Wilson's disease is a rare genetic disorder that causes copper to accumulate in the brain and other vital organs. MRI plays an important role in evaluating patients with suspected Wilson's disease. Key findings on MRI include signal changes in the basal ganglia, midbrain, pons, and cerebral white matter. Characteristic signs include T1 hyperintensity of the globus pallidus, the "face of the giant panda" sign, and CPM-like changes resembling osmotic demyelination. Serial MRI can show improvement in many patients responding to treatment, while extensive changes correlate with poorer prognosis. Advanced techniques like DTI reveal more widespread white matter abnormalities than conventional MRI and help assess disease severity.
Kapan aneurysma yang belum ruptur memerlukan intervensi?
"In the decision-making process, the PHASES score may be considered for predicting a patient’s risk of aneurysm rupture."
Diffusion-weighted imaging or computerized tomography perfusion assessment with clinical mismatch in the triage of wake up and late presenting strokes undergoing neurointervention with Trevo (DAWN) trial methods
Int J Stroke. 2017 Aug;12(6):641-652.
Thrombectomy 6 to 24 Hours after Stroke with a Mismatch between Deficit and Infarct
N Engl J Med. 2018 Jan 4;378(1):11-21.
A multicenter randomized controlled trial of endovascular therapy following imaging evaluation for ischemic stroke (DEFUSE 3)
Int J Stroke. 2017 Oct;12(8):896-905.
Thrombectomy for Stroke at 6 to 16 Hours with Selection by Perfusion Imaging
N Engl J Med. 2018 Feb 22;378(8):708-718.
This presentation starts with a description of what is MRI and how it generates an image. I feel that MRI is the investigation of our era and the applications will only grow, perhaps in some cases will drastically alter treatment protocols, being ignorant about the physics of MRI would be a crime!
I then introduce the concepts behind the common cardiac use cases. Cardiac MRI by itself is a huge topic, this presentation aims to prepare a base to understand this huge pandora's box.
Kapan aneurysma yang belum ruptur memerlukan intervensi?
"In the decision-making process, the PHASES score may be considered for predicting a patient’s risk of aneurysm rupture."
Diffusion-weighted imaging or computerized tomography perfusion assessment with clinical mismatch in the triage of wake up and late presenting strokes undergoing neurointervention with Trevo (DAWN) trial methods
Int J Stroke. 2017 Aug;12(6):641-652.
Thrombectomy 6 to 24 Hours after Stroke with a Mismatch between Deficit and Infarct
N Engl J Med. 2018 Jan 4;378(1):11-21.
A multicenter randomized controlled trial of endovascular therapy following imaging evaluation for ischemic stroke (DEFUSE 3)
Int J Stroke. 2017 Oct;12(8):896-905.
Thrombectomy for Stroke at 6 to 16 Hours with Selection by Perfusion Imaging
N Engl J Med. 2018 Feb 22;378(8):708-718.
This presentation starts with a description of what is MRI and how it generates an image. I feel that MRI is the investigation of our era and the applications will only grow, perhaps in some cases will drastically alter treatment protocols, being ignorant about the physics of MRI would be a crime!
I then introduce the concepts behind the common cardiac use cases. Cardiac MRI by itself is a huge topic, this presentation aims to prepare a base to understand this huge pandora's box.
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In my opinion this presentation is a road map for beginars.
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MRI in D.D. of MS
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Anatomy of Brain by MRI
In this presentation we will discuss the cross sectional anatomy of brain. Then we will discuss the Most common diseases to be evaluated by brain imaging.
In my opinion this presentation is a road map for beginars.
A 2019 update on the current role of robotics and simulation in neurosurgery with updates from the recent edition of Youman and Winn's Textbook of Neurosurgery. Videos in the presentation cannot be uploaded but can be viewed from youtube.
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Presentation Summary: Normal Cranial Development (Anatomy and Genetic), Imaging Technique (how to do 3D CT, when to do MRI, why to do not do Plain Film), Imaging Patterns of Craniosynostosis, Associated Complications, Pitfalls.
