Views & Reviews

Isolated angiitis of the CNS in children
S. Lanthier, MD; A. Lortie, MD; J. Michaud, MD; R. Laxer, MD; V. Jay, MD; and G. deVeber, MD

Article abstract—Objective: To clarify the clinical features and pathologic manifestations of isolated angiitis of the CNS
(IACNS) in children. Methods: The authors report two new cases and summarize the literature of childhood IACNS
confirmed by pathology. Results: IACNS affecting small vessels (n ⫽ 5)—Neurologic manifestations included headaches,
focal seizures, and progressive, behavioral, or multifocal neurologic impairment. MRI showed multifocal, T2-hyperintense,
cerebral lesions without mass effect or tumor-like lesions. CSF, erythrocyte sedimentation rate, and cerebral angiograms
were often normal. CNS biopsy disclosed a nongranulomatous vasculitis. Children were treated with prednisone alone or
combined with cyclophosphamide. One child died. Four children had a favorable outcome. IACNS affecting large and
medium arteries (n ⫽ 5)—Three children presented with acute ischemic stroke or TIA. Brain CT showed ischemic infarcts.
Two children presented with subarachnoid hemorrhage. In this group, CSF, erythrocyte sedimentation rate, and angiograms were often abnormal. No patient received immunosuppressive therapy. Five children died. Autopsy showed granulomatous IACNS (n ⫽ 5) Conclusions: Clinical and radiologic features correlate with the size of affected vessels. Prognosis
differs between groups. Potential markers of poor outcome are acute stroke presentation secondary to large and mediumsized artery involvement, granulomatous angiitis, and delayed institution of immunosuppressive therapy.
NEUROLOGY 2001;56:837–842

Isolated angiitis of the CNS (IACNS), also known as
primary angiitis of the CNS, is an idiopathic vasculitis confined to the CNS.1 A definitive diagnosis requires confirmation by pathology and exclusion of
disorders that mimic the disease.2-4 Experience in
adults shows that IACNS embraces a full spectrum
of clinical and pathologic manifestations and degrees
of severity.3 Literature of childhood IACNS is limited
to a few case reports,5-13 with fatal outcome in most
instances. We report two children with nongranulomatous IACNS proven by biopsy with good outcomes
after follow-up periods of 8 months and 8 years. We
also summarize pediatric cases documented by pathology and reported over the last 35 years. Our
objective is to clarify the spectrum of clinical and
pathologic manifestations in children with IACNS in
order to define a clinical approach tailored to this
age group.
We reviewed pediatric cases of IACNS identified
from two large stroke cohorts: Hôpital Sainte-Justine,
Montréal, 1991–1997,14 and The Hospital for Sick Children, Toronto, 1999 –2000. We also searched the English and French literature to identify reported cases in
children, using Embase (1980 –June 2000) and Medline
(1966 –May 2000) databases, and cross-checked references listed in retrieved articles. We contacted some
authors for additional data.5,7 We included all patients

age 0 to 18 years with IACNS proven by CNS biopsy or
autopsy. IACNS was classified as granulomatous (presence of giant cells or granulomatous inflammation
within vessel wall) and nongranulomatous.1 Artery
sizes as determined by angiograms were divided into
large (carotid arteries and vertebrobasilar system and
their primary branches, including A1, M1, and P1 segments), medium (secondary and tertiary branches of
the large arteries), and small (quaternary branches,
usually ⱕ1 mm and best seen on magnification).15 Using pathology and angiography to determine the size of
the CNS blood vessels that were predominantly affected, we classified cases with brain involvement as
IACNS affecting small vessels and IACNS affecting
large and medium-sized arteries.
Case 1. A 10-year-old girl presented with left-sided focal
seizures. The history and examination were unremarkable.
Electroencephalograms showed moderate right central and
posterior slowing. Brain MRI revealed two large, T2hyperintense, gadolinium-enhancing lesions involving the
right parietal cortex and white matter with no mass effect.
Four subcortical lacune-like lesions were also present in
the right middle and posterior cerebral artery distributions. Cerebral angiogram disclosed a single stenosis in a
quaternary branch of the right middle cerebral artery (figure 1). Erythrocyte sedimentation rate (ESR), C-reactive
protein, and other laboratory tests; renal angiography;

