7 cases, take you to a comprehensive understanding of multiple sclerosis|Clinical practice

Introduction Multiple sclerosis (MS) is a chronic, immune-mediated central nervous system (CNS) disease whose pathological features are inflammation, demyelination, and eventual loss of axons.

The diagnosis of MS is based on the concept of multiple occurrences of disease in space and time.

Although MS can be diagnosed based on its clinical cause, magnetic resonance imaging (MRI) can be used to support or confirm the diagnosis, and to track disease progression.

This article summarizes 7 cases to test everyone's understanding of MS, let's try it together.

Yimaitong compiles and organizes, please do not reprint without authorization.

Question 1 A 35-year-old female patient has an unbalanced gait, which has gradually worsened in the past few days.

The brain MRI examination is shown in the picture, and the doctor considers MS.

Regarding the location of MS lesions, which of the following are typical? A.
Cortical lesions B.
Periventricular lesions C.
Corpus callosum lesions D.
Brain stem and cerebellar lesions E.
All of the above included Slide to view the answer↓↓↓↓↓↓↓↓【Answer and analysis】E
As shown in the figure below, typical sites of MS lesions include the juxtacortex/cortex (yellow arrow) and periventricular white matter (red arrow), as well as the white matter of the brain stem, cerebellum, and spinal cord.

Periventricular lesions are usually located at the corpus callosum-septal interface and perpendicular to the long axis of the corpus callosum.

Although it was previously believed that the cortical gray matter was not affected, there is pathological evidence of gray matter involvement in MS, especially when using advanced imaging sequences, cortical lesions can be seen.

Question 2 As shown in the figure below, MRI shows that the patient's right occipital radiation crown has asymptomatic enhanced lesions.
Before the patient starts to receive disease modification therapy (DMT), which of the following should be performed? A.
Wait for the second clinical event to occur B.
Re-check MRI after 3 months to assess whether there are new lesions.
C.
Perform lumbar puncture to assess the oligoclonal band (OCB) and immunoglobulin G (IgG) index.
D.
Check whether the patient’s symptoms have been intravenously injected methyl in a short period of time After prednisolone cannot improve E.
None of the above, there is no need to delay DMT treatment.
Slide to view the answer↓↓↓↓↓↓↓↓↓【Answer and analysis】E.

Although the patient had only one clinical seizure, it met the diagnostic criteria for spatially frequent (at least one periventricular lesion and one near-cortical lesion) and temporally frequent diagnostic criteria (in this case, non-enhanced lesions and enhanced lesions).

DMT should be started as early as possible to prevent the second clinical attack.

The American Academy of Neurology (AAN) also recommended in its guidelines issued in April 2018 that early treatment should be considered for such patients.

 Clinical recurrence may be completely relieved or leave residual neurological deficits.

Over time, recurrent disease may transform into a course of secondary progression, which is characterized by a gradual increase in disability without acute recurrence.

The most typical course of MS (as shown in the figure below) starts in the recurrence phase of the disease, followed by the secondary advanced phase.

Whether DMT can prevent the occurrence of secondary progression is a controversial issue.

Question 3 A young woman has a tingling sensation from the spine to the arm when her cervical spine is flexed.
The MRI of her cervical spine is shown in the figure below.

MRI showed corpus callosum and periventricular lesions.

The neurologist suspected that the patient had a high risk of clinically isolated syndrome (CIS) converting to MS.
In order to rule out other possible causes, a lumbar puncture and cerebrospinal fluid (CSF) analysis were performed. Which of the following CSF analysis results does not meet the diagnosis of MS? A.
White blood cell (WBC) count is 200/uLB.
IgG index is elevated C.
OCB is present in the cerebrospinal fluid, but not in the serum D.
The protein is slightly higher.
Slide to see the answer↓ ↓↓↓↓↓↓↓↓【Answer and Analysis】A.

Cervical MRI showed a single T2 super-strong lesion at the C4 level, accompanied by enhancement.

The differential diagnosis of acute myelitis includes MS and other primary demyelinating diseases, such as neuromyelitis optica (NMO) and acute diffuse encephalomyelitis (ADEM), as well as infectious and rheumatic diseases.

If the cause of myelitis is not found, it can be diagnosed as idiopathic myelitis.

The cerebrospinal fluid white blood cell count is normal to the minimum (≤20/uL) consistent with the diagnosis of MS.

