פרק 30 Chapter 30: Intracranial Neoplasms and Paraneoplastic Disorders Flashcards

Question

תיאור של בחורה צעירה עם טרטומה של השחלה והופעת שינוי התנהגותי ופרכוסים 1. אנטי MA 2. אנטי רקוברין 3. אנטי yo 4. אנטי NMDA

Answer 1

ANTI NMDA ## Footnote * 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**

Answer 2

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**.

Answer 3

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 )

Answer 4

3. 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.

Answer 5

אמפיפיזין

Answer 6

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).

Answer 7

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**

Answer 8

**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;

Answer 9

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

Answer 10

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

Answer 11

כאב ראש וסימני עליית לחץ תוך גולגלתי

Answer 12

הסרה של הגידול.

Answer 13

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.

Answer 14

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.

Answer 15

primary meningeal melanosis דם בנוזל השדרה, עלייה בלחץ תוך גולגולתי עם פפילדמה ופרכוסים. ללא סימני צד. * Also known is a **rare primary malignant melanoma of the meninges** that *acts in a similar way to carcinomatous meningitis* but has the striking feature of **bloody CSF** (1,000 to 10,000 red blood cells per mm3). The origin of the neoplasm is from native melanotic cells in the meninges. The **prognosis is as bleak** as it is for metastatic carcinomatous meningitis as discussed by Liubinas et al. * **Carcinamatous:** The combination of a cranial neuropathy, such as unilateral facial weakness, hearing loss (suggestive of lymphoma), or ocular motor palsy, with bilateral asymmetrical limb weakness is particularly characteristic.

Answer 16

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.

Answer 17

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.

Answer 18

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.

Answer 19

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.**

Answer 20

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.

Answer 21

Intravascular lymphoma **Intravascular lymphoma**: a multifocal neoplasm of large anaplastic monoclonal lymphocytes that **infiltrate the walls of blood vessels and surrounding areas** or grow intravascularly and **cause occlusion** of small and moderate-sized vessels. The disease can be **distinguished from primary CNS lymphoma, which is also typically "angiocentric," meaning centered around vessels, but does not selectively invade and occlude vascular structures**. * In the brain and spinal cord there are lesions of various sizes that represent the **combined effects of occlusion of small vessels and concentric infiltration of the adjacent tissue by neoplastic cells**. In half of the cases, meningeal vessels are involved and in a few, the peripheral nerves, or more particularly the **endoneurial vessels** within spinal roots, have been involved by the neoplasm, and we have seen cases with a *flaccid paraplegia* on this basis. Because of the inconsistent location and size of the nervous system lesions there is no uniform syndrome, but **intravascular lymphoma should be suspected in patients with a subacute encephalopathy and indications of focal brain and spinal cord or nerve root lesions. Headache is a prominent early component in some cases**. * **MRI shows multiple nodular or variegated abnormalities on T2-weighted images throughout the white matter of the brain; most lesions are enhanced by gadolinium, and some demonstrate restricted diffusion resulting from microvascular occlusion and infarction**. * Definitive diagnosis is possible only through a **biopsy** of radiographically involved lung or nervous tissue that includes tissue with numerous intrinsic blood vessels.

Answer 22

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.

Answer 23

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**.

Answer 24

בצקת של תאי אנדותל 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**

Answer 25

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.**

Answer 26

**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)

Answer 27

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.

Answer 28

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.**

Answer 29

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.**

Answer 30

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

Answer 31

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.

Answer 32

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.

Answer 33

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.

Answer 34

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)

Answer 35

פרפרזיס עקב גרורות לחוליות הגביות.

Answer 36

מדולובלסטומה לא עושה קלציפיקציות Calcifications found in **Astrocytomas, Oligodendroglionas, Ependymoomas and Meningiomas** + pituitary adenoma, craniopharyngioma, Hand-Schuller-Christian disease, and sarcoidosis.

Answer 37

2. METHOTREXATE, CYTARABINE, RITUXIMAB בגלל שזה נשמע יותר לימפומה מטוקסופלזמה- האדרה הומוגנית וחולת איידס.

פרק 30 Chapter 30: Intracranial Neoplasms and Paraneoplastic Disorders Flashcards

(90 cards)