A 68-year-old woman with headache of one year's duration and frontal protrusion. She had a history of type 2 diabetes mellitus, hypertension, extrinsic bronchial asthma and osteoporosis.
The symptoms began with localised headache in the face and right hemicranium, and increased ESR and anaemia were detected, so empirical treatment with glucocorticoids was started on suspicion of temporal arteritis. At that time no biopsy of the temporal artery was performed. Four months later the patient suffered bilateral loss of vision. Ophthalmoscopic examination showed signs of intracranial hypertension. An MRI scan of the brain showed diffuse thickening of the calvarium and meninges. Analyses showed persistent anaemia, with a tendency to macrocytosis. CSF analysis showed hyperproteinorrachia (he was still on corticosteroid treatment) and no malignant cells were found. After a biopsy of the temporal artery, arteritis was ruled out and she was referred to our hospital for further investigation.
The aforementioned clinical manifestations were associated with jerking crises in the right hemibody that subsided with clonazepam, complex visual hallucinations, changes in character with increased irritability and loss of recent memory.
The general physical examination showed an apyretic patient, with cutaneous-mucosal pallor, right frontal protrusion and cutaneous signs of cranial hypervascularisation. She had oedema of both eyelids and right eyelid ptosis. No lymph nodes were palpable. Cardiopulmonary auscultation was normal. Neurologically she was temporarily disoriented and apathetic. Muscle fatigue was present. She scored 19 out of 30 in the Mini-mental test. There was a decrease in visual acuity in both eyes, to a greater extent in the left eye (with the right eye she counted fingers at one metre, with the left eye at half a metre). Fundus examination showed papillary oedema and bilateral cotton wool exudates, more marked in the left eye. Motor balance was symmetrical. He was walking with short strides and decreased brachiation.

Blood tests showed GGT 85 IU/l; LDH 779 IU/l; hypoproteinaemia (total protein 5.61 g/dl, albumin 3.4 g/dl); other biochemical parameters were normal. The haemogram showed anaemia (Hb 8.6 g/dl) with an inflammatory iron pattern, platelets 106,000/mm3 and normal leukocytes. Normal haemostasis. Elevated ESR (53 mm/h). Primary hypothyrodism was detected (TSH 1.1 mIU/l, free T4 0.6 ng/dl) and increased PTH (170 pg/ml). Proteinogram and FSR without pathological findings. Among the tumour markers only CA 15.3 was elevated (67.4 IU/ml). Cranial CT showed diffuse calcification suggestive of hyperostosis with scattered meningeal-dural thickening more marked in the left frontal region. Lumbar puncture showed clear cerebrospinal fluid with mild hyperproteinuria (48 mg/dl), glucose 74 mg/dl and 0 cells. No malignant cells were found. CSF cultures were negative. EEG study showed diffuse slowing with intermittent frontal slow activities and lesion-irritative signs in the left temporal area. Brain MRI detected diffuse hyperostosis of the cranial calvarium with diffuse abnormal meningeal enhancement more prominent in the left frontal region. Mammography was normal. Metastatic bone series showed generalised osteosclerosis. Bone scan showed increased activity in the skull, especially at the fronto-parietal level, compatible with hyperostosis. The iliac crest biopsy showed artefacted trabeculae without haematopoietic activity. The thoraco-abdominal-pelvic CT scan found no alterations.

In the presence of pachymeningitis with secondary hyperostosis, with no malignant cells detected in CSF in serial lumbar punctures and without knowledge of the existence of a primary cancer, a biopsy of the cranial bone and meninges was performed with the following anatomo-pathological result: calotte with hyperostosis, bone marrow and dura mater with extensive infiltration by metastatic adenocarcinoma. The immunohistochemical profile did not indicate the origin of the primary tumour.
Cranial radiotherapy treatment was indicated, which the patient refused, and palliative treatment with tamoxifen was started due to the suspicion of a breast tumour. In fact, a few months after the biopsy, a new thoraco-abdomino-pelvic CT scan was performed, which showed an irregular right axillary nodule. The patient refused aggressive treatment and died at home two months after discharge, so no post-mortem examination was performed.
