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Neuropsychiatric Dysfunction in a Patient With Whipple's Disease
Neuropsychiatric Dysfunction in a Patient With Whipple's Disease: Effects ofAntibiotic Treatment RALPH E. TARTER, PH.D. NORANN EDWARDS, M.S.W. AMY HAYS, B.S. DAVID H. VAN THIEL. M.D.
'lXThipple's disease is an infection caused by VV an unidentified Bacillus. The characteristic histopathologic lesion shows deposition of PAS-positive "sickle-like" particles within macrophages (SPC-cell) in the intestinal lamina propria mucosae. Although presentation is gastrointestinal in nature. Whipple's disease has been reported to include neurological involvement. implying blood-borne transmission. I In the central nervous system. PAS-positive deposits are seen in perivascular macrophages compressing adjacent neurons. 2 These cells are distributed throughout the cerebral cortex and cerebellum; however. they are concentrated within the temporal cortex. hippocampus, putamen, amygdala. thalamus, periventricular region. mammillary nuclei and the gray matter surrounding the aqueduct of Sylvius. 3 If untreated. the neurological course shows a variable progression characterized by pyramidal and extrapyramidal signs and. in advanced cases. akinetic mutism that may presage death. Several neuropsychiatric disturbances have been described in patients with Whipple's disease, most commonly affective dyscontrol with dementia. To date. however. no objective studies have investigated the functional impairments characterizing the putative dementia. which has been compared to that of Alzheimer's disease. 2.4.5 In the present case study. psychometric tests were used to evaluate cerebral integrity in an VOLUME 31· NUMBER 2· SPRING 1990
individual with Whipple's disease who had no overt neurologic disease on physical examination. Neuropsychologic tests were used to differentiate the dementia seen in Whipple's disease from that of other dementias. particularly Alzheimer's disease. The reversibility of neurologic deficits seen in Whipple's disease following long-term antibiotic therapy was then investigated by following the patient prospectively.
Case Report A.H., a 41-year-old white single male with 12 years of education, was employed as a produce manager in a supermarket. He was referred for a neuropsychologic evaluation following histologic confirmation of intestinal Whipple's disease. For several months prior to presentation, the patient noted rapid unexplained weight loss and diarrhea. At 5' I I". he weighed 122 Ibs, the least he had ever weighed as an adult. Results of several laboratory tests ordered during the course of his work-up are presented in Table
Received April 5. 1988; revised June 17. 1988; accepted January 4. 1989. From the Depanment of Psychiatry. University of Pittsburgh School of Medicine. Pittsburgh. Address reprint requests to Dr. Taner, Depanment of Psychiatry. University of Pittsburgh School of Medicine. 3811 O'Hara Street. Pittsburgh. PA 15213. Copyright © 1990 The Academy of Psychosomatic Medicine.
225
Case Reports
TABLE I. Laboratory measures or a patient with Whipple's disease before and after antibiotic treatment Measure
Before Treatment
After Treatment
143
118
1.9 745 359 122.6
3.9 241
Angiotensin-converting enzyme (units) Albumin (gld!) Alkaline phosphatase (lUlL) Stool weight (g/72 hr) Stool fat (g/72 hr) Stool osmolarity: mosm/L. Na+mMoVL K+ mMollL Folic acid (mglm!) (mglm!) Iron (l1g) Vitamin E (mgld!) Hemoglobin (gld!)
812
Mean corpuscular volume (M J )
1373 226 168 3.0
559 13.0 320 86 110 12.0
10.5
980 120 1.2 15.4
75.2
91.7
500 75 0.6
I. These. in combination with biopsies of the duodenum. small bowel and rectal-sigmoid colon. led to the diagnosis of Whipple's disease. Figures I and 2 illustrate characteristic biopsy findings by light and electron microscopy. Following the diagnosis of Whipple's disease the patient was given a battery of neuropsychologic tests and then started on a regimen of oral phenoxymethylpenicillin (2 g/day). The second neuropsychiatric evaluation was obtained 17 months later, after repeat small bowel biopsy showed resolution of the infection. At this time. the patient weighed 183 Ibs. The second test session was requested to ascertain the presence of any residual cognitive or psychomo1or disorder following successful treatment. In addition, a structured interview was conducted to evaluate psychiatric status on each of two occasions. There was no history of psychiatric or neurologic symptoms.
