| Article Access Statistics | | | Viewed | 12423 | | | Printed | 323 | | | Emailed | 7 | | | PDF Downloaded | 169 | | | Comments | [Add] | | | Cited by others | 12 | | |
|
Click on image for details.
|
|
| CASE REPORT |
|
|
|
| Year : 2010 | Volume
: 58
| Issue : 1 | Page : 115-117 |
Intrathecal baclofen pump implantation in a case of stiff person syndrome
Bobby Varkey Maramattom
Department of Neurology, Lourdes Heart Institute and Neuro Centre, Kochi, Kerala, India
| Date of Acceptance | 02-Nov-2009 |
| Date of Web Publication | 8-Mar-2010 |
Correspondence Address:
Bobby Varkey Maramattom
Department of Neurology, Lourdes Heart Institute and Neuro Centre, Kochi, Kerala
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.60420
Stiff person syndrome (SPS) is a rare disorder characterized by severe axial and/or limb rigidity, spasms and continuous motor unit activity on EMG. Symptomatically these patients are usually treated with GABAergic medications. We present a 45-year-old woman with SPS, who failed high dose of GABAergic medication. With intrathecal baclofen by intrathecal baclofen pump she had a significant improvement in her symptoms and quality of life.
Keywords: Intrathecal baclofen pump, stiff man syndrome, stiff person syndrome
How to cite this article:
Maramattom BV. Intrathecal baclofen pump implantation in a case of stiff person syndrome. Neurol India 2010;58:115-7 |
| » Introduction | |  |
Stiff person syndrome (SPS) is a rare progressive disorder characterized by severe axial and/or limb rigidity, spasms, and continuous motor unit activity on electromyography (EMG). [1] The estimated incidence of SPS in one in a million. Different authors have proposed different classifications for SPS based on clinical characteristics and antibody profile. Barker and colleagues [2] have classified SPS into three types: Progressive encephalomyelitis with rigidity, stiff man syndrome (SMS), and stiff limb syndrome (SLS). Progressive encephalomyelitis with rigidity is a fulminant condition characterized by widespread rigidity and death usually occurs within four months of onset. Stiff man syndrome is characterized by rigidity and painful spasms of the lumbar paraspinal, abdominal and proximal leg muscles with Lumbar hyperlordosis and often associated with anti-GAD antibodies and concurrent autoimmune disease such as insulin-dependent diabetes mellitus (IDDM) and thyroditis. Stiff limb syndrome is characterized by rigidity, spasm and abnormal posturing of a distal limb, usually the leg, simulating dystonia. The other classification proposed by Grimaldi and colleagues divides SPS into three types: Type I, associated with other autoimmune diseases and often anti GAD +ve (55-60% of patients); Type II: Paraneoplastic, anti amphiphysin positive (5% of patients); and Type III: Idiopathic, antibody negative (35-40% of patients). [3] Only a few cases of SPS have been reported from India. [4],[5],[6],[7]
Though most patients are treated with GABAergic medications, only few achieve a satisfactory quality of life (QoL). Intrathecal baclofen (ITB) pump therapy has been tried in a few patients with benefit. However, its use remains sporadic. We report ITB use in an Indian patient with SPS and discuss issues pertinent to our country.
| » Case Report | | |
A 45-year-old woman presented with pains in both legs and difficulty in walking of four years duration. Her dominant symptoms were stiffness and pain in both legs and lower back. She had a history of occasional falls related to sudden spasms in the legs precipitated by loud sound. She sustained a recent left patellar fracture after a fall. She was admitted with an acute exacerbation of stiffness and pain. For the past three years she had required assistance for all activities of daily living. She had diabetes for which she was on insulin.
