MS and demyelinating diseases Flashcards
What is the typical age and gender when MS begins?
ages 20 - 40 , female:male 2:1
The excact trigger of MS is uknown, but what are some possible triggers?(4)
viruses /other infectious - EBV, HHV-6, HTLV-1
environmental toxins
lack of nutrients - inverse vit D/sunlight correlation
genetic - IL2/7 R
Where is the prevalence of MS the highest?
furtherst away from the equator (both hemispheres)
What antibody is found in 47% of people with MS?
antibodies to KIR4.1 (potassium channel ab)
What pathology can be seen in CNS white matter in patients with MS?
Demyelinating plaques
- predilection for periventricular regions, brainsterm, optic nerves, spinal cord
Is MS a purely inflammatory demyelinating disease?
No, axonal loss occurs even at early stages
What can be clinical signs of MS? (13)
OPTIC NEURITIS (50% early, 75% late) Limb weakness Vision disturbances Sensory loss loss of balance/coordination Sphincter disturbances Cognitive decline spasticity (70%) tremor fatigue depression sexual dysfunction pain
What four clinical types of MS exists?
Remitting-relapsing
Sexondary progressive
Primary progressive
Progressive-relapsing
What can be triggers for MS relaps?(4)
viral infections
bacterial infections
3 months - post partum
stress?
clinical isolated syndrome is usually the first clinical event in MS. What are the subtypes?(9)
optic neuritis brainstem syndrome spinal cord syndrome transverse myelitis multifocal lesions cerebral lesions lhermitte's sign sensory sympt bladder dysfynction
What is needed to diagnose MS?
Damage to the CNS which is:
- Disseminated in time (2 relapses)
and - Disseminated in space (2 locations)
evidence in time/space can also be on MR (T2)
(lesions in brainstem/spinal cord doesn’t count)
What can be seen in CSF in MS?
oligoclonal bands
What other diseases can look like MS clinically and on MRI?(11)
Acute disseminated encephalomyelitis Progressive multifocal leukoencephalopathy Transvere myelitis Neuromyelitis optica (Devic syndrome) small vessel disease brain vasculitis metastasis (melanoma) Neuroborreliosis Neurosarcoidosis Bechet disease central pontine myelinolysis
What can indicate worse prognosis of MS?(2)
- shorter first attack interval
- incomplete recovery from first attack
How is a MS relapse treated?
corticosteriods
- methylprednisone 1g I.V for 3-5 days
OBS avoid in first trimester and breastfeeding
What disease-modyfing drugs are used in MS?(4)
- Interferon Beta1 a/b
- Glatimer acetate
- Natalizumab (alpha4integrin ab) - relapsing/remitting
- Mitoxantrone - secondary progressive
What is the mechanism of Mitoxantrone?(2)
Topoisomerase inhibitor
intercalating agent
What are used to manage spasticity in MS?(6)
Baclofen (GABAb agonist) Tizanidine (alpha 2 agonist) Tetrazepam Diazepam Botulinum toxin physiotherapy
What are used to manage chronic fatigue in MS?(2)
Amantadine(NMDA antag, dopa reuptake, dopa release)
Modafinil (dopamine reuptake inhibitor)
What are used to manage depression in MS?(2)
TCAs (amitryptiline)
SSRIs (Fluoxetine)
What are used to manage pain in MS? (7)
Acute - Carbamazepine - Benzodiazepines - Baclofen Chronic - Amitryptiline - Carbamazepine - Baclofen - Physiotherapy
What are used to manage “autonomic bladder” in MS?(2)
oxybutinine (anticholinergic)
tolterodine (antimuscarinic)
What are used to manage atonic bladder in MS?(2)
self-cathetherization
distigmine (blocks ACHE)
What are used to manage atonic bladder and hyperactive sphincters in MS?(3)
Terazosine (alpha 1 block)
Doxazosine
Tamsulozine
What are used to manage tremor in MS?(6)
Carbamazepine Clonazepam Primidone (barbituate) Propanolol Surgery (Thalamotomy, deep brain stimulation)
What are used to manage chronic vertigo in MS?
betahistine (Histamine 3 antag, H1 agonist)
What are used to manage sexual dysfunction in MS?
Sildenafil
What are used to manage seizures and other paroxysmal symptoms in MS?
anticonvulsants
What agents might be a trigger of Acute disseminated encephalomyelitis(ADEM)
viral infection
vaccination
What is ADEM?
Acute/subacute disorder
multifocal symptomatology
immunological
response to immunosuppression
What are the clinical features of ADEM?(7)
prodromal symptoms altered consciousness/confusion ataxia bilateral optic nerve damage aphasia spinal cord-related signs dysarthria, dysphagia
What examinations (2) should be perfomed in ADEM, and what will they show?
MRI:
multiple hyperintense T2 leisons
edeme
affected gray matter(thalamus, basal ganglia)
CSF:
lymphocytes increased
protein increased
oligoclonal bands
What is the treatment for ADEM?(4)
Methylprednisone
Plasmapheresis
IVIG
Mitoxantrone
50-70% recover
What is transvere myelitis?
ACUTE focal inflammatory disorder of spinal cord
motor,sensory, autonomic disturbances
prognosis 1/3 rule
What are the clinical features of transverse myelitis?
UMN syndrome, sensory level, autonomic disturbance
What are the causes of transverse myelitis?(4)
idiopathic 2/3
infection related
collagenosis related
secondary to MS, ADEM, NMO
What needs to be excluded in the diagnosis of transverse myelitis?
exclude cord compression - MRI
Exclude primary MS, ADEN, NMO
also confirm inflammatory CSF markers
What is the treatment of transverse myelitis?(4)
IV methylprednisone - then oral predn.
Plasmapheresis
cyclophosphamide
sympt treatment + physiotherapy
What is neuromyelitis optica?(NMO)
Chronic inflammatory demyelinating disorder
with severe recurrent myelitis and optic neuritis
What gender and race are more commonly affected by NMO?
Females (9x), Asians
Why does NMO sometimes cause dysphagia and dysarthria?
cervical lesion extends to brainstem
What antibody are found in 85% of patients with NMO?
NMO-igG autoantibodies against aquaporin4
What examinations should be performed in NMO?(3)
And what do they show?
MR: longitudinal T2 hyperintense lesions
CSF: elevated neutrophils, sometimes oligoclonal bands
Serum: NMO-IgG antibodies
What is the treatment of NMO?
Relapses: IV methylprednison / plasmapheresis
Prevention: Azathioprine+prednisone, rituximab,IVIG
OBS: interferons/glatimers may be even harmful!!
What are the major causes of neurological disabillity in young people?
Demyelinating diseases!