Neuroradiology in multiple sclerosis
MRI in diagnosis of MS
MRI in D.D. of MS
MRI in monitoring disease progression and response to DMT
New imaging techniques
Vertebral osteomyelitis( spondylodiskitis )
usually seen in adults (median age is 50 to 60 years)
Location
50-60% of cases occur in lumbar spine
30-40% in thoracic spine
~10% in cervical spine
the presentation gives a detail information about the seronegative spondyloarthropathy. this ppt also provide recent evidences to frame the rehab protocol.
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it describes the bony anatomy including the femoral head , acetabulum, labrum . also discusses the capsule , ligaments . muscle that act on the hip joint and the range of motion are outlined. factors affecting hip joint stability and weight transmission through the joint are summarized.
This slide is special for master students (MIBS & MIFB) in UUM. Also useful for readers who are interested in the topic of contemporary Islamic banking.
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Dear Dr. Kornbluth and Mr. Gorenberg,
The US House of Representatives is deeply concerned by ongoing and pervasive acts of antisemitic
harassment and intimidation at the Massachusetts Institute of Technology (MIT). Failing to act decisively to ensure a safe learning environment for all students would be a grave dereliction of your responsibilities as President of MIT and Chair of the MIT Corporation.
This Congress will not stand idly by and allow an environment hostile to Jewish students to persist. The House believes that your institution is in violation of Title VI of the Civil Rights Act, and the inability or
unwillingness to rectify this violation through action requires accountability.
Postsecondary education is a unique opportunity for students to learn and have their ideas and beliefs challenged. However, universities receiving hundreds of millions of federal funds annually have denied
students that opportunity and have been hijacked to become venues for the promotion of terrorism, antisemitic harassment and intimidation, unlawful encampments, and in some cases, assaults and riots.
The House of Representatives will not countenance the use of federal funds to indoctrinate students into hateful, antisemitic, anti-American supporters of terrorism. Investigations into campus antisemitism by the Committee on Education and the Workforce and the Committee on Ways and Means have been expanded into a Congress-wide probe across all relevant jurisdictions to address this national crisis. The undersigned Committees will conduct oversight into the use of federal funds at MIT and its learning environment under authorities granted to each Committee.
• The Committee on Education and the Workforce has been investigating your institution since December 7, 2023. The Committee has broad jurisdiction over postsecondary education, including its compliance with Title VI of the Civil Rights Act, campus safety concerns over disruptions to the learning environment, and the awarding of federal student aid under the Higher Education Act.
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A Strategic Approach: GenAI in EducationPeter Windle
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3. Wilson’s disease at NIMHANS
• Prof HS Swamy : Initiated in late 1970
• Specialized WD clinic: Every Wednesday
Neurologist, Social worker
Free care
• Funded & Non-funded Research Projects
Dissertation : 16
Presentation : 30
Peer- reviewed Publications: 34
• Corpus fund
• Registry of >700 patients with
125 – 150 on regular follow up
0
50
100
150
200
250
300
1970-79 1980-89 1990-99 2000-10
Series1
4. Williams and
Walshe, 1981
(n=60) %
Jha et al, 1998
(n=21) %
Present report,
2006 (n=116)
%
Cortical atrophy 63 9.5 44.8
Ventricular dilatation 73 19.0 44.0
Caudate atrophy NA 9.5 25.0
Brainstem atrophy 55 NA 31.9
Cerebellar atrophy 10 9.5 19.0
Hemispheric hypodensity NA 9.5 29.3
Basal ganglionic
hypodensities
NA NA 19.8
Thalamic hypodensities NA NA 10.3
Brainstem hypodensity NA 28.6 NA
Normal 18 14.3 NA
Taly AB, Meenakshi-Sundaram S, Sinha S, et al. Medicine 2007;82 (2): 112-119
CT Scan Observations in Wilson’s Disease
(n=116)
D Bhattacharya. Follow up study of CT scan brain and clinical correlation in Wilson’s disease.