From the Department of Pediatrics, Divisions of Neurology (Drs. Lanthier and deVeber) and Rheumatology (Dr. Laxer), and Department of Pathology (Dr.
Jay), The Hospital for Sick Children and University of Toronto; the Département de Médecine, Service de Neurologie, Centre Hospitalier de l’Université de
Montréal (Dr. Lanthier) and Département de Pédiatrie, Service de Neurologie, Hôpital Sainte-Justine (Dr. Lortie), and Université de Montréal; and the
Department of Pathology (Dr. Michaud), Children’s Hospital of Eastern Ontario and University of Ottawa, Canada.
Supported by grants from the Bloorview Children’s Hospital Foundation and the Heart and Stroke Foundation of Ontario.
Presented at the 52nd annual meeting of the American Academy of Neurology; May 4, 2000; San Diego, CA.
Received August 22, 2000. Accepted in final form December 23, 2000.
Address correspondence and reprint requests to Dr. Gabrielle deVeber, Division of Neurology, The Hospital for Sick Children, 555 University Avenue,
Toronto, Ontario, Canada, M5G 1X8; e-mail: deveber@sickkids.on.ca
Copyright © 2001 by AAN Enterprises, Inc.

837

Figure 2. Case 1: Right temporal lobe open biopsy. White
matter small venule surrounded by a chronic inflammatory infiltrate obliterating the vessel lumen. Reactive gliosis is present in the white matter. Insert: CD3 antibody
showing a predominance of T-lymphocytes.
Figure 1. Case 1: Selective cerebral angiography (midarterial phase). Single small-artery stenosis in a quaternary branch of the right middle cerebral artery. Insert:
magnification of the stenosis.

chest radiography; transthoracic echocardiography; and
abdominal ultrasonography showed normal hemostasis
and no evidence of rheumatic disease, infection, or neoplasm. Results of CSF analysis and cultures and spinal
MRI were normal.
The patient’s seizures were controlled with carbamazepine and clobazam. Six weeks after presentation, she developed headaches, nausea, and a left hemiparesis. Repeat
brain MRI showed an increase in size of the pre-existing
parietal lesions, with mass effect and additional small T2hyperintense lesions. A right temporal lobe open-brain and
leptomeningeal biopsy was performed. A multifocal, vascular and perivascular, predominantly T-lymphocytic infiltrate with occasional eosinophils and B-cell lymphocytes,
including plasma cells, was found in the arterioles, capillaries, and venules of the leptomeninges and white matter
(figure 2). There were no giant cells, granulomas, or areas
of fibrinoid necrosis. The cortical vasculature was minimally affected. However, within the neocortex, small ischemic infarcts and areas of neuronal loss containing
macrophages were identified, some adjacent to the infiltrated leptomeningeal vessels. Within the white matter,
small areas of necrosis with numerous macrophages, reactive gliosis, and occasional axonal swellings were found.
Few small vessels with fibrosis and scanty inflammatory
cells were also present. Relatively diffuse myelin pallor,
without perivascular distribution, was also noted. There
were no microglial nodules, viral inclusions, or evidence of
parasitic infection. Stains, cultures, and immunofluorescence testing of the biopsied brain tissue for Ig, C1q, C3,
and fibrinogen were negative.
Nongranulomatous IACNS was diagnosed. Treatment
with prednisone 2 mg/kg/day was initiated 7 weeks after
onset. At 15 weeks, owing to development of truncal obesity and mild hypertension, slow prednisone tapering was
838 NEUROLOGY 56

