Inflammatory and Demyelinating Diseases Flashcards
Name the subtypes of Multiple Sclerosis
Relapsing-Remitting (RRMS), Primary Progressive (PPMS), Secondary Progressive (SPMS), Clinically Isolated Syndrome (CIS), Radiologically Isolated Syndrome (RIS),
What is the typical disease presentation of Relapsing-Remitting MS?
RRMS typically presents as sporadic episodes of new or worsening symptoms (over 2-10 days) with variable improvement over 1-6 months. 85% of cases present this way. Relapse = Attack = Exacerbation
What is the typical disease presentation of Primary Progressive MS?
PPMS typically is a gradually progressive disease from the outset, with no true relapses. 15% present this way.
What is the typical disease presentation of Secondary Progressive MS?
RRMS which converts to progressive disease. 50% of all RRMS progress to SPMS.
What is the typical disease presentation of Clinically Isolated Syndrome?
CIS is the designation for a patient that presents with a single relapse, but does not yet fulfill all criteria for MS. These patients have a high risk for having more attacks.
What is the typical disease presentation of Radiologically Isolated Syndrome?
RIS patients have had MRI scans for non-MS symptoms which showed apparent MS on the MRI. No other symptoms, but are at a high risk of developing MS over time.
Describe the basic epidemiology of MS
3/4 present between ages of 15-45; 2/3 are women; highest incidence in caucasians; higher incidence with increasing distance from equator; 10,000 new cases/year; 1:500 in CO and WY, HIGH prevalence; most common cause of CNS inflammatory disease
What are the early clinical symptoms of MS?
Multifocal defects: parasthesias, loss of vision, gait problems, weakness, diplopia, Ihermitte’s (tingling down spine), urinary urgency and frequency, constipation, vertigo
What are the later clinical symptoms of MS?
Multifocal and more general sx: same as early, plus fatigue, sexual and cognitive dysfunction, depression, pain syndromes, dysphagia, secondary problems (skin breakdown, infections, immobility)
Neurological exam findings in MS?
Asymetric, Corticalspinal: weakness, spasticity, exagerated reflexes; Sensory: loss/added sensation; Visual: acuity loss, eye movement abnormalities; Cerebellar: ataxia, tremor, dysarthria; Mood: depression, emotional lability; Cognitive impairment: short term memory, word finding, eye/hand coordination
Lab findings in MS?
MRI: for diagnosis and prognosis; T1: holes suggest axon damage; T2: hyper intense bright lesions; Atrophy: focal white and/or grey matter. CSF analysis: mild elevation of Protein, Modest elevation of WBC (
What are the treatments for acute MS attacks?
High dose steroids, plasma exchange if steroids are ineffective.
What are immunomodulatory treatments for MS?
Interferon beta 1a, Interferon beta 1b, glatiramer acetate, all reduce attacks, slow progression, and reduce changes in MRIs. Natalizumab is a mAb against T4 integrin, highly effective, side effect is increased risk of Progressive Multifocal Leukoencephalopathy.
What is the general path of immunopathogenesis of MS?
Activated T-cells express adhesion molecules, move through the BBB, interact with microglia and B-cells, pro-inflammatory interactions with Th1/Th17 cells occur and the T-cells damage the axons.