פרק 30 Chapter 30: Intracranial Neoplasms and Paraneoplastic Disorders Flashcards
מה נכון לגבי
paraneoplastic opsoclonus myoclonus ataxia?
א. התסמונת מופיעה בדרך כלל במבוגרים
ב. בפתולוגיה נראה הרס נרחב של תאי פורקינייה
ג. טיפול בגידול הממאיר עשוי להביא לשיפור בסימפטומים הנוירולוגיים
ד. מרבית המקרים במבוגרים הם של נשים עם גידול בשחלה
ה. טיפול בסטרואידים אינו אפקטיבי
תשובה ג. טיפול בגידול הממאיר עשוי להביא לשיפור בסימפטומים הנוירולוגים
Most commonly in children with Neuroblastoma – but occures in adults as well – in relation to breast Ca and SCLC (anti ri- ANNA 2= breast. SCLC= anti glycine receptor ab ).
Pathology reveals mild loss of purkinje cell (not as significant as in cerebellar degeneration), inferior olives and brainstem
steroid and ACTH therapy may be affective when treating neuroblastoma
“search the tumour and resect it if possible”
-Table 30-6
THE MAIN PARANEOPLASTIC DISORDERS AND THEIR ASSOCIATED AUTOANTIBODIES
aIn many cases, a particular autoantibody is associated with a specific tumor type rather than with the clinical syndrome (e.g., small cell lung cancer and polyneuropathy with ANNA 1, breast cancer with anti-Purkinje cell antibody, testicular tumors with anti-Ma). Clinical syndromes similar to each of these may occur with non–small cell lung cancer and lymphoma, most often in the absence of detectable antibodies.
מהו הגידול הראשוני האינטרה קרניאלי השכיח ביותר?
א. גידולים של ההיפופיזה
ב. גליומות
ג. מנינגיומה
ד. נוירינומה
מנינגיומה
Table 30-1
FREQUENCY OF PRIMARY BRAIN AND CENTRAL NERVOUS
SYSTEM TUMORS TAKEN FROM CBTRUS STATISTICAL
REPORT, 2008–2012 (N = 356,858)
אפנדימומה
.שלושה שבועות לאחר לידהמטופלת מתלוננת על כאב ראש פתאומי, לא נעלם בשינה, ללא מרכיב תנוחתי . מההאתיולוגיה?
1. PRES
2. PITUITARY APOPLEXY
3. דימום אפידורלי
4. SAH
pituitary Apoplexy
ההתלבטות פה היא בין
PRES, APOPLEXY & SAH
מכוון לאפופלקסיה משך הזמן לאחר הלידה וכן העדר מידע המכוון ל-
PRES או SAH
כמו יתר לחץ דם, או הקאה פתאומית עם הופעת כאב הראש וכדומה..
מטופלת עם הפרעת פוריות, כאב ראש עז ולאחריו מנינגיזמוס עם הפרעה בתנועות עיניים.
1. אפופלקסיה של ההיפופיזה
2. דימום סאב-דורלי
אפופלקסיה
תיאורשלחולהעםהתפתחותהדרגתיתשלשיתוקעצב3בצדאחדוירידהבשמיעהבאותוצדעםווברלצדהשני,בהמשךחולשהפרוקסימליתבפיזורשלמספרשורשים,בMRIהאדרתקרומים,מההבדיקההחשובהמCSF:
א.PCRלWNV
ב.ציטולוגיה
ג.פרופילפאראנאופלסטי
ציטולוגיה. מכוונים פה לקרצינומטוס מנינגיטיס.
הקומבינציה של חולשת פנים, חירשות (מרמז תמיד על לימפומה) ושיתוק אוקולורי עם חולשת גפיים בילטרלית אסימטרית מרמז מאוד לפיזור לפטומנינגיאלי.
בת 70 עם בלבול חדש ופגיעה בעצבים קרניאלים. בציטולוגיה מניקור מותני נמצאו תאי אדנוקרצינומה.
מה הטיפול?
1. IV steroids
2. IV methotrexate
3. intrathecal methotrexate
intrathecal methotrexate
treatment of Carcinomatous and Lymphomatous Meningitis
This consists mainly of radiation therapy to the symptomatic areas (cranium, posterior fossa, or spine) followed in selected cases by the intraventricular/intrathecal administration of methotrexate, but these measures rarely stabilize neurologic symptoms for more than a few weeks or months. The methotrexate is typically administered into the lateral ventricle via an Ommaya reservoir
תמונהשלגידולפריונטריקולרישנרשםשעוברהאדרה,דיפוזיהמוגבלתבDWI,אפקטמסה.במיקום הנל-
מההטיפולהנכון:
א.כריתהוהקרנה
ב.כימותרפיהוכריתה
ג.כימותרפיהוהקרנה
ד.כימותרפיההקרנהוכריתה
כימותרפיה הקרנה וכריתה
איך אפשר לדעת מה האאוטקאם הצפוי של האסטרוציטומות השונות?
לאחרונה התגלה כי הגן IDH1 או IDH2 (מקודדים לאנזים isocitrate dehydrogenase) אחראי להתקדמות של אסטרוציטומות ואוליגודנדרוגליומות לצורות ממאירות יותר. לחולים עם מוטציות בגנים אלו יש OC טוב יותר והתקדמות איטית יותר.
מה מהסיפמטומים הבאים הכי שכיח כסימפטום ראשון של גליומה מדרגה נמוכה בגיל מבוגר?
1. שינוי התנהגותי
2. פרכוסים
3. אטקסיה
4. כאבי ראש
פרכוסים.
ב 2/3 מהחולים, הסימפטום הראשון יהיה פרכוס, או פוקלי או כללי וב 65-70% מחלת הפרכוסים תמשיך. כאבי ראש וסימני ICP מוגברים, הם סימנים פחות נפוצים.
איזה רצף בMRI יבדיל בין חזרת גידול לשינויים פוסט קרינתיים / בצקת?
1. פרפוזיה
2. דיפוזיה
3. רצף T1 עם גדוליניום
4. פלייר
פרפוזיה.
CT and MRI show a contrast-enhancing lesion, and by angiography there is an avascular mass. Small calcifications may appear many years after the radiation. MRI is somewhat more sensitive in distinguishing radiation necrosis from tumor and peritumor products, but PET is the most reliable way of differentiating the two, perhaps obviating the need for biopsy (Glantz et al, 1991). Single-photon emission tomography (SPECT) can be useful for this purpose as well (Carvalho et al). CT or MRI perfusion imaging can also be used to differentiate radiation necrosis from tumor progression; cerebral blood volume is reduced in necrosis and most often elevated in the progression.
איךאתהמבדילביןגידוללנזקמוחיקרינתי?
א.perfusion imaging
ב.diffusion imaging
ג.T2
ד.T1
פרפוזיה.
