Neurocase (2001) Vol. 7, pp. 515–521

© Oxford University Press 2001

Gerstmann Syndrome in Systemic Lupus Erythematosus:
Neuropsychological, Neuroimaging and Spectroscopic
Findings
Rex E. Jung1,2, Ronald A. Yeo2, Wilmer L. Sibbitt Jr3,4, Corey C. Ford1,4, Blaine L. Hart5 and
William M. Brooks1,6
1Clinical and Magnetic Resonance Research Center, and Departments of 2Psychology, 3Internal Medicine, 4Neurology,
5Radiology and 6Neurosciences, University of New Mexico, Albuquerque, New Mexico, USA

Abstract
Gerstmann syndrome (GS) comprises four interlaced neuropsychological symptoms including finger agnosia, right–left
confusion, agraphia, and acalculia. While GS is commonly associated with focal lesions to the region of the left angular
gyrus, it has also been associated with numerous diffuse etiologies including atrophy, alcoholism, carbon monoxide
poisoning, lead intoxication and anaphylactic shock. Thus, a vigorous debate has emerged as to whether GS represents
a syndrome arising from general brain decline or a distinct and localizing lesion. We report a right-handed patient who
developed neuropsychological dysfunction secondary to systemic lupus erythematosus (SLE). Neuropsychological
evaluation found the patient to exhibit symptoms consistent with the GS tetrad, as well as general cognitive decline.
Magnetic resonance imaging revealed a distinct focal lesion of the left parieto-occipital white matter underlying the
angular gyrus as well as diffuse atrophy. 1H-magnetic resonance spectroscopy revealed substantial metabolic
derangement in a voxel placed within the visible lesion, although substantial metabolic derangement was observed in
regions remote from the focal pathology. Thus, GS in this first case in SLE would appear to comprise a focal
neurological tetrad of disorders within a more general pattern of cognitive decline and metabolic derangement.

Introduction
Josef Gerstmann’s name is eponymous for a syndrome of
four interlaced neuropsychological symptoms comprising:
inability to identify one’s own fingers (finger agnosia), right–
left disorientation, inability to write (agraphia), and inability
to perform mathematical operations (acalculia)(Gerstmann,
1940). This tetrad of the Gerstmann syndrome (GS), which
arose from lesions of ‘the angular and second occipital
convolution’ of the left hemisphere, highlighted a period of
increasing attention to localization of cognitive function
within centers of the brain. However, even in his initial
reports, Gerstmann acknowledged that other ‘signs’ of cognitive dysfunction were often associated with the ‘pure’ form
of the disorder. While the syndrome found wide acceptance
within the neurological community of the time, the advent
of modern neuroimaging techniques and lesion analysis has
challenged the sensitivity and specificity of this syndrome
to lesions of the inferior parietal region of the dominant
hemisphere.
Prior to Gerstmann’s case reports, a French ophthalmologist
named Jules Badal (1888) described a visually disoriented

patient secondary to eclampsia. This patient was unable to
dress or feed herself (dressing apraxia), unable to read or
write (alexia, agraphia), walked slowly and cautiously, could
not trace the letters of the alphabet (constructional apraxia),
was unable to distinguish left from right (right–left disorientation), and could not identify the fingers on her hands by
name or number (finger ‘agnosia’). While GS was commonly
associated with focal lesions to the left angular gyrus, it has
been, even in this earliest case, associated with numerous
diffuse etiologies including neurodegenerative disorders,
alcoholism, carbon monoxide poisoning, lead intoxication
and anaphylactic shock (Critchley, 1966). Thus, a vigorous
debate has emerged as to whether GS represents a syndrome
arising from general brain decline or a distinct and localizing
focal lesion.
That GS may occur in a variety of syndromes and in the
presence of myriad lesion sites was not lost on Benton
(1961), who designed an elegant test of the localizing efficacy
of this disorder. Perhaps most striking was the observation
that GS often occurred as a syndrome in the presence of

Correspondence to: Rex E. Jung, Clinical and Magnetic Resonance Research Center, 1201 Yale NE, Albuquerque, NM 87131, USA. Tel: ⫹1 505 897 2269;
e-mail: rexjung@yahoo.com

