J Neurol Neurosurg Psychiatry 2000;68:246–256

248

Correspondence to: Dr Marek Czosnyka, Academic Neurosurgical Unit, Box 167, Addenbrooke’s
Hospital, Cambridge CB2 2QQ, UK email
MC141@MEDSCHL.CAM.AC.UK
1 Marmarou A, Foda MA, Bandoh K, et al. Posttraumatic ventriculomegaly: hydrocephalus or
atrophy? A new approach for diagnosis using
CSF dynamics. J Neurosurg 1996;85:1026–35.
2 Shapiro K, Fried A, Takei F, et al. EVect of the
skull and dura on neural axis pressure-volume
relationships and CSF hydrodynamics. J Neurosurg 1985;63:76–81.
3 Czosnyka M, Whitehouse H, Smielewski P, et al.
Testing of cerebrospinal compensatory reserve
in shunted and non-shunted patients: a guide
to interpretation based on observational study.
J Neurol Neurosurg Psychiatry 1996;60:549–58.
4 Hochwald GM, Epstein F, Malhan CrashoV J.
The role of skull and dura in experimental
feline hydrocephalus. Dev Med Child Neurol
1972;14(suppl 27):65–9.

Diencephalic amnesia and apraxia after
left thalamic infarction
Amnesia and apraxia are unusual manifestations of unilateral thalamic lesions. A patient
in whom severe amnesia and apraxia were the
presenting features of a left thalamic infarct is
presented. The findings support the concept
that memory and praxis both utilise circuits
which include the dominant thalamus.
A 78 year old right handed Hungarian
woman presented with memory loss and
disorientation. She had been well and conversed normally with her daughter on the
evening before presentation. The next morning, her daughter was alarmed to find her
mother’s house in a state of disarray. Dishes
were unwashed, lights left on, and doors
open. The patient seemed baZed by eating
utensils, attempting to scoop food with her
knife. Later that morning, she failed to recognise longstanding Hungarian friends. She was
unable to recall her address, the name of the
city in which she lived, or the names of her
grandchildren. She subsequently failed to
recognise her family doctor of 7 years.
History included non-insulin dependent
diabetes, hypertension, hyperlipidaemia, and
atrial fibrillation. Medications were digoxin,

glibenclamide, and metoprolol. Captopril
had been prescribed 4 weeks previously but
was ceased 2 days before presentation due to
presyncopal symptoms. The patient consumed no alcohol. There was no history of
cerebrovascular events.
Cognitive functions were examined at the
bedside with the assistance of an interpreter, as
the patient spoke no English, although she
conversed freely in her native Hungarian. She
had no recollection of events since emigrating
to Australia 50 years previously, gave her
correct maiden name, and could not recognise
or name her grandchildren, although she
recognised her daughter. She acknowledged
she was in a hospital, but maintained it was in
Budapest and the year was 1947. Although her
recollections regarding her early life and
wartime Hungary seemed accurate, she confabulated when asked for details of recent
events. Short term recall of verbal material and
people was poor. The patient was able to name
objects such as a pencil and a watch, and obey
two and three stage commands. She wrote her
name and copied simple designs correctly, and
could imitate gestures such as waving goodbye
or blowing a kiss. However, she was unable to
use eating utensils or a toothbrush, either in
pantomime or when provided with the object
itself. Movements of the face and limbs were
normal, and there were no sensory abnormalities. Knee and ankle jerks were absent bilaterally and both plantar responses were extensor.
General examination revealed atrial fibrillation
and mild cardiomegaly.
The patient continued to display severe
impairment of anterograde memory. She was
reluctant to leave her bed, and quickly
became lost unless supervised. She did not
recognise familiar staV members and was
unable to use ward landmarks to reorientate
herself. She required assistance to feed
herself, brush her teeth, and shower. When
reviewed 3 months later, her memory disturbance and apraxia for simple activities of
daily life (such as brushing her teeth)
persisted, necessitating care in a supervised
environment.

