Belatedly diagnosed acute motor axonal neuropathy after cardiac surgery

doi:10.4236/crcm.2013.27105

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Vol.2, No.7, 395-398 (2013) Case Reports in Clinical Medicine

http://dx.doi.org/10.4236/crcm.2013.27105

Belatedly diagnosed acute motor axonal neuropathy

after cardiac surgery

Chong Tae Kim1,2, Todd Beery2

1Division of Rehabilitation Medicine, The Children’s Hospital of Philadelphia, Philadelphia, USA;

*Corresponding Author: [email protected]

2Department of Physical Medi cine an d R ehabilitatio n, University of Pennsylvania, Philadelphia, USA

Received 18 March 2013; revised 18 April 2013; accepted 10 May 2013

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Surgery has been reported a rare cause of Guil-

lain-Barré syndrome (GBS), but a recent retro-

spective study reported a much higher inci-

dence rate for post-surgical patients. There are

several case reports of GBS presenting after

cardiac surgical procedures. All these cases

were diagnosed as acute inflammatory demye-

linating polyradiculoneuropathy (AIDP). We de-

scribed a case of acute motor axonal neuropa-

thy (AMAN) after cardiac surgery. Clinical fea-

tures were reviewed along with spinal magnetic

resonance imaging (MRI) and cerebral spinal

fluid (CSF) analysis. Sequential electrodiagnos-

tic studies (EDx) were performed. This case

represented a rare complication of AMAN with

urinary retention after cardiac surgery.

Keywords: Guillain-Barré Syndrome; Acute Motor

Axonal Neuropathy; Cardiac Surg ery; Sequential

Electrodiagnosis; Urinary Retention

1. INTRODUCTION

Gullain-Barre syndrome (GBS) most commonly de-

velops following antecedent upper respiratory or gastro-

intestinal infections. Other risk factors for GBS include

vaccination and surgery. GBS is classified into three

types, based on electrodiagnostic findings, which in-

clued acute inflammatory demyelinating polyradiculon-

europathy (AIDP), acute motor axonal neuropathy

(AMAN), and acute motor sensory axonal neuropathy

(AMSAN). AIDP is the most common, and AMSAN is

the rarest. Several cases of post-operative GBS have

been reported [1-6]. To our knowledge, there are no

reported cases of AMAN after an operative procedure.

Herein, we reported a case of AMAN with urinary re-

tention that developed after cardiac surgery. The diagno-

sis of AMAN was confirmed by serial electrodiagnostic

studies (EDx).

2. CASE PRESENTATION

A 24-year-old female with congenital heart disease

(tetralogy of Fallot and absent pulmonic valve) and a

history of prior cardiac heart surgery (main pulmonary

artery banding, ventricular septal defect closure, right

ventricle to pulmonary artery conduit insertion, and right

pulmonary artery placation) as a 2-year-old presented to

a cardiology department of a university children’s hospi-

tal with a gradual decline in general strength and endur-

ance. Prior to developing these symptoms, her physical

function was normal. She was found to have interval

changes of pulmonary stenosis, tricuspid regurgitation,

and aneurismal formation of the left pulmonary artery.

Consequently, she underwent an elective pulmonic valve

replacement, revision of the right ventricle to pulmonary

artery conduit, and left pulmonary artery placation under

general anesthesia. The procedure was completed using

the standard cardiopulmonary bypass and was uneventful.

