Autoimmune and demyelinating diseases Flashcards
What is the etiology and pathogenesis of multiple sclerosis?
Etiology: genetic (MZ concordance, a/w HLA genes, CLEC16, IL receptors, CD226), environmental (sunlight exposure, vit D, EBV exposure)
Pathogenesis likely immune mediated
* Activated T lymphocytes cross BBB and recognize myelin derived antigens
* Cytokine release –> acute inflammation –> acute clinical deficit (steroid amenable)
* MQ destruction of oligodendrocyte myelin unit –> demyelination plaques –> decreased conduction –> chronic deficit
* Repeated attacks –> axonal loss –> progressive deficit
What are the clinical subtypes of multiple sclerosis?
- Clinically isolated syndrome, CIS: 1st attack of a disease compatibloe with MS
- Relapsing and remitting MS (RRMS, 85-90%): episodes of acute worsening with recovery and a stable course between relapses
- Secondary progressive (SPMS, ~50% at 15y): gradual neurological deterioration
- Primary progressive (PPMS, 10-15%): variable progressive course from the beginning
- Progressive relapsing (PRMS, ~5%): relapses superimposing on a progressive course
What is the course of MS?
What are common presenting symptoms?
What are classical SS?
What are suggestive features of MS?
What are atypical features of MS?
What are the clinical features of multiple sclerosis (sensory, motor, ocular, cerebellar, autonomic, higher function, paroxsymal attacks)?
What are the Ix done for multiple sclerosis?
What are the typical findings?
How to make a dx of multiple sclerosis?
What is the ddx for multiple sclerosis?
What is the Tx for acute attacks of MS?
What is prophylactic Tx?
What is symptomatic Tx for MS?
□ Limb spasticity: baclofen, BZD, dantrolene,
□ Neuropathic pain and TN: carbamazepine, oxcarbazepine, gabapentin, pregabalin, antidepressant
□ Fatigue: amantadine, methylphenidate
What are the poor prognostic factors of multiple sclerosis?
□ Demographics: male gender
□ Symptomatology: motor and cerebellar signs at presentation, multiple MRI cranial lesions
□ Course: primary progressive, frequent relapses in first 2y, short interval between first two relapses, poor recovery from relapses
What is the characteristics of neuromyelitis optica (NMO, Devics disease)?
Associated with which genes?
Clinical features?
What are the Ix done for neuromyelitis optica?
What is the diagnostic criteria?
What is Mx and prognosis for neuromyelitis optica?
What is the etiology of acute disseminated encephalomyelitis?
What are the clinical features?
What are the Ix, Mx and prognosis for acute disseminated encephalomyelitis?
What are the causes of transverse myelitis?
What are the types of TM?
What are the clinical features of trnasverse myelitis?
What are the important Ix to be done?
What is the ddx of transverse myelitis?
What is the Mx?
Prognosis?
