[Magnetic resonance imaging of the olfactory pathways in Kallmann de Morsier syndrome].
BACKGROUND
Kallmann syndrome is a disease clinically characterized by the association of hypogonadotrophic hypogonadism and anosmia or hyposmia. Most cases have been recorded among men. It is a genetic disorder with a specific gene location on the X chromosome. The cells that normally express luteinizing hormone-releasing hormone or LHRH fail to migrate the olfactory placode to the forebrain. The lateral projections of the olfactory placode also fail to induce development of the olfactory bulbs and tracts.


METHODS
The aim of this study was to compare the MRI appearance of the olfactory sulci, the olfactory bulbs and frontal lobe between groups. The first reference group was composed of 20 subjects and the second group of 18 patients suffering from Kallmann syndrome. For all studies we used a 1.5 T magnet system (Signa GE). We performed two sagittal and coronal T1-weighted sequences in spin echo (TR = 600 ms, TE = 12 ms) with interleaved 3 mm slices and a 14 cm field of view.


RESULTS
In the first group, the two olfactory bulbs were always seen on coronal slices just behind the crista galli measuring 2 to 3.2 mm transversally. On sagittal slices, in 60% of the cases two bulbs were seen (3 mm laterally of the pituitary stalk) and in the other 40% only one bulb was seen. The length of the bulb has been measured between 6 and 11 mm. We noticed a plat frontal lobe in 85% of the cases. In the second group the olfactory bulbs were never visible among the 18 patients suffering from Kallmann syndrome. The hypoplasic sulci were hardly visible and their size was less or equal to 1 cm and the frontal lobe was triangular in 80% of the cases. One patient had hypoplasia of corpus callosum.


CONCLUSIONS
MRI is helpful tool to demonstrate abnormalities of the olfactory system which are always present among patients suffering from Kallmann syndrome. MRI can also show, at the same time, a possible associated brain abnormality.
Fuerxer
F
F
Service de Radiologie, Hôpital Raymond Poincaré, Garches.
Carlier
R
R
Iffenecker
C
C
Schaison
G
G
Doyon
D
D
D000328:Adult; D061085:Agenesis of Corpus Callosum; D003337:Corpus Callosum / Q000473:pathology; D003392:Cranial Sinuses / Q000473:pathology; D005004:Ethmoid Bone / Q000473:pathology; D005260:Female; D005625:Frontal Lobe / Q000002:abnormalities / Q000473:pathology; D007987:Gonadotropin-Releasing Hormone / Q000235:genetics; D006801:Humans; D017436:Kallmann Syndrome / Q000175:diagnosis* / Q000235:genetics; D008279:Magnetic Resonance Imaging; D008297:Male; D000857:Olfaction Disorders / Q000175:diagnosis; D009830:Olfactory Bulb / Q000002:abnormalities / Q000473:pathology; D009833:Olfactory Pathways / Q000002:abnormalities* / Q000473:pathology; D010902:Pituitary Gland / Q000473:pathology; D014960:X Chromosome / Q000235:genetics
D007987:Gonadotropin-Releasing Hormone
L'imagerie par résonance magnétique des voies olfactives dans le syndrome de Kallmann de Morsier.