What is Autoimmune Autonomic Ganglionopathy?
Autoimmune Autonomic Ganglionopathy (AAG) is a rare autoimmune condition where your body’s immune system mistakenly attacks the autonomic nervous system. This system controls vital involuntary functions like heart rate, blood pressure, digestion, and temperature regulation. In AAG, your immune system attacks your autonomic nervous system. You may experience fainting, low blood pressure upon standing or gastrointestinal symptoms.
The condition affects approximately 100 people annually in the United States, making it extremely rare. Approximately 100 Americans are diagnosed with AAG per year. Similar prevalence rates are expected in the UK, suggesting fewer than 70 new cases each year across the country.
Understanding Your Autonomic Nervous System
Your autonomic nervous system (ANS) acts like your body’s autopilot, managing essential functions without conscious thought. It comprises three main parts:
- Sympathetic nervous system: Your “fight or flight” response
- Parasympathetic nervous system: Your “rest and digest” functions
- Enteric nervous system: Controls digestion in your gut
When AAG strikes, it disrupts communication between these systems and your organs, leading to widespread symptoms that can significantly impact daily life.
Key Symptoms of AAG
Primary Symptoms
The symptoms of AAG can vary considerably between individuals, but typically include:
Cardiovascular Symptoms:
- Fainting (syncope) and near-fainting episodes
- Low blood pressure when you move to stand (neurogenic orthostatic hypotension)
- Rapid heart rate upon standing
- Blood pressure fluctuations
Gastrointestinal Symptoms:
- Constipation and severe gastroparesis (delayed stomach emptying)
- Abdominal pain and bloating
- Nausea and vomiting
- Difficulty swallowing
Autonomic Dysfunction:
- Dry mouth or dry eyes (sicca syndrome)
- Dilated pupils (Adie’s pupils)
- Urinary retention and bladder dysfunction
- Temperature regulation problems
- Excessive or absent sweating
Secondary Symptoms:
- Chronic fatigue and weakness
- Sleep disturbances
- Sexual dysfunction
- Cognitive difficulties (“brain fog”)
Symptom Onset Patterns
The onset can be acute, subacute, or gradual. Some patients experience rapid symptom development over days or weeks, whilst others notice a gradual progression over months or years. Understanding your symptom pattern helps healthcare providers determine the most appropriate treatment approach.
What Causes AAG?
Primary Causes
The exact cause of AAG remains largely unknown, but researchers have identified several potential triggers:
Idiopathic AAG (No known cause):
- Accounts for the majority of cases
- About 3 in 5 people with AAG develop it after getting another illness or infection
- May follow viral infections, including COVID-19
Paraneoplastic AAG (Cancer-related):
- Paraneoplastic AAG occurs when your immune system makes certain antibodies in response to cancer
- Most commonly associated with:
- Small cell lung cancer
- Thymoma
- Breast cancer
- Lymphoma
The Role of Antibodies
About 2 in 3 people with AAG have high levels of antibodies called ganglionic acetylcholine receptor antibodies (g-AChR antibodies). These antibodies target specific receptors in your autonomic nervous system, disrupting normal nerve communication.
Importantly, Researchers think that your g-AChR antibody levels correlate to how severe your symptoms are, meaning higher antibody levels often indicate more severe symptoms.
