PATIENT SELECTION IN STUDIES OF APHASIA ACQUIRED IN CHILDHOOD

Bryan T. Woods, M.D.

McLean Hospital, Department of
Neurology and Brain Imaging Center
115 Mill street
Belmont, MA 02178, U.S.A.

ABSTRACT. The history of the study of acquired aphasia of childhood

is filled with examples of erroneous conclusions that could be traced
to an imperfect understanding of the underlying pathological process
that caused the aphasia. Ideally, one wishes to be certain about
both the spatial and temporal extent of the pathological process.
Strokes, with their restriction to one major vessel territory,
sudden onset, and lack of progression best fit these criteria.
Other lesions can also be utilized effectively, but the greatest
single confounding clinical variable when one wishes to study effects
of discrete lesions is severe or prolonged generalized seizures.

1. INTRODUCTION

Acquired childhood aphasia has always been an uncommon medical

problem, but its study assumes much greater importance than the
numbers alone might warrant because of its potential for giving
otherwise unavailable insights into both the development of
normal higher-order brain functions and into the far more common
congenital disorders of that development. Somewhat paradoxically
a history of acquired aphasia, defined simply as a loss or reduc-
tion in previously achieved levels of language functions, is not a
necessary condition for patient inclusion in such studies. What is
a necessary condition, however, is the occurrence of a pathological
process involving the patient's cerebrum, and it is my belief that
progress in understanding childhood aphasia has resulted in large
part from an increasingly precise specification of the pathological
processes that not only produce it but fail to produce it.

2. EXAMPLES

The first example of this proposition can be taken from the

beginnings of modern aphasiology; the observation that patients with
infantile hemiplegia due to left hemisphere lesions involving the

27
/. P. Martins et al. (eds.), Acquired Aphasia in Children, 27-34.
(j) 1991 Kluwer Academic Publishers.
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language area are not aphasic (eotard, 1868). The second example is
the increasing awareness about 15 years ago by ourselves and others
that lesions truly localized to the right hemisphere rarely produce
even short-term aphasia in right-handed children. A review of the
older literature led us to speculate that the earlier observations
had been confounded by looking at patients with pathological processes
that appeared to be lateralized but actually affected functions
bilaterally (Woods and Teuber, 1978).
The final example of the importance of precise pathological
localization is a recent patient in whom the presence of the patho-
logical process was only initially revealed by magnetic resonance
imaging (MRI). The patient was a 29-year-old, left-handed man
admitted to a psychiatric facility because of impulsive and
inappropriate behavior, including making obscene remarks to strangers
in public places. Initially his only reported neurological history
was of a single grand mal seizure at age 21. A Sleep/Awake EEG was
normal but an MRI scan revealed an old infarction in the territory of
the left posterior cerebral artery (Fig. 1). Subsequent inquiry from
surviving family members (unfortunately limited to younger siblings)
elicited the information that he had had a sudden neurological
"problem" when very young that had left him with a "lazy" eye. The
best estimate was that this occurred at about age two. The patient
had performed adequately in elementary school and had gone on to
finish high school and complete several semesters of college before
dropping out.
Neurological examination confirmed the presence of a previously
undocumented right upper quadrant visual field defect, but good
reading skills and intact color naming (9/9). Figure 1 shows axial
plane MRI images showing the extent of the lesion's involvement of
the left lingual and parahippocampal gyri, calcarine cortex, and the
forceps major outflow from the splenium of the corpus callosum. This
is the classic lesion location to produce the adult alexia without
agraphia syndrome. The almost complete absence of surrounding gliosis
on other appropriate scan indicated the early-life origin of the
injury.
Anatomically more extensive abnormalities were elicited by the
auditory and visual evoked response portion of brain electrical
activity mapping (BEAM). The abnormalities were centered in the more
lateral portion of the posterior left temporal lobe.
Table 1 shows a summary of the results of neuropsychological
testing. The first striking finding is the 38-point superiority of
verbal IQ score over performance IQ score. The next point of note
is the low memory quotient, due primarily to problems with memory
passages, associate language, and visual reproductions. other test
results show a mixture of visual and verbal deficits intermixed with
intact performance on tasks such as face recognition.
Although single case interpretation must be tentative the clini-
cal and neuropsychological findings in this patient are consistent
with a shift of both handedness and control of language function to
the intact right hemisphere, with normal reading and color-naming but
incomplete compensation for left medial temporal functions, primarily