non-vasculitic inflammatory brain disease

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Inflammatory and Autoimmune Diseases
of the Pediatric Central Nervous System
Sangam Kanekar, MD
Dept of Radiology and Neurology
Penn State Milton S Hershey Medical Center
Hershey, PA, USA
Conflict of interest
Neither I nor my immediate family members have a financial
relationship with a commercial organization that may have a direct
or indirect interest in the content.
Sangam Kanekar, MD
Introduction
CNS inflammatory demyelinating diseases (CIDD) are rare. At first presentation
children are diagnosed with acute disseminated encephalomyelitis (ADEM) or a
clinically isolated syndrome (CIS) such as transverse myelitis (TM) or optic neuritis
(ON). Many of these disorders are monophasic, however a small number of children
relapse and are diagnosed with multiple sclerosis (MS) or neuromyelitis optica (NMO).
Paediatric onset Multiple Sclerosis (POMS) is a chronic inflammatory
neurodegenerative demyelinating disease of the CNS that is usually relapsingremitting at onset.
The diagnosis of infl ammatory brain disease is based on a thorough clinical
evaluation, including features of systemic inflammatory illnesses, blood and CSF
analysis, neuroimaging studies, supportive testing such as electromyography / nerve
conduction studies and targeted tests such as specific antibodies or brain biopsies
Inflammatory and Autoimmune Diseases
NON-VASCULITIC INFLAMMATORY
BRAIN DISEASE
• Demyelinating disease
• Antibody mediated inflammatory
brain disease
• T-cell mediated inflammatory
brain disease
• Granulomatous inflammatory
brain disease
• Miscellaneous
VASCULITIC INFLAMMATORY BRAIN
DISEASE
Primary CNS vasculitis (cPACNS)
Angiography positive, large-medium vessel
cPACNS
Angiography negative, small vessel cPACNS
Secondary CNS vasculitis
• Infection associated
Viral Bacterial Fungal Spirochaete
• Post-infectious
• Systemic vasculitis
ANCA-associated vasculitis
• Systemic rheumatic disease
• Systemic inflammatory disease
• Exposures
Radiation
Drugs (cocaine, amphetamines)
NON-VASCULITIC INFLAMMATORY BRAIN
DISEASE
Demyelinating disease
Acute demyelinating encephalomyelitis (ADEM)
Multiple sclerosis
Transverse myelitis (when demyelinating)
Antibody mediated inflammatory brain disease
Anti-NMDAR encephalitis
Antibody-mediated limbic encephalitis
Neuromyelitis optica
Hashimoto encephalitis
T-cell mediated inflammatory brain disease
Rasmussen’s encephalitis
Granulomatous inflammatory brain disease
Neurosarcoidosis
Blau syndrome
Others
Febrile infection-related epilepsy syndrome (FIRES)
Demyelinating Syndromes
Monophasic
Clinically Isolated Syndrome
(CIS)
Monophasic Acute
Disseminated
Encephalomyelitis (ADEM)
Monophasic Neuromyelitis
Optica (NMO)
Polyphasic
Multiphasic ADEM
Multiple Sclerosis (MS)
Relapsing Neuromyelitis Optica
(NMO)
Acute demyelinating encephalomyelitis (ADEM)
ADEM is considered as an immune-mediated disorder resulting from an autoimmune reaction to
myelin. ADEM typically occurs after preceding triggering events such as viral infection,
nonspecific upper respiratory tract infection or recent vaccination. ADEM presents with
polyfocal neurological deficits ranging from short prodromal phase consisting of fever, malaise,
headache, nausea, or vomiting to seizures, encephalopathy and/or coma. Other neurological
features include pyramidal signs; cranial nerve palsies; hemiparesis; ataxia; hypotonia; and
visual loss due to optic neuritis.