April (1 of 2) 2001

attempted. Her headaches recurred at 25 weeks, and repeat laboratory testing showed elevated blood leukocytes
(13.3 ⫻ 109/L), C-reactive protein (21.2 mg/L, normal ⱕ 6
mg/L),and ESR (26 mm/hour, normal ⱕ 10 mm/hour). Repeat brain CT showed only small residual hypodensities in
the right parietal lobe and right basal ganglia. She was
treated for an additional 20 months with combined prednisone 2 mg/kg/day and cyclophosphamide 2 mg/kg/day.
She has now been off all immunosuppressive treatment for
6 years and has had no recurrent symptoms. Neurologic
examination shows only mild residual left hemiparesis.
Case 2. A 16-year-old girl presented with severe headaches, neck pain, nausea, and vomiting for 2 days. Over
the previous year, she had had mild headaches and depression that was improved by fluoxetine. One month before
admission, she developed a cough and sore throat that
resolved, but headaches increased and became unresponsive to regular analgesics. The history was otherwise unremarkable. The examination showed intact cognition,
bilateral optic disk swelling, persistent conjugated right
gaze-evoked nystagmus, diffuse hyperreflexia, neutral
plantar responses bilaterally, right-sided dysmetria and
dysdiadochokinesis, and ataxic gait.
Blood leukocytes were elevated at 11.9 ⫻ 109/L (predominantly polymorphonuclears). Toxicology screening
was negative. Brain MRI revealed a right cerebellar lesion
producing mass effect on the fourth ventricle with obstructive hydrocephalus (figure 3). She received antibiotics, acyclovir, and dexamethasone. A right frontal external
ventricular drain was inserted and the initial CSF flow
was noted to be under high pressure. CSF analysis showed
747 leukocytes per liter (74% lymphocytes, 18% monocytes,
6% eosinophils, and 2% polymorphonuclears); protein and
glucose levels were not measured. CSF stains and cultures
were negative. PCR testing was positive for human
herpesvirus-7 (HHV-7), but negative for other herpesviruses, Mycobacterium pneumoniae, and Mycoplasma
tuberculosis.
The following day, the patient deteriorated with pro-

Figure 3. Case 2: T2-weighted brain MRI. Right cerebellar, hyperintense, pseudo-tumoral lesion producing mass
effect on the fourth ventricle.

gressive loss of consciousness and decerebration. Brain CT
revealed increased hydrocephalus. She underwent drain
repositioning and suboccipital craniotomy for posterior decompression and cerebellar biopsy. On pathology, the most
striking abnormality was a dense, perivascular and transmural, inflammatory infiltrate affecting small arteries and
venules. The infiltrate consisted predominantly of T lymphocytes, as well as many eosinophils and a few neutrophils. There was no evidence of granulomatous angiitis or
fibrinoid necrosis. Leptomeningeal vessels were more affected than those of the cerebellar cortex and white matter.
The small amount of necrotic and hemorrhagic cerebellar
tissue indicated some acute damage, but the marked Purkinje cell loss, Bergmann gliosis, and gliosis of the white
matter implied a more chronic process. There were no intraparenchymal granulomas, viral inclusions, microglial
nodules, or evidence of a parasitic infection. Tissue stains
and cultures disclosed no infection. Electron microscopy
showed endothelial cell necrosis in some vessels and transmural inflammatory infiltrate indicating vasculitis. There
were no viral inclusions, tubuloreticular inclusions, or Birbeck granules (specifically evaluated in view of the presence of eosinophils in the inflammatory infiltrate).
Investigations performed after the surgery showed elevated ESR (20 mm/hour, normal ⱕ 10 mm/hour) and
C-reactive protein (45.6 mg/L, normal ⱕ 8 mg/L), normal
hemostasis, and no evidence of rheumatic disease, infection, or neoplasm. Findings of chest radiography and selective cerebral angiography were normal. A diagnosis of
nongranulomatous IACNS was made. The patient improved on prednisone 1 mg/kg/day. C-reactive protein and
ESR are normalized. After 8 months of follow-up, she is
asymptomatic on a tapering dose of prednisone.

