1 .
bis inflexibly stereotyped behaviour towards these toys). . :