Use of pacemaker in GBS dysautonomia

Abstract

Autonomic dysfunction in Guillain-Barrè syndrome (GBS) involves labile hypotension, hypertension, resting tachycardia and sweating. While autonomic involvement affects 66% of patients with GBS, the changes are usually transient and reversible. We hereby delineate a case of a female who presented to our medical centre with flaccid, painless progressive quadriparesis with features of dysautonomia. She had resting tachycardia, was tachypneic with reduced chest expansion and required immediate invasive mechanical ventilation. After pertinent laboratory evaluation, nerve conduction studies were promptly performed at the bedside and findings were concordant with acquired acute inflammatory demyelinating polyneuropathy. The diagnosis of GBS was made on the standard set of investigations and plasmapheresis was initiated on the same day. Her intensive care unit stay was complicated by the multiple episodes of asystole. Even though a temporary transvenous pacemaker was inserted, she, unfortunately, succumbed to a sudden episode of asystole. This paper illustrates that GBS-associated autonomic dysfunction can be severe and close cardiac monitoring is imperative in these patients.

Background

In addition to the motor and sensory disturbances caused by GBS, approximately 66% of patients may also have involvement of sympathetic and parasympathetic nervous systems.¹ Autonomic dysfunction features include: tachyarrhythmia, bradycardia, bradycardia leading to asystole, orthostatic hypotension and labile hypertension.² Besides, focal myocarditis is another serious complication due to cardiovascular involvement.³ Nonetheless, asystole requiring a pacemaker has been rare in literature. So far, few cases have been reported requiring a permanent pacemaker to prevent serious bradycardia. There is a case report in literature from 1984 Dev Naryan et al⁴ reporting a successful case requiring a transvenous permanent pacemaker for fatal cardiovascular autonomic neuropathy due to GBS. Our main purpose to report this case is to spread awareness among clinicians to consider pacing as a viable option and perform meticulous care of the pacemaker in patients with severe sympathetic and parasympathetic autonomic involvement in GBS.

Case presentation

A 55-year-old woman presented to our hospital with acute-onset, painless, symmetric and bilateral lower limb weakness for the past 5 days on 25 December 2019. The patient initially experienced difficulty in walking properly. Subsequently, the weakness progressed to involve her upper limbs. It started distally, with difficulty holding things such as a glass of water and needed assistance in daily activities. Later, weakness progressed more proximally with difficulty in raising her arms above her head and unable to dress and undress without assistance. She then became bed-bound and felt heaviness in her legs and arms but experienced no paresthesia and/or numbness. She also had dizzy spells, on sitting up from a lying position. She reported diarrhoea associated with fever 1 week before her current presentation, which resolved after taking over-the-counter medications. There was no history of diaphoresis, urinary and faecal incontinence, recent vaccinations or respiratory infections. Her medical history included diabetes mellitus type 2 for the past 10 years, with good glycaemic control on insulin 70/30. There was no prior evidence of underlying diabetic neuropathy.

On admission to the emergency department (ED), she was conscious, aware and followed commands. Although the speech was coherent and intelligible, she had difficulty completing her sentences due to breathlessness. Her blood pressure was 150/100 mm Hg in supine and 130/80 mm Hg after 3 min of sitting. She had a resting tachycardia at 138 beats/min, with the respiratory rate at 38 breaths/min. She was maintaining oxygen saturation of 98% on room air but was using accessory muscle for breathing. She was unable to cough and her single breath count was 10. Chest expansion was less than 2 cm. On motor examination of limbs, muscle strength was 1/5 in both upper limbs and 0/5 in both lower limbs on Medical Research Council UK Scale (MRC) both proximally and distally. Muscle tone was decreased in all limbs, whereas muscle bulk was normal. Tendon reflexes were diminished. No numbness and paresthesia were evident on sensory examination.

Investigations

Electro diagnostic studies revealed delayed motor latencies, conduction block and prolonged F waves, consistent with the diagnosis of acquired acute demyelinating motor neuropathy with generalised polyradiculopathy. Sensory nerves were spared. Cerebrospinal fluid showed increased protein 75 mg/dL, with 10 white cells, and glucose 45 mg/dL, depicting albumin-cytological dissociation.

Antiganglioside antibodies were not carried out due to non-availability at our institute.

Serum levels of sodium and potassium were 138 and 4.5 mEq/L, respectively. Serum alanine aminotransferase was 25 IU/L, aspartate aminotransferase 28 IU/L, and serum creatinine 0.9 mg/dL, and serum albumin 2.5 mg/dL.

During her stay in ED, her respiratory rate increased to 45 breaths/min, and she became hypotensive (70 mm Hg systolic blood pressure). Her arterial blood gases analysis revealed pH 7.25, PaO₂ 55 mm Hg, PaCO₂ 50 mm Hg.