Getting Diagnosed with AAG in the UK
Initial Assessment
Diagnosing AAG requires careful evaluation by specialists familiar with autonomic disorders. In the UK, this typically involves referral to:
- NHS Autonomic Units at major hospitals
- Neurologists with autonomic expertise
- Specialist dysautonomia clinics
Key NHS Centres for Autonomic Disorders
National Hospital for Neurology and Neurosurgery (UCLH)
- The autonomic unit provides a comprehensive national tertiary clinical service (combining both investigation and management) for a wide variety of autonomic disorders
- Queen Square, London
- Accepts referrals through NHS e-referrals
St George’s University Hospitals NHS Foundation Trust
- As a regional specialist centre, the autonomic disorders team work closely with clinical teams from across St George’s and other hospitals in south west London and Surrey
- Provides comprehensive autonomic investigations
Diagnostic Tests
Your healthcare provider may recommend several tests:
Autonomic Function Tests:
- Tilt table test to check your heart rate and blood pressure when changing positions
- Quantitative sudomotor axon reflex test (QSART) to measure how your sweat glands work
- Thermoregulatory sweat test to find the cause of decreased or increased sweating
Blood Tests:
- You may also have a blood test to measure your g-AChR antibody levels. High levels of g-AChR antibodies may point to AAG
- Complete blood count and inflammatory markers
- Cancer screening tests if paraneoplastic AAG is suspected
Additional Investigations:
- Gastrointestinal tests, such as gastric emptying, to check your digestion
- Urinalysis to check your bladder function
- ECG and echocardiogram for cardiac assessment
Treatment Options Available in the UK
Immunomodulatory Treatments
AAG is so rare that healthcare providers don’t have standardised treatment for it. Usually, your treatment plan focuses on symptom management. However, several evidence-based approaches are available through the NHS:
First-Line Treatments:
Intravenous Immunoglobulin (IVIG)
- Provides immune system modulation
- Immunomodulatory treatment can be effective in both seropositive and seronegative putative autoimmune autonomic ganglionopathy
- Usually administered as hospital treatment
Plasma Exchange (Plasmapheresis)
- Plasma exchange (plasmapheresis): A process of drawing blood, removing harmful cells from plasma and returning blood
- Plasma exchange or combined therapy with immunosuppressive agents should be considered in patients who do not benefit from IV immunoglobulin alone
Long-term Immunosuppressive Therapy:
- Corticosteroids (prednisolone)
- Rituximab
- Mycophenolate mofetil
- Azathioprine
Symptomatic Management
Orthostatic Hypotension:
- Fludrocortisone to increase blood volume
- Midodrine to constrict blood vessels
- Compression stockings and physical countermanoeuvres
- Increased salt and fluid intake
Gastrointestinal Symptoms:
- Prokinetic agents (domperidone, metoclopramide)
- Dietary modifications (small, frequent meals)
- Nutritional support when needed
Autonomic Support:
- Artificial tears for dry eyes
- Saliva substitutes for dry mouth
- Bladder management strategies
Recent Treatment Advances
Recent research has shown promising results with combination therapies. Fortunately, multiple plasma exchange treatments improved the patient’s symptoms. In the end, low-dose oral prednisone and mycophenolate mofetil provided significant improvement in this patient during long-term follow-up.
NHS Resources and Support Services
Getting NHS Treatment
Primary Care Pathway:
- Discuss symptoms with your GP
- Request referral to neurology or autonomic specialist
- Attend NHS autonomic unit for assessment
- Receive ongoing care through shared care arrangements
NHS Support Services:
Specialist Nursing
- We also work with GPs and community nursing teams to help patients manage their condition and symptoms outside of hospital
- Autonomic nurse specialists provide ongoing support
- Medication monitoring and adjustment
Multidisciplinary Care
- Cardiology for heart-related symptoms
- Gastroenterology for digestive issues
- Physiotherapy for mobility and strength
- Occupational therapy for daily living adaptations
Accessing Treatment Through the NHS
What to Expect:
- Initial consultation within 18 weeks of referral
- Comprehensive autonomic testing
- Treatment plan development
- Regular follow-up appointments
- Emergency contact arrangements
Preparing for Your NHS Appointment:
- Keep a detailed symptom diary
- List all medications and supplements
- Bring family medical history
- Prepare questions about treatment options
UK Support Organisations and Charities
National Support Groups
The Brain Charity
- We are the only charity in the UK to be here for every one of the more than 600 different neurological conditions in existence
- Based in Liverpool with UK-wide support
- We provide practical help on all aspects of living with dysautonomia, emotional support such as counselling, phone befriending and group therapy
- Website: thebraincharity.org.uk
Dysautonomia International