Discussion. One child with granulomatous IACNS
limited to the spinal cord has been reported.13 He
presented with progressive myelopathy and responded to prednisone therapy. Eight children with
IACNS with brain involvement have been previously
reported5–12; they are summarized together with
Cases 1 and 2 from the current report in the table
and below in terms of caliber of involved vessels.
IACNS affecting small vessels. Children in this
group (n ⫽ 5, including Cases 1 and 2)5-7 had gradual
symptom onset consisting of persistent headaches (n
⫽ 3), additive multifocal neurologic deficits (n ⫽ 2),
cognitive decline (n ⫽ 1), and mood disorder (n ⫽ 1),
and presented with focal seizures (n ⫽ 3). Brain MRI
was abnormal in all children. In two of them, it showed
a single, large, cortico–subcortical, gadoliniumenhanced lesion producing mass effect and suggesting
the diagnosis of brain tumor. Cerebral angiography
was normal in all except one child (Case 1). In this
group, the diagnosis of IACNS was established by CNS
biopsy, which revealed a nongranulomatous (lymphocytic) vasculitis affecting small vessels (n ⫽ 5) and medium arteries (n ⫽ 1). Fibrinoid necrosis was not
reported. The two children with tumor-like lesions on
MRI remained clinically stable without recurrence at 8
months (Case 2) and 2 years after initiation of prednisone therapy.7 One child (Case 1) had recurrent
symptoms after a 4-month course of prednisone therapy but had no further recurrence over 6 years after an
additional 20 months with combined prednisone and
cyclophosphamide. A fourth child first received prednisone 60 mg/day, which was tapered over 6 weeks.5
After he developed a pontine hematoma 4 months after
presentation, he was treated with combined prednisone
and cyclophosphamide but experienced seizures and
progressive cognitive decline. He died of status epilepticus 18 months after presentation. Autopsy disclosed a
granulomatous IACNS limited to small cerebral arteries and veins. The fifth child in this group was reported
to improve on combined prednisone and cyclophosphamide therapy during a 20-month follow-up
period.6
IACNS affecting large and medium-sized arteries.
In this group (n ⫽ 5),8-12 reasons for seeking medical
attention were large arterial ischemic stroke (n ⫽
2),8,10 TIA (n ⫽ 1),12 and subarachnoid hemorrhage
(n ⫽ 2).9,11 Minor prodromal symptoms were reported
in two children. Four children in this group died
within 10 days of presentation.8-11 The fifth child developed cerebral hematomas secondary to recurrent
aneurysm ruptures.12 Pathologic analysis of one resected aneurysm revealed an inflammatory infiltration of the aneurysm wall. This child did not receive
immunosuppressive therapy. He lapsed into a persistent vegetative state and died 7 years after presentation. In this group, autopsy showed a granulomatous
infiltration of large and medium CNS arteries (n ⫽
5), inconsistent extension to small CNS arteries (n ⫽
2),9,11 and vessel wall (fibrinoid) necrosis (n ⫽ 2).9,10
Tuberculosis, sarcoidosis, Wegener granulomatosis,
Churg–Strauss syndrome, and other granulomatous
April (1 of 2) 2001

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Table Clinical manifestations, treatment, and outcome in 10 cases of childhood IACNS with brain involvement
IACNS affecting large and medium-sized arteries
IACNS affecting small
vessels*

AIS (n ⫽ 2)8,10 and
TIA (n ⫽ 1)12

SAH (n ⫽ 2)9,11

6–16 (2/3)

3–15 (1/2)

1.25–15 (1/1)

Neurologic
manifestations

Headache with progressive,
behavioral or multifocal
neurologic impairment
for ⱕ1 y (3/5); focal
seizures (3/5)

Mild headaches and dizziness
for 2 wk (1/3); acute
headache and sudden focal
neurologic deficit (3/3)

Mild headaches for 6
mo (1/2); acute
headache and loss of
consciousness or
sudden death (2/2)

Initial neuroimaging

Brain MRI:
multiple lacunes (2/5),
large T2-hyperintense
cortico-subcortical areas
without mass effect (2/5),
large tumorlike lesion
(2/5)

Brain CT:
multiple lacunes and a
single small hypodense
area (1/2); large hypodense
area (1/2)

ESR

Elevated (1/3);†
normal (2/3)

Elevated (1/1)

CSF

Inflammation (1/3);
normal (2/3)

Inflammation (2/3);
normal (1/3)

Hemorrhagic (1/1)

Cerebral
angiography

Normal (4/5);
single small-artery
stenosis (1/5)

Normal (1/2);
multiple medium-sized
artery stenoses and
aneurysms (1/2)

Single aneurysm (1/1)

Treatment

Immunosuppression (5/5)

No immunosuppression (3/3)

No immunosuppression
(2/2)

Outcome

Death 18 mo after
presentation (1/5);
alive, no recurrence (4/5)

Death within 3 d (2/3) and 7
y after presentation (1/3)

Death within 10 d after
presentation (2/2)

Variable
Age, y (M/F)