Summary of revised criteria
International MS Study Group monophasic ADEM criteria
• No history of prior demyelinating event
• First clinical event with presumed inflammatory
or demyelinating cause
• Acute or subacute onset
• Affects multifocal areas of central nervous system
• Must be polysymptomatic
• Must include encephalopathy (ie, behavioral change or altered
level of consciousness)
• Neuroimaging shows focal/multifocal lesion(s)
• predominantly affecting white matter
• No neuroimaging evidence of previous destructive
white matter changes
• Event should be followed by clinical/radiologic
improvements
• No other etiology can explain the event
• New or fluctuating symptoms, signs, or MRI findings
occurring within 3 months are considered part of the
acute event
•
First polyfocal clinical CNS event from a
presumed inflammatory cause
•
Encephalopathy that cannot be explained by
fever is present
•
MRI lesions are diffuse, poorly demarcated,
1-2cm white matter lesions; T1-hypointense
white matter lesions are rare
•
Deep gray matter lesions can be present
•
No new symptoms, signs or MRI findings
after 3 months of ADEM event
Acute demyelinating encephalomyelitis
(ADEM)
ADEM is characterized by perivenous edema, demyelination, and infiltration with macrophages
and lymphocytes, with relative axonal sparing. ADEM is classically associated with large,
confluent, and symmetric white-matter lesions. the typical MR imaging findings described in
ADEM are widespread, bilateral, asymmetric patchy areas of homogeneous or slightly
inhomogeneous increased signal intensity on T2-weighted imaging within the white matter,
deep gray nuclei, and spinal cord. Within the white matter, juxtacortical and deep white matter
is involved more frequently than is periventricular white matter,
More advanced imaging techniques, such as magnetic resonance spectroscopy, have
demonstrated elevation of lipids and reduction of the myo-inositol:creatinine ratio during the
acute phase, followed by reduction in lipids and increased myo-inositol:creatinine ratios in the
chronic setting
Acute demyelinating encephalomyelitis ACUTEBilateral
loss of vision
(ADEM)
Acute demyelinating encephalomyelitis
(ADEM)
Four MR imaging patterns of cerebral involvement in ADEM have been described:
(1) ADEM with small lesions (less than 5 mm);
(2) ADEM with large, confluent, tumefactive lesions;
(3) ADEM with symmetric thalamic involvement; and
(4) acute hemorrhagic encephalomyelitis characterized by evidence of hemorrhage within some
of the lesions.
Acute demyelinating encephalomyelitis
(ADEM)
large, confluent
lesions
INFLAMMATORY
ADEM
48 HRS
12 yrs old child presented with motor and sensory deficit.
Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the CNS, usually
occurring after infections and vaccinations. (children and young adults). C/F: motor/sensory deficits, brain
stem signs, and Ataxia. On MR: ill-defined hyperintense lesions on T2-WI are seen in the spinal cord. Lesions
are usually large and extend over a long segment of the spinal cord with cord expansion.
Associated involvement of brain helps in diagnosis of ADEM which shows involvement of the basal ganglia
and thalamus, cortical lesions, and brainstem involvement.
Multiple sclerosis
Multiple sclerosis (MS), the most common demyelinating disorder of the central nervous system
(CNS), characterized by multifocal areas of CNS demyelination disseminated in time and space, is
diagnosed primarily on clinical grounds. 3–10 % of patients will have an onset before the age of
18 years. In Pediatric age Relapsing-Remitting MS (RRMS) Acute relapses followed by periods of
remission is the most common type.
Multiple sclerosis (MS) Defined by any one of the
following:
• Two or more clinical nonencephalopathic
inflammatory CNS events (CIS episodes)
separated in time (>30 days apart) and space.
• Single episode of CIS with MRI brain meeting
2010
Revised
MacDonald
criteria
for
dissemination in space and time (only in children
_12 years); or
• ADEM followed >3 months later by a
nonencephalopathic clinical event (CIS) with new
lesions on MRI consistent with MS.
