///2017 Abstract Details
2017 Abstract Details2019-08-02T15:54:53-06:00

Labor Analgesia in a Parturient with Isaac’s Syndrome

Abstract Number: T-76
Abstract Type: Case Report/Case Series

Jordan Brice MD1 ; Natesan Manimekalai MD2

INTRODUCTION: Isaac’s syndrome also known, as Neuromyotonia is a rare neuromuscular disorder characterized by muscle cramping, stiffness, muscle twitching at rest, and delayed muscle relaxation. The symptoms are secondary to antibodies directed against voltage gates potassium channels (VGKC). There have only been approximately 200 cases of this syndrome that have been described and even fewer in gravid patients. The syndrome can be either inherited or acquired. With the number of described cases being so few there is minimal amount of information of the best way to proceed with labor analgesia.

CASE REPORT: A 37-year-old white female G1P0 at 40 weeks gestation presented for induction of labor at term. Patient has a past medical history significant for Isaac’s Syndrome that was diagnosed in 2011 after an extensive workup that included EMG changes consistent with Isaac’s syndrome as well as antibodies against VGKC. At the time of presentation patient reported that her symptoms, which included back and upper extremity spasms, has been well controlled with Oxcarbazepine 600mg four times daily. Patient reported symptoms were worse after strenuous activity or in the days preceding her menstrual cycle. After history and physical examination patient was offered regional anesthesia (epidural) for labor analgesia, which she accepted. Epidural was placed without complication and patient delivered a 3.2 kg healthy infant and was discharged two days later.

DISCUSSION: Isaac’s syndrome is a rare neuromuscular disorder that is characterized by antibodies against the voltage gated potassium channels that results in peripheral nerve hyperexcitability. This hyperexcitability translates to muscle cramping, twitching, and delayed muscle relaxation that is often seen in these patients. These myokymic discharges have been shown to persist under general anesthesia as well as peripheral nerve blocks, which could add a layer of complexity to a caesarean section. There have been two forms described in the literature, an inherited type which follows an autosomal dominant inheritance pattern and a much more common acquired type. The mainstay of treatment has been anticonvulsants such as phenytoin and carbamazepine. There have also been cases that have been treated with botulinum toxin, plasma exchange, and corticosteroids. A multidisciplinary discussion is important in coming up with an appropriate treatment plan in the gravid patient as anticonvulsants that are first line therapy are known to be teratogens and have been implicated in neural tube defects, cardiac defects and cleft lip/palate.


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SOAP 2017