1. Facial Nerve Palsy and Kawasaki Disease:
Case Report and Review of the Literature
Robert C. Stowe, M.D.
Department of Pediatrics, Baylor College of Medicine-PGY1
Abstract
Results
There are multiple known neurological complications associated with Kawasaki disease (KD). A well-established, but still rare, complication is a cranial nerve VII palsy. Previous reports suggest these patients tend to be younger, female, and have a higher rate of coronary artery (CA) aneurysm compared to KD patients without facial palsy. In this report, a 15-month-old patient diagnosed with KD who develops an acute, transient, left-sided facial palsy is described. Her case makes 39 cases described in the literature; these cases fail to demonstrate a clear sexual predilection but there is a clear risk association with CA aneurysm evident of which clinicians should be aware.
With the inclusion of this case, there have been a total of 39 cases of cranial nerve VII palsy described (Table 1). The age of KD patients who develop facial palsy tend to be younger than 25 months of age. The median age is 8.5 months with an average of / months. Using Grubbs’ test, the 156-month-old child is a statistically significant outlier; removal of this data point produces a median age of 8 months and average of / months. The median onset of facial palsy is 16 days with an average of /- 7.3 days. The median time to resolution of the facial palsy is 16 days with an average of / days.
From the available data, there were 17 females and 15 males; this female to male ratio is 1.1:1. The vast majority of cases are unilateral with 10 cases affecting the right side and 21 cases affecting the left side; only one case has described bilateral facial palsy (with unilateral onset and resolution at differing times albeit occurring concurrently). Coronary artery aneurysms were noted in 23 of 34 patients with facial nerve palsy (67.6%).
Introduction
Kawasaki disease (KD) is an acute febrile illness of childhood characterized by vasculitis of medium-sized extra-parenchymal arteries, with a predilection for CA. The diagnosis of classical KD is made on basis of prolonged fever at least 5 days in length with at least 4 of 5 principal criteria: changes in extremities, polymorphous exanthem, bilateral conjunctival injection, changes in lips and oral cavity, and cervical lymphadenopathy (1). Multiple organ systems may be affected in KD; up to 25% of children who do not receive intravenous immunoglobulin (IVIG) develop CA aneurysms compared to 3-5% of children who are appropriately treated (2).
Neurological complications have been described in between 1% and 33% of patients; the primary manifestation is extreme irritability, but may also include lethargy, aseptic meningitis, ataxia, seizures, focal encephalopathy, cerebral infarction, transient hemiplegia, sensorineural hearing loss, and cranial nerve palsies (3-5). The last comprehensive review regarding facial nerve palsy and KD was conducted nearly 15 years ago amongst a total of 28 patients and demonstrated a 1.4:1 female predominance (in contrast to 1.5:1 male predilection in KD in general), age of onset between 3- and 25-months, and 54% were noted to have CA aneurysms (6). The latter finding is suggestive that the occurrence of facial nerve palsy is an indicator of increased severity of disease.
Study
Age in Months
Sex
Onset of Palsy (Days)
Side Affected
Duration of Palsy (Days)
CA Aneurysm?
Present Report 15 F 6 L 1
Yes
Kocabaş et al 2014 8 14 2
Alves et al 2011 - 26 30
Kaur et al 2010 4
Lim et al 2009 72 M
R + L
Wright et al 2008 3 25 7
Li et al 2008
Larralde et al 2003 5 11 R 42
Biezeveld et al 2002
156 18 10
Poon et al 2000 24 17 32 No
McDonald et al 1998 12
Busharu et al 1997
1.5
Park et al 1991
Gallagher 1990 90
Kleiman et al 1988
Nigro et al 1988 13 16
Hattori et al 1987 60 9 19
Salo et al 1986
Sabatino et al 1984
Tamai et al 1984 28
Aso et al 1984 20
Terasawa et al 1983 21 22
Meade et al 1982
Amano et al 1980
Morens et al 1980
Death
Nakane et al 1977 37
Murayama et al 1974
Case Report
A 15-month-old, obese but otherwise previously-healthy Hispanic girl presented with a 2-day history of fever to 103.8°F and erythematous, urticarial-appearing rash. Acetaminophen was provided to which the fever was poorly responsive. Initial examination demonstrated an exceedingly irritable female with tachycardia ranging from bpm and a blanchable, erythematous, polymorphous, macular rash with areas of confluence on chest, abdomen, back, extremities, and groin. Initial laboratory investigation was notable for AST 212 U/L (normal U/L), ALT 313 U/L (normal 6-45 U/L), CRP 1.7 mg/dL (normal <1.0 mg/dL), ESR 26 mm/hr (normal 0-20 mm/hr), WBC 15,750/UL with 31.5% bands, B-type natriuretic peptide (BNP) pg/mL (normal <100 pg/mL), and normal urinalysis. Electrocardiogram demonstrated sinus tachycardia without other abnormalities and chest X-ray was normal. Blood cultures and viral studies for Coxsackievirus, enteroviruses, parvovirus B19, and CMV were sent and patient was empirically started on IV cefotaxime.
