SHORT COMMUNICATION

Acta Neurol Scand., 1986:74:75-77
Key words: systemic lupus erythematosus;central nervous
system; multiple sclerosis.

Cerebral vasculitis as presenting symptom
of systemic lupus erythematosus
E.A.C.M. Sanders’, L.A.H. Hogenhuis’
’Department of Neurology, University Hospital, Leiden
and *“De Goddelijke Voorzienigheid” Hospital,
Sittard, The Netherlands.

ABSTRACT - A 42-year-old woman developed right-sided hemiparesis due to left-sided
encephalomalacia revealed by CT scan. Subsequent angiography revealed vasculitis of several intra-cranial arteries. The ESR was 65 mm/h. Further laboratory tests revealed no evidence of systemic disease so that no causal diagnosis could be posed. Treatment with prednisone (3 X 30 mg daily) led to complete cure of the hemiparesis within 6 weeks. Sixteen
months later, the patient developed cutaneaus lesions in the neck. Histological examination
of these lesions indicated the presence of systemic lupus erythematosus (SLE). Neurological
presentation of SLE is exceptional, while cerebral vasculitis as initial symptom of SLE has
never been described before.
Accepted for publication February 20, 1986.

Central nervous system (CNS) involvement in systemic
lupus erythematosus (SLE) is common (1) and includes: 1) hyaline degeneration of the meningeal, subcortical and cortical arterioles in 54% of the cases; 2)
perivascular lymphocytes in 28%; and 3) endothelial
proliferation in 21 %. True cerebral vasculitis, usually
involving the small arteries, is infrequent (6-137’0)
(2,3).
Neurological complications are regarded as a frequent cause of death in SLE (4). The CNS is usally involved during the course of the disease. Neurological
signs and symptoms may be focal and short-lived. It is
most exceptional to find neurological symptoms heralding SLE. Siekert and Clark (9,M e n et a1 (6) and
Fulford et al(7) each reported an instance of SLE presenting as multiple sclerosis (MS). April and van Sonnenberg (8) reported a case of SLE presenting as neuromyelitis optica. Other authors mention brachial plexus neuropathy (9), pseudobulbar palsy (lo), multiple
mononeuropathy (11) and epilepsy (12) occurring before the onset of other features of the disease.
Cerebral vasculitis usually develops in patients with
diagnostically established SLE (3). The present report
describes radiologically confirmed cerebral vasculitis of
unknown origin in a patient who developed systemic
skin manifestations of SLE 16 months after the appearance of neurological symptoms.

Case report
A 42-year-old woman had been treated for hyperthyroidism at the age of 33. In August 1982 she suddenly
qoticed slight weakness in her right arm, which cleared
up spontaneously within a week. Three weeks later, recurrence of the right arm symptom led to hospitalization.
Upon admission the patient had a body temperature
of 37.6‘C, blood pressure 150/90 and a regular pulse
of 6O/min. Physical examination revealed slightly
right-sided central facial paresis and right-sided hemiparesis, with increased tendon reflexes and extensor
plantar reflex on that side.
Laboratory data: ESR 65mm/hr, haemoglobin 7.9
g/dl, haematocrit 0.35, white blood cell count
11.15X109/L, SGPT 6 3 p/l, SCOT 38 $1, alkaline
phosphatase 254 p/l, LDH 800 ~ / l normal
,
renal
function, serum glucose 4.6 mmol/ 1. Rheumatoid serology normal. There were no serological LE signs. The
A N F and anti-DNA were negative. Cerebrospinal fluid
(CSF) analysis showed 3 lymphocytes/mm3, total albumin 0.50 g/l, IgG 0.032 g / l , Ig and IgM absent.
Agar electrophoresis revealed sign of transudation.
CSF glucose concentration was 3.4 m m o l / l . (JT scan
showed a left-sided central encephalomalacia (Fig. la).
Angiography showed non-filling of the left perical-

