A 68-year-old woman with a one-year history of headache and frontal protrusion.
She had a history of type 2 diabetes mellitus, hypertension, bronchial asthma and osteoporosis.
The clinical picture began with localized headache in the right face and hemicrania, with an increase in ESR and anemia, so empirical treatment with glucocorticoids was started when a temporal arteritis was suspected.
Biopsy of that artery was not performed at that time.
Four months later the patient suffered bilateral vision loss.
The endoscopic examination revealed signs of intracranial hypertension.
Brain MRI showed diffuse thickening of callus and meninges.
Analytically anemia persisted, with tendency to macrocytosis.
CSF analysis showed hyperproteinorrhachia (follow-up on corticoids) and no malignant cells were observed.
After performing temporal artery biopsy, the existence of arteritis was ruled out and she was referred to our hospital for further study.
The above-mentioned symptoms were associated with shocks in the right hemibody that resolved with clonazepam, complex visual disturbances, changes in character with greater irritation and recent memory loss.
The general physical examination showed an apyretic patient with cutaneous-mucosal deformity, right frontal protrusion and cutaneous signs of cranial hypervascularization.
He had edema in both eyelids and right ptosis.
No lymphadenopathies were detected.
Cardiopulmonary auscultation was normal.
Neurologically, he was temporarily disoriented and apathetic.
He had muscle fatigue.
He scored 19 out of 30 on the Mini-mental test.
A decrease in visual acuity was observed in both eyes, to a greater extent in the left (right had fingers at one meter, with the left at half a meter).
Ocular fundus examination revealed papilledema and bilateral cotton-wool exudates, more marked in the left eye.
Motor balance was symmetrical.
He walked in short steps with a decrease in stroke.
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Blood analysis showed GGT 85 IU/l; LDH 779 IU/l; hypoproteinemia (total proteins 5.61 g/dl, albumin 3.4 g/dl); other biochemical parameters.
The blood count showed anemia (Hb 8.6 g/dl) with an inflammatory ferric pattern, platelets of 106,000/mm3 and normal leukocytes.
Normal hemostasis.
Elevated ESR (53 mm/h).
Primary hypothyroidism was detected (TSH 1.1 mIU/l, free T4 0.6 ng/dl) and increased PTH (170 pg/ml).
Proteinogram and PSI showed no pathological findings.
Among the tumor markers, only CA 15.3 was elevated (67.4 IU/ml).
Cranial CT showed diffuse thickening of the calcaneus suggestive of hyperostosis with meningeal-dural thickening more marked in the left frontal region.
In the lumbar puncture a clear cerebrospinal fluid was obtained, with mild hyperproteinorrhea (48 mg/dl), glucose 74 mg/dl and 0 cells.
No malignant cells were found.
CSF cultures were negative.
An EEG study showed diffuse slowing with slow intermittent frontal activities and damaging-irritative signs in the left temporal area.
Brain MRI detected diffuse hyperostosis of the skull base with more prominent diffuse abnormal enhancement in the left frontal region.
Mammography was normal.
In the metastatic bone series generalized osteolysis was observed.
The bone scan showed a reinforced skull activity, especially at the frontoparietal level, compatible with hyperostosis.
The iliac crest biopsy showed artifacted trabeculae without hematopoietic activity.
TAC-abdominopelvic was normal.
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malignant cells in CSF were detected in serial lumbar punctures and without knowledge of the existence of a primary cancer.
The immunohistochemical profile did not suggest the origin of the primary tumor.
Cranial radiotherapy was indicated that the patient refused, starting palliative treatment with tamoxifen when a breast tumor was suspected.
In fact, a few months after the biopsy, a new TAC-non-pelvic, showed an irregular right axillary nodule.
The patient refused aggressive treatments and died at home two months after discharge, so no autopsy study was performed.
