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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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respiratory and urinary tract. The presence of a plausible
infectious process, along with an MRI showing no acute
lesion, is generally taken to diagnose a pseudorelapse.
Occasionally, patients with known MS will also present with
psychologically based symptoms in the setting of depression
or severe life stresses.
8
However, some caution is justified in making the diagnosis of
pseudorelapse. There is evidence that new MS lesions can be
triggered by infection, so the presence of a urinary tract
infection (UTI) or viral respiratory illness does not in itself
exclude a true relapse. Furthermore, current MR technology
does not detect all acute lesions; gray matter lesions, for
example, are not well seen without specialized sequences.
Given that MRI lesions accumulate at a rate at least 5 to 10
times greater than clinical relapses, it seems likely that some
true attacks are misdiagnosed as pseudorelapses.
Initial Presentation of MS
A single focal attack of demyelination does not in itself
constitute MS. The diagnosis of MS requires the
demonstration of CNS lesions disseminated in both space and
time, based on either clinical findings alone or a combination
of clinical findings and MRI or CSF-specific oligoclonal
bands (OCBs). 9 The initial presentation of MS may be a CIS,
that is, an attack compatible with MS but not yet fulfilling
diagnostic criteria. For patients with a CIS and an otherwise
normal MRI, the long-term likelihood (>10 y) of developing
MS is approximately 20%.
Just as there are no clinical presentations that are unique to
MS, any of the manifestations of MS can be an initial
presentation. Common presenting syndromes include optic
neuritis, spinal cord sensory or motor symptoms, transverse
myelitis, and brainstem and cerebellar syndromes.
Optic Neuritis
Optic neuritis typically begins with eye pain, worse with eye
movement, followed by blurred or dimmed central vision.
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Complete unilateral loss of vision is rare. On examination,
there is usually an afferent pupillary defect. Retinal
examination may reveal mild optic disc swelling (papillitis),
although if the lesion is located some distance from the fundus
(retrobulbar neuritis), the retinal examination may be normal.
The presence of retinal hemorrhages or exudates should
suggest a diagnosis other than acute demyelination.
10
Spinal Cord Syndromes
Spinal cord lesions can cause a variety of sensory and motor
symptoms or mixtures of the two. Persistent tingling and
numbness are common and may be bilateral, leading them to
be mistaken for the intermittent tingling of hyperventilation.
Strange sensations that the legs are swollen, cold, tightly
wrapped, or swaddled in soft gauze or severe pains that radiate
upward from the feet are characteristic of spinal cord lesions
and are easily dismissed as anxious or delusional. Motor
symptoms include leg weakness or clumsiness, often with
enhanced reflexes and extensor plantar responses. Transverse
myelitis is the most fulminant spinal cord syndrome, with
profound bilateral weakness, sensory loss, and bowel and
bladder dysfunction. Partial and incomplete spinal syndromes
with asymmetric signs are much more common.
11
Brainstem and Cerebellar Lesions
Brainstem and cerebellar lesions can cause various
combinations of vertigo, diplopia, dysarthria, limb
incoordination, and tremor. An internuclear ophthalmoplegia
is common and if bilateral is almost pathognomonic of MS.
MS can cause an attack of acute vertigo, but there are
generally other brainstem symptoms and signs such as
diplopia, dysarthria, facial numbness, limb weakness, or
clumsiness to indicate that this is not simply a peripheral
vestibular syndrome.
New Paroxysmal Symptoms
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New paroxysmal symptoms lasting seconds to minutes at a
time can indicate an acute attack if they recur over a period of
weeks or more. An example of this is Lhermitte symptom, an
electric shocklike sensation, typically induced by neck flexion,
which radiates down the back into the legs and often indicates
a lesion within the cervical spinal cord affecting the
spinothalamic tracts. Other paroxysmal symptoms include
tonic contractions of the face or limbs and fleeting episodes of
dysarthria or sensory disturbance. Trigeminal neuralgia
associated with atypical features such as onset before age
50 years, bilateral presentation, or facial numbness should
raise suspicion for MS. Painful paroxysmal symptoms
associated with MS are best treated with agents such as
carbamazepine and gabapentin, rather than opioids.
Imaging
Although computed tomography can sometimes demonstrate
MS lesions, MRI is currently the imaging modality of choice.
