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Neurological Manifestations of Selected Medical Conditions 1. Lupus 2. HIV/AIDS

Neurological Manifestations of Selected Medical Conditions 1. Lupus 2. HIV/AIDS. Chenjie Xia Neurology AHD Wednesday, February 2, 2011. Lupus. Lupus. What are the diagnostic criteria for SLE?. Lupus. Need 4 / 11 of the following: Discoid rash Oral ulcers Photosensitive rash Arthritis

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Neurological Manifestations of Selected Medical Conditions 1. Lupus 2. HIV/AIDS

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  1. Neurological Manifestations of Selected Medical Conditions1. Lupus2. HIV/AIDS Chenjie Xia Neurology AHD Wednesday, February 2, 2011

  2. Lupus

  3. Lupus • What are the diagnostic criteria for SLE?

  4. Lupus • Need 4 / 11 of the following: • Discoid rash • Oral ulcers • Photosensitive rash • Arthritis • Malar rash • Immunologic criteria (anti-dsNDA, anti-Sm, VDRL) • NEuropsychiatric manifestations • Renal involvement (proteinuria, cellular casts) • ANA +ve • Serositis (pleural or pericardial) • Hematologic abnormalities (plt / Hgb / WBC)

  5. Lupus • Need 4 / 11 of the following: • Discoid rash • Oral ulcers • Photosensitive rash • Arthritis • Malar rash • Immunologic criteria (anti-dsNDA, anti-Sm, VDRL) • NEuropsychiatric manifestations • Renal involvement (proteinuria, cellular casts) • ANA +ve • Serositis (pleural or pericardial) • Hematologic abnormalities (plt / Hgb / WBC)

  6. Lupus • Clinical pearls: • Malar rash spares NLF, whereas rash in dermatomyositis involves NLF • Oral ulcers are painless

  7. Lupus • Which of the following is not a neurological manifestation of SLE? • A) Headache • B) Acute confusional syndrome • C) Seizure • D) Chorea • E) Myelopathy • F) AIDP / Guillain-Barre syndrome • G) Myasthenia gravis • H) Peripheral neuropathy

  8. Lupus • They all are! • American College of Rheumatology: • Neuropsychiatric SLE  NPSLE

  9. Greenberg, The Neurologist, 2009

  10. Bertsias and Boumpas, Nature Reviews, 2010

  11. Lupus • Some key points: • Lupus can virtually cause any neurological sign and symptom… • Neuropsychiatric manifestation can occur anytime • Preceding, during, or after Dx of SLE • Both in active and inactive states of SLE

  12. Seizing in SLE…

  13. Lupus and Seizures • 42F, known SLE, presents to the ER with one episode of GTC seizure lasting 2 mins, and is now back to baseline. What is your next step? • A) Obtain urgent EEG • B) Load with IV anti-epileptic • C) Start steroids • D) Start steroids and cyclophosphamide • E) Admit and observe in hospital • F) Consult rheumatology

  14. Lupus and Seizures • Seizures • 7-20% of SLE patients report seizures • Pathophysiology: • Direct antibody effect with neuronal binding capability • More likely APLA-mediated ischemia small epileptogenic foci • Management • Screen for APLA antibodies, treat for thrombosis (if indicated) • AED (may be withheld if no brain lesions or EEG abnormalities) • immunosuppressants considered (if seizure 2/2 inflammation)

  15. Lupus and Seizures • 42F, known SLE, presents to the ER with one episode of GTC seizure lasting 2 mins, and is now back to baseline. What is your next step? • A) Obtain urgent EEG • B) Load with IV anti-epileptic • C) Start steroids • D) Start steroids and cyclophosphamide • E) Admit and observe in hospital • F) Consult rheumatology

  16. Lupus and Seizures • None of the above! • First step: r/o other causes • obtain imaging, as we do for all other patients who present with first episode of seizure! (CT on urgent basis, then MRI)

  17. Bertsias and Boumpas, Nature Reviews, 2010

  18. Lupus and Seizures • Important THM: • Never automatically attribute neurological S/Sx to SLE • Always r/o other causes • E,g hypo/hyperglycemia, uremia, lytes abnormalities, liver/thyroid disease, vitamin deficiencies, medication side-effects • Avoid framing bias!

