Progressive Supranuclear Palsy

1. Case Presentation
Dr. Md Rashedul Islam
FCPS, MRCP(UK)
Registrar, Neurology, BIRDEM

2. A 60 years old diabetic male hailing from
Baridhara,Dhaka got admitted in BIRDEM
General Hospital under department of
Neurology, on 04.05.14 with the complaints of :
• Difficulty in walking for 1 year
• Slurring of speech for 10 months
▫

3. According to the statement of the patient, he
was reasonably well 1 year back. Then he
developed walking difficulty which was
progressive, associated with problems with
maintaining balance & recurrent history of fall.
It was not associated with low back pain,
tingling of feet, incontinence of urine, head
injury, fever, weight loss.

4. H/O Present illness
He also gave history of slurring of speech for 10
months which was progressive, monotonous,
hypophonic, indistinct. He had difficulty in
pronouncing consonants but no fatigability,
swallowing difficulty was present. On detailed
query, he gave history of memory disturbance,
behavioral problems, insomnia, clumsiness &
impaired hand writing.

5. H/O PAST ILLNESS:
Nothing contributory
SOCIOECONOMIC HISTORY:
He belongs to a upper class family
PERSONAL HISTORY:
He is non alcoholic, non smoker.

6. FAMILY HISTORY
Nothing significant
TREATMENT HISTORY:
Tab. Metformin
Tab. Vit B complex

7. General examination:
Appearance:
Masked face
Startled expression

8. General examination:
Built: Overweight(BMI- 27.4 kg/m2)
Decubitus: on choice
Oedema
Anaemia
Jaundice
Cyanosis
Dehydration
Clubbing Absent
Koilonychia
Leukonychia

9. General examination:
Neck vein: not engorged
Thyroid: not enlarged
Lymph node: not palpable
Skin pigmentation & body hair distribution:
normal
Pulse: 86 b/min
BP: 130/80 mmHg
Temp:98 F
RR: 16 breaths/min

10. Higher psychic function :
Mild cognitive dysfunction(MMSE- 27/30)
Slowed cognitive processing, sequencing,
planning.
Apathy
Speech: Spastic type of dysarthria
Cranial nerves :
Slow vertical saccades
Vertical gaze palsy was improved with
vestibular ocular reflex & Bell phenomenon
GCS: 15/15
NERVOUS SYSTEM EXAMINATION

11. Speech

12. Muscle Rt. UL Lt. UL Rt. LL Lt. LL
Bulk Normal Normal Normal Normal
Tone Increase
d
Increase
d
Increase
d
Increased
Power 4/5 4/5 4/5 4/5
Involunt
ary
moveme
nt
Absent Absent Absent Absent
MOTOR FUNCTION:

13. ReflexReflex B T S K A Abd. PlantarPlantar
RightRight ++ ++ ++ ++ ++ N FlexorFlexor
LeftLeft ++ ++ ++ ++ ++ N FlexorFlexor

14. Sensory system:
Pain Temp Touc
h
Vibrati
on
Positi
on
sense
Right upper
limb
N N N N N
Right lower
limb
N N N N N
Left upper
limb
N N N N N
Left lower
limb
N N N N N

15. • Sign of Meningeal irritation - Absent
• Cerebellar sign : Absent
• Gait – Broad based unstable gait
• Others-
Bradykinesia
Axial rigidity was more pronounced that appendicular

16. Systemic examinations
Cardiovascular system
Respiratory system:
Alimentary system: no abnormality detected
Genitourinary system:

17. SALIENT FEATURE
A 60 years old diabetic gentleman presented with
progressive walking difficulty for 1 year with
problems of maintaining balance, recurrent
history of fall. It was not associated with low back
pain, tingling of feet, incontinence of urine. He
also had progressively slurring, monotonous,
hypophonic, indistinct speech with difficulty in
pronouncing consonants but no fatigability.

18. He also had history of features of dementia,
insomnia, clumsiness & impaired hand writing.
On examination, he had masked face, mild
cognitive impairment, Spastic dysarthria, vertical
gaze palsy & broad based, unstable gait.
Bradykinesia, marked postural instability,
predominant axial rigidity, diminished muscle
power was also evident. Other systemic
examination was normal.
SALIENT FEATURE

19. Provisional Diagnosis
• Diabetes Mellitus Type 2
• Parkinson-plus syndrome possibly
progressive supranuclear palsy

20. Differential Diagnosis
• Other Parkinson-plus syndromes
Parkinsonism-dementia complex
Corticobasal degeneration
• Pseudobulbar palsy due to bilateral hemishpere
infarction

21. Investigations
CBC: Hb % - 14.2 mg/dl
WBC -7000 cu/mm
Neu-65 %
Lymph- 17.8 %
Mono -5.9 %
Eosino- 1.1%
Platelet- 195000
ESR- 20mm in 1st
hour

22. ALT: 28 iu/L
AST: 32 iu/L
Lipid profile:
TG: 136 mg/dl
T. Chol : 122 mg/dl
LDL: 55 mg/dl
HDL: 40 mg/dl
Liver Function test:

23. Serum electrolytes
Na: 141 mmol/l
K: 4.8 mmol/l
Cl: 108 mmol/l
HCO3: 23 mmol/l
Renal Function Test
SS. Urea: 21 mg/dl
S. Creatinine: 1.0mg/dl
S. Urea: 32 mg/dl.

