Emotional Lability in MND: Relationship to cognition and psychopathology and impact on caregivers

Abstract

Emotional Lability (EL) is a well recognized symptom of cortico-bulbar pathway dysfunction in Motor Neuron Disease/Amyotrophic Lateral Sclerosis (MND/ALS), and is reported to occur in 19-49% of patients. The Emotional Lability Questionnaire (ELQ), is specifically designed to detect EL as reported by MND patients and as observed by their carers. The aims of this study were to 1) validate the Italian version of the ELQ; 2) investigate the relationship between EL and presence of cognitive dysfunction; 3) investigate the relationship between EL and presence of psychopathology. Forty one MND patients, 39 caregivers and respective control groups composed of 39 subjects and 39 partners/friends were tested. The Italian version of the ELQ was found to have good psychometric properties. Seventy-one per cent of patients reported suffering from EL. Correlations were found between bulbar involvement and EL, and between bulbar involvement and low performance on tests of fluid intelligence and working memory. However, the cognitive profile did not correlate with any aspect of EL. The findings suggests that damage to different neurological pathways underlie cognitive change and EL, which supports the concept of MND/ALS as a multisystem disorder. Moreover the outcomes suggest that EL affects patients' everyday life with the increased anxiety and emotional frailty. The findings suggest that those involved in the care of MND patients should be more aware of the effects of EL in the management of the disease.

Full text

Emotional Lability in MND: Relationship to cognition and
psychopathology and impact on caregivers
A. Palmieri
a,
⁎, S. Abrahams
b
, G. Sorarù
a
, L. Mattiuzzi
c
, C. D'Ascenzo
a
, E. Pegoraro
a
, C. Angelini
a
a
Department of Neurosciences, University of Padova, Italy
b
Department of Psychology, University of Edinburgh, Scotland, UK
c
Department of Psychology, University of Padova, Italy
abstractarticle info
Article history:
Received 5 August 2008
Received in revised form 23 October 2008
Accepted 30 October 2008
Available online 21 December 2008
Keywords:
Emotional Lability
ELQ
MND
ALS
Neuropsychology
Psychopathology
Emotional Lability (EL) is a well recognized symptom of cortico-bulbar pathway dysfunction in Motor
Neuron Disease/Amyotrophic Lateral Sclerosis (MND/ALS), and is reported to occur in 19-49% of patients.
The Emotional Lability Questionnaire (ELQ), is specifically designed to detect EL as reported by MND
patients and as observed by their carers. The aims of this study were to 1) validate the Italian version of
the ELQ; 2) investigate the relationship between EL and presence of cognitive dysfunction; 3) investigate
the relationship between EL and presence of psychopathology. Forty one MND patients, 39 caregivers and
respective control groups composed of 39 subjects and 39 partners/friends were tested. The Italian version
of the ELQ was found to have good psychometric properties. Seventy-one per cent of patients reported
suffering from EL. Correlations were found between bulbar involvement and EL, and between bulbar
involvement and low performance on tests of fluid intelligence and working memory. However, the
cognitive profile did not correlate with any aspect of EL. The findings suggests that damage to different
neurological pathways underlie cognitive change and EL, which supports the concept of MND/ALS as a
multisystem disorder. Moreover the outcomes suggest that EL affects patients' everyday life with the
increased anxiety and emotional frailty. The findings suggest that those involved in the care of MND
patients should be more aware of the effects of EL in the management of the disease.
© 2008 Elsevier B.V. All rights reserved.
1. Introduction
Emotional Lability (EL), is the involuntary occurrence of laughter
and crying in the absence of a corresponding change in affect. It was
first reported in Motor Neuron Disease (MND) in the last century and
was found to occur in 19%-49% of Amyotrophic lateral Sclerosis (ALS)
patients [1,2], the most common form of MND. EL is most often
present in patients who have pseudo-bulbar symptomatology [1,3].
Nevertheless, few studies have undertaken a systematic investigation
of this phenomenon and hence it remains poorly understood.
