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Neuropsychological syndromes in multiple sclerosis.

Multiple Sclerosis (MS) is a chronic autoimmune disease of the Central Nervous System (CNS) characterized by inflammation, demyelination and axonal degeneration (Kornek & Lassmann 2004). It is the second cause of disability in young adults (Nieto, Sanchez, Barroso, Olivares, & Hernandez, 2008). Typically, MS leads to disability because of cumulative motor, sensory and or visual deficit. Between 45 and 65% of the patients develop variable cognitive dysfunction over the course of the disease, being generally more frequent and severe in late phases (6-10%) and coexisting with physical impairment (Halligan, Reznikoff, Friedman, & La Rocca, 1988). However, cognitive dysfunction can be a major complaint and the main cause of disability in the initial stages and, in contrast to motor or sensory deficits, it does not tend to improve (Calabrese et al., 2009; Staff, Lucchinetti, & Keegan, 2009). When cognitive deterioration in MS becomes the predominant symptom, it has been named 'Cortical MS' by some authors (Zarei, Chandran, Compston, & Hodges, 2003).

Although in MS there is no specific pattern of cognitive dysfunction, the functions most commonly impaired are speed of information processing (SIP), attention, memory, executive functions (EF) and visuospatial and visuoconstructive abilities (Arango-Lasprilla, DeLuca, & Chiaravalloti, 2007; Genova, Sumowski, Chiaravalloti, Voelbel, & DeLuca, 2009; Patti, 2009). The brief batteries designed for the routine study of cognitive dysfunction in MS basically include the assessment of these functions (Boringa et al., 2001; Duque et al., 2012). The presence of neuropsychological syndromes (NPS) (cognitive and behavioural disorders observed in cerebral disease with involvement of cortical areas: agnosia, aphasia, apraxia) has only been described in isolated cases of MS, largely without regulated neuropsychological evaluation. Anecdotal cases have been published referring to acute aphasia (Devere, Trotter, & Cross, 2000), alexia with agraphia (Day, Fisher, & Mastaglia, 1987), alexia without agraphia (Dogulu, Kansu, & Karabudak, 1996; Mao-Draayer & Panitch, 2004), visual agnosia (Okuda et al., 1996) and patients with more than one severe cognitive symptom, with criteria for dementia (Staff et al., 2009; Stoquart-ElSankari, Perin, Lehmann, Gondry-Jouet, & Godefroy, 2010). When cognitive impairment is the initial and predominant symptom, it constitutes a diagnostic challenge (Calabrese, Filippi, & Gallo, 2010; Rinaldi, Calabrese, & Grossi, 2010).

The objective of this study is the cognitive assessment and the clinical and radiological description of five patients with MS presenting with NPS, evaluated by means of specific cognitive tests.

Method

Participants and procedure

This study was conducted in the Multiple Sclerosis Unit, at the Getafe University Hospital. From a total of 164 patients with MS (McDonald 2005 diagnostic criteria) (Polman et al., 2005) and neuropsychological evaluation, five were selected for presenting neuropsychological symptoms involving changes in cognitive processes such as language and visual gnosis.

An analysis of demographic data, course of the disease and degree of physical disability (Kurztke disability status scale, EDSS) (Kurtzke, 1983) was carried out at the moment of diagnosis of the NPS.

An estimate of the lesion volume was made by Magnetic resonance imaging (MR) axial T1- and T2-weighted sequences and coronal fluid attenuated inversion recovery (FLAIR). A low lesion volume (LV) was defined by the presence of 9 or fewer small size lesions (< 1 cm of larger diameter), a moderate LV by the existence of more than 9 lesions, at least 2 being large (diameter between 1 and 2 cm) and high LV if there was at least one large confluent plaque (larger than 2 cm in diameter). Cerebral atrophy was qualitatively measured by inspection of the width of sulci and of the third and the lateral ventricles in relation to age. Finally, we performed a second neuropsychological evaluation in some patients in order to estimate the progression or remission of the cognitive deficits.

Instruments

From October 2004 to March 2012, an extensive routine neuropsychological evaluation was performed including the functions typically impaired in MS (IPS, memory, learning, attention and EF), language and visuospatial abilities. The degree of anxiety and depression was also measured with the Beck Depression Inventory (BDI) and Spielberger's State-Trait Anxiety Inventory (STAI). Values lower than the normative values of each test were considered pathological.

Clinical cases

A summary of the clinical and radiological characteristics, as well as the results of the cognitive tests, is found in Tables 1-5.

