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+ Paralisis Supranuclear Progresiva PSP

Anki
TARGET DECK: Medicina::Neuro
FILE TAGS: #Paralisis_Supranuclear_Progresiva

Diferencial

Differential Diagnosis

The differential is broad, and most sources do not formally rank alternatives by frequency; when tiering was not explicitly provided, alternatives were placed in the default bucket. [1][3]

Must-Not-Miss / High-Impact Alternatives

• Alternative: Autoimmune mimics (especially IgLON5 disease, anti-Ma2 encephalitis, and reported DPPX/LGI1 encephalitis). [3]
  Why considered: These were highlighted because some mimics can respond favorably to immunotherapy. [3]
  Key discriminators: IgLON5 may cause bulbar symptoms, gait difficulty, supranuclear palsy that mainly affects upward gaze, parasomnias, chorea, ataxia, and cognitive decline; anti-Ma2 encephalitis is usually associated with testicular cancer; DPPX encephalitis is linked to hyperexcitability and dysautonomia; DPPX/LGI1-related mimics typically progress faster and have more prominent cognitive symptoms. [3]
  Notes/uncertainty: The PDFs identify these as mimics but do not provide a unified bedside algorithm for separating them from PSP. [3]
• Alternative: Infectious mimics (Whipple disease and neurosyphilis). [3][4]
  Why considered: These are high-impact because treatment is possible if recognized early. [3]
  Key discriminators: Whipple disease should be considered when symptoms evolve rapidly, with or without gastrointestinal symptoms or arthralgias; neurosyphilis should be considered when light-near dissociation is present. [3]
  Notes/uncertainty: The broader MDS PSP criteria also flag Whipple disease and neurosyphilis as exclusionary conditions in appropriate clinical contexts. [4]
• Alternative: Creutzfeldt-Jakob disease / prion disease. [3][4]
  Why considered: This is a high-impact mimic because it can present with PSP-like features but typically reflects a much more rapid neurologic decline. [3]
  Key discriminators: Faster progression than typical PSP. [3][4]
  Notes/uncertainty: No additional bedside discriminator was provided beyond rapid progression. [3][4]

Common / First-Line Alternatives

• Alternative: Parkinson disease. [1][2]
  Why considered: This is one of the key differentials, especially early in the course and particularly for PSP-parkinsonism. [1][2]
  Key discriminators: PSP is described as symmetrical, with axial rigidity, severe global akinesia, early spontaneous falls, vertical paresis, dysarthrophonia, marked early executive dysfunction, and poor levodopa response; Parkinson disease is more typically asymmetric, limb-rigid, tremulous, later-falling, and strongly levodopa responsive. [1][2]
  Notes/uncertainty: PSP-parkinsonism may still resemble Parkinson disease for several years. [2]
• Alternative: Corticobasal syndrome. [1][2]
  Why considered: It is repeatedly listed among the main PSP mimics. [1][2]
  Key discriminators: Early asymmetric akinesia, apraxia, dystonia, myoclonus, cortical sensory loss, or alien-limb features favor corticobasal syndrome over PSP. [1][2]
  Notes/uncertainty: Overlap is substantial, and some PSP phenotypes themselves can present with corticobasal features. [2]
• Alternative: Multiple system atrophy. [1][2]
  Why considered: It is a core degenerative parkinsonian alternative. [1][2]
  Key discriminators: Predominant autonomic features and cerebellar signs favor MSA; anterocollis is described as more typical of MSA, whereas retrocollis supports PSP. [1][2]
  Notes/uncertainty: Imaging can help exclude or support alternatives, but the PDFs do not provide a single decisive bedside rule for all cases. [1][2]
• Alternative: Dementia with Lewy bodies. [1][2]
  Why considered: It is explicitly listed among degenerative parkinsonian alternatives. [1][2]
  Key discriminators: Hallucinations and fluctuations are common in DLB. [1]
  Notes/uncertainty: The PDFs do not provide a fuller discriminator set here beyond those features. [1]
• Alternative: Frontotemporal dementia. [1]
  Why considered: It is listed as a major mimic in the cognitive-behavioral spectrum. [1]
  Key discriminators: Predominant behavioral features and marked atrophy support FTD. [1]
  Notes/uncertainty: Behavioral overlap with PSP-frontal is acknowledged, so separation may remain difficult clinically. [1][3]
• Alternative: Normal pressure hydrocephalus. [1][2]
  Why considered: It is repeatedly listed as a key nondegenerative alternative. [1][2]
  Key discriminators: Supportive imaging findings are emphasized. [1]
  Notes/uncertainty: The PDFs do not provide a more detailed bedside discriminator set in the sections retrieved. [1]
• Alternative: Vascular parkinsonism / vascular disease. [1][2]
  Why considered: It is a recurrent alternative diagnosis in PSP-like presentations. [1][2]
  Key discriminators: Supportive imaging findings and vascular risk factors favor vascular disease. [1]
  Notes/uncertainty: Extensive small-vessel disease is specifically mentioned as an alternative that MRI may help exclude. [1]
• Alternative: Alzheimer disease. [1]
  Why considered: It is included among the major alternatives in cognitive presentations. [1]
  Key discriminators: Disproportionate memory impairment, hippocampal atrophy on MRI, and suggestive CSF biomarkers favor Alzheimer disease. [1]
  Notes/uncertainty: The PDFs do not provide a broader PSP-vs-AD clinical comparison beyond these features. [1]
• Alternative: Structural lesion. [1]
  Why considered: Structural brain disease can produce PSP-like presentations and must be excluded. [1]
  Key discriminators: Supportive imaging findings, including frontal mass lesions, favor a structural cause. [1]
  Notes/uncertainty: The PDFs frame MRI mainly as useful for excluding these alternatives rather than defining a specific clinical syndrome. [1]

