Lateral rectus palsy in Kawasaki disease.

Abstract

Neurologic findings and complications reported with Kawasaki disease include encephalitis, hemiplegia, ataxia, cerebral aneurysm, cerebral embolus, subarachnoid hemorrhage and sensorineural hearing loss.1-10 The only isolated cranial nerve finding reported to be associated with Kawasaki disease is facial nerve palsy.1, 2, 4 We report a child with Kawasaki disease in whom right lateral rectus palsy developed during the subacute phase of the illness. Case report. A 7-year-old girl from the Dominican Republic was admitted to the Emergency Department with a 3-day history of fever to 102-103°F and a pruritic rash. The rash began on her back and thighs and later spread to cover her entire body. The patient also reported a 2-day history of neck pain and frontal headache. She had six to eight episodes of nonbloody, nonbilious vomiting accompanied by nonbloody, nonmucoid diarrhea. There was no history of cough, rhinorrhea, dysuria, photophobia, recent travel or medication use. She was well-nourished, well-developed and tired but cooperative, with a temperature of 103.5°F, heart rate of 160/min, respiratory rate of 28/min and blood pressure of 94/50 mm Hg. There was mild conjunctival injection, dry red lips, injected pharynx, decreased range of motion of the neck on flexion and a positive Brudzinski's sign on physical examination. There was no photophobia, hepatosplenomegaly, costovertebral angle tenderness, lymphadenopathy or extremity changes. On neurologic examination cranial nerves II to XII were intact with 5/5 strength throughout and intact sensation. There were blanching erythematous papules with central clearing over her entire body, including the palms and soles. She was hospitalized with a diagnosis of erythema multiforme and viral syndrome. She received intravenous hydration, acetaminophen and diphenhydramine. The white blood cell count was 9500/mm3 with a differential of 92% segmented neutrophils, 4% lymphocytes, 3% monocytes and 1% eosinophils. The hematocrit was 30% and the platelet count was 203 000/mm.3 Serum electrolytes were sodium of 131 mEq/l, potassium of 4.1 mEq/l, chloride of 102 mEq/l, bicarbonate of 20 mEq/l, blood urea nitrogen of 26 mg/dl, creatinine of 0.9 mg/dl and glucose of 91 mg/dl. Erythrocyte sedimentation rate was 70 mm/h and rapid Group A Strep test (Strep A OIA, Biostar, Boulder, CO) of the pharynx was negative. Cerebrospinal fluid analysis revealed 0 white blood cells/mm3, 17 red blood cells/mm3, glucose of 88 mg/dl and protein of 7 mg/dl. Urinalysis showed a specific gravity of 1.012, pH 5.0, trace protein, 5 to 10 white blood cells/high power field and no red blood cells or bacteria. Noncontrast head computerized tomography was read as normal. She remained febrile, spiking temperatures to 104°F and became increasingly more irritable. On the second hospital day she became tachypneic and oliguric and developed a gallop rhythm. A chest roentgenogram showed cardiomegaly and diffuse pulmonary infiltrates. The patient was given a dose of intravenous furosemide (0.5 mg/kg) for presumed congestive heart failure, with some improvement. Azithromycin therapy was given for presumptive Mycoplasma pneumoniae infection. On the third hospital day the gallop rhythm disappeared, but pulmonary rales and hepatomegaly developed. On the fourth hospital day the patient continued to show signs of congestive heart failure, requiring two more doses of intravenous furosemide (0.5 mg/kg). The rash began to fade but fever, conjunctival injection and erythematous dry lips persisted, and she had a swollen red tongue, bilateral conjunctival injection, tachypnea and diffuse rales. The precordium was hyperactive with normal S1 and S2 sounds, a gallop rhythm and a II/VI systolic heart murmur at the left middle to lower sternal border. The liver edge was 2.5 to 3 cm below the right costal margin, and she had mild pitting edema of both ankles. The diagnosis was changed to Kawasaki disease, and an echocardiogram was performed which revealed a dilated left coronary artery measuring 3.6 to 3.8 mm, a dilated left ventricle with normal shortening fraction, tricuspid regurgitation, mild increase in right ventricle pressure and a posterior pericardial effusion measuring 7 to 8 mm. Intravenous gamma-globulin and high dose aspirin were given. By her fifth hospital day the patient's fever began to abate and desquamation of both upper extremities appeared. Later that evening she complained of double vision. Examination revealed a right sixth cranial nerve palsy. Slit lamp examination was normal. On the sixth hospital day the patient was afebrile, clinical signs of congestive heart failure improved and she became more playful and interactive. The platelet count was now 501 000/mm3 and the following day it rose to 713 000/mm3. She continued to be afebrile and playful, but the right sixth cranial nerve palsy persisted. On the 11th hospital day noncontrast magnetic resonance