Multiple sclerosis
Classic MR appearance
Relapsing-Remitting MS
2003
2005
Multiple sclerosis
Most frequent presenting symptoms included motor dysfunction (30 %), sensory dysfunction
(15–30 %), brainstem function (25 %), optic neuritis (10–22 %), and ataxia (5–15 %) [ 76 ]. There
is no specific laboratory test for MS. MRI findings, supportive laboratory studies, and the clinical
course are used to make the diagnosis. Initial specific images of MS on MRI (100 % specific of
MS) consist of welldefined lesions and the presence of lesions perpendicular to
Dissemination in space
•
> 1 T2- lesion in at least 2 of 4 typical
locations
• Periventricular
• Juxtacortical
• Infratentorial
• Cord
Dissemination in Time
•
New lesion on follow-up scan
(irrespective of time interval)
•
At least 1 enhancing lesion and 1
non-enhancing lesion on baseline
scan
Optic Neuritis (ON)
Monocular Blindness
ON remains a clinical diagnosis, and the main role of MR is in the
identification of white matter lesions and prognosis for MS
Multiple sclerosis with optic neuritis
ADEM
MS
children, 5-8 years
Middle age female
females = males
F>>M
pathogenesis of a preceding
infection
Autoantibody/perivenular
inflammation
mild increase in
total protein.
Oligoconal bands
Complete recovery has been
reported in 53% to
94% of cases
Recovery-recurrent-progressive
MR BRAIN
Patchy – asymmetrical
Gray matter involved in the first
attack
Peripheral or blotchy type of
enhancement
Not periventricular
Corpus callosum not involved
MR BRAIN
Flame shaped fairly symmetric
Gray matter NOT involved in the
first attack
Broken enhancement
Periventricular VERY COMMON
Corpus callosum involved
Both optic nerves are involved
Uni lateral optic nerve
Neuromyelitis optica
Inflammatory disease primarily affecting optic nerves, spinal cord, and occasionally brain
parenchyma (especially in children). It is an auto-immunity to CNS predominant water channel
aquaporin-4. Adults affected much more commonly than children. Median age at presentation
10-14 years. Strong female preponderance if NMO-IgG positive.
New 2012 IPMSSG Revised Criteria
All are required:
•
Optic neuritis
•
Transverse myelitis
•
2 of 3 of the following supportive criteria:
•
Contiguous cord lesion involving more
than 3 vertebral segments
•
Anti-aquaporin-4 IgG seropositivity
•
Brain MRI not meeting criteria for MS
Spinal cord cross‐section demonstrating
extensive demyelination involving both the
grey and white matter
Neuromyelitis optica
Neuromyelitis optica (NMO) Patients presenting with optic neuritis, acute myelitis and
any two of:
Spinal MRI lesion extending over three or more vertebral segments;
Positive NMO antibody testing (serum anti-aquaporin-4); or
Brain MRI not meeting diagnosis criteria for MS.
INFLAMMATORY
Middle age
higher age at onset
steroids first line of treatment
steroids not helpful
Cerebral white matter is the primary target
Spinal cord lesions are focal < 2-3 segments
Spinal cord atrophy. cranial nerves or cerebellar
involvement are common in MS but are not resent in
DNMO
No cerebral white matter lesions are present
spinal cord lesions are confluent and extend to multiple
segments
spinal cord atrophy. cranial nerves or cerebellar
involvement are not preresent in DNMO
Serum autoantibody, NMO-IgG is highly sensitive and
specific
Good prognosis
Poor prognosis
Acute Necrotizing Encephalopathy (ANE)
ANE is a rare immune-mediate disorder with a poor prognosis and elevated mortality rates. ANE
has been related to intracranial cytokine storms, usually triggered by influenza or other viral
infection, causing BBB damage that results in edema and necrosis. ANE can be isolated,
in which case it is sporadic and nonrecurrent, or familial, in which case it is recurrent and
associated with mutations in the RANBP2 gene on chromosome 2.
a
b
c,d
MRI shows a very characteristic concentric lesion pattern in the thalami, frequently with
accompanying lesions in the brain stem tegmentum, periventricular white matter, putamina,
and cerebellum. These areas are hyperintense on T2-weighted images, show diffusion
restriction, and enhance concentrically; susceptibilityweighted and gradient-echo T2*-weighted
images show corresponding hypointensities, consistent with petechial hemorrhages.
