Susac’s Syndrome

What is Susac's Syndrome?

Susac's syndrome is a rare immune-mediated disease that damages the lining of the small blood vessels in the brain, the retina and the inner ear. It mainly affects young women.^{[1, 2]} Because it can cause lasting loss of thinking, vision and hearing, it is treated urgently by specialists, and complementary care has no role in controlling the disease.

Common symptoms

The classic picture is a triad of brain dysfunction (encephalopathy), blockage of small branches of the retinal arteries, and sensorineural hearing loss.^{[3, 4]} Only a minority of people have all three at the start, which makes the diagnosis difficult.^[3]

Symptoms can include:

• Confusion, memory problems and behavior changes that can look psychiatric, with an unusual headache^[2]
• Areas of lost or blurred vision, sometimes without much awareness of it^[2]
• Hearing loss, tinnitus and dizziness^{[2, 3]}

Causes and risk factors

The cause is not fully understood. The disease is thought to be an autoimmune attack on the cells lining small vessels. Studies of tissue suggest that immune T cells stick to the vessels, causing swelling, narrowing and blockage that lead to tiny areas of tissue death.^[3] Some patients have antibodies against these cells, but it is unclear whether they cause the disease.^[3] Some earlier descriptions blamed small clots, but current reviews describe an inflammatory small-vessel disease.^[2]

How it is diagnosed and treated conventionally

Diagnosis relies on the clinical picture, MRI of the brain, fluorescein angiography of the retina and hearing tests. There are no reliable blood markers to track disease activity.^{[3, 4]} Formal diagnostic criteria were proposed to reduce delays in treatment.^[4]

First-line treatment is usually high-dose corticosteroids. People who relapse or do not respond may be given intravenous immunoglobulin or drugs such as mycophenolate mofetil, cyclophosphamide or rituximab.^{[2, 5]} Treatment is based mainly on case series and expert opinion because the disease is too rare for large trials.^[3] The active phase lasts about two years in many people, relapses are common during it, and hearing loss and cognitive problems are the most frequent lasting effects.^[2]

Why Susac's Syndrome calls for a whole-person approach

The disease itself is driven by the immune system and is managed by neurologists, ophthalmologists and other specialists. Living with it also involves symptoms that continue after the inflammation is controlled, and effects of treatment.

This is where supportive care may have a place. The evidence below concerns those symptoms in other groups of people, not Susac's syndrome.

Immune activity in small vessels

The central problem is inflammation of the vessel lining, and treatment is aimed at suppressing it.^{[2, 3]} There is no research showing that acupuncture, Ayurveda or herbs change this process.

Hearing, tinnitus and balance

Hearing loss and tinnitus are common and can persist after treatment.^[2] Tinnitus in particular is distressing and affects sleep and concentration, and it is a symptom where acupuncture has been studied in other patients.

Headache

Headache is a recognized feature, and the headache of this disease can be unusual.^[2] Any new or changed headache in someone with Susac's syndrome should be reported to the treating team, because it can signal active disease.

Treatment side effects, sleep and stress

Long-term corticosteroids can thin the bones, and the American College of Rheumatology publishes guidance on assessing and treating glucocorticoid-induced osteoporosis. That care belongs with your physician.^[6] Stress and poor sleep are common in people living with a rare, unpredictable illness.

Acupuncture as supportive care in Susac's Syndrome

What the research shows

No clinical trials of acupuncture for Susac's syndrome were found in PubMed. The evidence below is for symptoms that may overlap and comes from other patient groups.

For tinnitus, a meta-analysis of eight trials (504 people) found no significant difference in the main symptom score between acupuncture and controls, although two secondary tinnitus scales favored acupuncture. The authors judged the trials to be of low quality and the evidence insufficient for a firm conclusion.^[7] An earlier review of electroacupuncture for tinnitus found five trials with weaknesses in all of them and no convincing evidence of benefit.^[8]

For sudden sensorineural hearing loss, a meta-analysis of 20 trials suggested better hearing test results when acupuncture was added to conventional drug treatment, but the authors said high-quality evidence was lacking.^[9] That condition differs from Susac's syndrome, and no study shows acupuncture restores hearing damaged by it.

For chronic pain including chronic headache, a meta-analysis using individual data from 39 trials with 20,827 patients found acupuncture better than sham and no-acupuncture controls, with effects that persisted for a year.^[10] For sleep, a meta-analysis of 24 trials in insomnia found improvement over drug treatment after about three weeks, with very different results between trials.^[11]

How acupuncture may work

A review of anti-inflammatory effects proposes that acupuncture may release nerve signaling molecules that affect blood vessels and inflammatory messengers, and notes the lack of large trials showing a lasting effect.^[12] This is a proposed mechanism and has not been shown to apply to Susac's syndrome.

