Correspondence: Lucio Tremolizzo, Neurology Unit, University of Milano-Bicocca, Via Cadore 48, 20900 Monza, Italy. Fax: 39 02 6448 8108. E-mail:
lucio.tremolizzo@unimib.it
(Received 4 October 2012; accepted 28 October 2012)
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2013; 14: 157–158
ISSN 2167-8421 print/ISSN 2167-9223 online © 2013 Informa Healthcare
DOI: 10.3109/21678421.2012.746990
in Raaphorst’s one, we agree that motor impairment
is a major determinant of this outcome, as did Raa-
phorst (1). Interestingly, previous works have used the
FAB without reporting problems; however, on closer
inspection, ALS patients enrolled in these papers had
milder disease, as documented by higher ALSFRS-R
mean scores (e.g. Oskarsson et al., 2010, ALSFRS-R:
35.19 5.81, n 16 (2); Floris et al., 2012, ALS-
FRS-R: 40 in 15 patients out of 20 (3); Ahn et al.,
2011, ALSFRS-R: 38.4 3.5, n 61 (4); Morimoto
et al., 2012, ALSFRS-R: 40.4 6.4 and 37.5 3.6,
n 23 and 12 (5)). Most probably, the FAB is a fea-
sible tool only during the first stages of ALS (3), and
its value progressively diminishes with advancing
motor impairment. This might be not the case of
behavioural impairment that has been reported to be
independent from motor impairment (6). In addition,
also the sensitivity of the FAB as a screening tool
seems not to be perfect, as suggested by the fact that
in the Raphorst subgroup of eight ALS patients with
a diagnosis of behavioural variant of frontal dementia,
at least one had a FAB score of 18, i.e . at the top.
In general, our data are, therefore, in agreement
with those of Raaphorst et al. (1); raw FAB might
return a low rate of completion and other tests less
affected by motor performances might offer advan-
tages. We decided to use WST just for further stress-
ing this point, since this test can be administered with
a minimum motor involvement, but the test alone
may be hardly considered a suitable screening tool
because it explores only a single executive function,
i.e. sorting ability, as opposed to the FAB, which is a
mini-battery.
In conclusion, the need for dedicated tools for
screening cognitive and behavioural dysfunctions in
ALS patients is stronger than ever, especially when
Dear Sir,
We read with great interest the study by Raaphorst
et al., reporting significant limitations of the frontal
assessment battery (FAB) for cognitive impairment
screening in amyotrophic lateral sclerosis (ALS)
patients (1). The authors touch on an issue relevant
to all ALS clinicians trying to assess frontal lobe
impairment in their patients the impossibility of
achieving a full completion rate (lower than 80% in
their series). The authors further explore this issue
by proposing an item-adjusted FAB score seemingly
demonstrating more reliability with respect to the
raw score. However, the various items of the FAB
tap different executive functions that may be vari-
ably affected in each single ALS patient; thus, to
pro-rate a FAB raw score due to missed items does
not seem to be an optimal solution from a cognitive
point of view.
We performed a similar work in our ALS clinic
assessing FAB and Weigl’s sorting test (WST) in 10
consecutive definite non-demented ALS patients
(four bulbar/six spinal; mean ALSFRS-R: 25.8 6.7;
age 67.4 10.9 years; median disease duration 25.5
months, first third quartiles 20.5 74, range 9 105).
Incomplete testing was obtained at the FAB in 50%
of our patients and the mean item completion rate
was 85%. Bulbar patients could complete FAB in
75% of the cases, while spinal patients only in 33%
(mostly due to the already reported relevant impact
of the Luria item); by contrast, all patients could
undergo the WST. Cohen’s kappa score between the
item-adjusted FAB and WST was 0.44, which was not
surprising since the two tests assess different functions
within the frontal domain. Considering that the ALS-
FRS-R score in our case series was even lower than
LETTER TO THE EDITOR
Exploring limits of neuropsychological screening in ALS: The FAB
problem
LUCIO TREMOLIZZO, CARLO FERRARESE & ILDEBRANDO APPOLLONIO
ALS and Memory Clinics, Department of Neurology, San Gerardo Hospital and University of Milano-Bicocca, Italy