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Cerebrovascular disease and systemic lupus erythematosus.

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gen vascular studies, and serological tests including HIV antibody were negative. The spinal fluid was under normal pressure with a glucose of 70 mg/dl, protein of 42 mg/dl, and 14
white cells (98% lymphocytes). All CSF cultures and stains
were negative as were tests for myelin basic protein and
oligoclonal bands. Homovanillic acid, the stable metabolite
of dopamine in CSF, was 31.6 ng/ml (normal, 48.8
and 3-methoxy-4-hydroxy-phenolglycol,
the stable derivative
of norepinephrine, was 7.3 ng/ml (normal, 12.8 ? 1.2). The
level of 5-hydroxyindoleacetic acid, a derivative of serotonin,
was normal (22.4 ng/ml). EEG showed diffuse slowing but no
epileptiform activity, and cerebral arteriography was unremarkable. A magnetic resonance image (MRI) of the brain
using a 1.5 Tesla GE magnet with T1, T2, and spin density
images was completely normal.
Although the patient had no gastrointestinal symptoms the
diagnosis of Whipple’s disease was suspected and an intestinal biopsy specimen from the region of the ligament of
Treitz demonstrated abnormalities in the lamina propria, including acute and chronic inflammatory cells, fibroblasts,
macrophages with periodic acid-Schiff-positive, diastase
nondigestible material. Electron microscopy showed degenerative rodlike structures varying in length from 1 to
4 pm within macrophages consistent with but not diagnostic of Whipple’s disease.
The patient was treated with parented penicillin and
streptomycin for 2 weeks. At the same time he was started
on oral trimethopridsulfamethoxazole which he has continued. After approximately 6 weeks of this therapy the patient’s OMM has diminished as have his periods of somnolence. His mental status markedly deteriorates after he
smokes a single cigarette and he continues to have impairment of voluntary eye movements.
We suspected the diagnosis of Whipple’s disease because
of the triad of OMM, loss of voluntary eye movements, and
hypersomnolence. We agree with Schwartz and colleagues
[l] that this symptom complex should strongly suggest the
diagnosis of Whipple’s disease of the CNS. Since the disease
may affect the central nervous system without involvement
of other organ systems, a brain and meningeal biopsy is indicated when this symptom complex is present even when an
intestinal biopsy is negative [2-51. In our case, a meningeal
biopsy was not performed because the patient had already
begun to improve on empiric therapy for Whipple’s disease
by the time the results of the intestinal biopsy were available.
CNS Whipple’s disease may occur in immunocompromised patients [6, 71 and the MRI scan may be abnormal
151. The present case indicates, however, that the disease
may occur in previously healthy individuals without MRI
abnormalities or other distinctive diagnostic clues. CSF may
be normal or, as in our case, demonstrate a mild pleocytosis.
The neurotransmitter studies in our patient are nonspecific
and probably reflect damage to the brain stem. A high index
of suspicion and recognition of the distinct clinical triad of
OMM, loss of volitional gaze, and hypersomnolence are necessary to detect what may be a more common clinical entity
than previously recognized.
Departments of Neurology and Pathology
University of Texas Medical School
Houston, T X
1. Schwartz MA, Selhorst JB, Ochs AL, et al: Oculornasticatory
myorhythmia: a unique movement disorder occurring in Whipple’s disease. Ann Neurol 1986;20:677-683
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Cerebrovascular Disease
and Systemic Lupus
G. Kujala, MD
I read with interest the article by Shiozawa and colleagues [5l
describing a patient with recurrent superior sagittal sinus
thrombosis and systemic lupus erythematosus. I wondered
whether the patient had a serological test for syphilis performed, an abnormality of coagulation or evidence of a lupus
anticoagulant, or whether an assay for anticardiolipin antibodies was performed.
The association between thrombosis and the presence of
the lupus anticoagulant [l, 41 or the related antibodies to
cardiolipin 12, 31 has been recognized with increasing frequency in recent years. It is important to use such assays to
predict better the risk of thrombotic events.
Section of Rheumatology
West Virginia University School of Medicine
Morgantwn, WV
1. Elias M, Eldor A: Thromboembolism in patients with the “lupus”type circulating anticoagulant. Arch Intern Med 144:510-515,
2. Harris EN, Boey ML, Mackworth-Young CG, et al: Anticardiolipin antibodies: detection by radioimmunoassay and association
with thrombosis in systemic lupus erythematosus. Lancet 11:
1211-1214, 1983
3. Harris EN, Gharavi AE, Asherson RA, et al: Cerebral infarction
in systemic lupus: association with anti-cardiolipin antibodies.
Clin Exp Rheumatol2:47-51, 1984
4. Mueh JR, Herbst KD, Rapaport SJ: Thrombosis in patients with
lupus anticoagulant. Ann Intern Med 92:156-159, 1980
5. Shiozawa 2, Yoshida M, Kobayashi K Superior sagittal sinus
thrombosis and systemic lupus erythematosus. Ann Neurol 20:
272, 1986
396 Annals of Neurology Vol 22 N o 3 September 1987
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cerebrovascular, lupus, systemic, erythematosus, disease
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