Acute Necrotizing Encephalopathy (ANE)
The arthropod-borne viruses (arboviruses) are transmitted to humans by the bite of a mosquito or
tick. The epidemiology of arthropod-borne viral encephalitis is influenced by the season; the
geographic location; the regional climatic conditions, such as the amount of spring rainfall; and
the patient's age. Japanese encephalitis virus is a member of the St. Louis complex of flaviviruses
and is the most common cause of arthropod-borne human encephalitis worldwide. Epidemic
disease occurs in China and the northern parts of Southeast Asia, as well as in areas of India and
Sri Lanka. Imaging frequently shows bilateral thalami, brainstem, cerebellum and spinal cord
involvement. Hemorrhages are frequently seen.
Limbic encephalitis
Encephalitis of limbic system is most often due to infection or autoimmune etiology.
Autoimmune limbic encephalitis may be due to tumors (paraneoplastic) or nonparaneoplastic etiologies. Paraneoplastic encephalitis is most commonly seen in
pediatric patients either due to Ovarian Teratoma (anti-NMDAR) or in males due to
testicular germ cell tumor anti-Ma2. In adult similar pathology is seen due to small cell
lung cancer (anti-Hu, anti-CRMP, anti-amphyphysin, anti VGKC complex) or breast
cancer (anti-amphiphysin).
Voltage-gated potassium channel
encephalopathy. (A) Axial and (B)
coronal
FLAIR
images
how
hyperintensity in the hippocamppi
bilaterally, findings similar to limbic
encephalitis
Anti-NMDAR encephalitis
Encephalopathy caused by: anti-N-methyl-D-aspartate
receptor (NMDAR) antibodies. First described in 2007.
Associated with ovarian teratomas in young women (50%)
or with occult teratomas or unknown lesions. Idiopathic or
post-infectious in children
Symptoms: prodromal headache, psychosis, fever,
movement disorders, GI and upper respiratory symptoms
16 year old girl with paraneoplastic syndrome due to ovarian teratoma
Transverse myelitis
In TM a demyelinating lesion in the spinal cord
causes motor, sensory and/or autonomic
deficits, often accompanied by pain. Poorer
outcome is associated with younger age, rapid
progression (onset to nadir in less than 24
hours), flaccid legs at presentation, sphincter
involvement, and large and/or cervical lesions
on MRI. The risk of subsequent diagnosis of
MS is low (around 10%).
MS-associated myelitis extend less than three spinal segments in length in the longitudinal
plane. when MS patients develop myelitis it most often adheres to this pattern. Those with
longitudinally extensive spinal lesions (three or more spinal segments in length) should be
evaluated for NMO, as well as a variety of other disorders in the appropriate clinical setting,
including Sjo¨gren’s syndrome, BD, sarcoidosis, metabolic disturbances, and various infectious
agents.
Rasmussen’s encephalitis
-RE is a rare immune-mediated disease marked by progressive unilateral atrophy and
associated, intractable seizures. Widely considered a disease of childhood, up to 10%
of cases occur in adults. Incidence is sporadic 10% exhibit dual pathology, such as
coexistant low grade tumor or tuberous sclerosis. Bilateral disease very rare.
Pathophysiology
-A viral etiology is proposed, although no
specific pathogen has been identified.
-There is evidence of both a humoral and T-cell
mediated response.
-Oguni et al have suggested three distinct
clinical stages of prodromal, active and residual
disease.
-Over time, frequent seizures lead to atrophy
and progressive hemiparesis.