Neuropsychological Assessment An identical neuropsychological battery was administered by a trained psychometrician in two two-hour testing sessions before and 17 months after the initiation of antibiotic trea1ment. The battery contained the following tests: Diagnostic Interview Schedule (DIS); Shipley Institute of Living Scale; Token Test; 226
lG RE I. Duodenal biop ie of .H. Lowerpower photomi rograph obtained prior to antibioti therapy (Figure 1 ) demonstrates numer u P -posili e macrophag within the lamina propria mu 0 e. High-power photomicrograph obtained afler antibiotic lherapy Figur 18) how complete r olulion.
Animal Naming Tes1; Stroop Color Word Test; Trailmaking Test; Symbol Digit Modalities Test; Benton Visual Retention Test; Block Design Test; Finger Tapping Test; Grooved Pegboard; Digit Span; Rey-Osterreith Figure Test; and Digit Span + I. This battery was assembled to assess global cognitive and psychomotor capacities. Reliability and validity data have been previously described.6--X
Results The scores obtained by AH. in each test session and the percent change between sessions are presented in Table 2. Table 2 also lists the scores for each test that distinguish neurologically normal from abnorPSYCHOSOMATICS
Case Reports
leetroD photomicrographs of th duodenal mue a obtain d from .M. Low-power photomicrograph (Figure 2A) demon trate bacillary bodi in macropha e obtained b fore antibi lic ther. ap.. Higher-power photomicrograph ( igure 28 ,also taken prior to antibioti therapy, demon trat the pre ence of bacillary bodies within macrophag . Low-power pholomi rograph obtain dafter treatmenl igure 2 demon Irat the absence of bacillary bodie within macrophag in the int linallamina propria muc ae. Higher·power ph lomi rograph obtained aft r Irealment Figure 20) demon trate the ab ence of bacillary bodie within macrophage in the intI'. tinallamina propria mucosae.
mal individuals (cut-off scores).7-9 These data indicate that A.H. is of average intelligence. The results of the first testing session revealed impairments in spatial sequencing (Trailmaking, Pan B), spatial scanning and concentration (Symbol Digit), psychomotor efficiency (Grooved Pegboard), learning (Digit Span + I), immediate memory (Digit Span), and shon-term memory (Benton Visual Retention Test). Some of these deficits were only mildly to moderately severe compared with absolute levels of performance; however, the impairments are striking in light of the patient's intellectual capacity. For example, A.H. 's IQ falls in the 60th percentile of the general population, while his visuospatial capacity, as measured by the Block Design Test, was in VOLUME 31 • NUMBER 2· SPRING 1990
only the sixth percentile, and immediate memory, as measured by Digit Span, was in the first percentile. Visual perception (Rey-Osterreith), perceptual speed (Stroop), and motor speed were not impaired. No verbal deficits were observed. The deficits observed at the time of diagnosis were only panially ameliorated following a 17month course of antibiotic treatment and full resolution of the intestinal infection. On three of the six tests in which A.H. scored in the neurologically impaired ranges in the pre-treatment period, he continued to perform in the deficit range after treatment. They included the Trailmaking Test, Pan B; the Digit Span; and the Benton Visual Retention Memory Test. In fact, visuopractic performance (Trail227
Case Repons
TABLE 2.
Neuropsychological test results of a patient with Whipple's disease before and after antibiotic treatment Before Treatment
After Treatment
37
42
13.5
<40
4 3
4 4
0 33.3
<5 <3
81 109
62 73
23.5 30.3
>80 >85
Finger tapping (taps per 10 sees.) dominant hand nondominant hand
44 46
52 50
18.2 8.7
<45 <40
Block design (best score. 48)
24
29
20.8
28
Stroop (sees.) words colors interference
10 15 35
10 29 29
0 20.0 17.1
>25 >33 >72
Test Symbol digit score Digit span (number of digits correct) forward backward Grooved pegboard (sees.) dominant hand nondominant hand
Percent Change
Cut-orr Score"
Tokens (number correCI)
II
10
-9.\
>9
Trails A (sees.)
21
31
-47.6
>39
Trails B (sees.)
