Inflammatory and Autoimmune Diseases of the Pediatric Central Nervous System

Sangam Kanekar, MDDept of Radiology and Neurology

Penn State Milton S Hershey Medical CenterHershey, 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

IntroductionCNS 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 relapsing-remitting 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

NON-VASCULITIC INFLAMMATORY BRAIN DISEASE

• Demyelinating disease • Antibody mediated inflammatory

brain disease• T-cell mediated inflammatory

brain disease• Granulomatous inflammatory

brain disease• Miscellaneous

Primary CNS vasculitis (cPACNS)Angiography positive, large-medium vessel cPACNSAngiography negative, small vessel cPACNS

Secondary CNS vasculitis • Infection associatedViral Bacterial Fungal Spirochaete• Post-infectious• Systemic vasculitisANCA-associated vasculitis• Systemic rheumatic disease• Systemic inflammatory disease• ExposuresRadiationDrugs (cocaine, amphetamines)

Inflammatory and Autoimmune Diseases

VASCULITIC INFLAMMATORY BRAIN DISEASE

NON-VASCULITIC INFLAMMATORY BRAIN DISEASE

Demyelinating disease Acute demyelinating encephalomyelitis (ADEM)Multiple sclerosisTransverse myelitis (when demyelinating)Antibody mediated inflammatory brain diseaseAnti-NMDAR encephalitisAntibody-mediated limbic encephalitisNeuromyelitis opticaHashimoto encephalitisT-cell mediated inflammatory brain diseaseRasmussen’s encephalitisGranulomatous inflammatory brain diseaseNeurosarcoidosisBlau syndromeOthers Febrile infection-related epilepsy syndrome (FIRES)

Monophasic

Clinically Isolated Syndrome (CIS)Monophasic Acute Disseminated Encephalomyelitis (ADEM)Monophasic Neuromyelitis Optica (NMO)

Multiphasic ADEMMultiple Sclerosis (MS)Relapsing Neuromyelitis Optica (NMO)

Demyelinating Syndromes

Polyphasic

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.

International MS Study Group monophasic ADEM criteria• No history of prior demyelinating event• First clinical event with presumed inflammatoryor 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 theacute 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

Summary of revised criteria

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

Bilateral ACUTE loss of visionAcute 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 someof the lesions.

Acute demyelinating encephalomyelitis (ADEM)

Acute demyelinating encephalomyelitis (ADEM)

large, confluentlesions

ADEM

48 HRS

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.

INFLAMMATORY

12 yrs old child presented with motor and sensory deficit.

Multiple sclerosis

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 (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.

Classic MR appearance

20052003

Multiple sclerosis

Relapsing-Remitting MS

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 Time• New lesion on follow-up scan

(irrespective of time interval)

• At least 1 enhancing lesion and 1 non-enhancing lesion on baseline scan

Dissemination in space • > 1 T2- lesion in at least 2 of 4 typical

locations• Periventricular• Juxtacortical• Infratentorial• Cord

Multiple sclerosis

ON remains a clinical diagnosis, and the main role of MR is in the identification of white matter lesions and prognosis for MS

Optic Neuritis (ON)

Multiple sclerosis with optic neuritis

Monocular Blindness

ADEM MS

children, 5-8 years Middle age female

females = males F>>M

pathogenesis of a precedinginfection

Autoantibody/perivenular inflammation

mild increase intotal protein.

Oligoconal bands

Complete recovery has been reported in 53% to94% 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 COMMONCorpus callosum involved

Both optic nerves are involved Uni lateral optic nerve

Neuromyelitis opticaInflammatory 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 CriteriaAll 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 opticaNeuromyelitis 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.

MS DNMO

Middle age higher age at onset

steroids first line of treatment steroids not helpful

Imaging: Cerebral white matter is the primary target Spinal cord lesions are focal < 2-3 segmentsSpinal cord atrophy. cranial nerves or cerebellar involvement are common in MS but are not resent in DNMO

Imaging: No cerebral white matter lesions are presentspinal cord lesions are confluent and extend to multiple segmentsspinal 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

DNMOMSINFLAMMATORY

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.

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.

a b c,d

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.

Acute Necrotizing Encephalopathy (ANE)

Encephalitis of limbic system is most often due to infection or autoimmune etiology. Autoimmune limbic encephalitis may be due to tumors (paraneoplastic) or non-paraneoplastic 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).

Limbic encephalitis

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 encephalitisEncephalopathy 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.

-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.

Brain biopsy in Rasmussen's encephalitis showing lymphocytic infiltrates staining for CD8 on immunohistochemistry

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.

Rasmussen’s encephalitis

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

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.

SARCOIDOSIS

Febrile infection-related epilepsy syndrome (FIRES)

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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.

VASCULITIC INFLAMMATORY BRAIN DISEASEPrimary CNS vasculitis (cPACNS): Angiography

positive, large-medium vessel cPACNS

Primary CNS vasculitis (cPACNS)Angiography positive, large-medium vessel cPACNSProgressiveNon-progressiveAngiography negative, small vessel cPACNS

Secondary CNS vasculitis Infection associatedViral (CMV, EBV, HIV)Bacterial (mycobacterium tuberculosis, mycoplasma pneumonia, streptococcus pneumonia, others)Fungal (candida albicans, aspergillus, actinomyces, others)Spirochaete ( Borrelia burgdorferi )

Post-infectiousVZV – post-varicella angiopathySystemic vasculitisANCA-associated vasculitis, polyarteritis nodosa, takayasu arteritis,Systemic rheumatic diseaseSLE, systemic vasculitis, scleroderma, dermatomyositis, othersSystemic infl ammatory diseaseHemophagocytic lymphohistiocytosis, Kawasaki disease, inflammatory bowel disease, celiac disease, graftversus host diseaseExposuresRadiationDrugs (cocaine, amphetamines)

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 inflamma tory changes in CNS blood vessels are secondary due to collagen vascular diseases, infections, tumors, and sub stance abuse it is called secondary vasculitis. Primary vasculitides may prefer entially 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 sys tem (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.

Primary CNS vasculitis (cPACNS)

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

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.

Bacterial MeningitisBacterial

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.

Viral Infections

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.

Post-infectious: VZV – post-varicella angiopathy

Vasculitis associated with systemic diseaseVarious 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.

6/19

6/24

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.

SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

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

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.

e.g. Hemophagocytic lymphohistiocytosis, Kawasaki disease, inflammatory bowel disease, celiac disease, graft versus host 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.

KAWASAKI DISEASE

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.

RADIATION ENCEPHALOPATHYRadiation 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.

Exposures

VASCULOPATHY IN DRUG ABUSERS

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.

Drugs & vasculitis

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.

THANK YOU

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Questions and suggestions to: skanekar@hmc.psu.edu

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