Autonomic & Neurovascular Medicine Unit, Faculty of Medicine, Imperial College London

Transcription

Autonomic & Neurovascular Medicine Unit, Faculty of Medicine, Imperial College London
Autonomic & Neurovascular Medicine Unit,
Faculty of Medicine, Imperial College London
at St Mary’s Hospital.
Autonomic Unit,
National Hospital for Neurology & Neurosurgery, Queen Square
& Institute of Neurology, University College London
The Autonomic Clinic - Why is it so special?
Dr Valeria Iodice, MD
Miss Catherine Best, RN
Dr David Low, PhD
The Autonomic Clinic
• 1. Postural Tachycardia Syndrome and
Syncope
– Dr Valeria Iodice, Clinical Research Fellow
• 2. Treatment Strategies
– Miss Catherine Best, Nurse Specialist
• 3. Clinical Research Aspects
– Dr David Low, Clinical Research Associate Fellow
Postural Tachycardia Syndrome
Aims of treatment
• Awareness and education
• Prevent collapse and near collapse
• Reduce the frequency and intensity of symptoms
• Improve quality of life
Postural Tachycardia Syndrome (PoTS) and
Autonomic (Neurally) Mediated Syncope
(AMS)
Dr Valeria Iodice, MD
Autonomic Neurovascular Clinical Team
Prof. C.J. Mathias
Clinical Director
Dr G. Ingle
Consultant Neurologist
Clinicians
SHO/Registrar
Clinical Fellows
1 Autonomic
Nurse Specialist
11 Clinical Autonomic
Scientists
4 Clinical
Secretaries
Overview
• PoTS and AMS definition
• Diagnosis/role of autonomic
evaluation
• Associations/allied features
Postural Tachycardia Syndrome (PoTS)
•
•
•
•
•
•
•
More females than males
25-40 years
Postural and exertional palpitations
Lightheadedness, fatigue, tremulousness
Syncope
Disproportionately disabled
Symptoms with heart rate rise of >30 beats/min
or >120 beats/min without orthostatic
hypotension
60 O Head up tilt
200
140
Normal
Heart Rate
bpm
Blood
Pressure
mmHg
(Portapres)
10 MIN OF 60°HEAD UP TILT
0
0
200
140
PoTS
10 MIN OF 60°HEAD UP TILT
0
0
Autonomic (Neurally) Mediated
Syncope
• Transient loss of consciousness in
response to a trigger
120
120
60°HEAD UP TILT
0
0
Triggers
• Orthostatic stress
• Blood phobia
• Exercise
• Food
• Hot environment
PoTS and AMS, what in common?
• Intermittent episodes of autonomic
dysfunction
OH
PoTS
AMS
Normotension
Bradycardia/hypotension
Orthostatic Tachycardia
Orthostatic Hypotension
Intermittent
autonomic
dysfunction
Fixed
autonomic
dysfunction
Diagnosis
• Clinical history
• Autonomic evaluation
- Autonomic screening tests
- Additional autonomic tests
60 O Head up tilt
60 O Head up tilt
200
140
Normal
Heart Rate
bpm
Blood
Pressure
mmHg
(Portapres)
10 MIN OF 60°HEAD UP TILT
0
0
200
140
PoTS
10 MIN OF 60°HEAD UP TILT
0
0
60 O Head up tilt
200
140
Normal
Heart Rate
bpm
Blood
Pressure
mmHg
(Portapres)
10 MIN OF 60°HEAD UP TILT
0
0
Vasovagal syncope
VENEPUNCTURE
120
120
140
60°HEAD UP TILT
0
160
60°HEAD UP TILT
0
0
0
Pressor Response
250
Cutaneous Cold
100
Normal
Heart Rate
bpm
Blood
Pressure
mmHg
(Portapres)
0
90 SECONDS
Isometric Exercise
140 SECONDS OF RIGHT ARM ISOMETRIC EXERCISE
0
Valsalva Manoeuvre
160
80
Normal
Heart Rate
bpm
Blood
Pressure
mmHg
(Portapres)
0
EXPIRATION 15 SECONDS
Deep Breathing
10 SECONDS OF DEEP BREATHING
0
Do we need additional
autonomic tests?
Modified Exercise testing
140
120
129
100
65
72
pre ex
stand
40
96
supine rest
Heart rate80
bpm 60
20
post ex
stand
supine rest
0
Liquid Meal challenge
Heart rate
bpm
Blood pressure
(mmHg)
60 O Head up tilt
Pre - meal
Post - meal
24 hour BP & HR profile in PoTS
180
180
STANDING
STANDING
AFTER
EXERCISE
STANDING
STANDING
STANDING
STANDING
Heart
rate
(bpm)
Blood
pressure
(mmHg)
LYING
LYING &
SITTING
SITTING
SITTING
Nocturnal Period
0
10:00
0
12:00
17:00
20:00
Time (hours)
00:00
07:00
10:00
Causes & Associations of PoTS
Partial peripheral
autonomic neuropathy
Noradrenaline
transporter
deficiency
Hypovolumeia
PoTS
Deconditioning
Panic attacks
Joint
hypermobility
syndrome
Mitral valve
prolapse
Chronic fatigue
syndrome
Migraine
Mast cell disorders
PoTS – AMS
A syndrome NOT a disease!
PoTS – AMS
Joint
Hypermobility
Syndrome
Joint Hypermobility Syndrome
Familial Connective tissue disorder
• Hyperlaxity of the joint
• Posturally induced headache
• Subset with pelvic/visceral symptoms affecting
upper and lower GI tract & urinary bladder
• Vascular pooling in the periphery while
standing
What happens on standing?
Joint Hypermobility Syndrome
• Venous pooling in the periphery when upright
PoTS and AMS
Multidisciplinary approach
Clinicians
Autonomic
Nurse Specialist
1) Clinical spectrum