96
99
-3.1
>91
Animal naming (number of responses)
26
30
15.4
<20
18 12.0 104
16 12.0 102
-10.8 0 -1.9
NA NA NA
3
4
25.0
<7
34
32
-5.9
<30
2
1
50.0
<3
Shipley Institute of Living Scale (best score. 20) vocabulary abstract (WAIS)IQ Bemon Visual Retention Test (best score. 10) Rey-Osterreith Figure (best score. 36) copy Digit span + I
h
"Cut-off scores indicate scores thaI suggest impaired functioning. ~ot applicable
making) declined from the first to the second testing session. In contrast. psychomotor efficiency (Grooved Pegboard) and visual scanning (Symbol Digit) were essentially normal at the second testing. The deficits that persisted following resolution of Whipple's disease were those involving visuopractic and memory processes. The DIS revealed no significant current or lifetime psychopathology.
Discussion
This case provided a unique opportunity to delineate the extent of cerebral dysfunction in the early 228
stages of Whipple's disease and to assess the efficacy of antibiotic therapy in reversing the impairments identified. Caution must be exercised in generalizing these findings to all patients with Whipple's disease. However, the observation of memory, visuospatial, and psychomotor deficits in a patient without overt signs or symptoms of neurologic abnormality implies some degree of cerebral pathology. This is consistent with earlier observations that histologic evidence of Whipple's disease can occur without clinical evidence of cerebral involvement. 10 This report, along with the report of Sierachi et aI., 10 suggests PSYCHOSOMATICS
Case Reports
that central nervous system involvement in Whipple's disease may be much more frequent than previously recognized and may occur early in the course of the disease. In addition, the finding of persistent memory and visuospatial deficits after antibiotic treatment suggests that the neuropathology of Whipple's may have long-tenn sequelae. Indeed, the mean change across all tests was only 10.2%, well within the acceptable range of test perfonnance variation. Thus, it can be concluded that no significant overall improvement occurred during the 17-month treatment period. Whether a more aggressive antibiotic regimen might reverse the persisting neuropsychological deficits is unknown; however, the possibility of pennanent cerebral dysfunction cannot be ruled out. The use of psychiatric and psychometric testing made it possible to identify dementia associated with Whipple's disease as well as to differentiate it from that of other dementing conditions. For example, unlike patients in the early stages of Alzheimer's disease, A.H. demonstrated no detectable behavioral or psychiatric disturbances. Nonetheless, on tests of language and memory, he perfonned similarly to demented indi viduals with other conditions, such as Alzheimer's disease. Finally, the present case study underscores the value of neuropsychologic testing for evaluating the cerebral dysfunction associated with metabolic or infectious diseases as well as for monitoring the progress of treatment. Inasmuch
as such testing is predictive, to a large degree, of social and vocational adjustment, II its use can enhance comprehensive medical management. Testing is especially valuable when compromised functional capacities may affect the safety of the patient or others. Thus, patients with serious impainnents in visuospatial or psychomotor functions may be cautioned to be aware of such limitations when driving or operating machinery. Although the present study clearly documents the presence of neuropsychologic deficits in a patient with Whipple's disease, the underlying mechanisms of the deficits remain unclear. Blood-borne transmission of the infectious agent responsible for Whipple's disease may explain its systemic effects. Other factors, such as malnutrition secondary to malabsorption, could also potentially have contributed to the deficits. However, biochemical measures of nutritional status, substantial weight gain, and resolution of the malabsorptive disorder discount the importance of malnutrition as the predominant etiological factor. Moreover, emotional distress secondary to a chronic illness could have contributed to the manifest neuropsychologic deficits, although in view of the absence of significant psychopathology revealed on the Diagnostic Interview Schedule, it is not likely that it was a major factor in this case. Nonetheless, it is most probable that the observed impainnents have a multifactorial etiology, which in addition to the direct eNS infection, includes psychological and other organically mediated disturbances.
References I. Maizel H. Raffin J. Dobbins W: Whipple's disease: a review of patients from one hospital and a review of the literalure since 1950. Medicine 49: 175-205. 1970 2. Lampen P. Tom M. Cummings J: Encephalopathy in Whipple's disease: a histochemical study. NeurolollY 12:65-71, 1962 3. Smith W. French J. Gottsman M. et al: Cerebral complication of Whipple's disease. Brain 88: 137-150. 1965 4. Lishman W: Orllanic Psychiatry. Oxford. Blackwell Scientific Publications. 1978 5. Badenoch J. Richard W. Oppenheimer D: Encephalopathy in a case of Whipple's disease. J Neurol Neurosur[? Psychiatry 26:203-210. 1963
VOLUME 31 • NUMBER 2· SPRING 1990
6. Robins L. Helzer J. Croughan J. et al: National Institute of Mental Health Diagnostic Interview Schedule. Arch Gen Psychiatry 38:381-389. 1981 7. Goldstein G. Taner R. Shelly C. et al: The Pittsburgh Initial Neuropsychological Testing System (PINTS): a neuropsychological screening battery for psychiatric patients. Journal of Behal'ioral Assessment 5:227-238. 1983 8. Lezak M: Neuropsycholollical Assessment (second ed). New York. Oxford University Press. 1983 9. Goldstein S. Deysach R. Kleinknecht R: Effect of experience and amount of information on identification of cerebral impairment. J Consult Clin Psychol41 :30-34.
