Multiple system atrophy (MSA) is a neurodegenerative condition characterised clinically by a combination of parkinsonian, autonomie, cerebellar and pyramidal signs and neuropathologically by neuronal loss and the presence of glial and neuronal cytoplasmic inclusions in the basal ganglia and ponto-cerebellar system. The recognition of MSA may be easy when the full-blown picture is encountered, but some patients may be misdiagnosed as Parkinson's disease early in the disease phase. Quinn first proposed in 1989 a list of diagnostic criteria for MSA, which have represented the 'gold standard' in this field for the last decade. Nevertheless, his criteria have some pitfalls which mainly consist in the unclear definition of some of the clinical features and in the excessive emphasis put on some laboratory investigations (e.g. urethral and anal sphincters EMG). This observation has been taken into account by the members of the Consensus Conference (Oilman et al., 1998), who recently proposed the new clinical guidelines for the diagnosis of this condition. In order to test these guidelines, we applied them retrospectively in a series of clinical cases of MSA already enrolled in the ESGAP study using Quinn's criteria. Forty-five cases of MSA (29 M and 16 F), presenting with predominant parkinsonian (n = 37) or cerebellar (n = 8) signs were studied. The agreement by the two observations was moderate (K = 0.59), even if 4 cases (8.8%) previously classified as possible or probable MSA according to Quinn's criteria, could not be diagnosed as having this condition using Oilman's criteria. We conclude that the latter appear, on purely clinical grounds, reliable, but their real sensitivity and specificity must be tested on MSA cases with pathological confirmation before recommending their widespread use.
|Number of pages||1|
|Journal||Italian Journal of Neurological Sciences|
|Publication status||Published - 1999|
ASJC Scopus subject areas
- Clinical Neurology