* MULTIPLE SCLEROSIS

* DEFINATION
MULTIPLE SCLEROSIS IS AN AUTOIMMUNE DISEASE OF THE CNS
WHITE MATTER CHARACTERIZED BY A RELAPSING OR
PROGRESSIVE COURSE
MS is characterized by inflammation, selective demyelination,
and gliosis.
Acute and chronic symptoms and can result in significant
disability and impaired quality of life.
 It was first defined by Dr. Jean Charcot in 1868 by its
clinical and pathological characteristics: paralysis and the
cardinal symptoms of intention tremor, scanning speech,
and nystagmus, later termed Charcot’s triad. Using
autopsy
studies he identified areas of hardened plaques and
termed the disease sclerosis in plaques.
 ONSET :
The onset of MS typically occurs between ages 20 and
40 years. MS is rare in children, as is the onset of symptoms
in adults older than age 50 years

The disease is more common in woman than in men by a

ratio of 2:1 to 3:1.
ETIOLOGY:
The risk of MS is increased in persons with an affected
family member.
Genetic studies have revealed many interacting alleles that may
contribute to MS susceptibility with mutations in the human
leukocyte antigen major histocompatibility complex (MHC) gene
most strongly correlated.
It appears that although individuals do not
inherit the disease, they may inherit a genetic susceptibility to
immune system dysfunction.
When persons with a genetic susceptibility are exposed to a viral
agent, the immune system responds
with activated myelin-reactive lymphocytes, a concept
known as molecular mimicry.
The viruses may be retained in the body, resulting in a self-
perpetuating autoimmune process.
DISEASE COURSE:
MS is highly variable and unpredictable from person-to-person
and within a given individual over time.
At one end of the continuum, there is benign MS, defined
as disease in which the patient remains fully functional
in all neurological systems 15 years after onset.
Benign MS affects fewer than 20% of cases.
At the other end of the continuum, there is malignant MS
(Marburg disease), a relatively rare disease course
characterized by rapid onset and almost continual progression
leading to significant disability or death within a relatively
short time after onset.
 Clinical manifestations:
There are four major disease courses:

1)Relapsing-remitting MS (RRMS) is the most common course, affecting

approximately 85% of patients with MS. It is characterized by discrete attacks
or relapses, defined as periods of acute worsening of neurological function.
Relapses are followed by remissions, defined as periods without disease
progression and partial or complete abatement of signs and symptoms.

3)Primary-progressive MS (PPMS) is a rare

form occurring in about 10% of cases. It is characterized
by a nearly continuous worsening of the disease from the onset without distinct
attacks

3) Secondary progressive MS (SPMS): begins with a relapsing-remitting course

followed by progression to steady and irreversible decline with or without
occasional
acute attacks.
.
Progressive-relapsing MS (PRMS):
begins with a progressive disease course from the onset and
steady deterioration (similar to PPMS), but with occasional
acute attacks. It affects approximately 5% of patients with
MS.
Pseudoexacerbation
refers to the temporary worsening of MS symptoms. The
episode typically comes and goes quickly, usually within 24
hours. The overwhelming majority of individuals with MS
demonstrate an adverse reaction to heat, known as Uthoff’s
symptom.
Anything that raises the body temperature can bring on a
pseudoattack.
SYMPTOMS:

Symptoms of MS vary considerably, depending on the

location of specific lesions. Early symptoms typically
include minor visual disturbances (e.g., episodes of double
vision) and paresthesias progressing to numbness,
weakness, and fatigability.
In more advanced stages, patients demonstrate multiple
symptoms with varying involvement.
1) Sensory

Complete loss of any single sensation (anesthesia) is rare.

Focal deficits can produce limited areas of diminished
sensation. Altered sensations are far more common and
can include paresthesias (pins-and-needles sensation) or
numbness of the face, body, or extremities. Disturbances
in position sense are also common, as are lower extremity
(LE) impairments of vibratory sense.
2) PAIN

Almost half experience chronic pain.