229
Case Reports
1973 10. Sierachi J. Fine G. Hom R. et al: Central nervous system involvement in Whipple's disease. J Neuropalhol Exp Neuro/19:7{}-75.1960
M~~lNp
PSYCHOSOMATIC MEDICINE
T
he Academy of Psychosomatic Medicine stresses the ongoing education of its members-through clinical practice, study. and research-to advocate a psychosomatic approach that considers biologic. psychological and social factors in the prevention. diagnosis. and treatment of illness.
Membership .. entitles you to receive
II. Heaton R. Pendleton M: Use of neuropsychological tests to predict adult patients' everyday functioning. J Consull Clin Psyc/IO/ 49:1I07-821. 19111
professionals with special qualifications may be invited and considered for membership if approved by the executive council. Now in its 35th year, the Academy continues to work for the goal set forth by its founders:
The integration of medical treatment for illness-organic and psychologicwith the practice of medicine to ensure the best comprehensive care for all patients. For further information about membership in the Academy. clip and send the coupon below.
Psychosomatics throughout the year... provides opportunities to meet and exchange information while Executive Director earning Continuing ~lediC'JI Education credit at the Academy of Psychosomatic Medicine SH24 Magnolia. Chicago. II. 60660 prestigious annual meeting ...enables you to participate. after three years. in the distinguished fellowship Please send me fur/ber information regarding program. culminating in certifkation as a Fellow of the membership. Academy of Psychosomatic \Iedicine... is open to physicians and psychologists. as well as social workers Name and nurses with a doctoral degree who document Street active participation in clinical practice or research in City the psychosomatic approach to medicine. Associate membership is open to qualified health professionals State Zip with_ masters level In special other _ __ _ training. ___ _ _instances, ___ _--L
230
_ _ _
_
.J
PSYCHOSOMATICS
'lXThipple's disease is an infection caused by VV an unidentified Bacillus. The characteristic histopathologic lesion shows deposition of PAS-positive "sickle-like" particles within macrophages (SPC-cell) in the intestinal lamina propria mucosae. Although presentation is gastrointestinal in nature. Whipple's disease has been reported to include neurological involvement. implying blood-borne transmission. I In the central nervous system. PAS-positive deposits are seen in perivascular macrophages compressing adjacent neurons. 2 These cells are distributed throughout the cerebral cortex and cerebellum; however. they are concentrated within the temporal cortex. hippocampus, putamen, amygdala. thalamus, periventricular region. mammillary nuclei and the gray matter surrounding the aqueduct of Sylvius. 3 If untreated. the neurological course shows a variable progression characterized by pyramidal and extrapyramidal signs and. in advanced cases. akinetic mutism that may presage death. Several neuropsychiatric disturbances have been described in patients with Whipple's disease, most commonly affective dyscontrol with dementia. To date. however. no objective studies have investigated the functional impairments characterizing the putative dementia. which has been compared to that of Alzheimer's disease. 2.4.5 In the present case study. psychometric tests were used to evaluate cerebral integrity in an VOLUME 31· NUMBER 2· SPRING 1990
individual with Whipple's disease who had no overt neurologic disease on physical examination. Neuropsychologic tests were used to differentiate the dementia seen in Whipple's disease from that of other dementias. particularly Alzheimer's disease. The reversibility of neurologic deficits seen in Whipple's disease following long-term antibiotic therapy was then investigated by following the patient prospectively.
Case Report A.H., a 41-year-old white single male with 12 years of education, was employed as a produce manager in a supermarket. He was referred for a neuropsychologic evaluation following histologic confirmation of intestinal Whipple's disease. For several months prior to presentation, the patient noted rapid unexplained weight loss and diarrhea. At 5' I I". he weighed 122 Ibs, the least he had ever weighed as an adult. Results of several laboratory tests ordered during the course of his work-up are presented in Table
Received April 5. 1988; revised June 17. 1988; accepted January 4. 1989. From the Depanment of Psychiatry. University of Pittsburgh School of Medicine. Pittsburgh. Address reprint requests to Dr. Taner, Depanment of Psychiatry. University of Pittsburgh School of Medicine. 3811 O'Hara Street. Pittsburgh. PA 15213. Copyright © 1990 The Academy of Psychosomatic Medicine.
