Interesting case

By
Dr Kishore.K .K
Fellow in Pediatric Neurology
Case senario
6 months male baby, 4
th
issue of NCM
brought with h/o fever since 3 days
Convulsions since 1 day
(GTC of Lt UL & LL)
Birth, developmental and family history
normal.
Course in hospital

Child was put on ventilator for 10 days
Had raised ICT with conus (treated)
Methyl prednisolone course given
Later was consious and irritable, vitals
stable.
Cranial nerves normal.
Moves all limbs (power is 3-4/5)
Tone increased (UL>LL), brisk reflexes.
MRI shows non enhancing focal areas
showing restricted diffusion in the
temporal and frontal lobes B/L.
Subtle enhancement in basal cisterns
B/L narrowing of ICA, MCA and ACA
S/O basal meningitis with vasculitis
MRI T1
MRI T 2
DWI - image
MR - angio
Differential diagnosis
Moya moya disease (stroke)
CNS vasculitis
Viral encephalitis
CNS Vasculitis
By
Dr Kishore.K .K
Fellow in Pediatric Neurology
Introduction
CNS vasculitis refers to a spectrum of
heterogeneous disorders characterized by
inflammation of the blood vessels, including
arteries and veins of all calibers.
Which results in a variety of clinical
neurologic manifestations related to ischemic
and/or hemorrhagic parenchymal damage
Introduction
If unrecognized and consequently untreated
CNS vasculitis leads to permanent neurologic
injury and disability.
Thus it is important for accurate diagnosis
and management of each one of the variant
in this group of disorders.
CNS vasculitis in children can occur as a
primary disease that is isolated to the CNS or
as a secondary manifestation of an
underlying systemic condition
Classification
1. Primary
Referring to those patients with vasculitis isolated primarily
to the brain, cord and their leptomeninges occurring in the
absence of recognizable triggers or conditions
2. Secondary
Referring to where such associated conditions or co-factors
are apparent.
occurring as a result of an underlying systemic infectious,
noninfectious immunologic, neoplastic, or other etiology
It can be broadly classified into two major categories
Classification
Primary Angiitis of the Central Nervous
System (PACNS) (Unknown Immunopathogenesis)
1. Granulomatous angiitis of the CNS (GACNS)
2. Benign angiopathy of the CNS (BACNS)

Secondary CNS Vasculitis in Children




Secondary CNS Vasculitis
Infective (either direct infection
or autoimmune mechanisms )
Bacterial (eg-N.mening)
Mycobacterial (TB)
Spirochetal (Lymes, syphilis)
Rickettsial (Rocky Mount spot fev)
Fungal (aspergillosis, mucormycosis)
Viral (HZV/VZV, HIV, hepatitis C)
Parasitic (Toxoplasma gondii)
Primarily immunologic
Systemic vasculitis
(ANCA) mediated
Wegener granulomatosis
Microscopic polyarteritis
Churg-Strauss syndrome
Direct antibody attack
mediated
Kawasaki disease
(antiendothelial antibodies)
Secondary CNS Vasculitis
Immune complex
mediated
HS purpura
Cryoglobulinemia
Serum sickness
Unknown
immunopathogenesis
Temporal arteritis
Takayasu arteritis
Polyarteritis nodosa
Collagen vascular
diseases
SLE, JRA
Behacets, Sjorgens
Vascular injury
Dissection
Irradiation
Drugs
Amphetamines
Contraceptives

Pathophysiology
CNS vasculitis is characterized by transmural
inflammation of cerebral blood vessels of all
types and sizes involving the endothelial
lining and/or the cellular components of the
vascular wall.
The inflammatory responses may be
triggered by microbial pathogens (antigen)
or by altered host proteins (molecular
mimicry)
Pathophysiology
Vascular inflammation causes local hemodynamic
derangements leading to luminal compromise,
vasospasm, distal embolization arising from
necrotic vascular endothelium.
Perifocal tissue injury attributed to
proinflammatory factors like NO, cytokines,
cytotoxic lymphocytes, endothelins and proteases
produced by the infiltrating inflammatory cells or
by the injured brain vascular endothelial cells
Pathophysiology
This results in damage of cerebral blood
vessels and disruption of the BBB and local
hemodynamic derangements.
The main pathologic correlate of vasculitis-
related neurologic dysfunction is hypoxic-
ischemic injury of the CNS parenchyma.
Primary Angiitis of the CNS
Primary angiitis of the CNS is a rare disease
characterized by vasculitis of small and
medium arteries in the brain and spinal cord.
Presentations are highly variable, but the
triad of headache, organic brain syndrome
and multifocal neurologic deficits is most
suggestive.