24. Sugar - Nil
Albumin – Nil
Ketone- Nil
Epi. cell: A few /HPF
Pus cell: 1-2 /HPF
RBC: Nil
Urine R/M/E

25. Chest X ray P/A view:
Normal

26. ECG: Normal

27. MRI of Brain: Atrophy
of dorsal midbrain
giving rise to “mouse
ears” appearance

28. MRI of Brain: Atrophy
of dorsal midbrain
giving rise to “mouse
ears” appearance

29. MRI of Brain:

30. Final diagnosis:
• Diabetes Mellitus Type 2
• Progressive supranuclear palsy

31. Management
• Counseling
• Balanced diet
• Tab Metformin
• Tab. Ropirinole
• Physiotherapy
• Rehabilitation specialist consultation
• Speech therapy
• Consultation with opthalmologist

32. Follow up
Patient visited us after 2 months with improvement
of postural instability & reduced frequency of falls

33. Progressive supranuclear palsy
(PSP)

34. Progressive supranuclear palsy
• Progressive supranuclear palsy (PSP), also
known as Steele-Richardson-Olszewski
syndrome, is a neurodegenerative disease that
affects cognition, eye movements, and posture.
• It was first described as a clinicopathologic
entity in 1964.

35. Pathologically, PSP is defined by the
accumulation of neurofibrillary
tangles in the brain. Different rates,
localizations, and patterns of the
accumulation of phosphorylated tau
protein may account for the variation
in clinical phenomena seen in
patients with PSP.

36. Pathology
Bilateral loss of neurons and gliosis in the
periaqueductal gray matter, superior colliculus,
subthalamic nucleus, red nucleus, pallidum, dentate
nucleus, and pretectal and vestibular nuclei, and to
some extent in the oculomotor nucleus.

37. Etiology
The cause of PSP remains
unknown. Most cases
appear to be sporadic.
Both environmental and
genetic influences have
been postulated.

38. History
The cardinal manifestations:
• Supranuclear ophthalmoplegia
• Pseudobulbar palsy
• Prominent neck dystonia
• Parkinsonism
• Behavioral, cognitive, and gait disturbances that
cause imbalance
• Frequent falls/impaired postural reflexes

39. History
• Focal or segmental dystonia in the form of
limb dystonia or blepharospasm
• Micturition disturbances, including urinary
incontinence
• Progressive apraxia of speech, nonfluent
aphasia, or a combination thereof
• Photophobia

40. Physical examination
• Poor postural reflexes, axial rigidity greater than
appendicular rigidity, and dysarthria
• Absence of cogwheeling or tremor
• Widely based and unstable gait
• Bradykinesia with masked facies and a startled
expression
• Retrocollis may be present

41. Visual signs
• Supranuclear ophthalmoplegia
• Downgaze typically involved
before upgaze
• Impairment of convergence eye
movements
• Eyelid retraction, eyelid opening
or closing apraxia,
blepharospasm, or lid lag

42. Diagnostic Considerations
The diagnosis of progressive supranuclear palsy
(PSP) is clinical. The key features typically develop
over time; although the full-blown picture may be
relatively easy to recognize, the early or restricted
cases are much more challenging.

43. Diagnostic aids
Atrophy of the dorsal
mesencephalon (superior
colliculi, red nuclei) giving
rise to a "mouse ears"

44. Diagnostic aids
CSF contains both extended and truncated
tau forms, and the truncated-to-extended
ratio is significantly lower in PSP than in
other neurodegenerative disorders

45. Management
• Treatment of progressive
supranuclear palsy (PSP) is
challenging at best.
• No medication is effective
• Dopamine agonists,
tricyclic antidepressants,
may provide modest
symptomatic improvement.

46. Management
• Only a few patients
respond to dopaminergic
or anticholinergic drugs,
and responses often are
short-lived and
incomplete.
• Electroconvulsive therapy
(ECT) may ameliorate
motor symptoms in some
patients with PSP.

47. Management
• Rehabilitation
• Treatment of the sleep
difficulties
• Observing the decline of
these patients and the
limitations of treatment is a
frustrating ordeal for all
involved.

48. Thank you