What treatment involves

Treatment uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes, over a course of several visits with reassessment.

In prospective studies, about 9 in 100 patients had a minor reaction such as bleeding, pain or a mark at the needle site, and serious events occurred in about 1 in 10,000 patients.^[13] Tell your practitioner first if you take blood thinners or antiplatelet medicine, have a bleeding disorder, are pregnant, or have a pacemaker. Also say that you take immune-suppressing medicines, since this can affect how infections are handled.

Ayurvedic medicine as supportive care in Susac's Syndrome

The Ayurvedic view

Ayurveda has no classical name for Susac's syndrome. Traditional practitioners may describe the combined problems with the head, eyes and ears and disturbed thinking in terms of imbalance of Vata and Pitta. This is a traditional framework used to guide supportive care, not a medical diagnosis.

Therapies that may be used

Ayurvedic care is chosen for the individual. In a supportive role it may include:

• Diet and daily routine advice aimed at regular meals, steady sleep and stress reduction
• Relaxation and breathing practices and gentle body massage
• Individually prescribed herbs, such as ashwagandha, traditionally used for stress and low energy

Panchakarma is not appropriate during pregnancy, acute illness or frailty. It is also best avoided during active disease or while immune-suppressing treatment is being started or adjusted, unless the treating specialist agrees. A herbal preparation applied to the skin (transdermal medication therapy) is a supportive option only, and we make no claim of benefit for it in this disease.

What the research shows

No studies of Ayurvedic medicine for Susac's syndrome were found. For related symptoms, a meta-analysis of 12 randomized trials found that ashwagandha reduced stress and anxiety scores compared with placebo, with low-certainty evidence.^[14] A meta-analysis of five trials found a small improvement in sleep, and noted limited long-term safety data.^[15] These trials enrolled adults with stress, anxiety or insomnia, not people with Susac's syndrome or on immunosuppressants.

Herbal medicine as supportive care in Susac's Syndrome

Herbs and formulas that have been studied

No herbal medicine or formula has been tested in people with Susac's syndrome, and none was found for this disease in PubMed.

What the research shows

There is no evidence for herbal medicine in this disease. The only related evidence is for ashwagandha and stress, anxiety and sleep, described above, and its certainty is low.^{[14, 15]} No herb should be used in place of corticosteroids or other immune-suppressing treatment.

Safety and herb-drug interactions

This matters more here than in many conditions, because people with Susac's syndrome take immune-suppressing medicines and may also take blood thinners or antiplatelet drugs. A review of herb-drug interactions describes St. John's wort lowering levels of cyclosporine and tacrolimus, and reports of bleeding when danshen, garlic or ginkgo were combined with warfarin or aspirin.^[16] A review of warfarin cases also lists dang gui, ginger and Boswellia.^[17]

Herbal and dietary supplements account for a notable share of drug-induced liver injury in the United States.^[18] About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[19] Herbs should come from tested sources and be prescribed individually after review of your full medicine list. Tell us if you are pregnant or breastfeeding or have liver or kidney disease.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine would describe blocked flow of qi and blood or stagnation in the vessels of the head, eyes and ears, and Ayurveda would describe disturbed Vata and Pitta. Researchers study ideas that loosely parallel such pictures, including inflammatory signaling, blood vessel function and the nervous system's response to stress and pain.^[12]

No study has shown that a traditional pattern corresponds to the immune injury of the vessel lining in Susac's syndrome. The traditional language is used to guide supportive care for the person, not to explain the disease.

How this care fits with your medical care

Supportive care here is complementary. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Keep your neurologist, ophthalmologist, hearing specialist and any other physicians, and do not stop or change corticosteroids or other prescribed treatment without your prescriber.

We do not diagnose or monitor the disease, judge whether it is active or adjust medication. Please bring your diagnosis, recent MRI, eye and hearing test results, your full list of medicines and supplements, and the names of your specialists.

When to seek urgent medical care

Susac's syndrome can relapse and cause permanent damage to vision, hearing and thinking, so new symptoms need prompt assessment by your specialists.^{[2, 3]}

• New or worsening confusion, memory loss, personality change or severe headache
• Sudden loss of vision, a new dark patch in your vision, or new blurring
• Sudden hearing loss, new ringing in the ears, or new dizziness or vertigo
• Seizure, weakness, trouble speaking or loss of balance
• Fever or a new infection while taking immune-suppressing medicine

If any of these occur, call your specialist right away. For sudden severe symptoms, call 911 or go to the nearest emergency department.