CT and MRI show a contrast-enhancing lesion, and by angiography there is an avascular mass. Small calcifications may appear many years after the radiation. MRI is somewhat more sensitive in distinguishing radiation necrosis from tumor and peritumor products, but PET is the most reliable way of differentiating the two, perhaps obviating the need for biopsy (Glantz et al, 1991). Single-photon emission tomography (SPECT) can be useful for this purpose as well (Carvalho et al). CT or MRI perfusion imaging can also be used to differentiate radiation necrosis from tumor progression; cerebral blood volume is reduced in necrosis and most often elevated in the progression.
ילד עם לוקמיה שקיבל מתותרקסט+קרינה ולאחר כמה חודשים פיתח אנצפלופתיה ובהדמיה נגעים קונפלואנטים גדולים מפושטים. במה מדובר?
acute necrotizing leukoencephalopathy
מהם סוגי הנזקים השונים של קרינה?
Table 30-5
CLINICAL SYNDROMES CAUSED BY TUMORS AT THE BASE OF THE SKULL
Table 30-4
HORMONAL TESTS FOR DETECTION OF PITUITARY ADENOMAS
Table 30-3
MAIN CATEGORIES OF THE WHO BRAIN TUMOR CLASSIFICATION
Table 30-2
WORLD HEALTH ORGANIZATION (WHO) GRADING OF CENTRAL NERVOUS SYSTEM TUMORS
מטופל עם סרטן כלשהו שהחל לפתח הבזקים בראיה ובהמשך הידרדרות דו עינית בראיה.
1. Anti Ma
2. Anti Ri (ANNA 2)
3. Anti Hu (ANNA 1)
4. Anti-CAR(antirecoverin)
ANTI-CAR =Anti recoverin
בן 60, לאחר טיפול בסרטן ריאה מתלונן על חולשה כללית, טשטוש בראייה ללא כאבים בעיניים, ללא כאב ראש. בבדיקת שדות ראייה:
bilateral central scotomas
פונדוס:
bilateral swallen discs
איזו בדיקה תתמוך באבחנה?
1. Anti Hu
2. Anti RI
3. Anti CAR
4. Anti Yo
5. Anti Ma
ANTI-CAR =Anti recoverin
- although rare, the clinical syndromes associated with the anti-Ma antibody and testicular tumors are diverse: limbic, brainstem, or hypothalamic inflammation and an ataxic–opsoclonic Syndrome that is more typical of anti-Ri antibody.
- The antibody to CRMP-5 is reported in some series to be second in frequency only to anti-Hu. Lung carcinoma has been the most common source in the series of Yu and colleagues, with thymoma, renal cell, and other neoplasms accounting for a few of the cases. The clinical features have been as diverse as for anti-Hu, including seizures, dementia, confusion, depression, as well as a
variety of peripheral and cranial neuropathies and, surprisingly, the Lambert-Eaton syndrome.. - An illness similar to the one caused by anti-NMDA antibodies, under the name of Ophelia syndrome, has been reported as a paraneoplastic syndrome that is due to antibodies directed against the metabotropic glutamate receptor, mGluR5
תיאור של חולה צעירה עם סרטן שד וסובלת מאטאקסיה, מיוקלונוס ואופסוקלונוס והשאלה היא מה תהיה הפתולוגיה?
1. פגיעה בצרבלום בתאי פורקינייה
2. פגיעה בתאים גרנולריים
3. פגיעה בחוט השדרה
4. פגיעה קשה בגזע המח
פגיעה בצרבלום בתאי פורקנייה
anti Ri
anti Ri נמצאים במקרים נדירים בסרטן ריאות
לדעתי גם 1 נכון- אם קורה בילדים אז קשור לנוירובלסטומה.
Opsoclonus-Myoclonus-Ataxia Syndrome
In children, this syndrome is usually a manifestation of Neuroblastoma, but it is more common and occurs in adults in relation to breast cancer and small cell lung
Cancer. The unique feature of the neuroblastoma is a response of this syndrome to corticosteroids and ACTH in most children and in some adults, and resolution of the neurologic signs when the neuroblastoma is removed. A subgroup of breast cancers produce an antineuronal Antibody directed against a different RNA-binding antigen from the anti-Hu antibody; thus it has been termed “anti-Ri” (antineuronal antibody type 2). This antibody is not found in the opsoclonus-ataxic syndrome of neuroblastoma and is present only rarely with small cell lung cancer. There have also been a limited number of positive serologic tests in children with opsoclonus, apparently without an underlying tumor. A few such patients have had a mild pleocytosis in the CSF; the MRI is usually normal. More complex syndromes have been reported with the anti-Ri antibody, manifest by rigidity and intense stimulus-sensitive myoclonus in addition to the core features of opsoclonus and ataxia. Neuroblastoma This, the most common solid tumor of childhood, is a different entity from medulloblastoma but of nearly identical histologic appearance, arising in the adrenal medulla and sometimes metastasizing widely. Usually it remains extradural even if it invades the cranial and spinal cavities. The main neurologic interest is a syndrome of polymyoclonus with opsoclonus and ataxia that occurs as a paraneoplastic complication as discussed further on. A rare form of neuroblastic medulloblastoma in adults tends to be more benign.
תיאור של בחורה צעירה עם טרטומה של השחלה והופעת שינוי התנהגותי ופרכוסים
1. אנטי MA
2. אנטי רקוברין
3. אנטי yo
4. אנטי NMDA
ANTI NMDA
- An illness similar to the one caused by anti-NMDA
antibodies, under the name of Ophelia syndrome, has
been reported as a paraneoplastic syndrome that is due to
antibodies directed against the metabotropic glutamate
receptor, mGluR5
תיאורשלחולהשהתפתחאצלהבהדרגהסימניםפסיכיאטריםואוטונומים, בCSFישמעטתאיםמונונוקלאריםוחלבוןמוגבר, ocb חיוביים. אילונוגדניםמתאימים:
א.AntiNMDA
ב. AntiMOG
ג. AntiGAD
ד. AntiRI
Anti NMDA
בן 54 , הופנה לפסיכיאטר בשל שינוי בהתנהגות ותנועות לא רצוניות, בבדיקתו בלבול, הזיות ו
FACIOBRACHIAL DYSTONIC SEIZURES , נשלח פאנל נוגדנים, איזה נוגדים מוגבר במצב זה ?
א. ANTI LGI1
ב. ANTI NMDA
ג. ANTI RI
ד. ANTI YO
Anti- LGI1
Anti-VGKC and Associated Proteins
Several unusual patterns have been observed from the effects of the anti-VGKC antibodies as well as antibodies to the associated proteins LGI 1 and CASPR 2. About half of patients with * LGI 1 antibodies have faciobrachial dystonic seizures in addition to limbic encephalitis.
* VGKC and CASPR 2 antibodies are associated with peripheral motor hyperexcitability in addition to limbic encephalitis.
נוגדנים לאנטי גאד נמצאים באדיופתי ולא בפרהנאופלסטי.