2) Complete autonomic evaluation
3) Tailored management
Autonomic
Scientists
Thank you for your attention
Creating a culinary masterpiece
Autonomic Management Plan
The Ingredients
•
•
•
•
•
•
•
Expectations
Identify symptom triggers
Other medical conditions and medication
Non-drug treatment
Drug treatment
Other health professionals
Occupational health, colleges/universities, DVLA
This will be unique for each individual
Tailor made treatment
The Recipe
Add non-pharmacological measures
Combine with medication if needed
Non-pharmacological measures
To minimise the effect of
• Environment – gravity, heat
• Daily activities – eating, exercise
• Psyche – pain, anxiety
Environmental
• Preventing pooling
• Activation Measures
• Exercise - especially strengthening of leg
musculature
• Physiotherapy
Daily activities
• Salt addition
• Fluid repletion
• Small meals
• Pace activity
• Occupational therapy
Fear
• Information ,support
Pain
• Management programs
Anxiety
• Relaxation
• CBT
Pharmacological
Mineralocorticoids - Fludrocortisone
Sympathomimetics that do not raise heart rate Midodrine
Cholinesterase inhibitors – Pyridostigmine
Beta-adrenergic blockers, cardioselective Clonidine
I(f) Channel blocker - Ivabradine
Tachycardia
v
partnership
autonomic team
non-pharmacological
medication
=
The Autonomic Clinic: Clinical Research Aspects
Dr David Low, PhD
The Autonomic Units’ Research Team
Prof. C.J. Mathias
Clinical & Research
Director
Dr David Low
Dr Ekawat Vichayanrat
Clinical Research Lead
UKIERI Clinical
Research Fellow
Dr Valeria Iodice
Dr Juan Carlos Sanchez-Manso
Sir Roger Bannister
Clinical Research Fellow
EFNS Clinical
Research Fellow
William Seligman
Andrew Owens
MBBS Clinical Research
Project Student
Clinical Research
Co-ordinator
Plus others!
The Autonomic Units’ Research Team
Prof. C.J. Mathias
Clinical & Research
Director
Research
Team
Clinical
Team
Fellows
Students
Co-ordinators
Doctors
Nurse Specialists
Autonomic Scientists
Administrative
Team
Research Aims
• Improve diagnosis
– define features of autonomic disease
– develop novel non-invasive techniques
• Understand causes of autonomic disorders
– determine pathophysiological mechanisms
• Devise and evaluate new treatments
– improve management of disorders and their
complications
Research Achievements
Detailed
phenotyping of
autonomic disorders
Clinical and
Research training
placements
Development of
treatment
strategies
Diagnostic
techniques for
central & peripheral
disorders
Novel investigations
of physiology/pathophysiology
Autonomic Nervous System Function
Autonomic Dysfunction Symptoms
Blood
Pressure
Continence
Reproductive
Autonomic
Dysfunction
Gastrointestinal
Temperature
Sleep
Autonomic Disorders
Intermittent
Primary/
Secondary
• Syncope
• Postural Tachycardia
Syndrome (PoTS)
• Acute/Chronic
• Metabolic/Trauma/Drugs
Autonomic Function Research
Clinical
Test
Research
Study
Autonomic Function Research
Autonomic Function Research
Autonomic Dysfunction
Blood
Pressure
Continence
Reproductive
Autonomic
Dysfunction
Gastrointestinal
Temperature
Sleep
Postural Tachycardia Syndrome and AMS
venous pooling
hyperadrenergic
tone
physical
deconditioning
relative chronic
hypovolemia
genetics
JHS/collagen
dysfunction
Familial PoTS/EDS III
Family B
Family A
Family C
I1
II1
III1
III2
III3
II2
II3
II 1
II1
II2
II 2
Temperature
Skin Neurovascular Function
Gastrointestinal Function
Pacemaker
area
Autonomic Dysfunction and Exercise Training
Research Process
Develop
Protocol
Ethical
Approval
Data
Collection
Data
Analyses
Publication
of Findings
Research Process
Develop
Protocol
Ethical
Approval
Data
Collection
Data
Analyses
Publication
of Findings
Recruitment
Clinical & Research Fellows
Dr David Low - Clinical Research Lead
Dr Valeria Iodice - Sir Roger Bannister Clin.Res. Fellow
Dr Ekawat Vichayanrat - Clinical Research Fellow
Dr Juan Carlos Sanchez-Manso – Clin. Res. Fellow
Clinical Autonomic Scientists
Senior Clinical Autonomic Scientists
Madeline Tippetts
Katharine Bleasdale-Barr
Lydia Mason
Clinical Automomic Scientists
Michael Peche
Vanessa Ponnusamy
Ian Skeavington
O. Osiguwa
Fiona Vaidya
Helen Cannon
Seema Maru
Kiran Sheri
Clinical Research Co-ordinator
Andrew Owens
Clinical NeuroendocrineScientist
Laura Watson
Consultant & Head of Department
Prof. CJ. Mathias
Consultant
Dr. G. Ingle
Autonomic Nurse Specialist
Catherine Best
Thank you for your attention
Autonomic Function in Health & Disease

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