Although cognitive dysfunction is known to occur in multiple sclerosis (MS), only few studies have reported cognitive performance in patients with primary progressive MS (PPMS). To find out the pattern of cognitive performance in PPMS, 28 PPMS patients underwent an extensive battery of neuropsychological tests. The results were compared to those of healthy controls (n = 20) and patients with secondary progressive MS (SPMS, n = 28). Furthermore, the results of neuropsychological tests in PPMS were correlated to magnetic resonance imaging findings. Our study showed that the PPMS patients have deficits in several cognitive domains when compared to age-matched and education-matched controls, but the cognitive impairment in the PPMS and SPMS patients appeared to be similar. Cognitive deficits in PPMS patients correlated with diffuse brain lesion, T1- and T2-lesion load, but no correlations were found with atrophy.
1 Rao SM Leo GJ Bernardin L Unverzagt F . Cognitive dysfunction in multiple sclerosis: frequency, patterns and prediction. Neurology1991; 41: 685–691.
2.
2 Amato MP Ponziani G Siracusa G Sorbi S . Cognitive dysfunction in early-onset multiple sclerosis: a reappraisal after 10 years. Arch Neurol2001; 58: 1602–1606.
3.
3 DeSousa EA Albert RH Kalman B . Cognitive impairments in multiple sclerosis: a review. Am J Alzheimers Dis Other Demen2002; 17: 23–29.
4.
4 Huijbregts SCJ Kalkers NF de Sonneville LMJ de Groot V Reuling IEW Polman CH . Differences in cognitive impairment of relapsing remitting, secondary and primary progressive MS. Neurology2004; 63: 335–339.
5.
5 Rao SM . Neuropsychology of multiple sclerosis: a critical review. J Clin Exp Neuropsychol1986; 8: 503–542.
6.
6 Zakzanis KK . Distinct neurocognitive profiles in multiple sclerosis subtypes. Arch Clin Neuropsychol2000; 15: 115–136.
7.
7 Rao SM Hammeke TA McQuillen MP Khatri BO Lloyd D . Memory disturbance in chronic progressive multiple sclerosis. Arch Neurol1984; 41: 625–631.
8.
8 Heaton RK Nelson LM Thompson DS Burks JS Franklin GM . Neuropsychological findings in relapsing-remitting and chronic-progressive multiple sclerosis. J Consult Clin Psychol1985; 53: 103–110.
9.
9 Rao SM Hammeke TA Speech TJ . Wisconsin Card Sorting Test performance in relapsing-remitting and chronic-progressive multiple sclerosis. J Consult Clin Psychol1987; 55: 263–265.
10.
10 Beatty WW Goodkin DE Beatty PA Monson N . Frontal lobe dysfunction and memory impairment in patients with chronic progressive multiple sclerosis. Brain Cogn1989; 11: 73–86.
11.
11 Feinsten A Kartsounis LD Miller DH Youl BD Ron MA . Clinically isolated lesions of the type seen in multiple sclerosis: a cognitive, psychiatric and MRI follow-up study. J Neurol Neurosurg Psychiatry1992; 55: 869–876.
12.
12 De Sonneville LMJ Boringa JB . reuling, IEW, Lazeron, RHC, Adèr, HJ, Polman, CH. Information processing characteristics in subtypes of multiple sclerosis. Neuropsychologia2002; 40: 1751–1765.
13.
13 Comi G Filippi M Martinelli V . Brain MRI correlates of cognitive impairment in primary and secondary progressive multiple sclerosis. J Neurol Sci1995; 132: 222–227.
14.
14 Foong J Rozewicz L Chong WK Thompson AJ Miller DH Ron MA . A comparison of neuropsychological deficits in primary and secondary progressive multiple sclerosis. J Neurol2000; 247: 97–101.
15.
15 Gaudino EA Chiaravalloti ND DeLuca J Diamond B . A comparison of memory performance in relapsing-remitting, primary progressive and secondary progressive multiple sclerosis. Neuropsychiatry Neuropsychol Behav Neurol2001; 14: 32–44.
16.