Thesis submitted towards partial fulfillment for DM degree in Neurology, NIMHANS, Deemed University, (1997)
Putaminal, Pallidal &
White matter hypodenisty
6. Brain MRI changes in Wilson’s disease
• Globus Pallidum,
S. Nigra causes T1
hyperintensity
• Reason:
?Manganese
• A combination of
T2hyperIntensity &
T1hyperintensity is
highly suggestive
of WD
This variable appearance is probably due to
combination of necrosis, cystic changes, gliosis,
and copper accumulation
7. •One hundred patients (M:F::57:43, Age: 19.3±8.9 years) underwent MRI evaluation
•Atrophy: cerebrum (70%), brainstem (66%) and cerebellum (52%)
•Signal Changes: putamen (70%), caudate (61%), thalami (58%), midbrain (49%), pons
(20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%).
•Characteristic features: T2W globus pallidal hypointensity (34%), ‘Face of Giant panda’ sign
(12%), T1W striatal hyperintensity (6%), central pontine myelinosis (7%) and bright claustral
sign (4%) were also detected.
•Clinico-MRI correlation: MRI correlated with disease severity scores (p< 0.001) but did not
correlate with the duration
•MRI changes were diverse and universal in symptomatic patients and involved almost all
the structures of the brain
8. MRI observations in WD
Parameter Roh
(1994)
Wassenaer
(1996)
King
(1996)
Saatci
(1997)
Sinha et al
(2006)
Number of
Patients
25 50 (49 MRI) 25 30 100
Treatment status
Drug naïve 0(0%) 3(6%) 0(0%) NA 18(18%)
Abnormal MRI All(100%) NA 22(88%) 23(76.6%) 93(93%)
Signal intensity changes (%)
Putamen 68 36 86 85.7 72
Globus Pallidus 20 22 41.1 88.8 40
Thalamus 92 18 54 47.6 58
Caudate NA 8 45 42.8 61
White matter 4 22 59 NA 25
Midbrain 76 22 77 76.2 49
Pons 68 18 82 85.7 20
Medulla NA NA NA NA 12
Cerebellum NA 8 50 NA 10
Atrophy (%)
Diffuse/cerebral 88 39 80 100 70
Brainstem NA NA NA NA 66
Cerebellum NA NA NA NA 52
Sinha S, Taly AB, Ravishankar S et al. Neuroradiology; 2006; 48 (9): 614-621
15. MRI and Wilson’s Disease
Sinha S, Taly AB, Ravishankar S et al. Neuroradiology; 2006; 48 (9): 614-621
Face of
Giant Panda
Bright
Claustrum
CPM like
T1W
HyperIntensity
19. Diffusion Restriction in WD
Internal capsule, G. pallidus Pons: CPM like
• Restricted Diffusion: Correspond to restriction of mobility of water molecules
and indicates the presence of cytotoxic edema (acute ischemia and infarct).
• In WD: Excess copper causes cell injury leading to inflammation & cell death
- represented cell swelling associated with inflammation, hence restriction of
diffusion
20.
21. CPM-like changes in Wilson’s Disease
Sinha S, Taly AB, Ravishankar S et al. J Neuroimaging 2007; 17:286-291
22. CPM in osmotic demyelination Vs WD
• CPM- like changes in WD share some similarity
with CPM secondary to ‘osmotic demyelination’.
• But, CPM- like changes in WD might differ in
certain other aspects like
– a) occurs in the setting of a chronic disease without any
obvious evidence of sodium imbalance,
– b) it is almost always contiguous with midbrain signal
changes mainly of tectal region,
– c) has two additional but distinct ‘bisected’ and
‘trisected’ patterns,
– d) infrequent occurrence of EPM.
23. Sinha S, Taly AB, Ravishankar S et al. J Neuroimaging 2007; 17:286-291
CPM-like changes in
Wilson’s Disease
24. MRI correlates of Neuropsychological deficits in Wilson’s Disease
(n=12)
• Tools
NIMHANS Neuropsychology Battery (2004): Administered with norms
considering age, education & gender
• Observations
Universal and variable deficits in domains of motor speed, sustained attention,
executive functions- in working memory, verbal fluency, set-shifting ability,
verbal learning & visual memory, information processing & encoding
• Putative Substrate
Frontal-subcortical & Frontal lobe involvement
Temporal lobe involvement: Rare
Hegde S, Sinha S, Rao S, Taly AB. Cognitive evaluation in Wilson’s disease. Neurology India 2010; 58(5): 708-713
25. Seizures in Wilson’s disease: Magnetic Resonance Imaging
(n=11)
Prashanth LK, Sinha , Taly AB. Seizures in Wilson’s Disease. J Neurol Sci 2010; 291:44-51
14 year girl
with 2 years
h/o WD:
Recurrent
seizures -
response to
de-coppering
agents & AEDs
unsatisfactory.