Occasionally this disorder (stiff man syndrome) occurs as a paraneoplastic disease. Lesser degrees of unexplained mild rigidity are seen from time to time, perhaps as a
consequence of loss of spinal cord intemeurons. In what might be called “stiff woman syndrome,” Folli and associates described 3 female patients with breast cancer who developed a state of generalized motor hyperexcitability and rigidity. These patients generally have no antibodies
to glutamic acid decarboxylase (GAD), as in the sporadic cases of “stiff man syndrome”; probably there are antibodies to other synaptic proteins. (anti amphyphisin )
בת 50, בעברה ניתוח קרצינומה של השד. מתלוננות על נוקשות והתכווצויות כואבות בשרירי הרגליים, הגו והבטן, הליכתה נוקשה ומוזרה “כמו רובוט”. בבדיקתה הנוירולוגית כוח גס תקין, החזרים שווים, ללא הפרעת תחושה. בדיקת EMG- יחידות מוטוריות תקינות.
מה מאפיין את מחלתה?
1. נוכחות נוגדנים לחיידק הטטנוס
2. נוכחות של נוגדנים anti Hu
3. נוכחות של נוגדנים כנגד אמפיפיזין המצוי על גבי הרצפטור של גאבא
4. נוכחות חלבון אוליגוקלונל גבוה בנוזל השדרה
5. מחלה מקבוצת הצ’אנלופתיות עם פגיעה בתעלות אשלגן
- נוכחות של נוגדנים כנגד אמפיפיזין המצוי על גבי הרצפטור של גאבא
Of interest is the finding that about two-thirds of the cases of stiff man syndrome display circulating autoantibodies that are reactive with glutamic acid decarboxylase (GAD), the synthesizing enzyme for gamma-arninobutyric acid (GABA; Solimena et al). On several occasions the test for this antibody has become positive after samples taken over 2 years were negative. An antibody to the glycine receptor (GlyR) has been identified in a subset of patients with stiff man syndrome with anti-GAD Antibodies (McKeon and colleagues). The GlyR antibody, mainly expressed in the brain stem and spinal cord, has also been associated with progressive encephalomyelitis with rigidity and myoclonus, but appears to be highly specific for stiff man syndrome in patients who have antiGAD antibodies.
There are rare paraneoplastic varieties of stiff man Syndrome, mostly accompanying breast cancer and associated in some cases with circulating antibodies directed against amphiphysin or gephyrin, proteins associated with synaptic GABA receptors. Some of the cases related to the antiamphiphysin antibodies also display more conventional types of paraneoplastic neurologic disorder such as encephalopathy or opsoclonus.
אמפיפיזין
anti Hu
כנראה מכוון
לanti hu
אבל יכול להיות גם אנטי MAG
- ANTI –MAG- Proprioceptive sensory loss with gait imbalance, tremor, and the Romberg sign are typical findings in the group with anti-MAG activity, while weakness and atrophy tend to appear later in the illness
- ANTI – HU- The initial symptoms in anti – Hu neuopathy and neuronopathy are numbness or paresthesia, sometimes painful, in a limb or in both feet. There may be lancinating pains at the onset. Over a period of days in some cases, but more typically over weeks, the initially focal symptoms become bilateral and may spread to all limbs and their proximal portions and then to the trunk. It is this widespread and proximal distribution and the involvement of the face, scalp, and often the oral and genital mucosa that mark the process as a sensory ganglionitis and radiculitis and when subacute are highly suggestive of a paraneoplastic process. As the illness progresses, all forms of sensation are greatly reduced, resulting in disabling ataxia and pseudoathetoid movements of the outstretched hands. The reflexes are lost, but not always at the outset, and strength is relatively preserved. Autonomic dysfunction-including constipation or ileus, sicca syndrome, pupillary areflexia, and orthostatic hypotension-is sometimes associated. Also, a virtually pure form of peripheral autonomic failure has been recorded as a paraneoplastic phenomenon (paraneoplastic dysautonomia).
בן 58 פתח תמונה של אטקסיה צרבלרית סימטרית בחודשיים האחרונים באופן הדרגתי. ב MRI מוח אטרופיה של המוחון. לאיזו בדיקת הדמייה הסבירות גבוהה ביותר לקדם את האבחנה:
- מיפוי גליום
- US אשכים
- CT חזה
- CT בטן
- קולונוסקופיה
CT חזה
Paraneoplastic Cerebellar Degeneration
In approximately one-third of the cases, the underlying neoplasm has been in the lung most often a small cell carcinoma)—a figure eflecting the high incidence of this tumor. However, the association of ovarian carcinoma and lymphoma, particularly Hodgkin disease, accounting for approximately 25 and 15 percent, respectively, is considerably higher than would be expected on the basis of the frequency of these malignancies. *Carcinomas of the breast, bowel, uterus, and other viscera have accounted for most
of the remaining cases**
Anti Yo
Anti-Purkinje cell antibodies (termed “anti-Yo”) can be found in the sera of about half of patients with paraneoplastic cerebellar degeneration and in the large majority of those related to carcinoma of the breast or female genital Tract, linking the clinical syndrome and this antibody closely. Antibodies against a nuclear antigen, termed antiHu, may also be present;
ילד בן 7 שמפתח אטאקסיה ובהדמיה גידול צרבלרי עם ציסטה ונודול בתוכה. .
1. מדולובלסטומה
2. המנגיובלסטומה
3. די-נט -DNET TUMOR
4. פילוציטיק אסטרוציטומה
המגנגיובלסטומה
Von hippel landau
* Genetic disease of multiple neoplasias
* AD inheritance- VHL gene chrom 3
* Hemangiblastomas (cerebellar most commonly – appear as cyst with nodule in wall – highly vascular ) – causes ataxia and headache
* Renal cell Cancer
* Rarely – pheochromocytomas, pancreatic tumors, cysts, cystadenomas.
* Retinal hemangiomas
* Polycythemia vera
תיאור של חולה עם גידול בגומה אחורית עם ציסטה ונודול בתוכו עם אנגיוגרפיה פתולוגית של הרטינה, והשאלה היא מאיזה גידול עוד היא עלולה לסבול?
1. גידול נזופרינגיאלי
2. גידול ריאה
3. גידול מסוג Renal Cell Carcinoma
Renal cell carcinoma.
Von hippel landau
* Genetic disease of multiple neoplasias
* AD inheritance- VHL gene chrom 3
* Hemangiblastomas (cerebellar most commonly – appear as cyst with nodule in wall – highly vascular ) – causes ataxia and headache
* Renal cell Cancer
* Rarely – pheochromocytomas, pancreatic tumors, cysts, cystadenomas.
* Retinal hemangiomas
* Polycythemia vera
מהו לרוב הביטוי הראשוני של מדולובלסטומה
כאב ראש וסימני עליית לחץ תוך גולגלתי
מהו הטיפול המומלץ בגידול מסוג
DNET
הסרה של הגידול.
