PANCE Blueprint Neurology (6%)

Multiple sclerosis (ReelDx + Lecture)

Patient presents as → a 21-year-old woman who is brought to your urgent care 30 minutes after an acute onset of double vision in the left eye. Two months ago, she had an acute episode of pain with movement of the left eye along with loss of both central vision and pupillary light reflex. These symptoms subsided spontaneously. Examination shows a delay in the adduction of the left eye with nystagmus of the right eye on lateral gaze.

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What is relapsing-remitting MS?
MS attacks of unpredictable frequency, interspersed with periods of almost complete recovery. This is the most common presentation.
What are some of the most common presenting symptoms of MS?
Blurred vision with decreased acuity, double vision, paresthesias (numbness and tingling) in the extremities, ataxia, fatigue, and focal motor symptoms

An autoinflammatory disease causing demyelination, neuronal loss, and scarring within the white matter of the brain and spinal cord.

  • Usually present with visual disturbances often over many years.
  • Recurrent visits for different neurologic symptoms, often the first visit is for visual problems, followed by weakness in distal limb, ataxia or paresthesias over several years. Distal to proximal muscle weakness.
  • Most common symptoms:
    • Sensory loss
    • Optic neuritis
    • Weakness
    • Paresthesias
  • Lhermitte's sign (patient in the video above) refers to an electrical shock sensation in the limbs and torso, brought on by movement (mostly flexion) of the neck. It is due to pathology of the cervical spinal cord and a common finding in early MS

Four recognized forms of multiple sclerosis (MS):

  1. Relapsing-remitting multiple sclerosis (RRMS) (85% MOST COMMON TYPE) episodic flare-ups occurring over days to weeks between periods of neurologic stability. During attacks, new symptoms may present, whereas previous symptoms may worsen. Complete recovery or residual deficits may ensue following each bout.

  2. Secondary progressive multiple sclerosis (SPMS) beginning as RRMS, progressive deterioration in neurologic function, unassociated with attacks, eventually ensues (progression may continue or halt). ∼2% risk per year of RRMS becoming SPMS

  3. Primary progressive multiple sclerosis (PPMS) (∼15%): steady decline of neurologic function from onset of disease without episodic flares

  4. Progressive relapsing multiple sclerosis (PRMS) (∼5%): steady decline of neurologic function from onset of disease with episodic flare-ups

DX with MRI looking for plaques associated with MS (McDonald Diagnostic Criteria) – The most sensitive and specific investigation for this disorder is MRI of the brain and spinal cord. The MRI scan will reveal plaque formation (a subsequent stage that results from the loss of the myelin sheath in different parts of the CNS) and spotty and irregular demyelination in the affected areas in a patient with MS.

"The targeted cells in the MS disease process are the oligodendrocytes of the CNS. These cells fabricate and maintain the myelin sheaths, the material covering the axons that is necessary for the normal conduction of nerve impulses."

Diagnosis criteria

  1. Two episodes, or attacks, of symptoms
  2. Two different areas of the CNS involved

Recommended medications include corticosteroids (for acute attacks), interferon betas (Avonex), fingolimod (Gilenya), natalizumab (Tysabri), dimethyl fumarate (Tecfidera), and mitoxantrone (Novantrone).

  • Physical therapy and exercise may help increase coordination and decrease spasticity.
  • Modafinil, amantadine, pemoline, and methylphenidate to treat fatigue
  • Urge incontinence can be treated with Oxybutynin
  • Drugs are useful in treating spasticityBaclofen, tizanidine, diazepam, and dantrolene 
  • Identifying factors that trigger symptoms is critical for avoiding exacerbations of the disease.

multiple-sclerosis-symptoms-and-diagnosis_5975_1492145264 Multiple sclerosis (MS) is a demyelinating disorder that demonstrates unclear pathogenesis but has significant evidence of autoimmune involvement. Clinical presentation is variable with subtypes delineated by symptom exacerbation, but it classically presents with Charcot’s neurologic triad, which consists of scanning speech, internuclear ophthalmoplegia, and nystagmus, with symptoms characteristically worsening after a hot shower. However, a wide range of clinical manifestations may be seen including optic neuritis, urinary and fecal incontinence, motor abnormalities such as trembling and paresis, sensory changes ranging from pain to numbness and depression, all of which typically, though not exclusively, display a relapsing pattern. Diagnosis of MS includes the gold standard presence of simultaneous periventricular plaques on MRI, as well as elevated CSF immunoglobulins, most commonly IgG, and finally identification of oligoclonal IgG bands on immunoelectrophoresis.