Case number 1 is a 33-year-old woman. In 2001, she had the first MS symptom, consisting of motor impairment of the right hand. The second flare occurred in 2006 with motor-sensory impairment; therefore, the patient was diagnosed of relapsing-remitting MS (RRMS). Examination revealed signs of pyramidal, sensory and gait impairment (EDSS score: 3). The patient did not experience symptoms of cognitive deterioration at any time. However, a protocol neuropsychological evaluation in 2007 found a normal intelligence and low to moderate changes in IPS, attention, EF and visuoconstruction. Because difficulties at reading and writing were observed during the evaluation, additional linguistic processing was examined using the PALPA battery (Spanish version EPLA: Valle & Cuetos, 1995). The patient presented adequate phonological processing in the differentiation of sounds, letters and words independently of the imageability, frequency of use or morphology. She repeated sentences well and exhibited a good level of picture naming. Significant difficulties were observed in reading and writing, and she showed significant effects of imageability, frequency of use and inflectional morphology. The patient also had difficulties reading non-words, writing functional words and she committed errors depending on derivational morphology. These difficulties resulted in mistakes in the orthographic input lexicon and in grapheme-phoneme conversion (see Table 1). Symptoms of fatigue, anxiety and depression were also present. Cranial MR showed multiple large demyelinating lesions, some confluent in the deep white matter (WM) in both cerebral hemispheres, predominantly in the left parietal-occipital-temporal area, clear cortical atrophy and a thinning of the corpus callosum (Figure 1). At a new neuropsychological evaluation in 2009, a remission of her alexia-agraphia syndrome was observed.

Case number 2 is a 30-year-old woman with no relevant medical history. She was admitted in 2008 with a subacute left hemisphere syndrome consisting of right hemiparesis and impossible verbal expression. Neurological examination revealed a mild confusional state with a trend to agitation and a slightly decreased level of consciousness. The severity of the neurological involvement did not permit an adequate neuropsychological examination but confirmed severe motor aphasia with absence of spontaneous language and inability to repeat, name and produce automatic sequences. The patient was only able to utter two-syllable words and understood basic commands. Right unilateral visual and sensory neglect was observed together with severe right hemiparesis. MR showed an extensive lesion in the left frontal-parietal WM and two small lesions in the WM of the right hemisphere. No cortical involvement was observed (Figure 2). A brain MR after 4 months showed conversion to MS by the appearance of two new lesions, one of them active in the left occipital region, and the patient was diagnosed of RRMS. In 2009, a neuropsychological examination was carried out in another centre. The reports from this evaluation pointed out to an impairment in linguistic processing in both written and oral expression, with a slight alteration in understanding, a mild impairment in processing speed, attention, executive function (planning and working memory), memory and verbal learning, with preservation of visuospatial memory. In our centre, a neuropsychological evaluation performed in 2010 revealed a near-complete remission of the aphasia. Using the PALPA and the Boston Naming Test (BNT) (Table 2), adequate performance was observed in picture naming, phonological discrimination (words/non-words), lexical decision (depending on imageability and frequency) and linguistic understanding of sentence reading and repetition of words regardless of length, in both reading and writing. Only mild alterations in the repetition of non-words (non significant), SPI and attention were observed. In the BDI, the patient showed a slight lowering in mood, and the scores in the STAI were slightly elevated.

Case number 3 isa 43-year-old woman with secondary progressive multiple sclerosis (SPMS) of 10 years of evolution and an EDSS score 5 by impairment of walking, associated with mild decrease of visual acuity (> 20/30) and fatigue. She complained of spatial disorientation, often getting lost in places close to her home. The first neuropsychological evaluation in April 2004 found difficulties in the visual perception of objects and space, and in the construction of figures under visual guidance. The patient had problems in integrating figures into a whole, in the mental rotation of figures and elements and also in using three-dimensionality. These deficits were explained by apperceptive visual agnosia, spatial agnosia and moderate constructional apraxia and were not justified by the degree of visual impairment (see Table 3). The patient also showed alterations in other cognitive functions: IPS, attention, EF and verbal and visual episodic memory. The cranial MR showed confluent periventricular demyelinating lesions, and other multiple non-confluent lesions in frontal and parietal and occipital areas, corpus callosum and also infratentorial, as well as signs of significant axonal damage and subcortical and cortical atrophy. In a second neuropsychological evaluation in 2009, cognitive performance was similar, and no progression of visual agnosia was found. However, she showed a slight decrease in SPI and EF. On the other hand, the patient displayed a high level of fatigue, symptoms of mild depression and mild anxiety.

Case number 4 is a 44-year-old woman with a very active form of RRMS, both clinical and radiological. She suffered her first episode of the disease in 2006, with visual problems that impeded her from moving into familiar places. In the following months, she experienced several relapses with sensory impairment and unstable gait. At a new neurological evaluation performed a year and a half later, cognitive impairment was observed together with pyramidal signs and mild cerebellar involvement, with an EDSS score of 3.5 (excluding cognitive impairment). In the neuropsychological evaluation, severe cognitive deficits were found: apperceptive visual agnosia, spatial agnosia, constructional apraxia, ideomotor apraxia and a significant alteration in coding and recovery of verbal episodic memory. She also exhibited dysfunctions in EF and naming (Table 4). A behavioural disorder was also observed, including infantilism and emotional liability. A mood disorder with marked symptoms of depression and anxiety was also present. The cranial MR showed multiple demyelinating lesions at periventricular and yuxtacortical level, with a relative predominance in the parietooccipital areas, and also an important number of lesions in the brain stem. There were an elevated number of black holes and several of the occipital lesions showed gadolinium enhancing. The patient was treated with different immunomodulating drugs from the beginning, but her cognitive dysfunction progressed incessantly to fully developed dementia. In 2011, after 5 years, the patient was in a state of absolute dependence, needing 24-hour care.

