פרק 35 Chapter 35 Multiple Sclerosis and Other Inflammatory Demyelinating Diseases Flashcards
table 35-1 CLASSIFICATION OF THE INFLAMMATORY DEMYELINATIVE DISEASES
table 35-2
DIAGNOSTIC CRITERIA FOR MS
מה ההבדל הפתולוגי בנגעים דהמיאלינטיבים בגילאים שונים?
pattern 1- inflamatory lesions made up of T cells and macrophages alone
pattern 2- an autoantibody lesion mediated by immunoglobulin and complement
pattern 3- those characterised by apoptosis of oligodendrocytes and abscence of immunoglobulin, complement and remeyelination
pattern 4- oligodendrocyte dystrophy and no remyelination
each case represented only one pattern of pathology.
the last two histopathologic types were considered to represent primary oligodendroglial cell degeneration implicating is that the pathologic charachteristics of the chronic progressive type of MS may differ from those of the typical relapsing type
3,4 מחלה פרוגרסיבית דגנרציה של אוליגודנטרוציטים
ב3 כנראה ריאמלינציה
genetic and genderial impotrance of MS
- the major histocompatability complex class 1 region. accounts for 20-60% of the genetic component of MS susceptibillity, and homozygosity constitutes a high vulnerability genotype.
- there is an equal distribution between males and females with primary progressive MS, unlike relapsing MS which occures with a higher incidence in females. other genetic and enviromental risk factors seem to be the same in PPMS and RRMS
CT, CTA
תיאור שיותר מתאים לשבץ
אינם מוסברים
2 מתאים ל
CADASIL
small T2 hyperintensitis in the external capsule and anterior temporal lobe, while found in multiple sclerosis, should raise the suspicion of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and other small vessel diseases.
מה ההבדל בין סוגי העיוורון של אקוואפורין 4 לעומת mog
עיוורון מתאים לאקוואפורין
דו”צ + פפיליטיס מתאים למוג (אופטיק נויריטיס אנטריורי, מעורבות סימולטנית של שני עצבי הראייה)
MOG הוא ADEM LIKE
עם נגעים ארוכים בעמ”ש וחיבה לקונוס.
אינטרנליזציה של הרצפטור ספינגוזין 1 פוספאט = ספינגולימוד
1-ריטוקסימאב b-cell depletion
2- הפחתת הביטוי של מולקולות קו-סטימולציה- copaxone interferon
3- depletion of cd4+ lymphocytes- copaxone & interferon
4. עיכוב קשירה של לימפוציטים למולקולות אדהזיה על פני האנדותל- נטליזומב (טיסברי)
סיכון לפיתוח
JCV תחת טיפול נטליזומאב
פרטים נוספים על טרשת נפוצה
איזה תסמין לא אופייני במטופלת בת 55 , אשר מאובחנת כסובלת מטרשת נפוצה במשך 20 שנה,
ומטופלת ב-
interferon-beta ?
א. תסמונת פסאודובולבארית
ב. דמנציה עם הפרעה שפתית משמעותית.
ג. הפרעה בשליטה על השתן.
ד. עייפות כרונית
דמנציה עם הפרעה שפתית משמעותית לא מתאימה לטרשת נפוצה
בת 25 המאובחנת עם טרשת נפוצה מגיעה למרפאה נוירולוגית להתייעץ לפני תכנון הריון.
מה המידע על התלקחות התקפי טרשת נפוצה סביב הריון?
