Agraphia in
Dementia of the Alzheimer
Type
Emily LaBarge, EdD; Deborah S. Smith, PhD; Leah Dick, MA; Martha Storandt, PhD \s=b\ This
simple
study
describes
sentence in 368
graphic errors made in writing a healthy older adults and individuals
in different stages of dementia of the Alzheimer type. Errors of agraphia were present in both healthy and demented people and, in general, increased with the severity of dementia. The errors of agraphia were not correlated with measures of aphasia or psychometric measures of language and motor performance. Writing skill may represent procedural memory, and agraphia errors indicate alterations in long-term memory in dementia of the Alzheimer type. (Arch Neurol. 1992;49:1151-1156)
study was on linguistic competence and errors related to syntax. At least two articles have investigated agraphia and familial Alzheimer's disease finding either inconclu¬ sive or no relationships.1213 As pointed out by Goldstein,14 there are two types of agraphia: (1) impairment of the mo¬ tor act of writing and (2) impairment of the linguistic con¬ tent. The major focus of our study is on the motor compo¬ nent of writing. Several common clinical linguistic errors have also been included. We describe the graphic errors made in writing a simple sentence at different stages of DAT.
Further, we describe correlations of these errors with
aphasia and other measures of psychomotor performance. A companion article by Kemper and col¬ measures
may be defined simply as a loss or impairment of the ability to produce written language, caused by brain damage.1 (pl21) Historically agraphia has been linked with aphasia.2 Indeed, tests of writing ability have been used to screen for aphasia.1 Reports of the close association between agraphia and aphasia have been based on cases that, in general, could be attributed to focal lesions3 or to disorders of language.4 Ogle5 noted that there were occa¬ sional instances wherein aphasia and agraphia were sep¬ arable, although he believed they usually co-occurred. Subsequent articles have also described the occurrence of agraphia without aphasia. For example, Chedru and Geschwind6 reported impaired writing, but normal spon¬ taneous speech, in 33 of 34 cases of acute confusional states. In recent years much attention has been paid to Alz¬ heimer's disease, a disorder characterized by diffuse neu¬
Agraphia
rologic impairment. Although some investigators7 suggest that aphasia is invariably present in dementia of the Alzheimer type (DAT), others disagree. For example, an
earlier article from our research team8 indicated that 44% of the individuals in the mild stage of the disease were not aphasie. The prevalence of aphasia in DAT is probably de¬ pendent on the severity of the dementia.9-10 Few studies, however, have examined the prevalence of agraphia or the association between aphasia and agraphia in DAT. Neils et al11 compared descriptive writing abilities in 15 mildly to moderately demented patients with 15 matched control subjects. Although they used letter errors as their dependent measure, the major emphasis of their
Accepted
for publication July 20, 1992. From the Department of Neurology and Neurological Surgery (Dr LaBarge), Division of Biostatistics (Dr Smith), and Department of Psychology (Ms Dick and Dr Storandt), Washington University, St Louis, Mo.
Reprint requests to the Department of Neurology and Neurological Surgery, Box 8111, Washington University School of Medicine, 660 S Euclid, St Louis, MO 63110 (Dr Storandt).
of
leagues,15 based on the same data set, examines syntax. PATIENTS AND METHODS
Sample The sample was drawn from the Washington University Alzheimer's Disease Research Center Patient Registry, St Louis, Mo. Only individuals with DAT or healthy control subjects were included. Individuals with severe hypertension, stroke, other neurologic diseases such as Parkinson's disease, psychiatric illness, severe medical problems, or medications that may pro¬ duce dementia were excluded. Diagnostic and exclusionary cri¬ teria have been described elsewhere.16 Of the 422 identified registrants, 13 (nine with moderate dementia) could not produce any written output. An additional five individuals were not administered the task for reasons such as agitation or time constraints. Only two of the 25 severely de¬ mented individuals could perform the task; no further analyses were conducted with the severely demented because of the small number. The final sample size was 404; 179 nondemented (Clin¬ ical Dementia Rating [CDR]=0), 81 individuals with questionable or very mild dementia (CDR=0.5), 101 with mild dementia (CDR=1), and 43 with moderate dementia (CDR=2). Informed consent was obtained from participants and collateral sources after the nature of the study was explained. The diagno¬ sis of DAT was based on a 90-minute clinical interview (with both the participant and a collateral source) and a neurologic exami¬ nation including evaluation of near vision. Staging of dementia was made using the Washington University CDR)17-18 The CDR is based on clinical evaluations of cognitive abilities in six areas: memory, orientation, judgment and problem solving, community affairs, home and hobby activities, and personal care. Both the reliability of the CDR and the validity of the diagnosis of DAT (based on autopsy confirmation) have been reported elsewhere.19"21 Subsequent clinical follow-up and autopsy find¬ ings have demonstrated that individuals in the questionable stage of DAT were, by and large, in the very mild stage of DAT.22-23 Of the 92 individuals diagnosed with DAT who have come to
Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 05/31/2015
Table
1.—Percentage of Persons in Each Stage of Dementia Making One
Error
Type (Kappa) Capitalization (.92)
Mild
Moderate
2
13
21
33*
32*
17.33+
47
62
86*4
20.06+ 22.49+
6
65* 8
20*
32*
is/is (.75)
28
27
11*
36
Paraphasias (.90)
51
63
69*
75*
6
4
9
7
3
0
11*
8
18*
Spelling (.82)
Added marks (.80) Illegible words (.85)
1
3 3 consistency (.88) hoc comparisons, significantly different from nondemented P
Dementia of the Alzheimer
Type
Emily LaBarge, EdD; Deborah S. Smith, PhD; Leah Dick, MA; Martha Storandt, PhD \s=b\ This
simple
study
describes
sentence in 368
graphic errors made in writing a healthy older adults and individuals
in different stages of dementia of the Alzheimer type. Errors of agraphia were present in both healthy and demented people and, in general, increased with the severity of dementia. The errors of agraphia were not correlated with measures of aphasia or psychometric measures of language and motor performance. Writing skill may represent procedural memory, and agraphia errors indicate alterations in long-term memory in dementia of the Alzheimer type. (Arch Neurol. 1992;49:1151-1156)
study was on linguistic competence and errors related to syntax. At least two articles have investigated agraphia and familial Alzheimer's disease finding either inconclu¬ sive or no relationships.1213 As pointed out by Goldstein,14 there are two types of agraphia: (1) impairment of the mo¬ tor act of writing and (2) impairment of the linguistic con¬ tent. The major focus of our study is on the motor compo¬ nent of writing. Several common clinical linguistic errors have also been included. We describe the graphic errors made in writing a simple sentence at different stages of DAT.
