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Medical grandrounds

Kathryn Hazel C. Trinidad M.D. First Year Medical Resident. Medical grandrounds. To be able to present a known case of SLE who developed seizure during the course of illness To be able to know the diagnosis, treatment, and prognosis of NPSLE

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Medical grandrounds

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  1. Kathryn Hazel C. Trinidad M.D. First Year Medical Resident Medical grandrounds

  2. To be able to present a known case of SLE who developed seizure during the course of illness To be able to know the diagnosis, treatment, and prognosis of NPSLE To be able to know the latest updates with regards to NPSLE Objectives

  3. H.M • 35 year old G3P2 (1112) female • right-handed • known case of SLE maintained on Prednisone 20mg 2x/day • known case of APAS, maintained on ASA 80mg OD • housewife The case

  4. Seizure Chief Complaint

  5. Interval History • May 2002  experienced on-off fever (Tmax 40C), malar rash, joint pains, vomiting, photosensitivity, and oral ulcers. diagnosed with SLE; started on Prednisone 5mg OD; regular OPD follow-up • Nov 2007  (+) miscarriage; OPD follow-up for clearance prior to dilatation and curettage

  6. Interval History • Feb 2008  diagnosed with APAS based on history and lab results: She was started on ASA 80mg OD

  7. Interval History 9 months PTA(July 2008)  1st prenatal check-up at 7wks AOG. No rashes, joint pains, and oral ulcers; Started on Heparin 5,000 IU SQ OD Prednisone 5mg OD and ASA 80mg OD continued. Monthly prenatal check-up with both Rheumatology and OB-GYN services

  8. Interval History • 2 months PTA(March 2009)  gave birth to a live preterm male via LTCS; Discharged with ASA 80mg OD and Heparin 5,000 IU/mL; Prednisone was not resumed

  9. History of present illness • 11days PTA, OPD ff up with • (+)occasional upper back pain, grade I bipedal edema, raised macules on both face and upper extremities. • Prednisone 5mg OD was resumed. CBC, urinalysis and ESR were requested.

  10. History of Present Illness • 4 days PTA  (+) diffuse headache & joint pains; (+) fever; inc. rashes in face & extremities; consult done  Prednisone inc. to 20mg BID; Advised ff up of lab requests • 3 days PTA  pancytopenia: (Hgb-9.5 hct-30.1 wbc-2,640 plt-120,000) and proteinuria(+4) hematuria(1171/hpf), pyuria(335/hpf) bacteriuria(404); ESR was elevated at 111. Ciprofloxacin 500mg BID

  11. History of Present Illness • 7 hrs PTA  severe headache (10/10)slightly relieved by Paracetamol. (-) vomiting nor blurring of vision • 30 mins PTA tonic-clonic seizure ~ 30 minutes MMC ER  Admission

  12. Non-hypertensive Non-diabetic s/p CS (2001) s/p dilation and curettage November 2007 No history of travel outside of Metro Manila. Past Medical History

  13. Family History: • No hypertension • No diabetes • No connective tissue disease. Personal and Social History: • Non-smoker • Non-alcoholic beverage drinker • Worked as a bank teller prior to being diagnosed with SLE • Currently stays at home and takes care of her children; can do light household chores

  14. Gen. Survey: Drowsy, not in cardio-respiratory distress • Vital signs: BP = 200/100  170/100 HR 81 RR 20 Temp 36.7C • Skin: raised maculeson the face and both upper extremities Physical Exam • HEENT: Anicteric sclerae, pink palpebral conjunctivae, (+) subconjunctival effusion, no tonsillopharyngeal discharge, no cervical lymphadenopathy • Chest: Symmetrical chest expansion, no retractions, clear breath sounds

  15. Heart: Adynamic precordium, distinct S1 and S2, normal rate and rhythm, no murmurs Abdomen: Flabby abdomen, soft, non-tender, normoactive bowel sounds Extremities: Full and equal pulses, no cyanosis and no edema Physical Examination

  16. Neurological Exam • MSE: Drowsy, opens eyes to verbal stimulation but non-sustained, does not follow commands • Cranial Nerves: Pupils 3mm EBRTL; Full EOMS; (+) visual threat on all planes; (-) facial asymmetry; (+) gag • Sensory: Withdraws to pain in all extremities • MMT: Moves all extremities non-purposely • Reflexes: +2 in all reflexes • Pathologic Reflexes: No Babinski • Meninges: Supple neck

  17. 35 year old G3P2 (1112) female known case of SLE on Oral steroids Steroids on hold for 1 month; resumed ~ 1 week prior to onset of symptoms known case of APAS, maintained on ASA 80mg OD Came in for tonic-clonic seizure History of fever, headache, rashes, and joint pains few days prior to seizure Salient Features