516 R. E. Jung et al.

numerous other neurological deficits. These deficits include,
but are not limited to: aphasia, constructional apraxia, alexia,
visuoperceptual defects, and general cognitive decline
(Benton, 1961). Neuropathologically, patients with GS had
large lesions of the left posterior hemisphere that were of a
‘progressive or recurrent nature’ (Benton, 1977). The most
compelling conclusion to these studies was that GS does not
occur in isolation from more general linguistic and cognitive
decline. While most of the studies that have contributed to
the literature on GS are behavioral and radiological in nature,
the discussion has not adequately addressed the underlying
metabolism and integrity of brain tissue both at the site of
lesion and removed from the site of focal injury.
Magnetic resonance spectroscopy (MRS) is a powerful
non-invasive method for studying cerebral metabolism and
neuronal integrity in vivo. Proton MRS (1H-MRS) detects
signals from metabolites such as N-acetylaspartate (NAA),
creatine (Cre), choline-containing compounds (Cho), and
lactate (Lac) (Ross and Michaelis, 1994). Changes in neurometabolites measured by 1H-MRS have been found in a wide
range of neurocognitively impaired patients. For example,
reduced NAA has been noted in many diseases and is
associated with neuronal injury or metabolic depression
(Brooks et al., 2000). The Cre peak represents the sum of
intracellular creatine and phosphocreatine, both of which
have important roles in tissue energetics. The Cho peak
reflects the sum of all 1H-MRS visible choline moieties, and
is elevated in stroke and multiple sclerosis due to membrane
breakdown, inflammation and demyelination (Ross and
Michaelis, 1994). It has been demonstrated that neuronal
metabolites, including NAA and choline, are strongly correlated with composite measures of neuropsychological performance in a cohort of patients diagnosed with neuropsychiatric
systemic lupus erythematosus (NPSLE) (Brooks et al., 1999).
Moreover, the sensitivity of metabolites measured by
1H-MRS to behavioral measures was highlighted by recent
findings that broad intellectual and neuropsychological functioning was correlated with NAA and choline in normal
human brain (Jung et al., 1999a, b).
NPSLE is characterized by complex physical and
behavioral manifestations encompassing a broad spectrum of
neuropsychological symptoms (Sibbitt et al., 1999a). Mild
central nervous system (CNS) complaints, life-threatening
neurological involvement, stroke, and chronic disability contribute to the diagnostic and treatment complexity of this
disease (Carbotte et al., 1995). Neuropsychological function
is often abnormal in NPSLE, and can reflect impairment of
intellect, attention, memory, language, visuospatial, sensory
motor, and problem-solving abilities (Skeel et al., 2000).
Although a wide range of neurodiagnostic tools are used to
evaluate CNS complaints in NPSLE, no single technique has
proved to be a definitive, diagnostic, or reliable measure
(Sibbitt et al., 1999b). However, 1H-MRS has contributed
significantly to the understanding of neuronal metabolism
critical to disease expression, course, and prognosis (Sibbitt
et al., 1997).

