Cerebrospinal fluid oestrone in pseudotumour cerebri

Abstract

SUMMARY The concentration of oestrone in the cerebrospinal fluid (CSF) from obese young women with pseudotumour cerebri was much greater than predicted and found in normal subjects. Each woman with pseudotumour cerebri, and a high level of CSF oestrone and a CSF protein less than 0-2 g/l, had clinical improvement when treated with an 800 calorie/day diet and dexamethasone 2 mg/day.Increased oestrone production in adipocytes by the aromatisation of androstenedione may be the factor responsible for obesity in the pathogenesis of pseudotumour cerebri in young fat women.' Oestrone could cause the menstrual irregularities in such women by a direct effect upon endometrium and, perhaps, by alteration of feedback regulation of gonadotrophin secretion. Discovery of large amounts of oestrone in the cerebrospinal fluid (CSF) of an obese young women with Turner's Syndrome, pseudotumour cerebri and unexpectedly low serum gonadotrophin levels prompted this study of CSF oestrogens in pseudotumour cerebri.2 Methods Oestrogen concentrations were determined in duplicate by one of us (EH) using commercially available radioimmunoassays: Wien Laboratories, Succasunna, NJ, for oestrone and oestriol; CIS Radiopharmaceuticals, Bedford, Mass., for oestradiol. Oestrogens were extracted from 8 to 10 ml of serum or CSF with methylene chloride for oestrone and oestriol determinations, and diethyl ether for oestradiol assays. Oestrone and oestriol levels less than 5 pg/ml and oestradiol levels less than 8 pg/ml were less than the sensitivity of our techniques. The antibodies used in the assays were highly specific, reacting only with closely related compounds. In the oestrone assay, 1000 pg oestradiol was equivalent to 15 pg oestrone, and 50 000 pg oestnol did not alter the oestrone determination. Inter- assay and intra-assay reliability was within 10% and 3% respectively.All patients with pseudotumour cerebri had papilloedema and normal CT scans. The CSF used for oestrogen assays was collected after 5 to 8 ml had been taken for routine diagnostic tests. Each CSF sample had a protein concentration less than 0*40 g/l with one exception. After the first lumbar puncture, pseudotumour patients numbers 1-5 were treated with an 800 calorie/day diet and dexamethasone 2 mg/day.

What Do Transgender Patients Teach Us About Idiopathic Intracranial Hypertension?

“…The predilection of the disease for a particular gender would suggest that hormones may play a role in pathogenesis; however, previous studies into the role of oestrogens were inconclusive and have not been replicated. 14,15 It may be that the class of hormones that need further investigation are the androgens, as highlighted by this case report and the previous cases (Table 1), coupled with the observation that another androgen dysregulated condition, PCOS, shares a strikingly similar clinical phenotype. Furthermore, one study reports an increased incidence (58%) of PCOS in FTM patients prior to hormonal therapy.…”

Pseudotumor Cerebri (Benign Intracranial Hypertension): Etiopathogenesis, Diagnosis, And Management

“…With one exception, 40 most available data oppose excessive CSF formation 16,41 as a cause of pseudotumor cerebri. In this one exception, 40 the author used a mathematical calculation on data obtained from lumbar pressure monitoring using an infusion test in only three patients and noted an increased rate of CSF formation.…”

“…In this one exception, 40 the author used a mathematical calculation on data obtained from lumbar pressure monitoring using an infusion test in only three patients and noted an increased rate of CSF formation. However, detailed numerical and clinical data from this study are not available for analysis, and using similar techniques, a later study on a much larger sample of patients 41 showed no evidence of increased CSF formation.…”

Pseudotumor Cerebri: Categorical Disease or Spectrum of Disease

“…Many theories exist on PTC's pathomechanism. The mainstream of clinical research link this disease to the water retention effect of estrogens on the central nervous system [5,6]. However, this theory is contradicted by reports of PTC occurrence in males or in females with Turner's syndrome [7,8].…”