א. ירידה בסיכון להתקפים ככל שמתקדם ההריון
ב. ירידה בסיכון להתקפים בחודשים הראשונים לאחר הלידה
ג. עלייה בסיכון להתקפים בשבועות שלפני הכניסה להריון
ד. העליה הגבוהה ביותר בסיכון להתקפים היא בטרימסטר השני
ירידה בסיכון להתקפים ככל שמתקדם ההריון
אחר איזה ת”ל מאוחרת של התרופה
alemtuzumab
צריך לעקוב
1. קרדיוטוקסיות
2. ITP
3. ממאירות כלשהי
ITP
Another monoclonal antibody that has been introduced for the treatment of MS is alemtuzumab, which targets CD-52 antigen expressed on T and B lymphocytes, thereby reducing the number of circulating B cells and for a longer period, T cells. It is used in an annual cycle of intravenous administration for 5 consecutive days. A randomized trial conducted over 36 months comparing the drug to interferon-β-1a found it to be superior in preventing relapses and in reducing the accumulation of disability (CAMMS223 Trial Investigators). A series of subsequent trials have confirmed its effectiveness in comparison to interferon (Cohen et al). The drug can produce idiopathic thrombocytopenic purpura and autoimmune thyroiditis that results in either hyper- or hypothyroidism
alemtuzumab- antigen cd52
- sphngosine 1 phosphate 1 (S1P1) receptor analouge- fingolimod (gilenia)
- anti cd20- ocrevus (ocrelizumab)
- dihydro-orotate dehydrogenase inhibitor- tecfidera (dimethyl fumarate)
מטופלתצעירהעםטרשת,הולכתהרבהמאודלשירותים (תיאור של תכיפות ולא אצירת שתן).איזותרופהתיתן?
א .bethanecole
ב.omnic
ג.nitrofurantoin
ד.oxybutinin
oxybutinin (ditropan)
- Disorders of bladder function may raise serious problems in management. Where the major disorder is one of urinary retention, bethanechol chloride is helpful. In this situation, monitoring and reducing the residual urinary volume are important means of preventing infection; volumes up to 100 mL are generally well tolerated. Some patients with severe bladder dysfunction, particularly those with urinary retention, benefit from intermittent catheterization, which they can learn to do themselves and which lessens the constant risk of infection from an indwelling catheter.
- More often the problem is one of urinary urgency and frequency (spastic bladder), in which case the use of propantheline (Pro-Banthine) or oxybutynin (Ditropan) may serve to relax the detrusor muscle (Chap. 25). These drugs are best used intermittently.
- Severe constipation is best managed with stool softeners and properly spaced enemas.
- Sexual dysfunction has been treated with sildenafil and similar drugs.
- When pain is a prominent symptom, its management follows the general principles of pain management outlined in Chap. 7.
- Carbamazepine or gabapentin are often helpful to reduce paroxysmal symptoms in MS, particularly truncal extensor spasms.
- Fatigue, a common complaint of MS patients, particularly in relation to acute attacks, responds to some extent to amantadine (100 mg morning and noon), modafinil (200 to 400 mg/d), pemoline (20 to 75 mg each morning), methylphenidate, or dextroamphetamine.
- For depression associated with the disease, there does not seem to be any superior antidepressant.
- donepezil has not been found to be helpful for cognitive problems.
- Oral baclofen or tizanidine are often used to reduce spasticity in patients with MS but the dose must be limited to avoid excessive sedation. In patients with severe spastic paralysis and painful flexor spasms of the legs, local injection of botulinum toxin can be very effective. In these cases, intrathecal infusion of baclofen through an indwelling catheter and implanted pump can also be considered. An alternative to oral baclofen is tizanidine.
- The severe and disabling tremor that is brought out by the slightest movement of the limbs, if unilateral, can be managed surgically by ventrolateral thalamotomy or implanted stimulator of the type used for the treatment of Parkinson disease. Most surgical series report that about two-thirds of patients achieve a satisfactory reduction in their intention tremor (Critchley and Richardson; Geny et al). In the experience of others, the results have not been quite this reliable. In the series reported by Hooper and Whittle, only 3 of 10 MS patients who underwent thalamotomy for a severe tremor had sustained improvement. Hallett and colleagues have reported that severe postural tremor of this type can be improved by the administration of isoniazid (300 mg daily, increased by weekly increments of 300 mg to a dose of 1,200 mg daily) in combination with 100 mg of pyridoxine daily. How isoniazid produces its beneficial effects is not known, and careful monitoring of liver tests is required. Variable success may also be achieved with carbamazepine or clonazepam.
- There are no valid studies to substantiate claims that have been made for the value of synthetic polypeptides other than copolymer, for hyperbaric oxygen, low-fat and gluten-free diets, or linoleate supplementation.