Preoperatively, she denied having any respiratory or gas-

trointestinal symptoms. Cefazolin was given prior to the

procedure. Total bypass time of the procedure was 89

minutes, and no blood products were transfused. She was

extubated without complications. The medications given

postoperatively consisted of intravenous vasopressors (dis-

continued within 24 hours), furosemide, docu sate, senna,

nalbuphine hydrochloride, and hydromorphone. On post-

operative day 2, she complained of paresthesia and weak-

ness in her bilateral lower extremities. Later that day, she

was unable to stand without assistance and had bilateral

foot drop. On physical exam, manual muscle testing re-

vealed 2/5 muscle strength in ankle dorsiflexors and 4/5

elsewhere in the lower extremities. Both ankle and knee

reflexes were not elicited. Cranial n erves remained intact

C. T. Kim, T. Beery / Case Reports in Clinical Medicine 2 (2013) 395-398

396

and upper extremities were full strength. Sensations to

pain, light touch, proprioception, and vibration were all

preserved symmetrically. An initial magnetic resonance

imaging (MRI) of the thoracolumbar spine performed on

post-operative day 3 indicated no evidence of spina l cord

infarction or abnormal contrast enhancement involving

the cauda equina. Her post-operative course was also

significant for urinary retention, and she was treated for a

urinary tract infection with oral antibiotics (cotrimoxa-

zole 160 mg twice a day) for ten days, however urinary

retention continued to persist. Over the next several days,

lower extremity weakness progressed to 4/5 in hip flex-

ors, 3/5 in knee extensors, and 0/5 in ankle dorsiflexors,

and 2/5 ankle plantar flexors. On post-operative day 5, an

initial EDx was performed and motor nerves (bilateral

peroneal, right tibial, and right ulnar nerves), sensory

nerves (right radial and sural nerves) conduction studies,

and F waves (right tibial and ulnar nerves) were normal,

respectively. A repeat thoracolumbar MRI on post-op-

erative day 6 demonstrated disseminated enhancement of

the peripheral nerve roots of the cauda equina. Lumbar

puncture was performed on post-operative day 7 and

CSF analysis showed elevated protein (86 mg/dL), nor-

mal white cell count, normal glucose (55 mg/dL), nega-

tive oligoclonal bands, and elevated myelin basic protein

(8.10 ng/mL). Compylobacter testing was not performed.

For treatment, a 5-day course of intravenous immu-

noglobulin (400 mg/kg/day) was completed with no cli-

nical improvement. 13 days after her cardiac procedure,

she was admitted to an inpatient rehabilitation unit with

continued bilateral lower extre mity weakn ess and urinary

retention.

During inpatient rehabilitation, she did not make sig-

nificant improvements, and her bilateral lower extremity

weakness and urinary retention persisted. A second EDx

was performed 7 weeks post-operatively showed re-

markably decreased amplitudes of compound muscle

action potentials (CMAP) of the bilateral peroneal nerves.

Right tibial CMAP amplitude was slightly decreased,

and conduction velocities of the bilateral tibial nerves

were minimally slowed. F wave latency of the left tibial

nerve by stimulation at the ankle was within normal

ranges (Table 1). No conduction blocks or temporal dis-

persions were observed. Sensory nerve action potentials

(SNAP) were within normal ranges in the bilateral sural

and left radial, median, and ulnar nerves. Copious fibril-

lation potentials and positive sharp waves with neuro-

genic recruitment patterns were noted in the left lower

extremity (tibialis anterior, gastrocnemius, biceps femo-

ris, and vastus medialis) and less in the lumbar paraspi-

nal muscles. These findings were consistent with AMAN.

At the time of discharge from inpatient rehabilitation, she

continued to have bilateral lower extremity weakness and

paresthesia in a similar pattern as onset. She used loft-

strand crutches to assist with ambulation, and also con-

tinued to suffer from a neurogenic bladder, requiring

intermittent self-catheterization.

3. DISCUSSION

Post-operative acute peripheral neuropathy may de-

velop after any surgical procedure. It can be caused by

various injury mechanisms, which includes mechanical

injury, ischemia, and altered immune system function

that causes an inflammatory neuropathy. The last is be-

lieved to be the p athophysiology of GBS af ter surgery.

Table 1. Electrodiagnostic study.

Side Nerve (M/S)* Distal latency (R/Ref)† (msec) Distal amplitudes (R/Ref) Conduction velocity (R/Ref) (m/s)

Right Tibial (M) 5.9/<6.1 2.7/3 mV 38/>40

Peroneal (M) 5.3/<6.5 0.55/2 mV 46.2/>40

Left Tibial (M) 5.1/<6.1 3.8/3 mV 39.5/>40

Peroneal (S) 5.4/<6.5 0.69/2 mV 42/>40

Ulnar(M) 3.3/<3.5 8/6 mV 58.2/>50

Median(M) 4.4/<4.2 4.3/4 mV 60/>50

Right Sural (S) 4.3/ 35.9 µV

Left Sural (S) 4.3/ 27.7 µV

Ulnar (S) 3.5/ 18.7 µV

Median (S) 3.5/ 31.7 µV

Left Tibial

‡ 49.5/42 - 56

*M/S, motor/sen s ory; †R/Ref, recorded/reference value; ‡, F wave.