Brain MRI (figure) showed a left anterior
thalamic lesion consistent with lacunar infarction and periventricular white matter disease.
Deficits of anterograde and retrograde
memory after thalamic lesions are well
recognised.1 The syndrome of diencephalic
amnesia after bilateral medial thalamic lesions typically involves striking disorientation
for time, loss of autobiographical information
(often extending back for many years),
confabulation, and severe anterograde amnesia for verbal and visual material, including
recognition of familiar faces.1 These features
were well illustrated by our patient, who
became “marooned” in an earlier place and
time. Amnesia after unilateral thalamic lesions is rare.2 There is increasing evidence
that thalamic lesions interrupt the multiple
brain networks which form the anatomical
substrate of memory,1–4 encompassing the
hippocampus, medial thalamus, frontal and
cingulate cortices, and overlapping with the
language areas of the left hemisphere.3 The
thalamus is activated in retrieval of episodic
(autobiographical) and semantic (encyclopaedic) information from long term storage4
and execution of learned motor tasks,5 which
may reflect its widespread connections with
other subcortical and cortical structures.
The patient’s ability to name or identify
objects was not tested systematically. However,
on the evidence available, it seems likely that her
diYculty in utilising common objects was a
manifestation of apraxia for daily tasks rather
than, for example, agnosia for the objects
involved. Apraxia is a rare manifestation of isolated thalamic lesions.5 The ability to access
stored motor representations is thought to be
crucial for normal execution of learned actions.5
These motor representations or “engrams” are
analogous to motor memories. Although praxis
is generally regarded as a function of distributed
cortical regions in the left hemisphere, apraxia
in association with thalamic amnesia has not
been emphasised in previous reviews of this
syndrome.1–2 Involvement of deep hemispheric
white matter in association with basal ganglia
pathology is thought to be critical for the
development of apraxia after lesions of subcortical structures.5 The conjunction of diencephalic amnesia and apraxia after thalamic
infarction in the present case may be interpreted as further evidence that retrievial of
episodic, semantic, and motor memories is
mediated by overlapping functional networks
in the dominant hemisphere.1 3 4
JD WARREN
PD THOMPSON
Department of Neurology, Royal Adelaide Hospital,
Adelaide, South Australia
PD THOMPSON
University Department of Medicine
Correspondence to: Professor PD Thompson, University Department of Medicine, Royal Adelaide
Hospital, Adelaide, South Australia 5000, Australia

T2 weighted axial MRI brain slice showing ischaemic infarction of the left anterior thalamus and
periventricular white matter ischaemic changes.

1 Hodges JR, McCarthy RA. Autobiographical
amnesia resulting from bilateral paramedian
thalamic infarction: a case study in cognitive
neurobiology. Brain 1993;116:921–40.
2 Clarke S, Assal E, Bogousslavsky J, et al. Pure
amnesia after unilateral left polar thalamic
infarct: topographic and sequential neuropsychological metabolic (PET) correlations. J
Neurol Neurosurg Psychiatry 1994;57:27–34.
3 Perani D, Bressi S, Cappa SF, et al. Evidence of
multiple memory systems in the human brain:
a [18F]FDG PET metabolic study. Brain 1990;
116:903–19.
4 Fletcher PC, Frith CD, Grasby PM, et al. Brain
systems for encoding and retrieval of auditoryverbal memory: an in vivo study in humans.
Brain 1995;118:401–16.
5 Pramstaller PP, Marsden CD. The basal ganglia
and apraxia. Brain 1996;119:319–40.

