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MembersMeets
UCLH Education Centre
London
14th May 2018
ATAXIA: conditions that affect co-ordination,
balance and speech
Prof Paola Giunti
Ataxia Centre, Institute of Neurology
UCL/UCLH
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Ataxia, definition, causes, epidemiology,
symptoms
Ataxia centre as a model of care in
partnership with patients
Research Projects at the Ataxia centre Dr. Nita Solanky, Research Coordinator
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Means « lack of order, coordination »
• Loss of balance with unsteady, irregular,
wide-based gait with swaying and risk of
falls
• Limb movements are irregular, fragmented,
tremulous.
• Eye movements are interrupted, not
calibrated, fixation maybe unstable with or
without double vision
• Speech is slurred, in speed and volume
• Tremor in the hands or legs
ATAXIA
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Inherited ataxias
–Hereditary ataxias may be
congenital, episodic, or progressive
–Inheritance may be autosomal
dominant, autosomal recessive, X-
linked and mitochondrial
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Other forms of ataxia
–Vascular, Inflammatory (eg: multiple
sclerosis)
–Traumatic Metabolic, Developmental,
–Toxic / drug-related (eg: alcohol)
–Neoplastic / paraneoplastic Epilepsy
(in children) Infectious
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Treatable Ataxias
• Gluten Ataxia
• Vitamin E deficiency
• Vitamin B12 deficiency
• CoQ10 (ubiquinone) deficiency
• Cerebrotendinous xanthomatosis
• Niemann-Pick type C (NPC)
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Treatable Ataxias Mostly in Children
Glucose transporter 1 deficiency
Hypobetalipoproteinaemia
Hartnup disease
Biotinidase deficiency
Pyruvate dehydrogenase deficiency
Structural disorders
Acute encephalopathis
Non-convulsive status epilepticus/ other
epilepsies
Sensory ataxias
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Urology
Blurred vision
Dysphagia Myoclonus
Dystonia
Spasticity
Tremor
Cognitive decline Bowel & Bladder
Imbalance
Muscle spasms
Cardio
Slurred speech
Loss of coordination
Common symptoms in different types of Ataxia
Pain
Parkinsonism
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Patients clinical pathway
Ataxic Patients
Ataxia Centre
Multidisciplinary
clinical setting
Research (descriptive,
experimental,
interventional)
Translational
Research
Clinical
Excellence
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The Ataxia Centre
…a truly multidisciplinary approach to
management
The first Ataxia UK Accredited Ataxia
Centre in the UK 2005
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Three Accredited Centres in the UK
• Partnership to
develop Centres
• Funding
• Ongoing
communications
• Volunteer scheme
London
Sheffield
Newcastle
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SPECIALIST ATAXIA CLINIC Diagnosis and symptomatic treatment
Improved service
Improved levels of support
Expanding research potential
Start date: February 2005
Received Accreditation in November 2005 by Ataxia UK as first operating Ataxia Centre of Excellence in UK
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• Continuity of care - the same neurologist
every visit.
• One hour for new patients, 30mins for
follow up.
• Once weekly clinic.
• Referrals from neurologists and GPs.
…a truly multidisciplinary approach
to management !
The Ataxia Centre
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The Ataxia Centre
…a truly multidisciplinary approach to
management !
• Support both during and between clinic visits
via the neurogenetic nurse. This nurse is
available to discuss medical, social or financial
queries.
• Close links with primary, community and
secondary care services for shared care and
education on the ataxias.
• Establishment of an ataxia database, facilitating
research.
• Validation of new genetic tests as they emerge.
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The Ataxia Centre …a truly multidisciplinary approach to management!
Related Specialties….
Named consultants for: ENT, Cardiology, Neuro-ophthalmology,
Orthopedics, Neuro-psychiatry, Neuro-Psychology, Neuro-urology,
Neuro-electrophysiologist
Establishment of a holistic interdisciplinary clinic…
Where patients see Physiotherapists, Occupational Therapists and
Speech and Language Therapists and Neurologists in one clinic for a
personalised management programme
Other team members…
Neurogenetics specialist nurse, research nurse, research fellow, Ataxia
UK representative
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PG203-PG204
Clinic Frequency
Feb 2005 1/ month
Oct 2005 2/ month
May 2006 1/ week
July 2013 2/ week
Patients seen per year
Feb 05/Jan06 62 patients
Feb 06/Jan07 170 patients
Feb07/Jan 08 229 patients
Feb 08/Jan 09 263 patients
Feb 09/Jan 10 300 patients
Feb 10/Jan11 420 patients
Feb 12/Jan13 540 patients
Feb 13/Jan14 630 patients
Feb 14/Jan 15 749 patients
Feb 15/Jan 16 818 patients
Success of the Ataxia clinic!
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90.8%
1.8% 1.5%
0.7%
Is it beneficial to attend an Ataxia specific clinic?
Yes
No
Fairly
Not Sure
Patient Feedback 2010-2017
Data from surveys compiled by Ataxia UK
clinic evaluation Sep 2010- now
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.
Yes 902 92.1%
No 1 0.1%
Fairly 12 1.2%
Incomplete Form/ NA 64 6.5%
No 23 2.8%
Yes 136 16.6%
Fairly 0 0.0%
Incomplete Form/ NA 659 80.6%
Patient Feedback 2010-2017
Data from surveys compiled by Ataxia UK
clinic evaluation Sep 2010- now
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45.0%
50.3%
4.7%
Do you feel that you have received support and advice
between clinic appointments?
I haven't needed help
Yes I received advicewhen I needed it
No, I didn't receiveadvice when Ineeded it
I haven’t needed help 219 45.0%
Yes I received advice when 245 50.3%
I needed it
No, I didn’t receive advice 23 4.7%
when I needed it
397
26
Is the Care at this Clinic an
Improvement on Previous Care?
Improvement
Not an Improvement
Improvement 397 93.9%
Not an Improvement 26 6.1%
Patient Feedback 2010-2017
Data from surveys compiled by Ataxia UK
clinic evaluation Sep 2010- now
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Reasons Patients Prefer Ataxia Clinics
0.00%
10.00%
20.00%
30.00%
40.00%
50.00%
60.00%
70.00%
80.00%
90.00%
100.00%
1 2 3 4 5 6 7 8 9
1 - Understanding of
Ataxia
2 - Confidence in
Neurologist
3 - Medical Advice on
Symptom Management
4 - Better able to Cope
5 - Precise Diagnosis
6 - Information about
Research
7 - Practical Advice about
Living with your
Condition
8 - Other
Data from response forms of 800 patients seen over the last
10 years
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Success of Ataxia Centre/s
Improved care - 95%
improvement compared
to other clinics Data: London Ataxia Centre 2005- 2017
Research focus Large database
Expertise
Networks
Ataxia Guidelines of
best practice
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Ataxia Centre multidisciplinary approach for
patients with rare and chronic conditions,
brings
• Quicker diagnosis
• Precise diagnosis
• More effective
management
• Cost reduction
• Improved patient
satisfaction
All this is due
to our tight
involvement in
research
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Referral to Ataxia Centre
https://www.uclh.nhs.uk/HP/Howtorefer/Pages/Home
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Support from the clinical team at the London ataxia centre…
Paralympic glory for NHS patient
THE LONDON ATAXIA PATIENTS ACHEIVEMENTS
Ain't no mountain high
enough for Ataxia patient