Multiple Sclerosis

 

Key Points

 

1.       Involvement of the optic nerve implies optic neuritis.

2.       Blindness which can be at worst (and rarely) total in both eyes

3.       Course unpredictable.

4.       Symptoms - a number of distinct patterns relating to the course of the disease.

5.       Relapse-remitting MS. Unpredictable relapses (exacerbations, attacks) during which new symptoms appear or existing symptoms become more severe. This can last for varying periods (days/months) and there is partial or total remission (recovery). The disease may be inactive for months/years. Freq: - approx 25%.

6.       Benign MS. One or two attacks with complete recovery, does not worsen with time and no permanent disability. Only identified when minimal disability offer 10-15 years offer onset. Initially categorised as relapsing-remitting MS. Associated with less severe symptoms at onset (e.g. sensory). Freq: approx 20%.

7.       Secondary progressive. Initially relapsing-remitting MS. Development of progressive disability later. Often with superimposed relapses. Frequency - approx 40%.

8.       Primary progressive. Characterised by a lack of distinct attacks. Slow onset, steadily worsening symptoms. Accumulation of deficits. Disability may level off at some point or continue over months or years. No set "attack". Frequency - approx 15%.

9.       Early MS may present itself as o history of vague symptoms.

10.  Medical history may include past record of signs and symptoms.

11.  Type of symptoms, their onset and pattern may suggest MS.

12.  Medical tests will be needed to confirm the diagnosis.

13.  Many signs could be attributed to a number of medical conditions.

14.  Possible diagnosis may be easier with classic symptoms (e.g. optic neuritis).

15.  Distinct chronology of attacks helpful for diagnosis.

16.  Neurological deficits.

17.  Indicate involvement of at least two different areas of the CNS.

18.  Effects occurring at two separate times.

19.  MS is a clinical diagnosis.

20.  No specific test is 100% conclusive

21.  No typical MS.

22.  Experience more than one symptom, but no person would have them all.

23.  Some symptoms common to all sufferers.

24.  Some symptoms immediately obvious.

25.  Some symptoms not obvious - fatigue, altered sensation, memory, concentration.

26.  Several tests and procedures are needed to establish a diagnosis of MS.

27.  Test for abnormalities in nerve pathways but not conclude cause of abnormality.

28.  Changes in eye movements (nystagmus).

29.  Limb co-ordination.

30.  Weakness.

31.  Balance.

32.  Sensation.

33.  Speech.

34.  Reflexes.

35.  Other possible causes which produce similar symptoms must be eliminated (PD/MND).

36.  MRI for pictures of brain and spinal cord but evidence of scarring not conclusive.

37.  MRI is only test in which MS lesions can be seen.

38.  Many other conditions can produce identical abnormalities.

39.  Significant indicator to confirm a diagnosis of MS.

40.  Lumbar puncture - cerebrospinal fluid is tested for antibodies.

41.  Initial symptoms may be transitory and vague and confusing to doctor and patient.

42.  Doctor may not say MS is suspected.

43.  Neurologist may wish to witness at least two distinct episodes.

44.  Symptoms separated by at least a month and persisting for at least 24 hours.

45.  Will become severely disabled.

46.  Life expectancy is near normal for MS.

47.  "5 year rule" - first 5 years probably indicate how the disease will continue (relapse-remitting or progressive).

48.  After 5-10 years becomes more reliable predictor.

49.  About 45% MS sufferers are not severely affected.

50.  Some 40% become progressive after several years of relapsing-remitting MS.

51.  Years of mild affect from an early age may later develop to severely disabled.

52.  Later onset (55 years) - mostly males - may indicate faster, more progressive course.

53.  One of most common diseases of CNS - inflammatory demyelinating condition.

54.  Interferes with smooth, rapid and co-ordinated muscular (limb) movements.

55.  Scars appear at different times and in different areas of brain and spinal cord MRI.

56.  Disease of young adults - twice as common in women than in men. Mean age of onset is 29-33 years.

57.  Total and Permanent Disabilities.

58.  Loss of Speech.

59.  Blindness.

60.  Paralysis.

61.  Difficulty in walking.

62.  Abnormal sensations such as numbness or "pins and needles".

63.  Pain and loss of vision due to optic neuritis, an inflammation of the optic nerve.

64.  Tremor.

65.  Lack of co-ordination.

66.  Slurred speech.

67.  Sudden onset of paralysis, similar to a stroke.

68.  A decline in cognitive function - the ability to think, reason, and remember.

69.  Primary symptoms - direct result of demyelination. Weakness, numbness, tremor, loss of vision,      pain, paralysis, loss of balance, and bladder and bowel dysfunction.

