Multiple Sclerosis Flashcards
Summarise a MS case in an introduction. What are the key points? (5)
Type
Duration
Treatment
Major neurological deficits + complications
Major functional + psychosocial consequences
“Mr CD is a 60 year old man with a 15-year history of multiple sclerosis, that has transitioned from RRMS to SPMS, currently being treated with beta-interferon, on a background of comorbid COPD and depression. Mr CD’s MS- related disability has been gradually progressing and he is now wheelchair-bound due to weakness and ataxia. Major issues identified are Mr CD’s dissatisfaction and poor adherence with his injection-based treatment, severe bladder dysfunction, poorly managed depression and low social support.
Symptoms of MS you need to ask for in P? (8) What is the key information you need to extract from the history?
Symptoms (start top-down)
Seizure
Cognitive impairment
Visual (acuity, pain on movement, loss of central vision – optic neuritis)
Cerebellar – ataxia, tremor, dysarthria
Spastic paresis, quadriparesis, hemiparesis
Limb paraesthesiae (posterior column, ML, internal capsule)
Urinary urgency / incontionence / retention
Faecal incontinence
Need to extract the type based on Hx.
- Is there a progression without flare-ups? (i.e. PPMS)
- Flare-ups followed by worsening symptoms (i.e. SPMS)
Risk factors/triggers for MS (attacks?) - for R in pricmcp (6).
Other than the FH - Think of getting someone pregnant
Bend-over (Lhermit’s - flexing the neck)
Hot/Heat (Utoff’s)/Being born in a temperate place
Exercise (sex)
Impregnate (infection + Pregnancy)
RF for disease: Family History, Place of Birth (x10 risk if born in a temperate climate)
RF for attacks:
Uhthoff’s phenomenon - heat worsens Sx
Lhermitte’s sign (flexing the neck)
Infection, fever, pregnancy & exercise
Types of MS (4) - in order of frequency.
Relapse-Remitting (90%)
Secondary progressive (50-75% within 15y)
Primary Progressive (10%)
Progressive relapsing (<5%)
What are common symptoms of MS and what is your approach to managing them?
Head-to-toe
Seizure - antiepileptics, rule out infection/tumour, assess fitness for driving
Cerebellar ataxia / tremor - falls prevention strategies, PT/OT, walking aids, clonazepam, propranolol
Heat-sensitive symptoms - CCBs
Cognitive impairment - memory aids, cholinesterase inhibitor
Painful spasms - baclofen, diazepam, dantrolene
Bladder dysfunction - incontinence pads, bladder training/habit, oxybutynin (overactive bladder), IDC or alpha-blocker (overflow)
Bowel - incontinence pads, monitor constipation
Sexual - lubricants, sildenafil, consider referral to IVF
MS - PRICMCP?
P: date dx, initial symptoms (head-to-toe) - seizure, stroke, cerebellar ataxia, optic neuritis, visual problems, stroke-like symptoms, paresis, parasthesiae, bladder/faecal. What is the type of MS? (i.e. RR, PS, SP…etc)
R: FH, place of birth (temperate), Heat (Uhtoff), flexing the neck (Lermit’s), Infection, Exercise, Pregnancy
I: how was the dx established? MRI, LP, clinical (met McDonald’s criteria - at least 2 lesions clinically/radiologically)?
C:
- Disease (above - especially disability resulting from the disease: incontinence, autonomic dysreflexia) + Falls, Aspiration, Contractures, Pressure sores.
- Drug side effects (especially PML, infection / meningitis, arrhythmia, Hepatitis, depression [IFN]). Injection site reactions, infusion reactions***.
M:
- Current & previous drug regime
- Symptom Mx: CCB (heat-sensitive symptoms), tremor (propranolol, clonazepam), spasticity (baclofen, diazepam), pain (anti-depressants, anticonvulsants), bladder dysfunction (oxybutynin if urge incontinence), constipation (laxatives), fatigues (amantidine), sexual (sildenafil), IDC/SPC…etc.
- Is the patient compliant?
C: current symptoms (mobility, sexual dysfunction, ADLs, work). Did symptoms resolve or still there? Plan for further therapy? Any active problems.
P: understanding of prognosis (15y from diagnosis - the majority will have disability that stops them from working / help with normal activities), understanding of complications
MS - clinical examination. Routine neuro + what 4?
A full CN, UL, LL exam and;
Look specifically for…
- INO + decreased VA + RAPD. ***MUST DO FUNDOSCOPY (optic atrophy)
- Cerebellar signs (ataxia, tremor, nystagumus)
- Transverse myelitis (LMN at the level of lesion + UMN below)
- Mention bradycardia (Fingolimod)
What is your approach to investigating this patient with MS? What investigations would you like to see?
T: confirm Dx.
- Based on symptoms (e.g. patient had 2 clinical attacks) the patient fulfills time criteria (+/- space).
