ACQUIRED CHILDHOOD APHASIA (ACA)

• Acquired aphasias of childhood are those language disorders that

appear after a period of normal language development and are secondary
to cerebral dysfunction.
• Child Speech-Language Disorders can be divided into developmental
disorders and acquired disorders (Ludlow, 1980).
• Developmental disorders are those which onset prior to the emergence
of language (i.e. between birth and one year of age). Consequently,
children with developmental language disorders have never developed
language normally.

• Developmental speech-language disorders can, however, occur

secondary to conditions such as peripheral hearing loss, mental
retardation, cerebral palsy, child autism, birth trauma and environmental
deprivation.

• Acquired speech-language disorders, on the other hand, are

disturbances in speech-language function that result from some form of
cerebral insult after language acquisition has already commenced
(Hecaen, 1976).

DEFINITION

 Acquired Childhood Aphasia is defined as a language disorder secondary

 to cerebral dysfunction in childhood, but appearing after a period of
normal language development (Lees, 1993).
The cerebral dysfunction may be the result of:
• a focal lesion of one of the cerebral hemispheres or other area primary
to language processing
• a diffused lesion of the central nervous system above the level of the
brain-stem, secondary to head injury or cerebral infection
• a diffuse lesion as in 2, but related to convulsive activity
• unknown etiology

• Typically, the affected child has commenced learning language normally

and has been acquiring developmental milestones at an appropriate rate
prior to the injury. (Murdoch,1999).

Incidence

• Robinson (1987, 1991) reported two series of children: One from a child
development center and one from a school for speech and language
disordered children, which suggested that acquired aphasias accounted
for between 4% and 7% of cases of language impairment in children.
 PROGNOSIS

• With the introduction of new techniques of brain imaging and the

development of linguistic & neuropsychological tests, the literature
suggests that recovery in children is not as complete as often stated.
• Factors that may influence the recovery of language in ACA include site,
size and side of lesion, etiology, associated neurological disturbances, age
of onset, type and severity of aphasia, and the presence of EEG
abnormalities.

• Satz and Bullard-Bates (1981) reviewed the literature relating to the

prognosis of ACA and concluded that, although spontaneous recovery
occurs in majority of children, 20-50 % of cases still present with aphasia.

TRADITIONAL TYPES OF ACA

• Whereas, CVA are the most common cause of aphasia in adults, TBI is
the most common cause of ACA (Murdoch,1999).
• Children with ACA who do not fit into the traumatic or convulsive
division can be termed as “anomalous or atypical aphasia”.
• It can be a condition where the traumatic and convulsive mechanisms
 coexist’or other conditions in which the loss of language skills in
childhood may be 1 symptom, e.g., late onset autism, Rett syndrome (Lee
& Urwin, 1991).

ETIOLOGY

Traditionally researchers have divided these language problems into two

broad groups:
• Aphasias of traumatic origin
• Aphasias of convulsive origin

Clinical features of acquired childhood aphasia

Although there is some variation between early and recent reports in the
literature, the symptoms of ACA include:

• initial mutism followed by a period of reduced speech initiative;

• a non-fluent speech output; simplified syntax (telegraphic expression);
• impaired auditory comprehension abilities
• an impairment in naming;
• dysarthria; and
• Disturbances in reading and writing

• Most authors suggest that fluent aphasia and receptive disorders of oral
speech such as literal and verbal paraphasic errors, logorrhea, and
perseverations are only rarely found in children with acquired aphasia.

• There is, however, evidence to suggest that the age of the child has a
role to play in determining whether or not these symptoms occur in a
particular case

• Non-fluency as the most prevailing clinical feature of ACA (Satz &

Bullard-Bates, 1981)
• Alajouanine & Lhermitte (1965) found that the predominant features of
the acquired aphasia demonstrated by children at <10 years of age
included decreased auditory comprehension, severe writing deficit, and
no logorrhea, paraphasias or perseveration.

• However, ACA > 10 years of age is a more fluent form of aphasia, with
paraphasia present, less frequent articulatory and phonetic disintegration
and disturbed written language.

