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A case of late-onset allgrove syndrome presenting with predominant autonomic dysfunction.
[triple a syndrome]
Allgrove
Syndrome
or
triple
A
syndrome
is
a
rare
familial
multisystem
disorder
characterized
by
achalasia
,
alacrima
and
adrenal
insufficiency
.
The
objective
was
to
describe
a
case
of
4
A
syndrome
where
autonomic
dysfunction
was
the
presenting
feature
.
A
22
-
year
-old
male
presented
with
erectile
dysfunction
and
loss
of
spontaneous
morning
erections
for
six
months
.
He
was
having
nocturnal
diarrhea
and
recurrent
postural
dizziness
for
three
months
.
He
was
found
to
have
hyperpigmentation
at
pressure
points
,
postural
hypotension
and
other
features
of
autonomic
dysfunction
.
Laboratory
investigations
and
imaging
studies
revealed
hypoadrenalism
,
achalasia
,
alacrima
and
peripheral
neuropathy
.
Autonomic
neuropathy
-related
features
persisted
even
after
correction
of
hypoadrenalism
.
Based
on
clinical
features
and
investigation
he
was
diagnosed
as
a
case
of
4
A
syndrome
presenting
with
autonomic
dysfunction
.
Allgrove
or
4
A
syndrome
should
be
considered
as
a
rare
differential
diagnosis
of
someone
presenting
with
features
of
autonomic
neuropathy
.