  18. Proteinuria, pyuria on urinalysis sample done few days PTA • On PE: • Drowsy, does not follow commands, opens eyes to verbal stimulation (GCS ~ 9 – 10) • Elevated BP upon admission • Raised macules on the face and both upper extremities • Withdraws to pain on stimulation • Moves extremities non-purposely • Intact reflexes • Supple neck Salient Features

  19. Neuropsychiatric SLE R/O CNS infection Lupus nephritis Hypertension, secondary UTI APAS Admitting Impression

  20. Course in the ward

  21. CBC, Urinalysis, C3, 24hour urine, ESR, antiSmith, antidsDNA Complete Blood Count revealed improvement in pancytopenia Hydrocortisone 100mg IV q8 Referral to neurology service NGT feeding started UPON admission

  22. UPON admission…. • 3 episodes of seizures at ER – Midazolam 5mg IV • Loading dose Phenytoin 300mg IV x 2 doses 1 hour apart Phenytoin 100mg IV q8 Citicholine 1g IV q12 Mannitol 20% 75ml IV q6

  23. Upon admission • Urinalysis • Co- amoxiclav 1.2 g IV every 8 hours

  24. Upon admission • Lumbar tap done • CT Scan revealed normal • EEG Methylprednisolone 1 g IV in D5W 500mL x 4hours for 3 days

  25. Upon admission… • BP 180-200/100 • Cardiology referral • Nicardipine drip started • ECG, CXR, 2Decho were normal

  26. 2nd hospital day • (-)seizures • (-)headache • still drowsy but opens eyes to verbal stimuli, moves all extremities spontaneously • BP was 140-150/100mmHg • Phenytoin was shifted to Leviteracetam(Keppra) 500mg ½ tablet BID • Nicardipine overlapped w/ Amlodipine 5mg OD

  27. 3rd hospital day • (-) seizures/headache • Awake, conversant • Mannitol was tapered down to 50mL Q8 then later D/C • Last dose Methylprednisolone pulse therapy • Hydrocortisone 100mg IV q8

  28. 3rd hospital day • 24 hr urine collection proteinuria with a creatinine clearance of 83.5mL/min. • Serum complement C3, anti-SM and anti-dsDNA were requested which showed low C3 levels and positive anti-SM and anti-dsDNA • Urine culture – no growth • Nephrology referral • Imidapril 5mg OD • UTZ guided kidney biopsy contemplated

  29. 4th hospital day • (-) seizures/headache • Awake, conscious, coherent • Tolerated soft diet, NGT removed • Placed on full diet • Hydrocortisone shifted to Prednisone 25mg BID

  30. 5th hospital day • No recurrence of seizures, headache, no fever • Co- Amoxiclav IV was shifted to oral Co- Amoxiclav 625mg BID • ASA discontinued anticipating kidney biopsy • Leviteracetam continued

  31. 6th hospital day • No recurrence of seizures, headache, no fever • Decrease in raised macules on face and UE • CBC showed decrease in platelet to 80,000 • Prednisone increased to 30mg BID • Kidney biopsy to be done as outpatient

  32. 7th hospital day • No recurrence of seizures, headache, no fever, significant decrease in malar rash and macules on upper extremities • Patient cleared for discharge from all services

  33. Patient discharged improved and stable 8th hospital day

  34. NPSLE Lupus Nephritis Hypertension, secondary Urinary Tract Infection, resolved APAS Final Diagnosis

  35. DISCUSSION

  36. Systemic lupus erythematosus (SLE) • multisystem autoimmune connective tissue disorder with various clinical presentations • Affects many organ systems, including the central and peripheral nervous systems and muscles. • 90% of patients are women of childbearing age • Incidence is 12-39 cases per 100,000 people • With full access to medical care, overall survival for SLE is 85% at 5 years and 63% at 15 years Background Harrison’s Principles of Internal Medicine 17th Ed.

  37. PATHOPHYSIOLOGY • SLE is caused by interactions between susceptible genes and environmental factor resulting in abnormal immune response • Hyper-reactivity and hypersensitivity of T and B lymphocytes • Ineffective regulation of antigen availability and ongoing antibody response Harrison’s Principles of Internal Medicine 17th Ed.

  38. End result: sustained production of pathogenic auto-antibodies and formation of immune complexes that bind target tissues, resulting in: • Sequestration and destruction of Ig-coated circulating cells • Fixation and cleaving of complement proteins • Release of chemotaxins, vasoactive peptides, and destructive enzymes into tissues PATHOPHYSIOLOGY

  39. ARA Criteria for diagnosis: 4 out of 11 needed over any span of time of diagnosis Harrison’s Principles of Internal Medicine 17th Ed.

