Worsening weakness & numbness in a young female

Question 1

What more Hx should you ask in a young female px with worsening weakness & numbness?

Answer 1

• No back pain
• No trauma
• Symptoms not worse with cough/sneeze, Valsalva (compressive aetiology)

No systemic auto-immune disease e.g.

• No rashes, arthralgia (SLE)
• No miscarriages (anti-phospholipid syndrome)
• No dry mouth, dry eyes (Sjogren’s syndrome)
• No recent infections (Para-infectious causes, GBS)
• No recent vaccinations (GBS)
• Unrestricted diet (B12 deficiency)
• No B symptoms/Paraneoplastic syndromes (malignancies)

Question 2

Hemisensory loss + contralateral face loss indicates the lesion is at…

Answer 2

brainstem

Question 3

Where is the lesion for unilateral arm and leg weakness?

Answer 3

Brainstem or hemisphere.

Look for any cortical signs for hemisphere.

Question 4

What is the pattern of lesion in patchy weakness with multiple named nerves involved?

Answer 4

Mononeuritis multiplex

Question 5

What is the pattern of lesion in variable weakness that fatigues?

Answer 5

Myasthenia gravis

If no fatigue: functional weakness

Question 6

Compare the signs of spinal cord vs. peripheral neuropathy localisation

Answer 6

Spinal cord lesion:

• sensory level
• hyperreflexia
• bowel & bladder problems

Peripheral neuropathy:

• glove & stocking/dermatomal / single nerve distribution
• loss of reflexes
• no bowel & bladder problems

Question 7

What are the causes of SUBACUTE paraparesis?

Answer 7

1. spinal cord:
   - multiple sclerosis
   - tumours
2. Peripheral nerve lesions:
   - Guillain Barre syndrome (acute inflammatory demyelinating polyradiculopathy). but clasically no sensory loss. Weakness pattern usually distal in UL & proximal in LL

Question 8

What is Uhthoff’s phenomenon?

Answer 8

Worsening of symptoms with heat.

Reversible and stereotypic decrements in physical and cognitive symptoms due to increased ambient body temperature (including exercise, fever, summer, hot showers)

Relatively specific for Multiple sclerosis

Question 9

Describe transverse myelitis

• definition
• causes

Answer 9

• Inflammatory demyelination of segment/s of spinal cord
• Functionally transects the cord

Causes

• Multiple Sclerosis (common)
• Infectious/parainfectious inflammation
• Other autoimmune disorders: vasculitis, systemic autoimmune diseases
• No specific aetiology (40%)

Question 10

What is clinically isolated syndrome of multiple sclerosis?

What are the common 1st Px of MS?

Answer 10

1st clinical presentation compatible with the diagnosis of MS but not fulfilling the MS diagnostic criteria

Common first presentations of MS:

• transverse myelitis
• optic neuritis
• brainstem/cerebellar presentations (ataxia, nystagmus, vertigo, diplopia)

Question 11

How do you diagnose MS?

Answer 11

Affected:
-Dissemination in space in the CNS. presence of documented neurological signs in at least two parts of the CNS, e.g., transverse myelitis AND optic neuritis

• Dissemination in time. attacks separated in time. E.g. 2 or more relapses at least 1 month apart or 2 or more of different age.

Question 12

DDx of MS

Answer 12

• neuromyelitis optica (NMO, Devic’s disease)
• other systemic autoimmune diseases
• acute disseminated encephalomyelitis (ADEM)
• sarcoidosis
• infections
• tumours
• conversion disorder, somatisation

Question 13

How can you predict the risk of a second attack of MS after the 1st one?

Answer 13

by number of inflammatory lesions on baseline MRI Brain

The presence of oligoclonal bands in the CSF confers additional risk

Question 14

What are the subtypes of MS?

Answer 14

1. Relapsing-remitting MS (RRMS)
   - most common 90%
   - relapses w/ incomplete/complete recovery & stable phase b/w relapses
2. Secondary progressive MS (SPMS)
   - 50-75% RR becomes SP within ~15 yrs
   - gradual neurological deterioration
   - w/ or w/o superimposed relapses
3. Primary progressive MS (PPMS)
   - 10% overall
   - gradual but continuous neurological deterioation
   - if relapses occur: progressive + relapsing MS

Question 15

Discuss the MS prognosis

• better prognosis with:
• in general the 1/3 rules

Answer 15

Better prognosis if:

- female, young (onset before 35)
- initial sensory symptoms and optic neuritis (as opposed to motor or cerebellar dysfunction)
- initial mono-symptomatic presentation
- sudden onset, good recovery, lower attack rates, longer attack intervals

In general:
1/3 – do well without accumulating significant disability
1/3 – accumulate neurological deficits to impair activities but not serious enough to prevent them from leading a normal life
1/3 – become disabled – requiring gait/mobility aids and even high level care

Question 16

What is the px of acute optic neuritis?

Answer 16

• pain behind eye, worse w/ eye movement
• reduced visual acuity
• colour desaturation (esp red-green)
• papilloedema on fundoscopy

Question 17

Describe a MS relapse

Answer 17

an episode of neurological disturbance consistent with MS

≥48 hours; duration: usually 2-4 weeks

not preceded by viral-like illness

≥30 days from the onset of the previous attack

usually good recovery, but residual disability may persist

Question 18

How do you Mx MS relapse? What about long term Mx of MS?

Answer 18

Acute relapse:
3-5 days of IV methylprednisolone, 1g/day
- Reduces severity of attack
- Shortens recovery time
- Effect on ultimate recovery uncertain

Long term:
Disease modifying therapy
- immunomodulatory and/or immunosuppressive effects

Question 19

Discuss the disease modifying therapies available for MS

Answer 19

immunomodulatory and/or immunosuppressive effects

1st tier:

- Interferons β (SC or IM injections 1-4/week)
- Glatiramer acetate (daily SC injections)
- Teriflunomide (daily tablet)

2nd tier:

- Fingolimod (daily tablet)
- Dimethyl fumarate (BD tablet)

3rd tier:

- Natalizumab (monthly infusion)
- Alemtuzumab (2 infusion courses over 5 years)