225
Case Reports
TABLE I. Laboratory measures or a patient with Whipple's disease before and after antibiotic treatment Measure
Before Treatment
After Treatment
143
118
1.9 745 359 122.6
3.9 241
Angiotensin-converting enzyme (units) Albumin (gld!) Alkaline phosphatase (lUlL) Stool weight (g/72 hr) Stool fat (g/72 hr) Stool osmolarity: mosm/L. Na+mMoVL K+ mMollL Folic acid (mglm!) (mglm!) Iron (l1g) Vitamin E (mgld!) Hemoglobin (gld!)
812
Mean corpuscular volume (M J )
1373 226 168 3.0
559 13.0 320 86 110 12.0
10.5
980 120 1.2 15.4
75.2
91.7
500 75 0.6
I. These. in combination with biopsies of the duodenum. small bowel and rectal-sigmoid colon. led to the diagnosis of Whipple's disease. Figures I and 2 illustrate characteristic biopsy findings by light and electron microscopy. Following the diagnosis of Whipple's disease the patient was given a battery of neuropsychologic tests and then started on a regimen of oral phenoxymethylpenicillin (2 g/day). The second neuropsychiatric evaluation was obtained 17 months later, after repeat small bowel biopsy showed resolution of the infection. At this time. the patient weighed 183 Ibs. The second test session was requested to ascertain the presence of any residual cognitive or psychomo1or disorder following successful treatment. In addition, a structured interview was conducted to evaluate psychiatric status on each of two occasions. There was no history of psychiatric or neurologic symptoms.
Neuropsychological Assessment An identical neuropsychological battery was administered by a trained psychometrician in two two-hour testing sessions before and 17 months after the initiation of antibiotic trea1ment. The battery contained the following tests: Diagnostic Interview Schedule (DIS); Shipley Institute of Living Scale; Token Test; 226
lG RE I. Duodenal biop ie of .H. Lowerpower photomi rograph obtained prior to antibioti therapy (Figure 1 ) demonstrates numer u P -posili e macrophag within the lamina propria mu 0 e. High-power photomicrograph obtained afler antibiotic lherapy Figur 18) how complete r olulion.
Animal Naming Tes1; Stroop Color Word Test; Trailmaking Test; Symbol Digit Modalities Test; Benton Visual Retention Test; Block Design Test; Finger Tapping Test; Grooved Pegboard; Digit Span; Rey-Osterreith Figure Test; and Digit Span + I. This battery was assembled to assess global cognitive and psychomotor capacities. Reliability and validity data have been previously described.6--X
Results The scores obtained by AH. in each test session and the percent change between sessions are presented in Table 2. Table 2 also lists the scores for each test that distinguish neurologically normal from abnorPSYCHOSOMATICS
Case Reports
leetroD photomicrographs of th duodenal mue a obtain d from .M. Low-power photomicrograph (Figure 2A) demon trate bacillary bodi in macropha e obtained b fore antibi lic ther. ap.. Higher-power photomicrograph ( igure 28 ,also taken prior to antibioti therapy, demon trat the pre ence of bacillary bodies within macrophag . Low-power pholomi rograph obtain dafter treatmenl igure 2 demon Irat the absence of bacillary bodie within macrophag in the int linallamina propria muc ae. Higher·power ph lomi rograph obtained aft r Irealment Figure 20) demon trate the ab ence of bacillary bodie within macrophage in the intI'. tinallamina propria mucosae.
