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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2745_Библиотеки_им_академика_М_И_Перельмана
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more of four areas of the CNS: periventricular, cortical or
juxtacortical, and infratentorial brain regions and the spinal
cord.
4
Disseminated in Time (DIT): The development or appearance
of new CNS lesions over time. This can be demonstrated by
the simultaneous presence of gadolinium-enhancing and
nonenhancing lesions at any time or by a new T2-hyperintense
or gadolinium-enhancing lesion on follow-up MRI, with
reference to a baseline scan, irrespective of the timing of the
baseline MRI.
4
If a patient has at least two attacks + one CNS lesion,
they will need the following:
DIS demonstrated by additional clinical attack
implicating a different CNS site or by MRI
If a patient has one clinical attack + at least two CNS
lesions, they will need the following:
DIT demonstrated by additional clinical attacks or
by MRI
Or
Cerebrospinal fluid (CSF)-specific oligoclonal bands
If a patient has one clinical attack + one CNS
lesion, they will need the following:
DIS demonstrated by additional clinical attack
illustrating a different CNS site or by MRI
And
DIT demonstrated by additional clinical attacks or
by MRI
Or
CSF-specific oligoclonal bands
In addition, the 2017 criteria provided further explanation for
CIS, and understanding the nuances of this definition is
important in the diagnostic algorithm of MS. CIS can be
defined as a monophasic clinical episode reflecting a focal or
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multifocal inflammatory demyelinating CNS event,
developing acutely/subacutely, lasting at least 24 hours, in the
absence of fever or infection, in a patient not known to have
MS. If the patient eventually satisfies the diagnostic criteria
for MS, the Clinical Isolated Syndrome will be known as the
patient’s first attack/relapse.
4
According to the 2013 revised classifications of MS
phenotypes, the disease onset can be defined by MS with an
attack onset or a progressive course onset. 2 Diagnosing
patients with the PP subtype of MS, which is defined by its
progressive disease course, also has its own unique diagnostic
criteria (see Table 3.2).
Patient with 1 year of disability progression independent
of clinical relapse, plus two of the following criteria:
One or more T2-hyperintense lesions characteristic
of MS in one or more of the following brain regions:
periventricular, cortical or juxtacortical, or
infratentorial
Two or more T2-hyperintense lesions in the spinal
cord
Presence of CSF-specific oligoclonal bands
Table 3.2
2017 McDonald Criteria for the Diagnosis of Primary Progressive
Multiple Sclerosis (MS)
Primary progressive MS can be diagnosed in patients with:
1 y of disability progression independent of clinical relapse
Plus two of any of the following:
≥1 T2-hyperintense lesion a that are characteristic of MS
≥1 of the following brain lesions
Periventricular
Cortical/juxtacortical
Infratentorial
≥2 T2-hypertense lesion a in the spinal cord
Presence of CSF-specific oligoclonal bands
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a
For 2017 McDonald Criteria, there is no requirement for a
distinction between symptomatic and asymptomatic MRI
lesions.
Table adapted with permission from Carroll WM. 2017 McDonald MS diagnostic
criteria: Evidence-based revisions. Mult Scler. 2018;24(2):92-95. Copyright ©
2018 SAGE Publications.
Signs and Symptoms
Identifying signs and symptoms can often be difficult, as the
disease process can be subtle and occur over a long time
period, with large gaps between initial symptoms. Patients will
often forget or downplay symptom significance, and it can be
crucial for practitioners to ask detailed questions to elicit all
necessary information. Furthermore, a detailed review of
systems is often extremely valuable for collecting vital
information. Often times, patients present to clinic for further
evaluation after symptoms have already started to affect work
performance, daily function, or quality of life. Identifying
disease symptoms and establishing an accurate timeline of
events is an essential step that will dictate further diagnostic
workup and aid in quicker diagnosis and earlier treatment.
Table 3.3 is a brief summary of common presenting symptoms
for MS. This is by no means a comprehensive list but rather
includes major neurologic red flags that a practitioner should
identify. Furthermore, these symptoms can also be present in
many other diseases and in a large severity spectrum, which
will be discussed in the next section.