אישה בת חמישים עם קליניקה נוירולוגית חדשה (פרכוסים?) ובהדמיה מספר נגעים עגולים העוברים האדרה עם גדוליניום וחלקם מדממים. מה בסבירות הגבוהה ביותר המקור ששלח גרורות?
1. שד
2. מלנומה
3. שחלה
4. ושט
מלנומה
גרורות של מלנומה נוטות לדמם
Metastases from melanoma and chorioepithelioma are often hemorrhagic, but it is not unusual for tumors originating in lung, thyroid, and kidney to exhibit this characteristic.
ווגנר
Wegener Granulomatosis
This is a rare disease of unknown cause, affecting adults as a rule and favoring males slightly. A subacutely evolving vasculitis with necrotizing granulomas of the upper and lower respiratory tracts followed by necrotizing Glomerulonephritis are its main features.
Neurologic complications come later in one-third to one-half of cases and take two forms:
(1) a peripheral neuropathy either in a pattern of polyneuropathy or, far more frequently, in a pattern of mononeuropathy multiplex (see discussion in Chap. 46).
(2) multiple cranial neuropathies as a result of direct extension of the nasal and sinus granulomas into adjacent upper cranial nerves and from adjacent to pharyngeal lesions to the lower cranial nerves. We have seen this disease produce the syndrome of episodic hemicrania, with periorbital ecchymosis.
The basilar parts of the skull may be eroded, with spread of granuloma to the cranial cavity and more remote parts.
A description is included here because cerebrovascular events, seizures, and cerebritis are less common but well described neurologic complications. Spastic paraparesis, temporal arteritis, Horner syndrome, and papilledema have been observed but are rare (see Nishino et al). The orbits are involved in 20 percent of patients and lesions here simulate the clinical and radiologic appearance of orbital pseudotumor, cellulitis, or lymphoma. Pulmonary granulomas, usually asymptomatic but evident on a chest CT, are also common.
The vasculitis implicates both small arteries and veins. There is a fibrinoid necrosis of their walls and an infiltration by neutrophils and histiocytes. The sedimentation rate is elevated, as are the rheumatoid and antiglobulin factors.
The presence in the blood of cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) has been found to be relatively specific and sensitive for Wegener disease but it may also be present in intravascular lymphoma.
בן 25 ללא מחלות רקע שמתקבל עם פרכוס כללי ראשון וסימני צד ובהדמיה נגע גדול עם גבולות חדים
מה יהיה בפתולוגיה:
1. תאי אפיתל
2. אוליגודנדרוציטים
3. מניגניגומה
אוליגודנדרוציטים
(, האתרים הנפוצים ביותר של הגידול הוא לרוב באונות הפרונטליות או האונות הטמפורליות (40-70% מהאוליגודנדרוגליומות), לרוב ממקומות עמוק בחומר הלבן עם משקעי סידן ומעט בצקת מסביב, גבולות חדים, ביטוי ראשוני של פרכוס)
The neoplastic oligodendrocyte has a small round nucleus and a halo of unstained cytoplasm (“fried egg” appearance). The cell processes are few and stubby, visualized only with silver carbonate stains. Some of the oligodendrocytes have intense immunoreactivity to GFAP,
similar to normal myelin-forming oligodendrocytes.
Microscopic calcifications are observed frequently, both within the tumor and in immediately adjacent brain tissue.
The most common sites of this tumor are the frontal and temporal lobes (40 to 70 percent), often deep in the white matter, with one or more streaks of calcium but little or no surrounding edema. It is rarely found in other parts of the nervous system.
Fried egg
The neoplastic oligodendrocyte has a small round nucleus and a halo of unstained cytoplasm (“fried egg” appearance). The cell processes are few and stubby, visualized only with silver carbonate stains. Some of the oligodendrocytes have intense immunoreactivity to GFAP, similar to normal myelin-forming oligodendrocytes. Microscopic calcifications are observed frequently, both within the tumor and in immediately adjacent brain tissue. The most common sites of this tumor are the frontal and temporal lobes (40 to 70 percent), often deep in the white matter, with one or more streaks of calcium but little or no surrounding edema. It is rarely found in other parts of the nervous system.
פריוונטריקולרי
Primary CNS lymphoma may arise in any part of the Cerebrum, cerebellum, or brainstem, with 60 percent being in the cerebral hemiSpheres; they may be solitary or multifocal. A periventricular localization is common. The neoplasm is highly cellular and grows around and into blood vessels (“angiocentric” pattern) but elicits no tendency to necrosis. The nuclei are oval or bean-shaped with scant cytoplasm, and mitotic figures are numerous. B-cell markers applied to fixed tissue define the lymphoblastic cell population as monoclonal and identify the tumor cell type.
כנראה כיוונו לפיזור לעצבי 8 דו”צ.
The combination of a cranial neuropathy, such as unilateral facial weakness, hearing loss (always suggestive of lymphoma), or ocular motor palsy, with bilateral symmetrical limb weakness is particularly characteristic.
בגלל האדרה הומוגנית כנראה שמתכוונים ללימפומה
* Primary CNS lymphoma may arise in any part of the cerebrum, cerebellum, or brainstem, with 60 percent being In the cerebral hemiSpheres; they may be solitary or multifocal. A periventricular localization is common. Vitreous, uveal, and retinal (ocular) involvement occurs in 10 to 20 percent of cases. The pia and arachnoid may be infiltrated, and a
purely meningeal form of B-cell lymphoma that involves peripheral and cranial nerves is also known. The neoplasm is highly cellular and grows
around and into blood vessels (“angiocentric” pattern) with no tendency to necrosis. Nuclei oval and bean shaped. **The finding on CT and MRI of one or several dense (hypercellular), homogeneous, enhancing, infiltrating, nonnecrotic, nonhemorrhagic, periventricular masses is Characteristic **. However, rim enhancement also occurs, and any part of the brain may be involved. Characteristic of primary CNS lymphoma is the disappearance on imaging of the lesions or complete but transient resolution of contrast enhancement in response to steorids.
Patients with AIDS and less-common immunodeficiency states, such as the WlSkott-Aldrich syndrome and ataxia-telangiectasia, and those who are receiving immunosuppressive drugs for long periods, as for example, in renal Transplantation, are particularly liable to develop this type of lymphoma. Many of the tumors in immunosuppressed patients contain the EBV genome, suggesting a pathogenetic role for the virus.
