Atypical parkinsonism is a group of neurodegenerative disorders where parkinsonism is a prominent feature but differs from IPD by associated atypical features. References-Harrison textbook of Internal medicine,Various sourcres

1. PROGRESSIVE
SUPRANUCLEAR PALSY
-MRI SPOTTER
(WITH EXTRA SPOTTERS)
Dr KANNAN NARAYANAN
JR,MD GENERAL MEDICINE
KMC

2. • 65 yr old male patient with presented with c/o
• slowness of movement which was symmetrical in onset- one year
• frequent falls - 6 months

3. • No h/o tremors
• No h/o bowel and bladder involvement
• No h/o any drug intake
• No h/o loss of weight;decreased food intake
• No h/o gain in weight;lethargy;fatigue;intolerance to cold
• No h/o dificulty in swallowing

4. Examination
• No Palor/icterus/cyanosis/clubbing/ln/edema

6. Procerus sign

7. Nervous system examination
• HMF-Alert
• oriented in time,place,person
• Good attention
• Speech-slow monotonous speech
• CN examination- Olfactory and optic nerve normal

8. • Ptosis left eye
• Pupil -normal
• Vertical gaze palsy
• Hypometric saccades
• No nystagmus

9. • Other cranial nerves normal

10. • Motor system
• Bulk -normal and equal in both upper and lower limbs
• Tone- bilateral increase in rigidity in upper limb-leadpipe rigidity
• Power- grade 5 in all limbs
• Reflexes-normal
• Sensory system-normal
• No Cerebellar signs

11. • Other systems- normal

12. • MRI brain was taken

15. • MICKEY MOUSE SIGN
• Reduced AP
midlinemidbrain diameter,
at the level of the sc on
axial imaging (from
interpeduncular fossa to
the intercollicular groove:
<12 mm.

16. • MORNING GLORY SIGN

18. • There is a flattening or
concave outline to the
superior aspect of the
midbrain which should
be upwardly convex.

19. • Useful indices
• Midbrain to pons area ratio-reduced area ratio on the midline sagittal
plane to approximately 0.12 (normal ~ 0.24)

20. PARKINSONISM
• Refers to hypokinetic movement disorder
• Characterised by bradykinesia,rigidity,resting tremor and postural
instability

21. PARKINSONISM
• Idiopathic PD
• Atypical (parkinson plus syndromes)
• Heredodegenerative parkinsonism
• Secondary parkinsonism- Drugs,Metabolic conditions like Wilsons
d/s,Whipples d/s,Manganese toxicity,Hypothyroidism.

22. • Idiopathic Parkinsons d/s
• Age of onset-after 50 years
• Assymetrical- Rigidity
Tremor-resting tremor is more prominent
Hypokinesia
Postural instability-Later stage
PD

23. INCLUSION CRITERIA SUPPORTIVE CRITERIA EXCLUSION OF ATYPICAL FEATURES
Bradykinesia
+
one of
• Rigidity
• Rest tremor
• Postural instability
• Assymetry at onset
• Remains asymmetric
• Rest tremor
• Slowly progressive
• Respond to levo dopa
• Symmetrical onset
• Rapid progression
• Early frequent falls
• Early dementia
• Early autonomic symptoms
THE UK PARKINSON'S DISEASE
SOCIETY CRITERIA FOR
PARKINSON'S DISEASE

24. PARKINSONS PLUS SYNDROME/ATYPICAL
PARKINSONIAN SYNDROME
• Group of neurodegenerative disorders where parkinsonism is a
prominent feature but differs from IPD by associated atypical
features.
• PSP
• CBD
• LB
• MSA

25. • In initial stage of Parkinson plus syndrome it may be misdiagnosed as
IPD
• It is important to differentiate b/w these two, because Parkinson plus
syndrome is rapidly progressive and difficult to treat

26. When to suspect Parkinson plus syndrome
• Rapid progression
• Symmetrical onset
• Absence of tremor
• Postural instability,gait disturbance and frequent falls in early course of
disease
• Ataxia and cerebellar signs
• Oculomotor abnormalities
• Early autonomic instability
• Poor response to levo-dopa
• Apraxia,alien limb phenomenon,myoclonus

27. PSP-Progressive supranuclear palsy
• Onset-60-65 yrs
• Atypical parkinsonism/Parkinsons plus syndrome
• Slow ocular saccades,eyelid apraxia,restricted vertical eye
movements(particularly downgaze)
• Hyperextension of neck with early gait disturbances and frequent falls
• Speech and difficulty in swallowing in later stages

28. PSP
• Subcortical dementia
• Taupathy-tau deposition in basal ganglia,dentate,pontine and
oculomotor nuclei sparing cortex
• Associated marked degeneration of midbrain,subthalamic
nucles,superior cerebellar peduncle