Table 3.3
Common Presenting Signs and Symptoms in Multiple Sclerosis (MS)
(Not a Comprehensive REVIEW OF SYSTEMS)
System Common Presenting Signs or Symptoms
General
Fatigue
“Brain fog”
Reduced stamina: There are many metabolic and psychologic
differentials for fatigue that need to be ruled out. But this can
be a hallmark symptom in MS
Uhthoff phenomenon: worsening of MS symptoms when
exposed to heat
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System Common Presenting Signs or Symptoms
HEENT
Decreased visual acuity
Blurred vision/unilaterally or bilaterally (like looking through
a smudged window
Double vision
Decreased intensity of color
Eye pain with movement
Dysarthria
Dysphagia
Weakness with muscles of facial expression
Hearing loss
Dizziness/vertigo
GU
Urinary incontinence
Urinary retention
Increased urinary frequency
Increased frequency of UTIs (as a result of the above)
Endo
Intolerance to heat
Psych
Emotional lability
Pseudobulbar affect
Depression
Anxiety
Neuro
Paresthesias
Dysesthesias
Numbness
Focal motor weakness
Problems with balance
Problems with coordination (both upper and lower extremity)
Muscle spasticity
Cognitive issues such as word finding difficulty or memory
problems
GU, genitourinary; HEENT, head, eyes, ears, nose, and throat.
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Physical Examination
A comprehensive neurologic examination is pivotal for
identifying neurologic deficits and asymmetries that can lead
to a proper diagnosis. This section will not delve into the
comprehensive details of a neurologic physical examination
but will rather highlight elements that are more characteristic
of MS. As we have discussed, the clinical presentation of MS
is quite variable and the physical examination findings may be
similar to other neurologic diseases, in particular, diseases that
affect upper motor neurons. As a nonneurologist, it is
important to quickly and effectively collect the most
significant data that can point to a diagnosis of MS. Here we
will discuss specific areas of the neurologic examination that
are the most pertinent for MS.
Mental Status
Patients often present to clinic reporting cognitive decline,
including difficulty with word finding, memory, or slower
processing speed at work or home. Although more extensive
neurocognitive testing may be required to elicit more subtle
cognitive deficits, the Montreal Cognitive Assessment
(MoCA) is frequently used as a screening tool used at the
point of care. 5 In addition, the Symbol Digits Modality Test
(SDMT), in which the patient is asked to substitute a random
list of symbols with digits based on a key, is used to assess
information processing speed and has been shown to be a
better measure of cognitive function over time in patients with
MS. 6 Other neuropsychological tests have been studied in
patients with MS, including the Brief International Cognitive
Assessment for MS (BICAMS) and the Paced Auditory Serial
Addition Test (PASAT), and will be covered more fully in the
chapter titled Cognitive Function in MS.
Cranial Nerves
Complete cranial nerve examination is essential for any
physical examination in a patient with concern for MS.
Although isolated cranial neuropathies have been reported in
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the literature, 7 they are not commonly seen as presenting signs
of MS and may be seen as a result of demyelinating lesions of
the brainstem. Below is a brief summary of common findings
with associated cranial nerves. Only the most common cranial
nerve findings are listed, and this is merely a quick reference
guide rather than a comprehensive reference.
CN II
Visual deficits are an extremely common presenting symptom
with MS. Deficits can present as decreased visual acuity,
blurred vision, diplopia, reduced color vision, or visual field
deficits (Table 3.4).
Table 3.4
Hallmark MS Findings—Visual System
9
Optic Neuritis
Initial finding in 20% of patients with MS
More often unilateral but can be bilateral
Commonly seen with disc pallor on funduscopic examination
Caution: common in NMO spectrum disease as recurrent optic neuritis
can be a red flag for neuromyelits optica
Internuclear Ophthalmoplegia (INO)
Inability of contralateral eye to medially deviate adduct with lateral gaze
Secondary to lesions affecting the medial longitudinal fasciculus
Charcot Neurologie Triad
Triad characterized by nystagmus, intention tremor, and scanning or
staccato speech
The most common initial testing includes:
Snellen eye examination
Visual field test
Funduscopic evaluation:
Assess for optic disc pallor, although disc may
appear normal on funduscopic examination.
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Optical coherence tomography (OCT) 8 :
Provides high-resolution image of retinal nerve fiber
layer. Although not a common first-line treatment,
this technique has become more present as of late
and can be a more advanced imaging technique to
evaluate for neurodegeneration or progression of
disease. Further discussion on OCT can be found in
the ophthalmology chapter.
CNs III, IV, VI
9
A thorough examination of extraocular movement is essential,
as dysfunction in the corresponding cranial nerves can
precipitate diplopia, blurry vision, or visual field deficits.
