Stiff Person Syndrome

What is Stiff Person Syndrome?

Stiff person syndrome (SPS) is a rare disorder of the central nervous system that causes muscle rigidity and painful spasms, mainly in the trunk and the muscles close to the body.^[1] It is an autoimmune condition in which the brain and spinal cord become overexcitable because inhibitory signaling is impaired.^{[2, 3]} It needs ongoing care from a neurologist, and complementary care cannot control the disease.

Common symptoms

Stiffness in the limbs and trunk is the core symptom, along with a stiff gait that can cause falls and episodic painful spasms.^[2] The spasms can be triggered by anxiety, sudden noise or touch, and startle.^{[1, 2]}

Other common problems include:

• Pain and reduced mobility
• Anxiety, phobias such as fear of crossing a street, and depression^[4]
• A higher rate of psychiatric illness than in the general population, with diagnosis sometimes delayed because symptoms are mistaken for a psychiatric cause^[5]

Classic SPS is the most common form. Variants include partial forms, stiff limb syndrome and a severe form called PERM, which also involves myoclonus and instability of the autonomic nervous system.^[1]

Causes and risk factors

SPS is thought to be autoimmune. Most people with the classic form have antibodies to an enzyme called glutamic acid decarboxylase (GAD), which helps make the calming brain chemical GABA, although the exact role of these antibodies is still uncertain.^{[1, 4]} Other antibodies, for example against the glycine receptor, occur in some patients.^[3] SPS can occur with other autoimmune conditions such as type 1 diabetes, autoimmune thyroid disease, pernicious anemia, celiac disease and vitiligo, and a paraneoplastic form is linked to some cancers.^[1]

How it is diagnosed and treated conventionally

Diagnosis rests on the clinical picture, antibody tests and tests of muscle electrical activity, and delay in diagnosis is common and linked to lasting disability.^{[2, 3]} Experts also warn that SPS is sometimes overdiagnosed in people with other conditions.^[2]

First-line treatment uses GABA-enhancing drugs such as baclofen, benzodiazepines such as diazepam, tizanidine and gabapentin, and people who do not respond well may be given immunotherapy. Intravenous immunoglobulin has the most supporting evidence, and plasmapheresis and rituximab are also used.^{[4, 6]} Combination treatment is common, and the long-term outlook varies, with some people progressing to disability if treatment is delayed.^{[1, 6]}

Why Stiff Person Syndrome calls for a whole-person approach

SPS is driven by the immune system and by overactive nerve circuits, and it needs specialist treatment. It also touches many parts of daily life, including pain, sleep, anxiety, movement and the effects of long-term medicines.

Supportive care is aimed at those parts of life. The evidence below concerns symptoms in other groups, and it is not evidence that any therapy changes the course of SPS.

Nervous system excitability

The disorder is described as a failure of inhibition in the nervous system, so that stimuli such as noise, touch or emotion can set off spasms.^{[2, 3]} Whether any complementary therapy changes this excitability has not been studied.

Stress, anxiety and spasms

Anxiety and stress can trigger spasms, and anxiety and phobias are part of the illness.^{[2, 4]} This makes stress reduction a reasonable supportive goal, alongside psychiatric or psychological care when needed.^[5]

Pain and sleep

Painful spasms and stiffness often disturb sleep and daily activity.^{[1, 7]} Pain and sleep are the symptoms where supportive therapies have been studied most, although not in people with SPS.

Medicine effects

Treatment commonly involves sedating medicines such as benzodiazepines, so sedation, falls and drug interactions are real concerns that your neurologist manages.^[6]

Acupuncture as supportive care in Stiff Person Syndrome

What the research shows

The evidence for acupuncture in SPS is very limited. A single case report described a 60-year-old woman with SPS caused by an ovarian cancer who received weekly acupuncture for 9 weeks in addition to her intravenous immunoglobulin treatment. Her forward and sideways bending and timed walking and stair tests improved, and most quality-of-life measures improved. A single case with no comparison group cannot show that acupuncture caused the changes, and the author said more studies were needed.^[7]