  19. Making sense of SLE, APLAs and ischemic stroke…

  20. Lupus and Stroke • What is antiphospholipid syndrome? • What are antiphospholipid antibodies?

  21. Lupus and Stroke • Antiphospholipid syndrome (APS): • Presence of antiphospholipid antibodies (confirmed on repeat testing, separated by 6-12 wks) AND • Presence of clinical event (developing thrombosis or miscarriage)

  22. Lupus and Stroke • Antiphospholipid antibodies • a) Lupus anticoagulant (LA) • Recognizes negatively charged phospholipids • b) Anticardiolipin antibody (aCL) • Recognizes negatively charged phospholipids • c) Plasma protein beta-2 glycoprotein I • Less well known, but may be more clinically relevant

  23. Lupus and Stroke • Lupus anticoagulant (LA) • Anticoagulant: PTT • A misnomer: prothrombotic • Non-specific inhibitor: • PTT • does not correct with adding normal plasma • PTT corrects when phospholipids added • dRVVT (diluted Russel viper venom time) •  clotting time des not correct with normal plasma •  clotting time corrects when phospholipids added

  24. Lupus and Stroke • Anticardiolipin (aCL) antibodies • Screened for with ELISA targeting aCL IgM, IgG and IgA

  25. Lupus and Stroke • True or false: All patients with SLE have antiphospholipid antibodies and vice versa.

  26. Lupus and Stroke • False • Not all patients with SLE have APS • APS is only 1 of 11 diagnostic criteria for SLE • Not all patients with APS have SLE • APS can be a secondary condition related to SLE • APS can be a primary condition on its own • Remember, presence of APLAs does NOT automatically mean APS • Vila et al. 1994: 552 normal donors, 15.9% had aCL antibodies and no thrombotic events within 12 mos of f/u

  27. Lupus and Stroke • True or false: The risk of ischemic stroke is increased in SLE patients only if they have antiphospholipid antibodies.

  28. Lupus and Stroke • False •  risk of ischemic stroke in: • Patients with APLAs, but no SLE • SLE patients with APLAs • SLE patients without APLAs (due to  whole blood viscosity?)

  29. Lupus and Stroke • Management of patients ischemic stroke and APLAs: • Controversial for both primary and secondary prevention management • Secondary prevention: • APLAs and Stroke Study  No difference b/w ASA and coumadin • NEJM 2003: No difference between INR 2-3 and INR 3-4 • Current guidelines • +APLA, -stroke: ASA only • +APLA, +stroke: coumadin INR 1.4-2.8 • +APLA, +++stroke: coumadin INR > 2.8 OR coumadin + ASA

  30. Lupus and Stroke • Management of ischemic stroke in SLE • Acute managementsimilar to non-SLE patients with ischemic stroke • Secondary prevention • Antiplt, lifestyle, control of vascular RFs, +/- CAE • +++APLAs: consider oral A/C target INR 2-3 • Recurrent events + APLAs: target INR > 3 or coumadin + antiplt

  31. Bertsias and Boumpas, Nature Reviews, 2010

  32. Lupus and Stroke • Types of strokes in SLE: • Ischemic stroke or TIA (>80%) • Multifocal disease (7-12%) • Intracerebra hemorrhage (7-12%) • SAH (3-5%) • Sinus thrombosis (2%)

  33. The Story of SLE and Demyelination…

  34. Lupus and Demyelination • Can you reliably differentiate multiple sclerosis from SLE with demyelinating disease? • A) No, patients presenting with a classic demyelinating episode need to be screened for systemic rheumatological conditions • B) Yes, demyelination is monophasic in SLE and recurrent in MS. • C) Yes, the McDonald’s criteria can be used to differentiate MS from SLE demylination • D) Yes, IgG index and  OCBs are not seen in SLE • E) Yes, MS patients do not have positive ANA