- Global organisation with UK members
- Our support groups are based on Facebook, but they also host in-person meetings and virtual meet-ups on Zoom
- Provides educational resources and advocacy
The Dysautonomia Project
- The Dysautonomia Project is the global leader in dysautonomia education with a mission to educate patients, healthcare providers, and communities
- Free educational resources and assessment tools
Local Support Networks
Regional Groups:
- Contact NHS autonomic units for local support group information
- Patient advocacy groups through major hospitals
- Online communities and forums
Family and Carer Support:
- The Brain Charity provides free support for carers, friends and family of people with any form of neurological condition, including dysautonomia, from anywhere in the UK
- Carer advocacy services in Liverpool area
- Respite care options through local councils
Living with AAG: Practical Management
Daily Life Adaptations
Managing Orthostatic Symptoms:
- Rise slowly from sitting or lying positions
- Wear compression stockings
- Increase salt intake (under medical supervision)
- Stay well-hydrated
- Avoid hot environments and prolonged standing
Dietary Modifications:
- Eat smaller, more frequent meals
- Avoid large carbohydrate loads
- Consider gastroparesis-friendly foods
- Maintain adequate nutrition with specialist support
Energy Conservation:
- Plan activities around energy levels
- Use mobility aids when needed
- Delegate tasks to family members
- Prioritise essential activities
Employment and Benefits
Workplace Accommodations:
- Flexible working hours
- Regular breaks and rest periods
- Ergonomic workspace setup
- Working from home options
UK Benefits You May Be Entitled To:
- Personal Independence Payment (PIP)
- Employment and Support Allowance (ESA)
- Disability Living Allowance (for under-16s)
- Blue Badge parking permits
- Access to Work scheme support
Emergency Preparedness
When to Seek Urgent Care:
- Severe orthostatic symptoms with falls
- Persistent vomiting preventing medication absorption
- Signs of dehydration or electrolyte imbalance
- Acute changes in symptoms
Medical Alert Information:
- Carry medical identification
- Keep medication list updated
- Share condition information with family
- Have emergency contact details readily available
Prognosis and Long-term Outlook
Understanding Your Prognosis
Although there’s no cure for AAG, many people successfully manage symptoms long-term. The outlook varies significantly:
Positive Factors:
- For about 1 in 3 people, AAG symptoms improve without treatment
- Early diagnosis and treatment improve outcomes
- Young age at onset often associated with better recovery
- Supportive care significantly improves quality of life
Ongoing Management:
- Most people with AAG deal with some symptoms for the rest of their lives
- Regular monitoring and medication adjustments needed
- Lifestyle adaptations become routine
- Strong support networks essential
Recent Research Developments
Clinical Trials:
- The purpose of the study is to see if administering intravenous immune globulin (IVIG) (putting immune globulin directly into your blood) helps to improve the symptoms of orthostatic hypotension
- Novel immunotherapy approaches under investigation
- Biomarker research for better diagnosis and monitoring
Future Treatments:
- Targeted antibody therapies
- Advanced immunomodulation techniques
- Personalised medicine approaches
- Regenerative treatments under development
Frequently Asked Questions
Is AAG hereditary?
AAG is not typically inherited, though genetic factors may influence susceptibility to autoimmune conditions.
Can AAG be prevented?
Currently, there’s no known way to prevent AAG, but early treatment of triggering infections may reduce risk.
How does AAG differ from other dysautonomia conditions?
AAG is specifically caused by antibodies attacking autonomic ganglia, unlike other forms of dysautonomia with different underlying mechanisms.
Will I need lifelong treatment?
Treatment needs vary greatly. Some patients require ongoing immunosuppression, whilst others may achieve stable remission.
Can I have children with AAG?
Pregnancy requires careful planning and specialist monitoring, but many women with AAG have successful pregnancies.
Key Takeaways
Remember:
- AAG is a serious but treatable autoimmune condition
- Early diagnosis and treatment improve outcomes significantly
- The NHS provides comprehensive care through specialist centres
- Strong support networks are essential for managing daily life
- Research continues to improve treatment options
Next Steps:
- Discuss symptoms with your GP if you suspect AAG
- Request referral to NHS autonomic specialist
- Connect with support organisations
- Keep detailed symptom records
- Stay informed about treatment advances
For immediate support, contact The Brain Charity on 0151 298 2999 or visit your nearest NHS autonomic unit.
This guide provides general information about AAG and should not replace professional medical advice. Always consult your healthcare provider for personalised treatment recommendations.
