Diagnosing parkinsonism with atypical features: a classification system
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the title of my presidential lecture is
opening the pandora's box of
parkinsonism
and the concept of the pandora's box is
that when zeus gave the pandora box as a
present
anybody that would open that box
uh would just
free a lot of trouble that was inside
the box and i make an analogy
with that because every time you face a
patient with a parkinsonian syndrome or
with parkinsonian symptoms
it really is the start of trouble
because we are never sure if we are
facing a case
of parkinson's disease
some of the atypical parkinsonisms the
classical forms like
msa or psp or corticobasal degeneration
or the more difficult to diagnose the
very atypical cases that are thousands
caused by different
etiologies like mitochondrial disease
genetic forms of parkinsonism even some
of the ataxias of the or the paraplegias
can present with parkinsonism so we're
really faced
with a significant problem every time we
see a patient with parkinsonian syndrome
and the major limitation we have at
present is that we don't have
a definitive biomarker to differentiate
all these disorders we cannot even
differentiate between parkinson's
disease and some of the atypical
parkinsonisms because of the lack of
these
biomarkers so we have to
use clinically defined criteria we have
to
analyze the phenomenology of the patient
we have to
take a
very detailed clinical history the
family history
the existence of atypical manifestations
the use of auxiliary methods of
investigation like
mri and some of the functional
neural imaging techniques in order to
try to tease out all these different
types of parkinsonism
and the other significant problem is
that
we know for a fact that
when we see a patient with parkinson's
or with what we believe is parkinson's
disease for the first time
this can change in the course of the
disease
and i i
propose to apply the five-year rule
that means that for the first five years
in the course of a person that has
something that we believe is parkinson's
disease may change after five years
once the disease has consolidated and we
have a significant response to the
treatment
we have sorted out that the patient
doesn't have any atypical symptoms in
the clinical examination then we can be
significantly sure that we are facing a
case of parkinson's disease
and i
i'm proposing a classification
of parkinsonism that
includes several categories
and a roadmap to analyze these different
categories and the categories are the
typical parkinsonian
syndromes the
atypical presentations of the typical
parkinson's or parkinson's disease
the
typical atypical parkinsonisms
like msa or psp and then we have the
atypical presentations of these
disorders and finally we have this
huge box of the atypical atypicals so
that's
the skeleton of my presentation
the basis is on the clinical features of
the patients
it's clinically oriented because we
don't have any other
any other
indicator or clue to lead us into a
certain diagnosis so we have to base in
a very careful comprehensive clinical
examination
and follow-up of the patient
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