Clinical Diagnosis and Management of Atypical Parkinsonian Syndromes

Atypical parkinsonian syndromes encompass a group of neurodegenerative disorders that share features of Parkinson’s disease but diverge in pathology, clinical progression and treatment response. Progressive supranuclear palsy (PSP), multiple system atrophy (MSA) and corticobasal syndrome (CBS) represent the core entities within this category. The clinical diagnosis relies on careful phenotyping of motor signs—such as early postural instability, supranuclear gaze palsy and asymmetric rigidity—alongside non-motor manifestations including autonomic failure, cognitive impairment and cerebellar dysfunction. Recent consensus diagnostic criteria have refined subtypes of PSP and CBS, emphasising combinations of akinesia, oculomotor disturbance, gait freezing and cortical features to improve diagnostic sensitivity and specificity. Neuroimaging biomarkers, incorporating structural MRI measures of midbrain atrophy and advanced diffusion techniques, augment clinical assessment and support differential diagnosis. Functional imaging of dopaminergic and tau pathology further refines patient selection for disease-modifying trials. Management remains predominantly symptomatic, with limited responses to levodopa and other dopaminergic agents. Multidisciplinary interventions—physiotherapy, speech and occupational therapy—are essential to maintain mobility and quality of life. Emerging strategies target neuroprotective pathways, cholinergic modulation and tau aggregation, signalling a shift towards personalised care frameworks and clinical trial readiness across global research networks.

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Clinical Diagnosis and Management of Atypical Parkinsonian Syndromes publication trend

The graph below shows the total number of articles in clinical diagnosis and management of atypical parkinsonian syndromes across all publications each year (not limited to Nature Index journals).

Technical terms

Progressive supranuclear palsy (PSP): A tauopathy presenting with early gait disturbance, vertical gaze palsy and axial rigidity.

Corticobasal syndrome (CBS): A syndrome characterised by asymmetric rigidity, limb apraxia and cortical sensory loss, often underpinned by tau or Alzheimer-type pathology.

Multiple system atrophy (MSA): A synucleinopathy manifesting with parkinsonism and prominent autonomic failure or cerebellar ataxia.

Fixel-based analysis (FBA): An advanced diffusion MRI technique quantifying fibre density and cross-section within white matter tracts.

Nucleus basalis of Meynert (NbM): A basal forebrain cholinergic nucleus critical for cortical arousal and cognitive function.

References

1. Progression of atypical parkinsonian syndromes: PROSPECT-M-UK study implications for clinical trials. Brain (2023).
2. Fiber-specific micro- and macroscopic white matter alterations in progressive supranuclear palsy and corticobasal syndrome. npj Parkinson's Disease (2023).
3. Nucleus Basalis of Meynert Degeneration Predicts Cognitive Decline in Corticobasal Syndrome. Biological Psychiatry (2024).