• Despite pronouncements that receptive disorders are rare, few recent

 studies have reported that syntactic comprehension is more impaired in
the acute period following predominately left-brain involvement.

• Mild lexical deficits have also been reported.

• A reduction in all expressive activities including oral, written and

gestural activities has been reported to occur in children with acquired
aphasia (Alajouanine & Lhermitte, 1965).
• Hecaen (1983) carried out a retrospective study involving 56 acquired
aphasic children ranging from 3.5 to 15 years of age, with brain lesions
resulting from a variety of etiologies including trauma, tumour and
haematoma
• Hecaen found mutism to be the predominant clinical symptom in the
initial stage post-onset, occurring in 47% of his child acquired aphasics.
• Following the period of mutism when speech returns, there is often a
period during which the aphasic child is unwilling to speak (Guttmann,
1942; Alajouranine & Lhermitte, 1965).
• This period has also been described as representing a loss or reduction
of speech initiative (Hecaen, 1976, 1983).
• Increased incentives and encouragements are required in this period to
get the child to produce even the few words they are capable of
producing.
• Alajouanine & Lhermitte (1965) proposed that the suppression of
spontaneous speech might be the result of a psychological reaction
experienced by children in response to their inability to communicate.
• Further, the authors noted that children tend to isolation, refusal and
 silence in response to conflict or difficulties.
• The unwillingness of aphasic children to speak after speech returns is
similar to the behavior of normal children when faced with a difficult
problem they cannot solve and wish to put aside.
• Although a number of authors have reported that children with
acquired aphasia exhibit telegraphic expression or simplified syntax
(Bernhardt, 1885; Guttmann, 1942; Alajonanine & Lhermitte, 1965).
• Aram, Ekelman & Whitaker (1986) examined the spontaneous spoken
syntax of 16 children with acquired cerebral lesions to either the left or
right hemisphere and compared it to that of a group of appropriately
matched control subjects.
They found that children with left cerebral lesions had a shorter MLU,
lower developmental sentence scores, lower % of total sentences correct,
a fewer no. of main verbs & interrogative reversals, fewer sentences
containing conjunctions or embedded clauses & produced a greater % of
complex sentences in error compared to the children in the control group.
On the other hand, children with right cerebral lesions had a shorter MLU,
produced more simple sentences in error and more negatives than the
control subjects.

• Early researchers in this field considered impairments in auditory

comprehension to be a rare occurrence.(Bernhardt, 1885).
Alajouanine & Lhermitte (1965) reported the presence of auditory
comprehension deficits in about one-third of their ACA cases. It has been
suggested by Hecaen (1983) that, when present disturbances in auditory
comprehension, occur almost exclusively in the early stages post-onset of
 aphasia and disappear rapidly and virtually completely.

• Diminished verbal stock or an impoverished lexicon is another

commonly reported symptom of ACA (Alajouanine & Lhermitte, 1965;
Collingnon, Hacaen & Angerlerques, 1968).

• Hecaen (1983) reported that 44% of his sample of acquired childhood

aphasics had naming problems (not of a paraphasic type) which are
commonly present when the child returns to school and consequently is
often mentioned explicitly in their school reports.

• Hesitations have also been noted (Berhardt, 1885; Guttmann, 1942). It

is possible that these are the result of either the dysarthria sometimes
reported to be present in these children (Guttman, 1942; Hecaen, 1976,
1983) or alternatively they may reflect the word-finding problems also
reported to occur in this population.
• According to Hecaen (1983), the factor having the greatest influence in
determining the occurrence of dysarthria is the localization of the
underlying lesion.