10 Patients often experience acute, paroxysmal pain
characterized by sudden and spontaneous onset. The pains are
described as intense, sharp, shooting, electric shock–like, and
burning.
The most common types are trigeminal neuralgia,
paroxysmal limb pain, and headache.
Trigeminal neuralgia (tic douloureux) results from
demyelination of the sensory division of the trigeminal nerve
innervating the face, cheek, and jaw. Eating, shaving, or
simply touching the face may trigger painful episodes,
3) Visual

Visual symptoms are common with MS and are found in

approximately 80% of patients. Involvement of the optic
nerve produces altered visual acuity; blindness is rare. Optic
neuritis, inflammation of the optic nerve, is a common
problem, and produces an icepick-like pain behind the eye
with blurring or graying of vision or blindness in one eye.
Eye movements can be disturbed in a variety of ways.
Nystagmus is common in patients with MS.
This involves involuntary cyclical movements of the eyeball
(horizontal or vertical) that develop when the patient looks
to the sides or vertically (gazeinduced nystagmus) or when
the patient moves the head.
4) Motor

Patients with corticospinal lesions demonstrate signs and

symptoms of upper motor neuron (UMN) syndrome.
Paresis, spasticity, brisk tendon reflexes, involuntary flexor
and extensor spasms, clonus, Babinski’s sign, exaggerated
cutaneous reflexes, and loss of precise autonomic control
all characterize UMN involvement.
5) Weakness

Patients with UMN syndrome demonstrate movements

that are slow, stiff, and weak, the result of loss of orderly
recruitment and reduced firing rate modulation of
motor neurons. Reduced muscle strength, power, and
endurance, along with impaired synergistic relationships,
are evident. Patients with cerebellar lesions demonstrate
asthenia or generalized muscle weakness along with
ataxia. Patients can also experience muscle weakness
secondary to inactivity. Muscle weakness can vary from a
mild paresis, often transient at first, to total paralysis of
the involved extremities.
6) Spasticity

Spasticity is an extremely common problem in patients

with MS, occurring in 75% of all cases. Spasticity can
range from mild to severe, depending on the duration of
the disease, number of relapses, and worsening symptoms
in recent months. It occurs in the muscles of the upper
extremities (UEs) and particularly the LEs
7) Fatigue

Fatigue has been defined by the Panel on Fatigue of

the MS Council for Clinical Practice Guidelines as “a
subjective lack of physical and/or mental energy that is
perceived by the individual or caregiver to interfere
with usual and desired activities.”
Fatigue comes on abruptly without warning and typically
worsens throughout the day. Patient complaints may include
feelings of overwhelming tiredness, exhaustion, and
weakness together with difficulty concentrating and mental
dullness.
8) Coordination and Balance

Demyelinating lesions in the cerebellum and cere bellar

tracts are common in MS, producing cerebellar symptoms.
Clinical manifestations include ataxia, postural
and intention tremors, hypotonia, and truncal weakness.
Ataxia is a general term used to describe uncoordinated
movements characterized by dysmetria, dyssynergia,
and dysdiadochokinesia.
9) Gait and Mobility

Individuals with MS experience difficulty walking as a

result of muscle weakness, fatigue, spasticity, impaired
balance, impaired sensation, visual problems, and ataxia.
Staggering, uneven steps, poor foot placement,
uncoordinated limb movements, and frequent loss of
balance characterize ataxic gait.
10) Speech and Swallowing
Speech problems are the result of muscle weakness,
spasticity, tremor, or ataxia and affect as many as 40% of
individuals with MS.
Dysarthria is characterized by slurred or poorly articulated
speech with low volume, unnatural emphasis, and slow rate.
Dysphonia is characterized by changes in vocal quality
including harshness, hoarseness, breathiness, or hypernasal
sounds.
Poor coordination of the tongue and oral muscles can also
result in dysphagia,difficulty in swallowing.
Signs of swallowing dysfunction include difficulty chewing
and maintaining a lip seal, inability to swallow (ingest food),
and spitting or coughing during or after meals.
11) Cognitive

Cognitive symptoms in MS are common, with approximately 50%

of patients demonstrating measurable impairments.