On the contrary, a large number of hyperplasia in the range of 200/uL instead suggests infection.

The normal to minimal increase in protein levels and the increase in lgG index in CSF are also consistent with MS.

The presence of OCB in CSF but not in serum is a sensitive but non-specific marker of MS.

Question 4 A 25-year-old woman with no previous medical history complained of onset of acute left-eye gaze and painless diplopia.

The patient's gaze was normal when staring to the right and edge, and there were no other neurological symptoms.

Eye movement examination showed that the patient's main vision and rightward vision were normal.

When staring to the left, the patient's right eye cannot be adducted, and there is abduction nystagmus (as shown in the picture).

Which part of the lesion is the cause of the patient’s symptoms? A.
Corpus callosum pressure B.
Red nucleus C.
Left cranium D.
Right medial longitudinal fascia E.
Right third cranial nucleus slide to see the answer↓↓↓↓↓↓↓↓↓ [Answer and analysis] D.

Intranuclear ophthalmoplegia with impaired ipsilateral adduction and contralateral abduction nystagmus are common symptoms in MS patients.

This is usually caused by the demyelination of the medial longitudinal tract, which is the white matter tract connecting the third cranial nerve nucleus and the contralateral sixth cranial nerve nucleus, and mediates the lateral conjugate gaze.

This picture shows an acutely enhanced medial longitudinal fascicle (red arrow).

Question 5 The MRI of the patient's head showed some other characteristic MS lesions, so it was decided to initiate DMT.

After the patient started treatment, no further neurological diseases occurred.

When the head MRI was performed again one year later, the following figure shows the contrast T1 sequence.

Which of the following is the most appropriate next treatment? A.
Continue the current treatment B.
Start intravenous methylprednisolone C.
Change DMTD.
Check for urinary tract infection Slide to view the answer↓↓↓↓↓↓↓↓↓【Answer and analysis】C.

This patient has multiple enhancement lesions (red arrows), and despite treatment, disease activity is still progressing.

Obviously, the current treatment method is not optimal, so there is no need to continue treatment.

In addition, there is no evidence to support the use of steroids to treat asymptomatic enhancement lesions.

Question 6 A 60-year-old man has been suffering from MS for 30 years and has never taken any medications.

Since the first relapse 15 years ago, the patient's function has continued to decline; for the past 10 years, he has been in a wheelchair.

Which of the following MRI features suggest the long-term presence or degenerative changes of MS? A.
Whole brain atrophy B.
T1-weighted low signal C.
Gadolinium enhancement D.
A and B Slide to see the answer↓↓↓↓↓↓↓↓↓【 Answer and analysis] D.

In many neurodegenerative processes, including long-lived MS, atrophy of the whole brain can be seen.

As shown in the figure below, the persistent low signal on the T1 sequence-the so-called black hole (red arrow)-indicates loss of axons and destruction of neuronal tissue.

When the blood-brain barrier is broken, gadolinium enhancement occurs during the active inflammatory process.

Problem A 741-year-old man has a history of MS recurrence and is currently progressing to acute left lower quadrant hemianopia and left numbness.

MRI imaging of the brain (as shown in the picture).

Which of the following is the most appropriate next treatment? A.
A course of intravenous steroid therapy, followed by a review of imaging B.
A biopsy of the lesion as soon as possible to obtain a tissue diagnosis C.
Change of DMTD.
Review of MRI within 1 year, slide to view the answer↓↓↓↓↓↓↓↓↓【Answer And analysis] A.

MRI of the head showed that the right parieto-occipital lesions were significantly enhanced and accompanied by edema.

MS lesions occasionally swell and are difficult to distinguish from true malignant tumors.

Ideally, biopsy of demyelinating lesions should be avoided as much as possible, as it may lead to permanent neurological deficits.

In this particular case, the best next step is to inject steroids intravenously and pay close attention to the MRI results to ensure the enhancement and the resolution of the associated edema.

If the lesion does prove to be a swelling lesion of MS, it may be reasonable to change the DMT; however, assessing the malignancy will be the first priority.

 After intravenous steroids, the patient's symptoms have improved significantly.

After 6 weeks, the MRI was rechecked, and it was found that the lesion was reduced and the enhancement effect improved (as shown in the picture).

If the lesion is a malignant brain tumor, this will not happen.

Yimaitong compiled from: Multiple Sclerosis and Other Enemies of Myelin.
Medscape.
June 18, 2019.