Diagnosis

In the light of history and examination, she was diagnosed with Guillain-Barre Syndrome, further confirmed with electrophysiological studies.

The most pressing matter was severe dysautonomia. The need for multidisciplinary team involvement was recognised immediately. Cardiology and intensive care teams were called.

Treatment

She was immediately transferred to Intensive Care Unit and rapid sequence intubation was done and she was put on pressure support ventilation. Inotropes were started with dopamine infusion and fluid boluses of normal saline were administered. A double lumen catheter was placed and urgent plasmapheresis was initiated on stabilisation of blood pressure.

On her day 2 of admission in ICU, in consideration of unpredictable blood pressure readings, inotropes were discontinued.

On day 3, her blood pressure recordings were high and sustained (table 1). She was then given a short-acting beta-blocker, labetalol. Her blood pressure remained labile, with a maximum of 200/120 mm Hg to a minimum systolic blood pressure of 70 mm Hg. She was also initiated on nitroprusside infusion for a short period to control labile hypertension. Numerous episodes of bradycardia and hypotension were noted.

Table 1.

Labile blood pressure (BP) recorded with swinging pulse rate, due to severe dysautonomia

Dates	Blood pressure (mm Hg)	Heart rate (beats/min)	Treatment given
25 December 2019 (First day of admission)	150/100	120	First session of plasmapheresis
	60 SBP		Dopamine infusion and 0.9% N/saline
	70/40
	100/60
	80/40
	190/120
26 December 2019	120/60	130
27 December 2019	190/60	125	Second plasmapheresis
	74/40	50	500 mL 0.9% N/saline fluid bolus
	240/120	40	Intravenous labetalol
	220/110 200/120		Intravenous labetalol
28 December 2019 (Fourth day)	90SBP Asystole	25	Atropine CPR
29 December 2019	70/40 100/40 80/50	60 160 170	Third plasmapheresis 500 mL 0.9% N/saline fluid bolus
	240/120 80SBP	140 30 Asystole	Nitroprusside infusion CPR
31 December 2019	160/100	120	Fourth plasmapheresis
	170/120	112
02 January 2020	80 SBP	45 (triggered by suctioning) Asystole	Fifth plasmapheresis Atropine CPR
	90 SBP	40	Atropine
05 January 2020	60 *SBP	30	Atropine+500 mL 0.9% N/ saline fluid bolus
	80SBP
06 January 2020
07 January 2020	Pacemaker inserted 80SBP 80/40 70SBP	80 90 85 90
08 January 2020	60SBP
	80SBP

*Systolic Blood Pressure

On her day 4 in ICU, during posture change, there was an episode of severe bradycardia, heart rate fell to 25 beats/min and systolic blood pressure to 70 mm Hg, which led to asystole (figure 1). She was revived after chest compressions. Asystole episode duration was noted to be 40 s. She then suffered a total of five episodes of asystole since her admission day and she was revived with chest compressions at each instance. The episodes of asystole were evoked by either tracheobronchial suctioning or posture change but spontaneous episodes also occurred. The mean duration of the asystole episode was documented to be of 40 s. The longest pause observed was of 60 s.

Figure 1.

ECG of patient showing bradycardia resulted in asystole.

After five sessions of plasmapheresis, a mild improvement in strength was observed. Motor power in upper limbs and lower limbs was 1/5 on MRC UK scale. The patient was weaned off the ventilator and extubated on day 10 in ICU. She suffered another episode of asystole on the same day and was reintubated.

After discussion with the cardiology team and based on available literature search, the decision to insert a temporary trans venous pacemaker was made. On day 12 in ICU, a single chamber, VVI mode, trans venous pacing was done by an interventional cardiologist. The pacemaker was programmed at 60 beats/min. She remained stable without the need for resuscitation for the next 2 days. Despite the pacing, she continued to experience hypotension. However, no episode of asystole was documented for 2 days.

Outcome and follow-up

On day 14 in ICU, she had hypotension that was refractory to fluids and to inotropic. On the same day, she had another episode of asystole and cardiopulmonary resuscitation was done but the patient could not be resuscitated declared dead. Electrolyte abnormalities (hypokalaemia) and acid–base disorder (hypoxia, Acidosis) were ruled out as a possible cause of malfunction of the pacemaker or cardiac arrest. Pacemaker failure was thought to be the cause of cardiac arrest; however, the reason for the malfunction could not be determined at that time, unfortunately. Later, we determined that lead displacement was the actual issue that led to the pacemaker failure.

