We read Flaherty-Craig et al's article with interest. [1] While a brief cognitive screen has the potential to aid clinical
judgments, there were three aspects of this study that may
warrant further consideration.
It appears that the authors used the standard COWA
verbal fluency test. Our group has also found verbal fluency to be a
sensitive measure of cognitive dysfunction in ALS.[2] We suggested a
modification to the verbal fluency test [3] which has been used in subsequent studies. Without such modification, it is not
possible to establish the proportion of patients that were deficient for
physical versus cognitive causes. Our data indicate
that without such a correction for motor/speech problems, verbal fluency
deficits may be exaggerated.[2]
We would also like to emphasize that there is a difference
between statistical significance and clinical significance. This study is
one of several recent papers which has used greater than 1.5 SDs below the mean as
a cutoff for impairment.[4] As with the Ringholz study, the control group
in Flaherty-Craig's article was not matched for years of education and no
data is presented for the control group's estimated premorbid IQ. As
performance on executive tests is strongly associated with years of
education and premorbid IQ, this makes interpretation more difficult.
Furthermore, although the authors report that pulmonary function was
measured, poor respiratory function does not appear to have been an
exclusion criteria. Our group has previously found that performance on
cognitive tests can be improved by use of non-invasive ventilation,
suggesting that respiratory function may play an important role in the
cognitive dysfunction of some individuals with ALS.[5]
Considering these factors, we urge some caution in
following the authors' inferences from the data. There is a risk that such
a small screening battery might inappropriately call into question
patients' autonomy in making difficult choices that might conflict with
the views of health professionals or family members.
While the detection
of neuropsychological impairments does raise the possibility of problems
with decision-making, screening should be followed by more extensive
neuropsychological assessment in addition to careful, patient-specific
assessments of the person's capacity to make important decisions about
their care, especially when they may involve life-sustaining
interventions.
References
1. Flaherty-Craig C, Eslinger P, Stephens B, Simmons Z. A rapid
screening battery to identify frontal dysfunction in patients with ALS.
Neurology 2006;67:2070-2072.
2. Abrahams S, Leigh PN, Harvey A, Vythelingum GN, Grise D,
Goldstein LH. Verbal fluency and executive dysfunction in amyotrophic
lateral sclerosis (ALS). Neuropsychologia 2000;38:734-747.
3. Abrahams S, Goldstein LH, Al Chalabi A, et al. Relation between
cognitive dysfunction and pseudobulbar palsy in amyotrophic lateral
sclerosis. J Neurol Neurosurg Psychiatry 1997;62:464-472.
4. Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz
PE. Prevalence and patterns of cognitive impairment in sporadic ALS.
Neurology 2005;65:586-590.
5. Newsom-Davis IC, Lyall RA, Leigh PN, Moxham J, Goldstein LH. The
effect of non-invasive positive pressure ventilation (NIPPV) on cognitive
function in amyotrophic lateral sclerosis (ALS): a prospective study. J
Neurol Neurosurg Psychiatry 2001;71:482-487.
Disclosure: The authors report no conflicts of interest.