Case number 5 is a 57-year-old male patient with a history of hypertension and high levels of cholesterol. His relatives requested medical attention after observing progressive cognitive deterioration in the last 2 years, which impeded him from performing the required tasks at work. The patient, a waiter by profession, forgot the customers' orders, confused spaces at home and at work, had expression difficulties and was notably indifferent about his mistakes. The neurological exam showed only cognitive impairment (EDSS score 0, without considering cognitive impairment). The neuropsychological evaluation confirmed visual impairment in the perception of shape and space (apperceptive visual agnosia) and in colour naming (colour anomie), while maintaining perception of colour, topographical spatial disorientation, visuoconstructional impairment, preserving gestalt and simplification and ideomotor praxis for transitive gestures. The patient maintained verbal comprehension and the repetition of words but failed in the repetition of non-words. When dealing with spontaneous oral and verbal expression, there was a notable loss in naming abilities, construction of simple, grammatically incorrect sentences and hesitation. A decrease in the use of functional words and the presence of phonological and semantic paraphasia were also observed. Reading was considered adequate albeit slow and with some difficulty depending on the length of the sentence and presence of non-words. Verbal expression was the most affected, with difficulties with substitution, addition and omission, especially in longer and less frequent words. Spontaneous writing was characterized by grammatically incorrect sentences and a decrease in the use of functional words. These deficits resulted in difficulties in the phonological output lexicon, in acoustic-phonological conversion and in phoneme-grapheme conversion. Finally, the patient showed a low to moderate decrease in verbal episodic memory (coding), IPS, attention and EF (Table 5) as well as marked apathy. In another neuropsychological evaluation carried out 8 months later, mild progression of language disorder (anomie), semantic memory and free verbal long-term memory were all found. In the case history file collected from the family, the great effect of these difficulties on this patient's daily life activities could clearly be seen. The MR showed multiple lesions in the periventricular WM of both hemispheres and confluent plaques in the parietal-temporal-occipital areas (Figures 3b and c). Two cerebrospinal fluid samples examined in two different laboratories showed intrathecal IgG synthesis. Another MR performed one year later showed a new large left parietal yuxtacortical lesion.

Discussion

The patients we have described herein represent a particular clinical form of MS characterized by impairment in cognitive functions rarely affected in this disease, such as aphasia, alexia, agraphia and visual agnosia. Even though these cognitive processes are generally disturbed in cortical lesions, different pathological processes of the subcortical white matter can cause similar clinical syndromes (Damasio, 1992; Naeser et al., 1982).

Our first reported patient developed mild alexia with phonological agraphia in the context of a disease relapse, coincident with lesions in the left parietal and occipital areas, all evolving to complete remission at a later date. This deficit presented only a very mild repercussion in her daily life and social and occupational activities. In the second case, motor aphasia due to a large lesion in the WM of the left hemisphere was the first sign of disease, and it was not associated with any change in other cognitive functions and it also subsequently remitted. In this patient, the neuropsychological exam showed a large discrepancy between verbal and visual performance, not in accordance with spontaneous expression observed in normal conversation. This incongruence and the slight reduction in performance on SPI and attentive tasks can be explained by the elevated degree of anticipatory anxiety observed during the evaluation, as other authors have already observed (Nieto et al., 2008; Schulz, Kopp, Kunkel, & Faiss, 2006). The neuropsychological exam of the last three patients showed apperceptive agnosia and constructive apraxia in correspondence with bilateral lesions in the parietal and occipital areas. The last patient also showed alterations in linguistic processing. All but one of the cases (Case 2) exhibited cognitive impairment typical of MS, with some deficit in the encoding of verbal memory, SPI, attention and EF, similar to those described by other authors (Day et al., 1987; Devere et al., 2000; Okuda et al., 1996). We would like to highlight in three of our cases the good correlation encountered between apperceptive visual agnosia and the lesions observed in the parietal and occipital WM areas. Also remarkable is the recovery of language alterations observed in two of the patients.

NPS may be the initial form of presentation of MS (cases 2, 4 and 5) (Devere et al., 2000; Dogulu et al., 1996; Mao-Draayer & Panitch, 2004) or, more frequently, they may appear in the course of the disease in association with physical disability involving other functional systems (Cases 1 and 3) (Day et al., 1987; Okuda et al., 1996). When they constitute the only symptom and occur progressively, as in our Case no 5, it can be difficult to establish a diagnosis of MS (Stoquart-ElSankari et al., 2010); in this setting, the presence of intrathecal synthesis of immunoglobulins may be of great help. A rapid progression of cognitive dysfunction along with severe functional deterioration and dementia over a short lapse of time has been described in some other sporadic cases, similar to our Patient 4 (Staff et al., 2009; Stoquart-ElSankar et al., 2010). Some patients with cognitive impairment also display changes in the emotional sphere, mostly euphoria, emotional instability, apathy and/or symptoms of depression, also observed in our patients (Zarei et al., 2003; Zarei, 2006).

