Intrathecal baclofen therapy for stiff-man syndrome and progressive encephalomyelopathy with rigidity and myoclonus

Stayer, C; Tronnier, V.; Dressnandt, J.; Mauch, E.; Marquardt, Gerhard; Rieke, K.; Müller-Schwefe, Gerhard; Schumm, F.; Meinck, Hans‐Michael

doi:10.1212/wnl.49.6.1591

Cited by 71 publications

(37 citation statements)

References 41 publications

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“…Other oral agents have been reported in isolated case reports as effective for symptomatic relief of SPS with a benzodiazepine sparing effect (table 4). Intrathecal baclofen can be used as a rescue therapy for severe SPS and PERM 90 91. However, catheter malfunction is not uncommon and caution should be taken as sudden interruption of the intrathecal supply may lead to severe symptomatic withdrawal and even death from dysautonomia 91.…”

Section: Treatmentmentioning

confidence: 99%

Stiff-person syndrome: insights into a complex autoimmune disorder

Baizabal‐Carvallo

Jankovic

2014

J Neurol Neurosurg Psychiatry

159

142

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Stiff-person syndrome (SPS) is characterised by progressive rigidity and muscle spasms affecting the axial and limb muscles. Since its initial description in 1956, marked progress has been made in the clinical characterisation, understanding of pathogenesis and therapy of this disorder. SPS can be classified according to the clinical presentation into classic SPS and SPS variants: focal or segmental-SPS, jerking-SPS and progressive encephalomyelitis with rigidity and myoclonus. Most patients with SPS have antibodies directed against the glutamic acid decarboxylase, the rate-limiting enzyme for the production of the inhibitory neurotransmitter γ-aminobutyric acid (GABA). Antibodies directed against GABA(A) receptor-associated protein, and the glycine-α1 receptor can also be observed. Paraneoplastic SPS is commonly associated with antiamphiphysin antibodies and breast cancer. Treatment of SPS with drugs that increase the GABAergic tone combined with immunotherapy can improve the neurological manifestations of these patients. The prognosis, however, is unpredictable and spontaneous remissions are unlikely.

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Section: Treatmentmentioning

confidence: 99%

Stiff-person syndrome: insights into a complex autoimmune disorder

Baizabal‐Carvallo

Jankovic

2014

J Neurol Neurosurg Psychiatry

159

142

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“…Pain crises are usually managed with intravenous or subcutaneous opiates. Patients in whom rigidity and spasms are, or become resistant to benzodiazepines can benefit from treatment with levetiracetam [99], intravenous propofol infusion [100], or intrathecal baclofen [101,102]. Focal intramuscular injection of botulinum toxin is a useful adjunct for treating severe rigidity [103], as is muscle afferent block [104].…”

Section: Managementmentioning

confidence: 99%

Stiff Person Syndrome

Duddy

Baker²

2009

Frontiers of Neurology and Neuroscience

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Stiff person syndrome (SPS), stiff limb syndrome, jerking SPS and progressive encephalomyelitis with rigidity and myoclonus (PERM) are a family of rare, insidiously progressive diseases of the central nervous system. They all share the core clinical features of appendicular and axial rigidity caused by continuous involuntary motor unit activity, and superimposed stimulus-sensitive spasms. There is good evidence for a primary auto-immune aetiology. Anti-glutamic acid decarboxylase (anti-GAD) antibodies, specifically to the GAD65 isoform, are present in serum or cerebrospinal fluid of 60-80% of patients with SPS and its variants. A paraneoplastic form of SPS is recognized in about 5%, associated with a different profile of auto-antibodies. Repeated intravenous immunoglobulin is the mainstay of disease-modifying therapy in SPS. Rigidity and spasms may be treated symptomatically with benzodiazepines, baclofen, tiagabine and levetiracetam. After an initial progressive phase, patients with SPS generally stabilize over a period of months to years. However, 10% will require prolongedadmission to intensive care at some stage during the disease. Sudden death has been reported in asmany as 10% of patients because of unexplained metabolic acidosis or autonomic crises. The prognosis in paraneoplastic SPS, jerking SPS and PERM, in terms of mortality, is generally worse than in primary SPS.

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“…The literature concerning the pathomechanisms of catheter dislocation is scarce. Extensive patient movements especially inclination and reclination [17], or muscle spasms [18] are thought to be causing factors.…”

Section: Discussionmentioning

confidence: 99%

Epidural Granuloma by Dislocated Catheter Tip Associated with Spinal Cord Compression in High-Dose Intrathecal Morphine Therapy

R¹

2013

General Med

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Intrathecal drug delivery systems are an option for selected patients with chronic pain. Complications such as intrathecal catheter-tip inflammatory masses are already reported in literature. In the present case report we describe a patient suffering from neuropathic pain located on the anterior aspects of both thighs following failed back surgery. Two years after implantation of an intrathecal morphine pump system the patient experienced symptoms of a spinal cord compression with increasing radicular pain, especially after application of morphine bolus. Within this time frame the average daily intrathecal morphine hydrochloride dose was 3.2 mg per day and the total neuraxial morphine hydrochloride dose was 2460 mg. Magnetic resonance imaging revealed spinal cord compression from an epidural mass measuring 13×11×10 millimeters, originating from an epidural dislocation of the intrathecal catheter. After surgical removal of the mass, histological examination confirmed a disseminated formation of a granuloma. What makes this case so special, is the fact that the granuloma is not located intrathecally, but in the epidural space (epidural granuloma). The formation of a purely epidural granuloma due to a dislocated catheter tip has -according to our research -not been reported about up to the present.

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Intrathecal baclofen therapy for stiff-man syndrome and progressive encephalomyelopathy with rigidity and myoclonus

Cited by 71 publications

References 41 publications

Stiff-person syndrome: insights into a complex autoimmune disorder

Stiff-person syndrome: insights into a complex autoimmune disorder

Stiff Person Syndrome

Epidural Granuloma by Dislocated Catheter Tip Associated with Spinal Cord Compression in High-Dose Intrathecal Morphine Therapy

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