Treatment of stiff‐man syndrome with intravenous immune globulin

Karlson, Elizabeth W.; Sudarsky, Lewis; Ruderman, Eric; Pierson, Susan; Scott, Mary Jane; Helfgott, Simon M.

doi:10.1002/art.1780370621

Cited by 53 publications

(30 citation statements)

References 14 publications

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“…Immunotherapy has been reported to be a good therapeutic approach in these patients, but results are controversial. The use of intravenous gammaglobulins in a subgroup of patients with stiff-person syndrome seems to be of therapeutic value [44], as in other immunological diseases affecting the nervous system.…”

Section: Clinicalmentioning

confidence: 99%

Movement disorders of autoimmune origin

Valldeoriola¹

1999

Journal of Neurology

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In recent years there has been renewed interest in the role of autoimmunity in many neurological disorders such as multiple sclerosis and neurodegenerative diseases. Research advances in this field have led to the discovery of new potential therapeutic strategies. There are, however, only a few neurological disorders in which an autoimmune origin has been adequately documented and definitely confirmed. The most important neurological diseases causing movement disorders in which an autoimmune basis has been demonstrated are summarized in this review. The possible role of immunity with the most frequent movement disorders such as parkinsonism or dystonia is also commented.

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Section: Clinicalmentioning

confidence: 99%

Movement disorders of autoimmune origin

Valldeoriola¹

1999

Journal of Neurology

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“…Patients not responsive to or unable to tolerate corticosteroids can be treated with intravenous immune globulin (IVIG) given over 3-5 days [9,10]. The duration of the beneficial effects of IVIG varied from 6 weeks to 1 year.…”

mentioning

confidence: 99%

Stiff person syndrome (SPS) complicated by respiratory failure: successful treatment with rituximab

Qureshi

Hennessy

2011

J Neurol

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Dear Sirs, SPS is characterized by axial rigidity, progressive stiffness, and spontaneous reflex-or action-induced painful spasms of the paraspinal, abdominal and occasionally proximal leg muscles associated with a lumbar hyperlordosis.Most patients have anti-glutamic acid decarboxylase (GAD) antibodies in both serum and cerebral spinal fluid (CSF) with additional evidence of autoimmune disease, including thyroid disorders, insulin-dependent diabetes mellitus (IDDM), adrenal insufficiency, vitiligo, pernicious anaemia and epilepsy [1][2][3][4]. According Solimena et al. [5,6], IDDM is the most thoroughly documented condition, observed in 25% of patients presenting with SPS.A 56-year-old male presented with a 21-year history of type 1 diabetes mellitus and hyperthyroidism, hypertension for 10 years and a 6-year history of SPS (anti-GAD antibody positive). He had a 5-year history of progressive stiffness and painful spasms of his limbs and axial region.Prior to this presentation, he was treated symptomatically for SPS. At the time of admission he was taking Levetracetam 500 mg bd, Gabapentin 1,200 mg tds and Diazepam 10 mg tds. As he was already on these medications for muscle spasticity, its seems unlikely that another symptomatic treatment would have been effective, such as baclofen.In the weeks prior to this admission, there was a gradual worsening of axial muscular spasms along with anxiety attacks, generalized weakness, and anorexia and weight loss.On the day of admission, he was found face down in a field, cyanosed, with occasional jerking movement of his shoulders. He was brought into a regional hospital with a Glasgow Coma Scale score of 6/15 and intubated because of type II respiratory failure. He was transferred to our care for further investigation and management. A trial of extubation with bilevel positive airway pressure failed as he progressively became hypercapnic with episodes of agitation. He was intubated again and treated with Propofol and Lorazepam.He was vitally stable and afebrile. He appeared anxious during his physical examination. His cardiac and pulmonary examinations were unremarkable. The abdominal musculature was firm on palpation. He was confused and had slurred speech. He was initially hypertonic; reflexes were hard to elicit. The plantars were bilaterally flexor. There were no sensory deficits, and cranial nerve examination was normal.His routine laboratory blood tests (FBC, U&E, ESR, CRP, LFTS and glucose) were normal. Chest X-rays, CT thorax, MRI brain and C spine were all normal. An electroencephalogram was normal. Electromyography was not available secondary to equipment malfunction. CSF analysis contained oligoclonal bands only in CSF. Anti-GAD antibodies were positive with high titres [1,000; anti-MUSK antibodies and anti-Ach receptor antibodies were negative. Paraneoplastic antibody screening (anti-Hu, antiYo, anti-Ri, VGKC, anti-Purkinje, VGCC, anti-Ma, amphiphycin, NMDAR) was negative.A number of trials of extubation failed, and a tracheostomy tube was inserted for long-term...

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“…Several case reports have also shown a beneficial effect in the treatment of SMS [20,21,24]. Our patient received IVIG treatment once per week for 3 months in addition to plasmapheresis and prednisone.…”

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confidence: 78%

“…Response to plasmapheresis has been inconsistent with both a beneficial response and no effect being reported [15][16][17][18][19]. Improvement following steroid treatment or intravenous immune globulin administration has also been reported in several cases [20][21][22][23][24]. We report a patient with both SMS and type 1 diabetes who did not fully respond to the conventional treatment with diazepam and baclofen.…”

mentioning

confidence: 90%