76

E. A. C. M. SANDERS & L. A. H. HOGENHUIS

Fig. I a. The CT scan reveals a hyperdense area on the patient’s left-side.
Fig. lb. Angiography shows a stenosis and vessel irregularities of frontal and parietal branches of the middle cerebral artery (arrows). All these abnormalities are on the patient’s lefi-side.
losal artery, irregularities of the vessel wall of the left
temporo-occipital artery and stenosis of the angular artery; all these signs are indicative of cerebral vasculitis
(Fig. lb).
However, no causal diagnosis could be made since
none of the investigations carried out gave any indication as to how this cerebral vasculitis had come about.
The only sign which was probably the result of some
systemic involvement was the elevated ESR. The pa-

tient was therefore given non-specific antiinflammentory treatment with prednisone (3 X 30 mg daily) over
a period of 4 weeks. She recovered completely within 6
weeks and was discharged.
In January 1984, she noticed red spots on the left
side of her neck (Fig. 2a). Histological examination of
a biopsy from one of these spots revealed a picture typical of SLE (Fig. 2b). The ESR was 20 mm/h and the
A N F 1:20 at that moment. Further serological tests for

Fig. 2a. Red spots in the neck found 16 months after the initial neurological symptoms.
Fig. 2b. Light-microscopic view of a section through a skin biopsy taken from one of the spots of Fig. 2a (H. & E.
X 19). Showing hyperkeratotic, keratotic plugging, epidermal atrophy and dermal perivascular infiltrate of mononuclear cells usually found in discoid lupus erythematosus.

VASCULITIS AS PRIME SYMPTOM IN SLE

rheumatoid arthritis were negative. This condition gave
the patient little trouble. Treatment with prednisone (3
X 30mg daily) was resumed and the-red spots healed
nicely within 6 weeks and all tests gave results within
normal ranges.

77

Acknowledgement
W e thank Dr. R. H. Batbgate (Eindhoven) for critically reviewing
this paper and Mrs. Ch. J. Th. Sanden-Bozon for preparing the
manuscript.

References
Discussion
To the best of our knowledge, the present report is the
first description of cerebral vasculitis as the primary
sign of SLE. At the time of the presenting neurological
symptoms, a systemic disorder could not be diagnosed;
the only indication of systemic disease was the elevated
ESR. Even at the time of systemic manifestation of
SLE on our patient, there was only one serological sign
of systemic disease: the slightly elevated ANF of 1:20.
The case history provided insufficient objective data
concerning the period of hyperthyroidism in 1973
mentioned by the patient herself. Thyroid function at
the time of the neurological disorder was slightly elevated T3:3.8 (normal 1.0-3.0) mmol/l, T4:180 (normal 65-160) mmol/l. Tests for antibodies against various tissue types revealed antibodies against smooth
muscle tissue. A microthyroid antibody titre of
225,000 was obtained in the first week of hospitalization. More generally, it may be possible to regard the
hyperthyroidism with the still histologically present
SLE, together as an expression of a manifest autoimmune deficiency.
No well-established therapy for CNS-SLE has yet
been developed. Only a few authors have evaluated the
role of corticosteroids in this context.
No double-blind studies have been performed. Gibson and Meyers (1) concluded that treatment with
massive doses of corticosteroids (> 120 mg daily) was
no more effective than that with smaller doses. Our patient did well on 90 mg prednisone per day; this dose
brought about a complete cure of her neurological
complaints, which have not recurred up to the time of
writing.
Hazelton et a1 (10) suggested in view of CNS-SLE
that extensive laboratory investigations should be c a n ed out on all patients with unusual neurological symptoms. Despite the. extensive investigation, we still had
no clear picture of what was wrong with the patient
during the first attack. The present report reminds us
that in some cases an adequate clinical picture can only
be obtained if extensive laboratory investigations are
combined with a detailed follow-up study.

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Address
L.A.H. Hogenhuis M D
Department of Neurology.
"De Goddelijke Voorzienigheid" Hospital,
Walramstraat 13,
Sittard,
The Netherlands