70.  Secondary symptoms - complications that arise as a result of the primary symptoms.

71.  Vision. Optic neuritis. May not affect vision but still develop MS. ON not always develops into MS - about 50-60% do. So 40-50% don't. While other disease processes can cause ON, MS is most likely. Compromise Total and Permanent Blindness condition?

72.  Blurring of vision.

73.  Double vision (diplopia).

74.  Involuntary rapid eye movement (nystagmus) at extreme lateral gaze.

75.  Total loss of sight (rare).

76.  Opsoclonus (related eye disorder) - "jumping vision".

77.  Diplopia (double vision). Imperfect co-ordination of eye muscles.

78.  Visual symptoms not uncommon in MS - rarely total blindness.

79.  Can happen and be permanent symptoms.

80.  Co-ordination. See also note 29, 65, 77, 86.

81.  Loss of balance.

82.  Tremor.

83.  Unstable walking (ataxia).

84.  Giddiness (vertigo).

85.  Clumsiness of a limb.

86.  Lack of co-ordination.

87.  Cognitive and emotional disturbance - problems with short term memory.

88.  Concentration.

89.  Judgement.

90.  Reasoning.

91.  About 50% will develop some cognitive dysfunction.

92.  Slowed ability to think, reason, concentrate or remember.

93.  Spasticity - altered muscle tone; muscle stiffness.

94.  Affect mobility.

95.  Walking spasms.

96.  Wide range of involuntary muscle stiffness or spasms.

97.  Common symptom of MS.

98.  Feelings of pain or tightness around joints can cause low back pain.

99.  Baclofen (Lioresol) and Tizonidine (Zonoflex) commonly used (muscle relaxants).

100.       Less common is diazepam (Valium). Not first choice. Sedative/dependence potential.

101.       Dantrolene (Dantrium) last resort - can cause liver damage and blood abnormalities.

102.       Phenol is for nerve blocking agent.

103.       Sensation - tingling, numbness or burning feeling in an area of the body.

104.       Other indefinable sensations.

105.       Pain may be associated with MS (facial pain - Trigeminal neuralgia) and muscle pains.

106.       Sensitivity to heat - symptom very commonly causes a transient worsening of symptoms.

107.       Speech and swallowing.

108.       Abnormal speech.

109.       Slurring of words.

110.       Changes in rhythm of speech.

111.       Difficulty in swallowing - dysphagia - is mostly in advanced stages.

112.       Silent aspiration is inhaling small amount of food or drink without knowing.

113.       Strength. Weakness can particularly affect the legs and walking.

114.       Fatigue. Debilitating general fatigue. Unpredictable. Common and troubling symptom.

115.       Bladder control - need to pass water frequently and/or urgently.

116.       Incomplete emptying at inappropriate times.

117.       Bowel problems include constipation and, infrequently, loss of bowel control.

118.       Sexuality - impotence, diminished arousal, loss of sensation.

119.       Tremor - uncontrollable shaking. Can occur in various parts of body.

120.       Intention tremor is shaking when at rest.

121.       Postural tremor is limb or whole body supported against gravity.

122.       Nystagmus is regarded as a tremor for these purposes.

123.       Dizziness and vertigo. Common symptom. Off balance or light-headed.

124.       Could be caused by benign tumours or middle ear disease/inflammation.

 

Types of MS

 

