Neurological Diseases - Degenerative disorders - Multiple Sclerosis Flashcards

1
Q

what is multiple sclerosis?

A

white matter disease
Demyelination-”inflammation”
focal disturbance of function-relapse
relapsing remitting course

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2
Q

does multiple sclerosis affect the PNS or CNS?

A

central nervous system

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3
Q

what do most patients with multiple sclerosis end up developing?

A

progressive disability

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4
Q

what is the prevalence of MS in scotland?

A

Prevalence ~190 per 100 000 in Scotland
Approx 5 new cases per week in Scotland
50-60 new cases per year in Aberdeen

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5
Q

what is the pathogenesis of MS?

A

Complex genetic inheritance
Association with autoimmune disease
Female:male 2-3:1

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6
Q

how does MS present initially?

A

relapse
Demyelination
central nervous system
White matter

Inflammation
Gradual onset over days
Stabilises days to weeks
Gradual resolution
Partial or complete recovery

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7
Q

what are a patients symptoms following relapse of MS?

A

optic neuritis

Sensory symptoms

Limb weakness

Brainstem
Diplopia
Vertigo/Ataxia

Spinal cord
bilateral motor and sensory symptoms
Bladder involvement

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8
Q

what is optic/retrobulbar neuritis?

A

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

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9
Q

what results in a patient following brainstem relapse of MS?

A

Cranial nerve involvement
Pons- internuclear ophthalmoplegia

Cerebellum -vertigo, nystagmus, ataxia

Upper motor neurone signs limbs
Sensory involvement

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10
Q

what are the implications of a spinal cord lesion?

A

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

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11
Q

what is the process of demylination?

A

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

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12
Q

how would demylination be described in MS?

A

episodes of demyelination disseminated in space and time

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13
Q

what results from further relapses of MS?

A

Variable site and severity
Sensory symptoms
Limb weakness
Diplopia
Vertigo
Ataxia
Sphincter disturbance etc etc

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14
Q

when will a further relapse occur following first relapse?

A

May occur within months or years of first relapse

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15
Q

what are indictors that MS disease is progressing in a patient?

A

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

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16
Q

what symptoms and signs accumulate during the progressive phase of MS?

A

Fatigue, temperature sensitivity
Dysesthesia/ paraesthesia
Stiffness or spasms
Balance, slurred speech
Bladder & bowel symptoms
Diplopia/ oscillopsia/ visual loss
Swallowing problems
Cognitive-memory/ emotional lability

17
Q

what are examination findings for mS?

A

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

18
Q

what are different types of MS?

A

Relapsing remitting-85% at outset (RRMS)
Secondary progressive (SPMS)
Primary progressive – 10-15% (PPMS)

19
Q

what is primary progressive MS?

A

Often presents in 5th and 6th decade
No relapses
Spinal symptoms
Bladder symptoms
Prognosis poor
M:F = 1:1

20
Q

how is MS diagnosed?

A

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)

21
Q

what are the two methods of MRI based diagnosis for MS?

A

Posers criteria (clinical) - less used

Macdonald Criteria (MRI)

22
Q

what will be present in a patient with MS MRI scan?

A

Areas of demyelination

23
Q

what other diagnostic procedures can be done for MS dependent on clinical picture?

A

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

24
Q

what are some differential diagnosises for MS?

A

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

25
Q

what are differential diagnosis for optic neuritis?

A

Neuromyelitis optica
Sarcoidosis
Ischaemic optic neuropathy
Toxic/ drugs/ B12 deficiency
Wegeners granulomatosis
Local compression
Lebers hereditary optic neuropathy
Infection-TB, HIV

26
Q

what are differential diagnosis for myelitis?

A

Inflammation
Neuromyelitis optica
SLE
sarcoidosis
Infection…or post infection
(HIV, HTLV-1, HSV, TB, borrelia, mycoplasma etc)
Tumour
Paraneoplastic process
Stroke

27
Q

what are the different treatment options for MS?

A

Treatment of relapse

Disease modifying treatment

General health and diet
Symptomatic treatment
Multi-disciplinary approach

28
Q

what is the treatment for an acute relapse of MS?

A

LOOK FOR UNDERLYING INFECTION
Exclude worsening of usual symptoms with intercurrent illness

oral prednisolone (Intravenous)
Rehabilitation
Symptomatic treatment - muscle relaxents

29
Q

what are some symptomatic based treatments for MS?

A

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

30
Q

what multidisciplinary members make up the team for an MS patient?

A

MS nurse
Physiotherapy
Occupational therapy
Speech and language therapy
Dietician
Rehabilitation specialists
Continence advisor
Psychology/psychiatry

31
Q

what are first line disease modifying treatments?

A

s/c or i/m injections
Beta-interferons
glatiramer acetate

Oral treatments
Teriflunomide
Dimethyl Fumarate

32
Q

what are second line disease modifying treatments?

A

Natalizumab
Fingolimod
Cladribine
Ocrelizumab (?PPMS)

Alemtuzumub

33
Q

what are immunosupressive risks of disease modifying treatment?

A

Progressive multifocal leukoencephalopathy (PML) - reactivation of common virus - deadly braincondition can be fatal