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Therefore, any insult during development or migration of these neurons result
in anosmia and hypogonadotropic hypogonadism.- How to test for anosmia?
Absence of history of anosmia/hyposmia does not exclude the possibility of
olfactory abnormalities in a patient with isolated hypogonadotropic hypogo-
nadism; therefore, formal clinical testing for anosmia should be performed in
all patients. Oil of clove, oil of peppermint, and asafoetida can be used for
bedside evaluation of anosmia, whereas University of Pennsylvania Smell
Identification Test (UPSIT) is an objective test for anosmia.- What are the nonreproductive abnormalities associated with Kallmann syn-
drome apart from anosmia?
A variety of nonreproductive abnormalities are associated with Kallmann syn-
drome. The neurological abnormalities include bimanual synkinesia (mirror
movements), neurosensory deafness, cerebellar ataxia and oculomotor abnor-
malities, and skeletal abnormalities which include clinodactyly, syndactyly,
camptodactyly, and short fourth and fifth metacarpals and metatarsals. Other
associations include cleft lip/palate, high-arched palate, ocular hypertelorism,
dental agenesis, and unilateral renal agenesis. - What is synkinesia?
Non-suppressible involuntary movements accompanied with voluntary move-
ments are known as synkinesia or mirror movement. Synkinesia is a physiolog-
ical phenomenon during childhood due to incomplete brain myelination and
can be associated with a variety of disorders like Kallmann syndrome, Klippel–
Feil disease, corpus callosum agenesis, Joubert syndrome, stroke, and
Parkinson’s disease. Approximately 40 % of patients with Kallmann syndrome
manifest synkinesia, which is classically seen in upper limbs, predominantly
involving hands (bimanual synkinesia), and is confined only to X-linked variant
of Kallmann syndrome (KAL1 mutation).- What is the mechanism of synkinesia?
Synkinesia can be considered as a manifestation of midline defect in patients
with Kallmann syndrome. Various theories have been proposed to explain the
phenomenon of synkinesia and include partial failure of decussation of7 Delayed Puberty