Neurology · MU Sofia

68. Parkinson's plus syndromes

Covers the group of neurodegenerative disorders that combine parkinsonism with extra neurological features and respond poorly to levodopa. Goes through progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration and dementia with Lewy bodies, each with its clinical features and key sign. A comparison table sets these against classic Parkinson disease, followed by red flags, examination findings, imaging signs such as the hummingbird and hot cross bun signs, and symptomatic treatment.

Inside: 1 section · 1 labelled picture · 1 table · 15 flashcards · 2 min

Topic 68 of 88 in the MU Sofia syllabus for Neurology (Medicine, Year 4). The free AceMed note covers it in 1 section, about 282 words (2 min of reading), with 15 flashcards.

In this note

  1. Parkinsons plus syndromes

Parkinsons plus syndromes

Definition: Parkinson’s plus syndromes are a group of neurodegenerative disorders that present with parkinsonism plus additional neurological features and show poor or transient response to levodopa, unlike classic Parkinson disease.

Main Types

1. Progressive Supranuclear Palsy (PSP)

Pathology: Tau protein accumulation → brainstem degeneration

Clinical Features

  • Early postural instability and falls (backward falls)
  • Vertical gaze palsy (especially downward gaze)
  • Axial rigidity > limb rigidity
  • Dysarthria, dysphagia
  • Cognitive impairment (frontal dementia)

Key Sign: “Staring face” + inability to look down

2. Multiple System Atrophy (MSA)

Subtypes: MSA-P (parkinsonian type). MSA-C (cerebellar type)

Clinical Features: Parkinsonism (poor levodopa response). Cerebellar ataxia (MSA-C)

Severe autonomic failure: Orthostatic hypotension. Urinary incontinence/retention. Erectile dysfunction

Key Sign: Early autonomic dysfunction is hallmark

3. Corticobasal Degeneration (CBD)

Clinical Features: Asymmetric rigidity and bradykinesia. Apraxia. “Alien limb phenomenon”. Cortical sensory loss. Myoclonus

Key Sign: One limb behaves “independently”

4. Dementia with Lewy Bodies (DLB)

(Overlap between dementia and parkinsonism)

Clinical Features: Dementia occurs early. Visual hallucinations (vivid, recurrent). Parkinsonism (mild/moderate). Fluctuating cognition

Key Sign: Early dementia + hallucinations + parkinsonism

Comparison with Parkinson Disease

Clinical Clues for Diagnosis

“Red Flags” for atypical parkinsonism: Early falls. Early dementia. Severe autonomic. dysfunction. Poor response to levodopa. Rapid progression

Examination Findings

  • General Parkinsonism Signs: Bradykinesia. Rigidity. Postural instability
  • Additional Syndrome-Specific Signs
  • PSP → vertical gaze palsy
  • MSA → autonomic failure
  • CBD → apraxia, alien lim
  • DLB → hallucinations, fluctuating cognition

Diagnosis

Clinical Diagnosis (most important): Based on pattern recognition + atypical features

Imaging

MRI may show:

  • PSP: midbrain atrophy (“hummingbird sign”)
  • MSA: pontocerebellar atrophy (“hot cross bun sign”)
  • CBD: asymmetric cortical atrophy

Treatment

  • General: Poor response to levodopa. Symptomatic treatment only
  • Symptomatic Management: Physiotherapy. Speech therapy. Autonomic symptom management (MSA). Cognitive support (DLB)

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