Discussion

Dysautonomia is a well-documented feature of GBS with respiratory failure in an intensive care unit. Sudden deaths have been attributed to dysautonomia. Sympathetic and parasympathetic over and under activity in GBS are common and is always a poor prognostic sign.⁵ Cardiovascular instability due to autonomic failure further complicates the course of disease in mechanically ventilated patients.⁶ Clinical manifestations of autonomic failure range from innocuous profuse perspiration to life-threatening tachy or bradyarrhythmias. It can also cause sinus node dysfunction, sinus arrest and refractory hypotensive shock.⁶ Knowledge of varied changes in haemodynamic parameters caused by sympathetic and parasympathetic nervous system dysfunction is essential in the management of a patient with GBS⁷. Presently, there are no recommendations on placement of temporary or permanent pacemaker in GBS dysautonomia. One study by Patel et al⁸ concluded that the effects of autonomic dysfunction are long term even after recovery. The patient in their study was inserted permanent pacemaker on discharge and followed up after 3 months, and revealed good outcome. The possibility of elective placement of a temporary pacemaker should be taken into consideration in every patient with life-threatening dysautonomia. The long-term outcome of a pacemaker in GBS with autonomic dysfunction could not be determined because of the pacemaker failure in our case report.

A literature search was done on PubMed with keywords using “asystole in GBS”, “severe autonomic dysfunction in GBS management”, “pacing an option in GBS dysautonomia” and we came across with total of eight case reports. The case reports summary is outlined in table 2.

Table 2.

Similar Case Reports in Literature

Authors	Year of publication	Country	Age/gender	Clinical presentation	Comorbid	GBS diagnostic testing	Dysautonomia type	Treatment	Outcome	Follow-up
Emmons et al12	1975		NA	NA	NA	NA	The idioventricular rhythm of 40 beats/min	Pacemaker	NA	NA
Maytal et al13	1989	USA	NA		NA	NA	Labile blood pressure Profound bradycardia Occasional asystole	Pacemaker	NA	NA
Tonelli et al14	2007	USA	NA	NA	NA	NA	Asystole Sustained ventricular tachycardia Peaked T-waves
Patel et al8	2009	USA	18/F	Bilateral limb weakness and paresthesia	Nil	CSF Albumino-cytological dissociation	Bradycardia asystole episodes	IVIGs Intravenous immune globulins Permanent pacemaker	Recovered
Ha et al15	2017	New York, USA	76/M	Acute onset diaphoresis Altered mental status		CSF Albumino-cytological dissociation	Bradycardia Refractory hypotension	Plasma- Pheresis	Death due to Multiorgan Failure	NA
Kordouni et al16	2007	North Chicago, Illinois, USA	NA	Miller Fischer Syndrome	NIL	NA	Bradycardia and asystole	NA Pacemaker		NA
Kaushik et al17	2005	46/M		Severe ataxia Ophthalmo plegia Areflexia	Liver transplant on tacrolimus	NA	Symptomatic Sinus pauses	IVIGs Discontinuation of tacrolimus Pacemaker	Recovered	NA
Shiraiwa et al18	2017	Tsukuba	68/M	Truncal Ataxia Dysarthria Diplopia		Positive Anti GQ1b Antibody and autoantigen Anti-ganglioside antibody	Bradycardia Sinus arrest	IVIGs	Sinus arrest resolved itself	Recovered

CSF, cerebrospinal fluid; NA, not available.

Although complete pacemaker failure is rare in literature. Pacemaker malfunction can result from varied reasons. Causes of pacemaker failure contains depletion of battery, fracture of wire, displacement of lead and issues related to pacing and sensing.⁹ Pacing failure is mostly due to the capture and or inability of the pacemaker to produce an impulse in a heart rate. Causes of failure to generate an impulse comprise a broken wire, and over sensing and crosstalk causing inhibition. Failure to capture occurs due to the dislodged wires, and fibrosis at the lead implantation site. Acidosis and hyperkalaemia are also contributing factors in capture failure.¹⁰ Sensing dysfunction includes undersensing and oversensing leads' asynchrony and inappropriate inhibitions¹¹

Pacemakers are complex electronic devices and most clinicians working in ICUs, EDs are not fully equipped or have the expertise to recognise and evaluate the problems originating from pacemaker failure. Therefore, the cardiology and the electrophysiological team should be involved in the care of the patient immediately.

Learning points.

• Hospital staff and doctors should be cognizant of the presence of irregularities in haemodynamic parameters and their sensitivity to the manipulation of patients in a critical care unit.

• When bradycardia and hypotension (sinus node dysfunction) is refractory to treatment with atropine infusion or other pharmacological measures, the pacing is life saving and should be taken into account.

• Medical staff and doctors should be extravigilant in not dispositioning the wires or tubes connected to the patient during routine care of the patient.

• A temporary pacemaker may be a plausible intervention to consider in patients with severe autonomic disturbances in Guillain-Barrè syndrome.

• The electrophysiological and cardiology team should be involved early in care and management not only for inserting the pacemaker device but also in routine care and evaluation of pacemaker on daily basis to avoid unfortunate circumstances.

Ethics statements

Patient consent for publication

Next of kin consent obtained.