Evidence for related symptoms comes from other conditions. A meta-analysis of 39 trials with individual patient data found acupuncture better than sham and no-acupuncture controls for chronic musculoskeletal pain, osteoarthritis and chronic headache, with effects that persisted for a year.^[8] For sleep, a meta-analysis of 24 trials in insomnia found improvement compared with drug treatment after about three weeks, with large differences between trials.^[9]

For muscle tone, an overview of 18 systematic reviews found moderate evidence for acupuncture as an add-on to routine care after stroke. A separate meta-analysis of 22 trials found that electroacupuncture added to usual care reduced spasticity after stroke, although the results varied and the authors called for better trials.^{[10, 11]} Spasticity after stroke is a different problem from the rigidity and spasms of SPS, so this does not show that acupuncture works for SPS.

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 tested in SPS.

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. Tell your practitioner about triggers for your spasms, such as sudden noise or touch, so that the visit can be planned around them.

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, have a bleeding disorder, are pregnant, or have a pacemaker (relevant to electroacupuncture). Also say if you take immune-suppressing treatment, and mention any falls or balance problems.

Ayurvedic medicine as supportive care in Stiff Person Syndrome

The Ayurvedic view

Ayurveda has no classical name for stiff person syndrome. Traditional practitioners may describe stiffness, spasm and pain as a Vata imbalance, since Vata is the principle said to govern movement and the nervous system. 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
• Gentle, warm oil massage and relaxation and breathing practices, adapted to your triggers and mobility
• 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 in people with SPS who have significant weakness, falls or unstable symptoms, or who are on sedating medicines, unless the treating neurologist 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 stiff person 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 SPS or on sedating medicines.

Herbal medicine as supportive care in Stiff Person Syndrome

Herbs and formulas that have been studied

No herbal medicine or formula has been tested in people with stiff person syndrome. Chinese herbal medicine has been studied for spasticity after stroke, which is a different problem from SPS.^[16]

What the research shows

There is no evidence for herbal medicine in SPS. A meta-analysis of 35 trials (2,457 patients) found that oral or topical Chinese herbal medicine added to routine care was associated with lower spasticity scores after stroke. The review noted quality concerns with the trials, and only 10 of 35 reported safety information.^[16] This does not show a benefit in SPS. Ashwagandha has been studied for stress, anxiety and sleep in other adults, and the certainty of that evidence is low.^{[14, 15]}

Safety and herb-drug interactions

This matters more in SPS than in many conditions, because people often take sedating medicines. A review of herb-drug interactions describes kava causing a semicomatose state when given with the benzodiazepine alprazolam, and St. John's wort lowering levels of immunosuppressants such as cyclosporine and tacrolimus. It also reports bleeding when danshen, garlic or ginkgo were taken with warfarin or aspirin.^[17] A review of warfarin cases also lists dang gui, ginger and Boswellia.^[18]

Herbal and dietary supplements account for a notable share of drug-induced liver injury in the United States.^[19] About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[20] 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 stiffness and spasm as stagnation of qi and blood or liver wind, and Ayurveda describes disturbed Vata. Researchers study ideas that loosely parallel these pictures, such as overactive nerve circuits, muscle tone, inflammatory signaling and the stress response.^{[2, 12]}

No study has shown that a traditional pattern corresponds to the loss of GABA and glycine inhibition in SPS. 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 and other physicians, and do not stop or change baclofen, benzodiazepines, immunotherapy or other prescribed treatment without your prescriber. Stopping some of these drugs suddenly can be dangerous.

We do not diagnose or monitor the disease or adjust medication. Please bring your diagnosis, antibody and test results, your full list of medicines and supplements, and the name of your neurologist.

When to seek urgent medical care

SPS can cause severe spasms and falls, and the severe PERM form can affect breathing and the autonomic nervous system.^{[1, 2]}

• Severe, prolonged spasms that do not ease, or trouble breathing
• A fall with injury, or a sudden worsening of walking or stiffness
• Fever, sweating, racing heartbeat or blood pressure swings along with rigidity
• Confusion, seizure or new weakness
• Sudden worsening after stopping or missing a medicine such as baclofen
• Thoughts of harming yourself (in the US, call or text 988, the Suicide and Crisis Lifeline)

If any of these occur, call 911 or go to the nearest emergency department.