When Your Head Hurts and Your Memory Fails- Is It Your Lupus? Petros Efthimiou, MD, FACR, Lincoln Medical and Mental Health Center Assistant Professor of Medicine, Weill Cornell Medical College, New York, NY
The body’s autoimmune system (“defense") attacks ITSELF Skin Kidney Lung Heart Joints Blood Nervous System Progressive Long Standing MULTISYSTEMIC
Epidemiology of SLE Incidence : 7.6/100,000/year (pooled from a number of studies) Prevalence: 14.5-50.8/100,000 Hochberg’s Prevalence : 372/100,000 US: close to 1 million people SLE Lupus Foundation: probably 1.5 million incidence 1950 2000 10 5 2
How Does Lupus happen? T Cell Abnormal Adhesion Molecule and Chemokine Expression Tissue Specific Pathology APC B7 MHC FcR CR RES AutoAb Drugs UV Infectious Agents Genetic Background Estrogens B Cell Increased Help Decreased Cytotoxicity Altered Cytokine Production IL-6, IL-10 IL-2 DC IFN AutoAg Apoptosis + Immune Complexes
American College of Rheumatology Criteria For Lupus Antinuclear Antibody 95% Immunologic Disorder 70% (aDNA, LE prep, aSm, lupus anticoagulant Hematologic Disorder 10% Neurologic Disorder 10% Renal Disorder 60% Malar Rash Serositis Arthritis Oral Ulcers Photosensitivity Discoid Rash
Neuropsychiatric (NPS) lupus Why does it happen? NO SINGLE PATHOGENIC MECHANISM Primary Manifestations of the Disease Secondary Complications of the Disease or Therapy Coincidental
Neuropsychiatric (NPS) lupus SEIZURES Generalized: whole body affected Partial: only one part or side of the body is affected Complex change in level of consciousness Simple (focal) no change in level of consciousness May present with: Twitching or shaking Temporary abnormal sensations Visual disturbances
Sudden numbness or weakness especially on one side of the body
with an associated tingling sensation
Sudden confusion or trouble speaking or understanding
Sudden trouble seeing
Sudden trouble walking, dizziness, loss of balance or coordination
Sudden severe headache
Neuropsychiatric (NPS) lupus HEADACHE An organic basis for the headaches in SLE is suggested by the sudden development in someone previously free of headaches, associated with Neurologic changes or changes in personality. Migraine and tension Headaches are the most common type of presentation. Migraine: Throbbing or pounding pain Nausea and vomiting Scalp tenderness Sensitivity to light or sound Worsening of pain with movement Visual disturbances Dizziness or vertigo Tension headache Pressing/tightening (nonpulsating) quality, located on both sides of the head Mild or moderate intensity Not aggravated by routine physical activity No nausea or vomiting Possible sensitivity to light or sound but not both
Temporary numbness, tingling, and pricking sensations (paresthesia)
Sensitivity to touch
Burning pain (especially at night)
Organ or gland dysfunction.
Difficulty digesting food, maintaining safe levels of blood pressure, sweat normally, or experience normal sexual function.
Neuropsychiatric (NPS) lupus Neurocognitive Dysfunction Manifested by impairments in mental activities Memory Judgment Abstract Thinking Simple/ Complex Attention Language Psychomotor speed
Neuropsychiatric (NPS) lupus Psychosis Characterized by: Presence of Delusions Presence of Hallucinations False belief despite evidence to the contrary Perceptual experience occurring in the absence of external stimuli.
Neuropsychiatric (NPS) lupus Diagnosis Predominantly fixed lesions in the periventricular and Subcortical White Matter Focal Neurologic Disease More Sensitive than CT Scan and T1- Weighted MRI for detecting Abnormalities in NP-SLE T2- Weighted MRI : Findings: Diffuse Neurologic Disease Transient Subcortical White Matter Lesions and patchy Hyperintensities in the Gray Matter