26. Wilson’s disease: MR spectroscopy and Clinical correlation
H MRS
P MRS
Forty patients & 30
controls underwent in-
vivo 2-D 31P and 1H
MRS of basal-ganglia
using an image-selected
technique.
There was reduced
breakdown and/or
increased synthesis of
membrane
phospholipids and
increased neuronal
damage in basal ganglia
in patients with WD
Sinha S, Taly AB, Ravishankar S, et al. Wilson’s disease: 31P and 1H MRS. Neuroradiology 2010;52(11):977-85
27. • We evaluated white matter (WM) abnormalities in 15 patients with drug naïve Wilson's
disease (WD) and 15 controls using the technique of diffusion tensor imaging (DTI).
• Fractional anisotropy (FA) and mean diffusivity (MD) values were analyzed
• Six patients showed lobar WM signal changes on T2-Weighted (T2W)/ Fluid attenuation
inversion recovery (FLAIR) images while remaining had normal appearing WM.
• MD was significantly increased in the lobar WM, bilateral IC and midbrain of WD
patients. FA was decreased in the frontal and occipital WM, bilateral IC, midbrain and
pons.
• Normal-appearing white matter on FLAIR images showed significantly increased MD and
decreased FA values in both frontal and occipital lobar WM and IC compared with those
in controls.
• Correlation of clinical scores and DTI metrics revealed positive correlation between
neurological symptom score (NSS) and MD of anterior limb of right internal capsule, Chu
stage and MD of frontal and occipital WM.
• Negative correlation was observed between the Modified Schwab and England Activities
of Daily Living (MSEADL) score and MD of bilateral frontal and occipital WM and IC.
• Conclusions: This is the probably the first study to reveal widespread
alterations in WM by DTI metrics in drug naïve WD. DTI analysis revealed
lobar WM abnormalities which is less frequently noted on conventional MRI
and suggests widespread WM abnormalities in WD. It may be valuable in
assessing the true extent of involvement and therefore the severity of the
illness.
28. • We evaluated the usefulness of Diffusion Tensor Imaging (DTI) metrics in
confirmed patients with Wilson’s disease (WD) who are either drug naïve
(n=15) or on de-coppering therapy (n=15) and healthy control (n=15).
Diffusion weighted & tensor imaging (DWI / DTI) in WD
FA maps (b=800 s/mm2 images) shows
ROI in white matter, basal ganglia,
thalamus, and midbrain regions
Mean diffusivity (MD) maps (b=800 s/mm2 images) shows ROI
in cerebral white matter, basal ganglia, thalamus, midbrain,
pontine and cerebellar white matter regions
• First comprehensive report of DTI findings in patients with Wilson’s disease.
• Abnormalities in DTI findings (high MD and FA) were noted in WD compared
to controls and more so in drug-naïve patients
• DTI showed additional tissue abnormalities in WD in various regions of brain
where conventional MRI sequences were normal.
• Differential involvement and variable degree of phenotypic-DTI correlation
was noted Jadav R, Saini J, Sinha S, et al Metabolic brain disease 2013; 28:455-62
29. Role of imaging in following patients
• Clinical & MRI improvement pari-passu in
most patients with neuropsychiatric form
• Liver involvement: Additional clue
• Newer tools - DTI metrics & MRS:
improving understanding at microstructural
levels
• Extensive MRI (+WM) changes: Helps in
prognostication
Sinha S, Taly AB, Prashanth LK et al. BJR 2007; 80:744-749
Lawrence et al, JIMD reports. 2016 vol 25: pp 31-38
30. Wilson’s disease (WD) is clinically & radiologically a dynamic disorder: 50 patients
were recruited prospectively for this study to evaluate the serial MRI and clinical
changes
Serial imaging: Improvement in MRI parameters - 35, No significant changes - 10,
Worsening - 4 and An admixture of resolving and evolving changes - 1.