The prevalence of NPS in MS is unknown. Possible explanations are the lack of studies focused on these specific cognitive deficits, and the fact that routine tests used in clinical practice only contemplate patterns of cognitive impairment typical of MS (Boringa et al., 2001; Duque et al., 2012). In addition, the tests may be administered by non-specialized personnel not skilled enough as to detect other possible deficits which could deserve a more specific investigation. In spite of this, their prevalence is presumably low, as these cognitive deficits demand specialized care because of the serious functional disability that they usually generate. In fact, the presence of aphasia only represented 0.8% of the 2700 MS patients tested by Larner and Lecky (2007). In spite of this probably low prevalence, it is important to consider cognitive deterioration as a main symptom of MS, even in cases where other neurological disorders are minor, given their potential repercussion on socio-occupational activities.

The substrate of cognitive impairment of MS, and in these particular NPS, is possibly brain atrophy. It may be that in the neuropsychological disorders involving typically cortical areas (aphasia, agnosia, apraxia), the grey matter (GM) atrophy is more relevant than WM atrophy. For decades, we have known that around 20% of the lesions in MS are found in the GM (Brownell & Hughes, 1962), and its clinical relevance and its important contribution to cognitive symptomatology are well recognized. Three types of lesions are found in the cerebral cortex, namely cortical-subcortical lesions involving the white and grey matter, small perivascular demyelinating intracortical lesions, and extensive subpial demyelination bands that cover several gyri and three or four layers (Rudick & Trapp, 2009). Given the strong correlation between cognitive deterioration and cortical atrophy, it is highly probable that a large part of cognitive symptoms of MS was the result of progressive damage to the GM rather than a consequence of WM atrophy (Amato et al., 2004; Benedict et al., 2006).

Cognitive impairment in MS may lead to severe and permanent disability (Wynia, Middel, van Dijk, De Keyser, & Reijneveld, 2008) with significant socio-occupational impact (Rao et al., 1991). It is therefore necessary to perform properly designed neuropsychological studies, conducted by specialized professionals, in order to diagnose and better characterize cognitive impairment in patients with MS.

doi: 10.7334/psicothema2012.308

Acknowledgements

This work has been partially financed with project PSI2010/19348 of the Ministry of Science and Innovation and by Merc-Serono y Biogen Idec laboratories.

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Ma Jose Gil Moreno (1), Marta Cerezo Garcia (2), Raluca Marasescu (2), Ana Pinel Gonzalez (2), Laudino Lopez Alvarez (3) and Yolanda Aladro Benito (2)

(1) Hospital de Mostoles, (2) Hospital Universitario de Getafe and (3) Universidad de Oviedo

Received: November 9, 2012 * Accepted: June 27, 2013

Corresponding author: Yolanda Aladro Benito

Servicio de Neurologia Hospital Universitario de Getafe 28905 Getafe (Spain)

e-mail: yaladro.hugf@salud.madrid.org

Table 1
CASE 1: Clinical details and cognitive results

Sex                                               Female
Level of education (years of schooling)             11
Age in the first neuropsychological                 33
  evaluation (NPSE).
EDSS in the first NPSE.                              3
Disease duration in the first NPSE (Years).          6

MRI:

* T1 lesion volume                                 High
* T2 lesion volume                                 High
* Cerebral atrophy                                  Yes

TESTS                                                   SCORES
                                                         2007

WAIS-III (Wechsler Adult Intelligence Scale)          Max Score

  Digit Symbol-Coding                                     7
  Block Design                                            4
  Symbol Search                                           5
  Letter-number sequencing                                7

WMS-III (Wechsler Memory Scale)                       Max Score

  Logical Memory I                                        10
  Family Pictures I                                       13
  Logical Memory II                                       11
  Family Pictures II                                      12

Rey Osterrieth Figure (ROCF)                          Percentile

  Type of the copy                                        75
  Accuracy of copy                                        60
  Time of copy                                            75
  Immediate recall condition                              70

BNT (Boston Naming Test)                              Raw Score

  Total Score                                             58

CTMT (Comprehensive Trail Making Test)               Standard. S

  Tests 1/2/3/4/5                                   28/29/24/19/27
  Index                                                   23

BADS (Behavioural Assess. of Disexecutive              Profile
Syndrome)

  Temporal Judgement                                      2
  Zoo Map                                                 2

WCST (Wisconsin Card Sorting Test)                    Percentile

  Total % of errors                                       90
  % Perseverative Responses                               94
  No. of categories completed                            >16
  Trials to complete 1st category                        >16
  Failure to maintain set                                >16
  Learning to Learn                                      >16

