www.thetinman.org Copyright © All rights reserved.
There is currently no cure for stiff-person syndrome. The goal of treatment is to manage the symptoms and make you as comfortable and functional as possible as long as possible.
The first line of treatment is finding the right combination of medications to manage spasticity, stiffness, and rigidity, usually with GABA-ergic drugs, steroids, pain medication, and anti-seizure medication. Learn more.
The second line of treatment is to support the immune system with intravenous immunoglobulin, which infuses your body with with donated blood products. Results are mixed. Learn more.
This could also include plasmapheresis, a process of filtering antigens from your blood plasma. Results are mixed. Learn more.
The third line of treatment includes the use of immunosuppressive drugs. Results are mixed. Learn more.
There are several experimental treatments including: botulinum toxin injections, cannabanoids, clomipramine, clonidine, interferon, L-dopa, and Milacemide. Learn more.
Physical therapy, and other modalities such as accupuncture, dry needling, and massage therapy have benefited some patients, depending on the physical capacity of the individual, progression of the disease, the methods used, and the experience of the staff in working with rare diseases. Learn more.
The fourth line of treatment is to restart the immune system with hematopoietic stem cell therapy. This is in the trial study phase. Early results are “cautiously optimistic.” Learn more.
w Initially, the spasms and startle symptoms seem to respond best to Valium (diazepam) or Xanax (alprazolam). These benzodiazepines are used to treat different forms of spasticity and anxiety. Learn more.
w Baclofen can work in conjunction with benzodiazepines, but rarely works alone. Baclofen can help with stiffness and rigidity. Some patients have benefited from the implantation of a baclofen pump, though this is not without risks. Learn more. Learn more.
w Skeletal muscle relaxers generally do not have any impact on spasticity, myoclonus, or stiffness, but are sometimes prescribed. Learn more.
w Every individual responds differently to drugs. Finding the right cocktail is a matter of trial and error.
w Medications, particularly benzodiazepines, may need to be changed or rotated to avoid receptor down-regulation. This is a process by which cells decrease the number of receptors to a hormone or neurotransmitter to reduce the cell's sensitivity to it due to overexposure or prolonged exposure.
w The drugs used to treat stiff-person syndrome may require titration up and down. Abrupt withdrawal can cause serious side-effects, even death. Never stop or change medication on your own. Always consult with your physician if you experience new side-effects or the medication stops working.
w Dosages for stiff-person syndrome are often higher than doses for the general population, especially the level of benzodiazepines and baclofen. One milligram of Xanax is the equivalent of twenty milligrams of Valium.
w Patients who are highly sensitive to drugs may need less or even pediatric doses. Only you can tell how much or little you can tolerate. Don’t hesitate to discuss the results with your physicians.
w It is helpful to understand how the drugs work to avoid taking medications that duplicate the effect or work against one another. Learn more.
w The drugs used to treat stiff-person syndrome can impact medications for other conditions. It is important for your team to work together to balance your mix of medications.
w Thyroid and diabetes medications can interfere with each other. Benzodiazepines can affect thyroid medications and diabetes medications. You may have to investigate drug interactions on your own or discuss them with a pharmacist you trust. Benzodiazepines have been shown to inhibit T3 uptake into the cells of the body. They may alter insulin secretion and insulin sensitivity. Benzodiazepines can cause an acute lowering of serum cortisol levels. Moreover, chronic use of opioids, antipsychotics, and benzodiazepines can cause GABA-induced suppression of CRH release leading to low cortisol levels. Endocrinologists are not well-versed in the intricacies of these drug interactions.
1. Khadem-Ansari MH, Ahani A, Mikaeili P, Rasmi Y. Effect of benzodiazepines on thyroglobulin, anti-thyroglobulin, anti-thyroid peroxidase, and thyroid stimulating hormone in rat. Clin Neuropharmacol. 2011 Nov-Dec;34(6):248-55. doi: 10.1097/WNF.0b013e31823429a7 Link to article.
WHAT ARE YOUR TREATMENT OPTIONS?
WHAT CAN YOU EXPECT?
2. Grandison L. Actions of benzodiazepines on the neuroendocrine system. Neuropharmacology 1983;22:1505-10. Link to article.
3. Beary MD, Lacey JH, Bhat AV. The neuro-endocrine impact of 3-hydroxy-diazepam (temazepam) in women. Psychopharmacology (Berl) 1983;79:295-7. Link to article.
4. Clark F, Hall R, Ormston BJ. Diazepam and tests of thyroid function. Br Med J 1971;1:585-6. Link to article.
5. Saldanha VF, Bird R, Havard CW. Effect of diazepam (Valium) on dialysable thyroxime. Postgrad Med J 1971;47:326-8. Link to article.
6. Eiris-Punal J, Del Rio-Garma M, Del Rio-Garma MC, Lojo-Rocamonde S, Novo-Rodriguez I, Castro-Gago M. Long-term treatment of children with epilepsy with valproate or carbamazepine may cause subclinical hypothyroidism. Epilepsia 1999;40:1761-6. Link to article.
7. Camoratto AM, Grandison L. Inhibition of cold-induced TSH release by benzodiazepines. Brain Res 1983;265:339-43.
8. Chevassus H, Mourand I, Molinier M, Lacarelle B, Brun JF, Petit P. Assessment of single-dose benzodiazepines on insulin secretion, insulin sensitivity and glucose effectiveness in healthy volunteers: a double-blind, placebo-controlled, randomized cross-over trial. BMC Clinical Pharmacology 2004, 4:3 doi:10.1186/1472-6904-4-3. Link to article.
9. Salice VS, Valenza FV, Pizzocri MP, et al. Benzodiazepines induce hyperglycemia in rats by affecting peripheral disposal of glucose. Critical Care 2013, 17(Suppl 2):P385 doi:10.1186/cc12323. Link to article.
10. Giordano R, Grottoli S, Brossa PC, et al. Alprazolam (a benzodiazepine activating GABA receptor) reduces the neuroendocrine responses to insulin-induced hypoglycaemia in humans. Clinical Endocrinology Volume 59, Issue 3, pages 314–320, September 2003. Link to article.
11. Tella SH, Gallagher JC. Relationship of Benzodiazepines with Serum Cortisol Levels: A Hidden Fact. Pharmacopsychiatry. 1986 Jan;19(1):19-22 Link to article.
w Medications have multiple brand and generic names. Make sure you are not being prescribed multiples of the same drug.
w The medications may have stringent government regulations for prescription and follow-up. Keep your appointments. Keep your prescriptions up to date. Sign up for alerts from your pharmacy. Give your doctor’s office enough time to call in refills. Learn more about the laws here. See a sample pain management agreement here.
w If you experience memory issues, a pill organizer is useful. Keep notes as to dosages and times if you are adjusting doses. Inform your emergency contacts of your medications and dosages. You can read more about managing your medications here.
w It is important that emergency care providers are made aware of your medications and the need to avoid abrupt withdrawal. Carry a card with your prescription information with you and a warning to not abruptly discontinue your medications. If emergency personnel mistakenly assume you are taking the drugs recreationally, they may try to detoxify you or wean you off your medications. You can read more about emergency care here.