MRI score improved from 8.2±5.7 to 5.9±6.6.
Patients with extensive changes, white-matter involvement and severe diffuse
atrophy had a poor prognosis
Conclusions: Majority of patients of WD on treatment showed variable
improvement in clinical and MRI features
31. Sinha S, Taly AB, Prashanth LK et al. BJR 2007; 80:744-749
Serial MRI and Wilson’s Disease
Improvement Improvement
Improvement Improvement
Worsening Differential
change
32. Objective: The purpose is to evaluate white matter (WM) abnormalities in 15 patients with Wilson's disease &
15 controls (WD) using the technique of diffusion tensor imaging (DTI).
Methods: DTI/conventional MRI was acquired (3T MRI): Fractional anisotropy (FA) and mean diffusivity (MD)
values were extracted from regions of interest placed in pons, midbrain, bilateral frontal and occipital cerebral
white matter, bilateral internal capsules (IC), middle cerebellar peduncles (MCP) and corpus callosum (CC).
Results: S
Six patients showed lobar WM signal changes on T2-Weighted (T2W)/ Fluid attenuation inversion recovery
(FLAIR) images while remaining had normal appearing WM. MD was significantly increased in the lobar WM,
bilateral IC and midbrain of WD patients.
FA was decreased in the frontal and occipital WM, bilateral IC, midbrain and pons. Normal-appearing white matter
on FLAIR images showed significantly increased MD and decreased FA values in both frontal and occipital lobar
WM and IC compared with those in controls.
Correlation of clinical scores and DTI metrics revealed positive correlation between neurological symptom score
(NSS) and MD of anterior limb of right internal capsule, Chu stage and MD of frontal and occipital WM. Negative
correlation was observed between the Modified Schwab and England Activities of Daily Living (MSEADL) score
and MD of bilateral frontal and occipital WM and IC.
Conclusions: This is the probably the first study to reveal widespread alterations in WM by
DTI metrics in drug naïve WD. DTI analysis revealed lobar WM abnormalities which is less
frequently noted on conventional MRI and suggests widespread WM abnormalities in WD. It
may be valuable in assessing the true extent of involvement and therefore the severity of the
illness.
33. WD (Neurological form) & DD:
How MRI can help?
• Helps to exclude mimickers
• Assists in deciding tests to exclude other
DD
• Some of the MRI findings: almost
pathognomonic of WD
35. A B C D
E F G H
Nagappa et al, JCoN 2016;27:91-94
36.
37. Imaging: Important observations
• CT scan: careful interpretation is essential and normal CT
scan do not exclude WD
• MRI: Definitely useful – often provide clue if not clinically
suspected
– Hepatic form: T1W pallidal hyperintensity
– Always abnormal in neurologically involved patients
– Extent & severity of changes: Protean
– Clinically severe form=extensive MRI changes
– Common: Putamen, thalami, caudate, midbrain, pons,
white matter
– Characteristics: Midbrain tectal change, CPM like
changes, Face of giant panda
38. Imaging: Important observations
– T1W pallidal hyperintensity: Liver
– Frontal white matter & adjacent cortical atrophy: Seizures (SE)
– CPM: 3 subtypes –“Mercedes Benz” sign is a novel observation
– Serial MRI: improves variably with decoppering in majority
– CPM like changes: different that those of osmotic demyelination
– Simultaneous involvement of basal ganglia, thalamus and
brainstem are virtually pathognomonic of WD.
– MR Spectroscopy: Evolving knowledge: Provide idea about
metabolites
– Diffusion tensor imaging: Additional areas and might help in
prognosis
– What is intriguing? Clinico-MRI discordance; Basis of
topographic preference; & Genetic-MRI correlation
39. HOPE
Thank you very much!Acknowledgment: Prof AB Taly
& colleagues/residents
& all the patients
Wilson’s disease Clinic
(Late Dr. HS Swamy- 1970s)