- However, I would like to review MRI looking for ≥2 lesions affecting ≥2 regions of the CNS (space) + simultaneous enhancing + non-enhancing lesion (time)
- LP (oligoclonal band of IgG1, elevated protein, mononuclear cell pleocytosis)
- VEP (slowed EP suggestive of myelin loss) - indicates previous optic neuritis - important if there is only one clinically detectable lesion present to fulfil the criteria
Exclude other dx
- Rule out infection (septic workup, CSF MCS), stroke, malignancy (send cytology & flow-cytometry)
Severity
- Review MRI trends & disease burden: number of lesions, areas affected, the rapidness of progression
- Look for Gadolinium enhancement suggestive of active disease
Treatment baseline bloods + Screen for Complications
- Bladder scan, consider urodynamics
- FBC (cytopaenia), EUC (GBM-GN), LFTs (hepatitis), TSH (alemtuzumab), CRP (infection), Vitamin D (replace if low)
- Urine MCS (alemtuzumab - GBM, UTI), ECG (fingolimod-bradycardia)
What are 3 common neurological findings of MS of Transverse myelitis?
Sensory, motor, autonomic impairment below the level of the lesion
C5-T1 lesion: UMN + LMN signs (upper limb), UMN signs (lower limb)
T1-T12 lesion: UMN (lower limb) – most common
L1-S5: UMN + LMN (lower limb)
Ask autonomic dysreflexia
What is autonomic dysreflexia? why do you get it?
- Cord involvement above T6
- Uninhibited (exaggerated) sympathetic response to noxious stimuli below the level of injury → vasoconstriction + HTN
- Due to loss of compensatory dilatation of splanchnic vascular bed
What is “objective clinical evidence” used in MacDonald’s criteria?
Abnormality on
- Neurological examination or
- MRI or
- Visual evoked potentials
That corresponds to the anatomical location suggested by symptoms of a current or previous attack
What qualifies as MS according to 2017 MacDonald’s criteria?
Key summary;
- 2** x dissemination in **time and space criteria
- If only 1 time or space criteria are met based on hx/exam, you can fulfill the rest with MRI (i.e. lesions ≥2 anatomical areas or old+new lesions demonstrating dissemination in time)
In more detail;
- History of ≥2 clinical MS attacks (i.e. time) and
- Objective clinical evidence of ≥2 lesions (i.e. space) or
- Clinical evidence of 1 + reasonable historical evidence of prior attack involving lesion in distinct anatomical location
- History of ≥2 clinical MS attacks (i.e. time) and clinical evidence of only 1 (but without evidence of prior attacking lesion in distinct location)
* Need dissemination in space – MRI (hyperintenst T2 – in ≥2 of 4 regions of CNS), or another attack in different site - History of 1 clinical MS attack (i.e. time) and clinical evidence of ≥2 lesions (i.e. space; clinically isolated syndrome – CIS)
* Need dissemination in time: MRI – simultaneous gad-enhancing/nonenhancing lesions, new hyperintense T2/Gad enhancing lesions on FU, new clinical evidence or presence of CSF – oligoclonal bands.
What are the 4 anatomical locations in the CNS for Space criteria for MS?
Juxta-cortical
Periventricular
Infratentorial (Cerebellar)
Spinal cord
What do you mean by dissemination in space? (2)
What do you mean by dissemination in time? (2)
What are the differentials in MS long case?
Vascular (ischaemic stroke, SVT)
Infection (syphilis, lyme, HTLV-1)
Toxins (drugs – PML – progressive multifocal leuko-encephalopathy)****
Autoimmune / inflammatory – vasculitis (SLE, Sjogrens, Behcets)
Metabolilc: B12 deficiency (SDSC)
Infiltrative – sarcoids
Neoplastic - CNS lymphoma, paraneoplastic
If spinal cord disease – rule out SC compression and Subacute combined degeneration of the cord
DDx for optic neuritis? (6)
MS-associated:
- MS – typically unilateral
- MOGEM (MOG-associated encephalo-myelitis) – bilateral
-
NMOSD (neuromyelitis optica spectrum disorder) – bilateral (
Transverse myelitis with longitudinally extensive SC lesion spanning ≥3 vertebral segments + bilateral optic neuritis)
General:
- Vascular (stroke, thrombosis)
- Infection/inflammatory/infiltrative
- Trauma/Toxins
- Autoimmnue - SLE
- Malignancy/Metabolic - paraneoplastic
What are the differential ddx of a large acute lesion on MRI in patients with MS on tx? (4)
-
Tumefactive demyelination – acute, tumor like MS variant with ≥2cm lesions, often with oedema + ring enhancement
- Rapidly responsive to steroid treatment
- PML
- CNS malignancy
- Otherwise as per VITAMIN
How does typical MS lesion appear on MRI (3)?
•Ovoid appearance
•T2 hyper-intense lesion
•Dawson finger appearance: characteristic peri-ventricular lesions arranged at right angles to corpus callosum