In 1997 Moore and colleagues validated the CNS-LS (Centre of
Neurological Study-Lability Scale) in ALS patients; a seven-item, self
report measure of affective lability composed of two subscales
measuring labile laughter and labile tearfulness [4]. The CNS-LS
represents a good clinical instrument, but lacks the details necessary
to permit a thorough exam of the nature and the pervasiveness of
emotional lability in the MND population. More recently, Newsom-
Davies and colleagues [3] developed the Emotional Lability Ques-
tionnaire (ELQ), a measurement scale specifically designed to assess
MND patients which includes a self-rated version for patients and
independent-rated version for caregivers. The ELQ was modified from
the Pathological Laughter and Crying scale [5] which was first
developed for stroke patients. The ELQ presents good psychometric
properties and in depth exploration of emotion and expression, such
as the introduction of the ‘abnormal smiling’subscale. We set out to
validate an Italian version of the ELQ, to produce a measure able to
detect lability in an Italian population of MND patients, and to
distinguish between pathological laughing, crying and smiling.
A second aim of this study was also to investigate the relationship
between EL and cognitive deficits, which are found in some patients
with MND. Although EL can be found in patients with frontal and
temporal lobe involvement, the traditional view is that EL is due to
damage to pathways that arise in the motor areas of the cerebral
cortex and descend to the brainstem and inhibit putative centres for
laughter and crying. Recent neuroanatomical findings suggest that the
critical areas are in the cerebro-ponto-cerebellar pathways [6,7]. The
abnormal cognitive profile found in MND/ALS is well-known, and
consists of predominant executive dysfunction and in some cases
language and memory dysfunction [8–11,43–47]. Visuospatial func-
tions appear well preserved. This profile of impairment is consistent
with frontotemporal involvement as found in neuroimaging studies of
MND patients [12–14]. In one of the largest studies of cognition in ALS
Journal of the Neurological Sciences 278 (2009) 16–20
⁎Corresponding author. Department of Neurosciences, University of Padova, via
Giustiniani 5, 35128 Padova, Italy. Tel.: +39 0498215321; fax: +39 0498751770.
E-mail address: arianna.palmieri@unipd.it (A. Palmieri).
0022-510X/$ –see front matter © 2008 Elsevier B.V. All rights reserved.
doi:10.1016/j.jns.2008.10.025
Contents lists available at ScienceDirect
Journal of the Neurological Sciences
journal homepage: www.elsevier.com/locate/jns

to date [15], 47% of patients showed executive dysfunction, and 15% of
them had severe cognitive impairment with features that were
consistent with fronto-temporal dementia. The presence of cognitive
impairment in MND appears more prominent in patients with
pseudo-bulbar palsy [10,16]. In previous studies emotional lability
was also found to significantly correlate with bulbar scores [3].
However, the relationship between EL and cognitive dysfunction in
MND has received little attention other than a small study reported by
McCullagh et al. [17] in which a difference was found between 8 ALS
patients with EL and 10 patients without, in errors on Wisconsin Card
Sorting Test.
Moreover, the relationship between EL and psychopathology has
not previously been investigated in MND. Estimated prevalence rates
for depression or mild depressive-symptomatology has appeared low,
between 0-44% in MND populations [18–21]. In recent studies
depression has appeared to be relatively more common (prevalence
rates up to 50%), as were other forms of psychological distress, and
was not associated with illness severity and functional status [22].
Estimated prevalence rates for anxiety have ranged 11-30% [23–25].A
number of studies have shown the importance of including patients'
families in psychological research on MND. Social factors have been
reported to be the best predictor of ALS patients' self-esteem during
the disease [26]. In addition Gauthier et al. [27] found that patients'
level of disability and caregivers' depression were related with
caregivers' perception of burden. While Goldstein [23] showed that
carers also demonstrated signs of anxiety and depression, with the
latter correlating with aspects of the patients' functional impairment.
Carers depression and strain appeared to be related to their
attributional style and perceived strain was greater in carers who
viewed their partners' illness as having a more global impact on their
lives [28]. Hence, our third aim was to investigate the relationship
between EL and psychological status in patients and their caregivers.