Further, we describe correlations of these errors with
aphasia and other measures of psychomotor performance. A companion article by Kemper and col¬ measures
may be defined simply as a loss or impairment of the ability to produce written language, caused by brain damage.1 (pl21) Historically agraphia has been linked with aphasia.2 Indeed, tests of writing ability have been used to screen for aphasia.1 Reports of the close association between agraphia and aphasia have been based on cases that, in general, could be attributed to focal lesions3 or to disorders of language.4 Ogle5 noted that there were occa¬ sional instances wherein aphasia and agraphia were sep¬ arable, although he believed they usually co-occurred. Subsequent articles have also described the occurrence of agraphia without aphasia. For example, Chedru and Geschwind6 reported impaired writing, but normal spon¬ taneous speech, in 33 of 34 cases of acute confusional states. In recent years much attention has been paid to Alz¬ heimer's disease, a disorder characterized by diffuse neu¬
Agraphia
rologic impairment. Although some investigators7 suggest that aphasia is invariably present in dementia of the Alzheimer type (DAT), others disagree. For example, an
earlier article from our research team8 indicated that 44% of the individuals in the mild stage of the disease were not aphasie. The prevalence of aphasia in DAT is probably de¬ pendent on the severity of the dementia.9-10 Few studies, however, have examined the prevalence of agraphia or the association between aphasia and agraphia in DAT. Neils et al11 compared descriptive writing abilities in 15 mildly to moderately demented patients with 15 matched control subjects. Although they used letter errors as their dependent measure, the major emphasis of their
Accepted
for publication July 20, 1992. From the Department of Neurology and Neurological Surgery (Dr LaBarge), Division of Biostatistics (Dr Smith), and Department of Psychology (Ms Dick and Dr Storandt), Washington University, St Louis, Mo.
Reprint requests to the Department of Neurology and Neurological Surgery, Box 8111, Washington University School of Medicine, 660 S Euclid, St Louis, MO 63110 (Dr Storandt).
of
leagues,15 based on the same data set, examines syntax. PATIENTS AND METHODS
Sample The sample was drawn from the Washington University Alzheimer's Disease Research Center Patient Registry, St Louis, Mo. Only individuals with DAT or healthy control subjects were included. Individuals with severe hypertension, stroke, other neurologic diseases such as Parkinson's disease, psychiatric illness, severe medical problems, or medications that may pro¬ duce dementia were excluded. Diagnostic and exclusionary cri¬ teria have been described elsewhere.16 Of the 422 identified registrants, 13 (nine with moderate dementia) could not produce any written output. An additional five individuals were not administered the task for reasons such as agitation or time constraints. Only two of the 25 severely de¬ mented individuals could perform the task; no further analyses were conducted with the severely demented because of the small number. The final sample size was 404; 179 nondemented (Clin¬ ical Dementia Rating [CDR]=0), 81 individuals with questionable or very mild dementia (CDR=0.5), 101 with mild dementia (CDR=1), and 43 with moderate dementia (CDR=2). Informed consent was obtained from participants and collateral sources after the nature of the study was explained. The diagno¬ sis of DAT was based on a 90-minute clinical interview (with both the participant and a collateral source) and a neurologic exami¬ nation including evaluation of near vision. Staging of dementia was made using the Washington University CDR)17-18 The CDR is based on clinical evaluations of cognitive abilities in six areas: memory, orientation, judgment and problem solving, community affairs, home and hobby activities, and personal care. Both the reliability of the CDR and the validity of the diagnosis of DAT (based on autopsy confirmation) have been reported elsewhere.19"21 Subsequent clinical follow-up and autopsy find¬ ings have demonstrated that individuals in the questionable stage of DAT were, by and large, in the very mild stage of DAT.22-23 Of the 92 individuals diagnosed with DAT who have come to
Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 05/31/2015
Table
1.—Percentage of Persons in Each Stage of Dementia Making One
Error
Type (Kappa) Capitalization (.92)
Mild
Moderate
2
13
21
33*
32*
17.33+
47
62
86*4
20.06+ 22.49+
6
65* 8
20*
32*
is/is (.75)
28
27
11*
36
Paraphasias (.90)
51
63
69*
75*
6
4
9
7
3
0
11*
8
18*
Spelling (.82)
Added marks (.80) Illegible words (.85)
1
3 3 consistency (.88) hoc comparisons, significantly different from nondemented P