* Toxoplasma gondii, a tiny (2- to 5-flm), obligate, intracellular parasite. Most often it is a subclinical process or manifested by a painless lymphadenopathy, a mononucleosis-like syndrome, or acute chorioretinitis. There is a rare fulminant, widely disseminated infection with a rickettsia-like rash, encephalitis, myocarditis, and polymyositis. Or the neurologic signs may consist only of myoclonus and Asterixis, suggesting a metabolic encephalopathy. Often,
there are signs of a meningoencephalitis, i.e., seizures, mental confusion, meningeal irritation, coma, and a lymphocytic pleocytosis and increased CSF protein. The brain in such cases shows one or more foci of inflammatory necrosis, essentially an abscess, (Fig. 32-6), with free and encysted T. gondii organisms scattered throughout the white and gray matter. Rarely, large areas of necrosis manifest themselves as one or more mass lesions. Sometimes, a nodular lesion is detected on MR or CT imaging that is performed for other reasons. Patients with a presumptive diagnosis are treated with oral sulfadiazine (4 g initially, then 4 to 6 g daily) and pyrimethamine (200 mg initially, then 50 to 100 mg daily). Leucovorin, 15 to 20 mg daily, should be given to counteract the antifolate action of pyrimethamine. Treatment must be continued for at least 6 weeks.
* Cytomegalo virus
Among the nonfocal neurologic complications of AIDS, the most common are CMV and cryptococcal infections. at autopsy, about one-third of AIDS patients are found to be infected with CMV. However, the contribution of this infection to the total clinical picture is often uncertain. Despite this ambiguity, certain features have emerged as typical of CMV encephalitis in the AIDS patient. According to Holland and colleagues, late in the course of AIDS and usually concurrent with CMV retinitis, the encephalopathy evolves over 3 to 4 weeks. Its clinical features include an acute confusional state or delirium combined in a small proportion of cases with cranial nerve signs including ophthalmoparesis, nystagmus, ptosis, facial nerve palsy, or deafness. In one of our patients, there were progressive oculomotor palsies that began with light-fixed pupils. Pathologic specimens and MRI show the process to be concentrated in the ventricular borders, especially evident as T2 signal hyperintensity in these regions. The lesions may extend more diffusely through the adjacent white matter and be accompanied by meningeal enhancement by gadolinium in a few cases. Extensive destructive lesions have also been reported; this has been true in two of our own cases. Such lesions may be associated with hemorrhagic changes in the CSF in addition to showing an inflammatory response. CMV may also produce a painful lumbosacral polyradiculitis in AIDS (Chap. 44). The diagnosis of CMV infection during life is often difficult. Cultures of the CSF are usually negative and IgG antibody titers are nonspecifically elevated. Newer PCR methods prove useful here. Where the diagnosis is strongly suspected, treatment with the antiviral agents ganciclovir and foscarnet has been recommended, but, as pointed out by Kalayjian and colleagues, the CMV disease may develop and progress while patients are taking these medications as maintenance therapy.
מה גורם סיכון ל-Primary CNS Lymphoma
א. Ataxia-telangectasia
ב. Churg-Strauss
ג. Tuberous sclerosis
Ataxia talangiectasia
Patients with AIDS and less-common immunodeficiency states, such as the Wiskott-Aldrich syndrome and ataxia-telangiectasia, and those who are receiving immunosuppressive drugs for long periods, as for example, in renal transplantation, are particularly liable to develop this type of lymphoma.
the EBV genome has also been found in the tumors of a few immunocompetent patients
related Sjogren syndrome. Another disorder, lymphomatoid granulomatosis (called by several different names), is also driven by EBV virus and has similarities to CNS lymphoma.
מה מהבאים לא גורם לבצקת ציטוטוקסית?
1. SIADH
2. Heptaic failiure
3. Hypoxic- ischemic
4. osmotic disequilibrium of dialysis
5. Glioblastima
Glioblastoma
- Vasogenic edema is the type seen in the vicinity of tumor growths and other localized processes as wellas in more diffuse injury to the blood vessels (e.g., lead encephalopathy; malignant hypertension). It is practically limited to the white matter and is evidenced by decreased attenuation on CT and by hyperintensity on T2-weighted MRI and elevated diffusivity (reduced anisotropy) on diffusion-weighted MRI. presumably, there is increased permeability of the capillary endothelial cells so that plasma proteins exude into the extracellular spaces.
- By contrast, in cytotoxic edema, all the cellular elements (neurons, glia, and endothelial cells) swell with fluid and there is a corresponding reduction in the extracellular fluid space. Because a shift of water occurs from the extracellular to the intracellular compartment, there is relatively little mass effect, the opposite of what occurs with the vascular leak of vasogenic edema. This cellular edema occurs typically with hypoxic-ischemic injury but it may also complicate acute hypoosmolality of the plasma, acute hepatic encephalopathy, inappropriate secretion of antidiuretic hormone, and the osmotic disequilibrium syndrome of hemodialysis. Like vasogenic edema, cytotoxic edema results in decreased attenuation in CT and hyperintensity on T2-weighted MRI. However, it is coupled with reduced diffusivity (increased anisotropy) rather than elevated diffusivity on diffusion-weighted MRI.
- In the case of cerebral infarction, an initial phase of cytotoxic edema precedes the onset of vasogenic edema, manifest as early
reduced diffusivity followed by later elevated diffusivity on diffusion-weighted MRI.
איך תראה בצקת ציטוטוקסית בהדמיה?
Cytotoxic Oedema
MRI As cytotoxic oedema represents the redistribution of water from extracellular to intracellular compartments, without a change in local constituents it stands to reason that no T1 or T2 changes are evident. As is the case with CT, the changes colloquially ascribed to ‘cytotoxic oedema’ are in fact mostly due to ionic oedema and are described separately.
The one sequence which is able to identify cytotoxic oedema, and was thus responsible for a revolution in the imaging of acute ischaemic stroke, is diffusion weighted imaging (DWI). As cells swell due to inward shift of water, the there is a commensurate decrease in diffusion, identified as high signal on DWI and low signal on ADC.
איך תראה בצקת וזוגנית בהדמייה?
Vasogenic cerebral oedema refers to a type of cerebral oedema in which the blood brain barrier (BBB) is disrupted (cf. cytotoxic cerebral oedema, where the BBB is intact). It is an extracellular oedema which mainly affects the white matter via leakage of fluid from capillaries.
It is most frequently seen around brain tumours (both primary and secondary) and cerebral abscesses, though some vasogenic oedema may be seen around maturing cerebral contusion and cerebral haemorrhage.
Radiographic features
* CT
grey-white matter differentiation is maintained and the oedema involves mainly white matter, extending in finger-like fashion secondary effects of vasogenic oedema are similar to cytotoxic oedema, with effacement of cerebral sulci, with or without midline shift.
* MRI
hyperintense T2 and FLAIR signals, which do not show restricted diffusion (cf. cytotoxic cerebral oedema, which shows diffusion restriction)
vasogenic edema
- True restricted diffusion, appearing hyperintense on the DWI sequence in acute infarction, is hypointense on a related sequence termed apparent diffusion coefficient (ADC).
- If the hyperintense DWI signal is also hyperintense on ADC, then diffusion is termed facilitated rather than restricted. This phenomenon is seen when the free movement of water within a tissue becomes increasingly isotropic, as with vasogenic edema. Therefore, the interpretation of DWI signal hyperintensity must be gauged in the context of the ADC signal in the same region.
