THE TIN MAN GUIDE TO STIFF-PERSON SYNDROME
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1. Baclofen, Kemstro, Lioresal, Liofen, Gablofen, Beklo, Baclosan


Baclofen is a derivative of gamma-aminobutyric acid (GABA). It is primarily used to treat spasticity. It is also used by compounding pharmacies in topical pain creams as a muscle relaxant. It is an agonist for the GABA receptors. Baclofen produces its effects by activating the GABA receptor.


Its beneficial effects in spasticity result from actions at spinal and supraspinal sites. Baclofen can also be used to treat hiccups. Tolerance does not seem to occur to a significant degree. Baclofen retains its therapeutic anti-spasmodic effects even after many years of continued use. Tolerance may develop in some patients receiving intrathecal baclofen treatment. However, baclofen does not have significant affinity for the GHB receptor, and has no known abuse potential. Oral dosage must be carefully regulated; significantly high doses of the drug, particularly 80 mg per day or higher, can cause excessive ataxia and/or drowsiness that can interfere with daily function. Avoid abrupt withdrawal. Drugs.com.


References for Baclofen:


1. Todman D. Stiff-Person Syndrome (Moersch-Woltman Syndrome). The Internet Journal of Neurology. 2006 Volume 7 Number 1  Link to article


2. Seitz RJ, Blank B, Kiwit JC, et al. Stiff-person syndrome with anti-glutamic acid decarboxylase autoantibodies: complete remission of symptoms after  intrathecal baclofen administration. Neurology 1995, 242:618–622. Link to article


3. Bardutsky J, Tronnier V, Schwab S, et al. Intrathecal baclofen treatment for stiff-man syndrome: life-threatening intermittent catheter leakage. Neurology 2003;60:1976-8.

Link to article


4. Ho BL, Shih PY. Successful intrathecal baclofen therapy for seronegative stiff-person syndrome: a case report. Acta Neurol Taiwan. 2008 Sep;17(3):172-6. Link to article


5. Maramattom BV. Intrathecal baclofen pump implantation in a case of stiff person syndrome. Neurol India. 2010 Jan-Feb;58(1):115-7. doi: 10.4103/0028-3886.60420.

Link to article


6. Meinck HM, Tronnier V, Rieke K, et al. Intrathecal baclofen treatment for stiff-man syndrome: pump failure may be fatal. Neurology 1994;44:2209-10.  Link to article


7. Miller F, Korsvik H. Baclofen in the treatment of stiff-man syndrome. Ann Neurol 1981, 9:511–512. Link to article


8. Penn RD, Mangieri EA. Stiff-man syndrome treated with intrathecal baclofen. Neurology 1993, 43:2412. Link to article


9. Seitz RJ, Blank B, Kiwit JC, Benecke R. Stiff-person syndrome with anti-glutamic acid decarboxylase autoantibodies: complete remission of symptoms after intrathecal baclofen administration. J Neurol. 1995 Oct;242(10):618-22. Review.  Link to article


10. Silbert PL, Matsumoto JY, McManis PG, et al. Intrathecal baclofen therapy in stiff-man syndrome: a double-blind, placebo-controlled trial.  Neurology 1995, 45:1893–1897.

Link to article


11. Stayer C, Tronnier V, Dressnandt J, et al. Intrathecal baclofen therapy for stiff-man syndrome and progressive encephalopathy with rigidity and myoclonus.  Neurology 1997, 49:1591–1597.  Link to article


12.Whelan JL. Baclofen in treatment of the stiff-man syndrome. Arch Neurol 1980, 37:600–601.  Link to article





2. GHB (γ-Hydroxybutyric acid)


Gamma Hydroxybutyric acid (GHB), also known as 4-hydroxybutanoic acid, is a naturally occurring substance found in the human central nervous system, as well as in wine, beef, small citrus fruits, and almost all animals in small amounts. It is also categorized as an illegal drug in many countries. GHB has been used as a general anesthetic, to treat insomnia, clinical depression, narcolepsy, and alcoholism, and to improve athletic performance. It is also used as an intoxicant or as a date rape drug. As a supplement or drug, it is used most commonly in the form of a salt, such as sodium gamma-hydroxybutyrate (Na.GHB, sodium oxybate, or Xyrem) or potassium gamma-hydroxybutyrate (K.GHB, potassium oxybate). GHB is also produced as a result of fermentation, and so is found in small quantities in some beers and wines.


GHB has at least two distinct binding sites in the central nervous system. GHB is an agonist at the newly characterized GHB receptor, which is excitatory, and it is a weak agonist at the GABA receptor, which is inhibitory. GHB is probably synthesized from GABA in GABAergic neurons, and released when the neurons fire. If taken orally, GABA itself does not effectively cross the blood-brain-barrier. However, at therapeutic doses, GHB reaches much higher concentrations in the brain and activates GABA receptors, which are primarily responsible for its sedative effects and is responsible for the addictive profile of GHB. Low concentrations stimulate dopamine release via the GHB receptor. Higher concentrations inhibit dopamine release via GABA receptors as do other GABA agonists such as baclofen and phenibut. After an initial phase of inhibition, dopamine release is then increased via the GHB receptor. Drugs.com.



3. GHV (y-Hydroxyvaleric acid)


Gamma-Hydroxyvaleric acid is a psychoactive drug and designer drug which is structurally and pharmacologically related to both gamma-hydroxybutyric acid (GHB) and gamma-aminobutyric acid (GABA). It is sometimes seen on the black market as a legal alternative to GHB, but with lower potency and higher toxicity, which has tended to limit its recreational abuse. Wikipedia.


GABA AGONISTS

Treatment options  GABAergic drugs   GABA A Agonists   GABA Modulators   GABA B Agonists   GABA P Agonists


GABA Transaminase   GABA RUI   GABA Analogues   GABA Supplement   Muscle Relaxers   Corticosteroids


Antiseizure   Opiate Analgesics   Experimental   IVIG   Plasmapheresis   Immunotherapy   Stem Cell Therapy  PT