2. Methods
2.1. Participants
We recruited 41 patients with Motor Neuron Disease via
Neurosciences Department of Padua, in the period from March 2007
and November 2007. The sample consisted of 16 females and 25
males; 32 of 41 had ALS, 6 had Primary Lateral Sclerosis,1 was affected
by Progressive Bulbar Palsy, 1 was affected by Progressive Muscular
Atrophy and 1 had Flail Arm Syndrome. Mean age of the sample was
58.19 (11.59sd, range 23-77). Mean educational level was 9.39 years.
All of the ALS patients fulfilled the criteria of “probable”or “definite”
ALS [29]. We excluded patients with a clinical diagnosis of dementia
according to Neary's criteria [30], those in receipt of 24-hour care and
those with cerebral injury or cerebrovascular accident. We assessed
their respective caregivers in 39 cases of 41; two patients reported not
to have a single referential caregiver. The carers sample consisted of 11
males and 18 females (mean age was 52.56, 15.42 sd); 74% of them
were the patient's spouse. More detailed data of patients and
caregivers are showed in Table 1.
We also tested as controls 39 healthy subjects. The control group
did not significantly differ from the patient group in gender, age and
educational level. Thirty-nine pseudo-caregivers (i.e. who spent the
day with the 39 healthy subjects) were also tested. The pseudo-
caregivers did not significantly differ from the carers in gender, age
and educational level. Similar exclusion criteria were applied.
2.2. ELQ translation and back-translation
The original Emotional Lability Questionnaire, [3] consisted of two
components: the self-rated version, given to MND patients, and the
independent rated version given to carers. Each questionnaire
contains 33 items, including three subscales measuring: Laughing
(11 items), Crying (11 items), and Smiling (11 items). The questions
refer to the patients' condition during the past four weeks and the
answers are given on a four-point Likert scale. The higher the score,
the higher the level of perceived emotional lability.
The translation and back-translation process included three phases:
the first, in which two native Italian speakers fluent in English, translated
the original version of the ELQ; then, using the back-translation approach,
two native English speakers fluent in Italian, translated the English version
to Italian. In the third phase, four raters compared the two versions. No
relevant differences between the original and back-translated versions of
the questionnaires were found.
2.3. Procedure
MND patients and caregivers were interviewed separately by a
psychologist, following neurological examination. The interview took
an average of 1.5 h to complete. The same method was employed to
assess controls and pseudocaregivers. The ELQ, both self-rated and
independent rated versions, and other psychodiagnostic instruments,
were administered as a structured interview to each participant. MND
patents were asked to return to our Neurological department no
longer than two months after first assessment, for a more compre-
hensive neuropsychological test battery. The great majority of patients
(93%) accepted. The complete neuropsychological evaluation took
an average of 1 h, however not every patient was able to complete
all of the tasks due to severity of motor impairment or bulbar
involvement.
2.4. Neuropsychological measures
Thirty-eight of 41 MND patients undertook the neuropsychological
battery, which was designed to assess intelligence, executive
functions, memory and language. An initial general screening was
undertaken using the Mini Mental State Examination [32]. Intelligence
was assessed with the Raven's Coloured Matrices [33] and with the
Brief Intelligence Test (T.I.B.), designed to estimate pre-morbid
intellectual ability [34]. Executive functions were explored using the
Modified Wisconsin Card Sorting Test [35],Phonemic Verbal Fluency (F,
A,S) [36],Digit Span Backward [37],Trail Making Test A and B. The latter
was performed according to Reitan's protocol [38]. Long and short-
tem Memory were respectively investigated with Babcock's Prose [36],
Verbal Digit Span Forward [37] and Corsi Blocks tapping test [36].