איזה טיפול מהבאים מקובל לטיפול ב-
fatigue בטרשת נפוצה:
א. Dalfampridine
ב. Modafinil
ג. methamphetamine
ד. Bupropion
Modafinil
* Fatigue, a common complaint of MS patients, particularly in relation to acute attacks, responds to some extent to amantadine (100 mg morning and noon), modafinil (200 to 400 mg/d), pemoline (20 to 75 mg each morning), methylphenidate, or dextroamphetamine.
* Disorders of bladder function may raise serious problems in management. Where the major disorder is one of urinary retention, bethanechol chloride is helpful. In this situation, monitoring and reducing the residual urinary volume are important means of preventing infection; volumes up to 100 mL are generally well tolerated. Some patients with severe bladder dysfunction, particularly those with urinary retention, benefit from intermittent catheterization, which they can learn to do themselves and which lessens the constant risk of infection from an indwelling catheter.
* More often the problem is one of urinary urgency and frequency (spastic bladder), in which case the use of propantheline (Pro-Banthine) or oxybutynin (Ditropan) may serve to relax the detrusor muscle (Chap. 25). These drugs are best used intermittently.
* Severe constipation is best managed with stool softeners and properly spaced enemas.
* Sexual dysfunction has been treated with sildenafil and similar drugs.
* When pain is a prominent symptom, its management follows the general principles of pain management outlined in Chap. 7.
* Carbamazepine or gabapentin are often helpful to reduce paroxysmal symptoms in MS, particularly truncal extensor spasms.
* For depression associated with the disease, there does not seem to be any superior antidepressant.
* donepezil has not been found to be helpful for cognitive problems.
* Oral baclofen or tizanidine are often used to reduce spasticity in patients with MS but the dose must be limited to avoid excessive sedation. In patients with severe spastic paralysis and painful flexor spasms of the legs, local injection of botulinum toxin can be very effective. In these cases, intrathecal infusion of baclofen through an indwelling catheter and implanted pump can also be considered. An alternative to oral baclofen is tizanidine.
* The severe and disabling tremor that is brought out by the slightest movement of the limbs, if unilateral, can be managed surgically by ventrolateral thalamotomy or implanted stimulator of the type used for the treatment of Parkinson disease. Most surgical series report that about two-thirds of patients achieve a satisfactory reduction in their intention tremor (Critchley and Richardson; Geny et al). In the experience of others, the results have not been quite this reliable. In the series reported by Hooper and Whittle, only 3 of 10 MS patients who underwent thalamotomy for a severe tremor had sustained improvement. Hallett and colleagues have reported that severe postural tremor of this type can be improved by the administration of isoniazid (300 mg daily, increased by weekly increments of 300 mg to a dose of 1,200 mg daily) in combination with 100 mg of pyridoxine daily. How isoniazid produces its beneficial effects is not known, and careful monitoring of liver tests is required. Variable success may also be achieved with carbamazepine or clonazepam.
* There are no valid studies to substantiate claims that have been made for the value of synthetic polypeptides other than copolymer, for hyperbaric oxygen, low-fat and gluten-free diets, or linoleate supplementation.
79איך נטפל בספאזם דיסטוני של הידיים של חולת טרשת - מתוארת חולה בין היתר עם תסמינים ספינליים תורקליים?
1. בקלוסל
2. טגרטול
3. קלונקס
טגרטול!
Paroxysmal attacks of neurologic deficit, lasting a few seconds or minutes and sometimes recurring many times daily, are a relatively infrequent but well-recognized feature of MS ,Usually the attacks occur during the course of relapsing and remitting phase of the illness, rarely as an initial manifestation. These clinical phenomena are referable to any part of the CNS but tend to be stereotyped in an individual patient. The most common phenomena are dysarthria and ataxia, paroxysmal pain and dysesthesia in a limb, flashing lights, paroxysmal itching, or tonic “seizures,” taking the form of flexion (dystonic) spasm of the hand, wrist, and elbow with extension of the lower limb. The paroxysmal symptoms, particularly the tonic spasms, may be triggered by sensory stimuli or can be elicited by hyperventilation. On a few occasions we have seen dystonic hand and arm spasms as the first symptoms; an acute plaque was detected in the opposite internal capsule. In advanced cases, the spasms may involve all 4 limbs and even a degree of opisthotonos. The cause of paroxysmal phenomena is uncertain. They have been attributed by Halliday and McDonald to ephaptic transmission (“cross-talk”) between adjacent demyelinated axons within a lesion.