29. PSP
• No/little response to l-dopa
• 2 clinical forms of PSP described
• PSP-p form- PSP parkinson form- closely resembles PD in early stages
including a positive response to levodopa
• PSP-R form-Richardson form-classic
• Also known as Steel-Richardson-Olschewski syndrome

30. Diagnosis -PSP
Clinical features Clinical signs Radiological signs
Parkinsonism Procerus sign Hummingbird sign
Vertical gaze palsy Rocket sign Mickey mouse sign
Gait instability Mona Lisa stare Morning Glory sign
Frequent falls Applause sign
Dysarthria and dysphagia-later
stages

32. Hot cross bun sign
• The hot cross bun sign refers to the MRI appearance of the pons
when T2 hyperintensity forms a cross on axial images, representing
selective degeneration of pontocerebellar tracts in a cruciform
pattern.
• Seen in multiple system atrophy

33. • MSA is a neurodegenerative disorder characterised by L-dopa
unresponsive Parkinsonism and/or cerebellar ataxia along with
autonomic dysfunction

34. EYE OF TIGER APPEARANCE

35. EYE OF TIGER APPEARANCE

36. EYE OF TIGER APPEARANCE
• This sign is seen in Hallevorden-Spatz syndrome - neurodegeneration
with brain iron accumulation or panthothenate kinase II associated
neurodegeneration.
• There is a low signal intensity surrounding a central small hyperintense
area in globus pallidus producing this appearance
• low intense signal- excessive iron accumulation
• central high intense signal-gliosis and neuronal loss

38. ELEPHANT SIGN
• Atrophy of the medial aspect of the temporal lobes and hippocampi.
• Characteristically seen in Alzheimer’s disease.
• Seen in diseases where there is atrophy of medial temporal lobe.

39. Dural tail sign

40. Dural tail sign
• Represents thickening and enhancement of the dura mater in
continuity with a mass, which on MR images, gives the appearance of a
tail arising from the mass.
• Traditionally considered as highly specific for meningioma.
• Seen only in 60% of meningiomas
• Also known as ‘Mouse tail sign’

41. Dural tail sign
• Also reported in
• Chloromas
• primary CNS lymphomas
• sarcoidosis
• schwannomas
• metastases

42. Dural tail sign
• Three criteria need to be met for a ‘positive’ dural tail sign
• The tail should be seen on two successive images through the tumor
• It should taper away from the tumor
• It must enhance more than the tumor.

43. Hyperdense MCA sign

44. Hyperdense MCA sign
• Hyperdense middle cerebral artery (MCA) seen on nonenhanced CT
scan images in acute stroke.
• Due to acute clot formation.
• The sign is said to be seen usually within approximately 90 min of
the event.
• High specificity of almost 100% but a very low sensitivity of 30% in
the diagnosis of acute stroke.

45. STRIPE SIGN/ TIGROID PATTERN/LEOPARD SKIN
APPEARANCE
• Seen as linear hypointensities radiating from the ventricular margins
within hyperintense periventricular white matter and the centrum
semiovale on T2W MRI images
• The sign represents a specific pattern of demyelination, with sparing
of perivascular white matter.

46. • The spared perivascular white matter is seen as dark spots or dark
linear areas against a background of bright affected white matter,
giving the appearance the skin of a leopard.

47. • Seen in metachromatic leukodystrophy(MLD)
• MLD- Autosomal recessive disorder due to the deficiency of the
lysosomal enzyme arylsulfatase.

49. BLEND SIGN

50. BLEND SIGN
• Within a cerebral hematoma there is blending of hypoattenuating and
hyperattenuating region with well defined margin in between the two
areas
• Blood clots within hematoma appears as hyperattenuating
lesion(darker).
• Hypoattenuating lesion-s/o more blood/active bleeding
• Blend sign predicts the hematoma growth/expansion.

51. CT ANGIO “SPOT SIGN”

52. CT ANGIO “SPOT SIGN”
• Unifocal or multifocal enhancemnt within an IC bleed visible on CT
angiography which is discontinuos from adjacent normal blood
vessels.
• It corresponds to a site of active hemorrhage.
• This sign is an independent predictor of hematoma growth and poor
outcome.

54. EMPTY DELTA SIGN
• This sign is created by a nonenhancing thrombus in the dural sinus
surrounded by triangular enhancing dura as seen on cross-
section,seen on contrast-enhanced CT scan images s/o dural
sinovenous thrombosis.
• It is a reliable sign of sinus thrombosis but seen only in 25-30% cases.

55. TRIANGLE SIGN AND CORD SIGN
• Signs of sinus thrombosis on nonenhanced CT scan.
• Both these signs represent clotted blood with SSS (triangle sign) and
cortical superficial veins (Cord sign).