J Neurol Neurosurg Psychiatry 2000;68:246–256
Transverse myelopathy in the antiphospholipid antibody syndrome: pinworm
infestation as a trigger?
The antiphospholipid antibody syndrome is a
disorder characterised by the production of
autoantibodies directed against negatively
charged cell membrane phosholipids. Antiphospholipid antibodies have been described in various neurological disorders.
It has been generally accepted that viral,
bacterial, and parasitic infections can serve as
a trigger factor for autoimmune reactions.
Despite the growing knowledge that has
accumulated, the relation between parasites
and autoimmunity has not been clarified.
Enterobius vermicularis (pinworm) is a
nematode rarely found outside the gastrointestinal tract, but allergic reaction due to
enterobiasis has been reported.1 We describe
the case of transverse myelopathy preceded by
intestinal pinworm infestation in the primary
antiphospholipid antibody syndrome. To our
knowledge, such an association has not been
reported previously. Pinworm therapy was
complicated by the Jarisch-Herxheimer reaction manifested by temporary exacerbation of
pre-existing neurological symptoms.
In March 1998, a 40 year old woman who
complained of perianal itching noticed the
presence of worms migrating from the anus.
Three days later itching and numbness
involved both legs, and the patient had weakness in the legs. These symptoms progressed
over the next 3 days to severe paraparesis and
urinary urgence. Her medical history was relevant for three unexplained miscarriages
which all occurred in midpregnancies. In the
local hospital, she underwent brain and
lumbar-sacral spine MRI and findings were
normal. Cerebrospinal fluid examination disclosed slightly raised proteins of 670 mg/l, 2
lymphocytes/mm3;
oligoclonal
immunoglobulin (Ig) G bands were absent. She was
treated with methylprednisolone (1 g/day)
intravenously for 5 days with subsequent
gradual tapering oV, which was associated
with substantial improvement of motor,
sensory, and sphincter disturbances. At the
end of April 1998, the patient was transferred
to our hospital for further investigation.
Neurological examination showed mild
spastic paraparesis, bilateral Babinski’s sign,
and a Th-12 sensory level.
Erythrocyte sedimentation rate was 34.
Complete blood count, tests for hepatic and
renal function, angiotensin converting enzyme, concentrations of IgG, IgM, IgA, IgE,
and immune complexes, screening for antinuclear (HEp-2 cells), anti-ds DNA, antineutrophil cytoplasmic, antimitochondrial, and
antiparietal cell antibodies, rheumatoid factor, and the search for antineurotropic virus
and antiBorrelia antibodies were normal or
negative. A venereal disease research laboratory flocculation test was negative. A medium
positive concentration of IgM anticardiolipin
antibody was detected, and lupus anticoagulant was negative. Raised titres of serum IgG
and IgM anti-GM1 (1:1600 and 1:3200,
respectively) and antisulfatide antibodies
(1:6400, for both classes) were also demonstrated. Class II human leucocyte antigen
(HLA) typing showed the presence of HLADR3, DR4, DR52, DR53, DQ2, and DQ3.
Cervical and thoracic spine MRI was normal.
Electromyoneurography was normal.
Because the patient complained of reappearence of worms and perianal itching, a
cellulose adhesive tape test was performed
and diagnosis of enterobiasis was established.

249

Mebendazole was given in a single dose of
100 mg and the next day the JarishHerxheimer reaction occurred, with deterioration of leg spasticity, inability to walk, and
development of urinary retention. At that
time, low positive IgG ACA was detected.
The dose of prednisone was raised to 60
mg/day and slowly tapered oV within the next
2 months. Sphincter disturbances resolved in
1 day and motor dysfunction gradually
improved with only mild spasticity left.
Diagnosis of antiphospholipid antibody
syndrome in our patient was based on the
presence of reccurrent fetal loss, transverse
myelopathy, and raised ACA. The ACA titre
was probably lowered by previously administered corticosteroid therapy. There are several reports of transverse myelopathy as a
manifestation of antiphospholipid antibody
syndrome in the past decade.2 3 A potential
pathogenic role of antiphospholipid antibodies in transverse myelopathy might be based
either on vasculopathy or on interaction with
spinal cord phospholipids.
Infection by helminths is universally associated with activation of T helper 2 (Th2)type cells. Regardless of the mechanisms and
protective value of antihelminthic Th2 responses, such responses may also be detrimental to the host. The presence of ACA,
anti-GM1, and antisulfatide antibodies in our
patient suggests a systemic response to E vermicularis, as it has been shown that nematodes contain cardiolipin, ganglioside GM1,
and sulfatides within their complex lipid
composition.4 When parasites share epitopes
with host tissue, such molecular mimicry may
exploit host immune tolerance against a self
determinant. Autoimmunity may occur if
immune tolerance is overriden in genetically
susceptible hosts. It has been proposed that
the presence of pathogenic cross reactive
autoantibodies could be the basis for the
relation between nematodes and autoimmunity. It may be also postulated that E vermicularis stimulated Th2 response which enhanced polyclonal autoantibody production
resulting in the presence of ACA, anti-GM1,
and antisulfatide antibodies. The association
of transverse myelopathy, ACA, and enterobiasis might be purely coincidental, which we
assume to be highly unlikely. The finding of
diVerent autoantibodies, as well as the isotype
switch of ACA, strongly suggests that pinworm infestation in our patient was the “triggering event” that increased the production
of autoantibodies against cardiolipin and led
to the development of transverse myelopathy.
The appearance of the spinal cord damage
caused by ACA in our patient might have
been facilitated by the simultaneous eVect of
anti-GM1 and antisulfatide antibodies. A
significant subset of the human anti-GM1
antibodies that reacted with the Gal(b1–
3)GalNAc determinant also bound to
oligodendrocyte-myelin glycoprotein which
is a constituent of the myelin of the CNS. As
for antisulfatide antibodies, their presence
has been already shown in some diseases
aVecting the CNS.
It is clear that parasitic infections can serve
as a trigger factor of autoimmune reactivity,
but the presence of autoantibodies or self
reactive T cells is rarely associated with clinical manifestations. They develop only in
patients with adequate immunogenetic and
hormonal background for autoimmune diseases. In several studies, increased frequencies of HLA-DR4, DR7, DR53, and DQ7
were found in patients with antiphospholipid
antibody syndrome,5 and in our patient

