Sugammadex in a patient with Sjogren’s syndrome and polymyositis

Abstract

Spontaneous intracranial hypotension (SIH) is a poorly understood entity resulting from CSF leakage or from venous hypotension causing increased CSF absorption. 1 It causes a range of symptoms from orthostatic headaches to coma. MRI and CT myelography are the investigations of choice in demonstrating CSF leaks as presumed sites of dural defects. CT myelography has traditionally been the first diagnostic tool, but this necessitates lumbar puncture for contrast injection and this is both invasive and of significant risk with infra-tentorial herniation. MR myelography has been shown to have comparable detection rates in nerve root leaks and high cervical retrospinal CSF collections, and superior detection rates in spinal leaks. 2 A diagnostic quandary arose in this patient who had serious contraindications to MRI and CT myelography. The presence of a PPM is widely recognized as a contraindication to MRI due to the risk of harmful arrhythmias and heating or displacement of the device or its leads. In a survey asking radiologists and cardiologists whether they would submit a patient to MRI with a PPM in situ, 97% radiologists responded that they would not, whereas 34% of cardiologists responded that they would under appropriate circumstances. 3 There is now a substantial evidence base providing safety data on a large number of MRI studies with PPMs. The devices for which there is most evidence include those manufactured after 1998, in place for over 6 weeks and without epicardial or abandoned leads. 3-7 The number of PPMs will continue to increase due to an ageing population and increasing indications. However, the number of patients with an expanding range of co-morbidities that will benefit from the diagnostic advantages offered by MRI will also multiply. MRI should only be considered when there is no lower-risk alternative to answer clinical questions essential to patient management. This case highlights the commonly overlooked pathology of SIH and raises awareness of the increasing evidence that PPMs are becoming relative rather than absolute contraindications to MRI.

Sugammadex: What do we Know and What do we Still Need to Know? A Review of the Recent (2013 to 2014) Literature

“…Aldosteroma 55 , amyotrophic lateral sclerosis 49,66 , Brugada syndrome 42,51 , Duchenne muscular dystrophy 73 , hepatectomy patients 56 , myasthenia gravis 54,62 , congenital myotonic dystrophy type 1 58 , spinal muscular atrophy type 3 47 , myotonic dystrophy and spinal muscular atrophy 60 , a parturient with myotonic dystrophy 61 , paediatric oncology patients 53 , acute porphyria 64 , pseudocholinesterase enzyme deficiency 63 , severe cardiac failure 65 , Sjögren's syndrome 40 , Strumpell-Lorraine disease 43 and a patient with trisomy 8 mosaicism combined with cerebral palsy 69 .…”

Secondary myopathy due to systemic diseases

“…Rarely, dermatomyositis may occur . Occasionally, Sjögren syndrome may be also associated with polymyositis . In some patients, Sjögren syndrome was even associated with inclusion body myositis .…”

Safety and Efficacy of Sugammadex for Neuromuscular Blockade Reversal

“…54 However, in another report, sugammadex (4 mg/kg) was used in a patient with Sjögren's syndrome and polymyositis, to reverse deep neuromuscular blockade, successfully and without delay, following laparoscopic anterior resection. 55 The authors of this report argue that polymyositis does not affect the neuromuscular junction itself and therefore should not affect the onset of rocuronium action or indeed its reversal with sugammadex. It was postulated that the delayed effect of sugammadex in the first report was because of diminished cardiac output in the elderly patient involved.…”