In many cases, MRI can establish a diagnosis of MS at the
time of the first attack by demonstrating not only the acute
lesion but also additional lesions disseminated in both time
and space. On MRI, brain lesions appear ovoid in shape and
are typically aligned at right angles to the corpus callosum,
forming a pattern on sagittal imaging referred to as “Dawson
fingers.” The most valuable sequences are T2/T2-fluid
attenuation inversion recovery, used to identify old (chronic)
lesions, and contrast-enhanced T1, which detects breakdown
of the BBB associated with acute white matter lesions.
Enhancement typically resolves within a month, usually
leaving a T2-hyperintense lesion that persists indefinitely. T2
lesions that are hypointense on T1 (“black holes”) are thought
to represent areas of severe white matter destruction and
axonal loss. Diffuse brain and spinal cord atrophy are also
commonly seen in advanced MS and correlate more strongly
with clinical disability than does total lesion burden. Spinal
cord lesions are common in patients with MS, although
isolated spinal involvement is uncommon.
12,13
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All patients with suspected MS should have MRI of the brain
with and without contrast. Neurology consultation can help
choose appropriate imaging to maximize diagnostic yield.
Oligoclonal Bands
Activation of an IgG (humoral) immune response leads to the
appearance on electrophoresis of multiple distinct bands of
IgG corresponding to the activation of multiple B cell clones.
This response can be seen in a variety of systemic infectious,
inflammatory, and neoplastic diseases. OCBs found in the CSF
and not in the serum indicate an immune response originating
within the CNS. This is seen not only in MS but also in
meningitis, neurosyphilis, progressive multifocal
leukoencephalopathy (PML), tumors, and many other CNS
disorders. CSF-specific OCBs are found in up to 95% of
patients with clinically definite MS.
14
Before the advent of MRI, OCBs were a vital factor in
establishing dissemination in time for the diagnosis of MS.
One meta-analysis found that the presence of OCBs in a
patient with a CIS was associated with an odds ratio of
approximately 10 for the development of MS. In the MRI era,
OCBs are often unnecessary for diagnosis. The neurology
consultant should decide whether lumbar puncture for OCBs is
needed, and because the results will not be immediately
available to influence management, the test is most
appropriately performed when the patient is admitted.
Differential Diagnosis
The differential diagnosis for a young adult who presents with
a classic history of two or more acute episodes of neurological
dysfunction with at least partial resolution is limited. For
patients with a single attack of CNS disturbance, the
differential is broad and includes many infectious,
inflammatory, ischemic, neoplastic and genetic disorders.
15
Given the complexities of the differential and the profound
consequences of a diagnosis of multiple sclerosis, emergency
medicine (EM) clinicians would be prudent to ask for
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neurology consultation to help with the evaluation of these
patients.
A number of clinical “red flags” suggest that a diagnosis other
than MS should be considered. Hyperacute onset of symptoms
(over seconds to minutes) or very short duration (minutes to
hours) suggests an ischemic or hemorrhagic lesion, seizure, or
syncope. Diffuse encephalopathy with confusion or depressed
level of awareness points toward a systemic process (toxic or
metabolic) or a multifocal CNS disorder such as encephalitis,
vasculitis, or posterior reversible encephalopathy syndrome.
A significant number of patients eventually receive an
incorrect diagnosis of MS, especially when difficult-tolocalize symptoms of dizziness, fatigue, and cognitive
difficulties are combined with nonspecific white matter T2hyperintense lesions. 16 Studies suggest that the most common
diagnoses for these patients are psychiatric disorders,
migraine, and chronic pain disorders such as fibromyalgia.
Complications of Established MS
Lower Urinary Tract Dysfunction
Lower urinary tract dysfunction is almost universal in MS,
appearing on average 6 years after onset. Normal bladder
function requires the bladder to fill at low pressure, preventing
damage to the kidneys, and then contract efficiently under
conscious control, avoiding urine stasis and infection. MS can
interfere at multiple levels—conscious control in the frontal
lobe, the reflex control center in the pons, and reflex arcs in
the spinal cord. Dysfunction can be broadly divided into
abnormal urinary storage (overactive bladder caused by
detrusor muscle overactivity) and abnormal emptying caused
by detrusor underactivity or detrusor-sphincter dyssynergia.
Overactive bladder symptoms classically include urgency,
frequency, and urge incontinence, whereas voiding
dysfunction causes hesitancy, weak stream, and sensation of
incomplete emptying. In patients with MS, however, urinary
symptoms correlate poorly with the underlying urodynamics.