HLA-DR4 and DR53 were present. Additional studies are necessary to further elucidate the complex mechanisms of involvement
of intestinal helminths in the processes of
autoimmune activity.
JELENA DRULOVIC
u
IRENA DUJMOVIC
u
NEBOJŠA STOJSAVLJEVIC
u
IVANA TRIPKOVIC
u
SLOBODAN APOSTOLSKI
ZVONIMIR LEVIC
u
Institute of Neurology, CCS, School of Medicine,
University of Belgrade, Dr Subotiæa 6, 11000
Belgrade, Yugoslavia
MARIJA MOSTARICA STOJKOVIC
u
Institute of Microbiology and Immunology
BRANKA BONAC
yI
SLADJANA ANDREJEVIC
u
Institute of Allergology
Correspondence to: Dr J Drulović, Institute of
Neurology, CCS, School of Medicine, University of
Belgrade, Dr Subotića 6, 11000 Belgrade, Yugoslavia

1 Montag A, Ulrich R. Chronic urticaria in
enterobiasis vermicularis (oxyuriasis). Hautarzt
1992;43:652–3.
2 Schantz V, Oestergaard LL, Junker P. Shrinking
spinal cord following transverse myelopathy in
a patient with systemic lupus erythematosus
and the phospholipid antibody syndrome. J
Rheumatol 1998;25:1425–8.
3 Ruiz-Arguelles GJ, Guzman-Ramos J, FloresFlores J, et al. Refractory hiccough heralding
transverse myelitis in the primary antiphospholipid syndrome. Lupus 1998;7:49–50.
4 Maloney MD, Semprevivo LH. Thin-layer and
liquid column chromatographic analyses of the
lipids of adult Onchocerca gibsoni. Parasitol Res
1991;77:294–300.
5 Wilson WA. Histocompatibility genes in antiphospholipid antibody syndrome. Lupus
1996;5:259–262.

Radiologically selective visual pathway
involvement in adult onset cerebral
adrenoleukodystrophy
A case of adult onset cerebral adrenoleukodystrophy is presented with serial MRI showing selective involvement of the visual system
with spread of disease along the fibre tracts of
this system.
Adult onset cerebral adrenoleukodystrophy is the rarest presentation of
adrenoleukodystrophy.1 2 It may present with
various symptoms often including visual
impairment.3 Brain MRI may show multiple
areas of symmetric high signal intensity
within cerebral white matter, usually aVecting
the occipital lobes.4–6 We present a case of
adrenoleukodystrophy, in whom serial MRI
demonstrated selective progression of demyelination through the visual pathways.
A thirty year old man presented in May
1996 with a 7 month history of deteriorating
vision, slurred speech, incoordination, poor
balance, generalised weakness, sleep disturbance, and headaches. His symptoms were
worse on the right. He had no symptoms of
postural hypotension.
His mother had been shown to be a carrier
of X linked adrenoleukodystrophy (XLALD). His two elder brothers had died of
XL-ALD at the ages of 6 and 7 years. In 1993
our patient had been shown to have abnormal
serum concentrations of very long chain fatty
acids (VLCFAs) and to be a carrier of the
XL-ALD gene. At that time he was asymptomatic and had no abnormal neurological
signs. Crohn’s disease had been diagnosed in
1987 after an ileal resection although this had
remained in remission.
On examination, visual acuities were 6/12
(right), 6/9 (left). Fields were full to confron-