Clinical features
A broad spectrum of clinical presentation
ranging from
Insidious onset of headache
Focal neurological deficit
Cognitive decline or behavior changes
Acute stroke like
Seizures or status epilepticus.
Optic neuritis(B/L) and cranial nerve palsies
Differential diagnosis
Patients with CNS Vasculitis are often
misdiagnosed initially.
Stroke
Viral encephalitis
Tuberculosis
Severe migraine headaches
Multiple sclerosis
Tumor
How to diagnose CNS vasculitis
CSF analysis not diagnostic but helps in
confirming CNS inflammation/infection
Patients may have mildly elevated ESR,
CRP, WBC counts and C3 complement
and IgG levels.
Neuroimaging MRI, MRA
Angiography
Brain biopsy
Neuroimaging
MRI brain classically reveals areas of acute
ischemia in a vascular distribution in large-
medium vessel disease.
These lesions are typically multifocal in
progressive disease and unilateral in non-
progressive disease involving gray and white
matter.
MRI in small vessel disease shows lesions
that may be bilateral or unilateral, multifocal
and involve both gray and white matter

This is T2-weighted MRI showing
involvement of bilateral basal
ganglia nuclei, thalami,
subcortical and deep white
matter, greater on the right.


The distribution of abnormality is
consistent with both anterior and
posterior circulation involvement.
MRSA
Magnetic resonance angiography and
conventional angiography provide detailed
assessment of the vasculature.
The classic angiographic findings in CNSV
include beading (alternating dilatations and
narrowings of blood vessels)
And other irregularities within blood vessels
like tortuosity, stenosis, and occlusion of the
vessels.
1. MRA (A) demonstrates bilateral proximal vessel stenoses of the middle carotid
artery (arrows).
2. The corresponding conventional angiography of the right middle cerebral artery
(B) demonstrates proximal (arrow) and distal (open circle) vessel stenoses
Brain biopsy
Biopsy of the brain and leptomeninges.
The presence of an inflammatory process
and fibrinoid necrosis in the walls of small
veins and arterioles remains the gold
standard for the diagnosis of primary angitis
of CNS.
But brain biopsy also has pitfalls and should
therefore be strongly considered in a child
with typical clinical features and suggestive
MRI lesions but normal angiography.
Many diseases may mimic CNS vasculitis
and because the treatments for CNS
vasculitis are potentially dangerous.
It is essential to confirm the diagnosis
before starting treatment.
Nearly all patients therefore require either
an angiogram or biopsy of the brain.
Treatment
Immunosuppressive through methyl
prednisolone and/or cyclophosphamide
LMWH/warfarin
Low dose aspirin in pts with no
complications
THANK YOU
Moyamoya disease
Moyamoya disease was first described in
Japan in 1957 (Suzuki)
Moyamoya is a Japanese word meaning puff
of smoke
It is a chronic progressive non-
atherosclerotic, non-inflammatory occlusive
arteriopathy of unknown cause
Epidemiology
The annual incidence of MM is 0.35 to 0.94
per 100.000 population.
The prevalence of MM is 3.2 to 10.5 per
100.000 population (Japan)
Asian: 0.28 per 100.000 population
Age distribution in child: 10 14 yr old
Male/Female: 1/1.8 to 1/2.2
Etiology unknown
Familial occurrence of appr 10% of cases
Familial MM has been linked to chromosomes
3P24,2-P26, 6Q25,12P12, and 17Q25
Pathophysiology
Characterized by progressive bilateral
stenosis of distal ICA extending to proximal
ACA and MCA
With involvement of circle of Willis and
development of extensive collateral network
(parenchymal, leptomeningeal or transdural)
at the base of the brain like a puff of smoke.
Intracranial aneurysms are seen, especially in
posterior circulation.

CLINICAL FEATURES
Transient ischemic attack (TIA)
Cerebral infarction
Repeated ischemic or hemorrhagic stroke
Seizures
Headache, movement disorders, mental
deterioration
In children, symptomatic episodes of ischemia
may be triggered by exercise, crying,
coughing, straining, fever or hyperventilation
NEUROIMAGING
CT scan: infarction may involve cortical and
subcortical regions.
In the patients with parenchymal
hemorrhage, cranial CT usually show a high
density area indicating blood in the basal
ganglia, thalamus and/or ventricular system
CTA: can also demonstrate the abnormal
vessels of MM.D, including the collateral
MM vessels in the basal ganglia
MRI: acute ischemic brain lesions, in some
cases, dilated collateral vessels
MRA,
The diagnosis of MM.D is based upon the
characteristic angiographic appearance of bilateral
stenoses affecting the distal internal carotid arteries &
proximal circle of willis vessels, along with the
presence of prominent basal collateral vessels
MCA block with multiple collateral
moyamoya vessels
(c) MRI Diffusion-weighted image shows bright signal of left insular and
temporal cortex indicating cytotoxic edema.
(d) MR-angiogram shows poor representation of distal branches of left middle
cerebral artery .

MANAGEMENT
Acute management is mainly symtomatic and
directed towards reducing elevated intracranial
pressure, improving cerebral blood flow, and
controlling seizures.
In patients with intracerebral hemorrhage,
ventricular drainage, and/or hematoma, removal is
often required.
There is no curative treatment for MM.D
Secondary prevention for patients with
symptomatic MM syndrome is largely centered on
surgical revascularization techniques(EDAS) and
Aspirin after surgery and in asymptomatic children
Thank You