C. T. Kim, T. Beery / Case Reports in Clinical Medicine 2 (2013) 395-398

397

Cases of GBS developing after surgery are reported

with incidence of 5% - 10 %, and are not related to the

type of anesthesia or surgical procedure [1,2]. A recent

retrospective study revealed the relative risk of develop-

ing GBS within the post-operative 6-week period is 13.1

times higher than the general population incidence rate

[2]. All previous cases of GBS after surgery were re-

ported as AIDP with one exception. A case of AMSAN

was reported after a thoracic spine surgery [7]. AMAN is

known to progress faster and reach nadir earlier than

AIDP [8]. This holds true when comparing the onset

times of the cases of post-operative AIDP to the onsets

of our case and the on e case of AMSAN [7] (AIDP post-

operative onset 6 - 40 days versus AMAN and AMSAN

6 - 48 hours). More substantial data is needed to deter-

mine that AMAN or AMSAN develop earlier than AIDP

after surgery.

The pathophysiology of post-operative GBS is not en-

tirely clear. Histologic studies reveal infiltration of ma-

crophages, causing inflammation into the nerve [1]. The

location of the infiltration being in either the axon or

myelin can assist in distinguishing the various subtypes.

Macrophages invade the space between the Schwann cell

and axon not involving myelin in AMAN [9]. Two im-

mune response hypotheses have been proposed to ex-

plain the infiltration process. One of these involves a

T-cell response against antigens on the nerve surface

causing a release of inflammatory mediators [10]. An-

other theory is based on a humoral response from anti-

bodies binding to epitopes on the nerve surface, causing

activation of the complement cascade [11]. There is no

clear understanding on how the two entities are related.

The existing theories regarding the pathophysiology re-

volve around the prin ciple that surgery inherently results

in systemic inflammation [12-14]. The causes of in-

flammation can possibly be induced by ischemia, reper-

fusion injury, and the general trauma of the surgery,

which can consequently cause a humoral and cytokine

response by the immune system. The inflammatory re-

sponse after a cardiopulmonary surgery has been shown

to activate the complement system, causing an increase

of cytotoxic mediators and activation of macrophages,

neutrophils, and T-cells [15]. However, there is no clear

evidence indicating that activating these cells is directly

related to the occurrence of GBS following cardiopul-

monary bypass surgery.

If axonal lesions are limited to the d istal segments, the

recovery is favorable because of good capability of ax-

onal regeneration [16-18]. The poor recovery of our case

can be explained by the lesions involved in proximal

nerve roots as well as distal segments of the peripheral

nerves. As demonstrated in this case, sequential EDx is

necessary to confirm GBS and to classify subtypes, es-

pecially since 10% of cases of GBS have a normal EDx

in the early phase [19,20].

Urinary dysfunction is a common impairment associ-

ated with GBS. The incidence of the impairment is re-

ported in 20% of AIDP cases and 50% of AMAN cases

[21]. Urodynamic studies have demonstrated both detru-

sor overactive and underactive bladders in cases of GBS

[21-23]. Cases of detrusor overactivity in peripheral

nerve lesion is paradoxical, but have been reported in

cauda equina and pelvic plexus lesions [24,25]. Possible

explanations for detrusor overactivity include pelvic

nerve irritation, emphatic transmissions, or an undefined

mechanism [21]. In our case, since the patient deferred

urodynamic study, it is unclear whether the bladder was

overactive or underactive. Since the patient had sustained

urinary retention, it is assumed to be an underactive

bladder.

4. CONCLUSION

To our knowledge, five cases of AIDP were reported

after cardiac operative procedures. This is the first report

of AMAN after a cardiac operative procedure, and re-

quired sequential EDx for diagnosis.

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