• He reported that 81% of children with anterior lesions exhibited a

dysarthria as part of their aphasic disturbance,

• while only 20% of cases with temporal lesions exhibited with paralytic
and dystonic features.
• ‘Reading problem’ is often included by in the list of symptoms of ACA.
• A writing deficit is also a commonly reported. Alajouanine & Lhermitte
(1965) noted that child subjects with acquired aphasia had a severe
disorder in spontaneous writing, writing to dictation and copying in over
half of their subjects.
• Dysarthrographia (the misspellings often being based on phonetic
disturbances) was also observed in the spontaneous writing.
• ACA has over past years, been generally regarded as being of a non-
fluent type, a number of publications in more recent years have
documented the occurrence of an initial fluent aphasia in children
(Loonen & Dongen, 1985).
• In addition, earlier studies also stressed the absence or rarity of
receptive speech disorders such as paraphasias, logorrhea and
perseveration, especially in children < 10 years of age, more recent
reports in the literature have documented the occurrence of these
features in the spontaneous speech.
• Van Hout, Evrard & Lyon (1985) found paraphasias in the language
output of the children once speech re-appeared after a period of initial
mutism. Furthermore, they studied, the evolution of paraphasias over
time.
• In one group the paraphasic errors resolved within a few days, in the
second group paraphasia resolved in a few months and in the third group
paraphasia was still present at greater than one year post-onset.
• Although it is not immediately clear why the clinical features of
acquired childhood aphasia reported by earlier researchers (e.g.
 Ajajouanine & Lhermitte, 1965) differ from those of more recent workers
(e.g. Van Dongen, Loonen & Van Dongen, 1985),

Assessment of Speech and Language in ACA

1. Few specific assessment techniques are available. Therefore, the clinician

needs to be informed about the use of the most appropriate ones for the
child’s age, background and needs.

2. A complex association of motor, cognitive, perceptual, emotional and

communication problems can arise from brain injury in childhood and
these make demands on the assessment procedure.

3. The deficits encountered in ACA are likely to require different assessment

techniques, both during the initial stages of often rapid improvement, as
well as for long term residual problems when the child’s progress has
reached plateau.

4. These problems are uncommon and potentially complex. This may mean
that the clinician will need to refer to a specialist colleague for advice
during any stage of the management of the child.
5. It is important that an appropriate longitudinal reassessment protocol is
used to map change, whether spontaneous recovery or as a response to
treatment.

6. Assessment should seek to establish a comprehensive profile of the child’s

speech and language skills in a wide range of situations both formal and
informal, including observations of the child in his or her environment
and family setting.

Assessment should include:

• auditory verbal comprehension,

• expressive language including word finding ability; and
• monitor the presence of jargon aphasia,
• phonemic and semantic paraphasias,
• the presence of errors including perseveration and
• nonverbal communication.

Regarding the timing of assessments during the first 3 months of recovery

assessment should be more or less continuous.
• During the remainder of the first year of recovery assessment should be
carried out regularly and at least every 3 months.
• After the first year of recovery long-term assessment plans should be
for every 6 months to 1 year until the child leaves school or recovery is
 thought to have maximized.

• Research suggests that a few severely impaired children can show

marked change in the long term and yet equally few recover fully, so
speech and language assessment needs to identify and document both
change and residual deficits as appropriate.

Choice of speech and language assessment procedures is determined by a

number of factors:
• Childs age and level of ability
• Presence of coexisting disabilities such as motor, perceptual, emotional
and communication problems
• Availability of assessment procedures
• Experience of the clinician
• Need for repetition the assessment at any stage during recovery

• Cross and Ozanne (1990) outlined one possible model for the
assessment of children with ACA which included standardized tests,
informal or non-standardized tests, and observations, as well as samples
of spontaneous language and play.
• Within the area of language assessment, they use the form/content/use
division of language to look at syntactic, semantic and pragmatic abilities
and additionally, reading, writing and speech production.
 Classifying Language Disorders in Childhood

A number of methods have been used to classify language deficits of

language-impaired groups.

The purpose of defining language disorder syndromes is:

 to outline natural history and substantiate prognosis,
 to allocate appropriate treatment and / or management,
 to enable appropriate comparisons between children,
 and to facilitate research.

• Some authors have used this classification for children with acquired
aphasias Van Dengon, Iconer & Van Dongen, 1985, Pageuess et al. 1989.
• The problem of appropriate terminology for language disorders in
childhood was addressed by Bishop and Resenbloom (1987), who agreed
that are consistent approach has yet been adopted.

• In developmental language disorder 6 language disorder subtypes were

proposed by Rapin & Allen.
 Traumatic Aphasia

I. Unilateral cerebellar lesions of vascular origin

• It is more common in adults than in children.

