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Case Reports
Background We describe a young woman who presented often associated with stroke. Only complete laboratory testing,
with minor stroke as a first clinical symptom of Sjogren's including SSB antibody studies, and ophthalmologic and sali-
syndrome (SS) in the absence of well-known risk factors for vary gland evaluation revealed the correct diagnosis.
cerebrovascular disease. Conclusions SjSgren's syndrome should be considered
Case Description The medical history included recurrent among the causes of stroke, especially in a young female
miscarriages and sun rashes, which directed the diagnosis patient. (Stroke. 1994^5:2276-2279.)
toward inununologic disorders such as systemic lupus erythe- Key Words risk factors Sjflgren's syndrome young
matosus and antiphospholipid antibody syndrome, which are adults cerebrovascular disorders
Magnetic resonance images show two areas of hyperintensity on T r welghted image (right panel) and corresponding hypointenstty on
T,-weighted image (left paneO In the territory of the left middle cerebral artery, involving the cortex of the insuia and the giobus paiiidum.
R indicates right; L, left.
showed two areas of hyperintensity on T2-weighted ening of the mitral valves, which is not in itself consid-
images and corresponding hypointensity on T r weighted ered an independent risk factor for cardiac embolism to
images in the territory of the left middle cerebral artery, the brain.11 Additionally, Hess12 found that only the
involving the cortex of the insuia and the giobus paiii- combination of antiphospholipid antibodies and mitral
dum (Figure). valve abnormalities was associated with brain embolism.
An ophthalmologic examination revealed an abnor- In our patient antiphospholipid antibodies were nega-
mal break time and a rose bengal score greater than 4, tive on more than one occasion. Finally, we did not find
diagnostic of keratoconjunctivitis sicca.9 Minor salivary any detectable cardiac arrhythmias.
gland biopsy revealed more than one focus of lympho- Because of her young age and the absence of vascular
cytes in 4 mm2 of tissue, thus determining a grade IV and cardiac risk factors for cerebrovascular disease,
lymphocytic adenitis according to the Chishol-Mason hematologic and immunologic abnormalities were con-
scale and confirming the diagnosis of SS.10 sidered in the etiopathogenesis of the stroke. A congen-
The patient was treated with steroid and immunosup- ital or acquired deficiency of inhibitory coagulation
pressive therapy (dexamethasone, 20 mg once a week, factor proteins such as antithrombin III, protein C, and
and methotrexate, 7.5 mg once a week). In the following protein S, which has been established as probable cause
months she also presented with an autoimmune thy- of ischemic stroke in both young and old patients,1314
roiditis, which improved after adequate therapy. We was ruled out. Possible immunologic abnormalities were
continued to follow up the patient for more than 2 then considered. The clinical clues that alerted us to the
years, and she did not present with any other cerebral presence of an underlying immunologic disorder were
ischemic symptoms. the presence of three miscarriages and sun rashes in her
medical history. The possibilities of antiphospholipid
Discussion antibody syndrome and systemic lupus erythematosus,
In this patient the onset and time course of the often associated with stroke,1516 were considered. Our
neurological symptoms as well as the MRI findings patient did not fulfill established criteria for systemic
suggest the occurrence of a minor stroke in the vascular lupus erythematosus or for antiphospholipid antibody
territory of the left middle cerebral artery. syndrome. On the other hand, the presence of antinu-
Normal carotid duplex scan and cerebral angiography clear antibodies and anti-SSB antibodies, a quite spe-
ruled out disease of the extracranial and intracranial cific marker for SS,17-18 led us to perform more thorough
large arteries as a possible cause of stroke. A possible testing for SS. The ophthalmologic testing and the
cardioembolic mechanism was considered, but transtho- salivary gland biopsy confirmed the diagnosis of SS,
racic and transesophageal echocardiography ruled out according to the criteria of the most recent classification
the presence of relevant cardiac and aortic abnormali- of SS.17 Moreover, the association with autoimmune
ties. The only detectable cardiac abnormality was thick- thyroiditis and vitiligo frequently described in SS, a
well-known polyglandular (exocrine and endocrine) au- causes of stroke and a complete hematologic and im-
toimmune disease,19 completed the clinical presentation munologic screening for disorders rarely associated with
in our patient. cerebrovascular disease revealed the diagnosis.
Approximately 25% of SS patients present with CNS In conclusion, we believe that young stroke patients
complications3-3-6 and approximately 10% to 20% with without well-known risk factors for stroke should also
PNS complications.3 The entire neuroaxis, the periph- be evaluated for SS, even in the absence of other
eral and cranial nerves, and the ganglia may be involved clinical symptomatology. The laboratory screening
during SS. A neurological presentation of SS is consid- should include immunologic tests such as antinuclear
ered unusual in either the PNS 2023 or the CNS.3 A antibodies and anti-SSB and/or anti-SSA antibodies;
stroke as first clinical presentation of SS has been ophthalmologic and salivary gland evaluation should
described in association with cardiomyopathy,8 but this be performed only if the immunologic screening for SS
was not true in our case. This is the first well-docu- is positive.
mented large-artery minor stroke occurring in a young
woman in whom no cause other than uncomplicated SS Acknowledgments
has been found. The authors wish to thank Dr Patrizia Franco for the
The etiopathogenesis of the neurological damage in neuropsychological evaluation of the patient and Janet Wilter-
SS seems to be immunologically mediated. The most dink, MD, and Edward Feldmann, MD, for their comments on
common mechanism of PNS and CNS involvement an earlier draft.
during SS is due to vasculitis of small vessels. Necrotiz- References
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