Language was investigated with Semantic verbal fluency (modified
Table 1
Features of MND patients and caregivers
Patients(n=41) Values
Men,n(%) 25(60.9)
Mean age(SD) 58.19(11.6)
Mean educational level (years)(SD) 9.4(4.5)
Mean length from diagnosis (months)(SD) 40.58(33.8)
Spinal onset(%) 30(73.2)
Married,n(%) 33(80.4)
Mean MRC upper limbs (SD) 38.6(10.9)
Mean MRC lower limbs(SD) 29.2(11.5)
Mean ALSFRS-R(SD)[31] 32.7(7)
Caregivers(n=39)
Men,n(%) 11(28.2)
Mean Age(SD) 52.5(15.4)
Mean educational level (years)(SD) 9.4(4.8)
Spouses 29(74.4)
Sons or daughters 7(17.9)
Parent 1(2.6)
Brothers or sisters 2(5.2)
ALSFRS-r = Amyotrophic Lateral Sclerosis Functional Rating Scale Revised; MRC upper
and lower = Medical Research Council scale applied to upper extremities (maximum
score= 50) and applied to lower extremities (maximum score = 40).
17A. Palmieri et al. / Journal of the Neurological Sciences 278 (2009) 16–20

version from Spinnler) [37]. To exclude the effect of dysarthria on
verbal fluency, both phonemic and semantic, a motor control
condition was performed in which patients were asked to read
aloud all the words generated previously during the task as quickly as
possible and the examiner recorded the time taken to perform this
phase. The difference between the specified time for the generation
condition and the time taken for the control condition, the fluency
index (fi) was calculated according to guidelines of Abrahams et al.
(Neuropsychologia, 38:734-747, 2000) [11].
2.5. Psychodiagnostic measures
Depression was assessed using the Beck Depression Inventory (BDI)
[39]. State and trait anxiety were respectively evaluated with
Spielberger's State and Trait Anxiety Inventory (STAIY-1andSTAIY-2)
[40]. Emotional fragility and sense of inadequacy were investigated with
Emotional Fragility questionnaire (FE) [41], a questionnaire focusing on
sense of frailty and inadequacy.
2.6. Functional measures
The Amyotrophic Lateral Sclerosis Functional Rating Scale Revised
(ALSFRS-R) is a 12-item scale, in which the patient's functioning for
each item is rated on a scale from 0 (unable to attempt the task) to 4
(normal function). This scale includes evaluation of swallowing,
speech, and respiratory function, and both strength and function of
the upper and lower extremities musculature. Medical Research
Council scale (MRC) was used to obtain a more specific strength
measure for upper and lower extremity muscles groups.
3. Statistics
3.1. Validation of the Italian version of the ELQ
Spearman's correlations were applied to establish the internal validity
of the ELQ, both self-rated and independent rated versions, between
Laughing, Crying, Smiling subscales and global scores. Spearman's
correlation was also undertaken to explore construct validity, separately
for patients and controls group, and for caregivers and pseudo-caregivers.
MND characteristics (including age, educational level, time since onset,
onset of disease in terms of bulbar or limb, functional scores such as
ALFRS-R and MRC for upperand lower districts) were compared with ELQ
scores with Spearman's correlation.
3.2. Neuropsychological and psychopathological battery and ELQ scores
The cognitive and psychopathological data were analyzed with
Mann Whitney U non-parametric tests (as the data were not normally
distributed), comparing patients' performance with controls.
In order to identify differences between ALS (33) and ALS patients
with prevalent UMN (PLS), LMN (PMA) or bulbar involvement (PBP)
(8) a Mann Whitney U non parametric test has been used for
neuropsychological test and psycopathological and emotional lability
questionnaires applied at the two groups.
Spearman's correlations between ELQ data and the neuropsycho-
logical and psychopathological variables were applied to those
variables on which the ALS group were impaired. Due to the number
of correlations significance was set at p= 0.01 to reduce the incidence
of Type 1 errors.
4. Results
No significant differences were identified between ALS, and ALS
patients with prevalent UMN (PLS), LMN (PMA) or bulbar involvement
(PBP) (Mann Whitney non parametric test pN0.05) (Supplemental Table).
4.1. Validation of the Italian version of the ELQ
Seventy-one per cent of patients indicated that they suffered from
at least of one of the three emotional lability domains (Laughter,
Crying, Smiling), as opposed to 5% of healthy controls.
