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Multiple Sclerosis

Title               

Ñ    Multiple Sclerosis (MS)

Definition

Ñ    Chronic inflammatory disorder of the central nervous system

Characterised by:

Ñ    Multiple plaques of demyelination within the brain and spinal cord

Aetiology

Ñ    Unknown

Ñ    Prevalence increases in residents in areas further from the equator

Ñ    Immigrants acquire the prevalence of their destination, if immigration occurs before the age of 10 years

Ñ    Weak association between MS and antigens HLA-A3, B7, D2, DR2 in Caucasians in northern Europe and USA

Pathogenesis

Ñ    Inflammatory process against myelin, mediated by CD 4 T cells

Ñ    Causes plaques of demyelination between 2-10 mm in size

Ñ    Perivenular plaques with predilection for:

·          Optic nerves

·          Periventricular region

·          Brainstem

·          Cerebellar connections of the brainstem

·          Cervical spinal cord:

Posterior columns

Corticospinal tracts

Ñ    Acute phase causes inflammatory demyelination and conduction block

Ñ    Remission occurs when inflammation subsides and remyelination occurs

Ñ    Permanent sequelae if damage is severe and secondary permanent axonal destruction occurs

Clinical Features

Patterns of MS

Ñ    Relapsing and remitting

Ñ    Primary progressive

Ñ    Secondary progressive:

Follows relapsing remitting

Ñ    Fulminant

History

Age

Ñ    20 to 40 years

Sex

Ñ    Females > Males

CNS

Ñ    Blurred vision

Ñ    Unilateral visual loss

Ñ    Ocular pain

Ñ    Uthoff’s phenomenon:

Worsening of vision in:

·          Hot weather

·          After exercise

·          Fever

Ñ    Diplopia

Ñ    Vertigo

Ñ    Facial numbness

Ñ    Facial weakness

Ñ    Dysarthria

Ñ    Dysphagia

Ñ    Difficulty walking

Ñ    Numbness of limbs

Ñ    Lhermitte’s phenomenon:

Electric shock like sensation radiating down the trunk and limbs when the neck is flexed

Ñ    Urinary incontinence

Ñ    Urinary retention

Examination

CNS

Ñ    Normal disc:

Retrobulbar neuritis

Ñ    Optic neuritis

Ñ    Sixth nerve lesion

Ñ    Internuclear ophthalmoplegia

Ñ    Nystagmus

Ñ    Relative afferent pupillary defect

Ñ    Spastic paraparesis

Ñ    Spastic tetraparesis

Ñ    Cerebellar ataxia

Ñ    Sensory abnormalities:

Paraesthesiae,

Proprioceptive loss

Unusual features

Ñ    Epilepsy

Ñ    Trigeminal neuralgia

Ñ    Organic psychosis

Complications

Ñ    Uraemia

Ñ    Bronchopneumonia

Investigations

Fluids

Cerebrospinal Fluid

Haematology

Ñ    5-60 mononuclear cells/mm³

Biochemistry

Ñ    Oligoclonal IgG bands

Imaging

MRI

Ñ    Multiple plaques

Electrophysiology

EEG

Ñ    Delayed visual evoked responses

Management

Control

Drugs

Ñ    Methyl prednisolone:

Acute relapse

Ñ    Beta interferon:

Prevent relapse in relapsing remitting disease, which is defined as 2 attacks over 2-3 years followed by reasonable recovery

Ñ    Immunosuppression:

·          Azathioprine, cyclophosphamide:

No benefit in trials

Ñ    Glatiramer:

Reduce relapse frequency

Support

Lifestyle Adjustments

Ñ    Help with adjustments at work

Staffing

Ñ    Physiotherapy

Ñ    Occupational therapy

Equipment

Ñ    Walking aids

Ñ    Wheelchairs

Ñ    Car conversions

Residence

Ñ    Alterations to house and garden

Symptom Relief

Drugs

Ñ    Muscle relaxants:

Baclofen, benzodiazepines, dantrolene, tizanidine

Ñ    Botulinum toxin:

Severe spasticity

Staffing

Ñ    Physiotherapy:

Reduce pain and discomfort of spasticity

Prevention

Staffing

Ñ    Nursing:

Prevent pressure sores with good skin care

Prognosis

Ñ    Many live without significant disability

Some gravely disabled

 

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