• There has been considerable research into the long-term effects on
speech and language development of early cerebral lesions, defined as
those acquired before the age of 1 year.
• In more recent research an experimental design has often been used
which has compared groups of children with early (acqd before 1 yr of
age) focal lesions of either the left or right hemispheres with groups of
children with late (acqd after 1 yr of age) focal lesions.
• The children are then asked to carry out different types of tasks like
linguistic, visuospatial or other cognitive tasks.
• Results supported the hypothesis that the left hemisphere is so
predisposed in the development of language.
• Eisele (1991) carried out a detailed study of language comprehension,
using two groups of children with unilateral lesions to right and left
hemisphere respectively and two control groups individually matched for
age, sex and race to the children in the first groups.
• The onset of the cerebral lesions ranged from the perinatal period to 9
 years of age, but none of the children was clinically aphasic at the time of
the study.
There are 3 levels at which the language of aphasic children can be
evaluated:
• clinical rating
• psychometric assessment and
• linguistic study

However, it is impossible to know which tests suit which subgroups of

aphasic children.

• Erupt in some individual cases, few studies have specifically

documented the natural history of aphasia subsequent to unilateral
cerebral lesions.
• As these are highly variable only preliminary conclusions can be drawn.
• Acute expressive aphasia appears to be rare in children.

In most reported cases, where the results of formal language tests are
included, the presence of complete disorders, as well as expressive
language difficulties, were seen.

2. A general picture of an initial period of relatively fast recovery, gradually

tailing off has been shown (Lees & Neville, 1990);
 Lees (1989) suggested that prognosis is usually good in cases where
children were tested for verbal comprehension up to 6 months post-onset
(reached the level of -2 SD for the mean).
Those failing to reach this level had significant long-term language deficits.

3. Paraphasic naming errors have been reported in children with unilateral

cerebral lesions.

• Physical deficits comprise those related to cerebral hemisphere function

(e.g. language expression and reception, epilepsy, hemiplegia) and those
caused by damage to the brainstem and cranial nerves (e.g. visual,
olfactory and auditory impairments).

– Fuld & Fisher (1977) have highlighted that impairments in working

memory are one of the most common complaints following
childhood CHI.
– Sarno et al. (1986) classified the linguistic processing deficits
following severe CHI as a “sub-clinical aphasia disorder” and
enlighted that this group also demonstrated dysgraphias and
impaired expression of oral language abilities and verbal fluency
and confrontation naming.

– Murdoch (1990) has termed it as ‘confused language’.

– Recently, however the term cognitive communication disorder has

 been used or specifically cognitive language disorder (Hagen, 1981).

• Jordan (1996) indicated that children with CHI do exhibit impaired

performance on high level language tasks as assessed by the test of
language competence expanded edition.

• These children exhibited poorer performance in the ability to create

sentences with reference to social stimuli and also to interpret ambiguous
/ figurative expressions.

Language characteristics of children with traumatic brain injury (TBI)

Pragmatics
• Difficulty with organization and expression of complex ideas.
• Off-topic comments.
• Ineffectual, inappropriate comments.
• Short narratives include story grammar and cohesion, as do those of
typically developing peers.

Semantics
• Word retrieval, naming and object description difficulties, although
vocabulary relatively intact.

• Automatized, overlearned language relatively unaffected.

Syntax/Morphology
• Sentences may be lengthy and fragmented.
Phonology

• Few phonological difficulties although there may be some dysarthria or

apraxia due to injury.

Comprehension

• problems due to inattention & speed of processing.

• Poor auditory and reading comprehension.
• Difficulty with sentence comprehension due to difficulty assigning
meaning to syntactic structure.
• Most routinized, everyday comprehension affected.
• Vocabulary comprehension usually unaffected, except for abstract
terms.
 1.Frenchay Dysarthria Test
(Enderby, 1983)

Though it is commonly used, it is difficult to use this test successfully with

children under 8 years of age and almost impossible where they are
uncooperative.

2. The use of informal material which include assessment of head position and
general posture, the presence or absence of oral reflexes, movement of
facial musculature etc.