  40. Neuropsychiatric SLE

  41. Historical background • Hebra and Kaposi (1875)  Noted first neurologic involvement in SLE Over the last 3 decades, appreciation of Clinical significance of antineuronal, antiribosomal P, and antiphospholipid antibodies as well as advances in brain Imaging have again altered our concept of NP-SLE • Baum (1904)  Related active delirium, aphasia and hemiparesis to probable disseminated LE • Daly (1945)  conducted 1st modern study of NP-SLE • Lewis (1954)  1st to focus on importance of EEG findings and psychometric testing in patients with NP-SLE Joseph FG, Lammie AG, Scolding NG. CNS lupus: A study of 41 patients. Neurology. 2007

  42. Incidence of neuropsychiatric manifestations in SLE ranges from 14 – 75% Classification and Clinical Presentation • Patients with NP-SLE can present with a myriad of diffuse and/or focal symptoms and signs involving the brain, spinal cord, or peripheral nervous system

  43. Pathologic Classification of CNS changes observed in SLE • Vasculopathy • Hyalinization • Peripheral inflammation w/o infection • Endothelial proliferation w/o infection • Thrombosis • Vasculitis • Hemorrhage • Subarachnoid • Microhemorrhages • Subdural • Intracerebral • Infarction • Microinfarcts • Large infarcts • Infection • Meningitis • Perivascular inflammation with infection • Septic hemorrhages • Focal cerebritis • Vasculitis with infection Joseph FG, Lammie AG, Scolding NG. CNS lupus: A study of 41 patients. Neurology. 2007

  44. Vasculopathy • Auto-antibodies • Others PATHOPHYSIOLOGY OF NERVOUS SYSTEM INVOLVEMENT Schur PH, Khoshbin S. Neurologic manifestations of systemic lupus erythematosus. www.Uptodate.com

  45. 1. Vasculopathy • characterized by small to moderate perivascular accumulation of mononuclear cells, without destruction of the blood vessel. • May have small infarcts • Pathogenesis not known • Antiphospholipid antibodies may play a role • Accelerated atherosclerosis may contribute to the risk of stroke in patients with SLE Schur PH, Khoshbin S. Neurologic manifestations of systemic lupus erythematosus. www.Uptodate.com Joseph FG, Lammie AG, Scolding NG. CNS lupus: A study of 41 patients. Neurology. 2007

  46. (+) Antineuronal antibodies found in one report in 45 percent of patients with CNS lupus Cognitive dysfunction associated with lymphocytotoxic antibodies Antiphospholipid antibodies  increase the risk of stroke syndromes, recurrent seizures and abnormal findings on MRI 2. Auto-antibodies Schur PH, Khoshbin S. Neurologic manifestations of systemic lupus erythematosus. www.Uptodate.com

  47. Antiribosomal P protein antibodies  associated with lupus psychosis and depression but not with cognitive dysfunction or psychologic distress • High levels of autoantibodies to a 50 kDa antigen located in the plasma membrane of brain synaptic terminals in 19 of 20 patients with SLE who had CNS involvement • Antiribosomal-P autoantibodies from psychiatric lupus target a novel neuronal surface protein causing calcium influx and apoptosis Soledad Matus,1,2,4 Patricia V. Burgos,1,2,4 Marcela Bravo-Zehnder,1,2,4Regine Kraft,6 Omar H. Porras,5

  48. Cytokines Neuropeptides Oxidative stress Nitric oxide Interference with neurotransmission Genetic heterogeneity 3. Others Schur PH, Khoshbin S. Neurologic manifestations of systemic lupus erythematosus. www.Uptodate.com

  49. Primary • Vascular occlusion/hemorrhage • Auto-antibody-mediated • Choroid Plexus dysfunction • Cytokine effects • Other mechanisms Pathogenic Mechanisms causing Neuropsychiatric Symptoms in SLE • Secondary • Infection • Medications • TTP • Hypertension • Uremia • Electrolyte imbalances • Fever • Thyroid disease • Atherosclerotic strokes • Subdural hematoma • Cerebral lymphoma • Reactive depression Schur PH, Khoshbin S. Neurologic manifestations of systemic lupus erythematosus. www.Uptodate.com

  50. Confirm diagnosis of lupus according to ARA criteria Careful history and PE Diagnostics Diagnostic approach to manifestations of neuropsychiatric lupus • Monitoring: • If patient improves: monitor history and PE • If patient gets worse: PET scan/MRI, LP Schur PH, Khoshbin S. Diagnostic approach to the neuropsychiatric manifestations of systemic lupus erythematosus. www.Uptodate.com

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