Table 1.

Comparison of clinical features in frontotemporal lobar degeneration (FTLD) and AD

CharacteristicsAgreement, %  (κ)*    FTLD, % (N)AD, % (N)p ValueOdds ratio§
* Percent agreement between raters is given for each feature. NA = not applicable (i.e., this is continuous data); NM = not measurable (in these cases, the absence of variability in at least one of the raters’ ratings for this feature in the subgroup of patients used for replicability testing made calculation of κ impossible).
† Percentage of patients in each group (of 30) that showed this clinical feature, and total number (N) of patients in this group (of 30) deemed to have sufficient evidence for the chart reviewers to make a judgment regarding presence of particular feature.
p Value for the comparison of proportions across groups using the χ2 test.
§ The odds in this group of having FTLD, as opposed to AD, if this clinical feature is present. NC = not calculable, because one group has no members with this feature.
** p < 0.05. ND = no difference between proportions.
¶ This variable used for logistic regression analysis (see Methods).
MMSE = Mini-Mental State Examination.
Demographic
    Age at presentationNA59.866.30.009**NA
    Women, %NA48520.711NA
    MMSE scoreNA19.4619.600.942NA
    Insidious onset100 (NM)100 (30)100 (30)ND1
    Gradual progression100 (NM)100 (30)100 (30)ND1
    Age at onset <65 y93 (0.76**)76 (30)47 (29)0.022**3.14**
    Family history100 (1**)57 (21)38 (29)0.222.10
General behavioral
    Social conduct disorder100 (1**)80 (26)0 (28)<0.001**NC
    Personal conduct disorder87 (0.44**)86 (28)31 (26)<0.001**11.0
    Emotional blunting93 (0.89)72 (25)13 (24)<0.001**13.5**
    Impaired insight87 (0.74)85 (26)56 (25)0.025**3.14**
    Mental rigidity80 (0.61**)88 (16)10 (10)<0.001**25.4**
    Decline in hygiene80 (0.57**)64 (25)27 (26)0.008**3.76**
    Alterations in diet87 (0.73**)56 (25)0 (25)<0.001**NC
    Stereotyped behavior93 (0.86**)87 (23)47 (15)0.007**6.57**
    Utilization behavior93 (0.76**)27 (18)23 (13)0.7681.8
    Narrowed preoccupations87 (0.61**)82 (17)22 (9)0.003**12.23**
    Parsimony100 (NM)20 (10)0 (10)0.136NC
    Incontinence93 (0.84**)38 (24)11 (19)0.044**6.0**
Speech and language
    Alteration in speech output87 (0.73**)80 (25)3 (13)0.01**10.0**
    Stereotyped utterances87 (0.6**)53 (17)18 (11)0.0666.0**
    Nonfluent speech100 (NM)21 (24)7 (30)0.1242.8
    Stuttering100 (NM)9 (23)0 (28)0.111NC
    Agrammatism100 (NM)0 (12)5 (21)0.443NC
    Phonemic paraphasias80 (0.53**)8 (13)14 (21)0.5620.31
    Anomia87 (0.74**)56 (18)65 (26)0.5100.38
    Impaired repetition87 (0.42)30 (27)21 (28)0.4851.46
    Echolalia87 (−0.07)8 (24)3 (26)0.5042.07
    Perseverative utterances80 (−0.1)55 (22)50 (16)0.7821.83
    Mutism93 (0.63**)33 (21)0 (9)0.048**NC
Neuropsychological
    Absence of severe amnesia87 (0.71**)66 (27)3 (30)<0.001**43.45**
    Impairment on frontal lobe tests80 (0.33)86 (21)47 (23)0.008**2.59
    Impaired working memory (WORLD)100 (1**)62 (26)58 (26)0.7771.14
    Absence of clinical perceptual difficulties93 (0.86**)85 (27)30 (26)<0.001**9.04**
    Sparing of pentagon copy100 (1**)35 (26)54 (26)0.1631.96**
Physical Findings
    Motor neuron disease100 (1**)10 (30)0 (30)0.076NC
    Bulbar palsy93 (0.76**)9 (22)0 (25)0.123NC
    Akinesia87 (0.6**)35 (23)7 (27)0.016**5.09
    Primitive reflexes100 (1**)25 (20)31 (26)0.6670.55