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Natalia Fornal, Dominik Grzebyk, Ewelina Bracik,
Tomasz Chromiec
Students’ Scientific Society, Departament of
Neurology, Medical University of Lublin, Poland
Scientific supervisor: dr n. med. Joanna Bielewicz
Case of patient with progressive supranuclear
palsy with untypical fast progression, after
ganglia stroke
Atypical Parkinsonism
corticobasal
degeneration
progressive
supranuclear
palsy
multiple system
atrophy
Lewy body
dementia
Progressive supranuclear palsy -
called Steele-Richardson-
Olszewski Syndrome
neurodegenerative
diesease, from group
of tauopathies, with
slowly progress,
shorter life expectancy
5-10 cases/100.000
population
neuronal loss, tau
protein deposits in
neurons
etiolology is unknown,
there are several
hypotheses, uknown
treatment
manifesting clinically
about 6th decade of life,
progresses to death
within a decade
PSP manifestations
 supranuclear ophthalmoplegia in vertical
movement
 pseudobulbar palsy – dysarthria,
dysphagia,
 early falls and postural instability
 atypical Parkinsonism – bradykinesia, axial
rigidity,
 dementia, cognitive impairments
 retrocollis
 without recovery after L-dopa treatment
Case report
A 67 year old female,
admitted to Neurological Departament in 2012
AILMENTS
 speech disorders
 signs of extrapiramidal syndrome, bradykinesia
 rigidity
MRI indicated basal ganglia stroke:
 right hemisphere
 subcortical, in caudate nucleus and putamen
Doppler USG without any impairments in vessels
Focus of stroke in
basal ganglia
Case report
Patient presented also other, unconected
with stroke symptom
 dysartria, intensifing during a day
 Additional examination: EMG, cervical
spine MRI
was insufficient to exact diagnosis
 Then, in 2012 atypical Parkinson sydrome
Case report
Recommendations:
-Regular check-up in
neurological clinic
-Medicines: -Acard
-Bisocard
-Simvastatin
-Madopar (L-dopa)
Case report
2017 - extension of last disorders
Neurological examination
- bradykineesia, without tremor
-rigidity in all muscle groups
-dysartria
- inability in vertical movement of
eyes
- falls and instability of posture
Case report
 atrophy of the midbrain, especially in tegmentum
increase of interpeduncular fossa length
 normal diameter of pons
MRI and all neurologial symtoms leaded to PSP
diagnosis
Radiographic features in PSP
 midbrain atrophy- reduced area ratio on the
sagittal plane
 mickey mouse sign- reduced anteroposterior
diameter of midbrain
 morning glory sign: tegmentum - loss of the
lateral convex margin
 hummingbird sign / penguin sign- is referring
to the axial MRI, showing atrophy of the
midbrain tegmentum, with reduction of the
midbrain tectum and cerebral peduncles
Atrophy of
midbrain
(„Mickey-
Mouse
sign”)
flattening
outline of the
midbrain
(„hummingbir
d sign” or
„penguin
sign” )
Conclusions
 Diagnosis od PSP, which is a rare diesease, still
constitutes
a challange
 PSP can be undiagnosed or misdiagnosed as a more
common, neurodegenerative brain dieseases, because
of simmilar symptoms:
 Parkinson’s disease (due the balance and movement difficulties and
rigidity)
 Alzheimer’s (due to the cognitive disturbances), and other.
 It is important, to take into consideration some
symtoms more
characteristic for PSP, than other atypical
parkinsonism:
Conclusions
 In PSP a slowly progression is
typical, however in this case a fast
intensificaton of symptoms along
with stroke may lead to obstacles,
during diagnosis, which was notably
difficult due to rare diesease’s
occurence.
Thank You for Your
attention!

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Fornal2

  • 1. Natalia Fornal, Dominik Grzebyk, Ewelina Bracik, Tomasz Chromiec Students’ Scientific Society, Departament of Neurology, Medical University of Lublin, Poland Scientific supervisor: dr n. med. Joanna Bielewicz Case of patient with progressive supranuclear palsy with untypical fast progression, after ganglia stroke
  • 3. Progressive supranuclear palsy - called Steele-Richardson- Olszewski Syndrome neurodegenerative diesease, from group of tauopathies, with slowly progress, shorter life expectancy 5-10 cases/100.000 population neuronal loss, tau protein deposits in neurons etiolology is unknown, there are several hypotheses, uknown treatment manifesting clinically about 6th decade of life, progresses to death within a decade
  • 4. PSP manifestations  supranuclear ophthalmoplegia in vertical movement  pseudobulbar palsy – dysarthria, dysphagia,  early falls and postural instability  atypical Parkinsonism – bradykinesia, axial rigidity,  dementia, cognitive impairments  retrocollis  without recovery after L-dopa treatment
  • 5. Case report A 67 year old female, admitted to Neurological Departament in 2012 AILMENTS  speech disorders  signs of extrapiramidal syndrome, bradykinesia  rigidity MRI indicated basal ganglia stroke:  right hemisphere  subcortical, in caudate nucleus and putamen Doppler USG without any impairments in vessels
  • 6. Focus of stroke in basal ganglia
  • 7. Case report Patient presented also other, unconected with stroke symptom  dysartria, intensifing during a day  Additional examination: EMG, cervical spine MRI was insufficient to exact diagnosis  Then, in 2012 atypical Parkinson sydrome
  • 8. Case report Recommendations: -Regular check-up in neurological clinic -Medicines: -Acard -Bisocard -Simvastatin -Madopar (L-dopa)
  • 9. Case report 2017 - extension of last disorders Neurological examination - bradykineesia, without tremor -rigidity in all muscle groups -dysartria - inability in vertical movement of eyes - falls and instability of posture
  • 10. Case report  atrophy of the midbrain, especially in tegmentum increase of interpeduncular fossa length  normal diameter of pons MRI and all neurologial symtoms leaded to PSP diagnosis
  • 11. Radiographic features in PSP  midbrain atrophy- reduced area ratio on the sagittal plane  mickey mouse sign- reduced anteroposterior diameter of midbrain  morning glory sign: tegmentum - loss of the lateral convex margin  hummingbird sign / penguin sign- is referring to the axial MRI, showing atrophy of the midbrain tegmentum, with reduction of the midbrain tectum and cerebral peduncles
  • 13. flattening outline of the midbrain („hummingbir d sign” or „penguin sign” )
  • 14. Conclusions  Diagnosis od PSP, which is a rare diesease, still constitutes a challange  PSP can be undiagnosed or misdiagnosed as a more common, neurodegenerative brain dieseases, because of simmilar symptoms:  Parkinson’s disease (due the balance and movement difficulties and rigidity)  Alzheimer’s (due to the cognitive disturbances), and other.  It is important, to take into consideration some symtoms more characteristic for PSP, than other atypical parkinsonism:
  • 15. Conclusions  In PSP a slowly progression is typical, however in this case a fast intensificaton of symptoms along with stroke may lead to obstacles, during diagnosis, which was notably difficult due to rare diesease’s occurence.
  • 16. Thank You for Your attention!