Over the next three days the patient’s fever was recalcitrant to antipyretics and she was persistently irritable. Her rash became more erythematous and macular, her lips became cracked, oral mucosa appeared inflamed, and her hands and feet became swollen. Echocardiogram was obtained that demonstrated a proximal RCA dilatation measuring 2.7mm in diameter (Z-score 2.46). With this constellation of symptoms and findings, she was diagnosed with KD and started on IVIG (2g/kg) and high-dose aspirin.
On the fifth day of admission, the patient was noted to have mild left ptosis, loss of her left nasolabial fold, and left facial droop. A stat non-contrast head CT demonstrated no focal abnormalities and a new-onset lower motor neuron facial palsy was diagnosed. A repeat echocardiogram was obtained that demonstrated interval increase in dilatation of proximal and mid RCA measuring 3.5mm in diameter (Z-score 4.83). The day after onset of the facial palsy, it had completely resolved. The patient was started on IV methylprednisolone (30mg/kg/day) and received magnetic resonance angiography that demonstrated the proximal RCA was 4.4mm x 4.2mm in diameter at the ostium, but quickly returned to normal caliber; no other vascular abnormalities noted (figure 1).
Over the course of her hospital stay, the patient developed mild anemia and thrombocytosis of 759,000/mm3 (reference range 150, ,000/mm3). Her transaminitis, BNP, CRP, and ESR all improved to normal or near-normal levels prior to discharge. Her hands and feet as well as groin began to have mild desquamation at day of discharge. The patient was discharged on a steroid taper and low-dose daily aspirin with follow-up in rheumatology and cardiology clinics. Repeat echocardiography at 1 month failed to recapitulate aneurysm or ectasia of the proximal right CA.
Table 1: Complete list of previously reported KD patients with facial palsy as denoted by source article.
Discussion and Conclusions
The patient described in this report is notable in that she demonstrated the fastest resolution of her facial palsy compared to all previous reports. Many of her initial laboratory findings are consistent with those previously reported in KD (2). Notable from the literature review is a reduction in presumed female predilection compared to previous reports. The trend of left-sided palsy and age of onset were largely unchanged. The associated risk/presence of CA aneurysm appeared to increase despite more consistent use of IVIG in these patients (data not shown).
The histopathologic features from autopsies of children who died due to KD include aseptic choriomeningitis, leptomeningitis, and ganglionitis and neuritis of both central and peripheral nerves (7). It has been hypothesized that both ischemic vasculitis of the arteries supplying the facial nerve and immunologic mechanisms contribute to the facial nerve dysfunction (3). The occurrence of facial palsy may be suggestive of a greater inflammatory burden such as to explain the increased incidence of CA aneurysms. Another explanation is that many of these patients tend to be outside the expected age range for classical KD. Children diagnosed with KD at extremes of the age spectrum (< 6 months or > 9 years) tend to present as atypical or incomplete KD and are more likely to develop CA aneurysms compared to children diagnosed between the typical age range of 1-4 years (8).
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Figure 1: Magnetic resonance angiography demonstrating a 4.4mm x 4.2mm dilatation of the RCA at the ostium.
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