Carbamazepine is usually effective in controlling such spontaneous attacks, and acetazolamide blocks the painful tonic spasms that are elicited by hyperventilation.
קודם לבדוק
PCR JCV
ורק אחר כך נשקול טיפול בסטרואידים…
PML Characterized by widespread demyelinating lesions, mainly of the cerebral hemispheres but sometimes of the brainstem and cerebellum, and, rarely, of the spinal cord. The lesions vary greatly in size and severity-from microscopic foci of demyelination to massive multifocal zones of destruction of both myelin and axons involving large parts of a cerebral or cerebellar hemisphere. The abnormalities of the glia cells are distinctive. Many of the reactive astrocytes in the lesions are gigantic and contain deformed and bizarre-shaped nuclei and mitotic figures, changes that are seen otherwise only in malignant glial tumors. Also, at the periphery of the lesions, the nuclei of oligodendrocytes are greatly enlarged and contain abnormal inclusions. Many of these cells are destroyed, accounting for the demyelination. Vascular changes are lacking, and inflammatory changes are present but usually insignificant, except in a small number of interesting cases
in which immune reconstitution by retroviral drugs for AIDS allows the emergence of intense inflammation.
גילניה
- Interferon: Flu like, malaise, headache, depression, may develop antibodies
- (glatiramer acetate) Copaxone: MBP analog subcut. Flushing, dyspnea, chest tightness, paplitations, anxiety.
- Tysabri natalizumnb: MAB Alpha-integrin agonist – inhibits lymphocyte migration. PML – JC virus, monitor LFTs
- Lemtrada almatizumab: ITP, autoimmune thyroiditis
ORALS:
- Gilenya / fingolimod: HSV VZV, bradycrdia, AV block, lymphopenia, adenopathy, macular edema, raised LFTs
- Aubagio\teriflunomide : Hair Loss, N&V, diarrhoea, LFTs
- Tecfidera\dimethyl fumarate: abdo pain, N&V, skin rash, flushing, lymphocytopenia, raised LFT, PML reported as well.
באיזותרופהתבדוקנוגדניםלהרפסלפניהתחלתהטיפול?
א.גליראמראצטט
ב.פינגולימוד
ג. אובג’יו
ד.נטליזומב
פינגולימוד (גילניה)
- Interferon: Flu like, malaise, headache, depression, may develop antibodies
- (glatiramer acetate) Copaxone: MBP analog subcut. Flushing, dyspnea, chest tightness, paplitations, anxiety.
- Tysabri natalizumnb: MAB Alpha-integrin agonist – inhibits lymphocyte migration. PML – JC virus, monitor LFTs
- Lemtrada almatizumab: ITP, autoimmune thyroiditis
ORALS:
- Gilenya / fingolimod: HSV VZV, bradycrdia, AV block, lymphopenia, adenopathy, macular edema, raised LFTs
- Aubagio\teriflunomide : Hair Loss, N&V, diarrhoea, LFTs
- Tecfidera\dimethyl fumarate: abdo pain, N&V, skin rash, flushing, lymphocytopenia, raised LFT, PML reported as well.
בקלופן (לציין לא מופיע בספר מהדורה 11… אבל למקרה שהכותבים משחזרים :P)
-
Baclofen is at least as effective as diazepam in reducing spasticity and causes less sedation. In addition, baclofen does not
reduce overall muscle strength as much as dantrolene. It is rapidly and completely absorbed after oral administration and has a plasma half-life of 3–4 hours. Dosage is started at 15 mg twice daily, increasing as tolerated to 100 mg daily. Adverse effects of this drug include drowsiness; however, patients become tolerant to the sedative effect with chronic administration. Increased seizure activity has been reported in epileptic patients. Therefore, withdrawal from baclofen must be done very slowly. - Tizanidine causes markedly less muscle weakness but produces a different spectrum of adverse effects, including drowsiness, hypotension, dizziness, dry mouth, asthenia, and hepatotoxicity.