mal individuals (cut-off scores).7-9 These data indicate that A.H. is of average intelligence. The results of the first testing session revealed impairments in spatial sequencing (Trailmaking, Pan B), spatial scanning and concentration (Symbol Digit), psychomotor efficiency (Grooved Pegboard), learning (Digit Span + I), immediate memory (Digit Span), and shon-term memory (Benton Visual Retention Test). Some of these deficits were only mildly to moderately severe compared with absolute levels of performance; however, the impairments are striking in light of the patient's intellectual capacity. For example, A.H. 's IQ falls in the 60th percentile of the general population, while his visuospatial capacity, as measured by the Block Design Test, was in VOLUME 31 • NUMBER 2· SPRING 1990
only the sixth percentile, and immediate memory, as measured by Digit Span, was in the first percentile. Visual perception (Rey-Osterreith), perceptual speed (Stroop), and motor speed were not impaired. No verbal deficits were observed. The deficits observed at the time of diagnosis were only panially ameliorated following a 17month course of antibiotic treatment and full resolution of the intestinal infection. On three of the six tests in which A.H. scored in the neurologically impaired ranges in the pre-treatment period, he continued to perform in the deficit range after treatment. They included the Trailmaking Test, Pan B; the Digit Span; and the Benton Visual Retention Memory Test. In fact, visuopractic performance (Trail227
Case Repons
TABLE 2.
Neuropsychological test results of a patient with Whipple's disease before and after antibiotic treatment Before Treatment
After Treatment
37
42
13.5
<40
4 3
4 4
0 33.3
<5 <3
81 109
62 73
23.5 30.3
>80 >85
Finger tapping (taps per 10 sees.) dominant hand nondominant hand
44 46
52 50
18.2 8.7
<45 <40
Block design (best score. 48)
24
29
20.8
28
Stroop (sees.) words colors interference
10 15 35
10 29 29
0 20.0 17.1
>25 >33 >72
Test Symbol digit score Digit span (number of digits correct) forward backward Grooved pegboard (sees.) dominant hand nondominant hand
Percent Change
Cut-orr Score"
Tokens (number correCI)
II
10
-9.\
>9
Trails A (sees.)
21
31
-47.6
>39
Trails B (sees.)
96
99
-3.1
>91
Animal naming (number of responses)
26
30
15.4
<20
18 12.0 104
16 12.0 102
-10.8 0 -1.9
NA NA NA
3
4
25.0
<7
34
32
-5.9
<30
2
1
50.0
<3
Shipley Institute of Living Scale (best score. 20) vocabulary abstract (WAIS)IQ Bemon Visual Retention Test (best score. 10) Rey-Osterreith Figure (best score. 36) copy Digit span + I
h
"Cut-off scores indicate scores thaI suggest impaired functioning. ~ot applicable
making) declined from the first to the second testing session. In contrast. psychomotor efficiency (Grooved Pegboard) and visual scanning (Symbol Digit) were essentially normal at the second testing. The deficits that persisted following resolution of Whipple's disease were those involving visuopractic and memory processes. The DIS revealed no significant current or lifetime psychopathology.
Discussion
This case provided a unique opportunity to delineate the extent of cerebral dysfunction in the early 228
stages of Whipple's disease and to assess the efficacy of antibiotic therapy in reversing the impairments identified. Caution must be exercised in generalizing these findings to all patients with Whipple's disease. However, the observation of memory, visuospatial, and psychomotor deficits in a patient without overt signs or symptoms of neurologic abnormality implies some degree of cerebral pathology. This is consistent with earlier observations that histologic evidence of Whipple's disease can occur without clinical evidence of cerebral involvement. 10 This report, along with the report of Sierachi et aI., 10 suggests PSYCHOSOMATICS
Case Reports
that central nervous system involvement in Whipple's disease may be much more frequent than previously recognized and may occur early in the course of the disease. In addition, the finding of persistent memory and visuospatial deficits after antibiotic treatment suggests that the neuropathology of Whipple's may have long-tenn sequelae. Indeed, the mean change across all tests was only 10.2%, well within the acceptable range of test perfonnance variation. Thus, it can be concluded that no significant overall improvement occurred during the 17-month treatment period. Whether a more aggressive antibiotic regimen might reverse the persisting neuropsychological deficits is unknown; however, the possibility of pennanent cerebral dysfunction cannot be ruled out. The use of psychiatric and psychometric testing made it possible to identify dementia associated with Whipple's disease as well as to differentiate it from that of other dementing conditions. For example, unlike patients in the early stages of Alzheimer's disease, A.H. demonstrated no detectable behavioral or psychiatric disturbances. Nonetheless, on tests of language and memory, he perfonned similarly to demented indi viduals with other conditions, such as Alzheimer's disease. Finally, the present case study underscores the value of neuropsychologic testing for evaluating the cerebral dysfunction associated with metabolic or infectious diseases as well as for monitoring the progress of treatment. Inasmuch
as such testing is predictive, to a large degree, of social and vocational adjustment, II its use can enhance comprehensive medical management. Testing is especially valuable when compromised functional capacities may affect the safety of the patient or others. Thus, patients with serious impainnents in visuospatial or psychomotor functions may be cautioned to be aware of such limitations when driving or operating machinery. Although the present study clearly documents the presence of neuropsychologic deficits in a patient with Whipple's disease, the underlying mechanisms of the deficits remain unclear. Blood-borne transmission of the infectious agent responsible for Whipple's disease may explain its systemic effects. Other factors, such as malnutrition secondary to malabsorption, could also potentially have contributed to the deficits. However, biochemical measures of nutritional status, substantial weight gain, and resolution of the malabsorptive disorder discount the importance of malnutrition as the predominant etiological factor. Moreover, emotional distress secondary to a chronic illness could have contributed to the manifest neuropsychologic deficits, although in view of the absence of significant psychopathology revealed on the Diagnostic Interview Schedule, it is not likely that it was a major factor in this case. Nonetheless, it is most probable that the observed impainnents have a multifactorial etiology, which in addition to the direct eNS infection, includes psychological and other organically mediated disturbances.