16 Wachowius U Talley M Silver N Heinze H-J Sailer M . Cognitive impairment in primary and secondary progressive multiple sclerosis. J Clin Exp Neuropsychol2005; 27: 65–77.
17.
17 Rao SM Glatt S Hammeke TA . Chronic progressive multiple sclerosis: relationship between cerebral ventricular size and neuropsychological impairment. Arch Neurol1985; 42: 678–682.
18.
18 Hohol MJ Guttman CR Orav J . Serial neuropsychological assessment and magnetic resonance imaging analysis in multiple sclerosis. Arch Neurol1997; 54: 1018–1025.
19.
19 Filippi M Tortorella C Rovaris M . Changes in the normal appearing brain tissue and cognitive impairment in multiple sclerosis. J Neurol Neurosurg Psychiatry2000; 68: 157–161.
20.
20 Edwards SGM Liu C Blumhardt LD . Cognitive correlates of supratentorial atrophy on MRI in multiple sclerosis. Acta Neurol Scand2001; 104: 214–223.
21.
21 Bermel RA Bakshi R Tjoa C Puli SR Jacobs L . Bicaudate ratio as a magnetic resonance imaging marker of brain atrophy in multiple sclerosis. Arch Neurol2002; 59: 275–280.
22.
22 Benedict RHB Weinstock-Guttman B Fishman I Sharma J Tjoa CW Bakshi R . Prediction of neuropsychological impairment in multiple sclerosis: comparison of conventional magnetic resonance imaging measures of atrophy and lesion burden. Arch Neurol2004; 61: 226–230.
23.
23 Lazeron RHC de Sonneville LMJ Scheltens P Polman CH Barkhof F . Cognitive slowing in multiple sclerosis is strongly associated with brain volume reduction. Mult Scler2006; 12: 760–768.
24.
24 Morgen K Sammer G Courtney SM . Evidence for a direct association between cortical atrophy and cognitive impairment in relapsing-remitting MS. Neuroimage2006; 30: 891–898.
25.
25 Rao SM Leo GJ Haughton VM Aubin-Faubert PS Bernardin L . Correlation of magnetic resonance imaging with neuropsychological testing in multiple sclerosis. Neurology1989; 39: 161–166.
26.
26 Swirsky-Sacchetti T Mitchell DR Seward J . Neuropsychological and structural brain lesions in multiple sclerosis: a regional analysis. Neurology1992; 42: 1291–1295.
27.
27 Arnett PA Rao SM Bernardin L Grafman J Yetkin FZ Lobeck L . Relationship between frontal lobe lesions and Wisconsin Card Sorting Test performance in patients with multiple sclerosis. Neurology1994; 44: 420–425.
28.
28 Brass SD Benedict RHB Weinstock-Guttman B Munschauer F Bakshi R . Cognitive impairment is associated with subcortical magnetic resonance imaging grey matter T2 hypointensity in multiple sclerosis. Mult Scler2006; 12: 437–444.
29.
29 Thompson AJ Kermode AG MacManus DG . Patterns of disease activity in multiple sclerosis: clinical and magnetic resonance imaging study. Br Med J1990; 300: 631–634.
30.
30 Thompson AJ Kermode AG Wicks D . Major differences in the dynamics of primary and secondary progressive multiple sclerosis. Ann Neurol1991; 29: 53–62.
31.
31 Thompson AJ Polman CH Miller DH . Primary progressive multiple sclerosis. Brain1997; 120: 1085–1096.
32.
32 Camp SJ Stevenson VL Thompson AJ . Cognitive function in primary progressive and transitional progressive multiple sclerosis. A controlled study with MRI correlates. Brain1999; 122: 1341–1348.
33.
33 Lublin FD Reingold SC . Defining the clinical course of multiple sclerosis: results of an international survey. National Multiple Sclerosis Society (USA) Advisory Committee on Clinical Trials of New Agents in Multiple Sclerosis. Neurology1996; 46: 907–911.
34.
34 Polman CH Reingold SC Edan G . Diagnostic criteria for multiple sclerosis: 2005 revision to the McDonald Criteria. Ann Neurol2005; 58: 840–846.
35.