- Dantrolene – muscle weakness
נטליזומב. אמנם עושה גם חרדה קצת אבל כנראה בכמות פחותה ביחס לאינטרפרון…
-
Beta interferon – develop Antibodies – reduce efficacy.
SE include flu like symptoms, sweating and malaise. Tendency to depression
Increase in LFT. - Glatarmer – copaxon - mimics action of MBP – Subcut daily. No Abs develop against it. SE: flushing, chest tightness, dyspnea, Palpitations, anxiety. inection site reactions mild.
- Natalizumab – Tysabri - once monthly inections, PML – check for JC virus side effects include infusion-related symptoms (headache, flushing, erythema, nausea, and dizziness), fatigue, allergic reactions, -anxiety-, infections (mainly urinary tract infection and pneumonia), pharyngitis, sinus congestion, and peripheral edema.
-
Alemtuzumab – Lemtrada - The drug can produce idiopathic thrombocytopenic purpura and autoimmune thyroiditis that results in either hyper- or hypothyroidism.
The main side effects of alemtuzumab were infusion reactions, infections, and autoimmune disorders. Infusion reactions occurred in approximately 90 percent of patients and were characterized by headache, rash, nausea, and fever. Infections, though generally not severe, were observed in two-thirds or more of the patients treated with alemtuzumab. Herpes viral infections occurred in 16 to 18 percent, leading to a change in the protocol of the in-progress CARE-MS trials with the addition of prophylactic acyclovir treatment during alemtuzumab infusion and for 28 days after infusion.
Oral:
* Dimethylfumarate - Aubagio : SE – may decrease lymphocyte count, elevation of LFTs, ALP, bilirubin, PML reported in 1 patient
- Teriflunomide: Tecfidera: Diarrhoea, nasuea, hair thining, elevated ALTs and heatoxoticity.
- Fingolimod – Gylenia – HSV, VZV infections, MS rebound, PML rarely, cryptococcal infection, cardiac arrhythmias and blocks. Contraindicated in MI, Unstable angina, stroke, TIA, HF.
- mitoxantrone, a drug with broad immunosuppressant and cytotoxic activity, at one time attracted interest but was limited by cardiotoxicity
מונע יציאת לימפוציטים מבלוטות הלימפה
One immunosuppressive drug that interferes with egress of lymphocytes from lymph nodes, fingolimod, has had a short-term effect on MRI lesion burden and relapse rate that is comparable or slightly superior to injectable agents The drug stands out because it is administered orally, once daily, and ostensibly has tolerable side effects. It causes a lymphopenia by restricting lymphocytes to the lymph nodes and causes adenopathy.
Discontinuation of the drug is sometimes required because of extremes of bradycardia or atrioventricular block, macular edema, herpes infections and elevations in liver function tests, the last of these, in approximately 10 percent of patients.
.איזהממצאהדמייתיאופיינילנגעים של טרשת נפוצה?
א. oval radial lesions periventricular
ב. punctuate lesions
ג. heterogenousely enhancing cortical lesions
oval radial lesions periventricular
התקדמות המחלה בחמש שנים ראשונות.
have identified a number of features of the early clinical course that were predictive, in a general way, of the outcome of the illness. Perhaps not surprisingly, they found that a high degree of disability, as measured by the Kurtzke Disability Status Scale, was reached earlier in patients with a higher number of attacks, a shorter first inter-attack interval, and a shorter time to reach a state of moderate disability. Kurtzke had earlier reported that the feature most predictive of long-term disability was the degree of disability at 5 years from the first symptom.
למי מהמטופלים הבאים הסיכוי ללקות בטרשת נפוצה זהה ביחס לאוכלוסייה הכללית בישראל?:
א. בן 31 לאם שסובלת מטרשת נפוצה .