Course of MS unpredictable. Some are minimally affected by disease while others have rapid progress to total disability, with most fitting between these two extremes. An exacerbation is a rapid progressive worsening of symptoms lasting at least a day in a particular area that has not had any new symptoms within the lost month. Temporary increase of symptoms experienced with heat or overexertion is not an exacerbation. Symptoms decrease with rest or cooling down. To reduce these exacerbations stress limitation (emotional and physical) recommended. Compromises diagnosis but limits problems. Sleep deprivation can bring on an attack. Flu can precipitate an attack. Stay well! Although every individual will experience a different combination of MS symptoms there are a number of distinct patterns relating to the course of the disease:

 

     Relapse-Remitting MS

85% Begin with this form of MS and more than half (50%) have this form of disease at any one time. May be inactive for months or years with minimal symptoms. Maybe one or two flare ups every 1-3 years followed by periods of remission. Symptoms may worsen with each recurrence and be alone or in combination, typically appear suddenly and lost a few weeks or months, then gradually disappear. Maybe residual impairment. Unpredictable. New symptoms appear. Frequency - approx 25%.

 

     Benign MS

Not all with MS develop more progressive form of disease. Between 10-15% symptoms are mild to moderate, do not worsen and do not lead to permanent disability. One or two attacks with complete recovery. Benign MS can only be identified when there is minimal disability. 10-15 years after onset and initially would hove been categorised as relapsing-remitting MS. Benign MS tends to be associated with less severe symptoms at onset (e.g. sensory). Frequency - approx 20%.

 

     Secondary Progressive MS

Usually after years of remitting-relapsing MS, at least one-half will enter a stage of continuous deterioration often with superimposed relapses. Sudden relapses may still continue to occur. Presumably some neurological function affected worse thon others. About 40% of cases.

 

     Primary Progressive MS

From first appearance of symptoms, neurological function deteriorates slowly without periods of remission. Lack of distinct attacks. Accumulation of deficits and disability which may level off at some point or continue over months or years. About 10-15% begin with this disease pattern.

 

     Progressive Relapsing MS

This is primary progressive MS with the addition of sudden episodes of new symptoms or worsened existing ones. Form quite rare, accounting for less than 5% of cases.

 

Symptoms

 

The initial symptoms of MS are most often:

 

     difficulty in walking

     abnormal sensations such as numbness or "pins and needles"

     pain and loss of vision due to optic neuritis, an inflammation of the optic nerve

 

Less common initial symptoms may include:

 

     tremor

     lack of co-ordination

     slurred speech

     sudden onset of paralysis, similar to a stroke

     a decline in cognitive function - the ability to think, reason, and remember

 

It is useful to divide the symptoms of MS into primary and secondary categories:

 

Primary Symptoms are a direct result of demyelination, the destruction of myelin - the fatty sheath that surrounds and insulates nerve fibres in the central nervous system. This impairs transmission of nerve impulses to muscles and other organs. The symptoms include weakness, numbness, tremor, loss of vision, pain, paralysis, loss of balance, and bladder and bowel dysfunction. Many of these symptoms can be managed effectively with medication, rehabilitation, and other medically-based methods.

 

Vision blurring and diplopia (double vision), optic neuritis, nystagmus (involuntary rapid eye movement) - could be brain tumour - but total loss of sight (rare) but still possible.

 

Double Vision (diplopia). Not perfect co-ordination of eye muscles causing particular movement. Visual Symptoms not uncommon in MS - rarely total blindness. But can happen and be permanent.

 

Optic Neuritis is an inflammation of the optic nerve. Temporary. Also referred to as retrobulbar neuritis (nerve is behind the globe of eye) or lesions along nerve pathways that control eye movements and visual co-ordination. May result in blurring of vision or blindness in one eye. Rarely occurs in both eyes at same time but not permanent. A scotoma (dark spot) may occur in centre of visual field. Estimate is 55% MS sufferers will have an episode of ON. May not necessarily affect vision but still develop MS. ON not always develop into MS - about 50-60% do. While other disease processes can cause ON, MS is most likely.

 

Uncontrolled Eye Movements - nystagmus. Horizontal or vertical is a common symptom. Maybe mild and occur with an extreme lateral gaze. Maybe severe enough to impair vision. May cause the related eye disorder, opsoclonus - "jumping vision".

