Hypothalamo-pituitary sarcoidosis: a multicenter study of 24 patients.

AIM: To assess clinical features, treatment and outcome of Hypothalamo-pituitary (HP) sarcoidosis and to determine whether HP is associated with a particular clinical phenotype of sarcoidosis. DESIGN: Multicentric retrospective study. METHODS: Retrospective chart review. Each patient was matched with two controls. RESULTS: Twenty-four patients were identified (10 women, 14 men). Their median age at the sarcoidosis diagnosis was 31.5 years (range: 8-69 years). HP involvement occurred in the course of a previously known sarcoidosis in 11 cases (46%), whereas it preceded the diagnosis in 13 patients (54%). All but two patients had anterior pituitary dysfunction, 12 patients presented with diabetes insipidus. The most common hormonal features were gonadotropin deficiency (n=21), TSH deficiency (n=15) and hyperprolactinemia (n=12). Magnetic Resonance Imaging (MRI) revealed infundibulum involvement (n=8), pituitary stalk thickness (n=12) and involvement of the pituitary gland (n=14). All but two patients received prednisone. After a mean follow-up of 4 years, only two patients recovered from hormonal deficiencies. MRI abnormalities improved or disappeared in 12 cases under corticosteroid. There was no correlation between the hormonal dysfunctions and the radiologic outcomes. Patients with HP sarcoidosis had significantly more frequent sinonasal localizations and neurosarcoidosis and required a systemic treatment more frequently than controls. CONCLUSION: Although HP sarcoidosis is unusual, physicians should be aware that such specific localization could be the first manifestation of sarcoidosis. HP involvement is associated with general severity of sarcoidosis. MRI abnormalities can improve or disappear under corticosteroid treatment, but most endocrine defects are irreversible.

Effect of stimulus frequency and stimulation site on the N1m response of the human auditory cortex.

The aim of the present study was to investigate the functional organization of the auditory cortex for pure tones of 1, 2, 4, 6, 8 and 12 kHz. Ten subjects were tested with a whole-head magnetometer (151 channels). The location, latency and amplitude of the generators of the N1m (the main component of the response, peaking approximately at 100 ms) were explored simultaneously in the right and left hemispheres under monaural stimulation. Our results revealed that tonotopy is a rather complex functional organization of the auditory cortex. From 1 to 12 kHz, tonotopic maps were found for contralateral as well as for ipsilateral stimulation: N1m generators were found to be tonotopically organized mainly in an anterior-posterior direction in both hemispheres, whatever the stimulated ear, but also in an inferior-superior direction in the right hemisphere. Furthermore, latencies were longer in the left than in the right hemisphere. Two different representations of spectral distribution were found in the right auditory cortex: one for ipsilateral and one for contralateral stimulation.