Important aspects to observe:
Symmetrical range of motion for ocular movement,
assessing for unilateral CN dysfunction
Saccadic movements
Nystagmus
Coordinated eye movement, i.e., internuclear
ophthalmoplegia
CN V
Facial numbness is a common presenting symptom with MS,
as well as other conditions of upper motor neuron origin.
Careful examination of the V1, V2, V3 branches of the facial
dermatomes with light touch and pin prick is considered an
important aspect for MS evaluation. In addition, absence of
corneal reflex can indicate pathology involving any pathway
of the reflex circuit.
CN VII
Proper assessment of the muscles of facial expression are
crucial for identifying focal asymmetries or more global sideto-side differences in facial muscle tone. This can be important
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for evaluating other common differential diagnosis that need
to be ruled out. Furthermore, in patients reporting changes in
speech quality, facial motor strength is important to evaluate.
CN VIII
Although isolated hearing loss is not a common presenting
feature of MS, it is still important to evaluate as part of an MS
examination. Initial evaluation can be performed in office with
standard high- and low-frequency tuning forks. It may be
important to establish a baseline hearing level with more
formalized audiological evaluation.
CN IX
Dysarthria and dysphagia can be common findings in early
MS. Evaluating for midline symmetrical movement of the
oropharynx and soft palate should not be overlooked. In some
cases, formal swallow evaluation with a speech and language
pathologist will be needed to conduct a barium swallow study
particularly if a patient is complaining of choking on certain
foods or any other swallowing complaints.
CN XI
Muscle testing should be performed on the
sternocleidomastoid and trapezius muscles to assess for
muscle tone symmetry.
CN XII
Midline tongue protrusion is important to evaluate for
centrally mediated process involving the hypoglossal nerve.
Motor Testing
Outside the muscles of facial expression, complete motor
testing should be performed involving C5-T1 and L1-S2
myotomes.
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Sensory Testing
Complete sensory testing involving pin prick, light touch,
vibration, and temperature are important for teasing out
differential diagnosis. It is important to evaluate if the
associated sensory deficits follow specific spinal root levels,
stocking/glove distribution, or focal peripheral nerves. In
addition, proprioceptive evaluation of the big toe/thumb, as
well as Rhomberg test is important to evaluate. Furthermore, it
is not uncommon for patients with MS to present with
bandlike/bear hug dermatomal sensory distribution around the
abdomen, often due to CNS lesions localized to thoracic spine.
Identifying potential etiologies for isolated sensory symptoms
could necessitate further evaluation with imaging or
electrodiagnostic testing.
Reflex Testing
Reflexes are often normal or hyperreflexic in MS. Performing
standard reflex testing at the biceps, brachioradialis, triceps,
patella, and Achilles is considered standard for any neurologic
examination.
High-yield tests for upper motor neuron involvement:
Babinski sign:
Dorsiflexion of the great toe after irritation to
plantar surface of the foot. Most common test
for upper motor neuron damage
Jaw jerk reflex:
Stretch reflex for the masseter muscle,
involving the trigeminal nerve CN V. Signifies
a lesion above the foramen magnum
Hoffman reflex:
Test involves loosely holding the middle finger
and flicking the fingernail downward. A
positive response is seen when there is flexion
and adduction of the thumb on the same hand
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Positive result signifies upper motor neuron
problem in the corticospinal tract
Clonus:
Involuntary, rhythmic, repetitive muscle
contractions
Often seen with upper motor neuron damage
and a sign of hyperexcitability
Most commonly elicited at the ankle, with
abrupt dorsiflexion of a relaxed ankle joint
Oppenheim sign:
Dorsiflexion of the great toe after irritation of
the medial tibia. Signifies upper motor neuron
damage, similar to Babinski sign, involving
corticospinal tract
Chaddock sign:
Dorsiflexion of the great toe with stroking of
the lateral malleolus. Signifies upper motor
neuron damage, similar to Babinski sign,
involving corticospinal tract
Lhermitte phenomenon:
Electrical sensation that runs up and down the
spine, often as a result of neck flexion.
Typically represents damage in the upper
cervical spine or lower brain stem.
Has been estimated to be present in 33% of
patients with MS, and 16% reported this
phenomenon in the first MS episode
10
Other important considerations:
Spurling compression test—Evaluate for potential
cervical radiculopathy as cause for upper extremity
paresthesias, pain, or focal weakness
Tinel test—Perform at the wrist and elbow to assess
for focal nerve entrapment involved with ulnar
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