The major problem in use of informal assessments is in developing a scoring

or recording scheme which allows the therapist to document the
presenting situation and which allows for reliable reassessment
measures.

3. The Paediatric oral skills package

(Brindley et al. 1993)
• Aims to provide a profile of a child’s oral skills and used with those
aged between 0-16 years.
• There are three scales – observation, examination (for eating and
drinking) and performance.
• The profile is then used as the basis upon which the clinician can
formulate a hypothesis about the improvement of oral function.
 
 Meningitis

• Childhood infections diseases of the central nervous system (CNS) are

summarized by Smyth, Ganne & Woodhouse (1990) and include
meningitis encephalitis and meningoencephalitis.

• It is an infection of the meninges usually involving the sub-arachnold

space and the cerebro-spinal fluid.
• The infections are commonly bacterial in origin although viral
meningitis infections can occur.
• Severe deficits of speech and language do still occur along with
deafness, cerebral palsy and learning difficulties.

Cerebral abscess

• Intracranial abscess of the left temporal lobe is the most common one
(Collingnon,1969).
• These usually arose as a complication of severe otitis media and
mastoiditis.
• Another area that is vulnerable to infection is the frontal lobes followed
by frontal sinusitis.
 Encephalitis

• It is common name for viral infection of the brain.

• Mortality and morbidity are common sequelae.

• Commonly, incomplete recovery results in a range of motor, sensory

and cognitive deficits, ranging from mild-severe.

Cerebral tumors

• Neoplasm is defined as abnormal new growth of tissue, which may be

benign or malignant.
• They may arise from the abnormal and increased division of a number
of different cell types, which accounts for the variety of locations and the
range of pronoses.
• Very few cases have been reported in the literature.
• Martins, Ferro & Trindale (1987) reported a case of ‘acquired crossed
aphasia in a child’.
• This was a 15-year old right-handed boy diagnosed as having a right
hemispheric tumor.
• He was severely aphasic and subsequently developed left focal seizures.
As the aphasia worsened, he showed continued growth of the tumor and
the child died 3 months after the initial diagnosis.
• As the boy was right handed and all the cerebral damage was confined
to the right hemisphere, they concluded that he had a crossed aphasia.

Cerebral anoxia

• Anoxia is defined as a condition in which the oxygen level in the body

tissue falls below the level required maintaining normal function, and
may be caused by a complete absence or deficiency of oxygen.
• Murdoch & Ozanne (1990) stated that both the grey and white matter of
the brain may be damaged in cerebral anoxia, and that anoxic lesions of
the cerebral cortex are usually bilateral, although they may be
asymmetrical.
• Damage to the cerebellum was reported as a common finding in all
types of cerebral anoxia.
• Regarding the brainstem nuclei, they reported that damage here has
been recorded as more severe in children than in adults.

Prolonged coma in childhood

 • There are a number of causes of coma in childhood, including traumatic
brain injury, infections disease of the CNS cerebral anoxia and prolonged
convulsive status.

• A 5-year retrospective study of 35 childhood survivors of coma was

conducted by Edwards & Lees (1990).
• Individual psychometric assessment was carried out on all the children
up to 5 years after the end of the coma period using the tests listed.

• The Wechsler Intelligence Scales for Children

• Neale analysis of Reading and Vernon graded world-spelling test for
those of school age.
• The test for reception of grammar for verbal comprehension of language
• Measures of memory, behavior problems and motor impairment

• Results indicated a significant relationship between severity of coma

and cognitive performance between etiology of coma and coma length
and between clinical measures of cerebral perfusion pressure etc. and
cognitive ability.
• the lower the cerebral perfusion pressures in the acute stage, the
greater the likelihood of learning, motor and language difficulties.
Assessment (for other traumatic aphasias)

– Comprehensive assessment of both speech and language skills

 – As a minimum, verbal comprehension and expressive language both
naming and spontaneous language should be tested
– For associated motor speech disorders FDA can be used or for lower
age uncooperative children paediatric oral skills package can be
used.