Recommended visual anchor: Figure 2 — Veilleux Carpentier and McFarland - 2023 - Progressive supranuclear palsy current approach and challenges to diagnosis and treatment.pdf, p. 5. (Shows overlap between PSP phenotypes and major mimics.)

Uncommon Alternatives

• Alternative: Rare genetic mimics. [1][2]
  Why considered: These are explicitly described as rare or relevant especially in early-onset or familial presentations. [1][2]
  Key discriminators: Young age at symptom onset and a relevant family history support this category; examples explicitly listed include Niemann-Pick type C, Kufor-Rakeb syndrome, spinocerebellar ataxias, and several non-MAPT genetic disorders. [1][2][4]
  Notes/uncertainty: Niemann-Pick type C is specifically noted as important because it is treatable, and its gaze palsy may be horizontal or global rather than selectively vertical. [1][2]
• Alternative: Primary lateral sclerosis variant of motor neuron disease. [1]
  Why considered: It is specifically mentioned as a disorder that can resemble PSP. [1]
  Key discriminators: It may cause akinesia without decrement, oculomotor slowing including horizontal slowing, and dysarthria. [1]
  Notes/uncertainty: The PDFs present this as a narrower mimic rather than a routine first-line differential. [1]

Notes on Uncertainty

• Formal frequency-based tiering across the full PSP differential was not specified in most PDFs; items not explicitly prioritized were placed in the default bucket. [1][3]
• Validated bedside likelihood ratios across the full differential: Not confirmed in uploaded sources.
• A complete ED-style “rule-out-dangerous-mimics first” algorithm: Not confirmed in uploaded sources.

References

2026-04-08
[1] Rowe et al. - 2021 - Progressive supranuclear palsy diagnosis and management.pdf — 40%
[2] Pantelyat - 2022 - Progressive Supranuclear Palsy and Corticobasal Syndrome.pdf — 30%
[3] Veilleux Carpentier and McFarland - 2023 - Progressive supranuclear palsy current approach and challenges to diagnosis and treatment.pdf — 20%
[4] Höglinger et al. - 2017 - Clinical diagnosis of progressive supranuclear palsy The movement disorder society criteria.pdf — 10%

Flashcards

START
Basic
Header: [2026-04-08 | PSP]
Which common differential diagnosis most closely mimics PSP early, especially in PSP-parkinsonism?
Back:
Parkinson disease.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
What features favor PSP over Parkinson disease in the uploaded sources?
Back:
Symmetry, axial rigidity, severe global akinesia, early spontaneous falls, vertical eye movement abnormalities, dysarthrophonia, early executive dysfunction, and poor levodopa response.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
Which features favor corticobasal syndrome over PSP?
Back:
Early asymmetric akinesia, apraxia, dystonia, myoclonus, cortical sensory loss, or alien-limb features.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
Which key discriminator favors multiple system atrophy over PSP?
Back:
Predominant autonomic features and cerebellar signs.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
Which two bedside/history clues in the uploaded sources point toward dementia with Lewy bodies rather than PSP?
Back:
Hallucinations and cognitive fluctuations.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
What features favor Alzheimer disease over PSP in the uploaded sources?
Back:
Disproportionate memory impairment, hippocampal atrophy on MRI, and suggestive CSF biomarkers.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
Which treatable infectious mimic of PSP should be considered with rapid evolution plus gastrointestinal symptoms or arthralgias?
Back:
Whipple disease.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
Which autoimmune mimic of PSP is described as causing upward-gaze-predominant supranuclear palsy with bulbar symptoms, parasomnias, chorea, and ataxia?
Back:
IgLON5 disease.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
What broad clue should raise concern for prion disease or some autoimmune mimics rather than PSP?
Back:
A faster or rapidly progressive course.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

START
Basic
Header: [2026-04-08 | PSP]
When should rare genetic mimics of PSP be considered?
Back:
Especially in young-onset or familial cases.
Adicional:
Tags: #ZObs/Nivel/2Internista
END

PROMT

FIN