On examination, she had hyper lumbar lordosis with rock hard lumbar paraspinal muscles and deep lumbar furrowing [Figure 1]. Both legs were stiff (left more than right) and there was clawing of the left toes [Ashworth scale 5]. Motor and sensory examination was normal. Her gait was slow, cautious and 'tin soldier' like. Anti GAD antibodies were negative and MRI of the spine was normal. Nerve conduction studies were normal. EMG studies showed continuous motor unit activity from the lumbar paraspinal muscles. A paraneoplastic work up was negative. She was diagnosed as SMS variant of SPS.
She was treated for two weeks with high dose of oral diazepam (90 mg/day), oral baclofen (90 mg/day) and continuous IV infusion of diazepam (50 mg/ day). Methylprednisolone was deferred because of brittle diabetic state and the patient was unwilling for plasma exchange. She had little improvement in stiffness but was extremely sedated. After two weeks, the option of ITB pump was offered for which she agreed. A test dose of ITB (50 mcg) dramatically reduced her stiffness, following which she opted for ITB pump implantation. Accordingly a Medtronic Synchromed II ITB pump was implanted with the intrathecal catheter tip placement at T8 level with an initial ITB dose of 50 mcg/day. Postoperatively, she showed good improvement in stiffness and pain. After 10 days, her doses of oral medications could be reduced to 10 mg of diazepam bedtime. At the last follow-up, two months, her ITB dose was readjusted to 200 mcg/day and a diazepam dose of 10 mg/day. Her gait and stiffness improved subjectively by 70-80% [Ashworth scale 2] and spasms disappeared. She became independent for all activities of daily living.
| » Discussion | | |
The SMS variant of SPS is the most amenable to benzodiazepines and other GABA enhancing drugs such as vigabatrin, valproate, tiagabine, gabapentin or baclofen, but the doses required often cause unacceptable side effects. Antibodies against glutamic acid decarboxylase can be detected in some patients. GABA (A)-receptor-associated protein (GABARAP) is a novel protein that is responsible for the stability and surface expression of the GABA (A)-receptor. Up to 65- 70% of SPS patients demonstrate GABA (A)-receptor associated protein antibodies. [8] These GABARAP antibodies significantly inhibit the surface expression of GABA(A)-receptor and thus impair GABAergic pathways.
Some patients require periodic plasma exchange, intravenous immunoglobulin or high dose steroids for symptom relief. [9] However, there are some inherent problems with these treatment modalities. Plasma exchanges require periodic visits and hospitalization for 5-10 days and IVIG is extremely expensive. The cumulative cost of PE and IVIG over time can outstrip that of ITB pump implantation and refill. ITB pump implantation has been shown to improve symptoms in some patients with SPS. [10] The advantages of ITB pump include a targeted baclofen delivery into the intrathecal space which delivers a continuous 24/7 GABAergic therapy and reduces spasms and stiffness at approximately 1/500 th the oral dose of baclofen thus minimizing sedation and systemic side effects. The disadvantages include rare instances of catheter infection, catheter leakage or pump failure with catastrophic muscle spasms and rhabdomyolysis and multiorgan failure. [11]
The cost of a Medtronic synchromed II pump is approximately Rs 3 lakh and the cost of implantation is roughly Rs 25,000. The ITB pump holds 40 ml of baclofen. Depending upon the patient's requirement for baclofen the refill interval can vary from 30-90 days. Each refill costs the patient approximately Rs 24-36,000 per year. The pump lasts for an average of seven years after which it has to be replaced. Thus economically ITB pumps may be beyond the reach of many Indian patients. Although ITB pump implantation is an expensive option, our case report highlights the option of considering it in selected cases of SPS to improve the quality of life.
