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1. Goldkamp J. Blaskiewicz R. Myles T. Stiff-person syndrome and pregnancy. Obstet Gynecol. 2011 Aug; 118(2 pt 2):454-7 doi: 10.1097?AOG.0b013e31821696b. Link to article.

A woman diagnosed with stiff person syndrome became pregnant 2 months after her diagnosis. Her medication regimen was adjusted because of pregnancy, and anesthesia was initiated early in labor to control her pain. She was able to have a full-term pregnancy with few complications. Stiff person syndrome may be successfully managed in pregnancy. Patients can deliver vaginally with adequate pain control to avoid muscle spasms.

2. Amyradakis G, Carlan SJ, Bhullar A, Eastwood, J. Pregnancy and Stiff Person Syndrome. The American Journal of Medicine Volume 125, Issue 3, Pages e1–e2, March 2012.  Link to article.

The patient was taken off diazepam at 10 weeks and started on methocarbamol. Three weeks later she had with difficulty swallowing and inability to close her right eye.  She was given artificial tears and prescribed gabapentin at 300 mg 3 times per day. She returned 6 weeks later at 19 weeks gestation with intractable facial paralysis and muscle pain. The neurology consultant prescribed diazepam again at 20 mg 3 times per day and the  duration of her pregnancy was characterized by gestational diabetes but no exacerbation of her muscle spasm. The patient returned at 35 weeks' gestation with spontaneous rupture of membranes in active labor. Nonreassuring fetal heart tone changes occurred, and she delivered by cesarean a 9-lb infant with Apgar scores 3 and 9. The postpartum course was complicated by endometritis treated by intravenous antibiotics and muscle stiffness treated with oral diazepam. She left the hospital on postoperative day 4 in stable condition.

3. Cerimagic D, Bilic E. Stiff-person syndrome first manifesting in pregnancy. Gynecol Obstet Invest. 2009;67(2): 134-6. Doi: 10.1159/000172804.

Link to article

We present a case of the autoimmune form of glutamate decarboxylase-positive SPS that initially manifested in pregnancy. The diagnosis was made based on clinical, laboratory and electromyoneurographic criteria. The patient was administered low doses of diazepam and Baclofen. Considering the clinical picture of SPS patients, caesarean section is the method of choice for pregnancy termination.

Stiff-person syndrome1

Stiff person syndrome (SPS) is a rare neurological dis­order characterized by the presence of fluctuating muscle rigidity and spasms of the trunk and more proximal body parts.

Patients with SPS may present as an emergency with severe pain and spasms of the lumbar paraspinal mus­cles and lower limbs. The spasms, often associated with intense pain, typically begin with an abrupt jerk followed by tonic activity that slowly subsides over seconds to, less commonly, minutes. Rarely, these spasms last days (sta­tus spasticus). The affected muscles are typically extremely hard to palpation with a board-like appearance, leading to hyperlordosis. Muscle spasms can occur spontaneously or be provoked by noise or movement. The spasms may be so forceful as to produce femoral fractures, joint subluxations and even herniation of abdominal contents.

Paroxysmal autonomic dysfunctions, such as transient hyperpyrexia, diaphoresis, tachypnea, tachycardia, pupillary dilation and arterial hypertension, are recognized. Rhabdomyolysis can be a potential complication of the excessive muscle con­tractions in SPS. Sudden death can also occur due to an acute autonomic failure. In a significant number of cases, SPS is believed to be mediated by autoantibodies to glutam­ic acid decarboxylase (anti-GAD) that limit the gamma amino-butyric acid (GABA) neuronal activity and lower the threshold for muscle spasms, other neurologic and psychi­atric features of the disorder. SPS with elevated serum anti-GAD levels may occur with other autoimmune disorders, including diabetes mellitus, Graves’ disease, Hashimoto’s thyroiditis and pernicious anemia. Ten percent of cases with normal levels of this antibody may be related to au­toantibodies against amphiphysin which commonly rep­resent a paraneoplastic syndrome related to breast cancer, mediastinal tumors, small cell lung cancer, Hodgkin’s dis­ease, and colon cancer.

The management of SPS is based on the use of drugs that promote GABAergic inhibition, for example, benzodi­azepines (diazepam and clonazepam) and baclofen. High-dose benzodiazepines can abolish the excessive motor unit activity. Oral baclofen provides relatively modest relief, while intrathecal administration seems to be much more effective. Finally, variable degrees of benefit have been reported with the use of antiepileptic drugs, such as valproic acid, levetirac­etam and gabapentin.

1. Munhoz RP, Moscovich M, Araujo PD, Teive HAG. Movement Disorder Emergencies. Arq Neuropsiquiatr. 2012 Jun;70(6):453-61. Link to article.

2. Baclofen Emergency Protocol (Full PDF)     Link to article.

3. Baclofen Call Flowsheet (Full PDF)     Link to article.

4. Benzodiazepine Toxicity  Link to article.

5. Focus On: Best Practices for Seizure Management in the Emergency Department  Link to article.

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