Neurological Diseases - Degenerative disorders - Multiple Sclerosis Flashcards
what is multiple sclerosis?
white matter disease
Demyelination-”inflammation”
focal disturbance of function-relapse
relapsing remitting course
does multiple sclerosis affect the PNS or CNS?
central nervous system
what do most patients with multiple sclerosis end up developing?
progressive disability
what is the prevalence of MS in scotland?
Prevalence ~190 per 100 000 in Scotland
Approx 5 new cases per week in Scotland
50-60 new cases per year in Aberdeen
what is the pathogenesis of MS?
Complex genetic inheritance
Association with autoimmune disease
Female:male 2-3:1
how does MS present initially?
relapse
Demyelination
central nervous system
White matter
Inflammation
Gradual onset over days
Stabilises days to weeks
Gradual resolution
Partial or complete recovery
what are a patients symptoms following relapse of MS?
optic neuritis
Sensory symptoms
Limb weakness
Brainstem
Diplopia
Vertigo/Ataxia
Spinal cord
bilateral motor and sensory symptoms
Bladder involvement
what is optic/retrobulbar neuritis?
Subacute visual loss
Pain on moving eye
Colour vision impaired
Usually resolves over weeks/ months
Initial swelling optic disc
Optic atrophy seen later
Relative afferent pupillary defect
what results in a patient following brainstem relapse of MS?
Cranial nerve involvement
Pons- internuclear ophthalmoplegia
Cerebellum -vertigo, nystagmus, ataxia
Upper motor neurone signs limbs
Sensory involvement
what are the implications of a spinal cord lesion?
Partial or Transverse (complete) Myelitis
Sensory level often with band of hyperaesthesia
Weakness/ upper motor neurone changes below level of demyelination
Bladder and bowel involvement
what is the process of demylination?
Auto immune process
Activated T cells cross blood brain barrier causing demyelination (cause - attack myelin sheath - repair (glyosis))
Acute inflammation of myelin sheath
Loss of function
Repair
Recovery of function
Post inflammatory gliosis
may have functional deficit
Lesions or plaques on MRI scan
how would demylination be described in MS?
episodes of demyelination disseminated in space and time
what results from further relapses of MS?
Variable site and severity
Sensory symptoms
Limb weakness
Diplopia
Vertigo
Ataxia
Sphincter disturbance etc etc
when will a further relapse occur following first relapse?
May occur within months or years of first relapse
what are indictors that MS disease is progressing in a patient?
Axonal loss important in disease progression and development of persistent disability
Black holes on MRI
Later seen as cerebral atrophy
Probably present from disease onset
what symptoms and signs accumulate during the progressive phase of MS?
Fatigue, temperature sensitivity
Dysesthesia/ paraesthesia
Stiffness or spasms
Balance, slurred speech
Bladder & bowel symptoms
Diplopia/ oscillopsia/ visual loss
Swallowing problems
Cognitive-memory/ emotional lability
what are examination findings for mS?
Depends on where demyelination has occurred and stage of disease:
Afferent pupillary defect
Nystagmus or abnormal eye movements
Cerebellar signs
Sensory signs
Weakness
Spasticity
Hyperreflexia
Plantars extensor
what are different types of MS?
Relapsing remitting-85% at outset (RRMS)
Secondary progressive (SPMS)
Primary progressive – 10-15% (PPMS)
what is primary progressive MS?
Often presents in 5th and 6th decade
No relapses
Spinal symptoms
Bladder symptoms
Prognosis poor
M:F = 1:1
how is MS diagnosed?
Evidence of demyelination separated in time and space
May be clinical (2 relapses involving different areas of CNS, months or years apart)
or MRI based diagnosis
Posers criteria (clinical) - less used
Macdonald Criteria (MRI)
what are the two methods of MRI based diagnosis for MS?
Posers criteria (clinical) - less used
Macdonald Criteria (MRI)
what will be present in a patient with MS MRI scan?
Areas of demyelination
what other diagnostic procedures can be done for MS dependent on clinical picture?
Lumbar puncture
Normal CSF glucose and protein
Few if any white cell count
oligoclonal bands present in CSF but not serum
Visual/ somatosensory evoked response
Relevant blood tests e.g.serology, autoantibody screen
Chest X Ray
what are some differential diagnosises for MS?
Acute Disseminated Encephalomyelitis (ADEM)
Other causes demyelination (NMO etc)
Other Auto-immune conditions eg SLE
Sarcoidosis
Vasculitis
Infection eg Lyme disease, HTLV-1
Adrenoleucodystrophy etc etc
what are differential diagnosis for optic neuritis?
Neuromyelitis optica
Sarcoidosis
Ischaemic optic neuropathy
Toxic/ drugs/ B12 deficiency
Wegeners granulomatosis
Local compression
Lebers hereditary optic neuropathy
Infection-TB, HIV
what are differential diagnosis for myelitis?
Inflammation
Neuromyelitis optica
SLE
sarcoidosis
Infection…or post infection
(HIV, HTLV-1, HSV, TB, borrelia, mycoplasma etc)
Tumour
Paraneoplastic process
Stroke
what are the different treatment options for MS?
Treatment of relapse
Disease modifying treatment
General health and diet
Symptomatic treatment
Multi-disciplinary approach
what is the treatment for an acute relapse of MS?
LOOK FOR UNDERLYING INFECTION
Exclude worsening of usual symptoms with intercurrent illness
oral prednisolone (Intravenous)
Rehabilitation
Symptomatic treatment - muscle relaxents
what are some symptomatic based treatments for MS?
Spasiticity-muscle relaxants/ antispasmodics/ physiotherapy
Dysaesthesia-amitriptyline, gabapentin etc.
Urinary-anticholinergic Rx, bladder stimulator/ catheterisation
Constipation-laxatives
Sexual dysfunction-sildenafil
Fatigue-graded exercise, medication
Depression-CBT, medication
Cognitive-memory aids etc
Tremor-aids/ medication
Vision/ oscillopsia-carbamazepine
Speech/ swallowing-SALT
Motor/ sensory impairment- multi-disciplinary team
what multidisciplinary members make up the team for an MS patient?
MS nurse
Physiotherapy
Occupational therapy
Speech and language therapy
Dietician
Rehabilitation specialists
Continence advisor
Psychology/psychiatry
what are first line disease modifying treatments?
s/c or i/m injections
Beta-interferons
glatiramer acetate
Oral treatments
Teriflunomide
Dimethyl Fumarate
what are second line disease modifying treatments?
Natalizumab
Fingolimod
Cladribine
Ocrelizumab (?PPMS)
Alemtuzumub
what are immunosupressive risks of disease modifying treatment?
Progressive multifocal leukoencephalopathy (PML) - reactivation of common virus - deadly braincondition can be fatal