ב. בת 25 שנמצאה נשאית של locus DR . על כרומוזום 6
ג. בת 30 עם עבר של optic neuritis . בגיל 22
. ד. בן 26 אשר עלה לישראל מדנמרק בגיל 8
בן 26 אשר עלה לישראל מדנמרק בגיל 8.
Several studies indicate that persons who migrate from a high-risk to a low-risk zone carry with them at least part of the risk of their country of origin and genetic makeup, that in persons who had immigrated before the age of 15, the risk was similar to that of native-born
ADEM
the CSF shows a slight increase in lymphocytes and protein content but these are variable.
בן25,10ימיםלאחרשפעת,התפתחחום38,כאביראש,קישיוןעורףקל,ספירתדםתקינה,החזריםגידייםמוגבריםברגלייםובבינסקידוצ,מההאבחנההסבירה?
1. מנינגיטיסחיידקי.
2. דימוםתתעכבישי.
3. ADEM
4. מנינגיטיסכימי
ADEM
acute disseminated encephalomyelitis
steroids IV
- Treatment of optic neuritis (see Chap. 12) The Optic Neuritis Treatment Trial, reported by Beck and colleagues, cautioned against the use of oral prednisone in the treatment of acute optic neuritis (see also Lessell). In this study, it was found that the use of intravenous methylprednisolone followed by oral prednisone did, indeed, speed the recovery from visual loss, although at 6 months there was little difference in visual outcome between patients treated in this way and those treated with placebo. They reported that treatment with oral prednisone alone slightly increased the risk of new episodes of optic neuritis. In a subsequent randomized trial conducted by Sellebjerg and colleagues, it was found that methylprednisolone 500 mg orally for 5 days had a beneficial effect on visual function at 1 and 3 weeks. However, at 8 weeks, no effect could be shown (compared with the placebo-treated group), nor was there an effect on the subsequent relapse rate. The putative deleterious effects of oral glucocorticoids on relapse of optic neuritis have been disputed and most clinicians consider them equivalent to intravenous administration for this disorder.
גיל צעיר בהופעה.
More than one-half of adult patients who present with optic neuritis will eventually develop other signs of MS. The prospective investigation of Rizzo and Lessell showed that MS developed in 74 percent of women and 34 percent of men by the fifteenth year after onset of visual loss; similar results were reported by the Optic Neuritis Study Group (Beck et al, 2003). The risk is much lower if the initial attack of optic neuritis occurs in childhood (26 percent developed after 40 years of followup
[Lucchinetti et al 1997]); this suggests that some instances of the childhood disease may be of a different type, perhaps viral or postinfectious.
NMO
This disease is characterized by a simultaneous or successive and usually severe involvement of optic nerves and spinal cord. Its principal features are the acute to subacute onset of blindness in one or both eyes, preceded or followed within days or weeks by a severe transverse or ascending myelitis
ANTI AQP4 Abs identifies.
Most cases of neuromyelitis optica stand apart
from MS by virtue of distinctive clinical and pathologic Features, mainly, a failure to develop cerebral demyelinating lesions typical of MS even after years of illness.
the absence of oligoclonal bands in the CSF; a tendency to CSF pleocytosis more so than in MS, and the necrotizing and cavitary nature of the spinal cord lesion, affecting white and gray matter alike with prominent thickening of vessels but with minimal inflammatory infiltrates. It is also quite unusual for MS to involve several contiguous longitudinal segments of the spinal cord, and this is a frequent finding in Devic disease
The spinal cord lesions in cases of neuromyelitis optica are often necrotizing, centrally located in the cord, and occupying several contiguous vertebral segments, leading eventually to cavitation. As would be expected, the clinical effects are more likely to be permanent than those of typical demyelination
מטופלת עם הופעה אקוטית של חולשת רגליים והפרעת סוגרים וההדמיה הבאה איזו בדיקה תתן אבחנה?