Significant results relating to validity are displayed in Tables 2
and 3. In addition, correlational analyses were used to explore the
relationship of EL to demographic variables and clinical data. MND
patients displayed a positive correlation between time since onset of
disease (in months) and Laughing (r= 0.48; pb0.00). Global Lability
revealed a significant correlation with ALFRS-R total scores (r= 0.36;
pb0.01); in particular, significant correlations were found between
Global Lability and ALSFRS-R 1-language (r= 0.50; pb0.000) and
ALSFRS-R 3 -swallowing (r=0.47; pb0.001). The MRC did not exhibit
any significant correlations.
4.2. Neuropsychological battery and ELQ
Neuropsychological results are reported in Table 4.Inthe
comparison of the patient and control groups, MND patients showed
a trend towards impaired performance on digit span backwards and
Corsi block tapping test and were significantly impaired relative to
controls on the Raven's Coloured Progressive Matrices. No significant
correlations were found between neuropsychological performance
and emotional lability. Semantic Verbal Fluency Index was found to
correlate with level of swallowing (ALSFRS-R 3) (r=0.49; pb0.00).
MRC did not exhibited any significant correlation with these cognitive
tasks.
4.3. Psychodiagnostic questionnaires and ELQ
Thirty-nine per cent of MND patients revealed BDI scores from 16
to 33; 34% showed the presence of state anxiety (STAY-Y1) and trait
anxiety (STAI Y-2) with greater than one standard deviation above the
Table 3
Statistically significant correlations between ELQ items for the controls and pseudo-
caregivers groups
ELQ scores correlating Spearman's
coefficient
PValue
Within self-rated version(ncontrols= 39):
Laughter-Total 0.91 0.000
Crying-Total 0.69 0.000
Smiling-Total 0.50 0.001
Within independent-rated version(npseudo-caregivers= 39):
Laughter-Total 0.94 0.000
Crying-Total 0.43 0.000
Smiling-Total 0.76 0.000
Smiling- Laughter 0.72 0.000
Between self-rated and independent-rated version
Self/Laughter-Independent/Smiling 0.82 0.000
Self/Laughter-Independent/Total 0.57 0.000
Table 2
Statistically significant correlations between ELQ items for the patients and caregivers
groups
ELQ scores correlating Spearman's
coefficient
PValue
Within self-rated version (npatients = 41):
Smiling-Total 0.52 0.000
Within independent-rated version (ncaregivers= 39):
Crying-Total 0.69 0.000
Between self-rated and independent-rated version
Self/Laughter-Independent/Laughter 0.57 0.000
Self/Total-Independent/Total 0.51 0.000
Self/Laughter-Independent/Total 0.46 0.000
Self/Total-Independent/Laughter 0.57 0.000
18 A. Palmieri et al. / Journal of the Neurological Sciences 278 (2009) 16–20

cut off; 51% of patients' scores were greater than the 75th percentile in
Emotional Fragility questionnaire. While, 18% of caregivers had
depression scores from moderate to severe on the BDI; 79% showed
STAI-Y1 scores over the clinical cut off, and 44% were over the clinical
cut off on the STAI-Y2; 46% went greater than the 75 percentile of
emotional fragility scores. Scores for the BDI, STAI Y1 and STAI Y2
revealed significant differences between patients and healthy con-
trols. Caregivers had significantly greater scores on the BDI and STAI
Y2 than pseudocaregivers. The results are presented in Table 5.
Finally, psychological results obtained from questionnaires admi-
nistered to the four groups were compared with Spearman correla-
tions with ELQ scores. In the ALS/MND patients ELQ Global Lability
correlated with state anxiety (STAI Y1) scores, (r=0.36. pb0.01) and
with Emotional Fragility questionnaire scores (r=0.4; pb0.00). The
Crying subscale showed a significant correlation with Emotional
Fragility (r=0.44; pb0.00), BDI scores (r= 0.41; pb0.00), STAI Y1
(r=0.38; pb0.01), STAI Y 2 (r=0.37; pb0.01). In caregivers, Smiling
subscale of independent rated version showed a strong trend towards
a significant correlation with Emotional Fragility questionnaire at our
raised significance threshold (r=0.33; pb0.05). No significant correla-
tion were found in healthy controls and pseudo-caregivers with
respect to emotional lability and psychopathological features.