Question 1
A 30-year-old woman from Minneapolis presents to your office complaining of paresthesias, weakness, lack of coordination, and difficulty with gait. Her symptoms are worse after a hot shower. Examination of the cerebral spinal fluid shows oligoclonal bands of immunoglobulin G (IgG). The most likely diagnosis is  
A
Multiple sclerosis
B
Huntington’s disease
C
Parkinson’s disease
D
Neurofibromatosis
E
Amyotrophic lateral sclerosis (ALS)
Question 1 Explanation: 
Multiple sclerosis is a slowly demyelinating disease that affects the CNS. It is characterized by remissions and exacerbations that are separated in time and involve different areas of the CNS. A second form identified is progressive. The cause is unknown but may be related to a combination of genetic factors and perhaps infection with a slow or latent virus. Women are affected more than men (2: 1), and there appears to be a geographic predominance, with those in the northern United States affected more than those in the southern United States. The onset is usually between 20 and 40 years of age, and the geographic factor is present even if the individual relocates to a tropical climate (as long as they spent their first 15 years in the north). The pathology involves multiple plaques of demyelination that are found throughout the CNS. Symptoms include paresthesias, including Lhermitte’s symptom (sensation of a momentary electrical current or shock when the neck is flexed), weakness, loss of coordination, or visual disturbances (monocular visual loss), initially followed by emotional lability, gait disturbances, and spasticity in more severe cases. Signs include optic neuritis, speech difficulties, cranial-nerve palsies, increased deep tendon reflexes, nystagmus, tremor, urinary incontinence, and impotence. Symptoms increase with exposure to heat. Diagnosis is usually made by the history, appearance of oligoclonal bands of IgG in the CSF, and MRI scans showing plaques of demyelination in the paraventricular white matter. Evoked potential nerve tests may also be abnormal. Treatment is usually supportive; however, steroids and immunosuppressive drugs have been used. Newer medications include interferon β-1b (Betaseron), interferon β-1a (Avonex), and glatiramer acetate (Copaxone), which are interferon-type medications used in the relapsing-remitting forms. In addition, antispasmodic drugs such as baclofen have been used to treat the spasticity.
Question 2
A 27-year-old woman comes to your office for assessment of symptoms including weakness, visual loss, bladder incontinence, sharp shooting pain in the lower back, clumsiness when walking, and sensory loss. These symptoms have occurred during three episodes (different combinations of symptoms each time) approximately 3 months apart, and each episode lasted approximately 3 days. The first episode consisted of weakness, bladder incontinence, and sharp shooting pains in the lower back (in both hip girdles). The second episode consisted of visual loss, clumsiness when walking, and sensory loss. The third episode (last week) consisted of sharp shooting pains in the lower back and sensory loss (bilateral) in the upper extremities. There are four clinical categories of this disease. Which of the following subtypes does the patient presented fit into?    
A
relapsing-remitting
B
secondary progressive
Hint:
In the secondary progressive category, gradual neurologic deterioration occurs with or without superimposed acute relapses in patients who previously had relapsing-remitting MS.
C
primary progressive
Hint:
In primary progressive MS, gradual continuous deterioration occurs from the onset of symptoms.
D
progressive relapsing
Hint:
In progressive relapsing MS, gradual neurologic deterioration occurs from the onset of symptoms but with subsequent superimposed relapses.
E
none of the above
Question 2 Explanation: 
The most common pattern or clinical category of MS is the relapsing-remitting category. In relapsing-remitting MS, episodes of acute worsening are followed by recovery and a stable course between relapses. In the secondary progressive category, gradual neurologic deterioration occurs with or without superimposed acute relapses in patients who previously had relapsing-remitting MS. In primary progressive MS, gradual continuous deterioration occurs from the onset of symptoms. In progressive relapsing MS, gradual neurologic deterioration occurs from the onset of symptoms but with subsequent superimposed relapses. A small fraction of patients have a relatively benign form that never becomes debilitating. The patient described most likely has relapsing-remitting MS.
Question 3
In the previous patient, if you had the opportunity to do only one diagnostic test, which of the following would you choose?
A
computed tomography scan of the head and spinal cord
B
magnetic resonance imaging (MRI) scan of the brain and spinal cord
C
serum vitamin B12 levels
D
Beck depression scale
E
Venereal Disease Research Laboratory test for syphilis
Question 3 Explanation: 
The most sensitive and specific investigation for this disorder is MRI of the brain and spinal cord. The MRI scan will reveal plaque formation (a subsequent stage that results from the loss of the myelin sheath in different parts of the CNS) and spotty and irregular demyelination in the affected areas in a patient with MS.