imaging of the brain was normal. A repeat lumbar puncture had a cerebrospinal fluid white blood cell count of 2 cells, red blood cell count of 0, glucose of 49 mg/dl and protein of 21 mg/dl. The patient was discharged on the 12th hospital day with low dose aspirin therapy. A follow-up examination approximately 6 weeks later demonstrated complete resolution of the right lateral rectus palsy. A repeat echocardiogram at that time was normal except for persistent left ventricle dilatation. Discussion. Kawasaki disease is diagnosed based on clinical findings, which include fever for >5 days, along with four of the following five criteria: bilateral conjunctivitis, mucositis, extremity changes, rash and cervical lymphadenopathy.11 In atypical Kawasaki disease there is fever, fewer than four criteria and coronary artery aneurysms.8, 12, 13 From 7 to 10% of patients with Kawasaki disease have atypical/incomplete presentations.13-14 Our patient only had fever and three of the five criteria (conjunctivitis, change in mucous membranes and rash) at the time of initial presentation and coronary artery dilatation demonstrated on echocardiogram. Central nervous system findings have been reported in Kawasaki disease. Although 10% of patients have some neurologic finding during the course of their illness, such as aseptic meningitis, irritability and lethargy, the reported rate of neurologic complications including facial nerve palsy, seizures, ataxia, encephalitis, hemiplegia and cerebral infarct is 1.1%.1, 3, 8-11, 15 Sensorineural hearing loss, usually transient, was associated with the acute vasculitis of Kawasaki disease in 7 of 23 children.7 Twenty-five patients have been reported in the literature as suffering from a facial nerve palsy.1, 2, 4 All cases were unilateral and all were of the lower motor neuron. The facial nerve palsy with Kawasaki disease is more common in female patients (1.75:1), although the overall incidence of Kawasaki disease is more common in male patients (1.5:1). Fifty-eight percent of children with facial nerve palsy had aseptic meningitis, whereas reports of cerebrospinal fluid pleocytosis in all children with Kawasaki disease who had lumbar puncture was only 25 to 40%.1, 3, 11, 16 Fifty-two percent had coronary artery aneurysms. Mortality was increased to 8% for Kawasaki disease associated with facial nerve palsy, as opposed to 1 to 2% without facial nerve palsy.1 Neuropathology changes found on autopsied patients with Kawasaki disease have included meningeal thickening, focal stroke, brain edema, venous congestion and tonsillar or uncal herniation.5 Perivascular infiltrates of lymphocytes and large mononuclear cells were seen in the meninges and brain parenchyma. Ganglionitis and neuritis of cranial and peripheral nerves were seen, consistent with a primary vasculitic inflammatory process in the nervous tissue.1, 5 Because Kawasaki disease is a systemic vasculitis of medium-sized blood vessels, it is most likely that the central nervous system findings are secondary to ischemic effects caused by either neuritis or vasculitis of the arteries supplying the nerves. Magnetic resonance imaging is better than computerized tomography scan in detecting these changes.3,10 To our knowledge the finding of a sixth cranial nerve palsy has not been reported. This patient had normal computerized tomography, magnetic resonance imaging and slit-lamp examination. Besides a right lateral rectus palsy causing a complaint of diplopia, she also initially complained of a frontal headache and neck pain. Cerebrospinal fluid obtained at initial presentation and again on Hospital Day 11 were normal. There were no other findings on neurologic examination, other than the right sixth cranial nerve palsy. This finding is most likely a vasculopathic phenomenon secondary to Kawasaki disease that resolved spontaneously. Clinicians must be aware of the atypical presentations of Kawasaki disease, so that this diagnosis can be made promptly and therapy can be instituted to prevent disastrous outcomes, most notably coronary artery aneurysms. Furthermore clinicians should be aware of the vast number of complications that can occur in numerous organ systems including, cardiovascular, neurologic, gastrointestinal, musculoskeletal, renal and pulmonary, given that Kawasaki disease is a systemic vasculitis of the blood vessels. For patients suffering from Kawasaki disease complicated by facial nerve palsy, the overall mortality rate is increased. It has been postulated that perhaps this isolated cranial nerve finding indicates more severe disease. Although the pathophysiology of lateral rectus palsy in our patient may be similar to that of patients with facial nerve palsy, it is unclear whether this finding indicates more severe disease. Brenda J. Wurzburger, M.D. Jeffrey R. Avner, M.D. Department of Pediatrics; Division of Pediatric Emergency Medicine; Albert Einstein College of Medicine; Montefiore Medical Center; Bronx, NY