Common Neuroimaging Findings in Autoimmune Disorders

R Prativadi, R Mangla, J Almast, A CocaUniversity of Rochester Medical Center

eEdE-25 #316

Disclosures

• None

Purpose/Educational Objectives

• The purpose of this exhibit is to review neuroimaging findings in Systemic Autoimmune Disorders (SAD) with a focus on SLE (systemic lupus erythematosus), RA (rheumatoid arthritis), SSC (systemic sclerosis/scleroderma), SS (Sjogren’s syndrome), PAN (polyarteritis nodosa), GCA (giant cell arteritis), Takayasu’s Arteritis, and Behcet’s Syndrome.

Background• Systemic autoimmune disorders (SAD) comprise of a group of

disorders whose common denominator is the presence of an idiopathic systemic autoimmune process. The normal mechanisms of immune self-tolerance fail.

• These conditions including systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis/scleroderma, Sjogren’s syndrome, polyarteritis nodosa, giant cell arteritis, Takayasu’s Arteritis, Behcet’s Syndrome, may have their initial presentation as CNS diseases.

• Early assessment and recognition of these neuroimaging findings may be helpful in averting the development of serious complications.

• Clinical and neuroimaging manifestations can be quite varied with much overlap in these SAD disorders

Systemic Lupus Erythematosus (SLE)

• Complex autoimmune disease involving multiple systems. Can be classified as a vasculitis. Affects multiple components of the immune system which generally tends to have a relapsing remitting course

• Neurologic findings : vasculitis, infarctions, polyneuropathy, mononeuritis multiplex, myopathy, cerebritis, seizures, headache, pseudotumor cerebri, transverse myelitis, meningitis, optic neuritis, venous sinus thrombosis, hemorrhage, abscess, encephalopathy

23 y/o female with flaccid lower extremities and altered mental status.

T2 FLAIR –abnormal FLAIR intensity in the bilateral basal ganglia

T1 +C –small irregular foci of enhancement in the basal ganglia with linear enhancement seen in both hemispheres

T2 Flair image (A) demonstrates abnormal increased signal in the basal ganglia and post contrast T1 image (B) demonstrates small irregular foci of enhancement in the basal ganglia with linear enhancement seen in both hemispheres. These findings are consistent with cerebritis secondary to SLE.

A B

39 y/o female with right extremity weakness and garbled speech.

DWI image (A) demonstrates abnormal restricted diffusion in the left MCA territory. Swan image (B) demonstrates blooming in the left basal ganglia. TOF MRA (C) demonstrates an irregular left M1 segment These findings are consistent with left MCA stroke with hemorrhagic conversion in the basal ganglia secondary to SLE.

A CB

52 y/o female with visual changes and hypertension.

T2 FLAIR image (A) demonstrates abnormal hyperintensity in the bilateral occipital lobes without corresponding restricted diffusion on DWI (B). These findings are consistent with posterior reversible encephalopathy syndrome (PRES) secondary to SLE.

A B

52 y/o female with vision loss.

T1 +C (A) image demonstrates two subtle foci of enhancement in the right optic nerve. These findings are consistent optic neuritis/active demyelination secondary to SLE.

A

52 y/o female with right sided weakness.

T2 image demonstrates abnormal T2 hyperintensity within the C2-C5 spinal cord with associated abnormal regions of enhancement at the same level on the T1 +C (A) image. These findings are consistent transverse myelitis/active demyelination secondary to SLE.

A B

Rheumatoid Arthritis (RA)

• Multiorgan systemic inflammatory disease typically affecting synovial tissue.

• Neurologic findings: Atlantoaxial subluxation, polymyositis, mononeuritis multiplex, peripheral neuropathy, rheumatoid nodules in central/peripheral nervous system, vasculitis causing stroke and/or neuropathy

A B

64 year old male presents with cognitive impairment and worsening lower extremity sensory symptoms

T2 Flair image (A) demonstrates abnormal increased signal in the anterior frontal lobes and post contrast T1 image (B) demonstrates abnormal parafalcine dural and leptomeningeal enhancement. These findings are consistent with vasculitis secondary to rheumatoid arthritis.