PALPA (Psycholinguistic Assessment of
Language)

Reading                                                Z score
  Letter discrimination W/NW                          0.63/-2.63
  Visual lexical decision HIHF/HILF/LIHF/          0/-9.2/-4.2/0.55
    LILF
  Visual lexical decision. flex./deriv./            -2.8/0.33/7.9
    non-word.
  Reading HIHF/HILF/LIHF/ LILF                   0.23/0.23/-3.27/0.58
  Reading regular/Irregular.                           0.35/0.1
  Non words reading 5 letters/6 letters              -1.38/-0.62
  Reading sentences                                     -1.91
Writing                                                Z score
  Writing length 5 letters/6 letters                    0/0.3
  Writing to dictation HIHF/HILF/LIHF/ LILF     -1.91/-2.1/-4.25/-0.35
  Writing to dictation Noun/Adjective/Verb/      -1.2/0.31/0.31/-3.4
    function
  Writing imageability. Nouns/functional W           -1.78/-3.71
  Writing to dictation Regular/ Derived/           -3.4/-3.9/-1.68
    Irregular
  Writing non-words 5 letters/6 letters               0.27/-1.72

TESTS                                                  SCORES
                                                        2009

WAIS-III (Wechsler Adult Intelligence Scale)          Max Score

  Digit Symbol-Coding                                     6
  Block Design                                            8
  Symbol Search                                           6
  Letter-number sequencing                                9

WMS-III (Wechsler Memory Scale)                       Max Score

  Logical Memory I                                       13
  Family Pictures I                                      16
  Logical Memory II                                      13
  Family Pictures II                                     19

Rey Osterrieth Figure (ROCF)                         Percentile

  Type of the copy                                       75
  Accuracy of copy                                       80
  Time of copy                                           50
  Immediate recall condition                             30

BNT (Boston Naming Test)                              Raw Score

  Total Score                                            57

CTMT (Comprehensive Trail Making Test)               Standard. S

  Tests 1/2/3/4/5                                  26/34/37/30/29
  Index                                                  29

BADS (Behavioural Assess. of Disexecutive              Profile
Syndrome)

  Temporal Judgement                                      2
  Zoo Map                                                 4

WCST (Wisconsin Card Sorting Test)                   Percentile

  Total % of errors                                      86
  % Perseverative Responses                              92
  No. of categories completed                            >16
  Trials to complete 1st category                        >16
  Failure to maintain set                                >16
  Learning to Learn                                      >16

PALPA (Psycholinguistic Assessment of
Language)

Reading                                                Z score
  Letter discrimination W/NW                         0.54/-0.73
  Visual lexical decision HIHF/HILF/LIHF/              0/0/0/0
    LILF
  Visual lexical decision. flex./deriv./           -0.92/0.33/0.45
    non-word.
  Reading HIHF/HILF/LIHF/ LILF                   0.23/0.23/0.23/0.58
  Reading regular/Irregular.                          0.35/0.1
  Non words reading 5 letters/6 letters              -0.33/-0.33
  Reading sentences                                       0
Writing                                                Z score
  Writing length 5 letters/6 letters                    0/0.3
  Writing to dictation HIHF/HILF/LIHF/ LILF     0.64/0.35/0.66/-0.35
  Writing to dictation Noun/Adjective/Verb/     -0.53/0.31/0.31/-0.35
    function
  Writing imageability. Nouns/functional W             -0.35/0
  Writing to dictation Regular/ Derived/            0/0.23/-0.36
    Irregular
  Writing non-words 5 letters/6 letters              0.27/-0.95

TESTS                                            Normative
                                                  values

WAIS-III (Wechsler Adult Intelligence Scale)     Max Score

  Digit Symbol-Coding                               10
  Block Design                                      10
  Symbol Search                                     10
  Letter-number sequencing                          10

WMS-III (Wechsler Memory Scale)                  Max Score

  Logical Memory I                                  10
  Family Pictures I                                 10
  Logical Memory II                                 10
  Family Pictures II                                10

Rey Osterrieth Figure (ROCF)                    Percentile

  Type of the copy                                  50
  Accuracy of copy                                  50
  Time of copy                                      50
  Immediate recall condition                        50

BNT (Boston Naming Test)                         Raw Score

  Total Score                                       54

CTMT (Comprehensive Trail Making Test)          Standard. S

  Tests 1/2/3/4/5                                   50
  Index                                             50

BADS (Behavioural Assess. of Disexecutive         Profile
Syndrome)

  Temporal Judgement                                 2
  Zoo Map                                            2

WCST (Wisconsin Card Sorting Test)              Percentile

  Total % of errors                                 50
  % Perseverative Responses                         50
  No. of categories completed                       >16
  Trials to complete 1st category                   >16
  Failure to maintain set                           >16
  Learning to Learn                                 >16