The administration of gadolinium, a paramagnetic agent that accelerates the process of proton relaxation during the T1 sequence of MRI, permits even sharper definition and highlights regions surrounding many types of lesions where the blood–brain barrier has been disrupted in the brain, spinal cord, or nerve roots.
גנגליוציטומה
The best characterized, albeit rare, type in this group is the gangliocytoma, a tumor that occurs in the adrenal gland, retroperitoneal and thoracic sympathetic chain, internal auditory canal, and in the spinal cord. One form is the dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). This is a slowly evolving lesion that forms a mass in the cerebellum; it is composed of granule, Purkinje, and glia cells. Reproduced therein, in a disorganized fashion, is the architecture of the cerebellum with no clear plane from normally structured cerebellar tissue. The importance of distinguishing this disease from other cerebellar tumors is its lack of growth potential and favorable prognosis. It should, however, be excised if symptomatic. The appearance on imaging is highly characteristic; a cerebellar hemisphere is occupied with an indistinct mass of “tiger stripe” appearance as a result of alternating layers of dysmorphic cerebellar cells.
carotid body tumor usually does not go through a malignant transformation.
Carotid Body Tumor (Paraganglioma)
This is a generally benign but potentially malignant tumor originating in a small aggregate of paraganglioma cells of neuroectodermal type. The normal carotid body is small (4 mm in greatest diameter and 10 mg in weight) and is located at the bifurcation of the common carotid artery. The cells are of uniform size, have an abundant cytoplasm, are rich in substance P, and are sensitive to changes in Po2, Pco2, and pH (i.e., they are chemoreceptors, not to be confused with baroreceptors). The tumors
that arise from these cells are identical in appearance to tumors of other chemoreceptor organs such as the glomus jugulare neoplasm described in the preceding section (paragangliomas). Interestingly, they are many times more frequent in individuals living at high altitudes.
The usual presentation is of a painless mass at the side of the neck below the angle of the jaw; thus it must be differentiated from the branchial cleft cyst, mixed tumor of the salivary gland, and carcinomas and aneurysms in this region. As the tumor grows (at an estimated rate of 2.0 em in diameter every 5 years) it may implicate the sympathetic, glossopharyngeal, vagus, spinal accessory, and hypoglossal nerves. Hearing loss, tinnitus, and vertigo are present in some cases. Tumors of the carotid body have been a source of transient ischemic attacks in 5 to 15 percent of the 600 or more reported cases. One of the most interesting presentations has been with sleep apnea, particularly with bilateral tumors (see later); respiratory depression as well as lability of blood pressure are common postoperative problems. Malignant transformation occurs in 5 percent of cases.
מנינגיומה פרונטלית
Meningioma of the Olfactory Groove
This tumor originates in arachnoidal cells along the cribriform plate. The diagnosis depends on the finding of ipsilateral or bilateral anosmia or ipsilateral or bilateral blindness—often with optic atrophy and mental changes. The tumors may reach enormous size before coming to the attention of the physician but as many are small and found incidentally with cerebral imaging (see Fig. 30-7B). If the anosmia is unilateral, it is rarely if ever reported by the patient. The unilateral visual disturbance may consist of a slowly developing central scotoma. Abulia, confusion, forgetfulness, and inappropriate jocularity (witzelsucht) are the usual psychic disturbances from compression of the inferior frontal lobes (see Chap. 21). The patient may be indifferent to or joke about his blindness. Usually there are radiographic changes along the cribriform plate. MRI with gadolinium is diagnostic. Except for the largest and most invasive tumors, surgical removal is possible
בת 50 החלה לסבול מחולשה של רגל שמאל ובהמשך יד שמאל, לאחר מכן יד ימין ולבסוף רגל ימין. מה הממצא הסביר?
1. מיאליטיס
2. מנינגיומה צווארית
3. שבץ ספינאלי
4. גליומה
מנינגיומה צווארת
A progressive syndrome of monoparesis, biparesis, usually of the arms, and then triparesis involving the leg on the side of the last affected arm (“around the clock“ pattern) is caused by tumors and a variety of other compressive lesions in the region of the foramen magnum and high cervical cord. This is putatively explained by the pattern of corticospinal fiber decussation at the cervicomedullary junction.
A meningioma of the foramen magnum, for example, may begin with spastic weakness of one limb, followed by sequential involvement of the other limbs in the above noted “around-the-clock” pattern. There are usually bilateral Babinski signs early in the process, but there may be few sensory findings.
———-
Formen Magnum Tumors:
Tumors in the region of the foramen magnum are of particular importance because of the need to differentiate them from diseases such as multiple sclerosis, Chiari malformation, syringomyelia, and bony abnormalities of the craniocervical junction.
If unrecognized, they terminate fatally by causing medullary and high spinal cord compression. these tumors are not common (approximately 1 percent of all intracranial and intraspinal Tumors). In all series, meningiomas, schwannomas, neurofibromas, and dermoid cysts are the most common types; others, all rare, are teratomas, dermoids, granulomas, cavernous hemangiomas, hemangioblastomas, hemangiopericytomas, lipomas, and epidural carcinomas.
- The cranial nerve signs most frequently conjoined and indicative of intracranial extension of a foramen magnum tumor are dysphagia, dysphonia, dysarthria, and drooping shoulder (because of vagal, hypoglossal, and spinal accessory involvement); included less often are nystagmus and episodic diplopia, sensory loss over the face and unilateral or bilateral facial weakness, and a Horner syndrome.
- Pain in the suboccipital or posterior cervical region, mostly on the side of the tumor, is usually the first, and by far the most prominent complaint. In some instances the pain may extend into the shoulder and even the arm. the pain may radiate down the back, even to the lower spine.
- One pattern is weakness of a shoulder and arm progressing to the ipsilateral leg and then to the opposite leg and arm (“around-the-clock” paralysis).
- Another configuration is triplegia that is a characteristic but not invariable sequence of events, caused by the encroachment of tumor upon the decussating corticospinal tracts at the foramen magnum.
- Occasionally, both upper limbs are involved alone; surprisingly, there may be atrophic weakness of the hand or forearm or even intercostal muscles with diminished tendon reflexes well below the level of the tumor.
- Involvement of sensory tracts also occurs; more often it is posterior column sensibility that is impaired on one or both sides, with patterns of progression similar to those of the motor paralysis. Sensation of intense cold in the neck and shoulders has been another unexpected complaint, and also “bands” of hyperesthesia around the neck and back of the head.
מנינגיומה בגובה הפוראמן מגנום
A progressive syndrome of monoparesis, biparesis, usually of the arms, and then triparesis involving the leg on the side of the last affected arm (“around the clock“ pattern) is caused by tumors and a variety of other compressive lesions in the region of the foramen magnum and high cervical cord. This is putatively explained by the pattern of corticospinal fiber decussation at the cervicomedullary junction.