Sjogren’s Syndrome (SS)

• Lymphocytic infiltration of exocrine glands (lacrimal and salivary) with resultant dysfunction of those glands. Associated with hyperactivity of B-lymphocytes.

• Neurologic findings: keratoconjunctivitis sicca (resulting from the involvement of lacrimal glands), xerostomia (resulting from that of salivary glands), polyneuropathy, myelopathy, motor neuron syndromes, cognitive dysfunction, ganglioneuropathy, myelitis, optic neuritis

A B

38 year old male presents with dry mouth

T1 image (A) and T2 image (B) demonstrates abnormal high signal in the bilateral parotid glands. Findings are consistent with fatty atrophy of the parotid glands secondary to Sjogrens Syndrome.

Systemic Sclerosis/Scleroderma (SSC)

• Multiple system connective tissue disorder. Characterized by widespread deposition of collagen. Common involvement includes joints of the hands and ankles. Esophageal involvement is common.

• Neurologic Findings: : Proximal myopathy, brachial plexopathy, intracerebral inflammation, cranial neuropathy, headache, seizures, stroke, vascular disease

• Neuroimaging is rare

Polyarteritis Nodosa (PAN)

• Systemic inflammatory vasculitis involving the small to medium sized arteries. Typically has necrotizing inflammation of the arteries leading to micro aneurysms and focal ruptures.

• Neurologic findings: infarction, aneurysm, mononeuritis multiplex, polyneuropathy, myopathy

• Neuroimaging is rare

High resolution vascular wall imaging on MRI

• Vasculitis is commonly seen in SAD• Vasculitis can commonly be the cause of neurologic

findings in SAD• High resolution vessel wall imaging is important in

evaluating for vasculitis• Our institution aquires a T1 fast spine echo with

contrast (GE CUBE)• T1 fast spine echo with contrast images

demonstrate a thickened enhancing wall, due to vessel wall inflammation.

46 y/o female with left sided weakness.

MRI CUBE +C image (A) demonstrates a thickened enhancing wall of the right cavernous ICA. These findings are consistent with vasculitis.

A

Giant Cell Arteritis (GCA)

• Granulomatous vasculitis affecting large and medium sized arteries. Propensity to involve extra-cranial carotid artery branches.

• Neurologic findings: headache, visual loss due to lesions of optic nerve or retina, papilledema, stroke (posterior circulation)

60 y/o female with right vision loss.

CTA (A) image demonstrates complete occlusion of the right petrous portion of the ICA. These findings are consistent with vasculitis secondary to Giant Cell Arteritis.

A

Takayasu’s Arteritis

• Large vessel granulomatous vasculitis predominantly affecting the aorta and its major branches.

• Neurologic findings: stroke. amaurosis fugax, visual loss, headache

A B

12 year old male presents vision changes and headache

CTA head image(A) demonstrates moderate narrowing of the cavernous segment of the right ICA and severe narrowing of the left cavernous segment of the left ICA. CTA neck image (B) demonstrates severe diffuse narrowing of the left cervical ICA. These findings are consistent with vasculitis secondary to Takayasu’s Arteritis.

Behcet’s Syndrome

• Multisystem chronic inflammatory vasculitis. Classic triad consists of oral ulceration, genital ulceration, and ocular manifestations.

• Neurologic findings: stroke, seizures, cranial nerve palsy, multifocal parenchymal involvement, meningoencephalitis, headaches, cerebral venous sinus thrombosis

A B

50 year old male presents with cognitive impairment and worsening sensory symptoms

T2 Flair image (A) demonstrates abnormal increased signal in the pons and post contrast T1 image (B) demonstrates abnormal ring enhancement of the lesion. Findings are consistent with active demyelination likely secondary to visculitis seen in Behcets disease.

Conclusion

• Systemic Autoimmune Disorders includes a wide spectrum of disease processes

• Neurologic sequela can be seen in SAD and must be kept in mind within the differential diagnosis

• Clinical and neuroimaging manifestations can be quite varied with much overlap in these SAD disorders

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