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Patients with chronic urinary symptoms may benefit from
urological referral.
17,18
UTIs are exceedingly common in patients with MS and are
responsible for up to half of all hospital admissions in these
patients. The likelihood of a UTI increases with longer
duration of disease and increasing disability; additional risk
factors include female gender, elevated postvoid residual
volume, and the presence of an indwelling urinary catheter.
Intermittent self-catheterization may be useful in decreasing
the risk in patients who develop recurrent UTIs.
Asymptomatic bacteriuria should not be treated, except
perhaps in patients about to begin high-dose steroid for an
acute relapse. Local symptoms and signs of possible UTI
include suprapubic or flank pain, worsening of chronic urinary
urgency or frequency, catheter blockage, and cloudy or
malodorous urine; general symptoms and signs include fever,
lethargy, and worsening of previous deficits. Patients with
significant sensory deficits may not perceive symptoms such
as dysuria but may have other complaints such as increased
fatigue and spasticity secondary to their infection.
Spasticity
Spasticity (increased tone and stiffness in limbs) as well as
spontaneous or movement-induced muscle spasms are
common in MS, especially with spinal cord involvement.
Spasms can be quite painful and interfere with activities of
daily living. Spasms may be triggered by touching a limb or
by lower-body conditions that the patient may not perceive,
such as bladder infection, fecal impaction, and decubitus
ulcers. Painful spasm may respond to oral magnesium;
spasticity may be treated with gamma-aminobutyric acid
agonists such as baclofen and tizanidine.
19
Constipation
Constipation is present in more than 30% of patients, whereas
fecal urgency or incontinence is much less common. Severe,
chronic constipation can lead to complications such as
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impaction, sigmoid volvulus, large-bowel obstruction, and
perforation.
Generalized Fatigue
Generalized fatigue, although poorly understood, is almost
universal in MS and an important cause of work-related
disability. Sleep disorders (sometimes from bladder
dysfunction), pain, depression, and anxiety can contribute to
fatigue in these patients. Pharmacologic agents such as
amantadine, modafinil, and methylphenidate are sometimes
prescribed.
Cognitive Dysfunction
Cognitive dysfunction affects a large proportion of patients
with MS and may be underrecognized in routine clinical
assessment. Most often, there are impairments of episodic
memory and information processing speed.
20
Pain
Pain is a common and underrecognized complication of MS.
Spinal lesions can cause both localized back pain and painful
dysesthesias felt in the limbs. Other causes of pain include
painful tonic spasms and paroxysmal conditions such as
Lhermitte sign and trigeminal neuralgia. Antidepressants, such
as the tricyclic antidepressants and serotonin-norepinephrine
reuptake inhibitors, or antiepileptics, such as gabapentin, are
currently recommended for chronic pain. Opioids are not
recommended as first-line agents because of their limited
efficacy and potential for causing tolerance and addiction.
Emergency Department Issues in
Multiple Sclerosis
Although the neurologist must attend to the nuances of
diagnosis and choice of long-term treatment, the emergency
physician has a different set of questions. For a previously
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healthy patient with new neurological symptoms, the primary
questions are: Could this patient have multiple sclerosis? What
evaluation and testing should be done in the ED setting?
Should the patient be admitted?
For a patient with an established diagnosis of MS and new or
worsened neurological symptoms, the questions include: What
evaluation should be performed to decide whether they are
having a relapse? What testing might help? How important is
this determination? Should this patient be admitted?
Emergency Department Identification of
Patients With a Potential First Attack
Because the median age of onset for MS is approximately
30 years, MS should be considered in the differential diagnosis
of young patients presenting with new neurological symptoms.
Unfortunately, several cognitive biases can prevent MS from
being considered. The possibility of serious disease is more
easily set aside in young healthy persons. Clinicians may be
tempted to attribute unusual symptoms to anxiety, emotional
stress, or even a somatiform disorder.
A 2013 retrospective study at Mount Sinai Medical Center in
New York analyzed ED visits before an initial diagnosis of
MS. Over a 5-year period, there were 49 MS diagnoses and 98
ED visits before the diagnosis, 50% of which were for
neurological symptoms. Of those, 88% were retrospectively
felt by the study reviewers to have been a first presentation of
MS and included in the study.