Brain biopsy in Rasmussen's encephalitis
showing lymphocytic infiltrates staining for CD8
on immunohistochemistry
Rasmussen’s encephalitis
Rasmussen’s encephalitis (RE) is a chronic inflammatory
disease of unknown origin, usually affecting one brain
hemisphere. Rasmussen (1958) in his original description
assumed a viral cause of the disease. Later, the condition was
linked to circulating auto-antibodies. More recently, however,
a cytotoxic T cell reaction against neurons was demonstrated
to play a causative role in RE. Serial MRI of RE patients reveals
a spread of the inflammatory lesion over the affected
hemisphere. In a given brain region, a characteristic course
from increased volume and T2/FLAIR signal to a final stage of
atrophy without signal abnormalities occurs.
PARENCHYMAL
SARCOIDOSIS
The histopathologic hallmarks include epithelioid granulomas without caseation or staining for
infectious agents. It is thought to represent an immune-mediated response to an as yet
unidentified antigen. The disease most commonly afflicts African Americans (female). Brain
parenchyma as well its coverings are involved: a) Dural mass lesions (d/d: meningioma), b)
Leptomeningeal infiltration typically involves the suprasellar and frontal basal meninges, c)
Parenchyma lesions: frequently affect hypothalamus, brain stem, cerebral hemispheres and
cerebellar hemispheres.
White matter lesions may be either enhancing or nonenhancing. Hydrocephalus is due to
granulomatous infiltration into the subependymal layers. Due to perivascular infiltration post Gd
images shows a typical linear enhancement along the Virchow Robin spaces. Involvement of
every cranial nerve has been described in association with sarcoidosis, most frequently, the
facial nerve is involved clinically.
Febrile infection-related epilepsy
syndrome (FIRES)
Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic and usually refractory
epilepsy syndrome that occurs after a febrile illness in previously normal children. The
pathogenesis of the syndrome is unknown and the diagnosis is typically made by exclusion after
an exhaustive negative workup for CNS infections and autoimmune or metabolic disorders. MRI
shows hippocampal abnormalities, typically found several months or longer after this initial
seiuzres. The development of early hippocampal atrophy with epileptic encephalopathy may
provide insight into pathogenesis and highlights the need for aggressive and effective
interventions early in the disease process.
8/5/2011
8/15/2011
9/6/2011
VASCULITIC INFLAMMATORY BRAIN DISEASE
Primary CNS vasculitis (cPACNS): Angiography
positive, large-medium vessel cPACNS
Primary CNS vasculitis (cPACNS)
Angiography positive, large-medium vessel
cPACNS
Progressive
Non-progressive
Angiography negative, small vessel cPACNS
Secondary CNS vasculitis
Infection associated
Viral (CMV, EBV, HIV)
Bacterial (mycobacterium tuberculosis,
mycoplasma pneumonia, streptococcus
pneumonia, others)
Fungal (candida albicans, aspergillus,
actinomyces, others)
Spirochaete ( Borrelia burgdorferi )
Post-infectious
VZV – post-varicella angiopathy
Systemic vasculitis
ANCA-associated vasculitis, polyarteritis
nodosa, takayasu arteritis,
Systemic rheumatic disease
SLE, systemic vasculitis, scleroderma,
dermatomyositis, others
Systemic infl ammatory disease
Hemophagocytic lymphohistiocytosis,
Kawasaki disease, inflammatory bowel disease,
celiac disease, graft
versus host disease
Exposures
Radiation
Drugs (cocaine, amphetamines)
Primary CNS vasculitis (cPACNS)
The vasculitides encompasses a heterogeneous group of disorders pathologically
characterized by inflammation of the blood vessel wall. Vasculitis may occur in the
context of a primary systemic disorder, such as giant-cell arteritis and polyarteritis
nodosa, or it may be localized to cerebral vasculature. When the inflammatory
changes in CNS blood vessels are secondary due to collagen vascular diseases,
infections, tumors, and substance abuse it is called secondary vasculitis. Primary
vasculitides may preferentially affect large (elastic), medium (muscular), or small (< 0.5
mm in diameter) arteries. An idiopathic form of vasculitis limited to the brain and
spinal cord is known as primary angiitis of the central nervous system (PACNS). Chapel
Hill Consensus Conference (CHCC) 2012 revised the International Nomenclature of
Vasculitides depending on the size of the vessel involved.