1. OCB
2. Anti Aquaporin 4
anti aquaporin 4
TUMAFECTIVE
The topography of the lesions is noteworthy. A periventricular localization is characteristic, but only where subependymal veins line the ventricles (mainly adjacent to the bodies and atria of the lateral ventricles). Other favored structures are the optic nerves and chiasm (but rarely the optic tracts) and the spinal cord, where pial veins lie next to or within the white matter. The lesions are distributed randomly throughout the brainstem, spinal cord, and cerebellar peduncles without reference to particular systems of fibers, but always confined predominantly to the white matter. In the cerebral cortex and central nuclear and spinal structures, the acute lesions destroy myelin sheaths but leave the nerve cells mostly intact. Severe and more chronic lesions, however, may destroy axons and neurons in the affected region, but the dominant lesion is still demyelinating
MRI בעוד 3 חודשים
diagnosing MS in a patiern with: one attack with objective evidence on examination of 2 or more lesions– dssmnaton over time: simulataneous enhancing and non enhancing lesions in the above locations, or interval development of new T2 hyperintense lesions.
מדובר בADEM
הטיפול הוא סטרואידים במינון גבוה
סרקואידוזיס
These oligoclonal bands in the CSF (after comparing to monoclonal proteins that may be present in the serum) are detected in more than 90 percent of cases of MS in some populations, but in a lower proportion of patients in Asian countries. Furthermore, oligoclonal bands are not specific for MS; such bands also appear in the CSF of patients with disorders such as syphilis, Lyme, and subacute sclerosing panencephalitis. The presence of bands in a first attack of MS is predictive of a chronic relapsing course,
Oligoclonal bands are usually reported as being present if there is more than 1 band; the meaning of a single band is not clear, and we have treated this result as a negative test. As will be pointed out, the conditions of necrotic myelopathy and Devic disease generally lack oligoclonal bands.
Figure 35-1.
MRI in multiple sclerosis. Upper left, axial T2FLAIR
image showing multiple discrete periventricular hyperintense plaques, as well as two subcortical
plaques in the right frontal and parietal lobes. Upper right, coronal T1post
gadolinium image showing abnormal enhancement of the right optic nerve
in a case of acute optic neuritis (arrow). Lower left, sagittal T2FLAIR
image showing two hyperintense plaques emanating radially from the body of the
corpus callosum (“Dawson fingers”). Lower right, sagittal T2 MRI showing multiple discrete hyperintense plaques within the cervical spinal cord. The
lesion at C3 is acute with accompanying expansion of the cord. The lesion at the T1 level of the cord is chronic and shows cord atrophy.
Figure 35-2.
Left Axial T2FLAIR
image of a tumefactive MS lesion in the left temporal lobe. Right T1post
gadolinium showing an “open ring” of abnormal contrast
enhancement, a common imaging feature of acute demyelinating plaques that is less typical of tumors or abscesses.
Figure 35-3.
MRI of the spinal cord in neuromyelitis optica. Sagittal T2 image showing a hyperintense, longitudinally extensive, confluent cervicothoracic
lesion.
Figure 35-3.
MRI of the spinal cord in neuromyelitis optica. Sagittal T2 image showing a hyperintense, longitudinally extensive, confluent cervicothoracic
lesion.
Figure 35-4.
Acute (postinfectious) disseminated encephalomyelitis (ADEM). Axial T2FLAIR
images showing left inferior frontal (left) and right anterior temporal
(right) edematous lesions.
Figure 35-5.
Acute necrotizing hemorrhagic leukoencephalitis. Axial T2FLAIR
MRI shows extensive abnormal hyperintensity throughout the hemispheric white
matter as well as within the deep gray nuclei. Additional signal abnormality in the cortical sulci is due to subarachnoid hemorrhage.
Figure 35-5.
Acute necrotizing hemorrhagic leukoencephalitis. Axial T2FLAIR
MRI shows extensive abnormal hyperintensity throughout the hemispheric white
matter as well as within the deep gray nuclei. Additional signal abnormality in the cortical sulci is due to subarachnoid hemorrhage.
Figure 35-6.
Abnormal T2 hyperintensity in the splenium of the corpus callosum in a patient with graftversushost
disease 2 years following allogenic bone marrow
transplantation.