| » References | | |
| 1. | Levy LM, Dalakas MC, Floeter MK. The stiff-person syndrome: An autoimmune disorder affecting neurotransmission of gamma-aminobutyric acid. Ann Intern Med 1999;131:522-30.  [PUBMED] [FULLTEXT] |
| 2. | Barker RA, Revesz T, Thom M, Marsden CD, Brown P. Review of 23 patients affected by the stiff man syndrome: Clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry 1998;65:633-40. |
| 3. | Grimaldi LM, Martino G, Braghi S, Quattrini A, Furlan R, Bosi E, et al. Heterogeneity of autoantibodies in stiff-man syndrome. Ann Neurol 1993;34:57-64. [PUBMED] |
| 4. | Pagarkar W, Khopkar S, Agrawal M. Stiff-man syndrome. Indian Pediatr 1998;35:904-6. [PUBMED] |
| 5. | Kuruvilla T, Bharucha NE, Thompson EJ, Hawa M, Chauhan V, Agarwal R. Stiff-man syndrome-diagnostic criteria and pitfalls. J Assoc Physicians India 1999;47:836. [PUBMED] |
| 6. | Nikhilesh J, Sayeed ZA, Saravanan PK, Paul J. Stiff person syndrome. J Assoc Physicians India 2001;49:568-70. [PUBMED] |
| 7. | Misra UK, Maurya PK, Kalita J, Gupta RK. Stiff limb syndrome: End of spectrum or a separate entity? Pain Med 2009;10:594-7. [PUBMED] [FULLTEXT] |
| 8. | Raju R, Rakocevic G, Chen Z, Hoehn G, Semino-Mora C, Shi W, et al. Autoimmunity to GABAA-receptor-associated protein in stiff-person syndrome. Brain 2006;129:3270-6. [PUBMED] [FULLTEXT] |
| 9. | Linker RA, Gold R. Use of intravenous immunoglobulin and plasma exchange in neurological disease. Curr Opin Neurol 2008;21:358-65. [PUBMED] [FULLTEXT] |
| 10. | Silbert PL, Matsumoto JY, McManis PG, Stolp-Smith KA, Elliott BA, McEvoy KM. Intrathecal baclofen therapy in stiff-man syndrome: A double-blind, placebo-controlled trial. Neurology 1995;45:1893-7. [PUBMED] |
| 11. | Bardutzky J, Tronnier V, Schwab S, Meinck HM. Intrathecal baclofen for stiff-person syndrome: Life-threatening intermittent catheter leakage. Neurology 2003;60:1976-8. [PUBMED] [FULLTEXT] |
[Figure 1]
| This article has been cited by | | 1 |
A Case of HSP Carrying c.1537-11A > G Mutation of the SPAST Gene Presented as Stiff-Person Syndrome |
|
| Jae-Han Bae, Hae-Bong Jeong, HyeRyoun Kim, Kwang-Sup Song, Sung-Taek Park, Suk-Won Ahn | | Neurology India. 2021; 69(4): 1053 | | [Pubmed] | [DOI] | | | 2 |
Stiff-Person Syndrome: A Treatment Update and New Directions |
|
| Juan Fernando Ortiz, Mohammad R Ghani, Álvaro Morillo Cox, Willians Tambo, Farah Bashir, Martín Wirth, Gustavo Moya | | Cureus. 2020; | | [Pubmed] | [DOI] | | | 3 |
Case Series: Intrathecal Baclofen Therapy in Stiff-Person Syndrome |
|
| Justin Ralph Abbatemarco, Mary Alissa Willis, Robert G. Wilson, Sean J. Nagel, Andre G. Machado, Francois A. Bethoux | | Neuromodulation: Technology at the Neural Interface. 2018; 21(7): 655 | | [Pubmed] | [DOI] | | | 4 |
Paraneoplastic neurological complications of breast cancer |
|
| Ibrahim Fanous,Patrick Dillon | | Experimental Hematology & Oncology. 2015; 5(1) | | [Pubmed] | [DOI] | | | 5 |
Trialing of Intrathecal Baclofen Therapy for Refractory Stiff-Person Syndrome |
|