PALPA (Psycholinguistic Assessment of
Language)

Reading                                           Z score
  Letter discrimination W/NW                         0
  Visual lexical decision HIHF/HILF/LIHF/            0
    LILF
  Visual lexical decision. flex./deriv./             0
    non-word.
  Reading HIHF/HILF/LIHF/ LILF                       0
  Reading regular/Irregular.                         0
  Non words reading 5 letters/6 letters              0
  Reading sentences                                  0
Writing                                           Z score
  Writing length 5 letters/6 letters                 0
  Writing to dictation HIHF/HILF/LIHF/ LILF          0
  Writing to dictation Noun/Adjective/Verb/          0
    function
  Writing imageability. Nouns/functional W           0
  Writing to dictation Regular/ Derived/             0
    Irregular
  Writing non-words 5 letters/6 letters              0

Table 2
CASE 2: Clinical details and cognitive results

Sex                                                          Female

Level of Education (years of                                   12
  schooling)
Age in neuropsychological evaluation                           30
  (NPSE).
EDSS in NPSE.                                                  3
Disease duration in the NPSE (years).                         1.8

MRI:

* T1 lesion volume                                           Medium
* T2 lesion volume                                            High
* Cerebral atrophy                                             No

TESTS                                        SCORE         Normative
                                              2010           Values

WAIS-III (Wechsler Adult Intelligence      Max Score       Max Score
Scale)

  Digit Symbol-Coding                          5               10
  Block design                                 10              10
  Symbol Search                                7               10
  Letter-Number sequencing                     8               10

WMS-III (Wechsler Memory Scale)            Max Score       Max Score

  Logical Memory I                             2               10
  Family Pictures I                            9               10
  Logical Memory II                            1               10
  Family Pictures II                           8               10

Rey Osterrieth Figure (ROCF)               Percentile      Percentile

  Type of the copy                             75              50
  Accuracy of copy                             90              50
  Time of copy                                 50              50
  Immediate recall condition                   50              50

BNT (Boston Naming Test)                   Raw Score       Raw Score

  Total Score                                  55              54

CTMT (Comprehensive Trail Making           Standard S      Standard S
Test)

  Tests 1/2/3/4/5                        33/29/29/29/38        50
  Index                                        29              50

BADS (Behavioural Assess. Of                Profile         Profile
Disexecutive Syndrome)

  Temporal Judgement                           1               2
  Zoo Map                                      2               2

WCST (Wisconsin Card Sorting Test)         Percentile      Percentile

  Total % of errors                            79              50
  % Perseverative Responses                    92              50
  No. of categories completed                 >16             >16
  Trials to complete 1st category             >16             >16
  Failure to maintain set                     >16             >16
  Learning to Learn                           >16             >16

PALPA (Psycholinguistic Assessment of       Z score         Z score
Language processing in Aphasia)

  Discrimination minimal pairs words       0.36/0.42           0
    Equal/
  Different Auditory lexical decision   0/0/0.23/-0.35/-       0
    HIHF/
  HILF/LIHF/ LILF                             0.6
  Repetition: Length 3L/4L/5L/6L        0.17/0.23/0.23/0       0
                                            -3.27/-
  Repetition: non-words 3L/4L/5L/6L      4.33/0.31/0.53        0

Table 3
CASE 3: Clinical details and cognitive results

Sex                                                       Female

Level of education (years of schooling)                      8
Age in the first neuropsychological evaluation               43
  (NPSE).
EDSS in the first NPSE.                                      5
Disease duration in the first NPSE (years).                  10

MRI:

* T1 lesion volume                                        Moderate
* T2 lesion volume                                          High
* Cerebral Atrophy                                          Yes

TESTS                                                      Scores
                                                            2004

WAIS-III (Wechsler Adult Intelligence Scale)            Scaled Score
  Digit Symbol-Coding                                        2
  Block design                                               5
  Digit Span                                                 8
  Symbol search                                              5
  Letter-Number sequencing                                   10
WMS-III (Wechsler Memory Scale)                         Scaled Score
  Logical Memory I                                           7
  Family Pictures I                                          6
  Logical Memory II                                          5
  Family Pictures II                                         7
  Spatial Span                                               5
Rey Osterrieth Figure (ROCF)                             Percentile
  Type of the copy                                           50
  Accuracy of copy                                           1
  Time of copy                                               10
  Immediate recall condition                                 10
BNT (Boston Naming Test)                                 Raw Score
  Total score                                                50
CTMT (Comprehensive Trail Making Test)                   Standard S
  Tests 1/2/3/4/5                                      18/18/18/18/18
  Index                                                      18
BADS (Behavioural Assess. of Disexecutive Syndrome)       Profile
  Temporal Judgement                                         3
  Zoo Map                                                    1
Verbal Fluency                                          Raw--Average
  Phonemic Fluency (FAS)                                     6
  Semantic Fluency (animals, fruit, supermarket)             12
WCST (Wisconsin Card Sorting Test)                       Percentile
  % total errors                                             5
  % Perseverative Responses                                  7
  No. of categories completed                                6
  Failure to maintain set                                   >16
  Learning to Learn                                         >16
VOSP (Visual Object and Space Perception Battery)        Raw Score
  Screening Test                                             20
  Incomplete Letters                                         16
  Silhouettes                                                12
  Object decision                                            14
  Progressive Silhouettes                                    14
  Dot counting                                               6
  Position Discrimination                                    20
  Number location                                            6
  Cube analysis                                              4

