Objective: To improve the differential diagnosis between patients with mult
iple system atrophy (MSA) and idiopathic PD (IPD) with autonomic failure. B
ackground: Some patients diagnosed with IPD are discovered to have alternat
ive diseases such as MSB, despite the application of stringent diagnostic c
riteria. This differentiation is particularly difficult if patients with IP
D also show symptoms of autonomic failure. In IPD, autonomic failure is cau
sed by damage of the postganglionic part of the autonomic nervous system, w
hereas in MSA, degeneration of preganglionic and central autonomic neurons
is revealed histopathologically. Methods: Scintigraphy with [I-123] metaiod
obenzylguanidine (MIBG) enables the quantification of postganglionic sympat
hetic cardiac innervation. Fifteen patients with IPD and 5 patients with MS
A underwent standard autonomic function tests and scintigraphy with MIBG. R
esults: In all patients, cardiovascular testing showed evidence of autonomi
c failure of varying severity. In all patients with IPD, the heart-mediasti
num (H/M) ratio of MIBG uptake was pathologically impaired, independent of
duration and severity of autonomic and parkinsonian symptoms. All patients
with MSA had a regular H/M ratio. Each patient could be assigned to the cor
rect diagnostic group based on the results of the MIBG scintigraphy, even i
f the duration of the disease was only 2 years or less. Conclusions: This p
opulation assessment of the heart-mediastinum ratio of [I-123]metaiodobenzy
lguanidine uptake showed a high sensitivity for the detection of autonomic
involvement in patients with idiopathic IPD and also a high specificity for
the discrimination between idiopathic PD and MSA.