Case report
JA was a 35-year-old woman who complained of severe
headaches, weakness, difficulty performing simple intellectual tasks, and disability. She had been disabled for 5 years
prior to the diagnosis of SLE and brain disease, and was
unemployable and living with her mother when the cognitive
disorder was recognized. Her medical history included nine
first trimester miscarriages, deep venous thrombosis, joint
pain, thrombocytopenia, severe depression, and mouth ulcers.
The patient had been told in the past that she had ‘lupus’, at
which time she was treated with high-dose corticosteroids.
The diagnosis of SLE was confirmed using the American
Rheumatism Association 1982 and American College of
Rheumatology 1997 revised criteria for SLE (Tan et al., 1982;
Hochberg, 1997). The presence of NPSLE was confirmed
and classified using the 1999 Case Definitions for NPSLE
Syndromes (American College of Rheumatology, 1999).
Medication at the time of the evaluation included oxycodone/acetaminophen for severe headaches. A physical
examination demonstrated extensive livedo reticularis over
the extremities, a depressed affect, hyperreflexia, bilaterally
upgoing toes (Babinski’s sign), and impaired cognition.
Antinuclear antibody was 1:320 speckled and diffuse, anti-dsDNA antibody was negative, anti-Sm antibody was negative,
urinalysis was normal, platelet count was 90 ⫻ 103/cm3,
C3 13 (normal range 24–44), C4 63 (normal range 74–120),
IgG (in standardized international units: MPL/GPL units)
(36 GPL) and anti-phospholipid antibodies IgM (39 MPL)
were strongly positive. A lumbar puncture was performed,
but demonstrated no cells, normal glucose, no oligoclonal
bands, negative myelin basic protein, normal IgG index,
normal protein, and normal albumin quotient.
The patient was positive for anti-phospholipid antibody
IgM (MPL), and in the presence of a thrombotic event
(stroke), indicated vascular injury secondary to anti-phospholipid antibody syndrome (APAS) in this case SLE–APAS
(Golstein et al., 1993; Gorman and Cummings, 1993). However, it should be recognized that the presence of antiphospholipid antibodies is now one of the criteria for the
diagnosis of SLE; thus, this syndrome should be construed
to be a complication of NPSLE, not primary APAS (Hochberg,
1997; American College of Rheumatology, 1999). As in this
case, stroke syndromes affect 10–15% of SLE subjects and
remain a devastating complication (West, 1994).
Neurological examination found the patient to be alert,
oriented, and able to follow simple commands. She had some
difficulty with left–right confusion. For example, when asked
to touch her left ear with her right thumb she was hesitant
and made errors with the choice of arm, digit and ear. She
had difficulties with calculations and was unable to perform
serial 7s or serial 3s or to make simple change. No abnormalities were noted in an evaluation of the cranial nerves. Extraocular movements were intact. No sensory abnormalities
were noted on the face. There was no weakness in the facial
muscles and her hearing acuity was normal bilaterally. Her

Gerstmann syndrome in SLE 517

palate elevation was midline and there was no dysarthria.
Shoulder shrug was normal, as was tongue function. Muscle
power was normal in all muscle groups of both the upper
and lower extremities. There was normal bulk and tone in
the arms and legs. There was a slight decrease in vibration
thresholds in the feet and occasional proprioceptive errors
were made identifying passive toe movements. Her reflexes
were 3⫹ and symmetric in the upper extremities and at the
knees. There was ankle clonus on the right and a 3⫹ reflex
at the left ankle with bilateral extensor plantar responses. No
ataxia was noted on finger-to-nose or heel-to-shin testing and
she performed rapid alternating movements normally. Her
gait was normally based and stable. However, she demonstrated ataxia on tandem gait and was unable to take more
than two steps without stepping to the side. She had a
negative Romberg.
The patient was fully anticoagulated with warfarin, but
refused treatment with prednisone. In spite of adequate
anticoagulation (international normalized ratio ⫽ 3.5), the
patient continued to have severe headaches and became
increasingly disabled. As serum complements remained low,
it was felt that the increasing neurological deterioration was
due to CNS vasculopathy secondary to SLE disease activity,
rather than to further antiphospholipid antibody-mediated
cerebrovascular thromboses. The patient was placed on
monthly intravenous cyclophosphamide (1.0 g/m2) and within
4 weeks noticed markedly fewer headaches and arthralgias.
By 8 weeks of therapy, serum complements had risen, and
the patient felt that her neurological functioning had markedly
improved. She made plans to move to her own apartment. A
physical examination at that time was unchanged, and
although the patient’s depression and pain were much less,
cognitive abnormalities remained.

Neuropsychological examination
JA was alert and oriented to person, place and situation. She
correctly identified the day and date, but stated that the year
was 1996 (actually 1998) and that the time was 2.30 p.m.
(actually 1.37 p.m.). JA complained of visual difficulty, but
was unable to specify the extent and effect her vision had
on her abilities. She wore contact lenses during the evaluation,
and quickly attributed her visual difficulties to the lack of a
current lens prescription. Informally assessed, she did not
exhibit symptoms characteristic of simultaneous agnosia,
visual neglect, or Balint–Holmes syndrome. Subjectively, her
speech was normally articulated, fluent, and demonstrated
correct prosody. She appeared to be somewhat labile, crying
one moment when recounting a family story, and laughing
minutes later. Depressive affect was assessed with the Beck
Depression Inventory, and she endorsed very few vegetative
or cognitive symptoms (total score ⫽ 6).
During the course of the neuropsychological assessment,
the patient complained of difficulty discriminating left from
right, trouble keeping financial records, and difficulty with
vision, particularly reading and writing. Formal screening for

