Rheumatology and Neurology (Neurology of Systemic Disease 2017)

Educational Objectives

The goal of this lecture is to improve diagnosis and treatment of systemic diseases with neurologic manifestations. After hearing and assimilating this lecture, the clinician will be better able to:

1. Recognize neurologic manifestations in a patient with systemic lupus erythematosus.
2. Use clinical history and neurophysiologic studies to diagnose neurologic ­findings in patients with connective tissue disorders and vasculitides.

Summary

Systemic lupus erythematosus (SLE): Neurologic manifestations vary; diagnostic criteria mention seizures, psychosis, mononeuritis multiplex, myelitis, peripheral neuropathy, cranial neuropathy, and acute confusional state; prevalence of neuropsychiatric lupus estimated at 50% to 60%; central nervous system (CNS) manifestations seen in 13% to 92% of patients; headache most common neuropsychiatric manifestation; others include mood disorders, general cognitive dysfunction, seizures, and cerebrovascular disease; inflammation component of vasculopathy and atherosclerosis; patients may develop atherosclerosis prematurely; peripheral nervous system (PNS) manifestations less common; peripheral neuropathy most common presentation in PNS, followed by cranial neuropathy and other mononeuropathies (eg, mononeuritis multiplex); peripheral neuropathy usually distal, sensory or sensorimotor, and symmetric; autonomic dysfunction common.

Pathophysiology: Many connective tissue diseases associated with vasculitis; inflammatory changes more likely to affect CNS than PNS; in patients newly diagnosed with neuropsychiatric SLE, general white matter hyperintensities most common finding on MRI and may represent central inflammation.

Rheumatoid arthritis (RA): Most common inflammatory arthritis; neurologic manifestations may arise from inflammation or external compression; atlantoaxial subluxation — concerning manifestation; subluxation usually anterior and may lead to compression of nerve root and spinal cord with myelopathy; vertical subluxation less common (associated with descent of foramen magnum and apparent upward migration of odontoid); condition may cause compression of brainstem and cervical spine, cranial nerve dysfunction, basilar artery stroke, or cardiac arrest; numerous central and peripheral manifestations complicate diagnosis; other associated peripheral neuropathies — systemic vasculitis; mononeuritis multiplex; cutaneous vasculitis with low levels of complement (especially C4) and involvement of more than two limbs predicts mortality.

Sjögren syndrome: Occurs in isolation (primary) or with other connective tissue diseases (secondary); peripheral neuropathy most common neurologic sign and often presenting feature; classic signs include sensory neuronopathy or ganglionopathy; non–length-dependent sensory dysfunction associated with lymphocytic infiltration of dorsal root ganglia; on electrophysiologic studies, reduced amplitude or loss of sensory response (particularly in upper limb) with preserved motor response strongly suggests Sjögren syndrome; unilateral or bilateral trigeminal neuropathy possible.

Scleroderma (progressive systemic sclerosis): Pathology of internal organs and neurologic manifestations may be disabling; types of systemic sclerosis include diffuse cutaneous, limited cutaneous, localized, and systemic sclerosis without scleroderma; localized scleroderma includes morphea, linear scleroderma, and coup de sabre; neurologic manifestations usually peripheral, but epilepsy possible, especially with coup de sabre; myopathy common (prevalence up to 90%) and usually inflammatory; corticosteroids effective but may cause renal crisis in patients with diffuse cutaneous systemic sclerosis.

Vasculitis: Clinician should determine whether condition confined to nervous system or systemic, then assess whether condition primary or secondary to infection, chronic inflammation, or connective tissue disease.

Isolated vasculitides of nervous system: Primary angiitis of CNS — rare disorder; affects small vessels; meninges and cortex often show fibrinoid necrosis or granulomatous disease; presentation may be vague or mimic other disorders; patients may present with headache, cognitive decline, encephalopathy, ischemic or hemorrhagic stroke, seizures, or inflammatory disease with meningitis or myelopathy; systemic symptoms suggest secondary CNS vasculitis rather than primary angiitis; disorder must be distinguished from reversible cerebral vasoconstriction syndrome (reversible cerebral vasoconstriction syndrome more likely in patient with normal MRI; infarcts on MRI suggest angiitis); diagnosis depends on biopsy and exclusion of other diagnoses, including systemic vasculitis; nonsystemic vasculitic neuropathy — most common vasculitic neuropathy; associated with better prognosis compared with systemic vasculitides; mononeuritis multiplex most common manifestation; usually responds to treatment; as in angiitis of CNS, sensitivity of peripheral nerve biopsy low (~50%); onset acute or subacute, with painful asymmetric mononeuritis multiplex; necrotizing vasculitis typically affects epineurial vessels, but perivascular inflammation possible; biopsy shows thickened vessel walls and ischemia.