A meningioma of the foramen magnum, for example, may begin with spastic weakness of one limb, followed by sequential involvement of the other limbs in the above noted “around-the-clock” pattern. There are usually bilateral Babinski signs early in the process, but there may be few sensory findings.
———-
Formen Magnum Tumors:
Tumors in the region of the foramen magnum are of particular importance because of the need to differentiate them from diseases such as multiple sclerosis, Chiari malformation, syringomyelia, and bony abnormalities of the craniocervical junction.
If unrecognized, they terminate fatally by causing medullary and high spinal cord compression. these tumors are not common (approximately 1 percent of all intracranial and intraspinal Tumors). In all series, meningiomas, schwannomas, neurofibromas, and dermoid cysts are the most common types; others, all rare, are teratomas, dermoids, granulomas, cavernous hemangiomas, hemangioblastomas, hemangiopericytomas, lipomas, and epidural carcinomas.
- The cranial nerve signs most frequently conjoined and indicative of intracranial extension of a foramen magnum tumor are dysphagia, dysphonia, dysarthria, and drooping shoulder (because of vagal, hypoglossal, and spinal accessory involvement); included less often are nystagmus and episodic diplopia, sensory loss over the face and unilateral or bilateral facial weakness, and a Horner syndrome.
- Pain in the suboccipital or posterior cervical region, mostly on the side of the tumor, is usually the first, and by far the most prominent complaint. In some instances the pain may extend into the shoulder and even the arm. the pain may radiate down the back, even to the lower spine.
- One pattern is weakness of a shoulder and arm progressing to the ipsilateral leg and then to the opposite leg and arm (“around-the-clock” paralysis).
- Another configuration is triplegia that is a characteristic but not invariable sequence of events, caused by the encroachment of tumor upon the decussating corticospinal tracts at the foramen magnum.
- Occasionally, both upper limbs are involved alone; surprisingly, there may be atrophic weakness of the hand or forearm or even intercostal muscles with diminished tendon reflexes well below the level of the tumor.
- Involvement of sensory tracts also occurs; more often it is posterior column sensibility that is impaired on one or both sides, with patterns of progression similar to those of the motor paralysis. Sensation of intense cold in the neck and shoulders has been another unexpected complaint, and also “bands” of hyperesthesia around the neck and back of the head.
בן 10 מתלונן על חולשה פרוקסימלית ודיסטלית בגפיים, שהתחילה לפני 4 חודשים, תחילה ביד ימין,
לאחר חודש הופיעה חולשה גם של יד שמאל, לאחר חודש נוסף הופיעה חולשה של רגל שמאל.
מהו הממצא הפתולוגי שעשוי להסביר את הקליניקה בצורה הטובה ביותר?
1. א. נגע דמיאלינטיבי מ- C3 עד C6 בצד השמאלי של חוט השידרה.
2. ב. מנינגיומה בגובה של foramen magnum עד C2
3. ג. פריצת דיסק עם לחץ על מרכז החוט בגובה C7
4. ד. נגע פתולוגי מ C2 עד C7 עקב חוסר של נחושת
מנינגיומה בגובה הפוראמן מגנום
A progressive syndrome of monoparesis, biparesis, usually of the arms, and then triparesis involving the leg on the side of the last affected arm (“around the clock“ pattern) is caused by tumors and a variety of other compressive lesions in the region of the foramen magnum and high cervical cord. This is putatively explained by the pattern of corticospinal fiber decussation at the cervicomedullary junction.
A meningioma of the foramen magnum, for example, may begin with spastic weakness of one limb, followed by sequential involvement of the other limbs in the above noted “around-the-clock” pattern. There are usually bilateral Babinski signs early in the process, but there may be few sensory findings.
———-
Formen Magnum Tumors:
Tumors in the region of the foramen magnum are of particular importance because of the need to differentiate them from diseases such as multiple sclerosis, Chiari malformation, syringomyelia, and bony abnormalities of the craniocervical junction.
If unrecognized, they terminate fatally by causing medullary and high spinal cord compression. these tumors are not common (approximately 1 percent of all intracranial and intraspinal Tumors). In all series, meningiomas, schwannomas, neurofibromas, and dermoid cysts are the most common types; others, all rare, are teratomas, dermoids, granulomas, cavernous hemangiomas, hemangioblastomas, hemangiopericytomas, lipomas, and epidural carcinomas.
- The cranial nerve signs most frequently conjoined and indicative of intracranial extension of a foramen magnum tumor are dysphagia, dysphonia, dysarthria, and drooping shoulder (because of vagal, hypoglossal, and spinal accessory involvement); included less often are nystagmus and episodic diplopia, sensory loss over the face and unilateral or bilateral facial weakness, and a Horner syndrome.
- Pain in the suboccipital or posterior cervical region, mostly on the side of the tumor, is usually the first, and by far the most prominent complaint. In some instances the pain may extend into the shoulder and even the arm. the pain may radiate down the back, even to the lower spine.
- One pattern is weakness of a shoulder and arm progressing to the ipsilateral leg and then to the opposite leg and arm (“around-the-clock” paralysis).
- Another configuration is triplegia that is a characteristic but not invariable sequence of events, caused by the encroachment of tumor upon the decussating corticospinal tracts at the foramen magnum.
- Occasionally, both upper limbs are involved alone; surprisingly, there may be atrophic weakness of the hand or forearm or even intercostal muscles with diminished tendon reflexes well below the level of the tumor.
- Involvement of sensory tracts also occurs; more often it is posterior column sensibility that is impaired on one or both sides, with patterns of progression similar to those of the motor paralysis. Sensation of intense cold in the neck and shoulders has been another unexpected complaint, and also “bands” of hyperesthesia around the neck and back of the head.
לא תהיה לו הפרעה באקומודציה
1.
Paralysis of upgaze and accommodation but sparing of other eye movements
2.
Normal-to-large pupils with light-near dissociation (loss of pupillary reflex to light with preservation of pupilloconstriction in response to convergence)
3.
Eyelid retraction
4.
Convergence-retraction nystagmus (eyes make convergent and retracting oscillations following an upward saccade)
מה מהגידולים הבאים לא עושה קלציפיקציות?
1. אסטרוציטומה
2. אפנדימומה
3. מדולובלסטומה
4. אוליגודנדרוגליומה
מדולובלסטומה לא עושה קלציפיקציות
Calcifications found in Astrocytomas, Oligodendroglionas, Ependymoomas and Meningiomas
+
pituitary adenoma, craniopharyngioma,
Hand-Schuller-Christian disease, and sarcoidosis.
Figure 30-1. A. Schematic representation of the astrocytes and endothelial cells of the capillary wall in the normal state (above) and in vasogenic edema (below). Heightened permeability in vasogenic edema is partly the result of a defect in tight endothelial junctions, but mainly a result of active vesicular transport across endothelial cells.
B. Cellular (cytotoxic) edema, showing swelling of the endothelial, glial, and neuronal cells at the expense of the extracellular fluid space of the brain. (Reproduced by permission from Fishman.)