 

Secondary Symptoms are complications that arise as a result of the primary symptoms. For example, bladder dysfunction can cause repeated urinary tract infections. Inactivity can result in disuse weakness (not related to demyelination), poor postural alignment and trunk control, muscle imbalances (adaptive shortening and/or stretch weakness), decreased bone density (increasing risk of fracture), and shallow, inefficient breathing. Paralysis can lead to the secondary symptom of bedsores. While secondary symptoms can be treated, the optimal goal is to avoid them by treating the primary symptoms.

 

     Bladder Control problems include the need to pass water frequently and/or urgently, incomplete emptying at inappropriate times. Bowel problems include constipation and, infrequently, loss of bowel control

 

     Co-ordination - loss of balance tremor unstable walking (ataxia) giddiness (vertigo) clumsiness of a limb lack of co-ordination.

 

     Cognitive and emotional disturbance - short term memory problems, concentration, judgement or reasoning. About 50% will develop some cognitive dysfunction. In MS generally means slowed ability to think, reason, concentrate or remember. Only 10% of this group (5% of all) will develop severe problems that interfere with everyday activities. Usually develops in long term disease but can occur at onset. Maybe subtle beginnings. Finding right words, remembering what to do on the job or daily routines at home. Changes in personality or personal habits noticed by family members first. Cognitive function can be affected by aging or medication so careful evaluation necessary to determine cause.

 

     Dizziness and Vertigo - common symptom. May feel off balance or light-headed. Vertigo less often but happens. Due to lesions (damaged areas). Could be caused by benign tumours of acoustic nerve (connects ear to brain) or middle ear disease/inflammation.

 

     Fatigue - a debilitating kind of general fatigue. Unpredictable or out of proportion to the activity. Fatigue is one of the most common symptoms of MS

 

     Numbness (paraesthesia) of face and body, or extremities one of most common symptoms of MS. Often first symptom experienced by those eventually diagnosed as having MS.

 

     Spasticity - altered muscle tone can produce spasticity or muscle stiffness. Can affect mobility and walking spasms. Wide range of involuntary muscle stiffness or spasms - sustained contractions or sudden movements. Common symptom of MS. May be mild feeling of tightness of muscles or so severe as to produce painful/uncontrollable spasms in extremities - usually legs. Feelings of pain or tightness around joints. Can cause low back pain. In flexor spasticity hamstrings involved. Hips and knees bent forward. Extensor spasticity (quadriceps) - legs close together, possibly ankles crossed.

 

Can also occur in arms but less common. Boclofen (Lioresol) and Tizonidine (Zonoflex) commonly used drugs. Muscle relaxants. Neither is cure of spasticity but good safety record. Less common is diazepam (Valium) - not first choice. Sedative and dependence potential. But longer effect to Baclofen. Small doses likely at bedtime to improve sleep patterns. Dantrolene (Dantrium) last resort can cause liver damage and blood abnormalities. Phenol is used as a nerve block agent.

 

     Sensation - tingling, numbness or burning feeling in an area of the body and other indefinable sensations. Pain may be associated with MS (facial pain such as trigeminal neuralgia) and muscle pains.

 

     Sensitivity to heat - this symptom very commonly causes a transient worsening of symptoms

 

     Speech and Swallowing - abnormal speech, slurring of words, changes in rhythm of speech, difficulty in swallowing - dysphagia - is mostly in advanced stages. Silent aspiration is inhaling small amount of food or drink without knowing.

 

     Strength - weakness can particularly affect the legs and walking 

 

     Sexuality - impotence, diminished arousal, loss of sensation

 

     Tremor - some degree of tremor or uncontrollable shaking. Can occur in various parts of body. Intention tremor - shaking when at rest. Tremor develops and becomes more pronounced on movement of hand or foot. Most disabling in MS. Different in PD - tremor may cease upon movement. Postural tremor - limb or whole body supported against gravity. Shaking while sitting or standing but not lying down. Nystagmus is regarded as a tremor for these purposes. Tremor most difficult symptom to treat. No consistently effective drugs. Isolation can lead to depression. Usually self-imposed to ovoid embarrassment.