Landau-Kleffner Syndrome

• The landau-kleffner syndrome must be the most puzzling language

disorder of childhood.
• the rarest disorder of childhood.
• It was first described by Landau & Kleffner in 1957;
• It is also called acquired aphasia with convulsive disorder and acquired
receptive aphasia.
• In the Landau-Kleffner syndrome, language regresses after a period of
normal language development, usually with an accompanying seizure
disorder.
• Beaumanoir (1985) described the two major symptoms as ‘an acquired
aphasia and a paroxysmal electroencephalographic recording with spikes
and spikes and waves, mostly multi-focal and unstable in the course of
evolution’, which may be associated with behavior disturbances and
epilepsy.
Two hypotheses have been advanced:
• first, the lesional hypothesis, which suggests that the EEG abnormalities
and the language problems are the product of the same pathology – an
inflammatory process in the cortex.

• The functional hypothesis suggests that the bilateral discharges lead to

a functional exclusion of the language centers in which even the possible
remedial function of the minor hemisphere is cut off by the abnormal
discharges.
• The exact relationship between the aphasia and the convulsive disorder
in the Landau-Kleffner syndrome has not been established.
• The paroxysmal discharges act in some way to block access to the
language areas of the cortex bears some relation to the phenomenon of
aphasic arrest, which was described by Penfield & Rasmussen (1950) in
their studies of adults undergoing electrical stimulation of the cortex
during neurosurgery for intractable epilepsy.
• Bishop (1985) reviewed 45 cases form the literature (followed up to 12
years of age) to discuss the variable of age of onset in relation to outcome.
• She agreed with the widely held clinical view that the older the child at
onset the better the prognosis.
• This is the opposite of the situation in children having aphasia from
unilateral cerebral lesions, in which prognosis for recovery is poorer with
increasing age.

Deonna et al. (1977) postulated that it was a heterogeneous syndrome,

with at least three courses:
 – The first group, with the better prognosis, had a rapid onset and a
rapid recovery of the aphasia.
– The second group showed progressive worsening of the aphasia
after repeated seizures, and there were subsequent aphasic
episodes.

– The third group also showed a progressive receptive aphasia, but

there were no clinical seizures and recovery was variable.

• Lees, (1989) reported that these children range in age at onset from 2 to
12 years.
• Some have presented acutely and in others there has been a period of
language deterioration lasting over several weeks to several months.
• Those with the longer period of deterioration seem to have poorer
prognosis.
• Complete verbal auditory agnosia and receptive language component
has been seen.
• Verbal language is absent or considerably reduced.
• Older children may present with a rather pedantic conversational style
with inappropriate prosody.
• The characteristic language problem of children with the Landau-
Kleffner syndrome is a severe receptive aphasia.
• Cooper & Ferry (1989) preferred to call it a verbal auditory agnosia
rather than aphasia. This suggests a view that in these children, this
syndrome is not a primary language disorder but rather an auditory
 processing disorder.
• Indeed many of the children are initially thought to be deaf, but on
examination peripheral hearing is normal.
• Bishop (1982) set up a study using three forms of a test for the
comprehension of grammatical structures.
• Three groups of children, one with Landau-Kleffner syndrome, one with
developmental language disorder of an expressive type, and normal
controls were tested with spoken, written and signed presentations of the
test.
• Results confirmed that the children with the Landau-Kleffner syndrome
had deviant comprehension of language, in auditory, signed and written
presentation.
• However, a subsequent experiment with deaf children demonstrated
that they had a very similar profile of difficulty in comprehension of
language to that of the children with Landau-Kleffner syndrome.

• Dugas et al. (1976) reported word finding difficulty, perseveration,

phonemic and semantic paraphasias in the verbal language of the 9 year
old girl they describe these were also observed to a lesser degree in her
written language.

• Van Der Sandt-Koenderman et al. (1984) also reported a child who

produced paraphasias and neologisms.
• They found that the frequency of these in spontaneous speech was a
very sensitive indicator of language breakdown and recovery.
• Beaumanoir (1985) supported the view that when the onset of aphasia
occurs after the child has acquired written language, and if this
knowledge is retained, then there is a better prognosis for educational
attainment.