Figure 30-2. Glioblastoma. Contrast-enhanced T1-weighted MRI
illustrates a large irregularly enhancing tumor with internal necrosis
deep within the left cerebral hemisphere.
Figure 30-3. Simplified molecular genetic analysis of gliomas as a supplement to histological classification. IDH, Isocitrate dehydroxygenase;
TP53, tumor (suppressor) protein 53; ATRX, alpha-thalassemia/mental retardation syndrome X-linked. (From Louis DN, Ohgaki H, Wiestler OD,
Cavenee WK: World Health Organization Histological Classification of Tumours of the Central Nervous System. France, International Agency for
Research on Cancer, 2016. Reproduced with permission from David N. Louis, MD.)
Figure 30-4. Astrocytoma of the left frontal lobe; the T2-weighted MRI shows an infiltrating tumor with minimal mass effect and mild edema. The degree of contrast enhancement is variable but most often less than glioblastoma.
Figure 30-5. Gliomatosis cerebri invading both hemispheres. T2-weighted MRI shows a large confluent area of involvement in the frontal lobes with effacement of overlying cortical sulci. There was slight enhancement along the margins of the lesions after gadolinium infusion. The patient was mentally slow but had no other neurologic signs.
Figure 30-6. A partially cystic oligodendroglioma of the right frontotemporal
region. There was no abnormal contrast enhancement.
Figure 30-7. A. Parafalcine meningioma; coronal image, MRI with gadolinium. Note the rightward displacement of an anterior cerebral artery
(hypointense flow void) trapped between the right lateral margin of the mass and the right medial frontal lobe. B. Small and asymptomatic left
olfactory groove meningioma, MRI with gadolinium.
Figure 30-8. Gadolinium-enhanced MRI of meningioma. Large subfrontal
extraaxial mass with central calcification and surrounding
vasogenic edema. Homogeneous avid enhancement is characteristic
of the tumor.
Figure 30-9. Primary central nervous system lymphoma. Left: Axial T2-FLAIR MRI showing hyperintensity in the right periatrial white matter,
without mass effect. Right: Contrast-enhanced MRI reveals two foci of nodular parenchymal enhancement.
Figure 30-10. Contrast-enhanced MRI (left) showing multiple metastases from renal cell carcinoma. Note the extensive hypointense edema
surrounding each lesion. The right image is a gradient echo MRI in which blood products appear hypointense (dark). This sequence can aid in
detection of small or nonenhancing hemorrhagic metastases, such as the lesion in the left occipital lobe.
Figure 30-11. Leptomeningeal carcinomatosis from breast cancer showing infiltration of the cortical and cerebellar subarachnoid spaces on
axial T1 post-gadolinium MRI. There is a small metastases in the pontine tegmentum.
Figure 30-12. Medulloblastoma. MRI in the sagittal (above) and axial
(below) planes, illustrating involvement of the cerebellar vermis and
neoplastic obliteration of the fourth ventricle.
Figure 30-13. Ependymoma of the fourth ventricle. A. Coronal T2 MRI shows an ependymoma growing out of the fourth ventricle. B. Axial T2 FLAIR MRI shows that the mass completely obliterates the fourth ventricle.
Figure 30-14. Hemangioblastoma. Contrast-enhanced MRI in the axial plane (left) shows the vascular tumor in the left cerebellar hemisphere. Selective left vertebral angiogram (right) defines a hypervascular nodule with dilated draining veins.
Figure 30-15. Pineal tumor. T2-weighted MRI in the sagittal plane
(above) demonstrates a tumor that compresses the tectum and the
cerebral aqueduct. Axial T2 FLAIR MRI (below) shows the tumor and
evidence of hydrocephalus and transependymal flow of CSF from the
lateral ventricles, resulting from aqueductal compression.
Figure 30-16. Lhermitte-Duclos disease. T2-weighted MRI showing the characteristic “tiger stripe” appearance of this hamartomatous tumor in the right cerebellar hemisphere.
Figure 30-17. Contrast-enhanced MRI of a cystic and nodular dysembryoplastic neuroepithelial tumor (DNET) of the left temporal lobe in an adult who had a single seizure. The nodule had an inflammatory component and the seizures ceased with resection.
Figure 30-18. Colloid cyst of the third ventricle. MRI in the axial and sagittal planes. Hydrocephalus due to obstruction at the foramen of Monro
can occur, but is not apparent here.
Figure 30-19. Bilateral vestibular schwannomas in neurofibromatosis type 2. T1-weighted MRI in the axial plane before (left) and after (right) contrast administration.
Figure 30-20. A. MRI of a small vestibular schwannoma emanating from the left porus acusticus, showing typical homogenous gadolinium enhancement.
B. A larger atypical vestibular schwannoma with rim enhancement, causing compression of the left middle cerebellar peduncle.
Figure 30-21. Pituitary macroadenoma. Coronal T1 MRI before (left) and after (right) contrast administration. A homogenously enhancing mass originating in the sella protrudes into the suprasellar cistern and displaces the optic chiasm and inferior hypothalamus. The lesion also extends into the adjacent cavernous sinuses.
Figure 30-22. Pontine glioma. Contrast-enhanced T1 MRI demonstrates a mass with prominent irregular peripheral gadolinium enhancement. The patient was a 3-year-old male with progressive cranial nerve and long tract deficits.
Figure 30-23. MRI demonstrating an epidermoid cyst in the left
cerebellopontine angle just above the foramen magnum. The cyst is
heterogenous and hyperintense on T2-weighted MRI (A) and demonstrates
reduced diffusivity (B), a characteristic feature.
Figure 30-24. Axial T2 FLAIR MRI from a woman with anti-voltagegated
potassium channel (VGKC) paraneoplastic limbic encephalitis
associated with thyroid cancer. The hippocampus and amygdala
appear abnormally T2 hyperintense.
figure 30-25. Paraneoplastic cerebellar degeneration. Coronal T2
FLAIR MRI showing subtle diffuse abnormal T2 hyperintensity of the
cerebellar cortex.
Figure 30-26. Radiation leukoencephalopathy. (Left panel) A patient who underwent proton beam radiation therapy for carcinoma of the left mastoid area presented several years later with radiation necrosis presenting as a seizure. There is extensive edema in the left hemisphere and obstructive hydrocephalus of the contralateral ventricle. (Right panel) Another patient with lung cancer who was treated with prophylactic whole-brain radiation, presented years later with gait difficulties and cognitive decline and was found to have extensive symmetric leukoencephalopathy with ex vacuo ventricular dilation.
Figure 30-27. Stroke-like migraine attacks after radiation therapy (SMART). A 68-year-old man who had prophylactic cranial radiation presented several years later with dysphasia and right hemiparesis. MRI showed abnormal T2 hyperintensity (left) and more prominent enhancement (right) of the cortex of the left hemisphere. These imaging abnormalities resolved after several weeks. (Images reproduced from Olsen et al with permission from Wolters Kluwer.)