GS was added to the neuropsychological assessment, and
was found to be defective in all criteria measured (Table 1).
The positive GS findings led to speculation that JA had a
major lesion within the left parietal region, a hypothesis that
was confirmed upon subsequent magnetic resonance imaging
(MRI) examination.
A broad neuropsychological examination was administered, utilizing standard methods, that included the following
tests: Wechsler Adult Intelligence Scale-III, Wide Range
Achievement Test-III reading and spelling subtests, California
Verbal Learning Test, Warrington Recognition Memory Test,
Boston Naming Test, Controlled Oral Word Association,
Sentence Repetition Test, Stroop Test, Reitan–Klove Sensory
Perceptual Examination, Grip Strength, Finger Tapping, Ninehole Peg Test, Right–Left Orientation, Trail Making Test A
and B, Positive Affect Negative Affect Scale and Beck
Depression Inventory. JA’s scores were compared with normal
age-matched controls commonly utilized in both research
and clinical settings (Spreen and Strauss, 1998). Normative
data on the Halstead–Reitan test and the California Verbal
Learning Test were generated via computer scoring. All
scores were converted to z-scores (individual score – mean/
standard deviation) for the purpose of comparison within a
standard metric. z-scores have a mean of 0, and a standard
deviation of 1, with scores falling below –1.3 considered to
be within the impaired range of functioning (Lezak, 1995).
Pre-morbid intellectual ability was estimated with the
Barona et al. (1984) equation, which suggested average
pre-morbid cognitive functioning (estimated full-scale
IQ ⫽ 100.7). Of note is the substantial decline in current
intellectual functioning from estimates of pre-morbid functioning (full-scale IQ ⫽ 65). It should also be noted that,
when the effects of working memory are removed from
intellectual functioning [as is the case with the Verbal
Comprehension Index (VCI)], JA’s intellectual ability falls
within the average range of functioning (VCI ⫽ 91). Finally,
very poor performance was evident on most measures of
non-verbal reasoning, relying heavily upon accurate visual
and spatial integration. Table 2 compares JA’s performance
on intellectual measures with that of a recent case of pure
GS with a subangular lesion (Mayer et al., 1999). Striking
similarities were observed between these individual cases on
measures of intellectual functioning, namely poor performance on measures of oral arithmetic, block design, and digit
symbol, probably representing the intellectual correlates of
GS (Fig. 1).
Verbal comprehension was adequate according to responses
to complex ideational material (total score ⫽ 11/12). Similarly, sentence repetition was within normal limits (total
score ⫽ 15). Performance on measures of right–left discrimination was similar to the GS patient described by Strub and
Geschwind (1974), in that right–left orientation was largely
intact for her own body and that of the examiner (seven of
eight correct), although she was unable to identify correctly
the right hand of the examiner. JA, however, did not possess
the ability to use orientation systems concurrently. For

518 R. E. Jung et al.
Table 1. Gerstmann symptoms for JA
Gerstmann domain

Test

Raw score

z-scorea

Acalculia
Agraphia
Finger agnosia

WAIS-III arithmetic
WRAT-III spelling
Reitan–Klove: tactile finger recognition right hand (errors)
Reitan–Klove: tactile finger recognition left hand (errors)
Benton right–left orientation

6
16
13
15
10

–2.40
–4.50
⬎–5.00
⬎–5.00
–4.04

Right–left confusion

az-scores have a mean value of 0 and a standard deviation of 1.

WAIS-III, Wechsler Adult Intelligence Scale; WRAT-III, Wide Range Achievement Test.

Table 2. Intellectual profile of HP and JA

Fig. 1. Scatterplot comparing JA’s intellectual subtest performance with that
of a recent case of pure Gerstmann syndrome with a subangular lesion (HP).