* N ⫽ 5, including our two cases.5-7
† Measured after brain surgery.
IACNS ⫽ isolated angiitis of the CNS; AIS ⫽ arterial ischemic stroke; SAH ⫽ subarachnoid hemorrhage; ESR ⫽ erythrocyte sedimentation rate.

processes affecting CNS vessels in children were excluded by the absence of systemic involvement and
pathologic hallmarks of these conditions.
We used the size of the affected vessels, as determined by angiography and pathology, to define two
groups of IACNS patients with brain involvement.
Although the groups are not mutually exclusive, as
shown by the coexisting involvement of small vessels
and large or medium arteries in two children9,11 and
some adults,16 their clinical manifestations and
short-term prognosis differ. This classification is
therefore relevant clinically and may help distinguish the approach to therapy.
IACNS affecting small vessels. Most adults with
IACNS documented by pathology have small vessel
involvement.3 Children and adults with IACNS and
small vessel involvement are similar in several aspects: they are more likely to experience a clinical
course that is gradual and variable, with headaches,
focal seizures, multifocal neurologic deficits, and
neurobehavioral impairment, reflecting multifocal or
diffuse brain damage. Neuroimaging reveals multiple cerebral ischemic lesions and tumor-like lesions.
In comparison with the adult series,3 seizures and
840 NEUROLOGY 56

April (1 of 2) 2001

tumor-like presentations were reported more frequently in children with small vessel disease. CSF
and ESR are often normal. Angiograms are generally
unremarkable, because the size of the affected vessels is often below the resolution of this technique.
CNS biopsy directed at cerebral lesions identified on
neuroimaging leads to antemortem diagnosis of
IACNS and initiation of immunosuppressive treatment. Response to treatment varies from long-term
remission, possibly achievable with prednisone
alone, to resistance to combined prednisone and cyclophosphamide, resulting in gradual neurologic decline and death.
In this series, CNS biopsy revealed a nongranulomatous form of IACNS in all children with small
vessel involvement. In the only patient who failed to
respond to immunosuppressive therapy, granulomas
were found within small vessel walls at autopsy performed 14 months after biopsy. In children with
IACNS affecting small vessels, the absence of granulomas on CNS biopsy could be due to sampling error,
given the segmental distribution and pathologic heterogeneity of vascular lesions in IACNS.1 However,
the existence of a lymphocytic (nongranulomatous)

form of IACNS has been repeatedly confirmed by
autopsy reports,16 and associated with a better outcome in adults.17 The lymphocyte infiltration may
represent one of the first changes in IACNS, whereas
the presence of granulomas may signal a more aggressive or advanced form of the disease.
In one of our patients (Case 2), PCR assay on CSF
was positive for HHV-7. This adolescent had no recent or current exanthema and no fever. Pathologic
examination and electron microscopy showed no evidence of viral encephalitis. HHV-7, like HHV-6, is a
causal agent of roseola, which can be complicated by
acute encephalopathy.18 We are not aware of any
report linking HHV-7 with IACNS. Several viruses
have been associated with CNS vasculopathies, including herpes zoster virus.19 The detection of
HHV-7 by PCR on CSF should be interpreted with
caution.18 HHV-6 DNA can persist in the CSF after
childhood infection.20 The same may be true for
HHV-7.
IACNS affecting large and medium-sized arteries.
Children in this group are more likely to present
with TIA, arterial ischemic stroke, or hemorrhagic
stroke, and to die of neurologic complications of
acute stroke. Elevated ESR, inflammatory CSF, and
abnormal angiograms are more frequent than in the
first group. For this group, granulomatous IACNS
was diagnosed at autopsy. We suspect that presumptive cases of IACNS are often treated on the basis of
clinical evaluation and “vasculitic” changes seen on
angiograms.21
Clinical approach. The clinical evaluation and
investigation should exclude drug exposure, systemic
infections, thromboembolic processes, childhood
rheumatic diseases, and other mimics of IACNS.2-4
Systemic lupus erythematosus and microscopic polyangiitis should be considered in children with involvement of small-sized CNS vessels.22 Polyarteritis
nodosa, Kawasaki disease, Churg–Strauss syndrome, and Wegener granulomatosis affect small
and medium arteries.22 IACNS affecting medium and
large arteries should be differentiated from benign
angiopathy of the CNS3 and post-varicella arteriopathy,23 which generally follow a monophasic course.
Typically, post-varicella arteriopathy occurs within 9
months of the chickenpox primary infection and affects the proximal segments of the middle and anterior cerebral arteries unilaterally. Takayasu arteritis
affects the aorta and its branches but spares intracranial vessels.22 Giant cell temporal arteritis rarely
or never affects children.22 Inflammatory bowel disease, sarcoidosis,24 and Behçet disease25 can be complicated by CNS vasculitis, which may involve arteries
and veins of any size. Systemic rheumatic diseases are
diagnosed based on their distinct pattern of systemic
manifestations and laboratory markers, although in
rare cases they may be unrecognizable early in their
course if the CNS is involved first.
In children with suspected IACNS, our approach
is to attempt to confirm the diagnosis of CNS vasculitis and to exclude intracranial infection and neo-