 

Diagnosing MS

 

Early MS may present itself as a history of vague symptoms. The medical history may include past record of signs and symptoms as well as current status of health. Type of symptom, their onset and pattern may suggest MS, but full physical examination and medical tests will be needed to confirm the diagnosis. Symptoms may have subsided and many signs could be attributed to a number of medical conditions. Period of time may elapse and a prolonged diagnostic process may be involved before MS is suggested. A possible diagnosis of MS may be more clear-cut with classic symptoms (e.g. optic neuritis) and o distinct chronology of attacks.

 

Neurologist requires evidence that the types of neurological deficits indicate involvement of at least two different areas of the CNS with effects occurring at two separate times. A definite diagnosis may take several months or even years. See Eric Stern - MS since 1980 but not diagnosed till 1986. Multiple Sclerosis is essentially a clinical diagnosis and there are no tests which are specific for the condition and no single test is 100% conclusive. No typical MS. Most will experience more thon one symptom but no person would hove them all though some are common to all sufferers. Some of the symptoms are immediately obvious but others like fatigue, altered sensation, memory and concentration problems are often hidden symptoms. Several tests and procedures are needed to establish a diagnosis.

 

Neurological Examination

 

A neurologist would test for abnormalities in nerve pathways. Some of more common neurological signs involve changes in eye movements, limb co-ordination, weakness, balance, sensation, speech and reflexes. However, this examination cannot conclude what is causing abnormality so other possible causes of illness which produce similar symptoms to MS must be eliminated. Demyelination (scarring) occurs conduction of messages along nerves may be slowed. Evoked potentials measure velocity. Normally instantaneous brain reaction to such stimuli. Delay may occur in demyelination. Classic for MS is optic neuritis followed several months later by tingling and weakness in legs. History very important.

 

MRI

 

This provides detailed pictures of brain and spinal cord, showing existing areas of sclerosis. It cannot be regarded as conclusive but is the only test in which MS lesions can be seen. Not all lesions may be seen and many other conditions can produce identical abnormalities. Nevertheless, it remains a significant indicator to confirm a diagnosis of MS.

 

Lumbar Puncture

 

Cerebrospinal fluid is tested for antibodies. These can occur with MS but also other neurological conditions. Test may indicate MS but not in itself conclusive. Diagnosis of MS not always clear cut. Initial symptoms may be transitory and vague and confusing to both the person and their doctor. Invisible or subjective symptoms are often difficult to communicate to doctors and are sometimes dismissed as being a neurotic or a hypochondriac. Following an episode for which sought medical advice, doctor may not say MS is suspected. Delay may be very reasonable because neurologist may wish to witness at least two distinct episodes with symptoms that are separated by at least a month and persisting for at least 24 hours. The diagnosis of MS does not mean cessation of work. Many people can lead productive, fulfilling and relatively normal lives. But at least 15% will become severely disabled (fulltime wheelchair). However, life expectancy is near normal for MS.

 

General

 

It is difficult to predict the course of MS for any individual. The first 5 years will probably indicate how the disease will continue. Relapsing-remitting or progressive, for example. But after 5-10 years it becomes a more reliable predictor. There are many variables. About 45% MS sufferers are not severely affected. But some 40% become progressive after several years of relapsing-remitting. Research suggests that an early onset (under 16) gives a more favourable prognosis but 10-15 years of mild affect from an early age may later develop after 20 or 30 years with MS sufferer as severely disabled. Total length of time. Others developing late (55 and on) - particularly in males may indicate a faster and more progressive course of the disease.

 

MS is one of most common diseases of Central Nervous System (CNS). It is an inflammatory demyelinating condition. The fatty material that insulates nerves is damaged. In MS, disruption is caused in the conducted electrical impulses. This interferes with smooth, rapid and coordinated muscular (limb) movements. Sites where myelin lost (plaques or lesions) appear as hardened (scar) areas. In MS these scars appear at different times and in different areas of brain and spinal cord. Seen on MRI. MS means many scars.

 

Cause not known (idiopathic). Maybe "autoimmune" disease - body attacks its own cells and tissues. Possibly virus lying dormant. Activated by a trigger and enters brain (tricks blood/brain barrier defences) then activate other elements of the immune system to attack and destroy myelin. MS is a disease of young adults - mean age of onset is 29-33 years - but can be anything from 10-59 years.

 

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