example, when asked to indicate the correct directional eye,
ear, or knee with her right or left hand, she performed at
chance levels (three of eight correct). Most striking was her
inability to use her right or left hand to indicate the directional
side of the examiner’s eye, ear or shoulder (none of four).
The Reitan–Klove Sensory Perceptual Examination was
notable for the lack of basic tactile, auditory, or visual sensory
deficiency (one left-hand suppression error in total), except
for the finding of finger agnosia described below. For example,
in the tactile examination, each hand is first touched separately
in order to determine that the subject is able to respond
with accuracy to the hand touched, followed by unilateral
stimulation interspersed with bilateral simultaneous stimulation. As a basic attentional component is required to perform
these sensory tasks, JA’s performance would tend to indicate
a lack of primary attentional or sensory deficit to explain the
dysfunction on other measures. Of more general interest was
the expected bilateral dysfunction on finger ‘gnosis’, as
well as unilateral right ⬎ left impairment on graphesthesia
(13 errors for the right hand, one error for the left hand).
The former task requires the subject to identify individual
fingers on each hand following tactile stimulation, while the
latter task requires the patient to report numbers written on
the fingertips of each hand, both without the use of visual
cues (Reitan and Davison, 1974).
JA’s performance on writing tasks is best characterized as
‘apraxic’ agraphia, in which letter formation and poor line
drawing is the central element of dysfunction. This pattern
was continued on tasks such as the Benton Visual Retention
Test, and clock drawing, both of which had to be discontinued

WAIS-III

HP

JA

Impaired

Verbal IQ
Information
Comprehension
Vocabulary
Similarities
Digit span
Arithmetic

97
14
14
13
12
3
3

79
9
6
8
8
5
3

–
–
–
–
–
⫹
⫹

Performance IQ
Picture completion
Picture arrangement
Block design
Digit symbol

79
8
7
5
4

54
2
4
2
1

⫹
⫹
⫹
⫹
⫹

WAIS-III, Wechsler Adult Intelligence Scale.

due to poor line formation. Interestingly, while generally able
to copy letters to dictation, JA indicated that she could not
‘think how to form a K’ in both single letter and word
spelling formats (i.e. ‘make’). The neuropsychological test
results are presented in Table 3.

MRI and 1H-MRS examination
The patient came to our center as part of a research cohort
of SLE patients undergoing 1H-MRS and neuropsychological assessment to determine the relationship of
metabolic changes to functional impairment. All magnetic
resonance acquisitions were carried out on a 1.5 Tesla
clinical magnetic resonance scanner using standard software (GE Medical Systems, Waukesha, WI, USA). Sagittal
T1-weighted images, axial T1-weighted three-dimensional
fast spoiled gradient-recalled acquisition sequence (FSPGR)
images and axial intermediate and T2-weighted images
from the midbrain through the vertex were obtained without
contrast. The ventricular system was normal in position
and somewhat prominent in size. Diffuse volume loss and
multiple focal areas of cortical volume loss and underlying
white matter gliosis were present. Volume loss and gliosis
were interpreted as probably resulting from multiple areas
of infarction. Representative axial T1, T2 and proton density
images are presented in Fig. 2. The diffuse atrophy is
readily observed, with the most prominent brain tissue
loss in the left posterior parietal and occipital regions.

Gerstmann syndrome in SLE 519

Fig. 2. Representative axial T1, T2 and proton density images from magnetic resonance imaging showing diffuse atrophy and focal lesion to the left posterior
brain region.

Table 3. Neuropsychological performance of JA
Test

JA raw z-scorea Impairedb

WRAT-III—reading
39
CVLT—total
42
CVLT—delay
8
Warrington Recognition Memory Test—words 44
Warrington Recognition Memory Test—faces 32
Sentence Repetition Test
15
Boston Naming Test
49
Verbal Fluency Test
16
Finger tapping—dominant
21.7
Finger tapping—non-dominant
30
Grip strength—dominant
22.3
Grip strength—non-dominant
22.7
Stroop Word Reading
30
Stroop Color Naming
14
Stroop Interference
2
Beck Depression Inventory
6
PANAS—positive affect scale
35
PANAS—negative affect scale
12

–1.4
–2.8
–3.0
–1.0
–3.0
–0.6
–2.7
–5.3
–2.9
–1.7
–1.2
–0.8
⬎–3.0
⬎–3.0
⬎–3.0
0.6
0.6
0.5

⫹
⫹
⫹
–
⫹
–
⫹
⫹
⫹
⫹
–
–
⫹
⫹
⫹
–
–
–

az-scores have a mean value of 0 and a standard deviation of 1.
bImpaired performance based upon a z-score less than –1.3.

WRAT-III, Wide Range Achievement Test; CVLT, California Verbal
Learning Test; PANAS, Positive Affect Negative Affect Scale.