TESTS                                                      Scores
                                                            2009

WAIS-III (Wechsler Adult Intelligence Scale)            Scaled Score
  Digit Symbol-Coding                                        2
  Block design                                               5
  Digit Span                                                 7
  Symbol search                                              4
  Letter-Number sequencing                                   8
WMS-III (Wechsler Memory Scale)                         Scaled Score
  Logical Memory I                                           7
  Family Pictures I                                          6
  Logical Memory II                                          5
  Family Pictures II                                         7
  Spatial Span                                               5
Rey Osterrieth Figure (ROCF)                             Percentile
  Type of the copy                                           50
  Accuracy of copy                                           1
  Time of copy                                               25
  Immediate recall condition                                 1
BNT (Boston Naming Test)                                 Raw Score
  Total score                                                52
CTMT (Comprehensive Trail Making Test)                   Standard S
  Tests 1/2/3/4/5                                      18/18/18/18/18
  Index                                                      18
BADS (Behavioural Assess. of Disexecutive Syndrome)       Profile
  Temporal Judgement                                         3
  Zoo Map                                                    1
Verbal Fluency                                          Raw--Average
  Phonemic Fluency (FAS)                                     6
  Semantic Fluency (animals, fruit, supermarket)             12
WCST (Wisconsin Card Sorting Test)                       Percentile
  % total errors                                             3
  % Perseverative Responses                                  1
  No. of categories completed                                2
  Failure to maintain set                                    6
  Learning to Learn
VOSP (Visual Object and Space Perception Battery)        Raw Score
  Screening Test                                             19
  Incomplete Letters                                         11
  Silhouettes                                                17
  Object decision                                            10
  Progressive Silhouettes                                    13
  Dot counting                                               1
  Position Discrimination                                    13
  Number location                                            7
  Cube analysis                                              6

TESTS                                                    Normative
                                                          values

WAIS-III (Wechsler Adult Intelligence Scale)           Scaled Score
  Digit Symbol-Coding                                       10
  Block design                                              10
  Digit Span                                                10
  Symbol search                                             10
  Letter-Number sequencing                                  10
WMS-III (Wechsler Memory Scale)                        Scaled Score
  Logical Memory I                                          10
  Family Pictures I                                         10
  Logical Memory II                                         10
  Family Pictures II                                        10
  Spatial Span                                              10
Rey Osterrieth Figure (ROCF)                            Percentile
  Type of the copy                                          50
  Accuracy of copy                                          50
  Time of copy                                              50
  Immediate recall condition                                50
BNT (Boston Naming Test)                                 Raw Score
  Total score                                               54
CTMT (Comprehensive Trail Making Test)                  Standard S
  Tests 1/2/3/4/5                                           50
  Index                                                     50
BADS (Behavioural Assess. of Disexecutive Syndrome)       Profile
  Temporal Judgement                                         2
  Zoo Map                                                    2
Verbal Fluency                                         Raw--Average
  Phonemic Fluency (FAS)                                   12-13
  Semantic Fluency (animals, fruit, supermarket)           17-18
WCST (Wisconsin Card Sorting Test)                      Percentile
  % total errors                                            50
  % Perseverative Responses                                 50
  No. of categories completed                               >16
  Failure to maintain set                                   >16
  Learning to Learn                                         >16
VOSP (Visual Object and Space Perception Battery)      Cut-off Score
  Screening Test                                            15
  Incomplete Letters                                        17
  Silhouettes                                               16
  Object decision                                           15
  Progressive Silhouettes                                   14
  Dot counting                                               8
  Position Discrimination                                   18
  Number location                                            7
  Cube analysis                                              6

Table 4
CASE 4: Clinical details and cognitive results

Sex                                                         Female

Level of education (years of schooling)                        8
Age in neuropsychological evaluation                          44
  (NPSE).
EDSS in NPSE                                                  3.5
Disease duration in NPSE (years).                             1.5

MRI:

* T1 lesion volume                                           High
* T2 lesion volume                                           High
* Cerebral atrophy                                            Yes

TESTS                                        Score         Normative
                                              2008          values

WAIS-III (Wechsler Adult Intelligence     Scaled Score   Scaled Score
Scale)

  Picture completion                           1              10
  Vocabulary                                   9              10
  Similarities                                 6              10
  Block Design                                 1              10
  Digit Span                                   5              10