plasm by leptomeningeal and brain biopsy of a
radiologically affected area before initiating immunosuppression. CSF and biopsied CNS tissue should
be analyzed and cultured. On pathology, vascular
changes consistent with a diagnosis of IACNS include multifocal and segmental infiltrates, fibrinoid
necrosis, and vessel fibrosis indicating healing lesions. The vessel infiltrates consist predominantly of
lymphocytes, variably associated with giant cells
that form granulomas and eosinophils. The risk of
false-negative CNS biopsy (17 to 53% in adults with
IACNS26) is greater if the disease is restricted to
large arteries not sampled by this procedure, and if
pathologic study is limited to the leptomeninges5 or
to evacuated necrotic tissue from cerebral infarction.10 Occasionally, systemic rheumatic diseases can
be identified by the presence of typical pathologic
findings (e.g., necrotizing vascular lesions with polymorphonuclear leukocytes infiltrating branching
points of small and medium arteries, associated with
microaneurysms, suggest polyarteritis nodosa).27
Subarachnoid hemorrhage is rarely reported and
usually mild in adults with IACNS.19 Subarachnoid
hemorrhage from rupture of aneurysm or nonaneurysmal dilatation is unusual in childhood28 and
should raise the possibility of CNS vasculitis, especially when atypical features are found (e.g., nonsaccular aneurysm, location distal to bifurcations) and
after infectious and traumatic causes have been excluded. Clipped aneurysms should be biopsied whenever feasible and analyzed by pathology. When an
intracranial aneurysm located in a peripheral
branch artery is treated by occluding its proximal
segment, the sacrificed distal segment of artery
should be submitted for pathologic analysis.
In children presenting with acute stroke, supportive care, appropriate antithrombotic treatment, and
measures to prevent stroke complications should be
initiated with the aim of decreasing early morbidity.29 A combination of cyclophosphamide and prednisone is the recognized treatment of IACNS in
adults.2,19 The efficacy of prednisone or cyclophosphamide alone in IACNS is undetermined and should
be assessed prospectively in patients selected on the
basis of prognostic factors. In children with IACNS,
potential markers of poor outcome are an acute
stroke presentation secondary to large and mediumsized artery involvement, a granulomatous IACNS
on pathology, and delayed institution of immunosuppressive therapy. Response to immunosuppressive
treatment should be monitored, its effects shown by
stabilization of clinical and radiologic manifestations
and by normalization of laboratory results, including
CSF analysis and ESR. Because CNS involvement
may herald rheumatic diseases in some patients, vigilant appraisal of delayed systemic manifestations is
an essential part of the follow-up.
Acknowledgment
The authors thank Ms. C. Tunney for her assistance in the search
of Embase and Medline databases; Ms. T. Domi, Dr. S. Mayank,
April (1 of 2) 2001

NEUROLOGY 56

841

and Dr. M. Salmon for their assistance in the preparation of this
manuscript; and Dr. M.W. Bojanowski, Centre Hospitalier de
l’Université de Montréal, for valuable discussion on the surgery of
intracranial aneurysm. This paper was prepared with the assistance of Editorial Services, The Hospital for Sick Children,
Toronto.

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Isolated angiitis of the CNS in children
S. Lanthier, A. Lortie, J. Michaud, et al.
Neurology 2001;56;837-842
DOI 10.1212/WNL.56.7.837
This information is current as of April 10, 2001
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