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Articles |
Fatal Infantile Polyarteritis NodosaWith Predominant CentralNervous System Involvement
From the Department of Neurological Surgery, Northwestern University, Evanston, Ill (D.G.E.), and the Departments of Neurology (S.M.G., W.G.E.), Pediatrics (S.M.G., K.A.T.), and Pathology (W.G.E., J.T.L.), University of California, Davis.
Correspondence to Sidney M. Gospe, Jr, MD, PhD, Child Neurology, University of California, Davis, Medical Center, 2315 Stockton Blvd, Sacramento, CA 95817.
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Abstract |
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Background Infantile polyarteritis nodosa usually presents in children under 2 years of age as a multiorgan system disease with signs of congestive heart failure or renal failure. This disease and Kawasaki disease may share certain clinical and pathological features.
Case Description We describe a child who first presented at 8 months of age with a febrile illness followed by a delay in motor and language development and a mild right hemiparesis. Five years later he died after developing oculomotor dysfunction, hypertension, and intracranial hemorrhage. Autopsy revealed focal segmental necrotizing vasculitis of cerebral arteries, without involvement of coronary or renal vessels.
Conclusions Although this child was evaluated on several occasions during this time period, the diagnosis was not made antemortem. The predominant central nervous system features, both clinical and pathological, together with the prolonged course are the two unique features of this child's disease that need to be emphasized.
Key Words: central nervous system • infant • vasculitis
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Introduction |
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Polyarteritis nodosa (PAN) is an uncommon form of systemic vasculitis in the pediatric age group. Infantile PAN usually presents in children under 2 years of age as a multiorgan system disease, with signs of either cardiac or renal failure. We present a case of infantile PAN with a prolonged clinical course in which arterial lesions were almost exclusively limited to the central nervous system.
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Case Report |
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The child was a healthy, full-term product of an uncomplicated pregnancy who acquired appropriate developmental milestones until approximately 8 months of age. At that time he had a febrile illness accompanied by vomiting, a macular rash over the upper extremities and trunk, leukocytosis, and thrombocytosis, which lasted several weeks. This illness was not associated with mucous membrane involvement, adenopathy, or neurological symptoms.
Thereafter, slow motor and language development and poor weight gain were noted. With the exception of increased lower extremity reflexes, his neurological examination at 11 months of age was unremarkable. He sat at age 12 months and walked at 20 months. After recovering from a left femur fracture sustained at 27 months of age, gait asymmetry was noted. Subsequent neurological examination at 34 months of age revealed a head circumference of 50.5 cm, immature dysarthric speech, and a mild right hemiparesis with increased lower extremity tone, foot drop, and ankle clonus. A magnetic resonance imaging scan of the head demonstrated a left temporal arachnoid cyst and a small cystic lesion in the right thalamus, neither of which accounted for his neurological deficits.
He was admitted to the hospital at age 4
years for evaluation
of a right pupil-sparing third nerve palsy. His neurological
examination showed right-sided hypertonia, hyperreflexia, and sustained
ankle clonus. Cerebrospinal fluid (CSF) examination revealed normal
glucose and protein determinations, 116 red blood cells per cubic
millimeter, and 10 white blood cells per cubic millimeter (86%
lymphocytes, 12% histiocytes, and 2% eosinophils). X-ray computed
tomographic and magnetic resonance imaging with gadolinium scans of the
head did not show new lesions. An infectious etiology was considered,
but serological studies for respiratory syncytial virus, adenovirus,
mycoplasma, Epstein-Barr virus, Lyme disease, and hepatitis were
negative, as was an evaluation for tuberculosis. CSF cultures were
negative, and an evaluation for CSF immunoglobulin production was
unremarkable. After 4 days, the patient was discharged with a
provisional diagnosis of viral meningoencephalitis. Within 2 days he
was readmitted; he was hypertensive (135/100 mm Hg), obtunded, and had
bilateral pupil-sparing third nerve palsies. Renal and cerebral
angiograms were normal, and no etiology for his encephalopathy was
established. Six weeks later he was discharged, receiving oral
antihypertensive medications and tube feedings. Bulbar function
improved rapidly, and within weeks he was taking a general diet. Within
6 months he was ambulatory, but he had persistent oculomotor
dysfunction.