WMS-III (Wechsler Memory Scale)           Scaled Score   Scaled Score

  Logical Memory I                             3              10
  Logical Memory II                            1              10
  Mental control                               3              10
  Information and Orientation             Percentile 1   Percentile 50

Verbal fluency                            Raw-Average     Raw-Average

  Phonemic fluency (FAS)                       10            12-13
  Semantic fluency (animals, fruit,            14            17-18
    supermarket)

VOSP (Visual Object and Space              Raw Score     Cut-off Score
Perception Battery)

  Screening Test                               18             15
  Incomplete Letters                           0              17
  Silhouettes                                  2              16
  Object decision                              5              15
  Progressive Silhouettes                      20             14
  Dot counting                                 4               8
  Position Discrimination                      11             18
  Number location                              0               7
  Cube analysis                                1               6

Table 5
CASE 5: Clinical details and cognitive results

Sex                                                      Male

Level of education (years of schooling)                   12
Age in the first neuropsychological evaluation (NPSE      57
EDSS in the first NPSE                                     4
Disease duration in the first NPSE (years)                2.2

MRI:

* T1 lesion volume                                       High
* T2 lesion volume                                       High
* Cerebral atrophy                                        Yes

                                                        Score
TESTS                                                March 2012

WAIS-III (Wechsler Adult Intelligence Scale)        Scaled Score
  Block Design                                            5
WMS-III (Wechsler Memory Scale)                     Scaled Score
  Logical Memory I                                        6
  Logical Memory II                                       5
Rey Osterrieth Figure (ROCF)                         Percentile
  Type of the copy                                       75
  Accuracy of copy                                        1
  Time of copy                                           10
  Immediate recall condition                              1
CTMT (Comprehensive Trail Making Test)                Standard
  TEST 1                                                 18
  TEST 5                                                 18
Verbal Fluency                                        Raw Score
  Phonemic fluency (P)                                    4
  Semantic fluency(animals)                              13
VOSP (Visual Object and Space Perception Battery)     Raw Score
  Screening Test                                         18
  Silhouettes                                             7
  Object Decision                                         9
  Number Location                                         3
  Cube Analysis                                           4
BNT (Boston Naming Test)                              Raw Score
  Total Score                                            42
M@T (memory alteration test)                          Raw Score
  Total Score                                            37
FAB (Frontal Assessment Battery)                      Raw Score
  Total Score                                            10

                                                          Score
TESTS                                                 November 2012

WAIS-III (Wechsler Adult Intelligence Scale)               --
  Block Design                                             --
WMS-III (Wechsler Memory Scale)                            --
  Logical Memory I                                         --
  Logical Memory II                                        --
Rey Osterrieth Figure (ROCF)                               --
  Type of the copy                                         --
  Accuracy of copy                                         --
  Time of copy                                             --
  Immediate recall condition                               --
CTMT (Comprehensive Trail Making Test)                     --
  TEST 1                                                   --
  TEST 5                                                   --
Verbal Fluency                                             --
  Phonemic fluency (P)                                     --
  Semantic fluency(animals)                                --
VOSP (Visual Object and Space Perception Battery)       Raw Score
  Screening Test                                           20
  Silhouettes                                              12
  Object Decision                                           7
  Number Location                                           4
  Cube Analysis                                             4
BNT (Boston Naming Test)                                Raw Score
  Total Score                                              37
M@T (memory alteration test)                            Raw Score
  Total Score                                              31
FAB (Frontal Assessment Battery)                           --
  Total Score                                              --

                                                       Normative
TESTS                                                   values

WAIS-III (Wechsler Adult Intelligence Scale)         Scaled Score
  Block Design                                            10
WMS-III (Wechsler Memory Scale)                      Scaled Score
  Logical Memory I                                        10
  Logical Memory II                                       10
Rey Osterrieth Figure (ROCF)                          Percentile
  Type of the copy                                        50
  Accuracy of copy                                        50
  Time of copy                                            50
  Immediate recall condition                              50
CTMT (Comprehensive Trail Making Test)                 Standard
  TEST 1                                                  50
  TEST 5                                                  50
Verbal Fluency                                         Raw Score
  Phonemic fluency (P)                                   12-13
  Semantic fluency(animals)                              17-18
VOSP (Visual Object and Space Perception Battery)    Cut-off Score
  Screening Test                                          15
  Silhouettes                                             15
  Object Decision                                         14
  Number Location                                          7
  Cube Analysis                                            6
BNT (Boston Naming Test)                               Raw Score
  Total Score                                             54
M@T (memory alteration test)                         Cut-off Score
  Total Score                                             36
FAB (Frontal Assessment Battery)                     Cut-off Score
  Total Score                                             16
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No portion of this article can be reproduced without the express written permission from the copyright holder.
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Title Annotation:texto en ingles
Author:Moreno, Ma Jose Gil; Garcia, Marta Cerezo; Marasescu, Raluca; Gonzalez, Ana Pinel; Alvarez, Laudino
Publication:Psicothema
Date:Oct 1, 2013
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