Inflammatory Disorders | The Massachusetts General Hospital Handbook of Neurology

Authors: Flaherty, Alice W.; Rost, Natalia S.

Title: Massachusetts General Hospital Handbook of Neurology, The, 2nd Edition

> Table of Contents > Adult Neurology > Inflammatory Disorders

Inflammatory Disorders

A. See also

Demyelinating Disease (p. 33), myositis (p. 83).

B. Vasculitis

Angiitis and vasculitis are synonyms. Arteritis is vasculitis restricted to arteries.

1. Angiitis, primary CNS: Small-medium arteries.
- a. CNS sx: HA, encephalopathy, stroke/bleed.
- b. PNS sx: None.
- c. Systemic sx: None.
- d. Tests: Angiogram, MRI, bx (leptomeninges and cortex).
- e. Rx: High-dose steroids cyclophosphamide.
2. Angiitis, hypersensitivity: Small vessels.
- a. CNS sx: Variable.
- b. PNS sx: Asymmetrical, painful distal sensorimotor neuropathy, mononeuritis multiplex.
- c. Systemic sx: Purpura.
- d. Tests: Evaluate potential causes (drugs, infections, cancer, connective tissue dz, cryoprotein).
- e. Rx: Treat cause.
3. Arteritis, giant cell (temporal arteritis): Extracranial branches of the aorta.
- a. CNS sx: HA (esp. unilateral temporal), ischemic optic neuropathy, transient monocular blindness, TIA/stroke.
- b. PNS sx: None.
- c. Systemic sx: Jaw claudication.
- d. Tests: ESR (often >100 mm/h), D-dimer, fibrinogen, CBC (anemia common), temporal artery bx.
- e. Rx: Prednisone 60 mg/d. Start at clinical suspicion short-term use will not affect bx results.
4. Arteritis, Takayasu's: Extracranial branches of the aorta.
- a. CNS sx: TIA/stroke (due to large-vessel stenosis).
- b. PNS sx: None.
- c. Systemic sx: None.
- d. Tests: Aortic angiography.
- e. Rx: Steroids.
5. Polyarteritis nodosa: Small-medium arteries.
- a. CNS sx: Cranial neuropathies, stroke/hemorrhage, encephalopathy, seizures.
- b. PNS sx: Mononeuritis multiplex, polyneuropathy.
- c. Systemic sx: Any organ, esp. kidney.
- d. Tests: p-ANCA, MRI, visceral angiogram, EMG, nerve/muscle bx.
- e. Rx: Cyclophosphamide.

C. Connective tissue dz

1. Beh et's syndrome: Small vessels. Higher prevalence along old silk route in Near East.
- a. CNS sx: Subacute brainstem dysfunction (cranial nerve palsies, cerebellar dysfunction, long-tract signs), HA, MS-like sx.
- b. PNS sx: None.
- c. Systemic sx: Uveitis, erythema nodosum, skin lesions, oral/genital ulcers.
- d. Tests: MRI often shows brainstem and subcortical nuclei lesions.
2. Rheumatoid arthritis:
- a. CNS sx: Atlantoaxial subluxation with cord compression, vasculitis (rare).
- b. PNS sx: Compression neuropathies.
- c. Systemic sx: Arthritis, cardiac complications.
- d. Tests: Rheumatoid factor, MRI.
- e. Rx: NSAIDS, TNF- inhibitors, steroids, methotrexate.
3. Systemic lupus erythematosus:
- a. CNS sx: Psychiatric sx, cognitive deficits, seizures, cranial nerve palsies, HA, myelitis, stroke/bleed, MS-like sx, vasculitis.
- b. PNS sx: Polyneuropathy.
- c. Systemic sx: Inflammation of joint, skin, heart, lungs, kidney.
- d. Tests: ANA (high sensitivity, low specificity), anti-dsDNA (low sens, high spec), antiphospholipid antibodies, MRI.
- e. Rx: Steroids, cyclophosphamide; warfarin or aspirin for thrombotic events due to antiphospholipid syndrome (q.v. p. 192).
4. Sjogren's syndrome:
- a. CNS sx: MS-like disease, vasculitis (rare).
- b. PNS sx: Dorsal root ganglionopathy (may lead to sensory ataxia), polyneuropathy.
- c. Systemic sx: Dry eyes, dry mouth.
- d. Tests: ANA, anti-Ro, anti-La (not sensitive or specific); antiphospholipid antibodies, Schirmer's lacrimal flow test, salivary gland bx.
- e. Rx: High-dose steroids cyclophosphamide.
5. Wegener's granulomatosis: Small-medium arteries.
- a. CNS sx: Cranial neuropathies, basilar meningitis, optic nerve sx, pituitary sx, stroke/bleed, seizures.
- b. PNS sx: Polyneuropathy.
- c. Systemic sx: Lung and kidney.
- d. Tests: MRI, c-ANCA, bx.
- e. Rx: Cyclophosphamide.
- f. Rx: High-dose steroids cyclophosphamide other immunosuppressants.