[Parkinsonian disorders: from clinical manifestations to diagnostic classification]

Abstract

Parkinsonism may include atypical clinical manifestations, which are warning signs for the clinicians and motivate further investigations to identify an etiology other than idiopathic Parkinson's disease. The dismemberment of pathological entities, the advances of morphological and functional imaging of the brain, and new insights into molecular biology have successively led to more precise clinical phenotype and mechanisms. Except for etiologies with specific treatment, such as Wilson's disease or Parkinsonism secondary to a lesion of basal ganglia, or the discontinuation of a culprit drug, the treatment of Parkinsonian syndrome is mainly based on a multidisciplinary approach, involving occupational therapist, physiotherapist, speech therapist, psychologist and social worker. L-Dopa may be tried but it is less effective in atypical Parkinsonian syndrome than in Parkinson's disease. Formal diagnosis, only achievable post-mortem, is not available during the lifetime of the patient. Although some additional tests provide undeniable assistance, the clinical approach remains an essential and critical step to avoid costly and unnecessary investigations.

Keywords: Atrophie multisystématisée; Corticobasal degeneration; Dégénérescence corticobasale; Démence à corps de Lewy; Lewy body dementia; Maladie de Wilson; Multiple system atrophy; Paralysie supranucléaire progressive; Parkinsonian disorders; Progressive supranuclear palsy; Syndromes parkinsoniens; Wilson's disease.