  35. Lupus and Demyelination • Can you reliably differentiate multiple sclerosis from SLE with demyelinating disease? • A) No, patients presenting with a classic demyelinating episode need to be screened for systemic rheumatological conditions • B) Yes, demyelination is monophasic in SLE and recurrent in MS. • C) Yes, the McDonald’s criteria can be used to differentiate MS from SLE demylination • D) Yes, IgG index and  OCBs are not seen in SLE • E) Yes, MS patients do not have positive ANA

  36. Lupus and Demyelination • First demyelinating event: • Screen for rheumatologic condition • Clinical symptoms • Serologic evidence • “lupus sclerosis” • May need acute aggressive immunosuppression (e.g. pulse steroids, cyclophosphamide)

  37. Greenberg, The Neurologist, 2009

  38. Lupus and Demyelination • Lupus and transverse myelitis • Patients with SLE are at risk for TM • Lesions in SLE tend to be larger, more edematous and cause more damage than in MS • MRI, CSF • Start antimicrobials empirically if CSF inflammatory • Initiate immunosuppression early (w/i hours) • Lesions similar to those in NMO and neurosarcoidosis (may be part of same spectrum?)

  39. Lupus and Neurology Hodgepodge

  40. Lupus and Headache • Types of headache • Migraine with/without aura, tension headache • Prevalence • Documented in over 50% of SLE patients • Strength of association • Convincing evidence for association lacking • If no red flags, manage as other HA patients

  41. Lupus and Cognition • Degree • Range of severity, definitions vague • Prevalence • If any cognitive deficit: up to 80% • If mild deficit discounted: <24% • Severe deficits uncommon: 2-5% • Pathophysiology • Not well understood (?autoimmune) • Not correlated with CNS events (e.g. stroke, seizure) • Tend to persist despite control of systemic SLE

  42. Bertsias and Boumpas, Nature Reviews, 2010

  43. Lupus and Confusion • Acute Confusional State (ACS) • Heterogeneous etiology • Antineuronal abs, CVST, ischemic stroke, seizures, white matter lesions, SiADH • Diagnosis of exclusion in SLE patients • Early imaging • R/o other causes (e.g. infection, metabolic)

  44. Lupus and Confusion

  45. Bertsias and Boumpas, Nature Reviews, 2010

  46. Lupus and Abnormal Movements • Types • Chorea *** • Others: hemiballism, parkinsonian • Screen for SLE in young patients presenting with abnormal movements • Pathophysiology • Direct antibody-mediated? • Small vessel ischemia

  47. Lupus and Polyneuropathy • Most common: symmetric, length-dependent polyneuropathy • May be related to glucose intolerance due to prolonged steroid use • Overall rare, SLE patients make up very small proportion of patients with neuropathy

  48. Final Words on ANA • Which of the following is false? • A) Serum ANA is often the most cost-effective way to screen for lupus. • B) Serum ANA is helpful only when systemic manifestations for SLE are present. • C) There are a variety of overlap syndromes in which ANA is positive. • D) Detailed history and physical exam help to screen for SLE.

  49. Final Words on ANA • Which of the following is false? • A) Serum ANA is often the most cost-effective way to screen for lupus. • B) Serum ANA is helpful only when systemic manifestations for SLE are present. • C) There are a variety of overlap syndromes in which ANA is positive. • D) Detailed history and physical exam help to screen for SLE.

  50. Lupus and Management • Immunosuppresive therapy • Corticosteroids (only FDA-approved Rx for SLE) • +/- another immunosuppressant (Imuran, Cyclophosphamide) • If refractory to above: PLEX, IVIG, rituximab • Antiplatelet +/- anticoagulation (when indicated) • Symptomatic treatment (when indicated) • E.g. anticonvulsants, antidepressants, antipsychotics

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