The location and appearance of this lesion suggest the
occlusion of a discrete medium-sized muscular cerebral artery,
indicating a classic stroke syndrome of NPSLE (American
College of Rheumatology, 1999). The stroke episode was
distant, as indicated by the involuted appearance of the
affected area on MRI. However, this subject had no identifiable clinical stroke episode; rather her disability appeared to
evolve slowly over a period of time. This presentation is
typical of multi-infarct processes, rather than a catastrophic
stroke syndrome, which is usually easily recognizable by the
clinician. Indeed, there were other smaller lesions obvious
by MRI, indicating a gradual accumulation of individual

lesions. Moreover, the patient lost cognitive rather than motor
function with this lesion, and although most physicians can
recognize gross loss of motor function, many have difficulty
recognizing cognitive disorders without a high level of
vigilance.
Spectroscopic voxel locations were selected from localizing imaging sequences—a T1-weighted FSPGR scan
(TE ⫽ 6.9 ms, TR ⫽ 17.7 ms, flip angle ⫽ 25 degrees,
3 mm slices) and a conventional T2-weighted series
(TE ⫽ 30/100 ms, TR ⫽ 2800 ms, 4 mm slices). A stimulatedecho acquisition mode (STEAM) pulse sequence, including
water suppression, was employed to sample four voxel
locations (TE ⫽ 30 ms, TR ⫽ 2000 ms, 128 averages) within
bilateral occipitoparietal white matter, and bilateral frontal
white matter. The voxels were positioned specifically to
maximize white matter and minimize gray matter and
ventricular contribution (Fig. 3). Following water filtering,
time-domain fitting of Gaussian lineshapes was carried out
by VARPRO, and the areas corresponding to NAA and Cho
were recorded. The data were quantified by employing the
internal water signal as a concentration reference standard
and correcting for metabolite and water T1 and T2 effects
during echo and recycle times using literature values.
Spectroscopic evaluation revealed global metabolic decline
within both white and gray matter tissue in this patient.
Measures of NAA within left occipitoparietal white matter
were reduced substantially (–8.5 standard deviations) from
normal control values (12.92 ⫾ 0.60) obtained in a cohort
of healthy neurologically normal adults reported previously
(Jung et al., 2000). Similarly, Cho was substantially elevated
within the same voxel location (–3.6 standard deviations)
compared with control values (1.75 ⫾ 0.25) obtained within
a similar cohort (Jung et al., 1999b). Of interest, while the
left occipitoparietal voxel revealed the greatest degree of
metabolic derangement, similar reductions in NAA and

520 R. E. Jung et al.

Fig. 3. Spectroscopic regions of interest and metabolic values for
N-acetylaspartate (upper value) and choline (lower value) within three voxel
locations.

elevations in Cho were apparent throughout the brain of this
individual (Fig. 3).

Discussion
This case represents the first description of Gerstmann’s
tetrad in a patient diagnosed with SLE. While this patient
exhibited numerous areas of neuropsychological dysfunction,
it is clear that various cognitive domains were differentially
affected by the disease course. For example, while the
intellectual profile of JA was quite reduced, and indicative
of global cognitive decline, it did correlate highly with the
intellectual performance of a case of pure GS reported in the
literature (Mayer et al., 1999). Indeed, JA’s verbal intellectual
abilities are probably close to pre-morbid levels in the low
average range. Other aspects of intellectual ability, including
digit–symbol substitution, arithmetic reasoning, and block
construction, were all profoundly affected in both JA and
HP, demonstrating the possibility, although preliminary, of a
common intellectual consequence of GS.
While GS has often been described as a focal neurological
disorder, analysis of the performance of our patient would
suggest the confounding influences of global cerebral pathology. Indeed, JA exhibited the tetrad of GS symptomatology
within the context of a general cognitive decline as well as
global neuropsychological dysfunction sparing only gross
motor and some language processes (e.g. word recognition,
sentence repetition). MRI data revealed both focal pathology
within the left parieto-occipital hemisphere and general
atrophic processes affecting the entire parenchyma. Spectro-