5. Discussion
The Italian version of the ELQ showed good internal validity as
determined by the relationship between the subscales (Laughing,
Crying, Smiling) and global scores. Construct validity was corroborated
by significant correlations between self rated version subscales and
independent rated version subscales. Seventy-one per cent of MND
patients reported that they suffered from at least one of the three
aspects of emotional lability. This percentage appears greater than
reported in previous studies [1,2,42]. MND patients displayed a
positive correlation between time since onset of disease (in months),
Laughing and Smiling. Global Lability (total score derived from sum of
the three subscales) was significantly related to ALFRS-R total scores
and in particular with Language and Swallowing items. Our findings
are therefore consistent with the view that the Emotional Lability
correlates with disease progression and bulbar symptomatology [3].
The neuropsychological battery revealed significantly impaired
performance in the MND group in the Raven's Coloured Progressive
Matrices only as compared with controls. Two tests of working
memory, Digit Span Backward and Corsi Block Tapping test, showed
trends towards an impairment. Although the Raven's matrices is
considered to be a test of fluid intelligence it can also be viewed as
reliant on other more specific cognitive processes such as working
memory, attention and problem solving and hence is dependent on
intact executive functions. However, our group, unlike many
previous studies, did not display a deficit on other tests of executive
processes such as Verbal Fluency, Trail Making Test and the
Wisconsin Card Sorting Test. Moreover, the neuropsychological
scores did not correlate significantly with any aspect of emotional
lability. This data are in contrast with McCullogh's and colleagues
study [17], who implicated an association between prefrontal cortex
dysfunction, as represented by deficits on the Wisconsin Card
Sorting Test and emotional lability. Cognitive change in MND has
been previously associated with extra-motor frontotemporal dys-
function [12–14] while it is possible that dysfunction of cerebro-
cerebellar pathways are involved in the regulation of emotional
expression [7].
The current findings suggest that not only the upper motor
neurons are involved with cognitive dysfunction in MND and
emotional lability, but other neuronal pathways not fully character-
ized yet may play a role, providing support for the concept of MND as a
multisystem disorder.
With regard to the presence of psychopathology in MND, the
scientific literature varies: the two most common features investi-
gated, depression and anxiety have been reported in 0-44% and 10-36%
of cases respectively. In our sample, 36% of patients showed a level of
depression from moderate to severe, while very high levels of anxiety
were reported with 70% exhibiting a high level of state anxiety and 60%
and high level of trait anxiety. Furthermore high levels of emotional
fragility were reported in 48% of cases. Wicks demonstrated that the
estimated prevalence of mood disorder amongst patients with ALS
may vary significantly depending on the measure used [21]. Regardless
of the lack of unanimous consensus of standard utilization of
psychopathological measures, it was clear that our sample of MND
patients revealed predominant anxiety, but with also depression and
emotional fragility in many cases.
Moreover, the current study revealed a relationship between total
scores on the ELQ and both state anxiety and Emotional Fragility
indicating an association between lability and psychopathology in
MND patients. The Crying subscale showed a significant relationship
Table 5
Participants psychopathological features and significant results
MND (SD)N=41 Controls (SD) n= 39 Mann Whitney UCoefficient Caregivers (SD) n=39 Caregiver's control (SD) n= 39 Mann Whitney UCoefficient
BDI 15.92 (11.55) 7.38 (6.03) U=1203 (pb0.00 0) 10.71 (6.48) 5.94 (6.30) U=1137 (pb0.000)
STAI Y1 43.48 (13.45) 35.20 (9.66) U= 1085 (pb0.005) 47.76 (10.05) 35.64 (9.19) U=1247 (pb0.000)
STAI Y2 44.39 (12.43) 32.56 (15.84) U=1155.5 (pb0.000) 41.97 (8.61) 37.76 (9.45) U= 966.5 (pb0.05)
FE 74.2 (29.53) 58.27 (24.49) U=1011 (pb0.05) 65.68 (24.21) 59 (27.04) U=86.5 (p=0.312)
Significant results are highlighted in bold.
