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disorderis rarer, and nearly always involves a mixture of problems with
language comprehension, language expression and articulation.
2 Pragmatic language impairment (formerly semantic-pragmatic disorder)
refers to a set of problems concerning the use and content rather
than the form of language. In a typical case, articulation and syntax
are normal and the child scores well on formal tests of language, but
there are problems with everyday conversation and comprehension
that parents and teachers find hard to describe. Understanding is highly
literal, and the child fails to use knowledge of the context to make sense
of what is said. The child’s own attempts to explain things or to tell a
story do not make allowances for the listener’s point of view, they miss
out key details, or fail to organise the account into a coherent sequence.
The child’s speech may be dominated by rambling monologues or
repetitive questioning. Prosodic impairments are common, for example,
a monotonous tone of voice or abnormal pattern of stressing syllables.
Many children with these pragmatic deficits meet all the criteria for
childhood autism or Asperger syndrome in terms of associated social
impairments, rigidity, and so on. Labelling such children as having both
an autistic spectrum disorder and a pragmatic language impairment is
not very helpful – it is not so much that these children have two separate
disorders; more a case of different professional groups using different
vocabularies. It may be more helpful to use ‘social communication
disorder’ to refer to pragmatic language difficulties that affect social
interaction, along with other mild or patchy features of autism that are
too mild to warrant the diagnosis of an ASD. Some degree of pragmatic
language impairment is common in children with severe phonologic-
syntactic language disorders.
3 Landau-Kleffner syndrome(also known asacquired epileptic aphasia)isa
rare disorder involving the loss of language skills after a period of normal
development, usually starting between the age of 3 and 9. The loss is
usually gradual, typically occurring over the course of months, though
it can be more rapid. Receptive loss is noted first, with the child be-
coming increasingly unresponsive to spoken language. This may lead to
deafness being suspected, though testing shows that hearing thresholds
are normal. Loss of understanding is followed by loss of expressive
language. The language changes are accompanied by EEG abnormalities,
involving paroxysmal discharges that affect both hemispheres, often
independently; these EEG changes are often most prominent during
non-REM sleep. Seizures occur in roughly 50–70% of affected individ-
uals, usually starting about the same time as the aphasia, and taking the
form of generalised or simple partial seizures that are infrequent and
mainly nocturnal. Perplexity, anxiety and temper tantrums are common
at the time of onset, and may result in the child being referred to a
child mental health professional, particularly when there are no evident
seizures and the child’s unresponsiveness is attributed to wilfulness.
Affected children do not generally show autistic impairments in social
interaction, but often show some degree of hyperactivity.