35 McDonald WI Compston A Edan G . Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol2001; 50: 121–127.
36.
36 Kurtzke JF . Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology1983; 33: 1444–1452.
38 Heinonen T Dastidar P Eskola H Frey H Ryymin P Laasonen E . Applicability of semi-automatic segmentation for volumetric analysis of brain lesions. J Med Eng Technol1998; 22: 173–178.
39.
39 Heinonen T Dastidar P Kauppinen P Malmivuo J Eskola H . Semi-automatic tool for segmentation and volumetric analysis of medical images. Med Biol Eng Comput1998; 36: 291–296.
40.
40 Ukkonen M Dastidar P Heinonen T Laasonen E Elovaara I . Volumetric quantitation by MRI in primary progressive multiple sclerosis: volumes of plaques and atrophy correlated with neurological disability. Eur J Neurol2003; 10: 663–669.
41.
41 Lycklama á Nijeholt, GJ, van Walderveen, MA, Castelijns, JA, et al. Brain and spinal cord abnormalities in multiple sclerosis. Correlation between MRI parameters, clinical subtypes and symptoms. Brain 1998; 121(Pt 4): 687–697.
42.
42 Beck AT Ward CH Mendelson M Mock J Erbaugh JK . An inventory for measuring depression. Arch Gen Psychiatry1961; 4: 561–571.
43.
43 Revesz T Kidd D Thompson AJ Barnard RO McDonald WI . A comparison of the pathology of primary and secondary progressive multiple sclerosis. Brain1994; 117: 759–765.
44.
44 Lucchinetti C Bruck W Parisi J Scheithauser B Rodriguez M Lassmann H . Heterogeneity of multiple sclerosis lesions: implications for the pathogenesis of demyelination. Ann Neurol2000; 47: 707–717.
45.
45 Parmenter BA Weinstock-Guttman B Garg N Munschauer F Benedict RHB . Screening for cognitive impairment in multiple sclerosis using the Symbol digit Modalities Test. Mult Scler2007; 13: 52–57.
46.
46 Rosti-Otajärvi E Hämäläinen P Koivisto K Hokkanen L . The reliability of the MSFC and its components. Acta Neurol Scand2008; 117: 421–427.
47.
47 Filippi M Iannucci G Tortorella C . Comparison of MS clinical phenotypes using conventional and magnetization transfer MRI. Neurology1999; 52: 588–594.
48.
48 Rovaris M Bozzalli M Santuccio G . In vivo assessment of the brain and cervical cord pathology of patients with primary progressive multiple sclerosis. Brain2001; 124: 2540–2549.
49.
49 Tortorella C Viti B Bozzalli M . A magnetization transfer histogram study of normal-appearing brain tissue in MS. Neurology2000; 54: 186–193.
50.
50 Rocca MA Iannucci G Rovaris M Comi G Filippi M . Occult tissue damege in patients with primary progressive multiple sclerosis is independent of T2-visible lesions. A diffusion tensor MR study. J Neurol2003; 250: 456–460.
52 Corsi PM; . Human memory and the medial temporal region of the brain (unpublished doctoral thesis). Montreal: Department of Psychology, McGill University; 1972.
53.
53 Gronwall DM . Paced auditory serial-addition task: a measure of recovery from concussion. Percept Mot Skills1977; 44: 367–373.
54.
54 Stroop JR . Studies of interference in serial verbal reactions. J Exp Psychol1935; 18: 643–662.
55.
55 Smith A . The symbol digit modalities test manual: revised.Los Angeles: Western Psychological Services; 1982.
56.
56 Heaton, RK, Chelune, GJ, Talley, JL, Kay, GG, Curtis, G. Wisconsin card sorting test manual. Revised and expanded.Odessa: Psychological Assessment Resources, Inc; 1993.
57.
57 Laine M Goodglass H Niemi J Koivuselkä-Sallinen P Tuomainen J Marttila R . Adaption of the Boston Diagnostic Aphasia Examination and the Boston Naming Test into Finnish. Scand J Logoped Phoniatr1993; 18: 83–92.
58.
58 Benton AL . The revised visual retention test.New York: Psychological Corp; 1963.