At age 5 years his condition again deteriorated. One day
after experiencing a brief episode of syncope, he developed diarrhea
and vomiting followed by a progressive depression in his level of
consciousness. On admission his blood pressure was 110/74 mm Hg, he
was afebrile, and he aroused only to painful stimuli. His pupils were
equal and reactive to light, and he had a left partial third nerve
palsy, right internuclear ophthalmoplegia, and right hemiparesis. The
CSF contained 21 000 red blood cells per cubic millimeter and 50 white
blood cells per cubic millimeter, with normal glucose and protein
determinations. There were no abnormalities of hemostasis. A computed
tomographic scan of the head showed subarachnoid, intraparenchymal, and
intraventricular hemorrhage (Fig 1
). Angiography did not
demonstrate an aneurysm but showed beaded vessels and vasospasm of
several arteries (Fig 2). To combat vasospasm and
prevent brain ischemia, he was treated with nimodipine and
intravascular volume expansion. At discharge 17 days later, he could
intermittently follow simple commands, he smiled to familiar faces, his
oculomotor signs were unchanged, and he had marked bilateral
spasticity. Six weeks later he was found unresponsive, pulseless, and
apneic. He died almost 5 years after the initial presentation of
his illness.
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At autopsy the brain was swollen and weighed 1300 g. Gross
neuropathologic findings included a 3-cm arachnoid cyst beneath the
left temporal pole and hemosiderin in the leptomeninges of the
brain base and in the interhemispheric and left lateral fissures. A
5-mm saccular aneurysm of the left middle cerebral artery trifurcation
and a 2-mm aneurysm at the junction of the left pericallosal and
anterior cerebral arteries were present. Cerebral coronal sections
showed mildly dilated ventricles; a 1-cm old hematoma in the anterior
septum pellucidum; a 3-mm right medial thalamic lacunar infarct; mild
gliosis in the adjacent lateral thalamus and right internal capsule;
and three large, partially cystic wedge-shaped infarcts of intermediate
age in the left superior frontal and cingulate gyri and corpus callosum
genu, in the right anterior cingulate gyrus, and in the left superior
temporal gyrus, insula, and inferior parietal lobule. In the rostral
midbrain, multiple small, irregular, partially cystic infarcts involved
much of the left oculomotor nucleus and nerve, the medial longitudinal
fasciculus bilaterally, the left red nucleus, and the medial one half
of the left substantia nigra and cerebral peduncle. The cerebral
vasculature showed inflammation ranging from mild mixed infiltrates to
complete destruction of vessel walls. The characteristic pattern of
focal segmental necrotizing vasculitis of the PAN type was
seen (Fig 3). Occasional healed arteritis lesions
were seen in the testes and pancreas. No coronary or renal vascular
lesions were found.
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Discussion |
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PAN is a systemic disease with multifocal, segmental, necrotizing vasculitis of small and medium-sized muscular arteries, which sometimes results in "blow-out–type" microaneurysms.1 PAN is uncommon in infants and children, and the infantile form is often considered a separate clinicopathologic entity from classic PAN.
Infantile PAN generally occurs before the age of 2 years and most commonly affects the coronary arteries, often resulting in aneurysm formation in these vessels.2 3 These patients commonly present with congestive heart failure. Other organs may be affected in a similar manner, leading to renal failure and severe gastrointestinal and central nervous system dysfunction. Pathologically, infantile PAN is frequently indistinguishable from fatal cases of Kawasaki disease, and these two conditions may share certain clinical features.3 4 5 6 7 8 However, while the diagnosis of Kawasaki disease is usually made on clinical grounds, infantile PAN is frequently diagnosed at autopsy,8 as was the case with our patient.
Childhood PAN, on the other hand, is more similar to the adult form in both the clinical manifestations and the anatomic distribution of vascular lesions. Neurological involvement, most commonly seen in the form of peripheral neuropathy, develops in 30% to 70% of patients.4 9 There are few reports of cranial nerve involvement. In a series of 36 biopsy-proven cases of childhood PAN, Topaloglu and colleagues10 described four patients with cranial nerve lesions. Central nervous system pathology is thought to occur in 15% to 65% of cases of childhood PAN,11 12 13 14 but cases with predominant central nervous system lesions are extremely rare.
At 8 months of age, our patient presented with a febrile illness that, in retrospect, was likely the first clinical manifestation of vasculitis. Although this illness was associated with prolonged rash and fever, it was not consistent with Kawasaki disease. In particular, the acute illness was not accompanied by either cervical lymphadenopathy or mucous membrane involvement. Almost 5 years later the patient died from infantile PAN, which predominantly affected the central nervous system without involvement of the coronary or renal vasculature. The prolonged duration of this child's illness and the predominance of central nervous system involvement are the two unique features of the present case that need to be emphasized.
Received November 29, 1994; revision received January 10, 1995; accepted January 10, 1995.
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