We present two cases of
Werner's syndrome associated with
intracranial meningioma. Characteristic clinical features of
Werner's syndrome include short stature with slender extremities, premature senility, juvenile
cataract, skin changes, a tendency to
diabetes mellitus and familial occurrence. A 44-year-old female, who had been treated for
diabetes mellitus, was diagnosed as having
Werner's syndrome because of various characteristic features. A falx
meningioma was incidentally found on CT scan, and was surgically removed. Her
diabetes mellitus improved. The second case was a 28-year-old male was diagnosed as having
Werner's syndrome,
diabetes mellitus, juvenile
cataract, together with
diabetes insipidus, and
liver dysfunction. He developed severe
headache, gait disturbance and then became unconscious with right
hemiparesis. He was found to have a
parasagittal meningioma by CT scan and angiography. After removal of the
tumor,
diabetes mellitus,
diabetes insipidus and
liver dysfunction improved. The reported incidence of
neoplasms associated with
Werner's syndrome is about 10%. The majority of associated
tumors were mesenchymal in origin. Ten
meningiomas, 1
neurinoma and 2
gliomas are reported as associated
tumors in the central nervous system. Most of the associated
meningiomas were asymptomatic and found incidentally at autopsies or CT scans.
Diabetes mellitus associated with
Werner's syndrome is generally mild with high immunoreactive
insulin value and is controllable by
diet therapy and oral
antidiabetic drugs. Daily profile of
blood sugar improved after the removal of
tumor in our cases. In 50 gm
glucose tolerance test, tendency of delayed appearance of peak value, which is common in
Werner's syndrome, was not altered in our cases. Discussion is made as to the association of
Werner's syndrome with
meningioma and
diabetes mellitus.