References I. Maizel H. Raffin J. Dobbins W: Whipple's disease: a review of patients from one hospital and a review of the literalure since 1950. Medicine 49: 175-205. 1970 2. Lampen P. Tom M. Cummings J: Encephalopathy in Whipple's disease: a histochemical study. NeurolollY 12:65-71, 1962 3. Smith W. French J. Gottsman M. et al: Cerebral complication of Whipple's disease. Brain 88: 137-150. 1965 4. Lishman W: Orllanic Psychiatry. Oxford. Blackwell Scientific Publications. 1978 5. Badenoch J. Richard W. Oppenheimer D: Encephalopathy in a case of Whipple's disease. J Neurol Neurosur[? Psychiatry 26:203-210. 1963
VOLUME 31 • NUMBER 2· SPRING 1990
6. Robins L. Helzer J. Croughan J. et al: National Institute of Mental Health Diagnostic Interview Schedule. Arch Gen Psychiatry 38:381-389. 1981 7. Goldstein G. Taner R. Shelly C. et al: The Pittsburgh Initial Neuropsychological Testing System (PINTS): a neuropsychological screening battery for psychiatric patients. Journal of Behal'ioral Assessment 5:227-238. 1983 8. Lezak M: Neuropsycholollical Assessment (second ed). New York. Oxford University Press. 1983 9. Goldstein S. Deysach R. Kleinknecht R: Effect of experience and amount of information on identification of cerebral impairment. J Consult Clin Psychol41 :30-34.
229
Case Reports
1973 10. Sierachi J. Fine G. Hom R. et al: Central nervous system involvement in Whipple's disease. J Neuropalhol Exp Neuro/19:7{}-75.1960
M~~lNp
PSYCHOSOMATIC MEDICINE
T
he Academy of Psychosomatic Medicine stresses the ongoing education of its members-through clinical practice, study. and research-to advocate a psychosomatic approach that considers biologic. psychological and social factors in the prevention. diagnosis. and treatment of illness.
Membership .. entitles you to receive
II. Heaton R. Pendleton M: Use of neuropsychological tests to predict adult patients' everyday functioning. J Consull Clin Psyc/IO/ 49:1I07-821. 19111
professionals with special qualifications may be invited and considered for membership if approved by the executive council. Now in its 35th year, the Academy continues to work for the goal set forth by its founders:
The integration of medical treatment for illness-organic and psychologicwith the practice of medicine to ensure the best comprehensive care for all patients. For further information about membership in the Academy. clip and send the coupon below.
Psychosomatics throughout the year... provides opportunities to meet and exchange information while Executive Director earning Continuing ~lediC'JI Education credit at the Academy of Psychosomatic Medicine SH24 Magnolia. Chicago. II. 60660 prestigious annual meeting ...enables you to participate. after three years. in the distinguished fellowship Please send me fur/ber information regarding program. culminating in certifkation as a Fellow of the membership. Academy of Psychosomatic \Iedicine... is open to physicians and psychologists. as well as social workers Name and nurses with a doctoral degree who document Street active participation in clinical practice or research in City the psychosomatic approach to medicine. Associate membership is open to qualified health professionals State Zip with_ masters level In special other _ __ _ training. ___ _ _instances, ___ _--L
230
_ _ _
_
.J
PSYCHOSOMATICS