Primary CNS vasculitis (cPACNS)
Primary Angiitis of the Central Nervous System (PACNS) is an autoimmune vasculitis
that exclusively involves CNS blood vessels, including the brain and spinal cord, in the
absence of an underlying systemic disease. Patients may present with headaches,
stroke like episodes, seizures, movement disorder, optic neuritis, or progressive
cognitive decline or multifocal myelopathies with spinal cord involvement.
Secondary CNS vasculitis:
Infection associated : Viral (CMV, EBV, HIV)
Various organisms can cause CNS vasculitis either by primary or secondary damage to the
vessels. True infectious CNS vasculitis can be due to organisms directly infecting the vessel wall
or damage to blood vessels as they traverse purulent exudate in cisterns and along the cerebritis
bed. Secondary vessel damage could be due to post-infectious inflammatory toxins such as
immunoglobulin, complement, lipoprotein, viral antigen or immune complex deposition, cold
agglutinin formation, or vascular endothelial cell proliferation leading to vessel damage.
Fungal agents such aspergillosis, candidiasis, coccidioidomycosis and mucormycosis have a
predilection for cerebral vessels, leading to vasculitis, particularly in immunocompromised and
neutropenic hosts. They may cause large and small vessel thrombosis, cerebral infarction, and
mycotic aneurysm
Bacterial
Bacterial Meningitis
13 months old male child with proven case of bacterial meningitis
presented with acute loss of consciousness. MRI shows bilateral
restricted diffusion in the temporal lobes, posterior frontal lobes,
thalami and brainstem suggestive of acute infarction, due to
vasculitis/stenosis from exudate. Follow up CT showed my changes
of atrophy in these region.
Post-infectious: VZV – post-varicella angiopathy
Viral Infections
Viruses may be responsible for many cases of vasculitis that are considered idiopathic. Hepatitis-B surface
antigen, immunoglobulin, and complement are found in the vessel walls of patients with polyarteritis
nodosa who have hepatitis-B antigenemia. HZV is the most well known and best documented of the viral
arteritides. The most common clinical syndrome is delayed brain infarction, usually causing hemiplegia
contralateral to herpes zoster ophthalmicus. Infarcts are usually hemispheral and cause hemiparesis,
hemisensory loss, and aphasia, or right-hemispheric types of cognitive and behavioral changes. Small or
large arteries may be involved. Single regions of focal ring-like stenosis and gradual longer segments of
stenosis and multifocal narrowings were found.
A 15 year old male patient with acute confusion and seizure. MRI at admission showed hyperintensity
involving right temporal lobe suggestive of Herpes encephalitis. On 4th day of admission pt developed left
sided weakness repeat MRI showed acute ACA infarction presumed to be due to viral vasculitis of ACA
artery. CT perfusion shows stroke in right ACA territory.
Vasculitis associated with systemic disease
Various connective tissue disorders such as systemic lupus erythematosus (SLE), scleroderma,
rheumatoid arthritis, Sjogren syndrome, mixed connective tissue disease, and Behcet disease
can involve vessels. Vessel wall involvement in most of these diseases is thought to be
autoimmune.
SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
Headaches that often share features with migraine, seizures, psychosis with decreased cognition, chorea,
and mononeuropathies and polyneuropathies are important features of SLE. Sudden-onset neurologic signs
do occur in patients with SLE and can be a prominent clinical feature. MRI often shows discrete focal lesions
in cortical and subcortical infarcts are most often caused by abnormalities of coagulation and cardiac-origin
embolism. Angiography often shows occlusion of intracranial artery branches.
SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
6/19
Myelopathy is an uncommon manifestation of SLE (1% to 3%). MR imaging
of the spinal cord is normal in 16% to 30% of patients with clinical myelitis.