scopic data revealed general metabolic derangement throughout the brain with a lack of specificity to the white matter
underlying the left angular gyrus. Thus, GS appears to be a
watershed disorder that tends to localize well to the dominant
parietal lobe and underlying white matter, but that does not
reflect a single underlying deficit (i.e. Grundstörung) of other
focal neurological disorders, including various syndromes of
aphasia, amnesia and alexia. However, GS symptoms in
neurological patients are ‘clinically meaningful’ in that they
point to a probable lesion within the dominant posterior
parietal hemisphere (Benton, 1992).
The interest in the cognitive models that underlie GS is
well placed, as GS appears to involve some aspects of
body schema, visuospatial orientation, and the developmental
processes inherent within arithmetic ability that often begins
with counting on one’s fingers (Benton and Sivan, 1993).
The specificity of this disorder, however, is fading as the
sensitivity of various imaging modalities exceeds the sensitivity of GS in diagnosing brain–behavior dysfunction. JA
exhibits the ‘classic’ symptoms that comprise the GS syndrome, as well as converging evidence of the characteristic
GS lesion. However, it is difficult to determine whether this
lesion also plays a role in the broad neuropsychological
deficit evident in this patient, or whether other brain events,
including global cerebral atrophy and concurrent metabolic
derangement, can better account for the general neuropsychological dysfunction evident in JA. Future research will be
valuable to disentangle the cognitive underpinnings of GS
that play a role in higher-level neuropsychological domains
including expressive language (both oral and written), visuospatial orientation, and abstract use of numerical concepts.

Acknowledgements
This research was supported by The Mental Illness and
Neuroscience Discovery (MIND) Institute and grant RO1
NS 35708 from the National Institutes of Health.

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Received on 11 April, 2001; resubmitted on 14 August, 2001; accepted
on 16 August, 2001

Gerstmann syndrome in systemic lupus
erythematosus: neuropsychological,
neuroimaging and spectroscopic findings
R. E. Jung, R. A. Yeo, W. L. Sibbitt Jr,
C. C. Ford, B. L. Hart and W. M. Brooks
Abstract
Gerstmann syndrome (GS) comprises four interlaced neuropsychological
symptoms including finger agnosia, right–left confusion, agraphia, and
acalculia. While GS is commonly associated with focal lesions to the region
of the left angular gyrus, it has also been associated with numerous diffuse
etiologies including atrophy, alcoholism, carbon monoxide poisoning, lead
intoxication and anaphylactic shock. Thus, a vigorous debate has emerged as
to whether GS represents a syndrome arising from general brain decline or a
distinct and localizing lesion. We report a right-handed patient who developed
neuropsychological dysfunction secondary to systemic lupus erythematosus
(SLE). Neuropsychological evaluation found the patient to exhibit symptoms
consistent with the GS tetrad, as well as general cognitive decline. Magnetic
resonance imaging revealed a distinct focal lesion of the left parieto-occipital
white matter underlying the angular gyrus as well as diffuse atrophy. 1Hmagnetic resonance spectroscopy revealed substantial metabolic derangement
in a voxel placed within the visible lesion, although substantial metabolic
derangement was observed in regions remote from the focal pathology. Thus,
GS in this first case in SLE would appear to comprise a focal neurological
tetrad of disorders within a more general pattern of cognitive decline and
metabolic derangement.

Journal
Neurocase 2001; 7: 515–21

Neurocase Reference Number:
O241

Primary diagnosis of interest
Gerstmann syndrome

Author’s designation of case
JA

Key theoretical issue
d Gerstmann syndrome in systemic lupus erythematosus, comprising a focal

neurological tetrad of disorders within a more general pattern of cognitive
decline and metabolic derangement
Key words: Gerstmann syndrome; systemic lupus erythematosus; spectroscopy;
nuclear magnetic resonance; neurochemistry; neuropsychological

Scan, EEG and related measures
MRI, 1H-MR spectroscopy

Standardized assessment
Wechsler Adult Intelligence Scale-3, Wide Range Achievement Test-3,
Naming Test, Controlled Oral Word Association, Sentence Recognition Test,
Sentence Repetition Test, Stroop Test, Reitan–Klove Sensory Perceptual
Examination, grip strength, finger tapping, 9-hole peg test, Benton right–left
orientation test, Trail Making test A & B, positive affect negative affect scale,
Beck depression inventory

Other assessment
Neurological, rheumatological

Lesion location
d Left posterior parietal region

Lesion type
Infarct

Language
English