Secondary vasculitides: Include Takayasu arteritis, giant cell arteritis (with possible involvement of temporal arteries), polyarteritis nodosa (PAN), Kawasaki disease, microscopic polyangiitis (no longer included with PAN), granulomatosis with polyangiitis (formerly Wegener granulomatosis), eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), cryoglobulinemia, Behçet disease, and Cogan syndrome; these disorders may cause neurologic impairment.

Large-vessel vasculitides: Granulomatous and more common in women; Takayasu usually found in patients <40 years of age, while giant cell arteritis found in patients >50 years of age (these entities may be same disease); disorders affect aorta and its branches; neurologic manifestations develop in minority of patients and may include dizziness, visual changes, headache, and ischemic stroke; diagnosis should be considered in patient with large-artery stroke; development of posterior reversible encephalopathy syndrome is possible.

Medium-vessel vasculitides: PAN — idiopathic or associated with chronic infection (especially hepatitis B); antineutrophil cytoplasmic antibody (ANCA)-negative systemic syndrome; PNS often involved; among patients with PAN, ~68% of those without hepatitis B and ~85% of those with hepatitis B have peripheral neuropathy; mononeuritis multiplex common; Kawasaki disease — pediatric disorder; neurologic involvement rare; presentation may be vague (ie, irritability) or include aseptic meningitis, meningoencephalitis, seizures, demyelination, mononeuritis multiplex, or cranial neuropathies.

Small-vessel disease: Often ANCA-positive; commonly affects PNS; may cause myalgia and inflammatory myopathy; microscopic polyangiitis and PAN are nongranulomatous; peripheral neuropathy found in half of patients; CNS involvement uncommon; granulomatous polyangiitis — necrotizing vasculitis of upper and lower respiratory tract and kidneys; cranial neuropathies common; inflammatory granulomas may compress nerves in ear, sinuses, or orbit; olfactory and optic nerves most often affected; granulomatous hypertrophic pachymeningitis unique to this disorder; granulomatous infiltration of pituitary gland possible; eosinophilic granulomatosis with polyangiitis — presents with allergic rhinitis, nasal polyps, and sinusitis; patients develop asthma, peripheral hypereosinophilia, and painful vasculitis with peripheral neuropathy; may be caused by leukotriene antagonists; other manifestations in PNS rare, but muscular pain common; ischemic or hemorrhagic stroke of brain or spinal cord rare; cryoglobulinemic vasculitis — occurs with type II and type III mixed cryoglobulinemia; presents acutely as painful asymmetric peripheral neuropathy; patients with cryoglobulinemia should be tested for hepatitis C; Behçet disease — affects vessels of various sizes; headache most common presentation of neuro-Behçet disease; patients may develop oral and genital ulcers, uveitis, skin lesions, arthritis, and gastrointestinal involvement; disease rare and responds to steroids; Cogan syndrome — variable-vessel vasculitis; causes interstitial keratitis and vestibulocochlear dysfunction.

Readings

Dimberg EL. Rheumatology and neurology. Continuum (Minneap Minn) 2017;23(3 Neurology of Systemic Disease).

Disclosures

For this program, the following was disclosed: Dr. Dimberg reports no disclosure. Unlabeled Use of Products/Investigational Use Disclosure: Dr. Dimberg reports no disclosure. To view disclosures of planning committee members with relevant financial relationships, visit: audiodigest.org/continuumaudio/committee. All other members of the planning committee report nothing to disclose.

 

Acknowledgements

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Audio Digest Foundation is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. Audio Digest Foundation designates this activity for 0 CE contact hours.

Lecture ID:

CA061101

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

To earn CME/CE credit for this course, you must complete all the following components in the order recommended: (1) Review introductory course content, including Educational Objectives and Faculty/Planner Disclosures; (2) Listen to the audio program and review accompanying learning materials; (3) Complete posttest (only after completing Step 2) and earn a passing score of at least 80%. Taking the course Pretest and completing the Evaluation Survey are strongly recommended (but not mandatory) components of completing this CME/CE course.

Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.

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