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A CNS white matter disease, demyelination at different times in different parts. |
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the hallmark of MS, an area of demyelination, relative axon sparing. acute plaque = oedema, inflammation chronic plaque = gliosis and scarring Plaques can be found in any part of the white matter of the spinal cord and brain. Particularly in the periventricular regions, optic nerves, corpus callosum, brain stem. |
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Demyelination disrupts conduction of nerve impulse and hence the neurological symptoms. Conduction along such segments is further impaired with higher temperatures e.g. fever, exercise, hot weather may worsen symptoms. |
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Unknown, likely multifactorial. Autoimmune process. Genetic: relative risk of 1st degree relative 2-4 x normal risk. Twin studies: mz (40%) dz (5%). Association with HLA antigens DR2 and DW2. Geographic: lower rates in tropical countries. If migrate from low prevalence area before age 15, take on the new risk. If migrate after age 15, remain at low risk. Viral: no evidence. Other factors often mentioned but no definite evidence: diet, sanitation, exercise, stress, trauma, socio-economic. |
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1/1000 in the UK, 80,000 in UK. F:M = 2:1 Peak age of onset 25-35, rare before 15 or after 60. |
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2 main patterns of disease. |
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1. Relapsing remitting form (80%) Clear relapses followed by recovery. Symptoms come and go in the form of "attacks", with abrupt worsening lasting >24hrs, often weeks or months of recovery, followed by a remission which may last weeks/months/years. May then become secondary progressive. 2. Primary Progressive Form (20%) Progressive from outset, tend to be older, gradual progressive decline without remission. |
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Clinical Features Sensory: Visual: Motor: Spinal Cord: Brainstem and cerebellum: Sphincter and sexual function: Cognitive: Lhermitte's phenomenon: |
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Clinical Features Sensory: commonest presentation (40%), numbness, tingling, cold, usually minimal signs Visual: optic neuritis common presentation (25-50% develop MS) Motor: weakness, legs more common than arms Spinal Cord: transverse myelitis: paraperesis (impairment in motor/sensory function of lower extremities), sensory level Brainstem and cerebellum: double vision, nystagmus, limb and gait ataxia, dysarthria, speech problems, dysphasia Sphincter and sexual function: urgency, frequency, incontinence, impotence, loss of sexual pleasure. Cognitive: depression, anxiety, euphoria. Fatigue. Epilepsy. Lhermitte's phenomenon:electric shock sensations down back when bending neck forward: cervical cord disease. |
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Precipitating factors (exacerbates MS) |
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trauma, infection, post partum (after had a baby, relapse) |
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Slight reduction of relapses during pregnancy, slight increases in relapses after, in general no overall change. |
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Occurs in 40%, so relatively common. Caused by lesions in brainstem, cerebellum or connecting pathways which lead to dysfunction of muscles of tongue, mouth, larynx, respiratory system. Results in spastic dysarthria, cerebellar scanning speech. Disorders of voice intensity, voice quality, articulation and intonation, rate, reduced tongue strength, endurance difficulties, rate of repetitive movements, even in many non-dysarthric patients. Episodic paroxysmal dysarthria, lasting a minute and recurring several times a day. |
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Surprisingly uncommon Usually mild Aspiration pneumonia in severly affected PEG feeding unusual |
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Made on the basis of a history of 2 or more attacks with abnormalities on examination which indicate dysfunction in different parts of the brain and/or spinal cord. Most patients with MS have an abnormal MRI. |
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Exclude other diagnoses, find changes consistent with MS. MRI brain/spinal cord: may find clinically silent lesions 90% CSF examination for oligoclonal bands:inflammatory proteins made in the CSF and not in the blood Visual evoked potentials:show delay in 90% even if no visual symptoms |
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Reduced life expectancy 5-10 years Up to 50% are in chronic progressive phase at 10 years. 50% require walking aids by 17 years 15% have a very benign course If disease mild after 5 years, tends to remain mild Sensory symptoms or optic neuritis at onset do better Men do worse Relapsing remitting disease better than primary progressive |
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Definition
No cure. Treatment of complications (spasticity, depression, bladder, erectile failure, pain, tremor) Symptomatic treatment for acute attacks (steroids) Disease modifying drugs - beta interferons, decrease number of attacks by 1/3? Less severe attacks, delays secondary progressive phase? No disease modifying drugs work for primary progressive phase at the moment |
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