BDI = Back Depression Inventory; STAI = State-Trait anxiety Inventory; Y1 = State Form; Y2 = Trait Form; FE = EmotionalFragility.
Table 4
Neuropsychological assessment of patients and controls
Patients nMean
(SD) n=38
Controls Mean
(SD) n=39
Mann-Whitney
UCoefficient
P
Value
General Screening
MMSE 28.26 (1.77)n=28 28.51(1.79) 326 0.375
Intelligence
Rs CPM 28.76(5.61)n=38 31.86(3.98) 332.5 0.002
TIB 108,78(6,54)n=30 104,41(9) 470 0.594
Memory
Digit Span Forward 5.51 (0.94)n=30 5.94(1.59) 468 0.233
Digit Span Backward 3.3(0,81)n=24 3.72(1.38) 411 0.052
Babcock Prose 12.36(2,56)n=30 12.97(2.40) 450.5 0.309
Corsi Blocks Tapping 5 (1.02) n=30 5.5(1.24) 490 0.037
Language
Semantic Fluency 35.19(12.46)n=30 37.02 (10.95) 381 0.714
Semantic Index(Sfi) 2,44 (0,95) n=30 2,51 (1,21) 429 0.838
Phonemic Fluency 30.55 (11.15) n=30 32.47 (13.36) 374 0.381
Phonemic Index (Pfi) 5.1(2.32)n=30 5.1(1.96) 466.5 0.714
Attention/planning
TMT A 44.6 (7.43) n=24 51.37 (30.50) 349 0.411
TMT B 126.88 (110.73) n=24 120.09 (33.62) 442.5 0.494
TMT A-B 82.28 (106.37)n=24 68.71(60.23) 467 0.381
WCST (categories) 3.90 (1.89) n=24 4.41(1.56) 324 0.309
WCST (errors) 17.35(13.38)n=24 13.37 (8.31) 447.5 0.199
WCST
(perseverations)
8.74(9.61)n=24 5.7(4.58) 439 0.254
pb0.01 andpb0.05 are highlighted in bold.
MMSE = Mini Mental State Examination; RsCPM = Raven's Coloured Matrices; TIB = Brief
Intelligence Test; TMT = Trail Making Test; WCST = Wisconsin Cards Sorting Test
Modified Version (Nelson).
19A. Palmieri et al. / Journal of the Neurological Sciences 278 (2009) 16–20

with all psychopathological measures. These findings suggest that the
presence of psychopathological symptoms in MND patients may be a
predisposing factor for emotional lability or in contrast that such
psychopathology may be a reaction to emotional lability. Interviews
with carers also demonstrated clear evidence of psychopathology with
20% reporting depression from moderate to severe levels on the BDI,
while 85% and 72% showed state and trait anxiety scores over the
clinical cut off, respectively. As for the relationship between patients
and carers mood, some data in the literature have reported an
association [23–28] but despite high levels of affective conditions in
our patients and carers we did not find an association.
In conclusion, emotional lability appears to be more prevalent in
the MND population than previously thought. However cognitive
change was not associated with the presence of lability suggesting
differential neurological pathways and indicating that extramotor
involvement in MND is widespread. Moreover, the lack of ability to
control one's own emotions and the expression of inconsistent
emotions, seems to relate to high levels of anxiety and emotional
frailty in people with MND. Hence, the relatively low number of
patients' asking for a drug treatment for emotional lability [42] may
not be representative of real discomfort caused by such emotion
dyscontrol. Clinicians should be more aware of the effects of emotional
lablity and provide appropriate support to the both the patients and
caregivers during the course of the illness.
Acknowledgements
We thank patients and caregivers for participating to this study.
This work was supported by COFIN 2006062912-001 and a
Telethon grant to C.A.
Appendix A. Supplementary data
Supplementary data associated with this article can be found, in
the online version, at doi:10.1016/j.jns.2008.10.025.
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