21
The initial ED impression was MS for only 10% of patients,
whereas 57% received no specific initial diagnosis and 16%
received an incorrect diagnosis, such as stroke, neuropathy,
tumor, or muscle spasm. However, 78% of the ED
presentations resulted in a neurology consult and 76% were
admitted to the hospital, 82% of those to the neurology
service. Ultimately, 61% received a diagnosis of MS during
their admission, and 74% were diagnosed within a week. Of
the remainder, an additional 4% were diagnosed within a
month, 8% within 6 months, and another 6% within the year.
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Demographic factors of age, sex, and insurance status did not
predict diagnostic delay, but admitted patients were diagnosed
sooner. The authors concluded that ED presentation for acute
neurological symptoms represents an opportunity for rapid
diagnosis and treatment of early MS and that neurology
consultation and admission expedites the process.
Emergency Department Evaluation of
Patients With Known MS
Surprisingly little study has been devoted to the evaluation of
established patients with MS in the ED setting.
A 2014 observational study from Mount Sinai Medical Center
in New York examined ED utilization by patients with
established MS over a 3-year period in a population that
included a significant number of poor and uninsured patients.
22
Almost 75% of visits were for nonneurological symptoms,
such as musculoskeletal pain, fever, and gastrointestinal
complaints. More than half of the patients had severe
disability, defined as an EDSS of 6 or greater (needing an
assistive device such as a cane or crutch to walk 100 m).
Presentations directly related to MS, such as exacerbations,
were diagnosed in only 20% of patients with mild to moderate
disease (EDSS < 6) and 13.2% of visits overall. For patients
with mild to moderate disease (EDSS < 6), most visits were
for medical issues unrelated to MS; for those with severe
disease, most visits were for medical issues that were related
to MS but indirectly, such as UTI or pneumonia, decubitus
ulcers, or falls. Only half of the patients with RRMS were
receiving disease-modifying treatment.
A 2016 observational study from Johns Hopkins examined the
value of ED MRI in the evaluation of patients with MS who
present with a possible exacerbation. Over a 2-year period,
115 encounters resulted in MRI; 37% had active MRI findings,
42% were diagnosed with a true exacerbation, and 18% were
diagnosed with a pseudoexacerbation. When spine imaging
was performed, 20% showed acute activity, although only
10% showed activity only in the spine. MRI only occasionally
(8%) revealed an alternative diagnosis, such as radiculopathy,
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compressive myelopathy, and stroke. Of those diagnosed with
an exacerbation, 92% were admitted and almost all received
steroids.
23
The study demonstrated clinicians’ reliance on MRI to decide
disposition and treatment in large centers with ready access to
this resource. Indeed, the authors point out that “no
(nonpregnant) patient with MS presenting to the ED was
admitted for treatment of an MS exacerbation without either
ED MRI imaging or inpatient MR imaging, despite the current
definition of MS exacerbations as a clinical diagnosis.”
A 2017 observational study from the Cleveland Clinic divided
ED visits into three categories: category 1 comprised new
neurological symptoms, category 2 comprised worsening of
preexisting symptoms, and category 3 comprised
nonneurological complications of MS. Over a calendar year,
there were 97 MS-related ED visits, divided almost equally
among the three categories. In category 1, 64% of patients had
an MRI performed, of which 52% showed acute activity.
Although 33% of patients in this category therefore had a
positive MRI, almost twice that number (61%) were diagnosed
with a true exacerbation. In category 2, 48% of patients had an
MRI, and 29% of studies were positive. Although only 14% of
patients in this category had a positive study, almost twice that
number (24%) were diagnosed with a true exacerbation. The
authors do not specify what proportion of the patients
diagnosed with an exacerbation in the absence of a positive
MRI had no MRI done (i.e., judged not to need one for
diagnosis) and what proportion had a negative MRI but
nevertheless received the diagnosis on clinical grounds. 24 The
authors state that ED mistriage results in a large number of
unnecessary MRIs and admissions to the neurology service,
but they do not present data regarding who (EM or Neurology)
decided to perform an MRI and who decided on admission.
The study refutes some common assumptions regarding the
diagnosis of true and pseudorelapses. Patients with clinical or
laboratory findings suggestive of infection, especially UTI, are
often assumed to have a pseudorelapse. In this study, however,
patients diagnosed with true relapse had an overall rate of
suspected infection (30%) only slightly lower than those with
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