| J. Camp Newton,Michael E. Harned,Paul A. Sloan,Sara S. Salles | | Regional Anesthesia and Pain Medicine. 2013; 38(3): 248 | | [Pubmed] | [DOI] | | | 6 |
Trialing of intrathecal baclofen therapy for refractory stiff-person syndrome |
|
| Newton, J.C. and Harned, M.E. and Sloan, P.A. and Salles, S.S. | | Regional Anesthesia and Pain Medicine. 2013; 38(3): 248-250 | | [Pubmed] | | | 7 |
Intrapleural migration of a spinal catheter in a patient with arachnoiditis and extensive epidural scarring after tethered cord release: A case report and review of literature |
|
| Anitescu, M. and DaSilva, A.N. and Frim, D.M. | | Neuromodulation. 2012; 15(3): 200-203 | | [Pubmed] | | | 8 |
Intrapleural Migration of a Spinal Catheter in a Patient With Arachnoiditis and Extensive Epidural Scarring After Tethered Cord Release: A Case Report and Review of Literature |
|
| Magdalena Anitescu,Arnaldo Neves DaSilva,David M. Frim | | Neuromodulation: Technology at the Neural Interface. 2012; 15(3): 200 | | [Pubmed] | [DOI] | | | 9 |
Polyanalgesic Consensus Conference 2012: Recommendations for the Management of Pain by Intrathecal (Intraspinal) Drug Delivery: Report of an Interdisciplinary Expert Panel |
|
| Timothy R. Deer,Joshua Prager,Robert Levy,James Rathmell,Eric Buchser,Allen Burton,David Caraway,Michael Cousins,José De Andrés,Sudhir Diwan,Michael Erdek,Eric Grigsby,Marc Huntoon,Marilyn S. Jacobs,Philip Kim,Krishna Kumar,Michael Leong,Liong Liem,Gladstone C. McDowell II,Sunil Panchal,Richard Rauck,Michael Saulino,B. Todd Sitzman,Peter Staats,Michael Stanton-Hicks,Lisa Stearns,Mark Wallace,K. Dean Willis,William Witt,Tony Yaksh,Nagy Mekhail | | Neuromodulation: Technology at the Neural Interface. 2012; 15(5): 436 | | [Pubmed] | [DOI] | | | 10 |
Polyanalgesic consensus conference 2012: Recommendations for the management of pain by intrathecal (intraspinal) drug delivery: Report of an interdisciplinary expert panel |
|
| Deer, T.R. and Prager, J. and Levy, R. and Rathmell, J. and Buchser, E. and Burton, A. and Caraway, D. and Cousins, M. and De Andrés, J. and Diwan, S. and Erdek, M. and Grigsby, E. and Huntoon, M. and Jacobs, M.S. and Kim, P. and Kumar, K. and Leong, M. and Liem, L. and Ii, G.C.M. and Panchal, S. and Rauck, R. and Saulino, M. and Todd Sitzman, B. and Staats, P. and Stanton-Hicks, M. and Stearns, L. and Wallace, M. and Dean Willis, K. and Witt, W. and Yaksh, T. and Mekhail, N. | | Neuromodulation. 2012; 15(5): 436-464 | | [Pubmed] | | | 11 |
Stiff-person syndrome (SPS) and anti-GAD-related CNS degenerations: Protean additions to the autoimmune central neuropathies |
|
| Ali, F., Rowley, M., Jayakrishnan, B., Teuber, S., Gershwin, M.E., Mackay, I.R. | | Journal of Autoimmunity. 2011; 37(2): 79-87 | | [Pubmed] | | | 12 |
Stiff-person syndrome (SPS) and anti-GAD-related CNS degenerations: Protean additions to the autoimmune central neuropathies |
|
| Fatima Ali,Merrill Rowley,Bindu Jayakrishnan,Suzanne Teuber,M. Eric Gershwin,Ian R. Mackay | | Journal of Autoimmunity. 2011; 37(2): 79 | | [Pubmed] | [DOI] | |
|
|
|
|
|
|
|
|