56. ECCENTRIC TARGET SIGN

57. ECCENTRIC TARGET SIGN
• Central enhancing core of the target is due to the leash of inflamed
vessels extending down the length of the sulcus that is surrounded
by concentric zones of necrosis,with impaired permeability
producing the peripheral enhancing rim.
• Seen in cerebral toxoplasmosis.

59. Medusa head sign
• Medusa head sign is seen in a developmental venous anomaly
(venous angioma), where multiple tributaries arranged in a radial
fashion drain into a larger vein.
• Best seen in T1W contrast MRI.
• Also seen in ct angiography.

60. Reversal sign

61. Reversal sign
• seen on CT scan images.
• diffuse decrease in the density of the cerebral hemispheres, with
loss of gray-white differentiation and a relative increase in the
density of the thalami, brainstem, and cerebellum.
• It is also known as the ‘white cerebellum sign

62. Reversal sign
• seen in severe head injury
• status epilepticus
• bacterial meningitis
• encephalitis
• birth asphyxia
• drowning
• Represents anoxic/ischemic cerbral injury

63. RADIAL BAND SIGN

64. RADIAL BANDS SIGN
• Seen in tuberous sclerosis.
• Sign refers to linear bands seen on MRI,radiating from periventricular
white matter to the subcortical region.
• Hyperintense on T2W
• Hypointense onT1W
• Represents abnormal migration of dysplastic stem cells along the
course of the radial glial neuronal unit

66. POPCORN OR MULBERRY APPEARANCE
• Seen in cavernous angioma or cavernoma
• Well circumscribed lobulated lesion with a reticulared core of
heterogenous signal intensity on both t1 and t2w.
• Blooming artifact is seen.

67. Dawsons fingers

68. DAWSONS FINGERS
• Seen in MS MRI imaging
• Related to white matter demyelination that occur ariund the
perimedullary vein.
• These are ovoid lesions with longest axis perpendicular to corpus
callosum.

69. Dawsons fingers

71. Molar tooth sign
• Seen in Jouberts syndrome
• Characteristic MRI appearance of brainstem which results from
cerebellar vermis hypoplasia ,thick and maloriented superior
cerebellar peduncles.

72. BOX CAR VENTRICLE SIGN
• Seen in Huntingtons disease
• D/t the degeneration of cells of caudate nucleus in HD which results
atrophy of caudate nucleus and lateral ventricle becomes square
shaped when viewed on axial and coronal sections.

74. Racing car sign
• Seen in corpus callosum agenesis resulting in widely spaced lateral
ventricles.

75. FIGURE OF EIGHT SIGN
• Lissencephaly;absence of sulci gyri pattern; a/k/a hourglass appearance

76. TRAM TRACK CALCIFICATION

77. TRAM TRACK CALCIFICATION
• Sturge-Weber syndrome (encephalo-trigeminal angiomatosis)
• U/l facial nevus (portwine stain) with ipsilateral intracranial gyral
pattern curvilinear calcifications
• m/c in occipital and posterior parietal lobe.

78. TRAM TRACK SIGN IN OPTIC NERVE
• Seen in optic nerve sheath meningioma.
• This sign is composed of two enhancing areas of tumor seperated
from each other by the negative defect of the optic nerve on contrast
MRI images.
• This sign helps to distinguish between optic nerve sheath
meningioma and optic glioma.Optic glioma arises from glial cells
within the optic nerve and there is no clear separation b/w nerve and
tumor.

80. Mount Fuji Sign
• Seen in pneumocephalus

81. STARRY SKY APPEARANCE
Classically described in Disseminated neurocysticercosis.

82. CORTICAL RIBBON SIGN

83. CORTICAL RIBBON SIGN
• Seen in sporadic Creutzfeldt-Jakob disease
• Hyperintensities in the cortical gray matter giving appearance like a
ribbon.
• D/t spongiform degeneration of brain

84. INSULAR RIBBON SIGN
• Insular ribbon is an area of grey white differentiation that is seen on
CT brain b/w the sylvian fissure and the basal ganglia
• Supplied by small perforating branches of the MCA
• Loss of the insular stripe is one of the earliest sign of MCA stroke.
• Ischemia results in intracellular edema of grey matter resulting in
hypodensity.

86. “PUFF” OF SMOKE/MOYA MOYA APEARANCE
• Represents angiographic appearance of basal telangectasias,which
consist of dilated collateral branches of the lenticulostriate and
thalamostriate arteries;usually seen in association with internal
carotid artery stenosis.
• Idiopathic ICA stenosis-Moya moya d/s
• When secondary to some other d/s-moya moya syndrome

87. • Moya moya syndrome is caused by
• Neurofibromatosis
• sickle cell disease
• TB meningitis
• Polyarteritis nodosa

89. • THANKS