12) Depression

Clinical depression is common in patients with MS, with at least

50% of individuals experiencing a major depressive episode.
Depressive symptoms can include feelings of hopelessness or
despair, diminished interest or pleasure in activities, changes in
appetite and significant weight loss or gain, insomnia or
hypersomnia (daytime sleepiness), feelings of lethargy or
worthlessness, fatigue or loss of energy, decreased concentration,
and recurrent thoughts of death and suicide.
13) Emotional

Affective disorders occur in approximately 10% of cases

and can include changes in mood, feelings, emotional
expression, and control. Pseudobulbar affect (PBA),
also known as involuntary emotional expression disorder
or emotional incontinence, is characterized by sudden
and unpredictable episodes of crying, laughing, or other
emotional displays.
*Diagnosis
*Medical management
 *CLINICAL
EXAMINATION
* Mental status
* Cranial nerves
* Motor system
* Reflexes
* Sensory system
* Coordination
* Station and gait
*PRESENTING HISTORY
* Involves symtoms reflecting CNS INVOLVEMENT
* Pt may present with paresthesia of hand that may
resolve, and a few months later followed by weakness in
limb or visual disturbances (diplopia)
* The symotoms have to last for more than 24 hours
* • Diagnosis involves evidence of damage occurring in at
least two separate areas of CNS and at two separate
points in time at least one month apart (dissemination of
lesions in space and time)
 *Mental Status
* MMSE SCALE
* Level of consciousness
* Attention
* Orientation
* Language — fluency, comprehension, repetition, naming,
reading, writing
* Memory — immediate recall, recent, remote
* Higher intellectual function—general knowledge, abstraction,
judgment, insight, reasoning
* Mood and affect CNI: O
 *Cranial Nerve Examination
* CNII: Optic nerve
Visual acuity • Visual fields • Fundoscopy • Afferent
limb of pupillary function
* CNII & III: Pupillary function
Normal pupils are equal in size and shape and are
situated in center of iris
* CNII & III: Light reflex
Shine bright light obliquely into each pupil. Look for
both direct (same eye) and consensual (opposite eye)
reaction
* CNII & III: Accommodation
Hold finger 10 cm from patient’s nose. Alternate looking
into distance and at finger
* CNIII Oculomotor nerve
* CNIV Trochlear nerve
* CNVI Abducens nerve
* Visual inspection: ocular alignment, lids,
Convergence, Smooth pursuits, Saccades,
Nystagmus, 6 cardinal directions of gaze
* CNV: Trigeminal nerve
* Corneal reflex (CN V-afferent limb CN VII-efferent
limb), Masseter strength, Jaw jerk
 *Motor Examination
Compare left to right, proximal to distal, arms to legs:
bulk(with tape), tone(modified ashwarths scale),
strength( mmt- group/ individual)
Stretch reflex: Biceps (C5, C6; musculocutaneous) • Triceps
(C6, C7; radial) • Patellar (L2-L4; femoral) • Ankle (S1-S2;
tibial)
Superficial reflexes: Plantar (L4-S2, esp S1; tibial nerve) –
Babinski, etc. – Normal response = flexion (toes go down) –
Abnormal response = extension (dorsiflexion of great toe as
the extensor hallucis longus is recruited).
 *Sensory Examination
* SUPERFICIAL
Pain and temperature – Light touch
* DEEP
-pressure – Vibration – Proprioception
* CORTICAL
Graphesthesia – Stereognosis – Two Point Discrimination
* Test for sensory loss in most commonly affected nerve and nerve
root distributions: Palmar aspect of index finger (median nerve).
Palmar aspect of 5th finger (ulnar nerve). Web space between thumb
and index finger on dorsal surface of hand (radial nerve). Lateral
surface of foot (L5). Posterior aspect of leg (S1).
 *Coordination
*Control, precision, rhythm, synergy of movement
*Test at rest and with action in trunk and limbs, Finger-
nose-finger, Rapid alternating movements, Heel-knee-
shin, Finger or toe tapping
*Romberg sign: Ability to maintain upright position with
feet together and eyes open. Sway/fall when eyes
closed , indicates impaired proprioception or vestibular
dysfunction
 *Gait
* Posture of body and limbs
* Length, speed, and rhythm of steps
* Symmetry and base of gait
* Steadiness
* Arm swing
* Turns
* Test with normal gait, toe walking, heel walking,
tandem walking
* Physical Rehabilitation (susan o’Sullivan)
* Internet

*REFRENCES