PURPOSE: To identify morphologic differences between Kallman syndrome
(KS) and idiopathic hypogonadotropic hypogonadism (IHH) and establish
a role for magnetic resonance (MR) imaging in these disorders. MATERIA
LS AND METHODS: Twenty-eight patients were compared with 10 eugonal ma
le volunteers. Eighteen patients had KS (hypogonadotropic hypogonadism
with anosmia) and 10 had IHH. All participants underwent hormone anal
ysis, a sniff-bottle smell test, and gadolinium-enhanced MR imaging. C
hanges in the hypothalamic-hypophyseal region and the rhinencephalon w
ere evaluated. RESULTS: MR imaging revealed intracranial morphologic c
hanges in all patients on plain T1-weighted sections. Seventeen patien
ts with KS demonstrated aplasia of an olfactory bulb; one olfactory su
lcus was absent in six, rudimentary in four, and normal in eight. Olfa
ctory bulbs were present in all 10 IHH patients and three showed one s
lightly hypoplastic bulb. Ten patients with KS and three with IHH show
ed an enlarged paranasal sinus system. Further MR findings were simila
r. CONCLUSION: MR imaging demonstrates abnormalities of the rhinenceph
alon present in KS patients and occasionally absent in IHH patients.