Birnbaum and colleagues: 2 subtypes of SLE myelitis: A) rapid onset (<6
hours in 72.7%) of flaccid paralysis, hyporeflexia, and urinary retention,
leading to a poor outcome with persistent paraplegia. B) subacute onset of
spasticity, hyperreflexia,and mild-to-moderate weakness for 1 To 30 days,
followed by a relapsing course.
6/24
SYSTEMIC VASCULITIS (COLLAGEN VASCULAR DISEASES)
Systemic vasculitis syndromes can be conveniently divided into polyarteritis nodosa, allergic
angiitis and granulomatosis (Churg-Strauss syndrome), hypersensitivity vasculitis, Wegener’s
granulomatosis, and overlap syndromes sharing features of other subtypes. All of these
syndromes have in common multisystem involvement. On imaging they are difficult to
differentiate. Final diagnosis is by documentation of the specific antigen or antibody with other
labortory investigations.
Polyarteritis nodosa (PAN) affects small- and medium-sized arteries, especially at branch points.
CNS involvement occurs in 20% to 40% of patients and the onset occurs usually after systemic
symptoms and signs and neuropathy. Occasionally, hemispheral, spinal cord, cerebellar and
brainstem infarcts occur.
Systemic inflammatory disease
e.g. Hemophagocytic lymphohistiocytosis, Kawasaki disease, inflammatory bowel disease, celiac
disease, graft versus host disease.
Celiac disease patient presented with ataxia. Sagittal, coronal T1 WI and
T2 axial images shows advanced cerebellar atrophy, disproportionate to
extent of cerebral volume loss and advanced for age.
KAWASAKI DISEASE
Kawasaki disease is an acute disorder
occurring predominantly in children that is
presumed to be caused by an unidentified
infectious agent. It causes vasculopathies
mostly in the form of coronary artery
aneurysm and the aorta. The carotid arteries
are sometimes also often involved. Rarely child
may present with small lacunar infarctions or
subarachnoid hemorrhage.
26 year old male patient with Kawasaki disease shows large aneurysm from both the carotid artery on CT coronary angiogram.
MR done for acute weakness of the right side shows stroke in the left MCA territory.
Exposures
RADIATION ENCEPHALOPATHY
Radiation therapy to the CNS typically causes vascular injury, specifically to the small vessels,
with resultant small vessel arteritis and ischemia.
The effects of radiation therapy on the CNS include focal CNS necrosis, white matter injury, CNS
atrophy, microangiopathic changes, and large vessel Vasculopathy. Injury includes edema,
arteritis, leukoencephalopathy, mineralizing microangiopathy, necrotising leukoencephalopathy,
PRES. Periventricular white matter.
VASCULOPATHY IN DRUG ABUSERS
Drugs & vasculitis
Ischemic stroke and vasculitis in drug abusers is not uncommon. It more frequently seen with
heroin addiction, amphetamine abuse, and in cocaine use. Brain as well as spinal cord
infarctions are seen following cocaine. The mechanism of ischemia is unknown but
vasoconstriction related to cocaine itself or its metabolites is the major posited mechanism.
Arterial constrictions is predominantly seen in the MCAs and PCAs following IV cocaine. Cocaine
use is also associated with SAHs and ICHs. There is also higher incidence of aneurysms and
vascular malformations in cocaine addicts.
A male patient presented to ER with acute onset right sided weakness. T2 and FLAIR images
show diffuse white matter vasculopathic changes. Axial DWI shows acute infarction involving
the left putamen and left temporal lobe. MRA shows narrowing of the both MCAs with acute
cut-off of left MCA.
Conclusion
• Children with primary or secondary inflammatory brain
diseases frequently present with acute symptoms which
includes seizures and status epilepticus, intractable movement
disorders, meningitis or encephalitis or stroke symptoms.
Clinically the underlying pathology is often challenging to
determine.
• Imaging especially MRI may facilitate the rapid evaluation and
limit the differential the differential diagnosis thus helping the
clinician to clinch the exact diagnosis.
Questions and suggestions to: skanekar@hmc.psu.edu
THANK YOU
Penn State Presentation
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