Question 4
Multiple sclerosis is associated with which of the following?
A
racial predilection: whites more often than African Americans
B
sex predilection: females more often than males
C
high socioeconomic status
D
environmental exposure
E
all of the above
Question 4 Explanation: 
The documented risk factors for MS include the following: white race more likely than African American race; female more likely than male (2: 1); environmental influences (latitude north > south) and suspected but unidentified environmental toxins; genetic (common human leukocyte antigen [HLA] histocompatibility antigen patterns); viral infections are suspected, but none has yet been identified; and high socioeconomic status.
Question 5
Which of the following statements regarding the behavior of the disease described is (are) true?  
A
in 80% to 90% of all cases, the first episode is followed by a cycle of relapses and remissions
Hint:
This is a true statement
B
50% of those with relapsing-remitting cases switch to a progressive course approximately 5 years after the onset of the first symptoms
Hint:
This is a true statement
C
10% of patients have progressive disease from the onset of symptoms
Hint:
This is a true statement
D
up to 10% of patients with this disease have a relatively “benign” course
Hint:
This is a true statement
E
all of the above
Question 5 Explanation: 
All of the following are correct: 80% to 90% of all cases after the first symptom have relapses followed by remissions; 50% of those who have relapsing-remitting cases switch to a progressive course approximately 5 years after the onset of the first symptoms; 10% have progressive disease from the onset; and 10% of patients have clinical courses that are benign. These patients have one or two relapses and then recover or have episodes of mild nondebilitating relapses with long-lasting remissions. These individuals have multifocal plaques at autopsy without evidence of an inflammatory demyelinating reaction. There is a very rare type termed acute multiple sclerosis of the Marburg type with rapid progression of symptoms.
Question 6
The target of this disease process is an attack on which of the following?
A
the neurotransmitter balance in the CNS
B
the oligodendrocytes of the CNS
C
the peripheral nerves in the posterior columns of the spinal cord
D
the cerebral hemispheres
E
the cerebellum
Question 6 Explanation: 
The targeted cells in the MS disease process are the oligodendrocytes of the CNS. These cells fabricate and maintain the myelin sheaths, the material covering the axons that is necessary for the normal conduction of nerve impulses. Destruction of oligodendrocytes occurs in clusters and is accompanied by loss of oligodendrocytes and their myelin sheath appendages with axon sparing (primary demyelination). The cluster destruction of oligodendrocytes– myelin sheaths forms multifocal plaques, the pathologic hallmark of the disease. Most of these plaques are in the white matter.
Question 7
What is (are) the treatment( s) of choice for the disease process described?
A
adrenocorticotropic hormone (ACTH)
B
corticosteroids
C
mitoxantrone (Novantrone)
D
interferon beta
E
natalizumab (Tysabri)
F
all of the above
Question 7 Explanation: 
The treatments of choice for MS are as follows:
  • Corticosteroids are the mainstay for treatment of initial and acute relapses of MS. Although corticosteroid therapy can shorten the duration of a relapse, it is uncertain whether the long-term course of the disease will be altered with their use.
  • ACTH has been replaced by high-dose intravenous methylprednisolone.
  • Interferon beta has been the most common long-term treatment for patients with relapsing-remitting MS. Interferon beta is available in two forms, 1a and 1b. Both types are generally well tolerated by most patients. Influenza-like symptoms are common after each injection, and questions about different responses in different people remain. Therefore, interferon beta doses should be individualized.
  • Newer treatments are becoming more common and are usually administered in special clinics by experts in multiple sclerosis. Glatiramer acetate is an alternative for those who have failed to respond to interferon beta. Glatiramer helps prevent the body’s immune system from attacking nerve fibers. Mitoxantrone slows disability and reduces the relapse rate. Natalizumab reduces the relapse rate.
Question 8
Which of the following statements regarding the use of interferon in this disorder is (are) true?
A
interferon has been shown to reduce the rate of relapse
B
interferon may delay the progression to disability
C
interferon reduces the development of new lesions as seen by MRI
D
interferon delays the increase in volume of lesions as seen by MRI
E
all of the above
Question 8 Explanation: 
Interferon beta-1a and beta-1b are used in the treatment of MS. Both interferons have been shown to reduce the rate of clinical relapse, to reduce the number of new lesions seen on MRI, and to delay the increase in volume of new lesions seen on MRI. Interferon beta-1a also may delay the progression to disability in some patients. Glatiramer acetate, mitoxantrone, and natalizumab also have been shown to reduce the rate of relapse, although much uncertainty remains about their precise use.
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