פרק 32 Chapter 32 Viral Infections of the Nervous System and Prion Diseases Flashcards
מה מהווה פרוגנוזה טובה להרפס אנצפליטיס?
1. מצב הכרה בקבלה
2. EEG תקין
3. ממצאים הדמייתים
4. ממצאים בניקור מותני
מצב הכרה תקין בקבלה
בת 13 , בריאה בד”כ. אושפזה עקב חום, כאב ראש שהופיעו יום לפני קבלתה, ובהמשך פרכוסים,
דליריום, הפרעה אפזית בדיבור. CT מח במיון בתמונה להלן:
הרפס סימפלקס אנצפליטיס
בחור צעיר עם אפיזודה שניה של מנינגיטיס לימפוציטרי, מה המחולל הסביר?
1. HSV2
2. CMV
3. VZV
HSV
Mollaret recurrent meningitis, many instances of which have been associated with HSV-1 (Steel) and others (perhaps most) with HSV-2 infection (Cohen et al).
The syndrome is characterized by episodes of acute meningitis with severe headache and sometimes low-grade fever, lasting for about 2 weeks, and recurring over a period of several months or years. In a few patients of ours, in whom no virus could be identified in the CSF, antiviral therapy met with some success, although corticosteroids seemed to reduce the severity of acute episodes.
A proportion of these cases follow bouts of genital herpes and individual cases have been reported with EBV, herpes-6 in children, and other viruses. A special syndrome that has been associated with HSV-2 is that of aseptic meningitis and bladder failure and vaginal or vulvar pain after a bout of genital herpes (Elsberg Syndrome).
—–
The CSF formula in a number of other chronic or acutely recurring meningitides corresponds to that of aseptic meningitis. These include (1) the Vogt-KoyanagiHarada syndrome, which is characterized by combinations of iridocyclitis, depigmentation of a thick swath of hair (poliosis circumscripta) and of the skin, vitiligo, around the eyes, loss of eyelashes, dysacusis, and deafness (the pathologic basis of the syndrome is not known);
and (2) Mollaret recurrent meningitis, many instances of which have been associated with HSV-1 (Steel) and others (perhaps most) with HSV-2 infection.
Table 32-1_CAUSES OF CHRONIC AND RECURRENT ASEPTIC MENINGITIS
Infectious (10)
Granulomatosis & vasculitis (5)
Neoplastic (3)
Allergic (4)
Chemical (2)
Idiopathic (2)
– בן 35, בריא, כאבי ראש, פוטופונופוביה. לפני 6 חודשים ולפני שנה חווה סימפטומים דומים שחלפו כעברו שבועיים. בנוזל השדרה : 64 תאים רובם מונונוקלארים, חלבון 87, סוכר 55/90 בדם. איזה וירוס?
1. Coxackie
2. HSV
3. HIV
4. Lymphocytic choriomeningitis
5. VZV
HSV
Mollaret recurrent meningitis, many instances of which have been associated with HSV-1 (Steel) and others (perhaps most) with HSV-2 infection (Cohen et al).
The syndrome is characterized by episodes of acute meningitis with severe headache and sometimes low-grade fever, lasting for about 2 weeks, and recurring over a period of several months or years. In a few patients of
ours, in whom no virus could be identified in the CSF, antiviral therapy met with some success, although corticosteroids seemed to reduce the severity of acute episodes.
A proportion of these cases follow bouts of genital
herpes and individual cases have been reported with EBV, herpes-6 in children, and other viruses. A special syndrome that has been associated with HSV-2 is that of aseptic meningitis and bladder failure and vaginal
or vulvar pain after a bout of genital herpes (Elsberg Syndrome)
—–
The CSF formula in a number of other chronic or
acutely recurring meningitides corresponds to that of aseptic meningitis. These include (1) the Vogt-KoyanagiHarada syndrome, which is characterized by combinations of iridocyclitis, depigmentation of a thick swath of hair (poliosis circumscripta) and of the skin, vitiligo, around the eyes, loss of eyelashes, dysacusis, and deafness (the pathologic basis of the syndrome is not known);
and (2) Mollaret recurrent meningitis, many instances of which have been associated with HSV-1 (Steel) and others (perhaps most) with HSV-2 infection.
אמיטריפטילין
Post Herpetic Neuralgia
This severely painful syndrome follows shingles in 5 to 10 percent of patients but occurs almost three times more often among individuals older than age 60 years. The possible effect of acute treatment on the severity of postherpetic neuralgia is mentioned above but potential prevention with the vaccine is even more appealing. It is likely that incomplete interruption of nerve impulses results in a hyperpathic state in which every stimulus excites pain. amitriptyline proved to be an effective therapeutic measure. initially, it is given in doses of approximately 50 mg at bedtime; if needed, the dosage can be increased
gradually to 125 mg daily. The addition of carbamazepine, gabapentin, pregabalin, or valproate may further moderate the pain, particularly if it is of lancinating type. Capsaicin ointment can be applied to painful skin, as noted above. A salve of two aspirin tablets, crushed and mixed with cold cream or chloroform (15 mL) and spread on the painful skin, was reported to be successful in relieving the pain for several hours (King). The effect of nerve root blocks is inconsistent, but this procedure may afford temporary relief. In one randomized trial, the preemptive use of epidural steroids at the onset of the rash had minimal effects (van Wijck et al). It should be emphasized that postherpetic neuralgia eventually subsides even in the most severe and persistent cases but the short-term use of narcotics is appropriate when the pain is severe. Until the pain subsides, the physician must exercise skill and patience and avoid the temptation of subjecting the patient to one of the many surgical measures that have been advocated for this disorder
מה מחייב טיפול דרך הוריד באציקלוביר
1. רמזי האנט
2. אופתלמיק VZV
3. בחור צעיר בן 22 עם מעורבות דרמטומלית T3-T6
4. גבר בן 81 עם מעורבות דרמטום T1
מעורבות של 3 דרמטומים
בן 42, מפתח זוסטר אופתלמיקוס. איזה סיבוך יש להגיד למטופל שיכול להתפתח בהמשך ?
1. אוטם איפסילאטרלי
2. Ramsay hunt
אוטם איפסילטרלי
Among the most important considerations in this disease is the cerebral arteritis caused by varicella zoster virus of the ophthalmic division of the trigeminal nerve; it simulates in imaging appearance granulomatous arteritis and giant cell arteritis ,
בן 64, מפתח הפרעה בשינה ותגובת בהלה מוגזמת .
(Startle)
באיזו מחלה יש לחשוד?
1. FTD
2. LBD
3. Prion
4. Hepatic encephalopathy
Prion
בת 68 הסובלת מירידה קוגניטיבית מהירה תוך 4 חודשים עם שינויים התנהגותיים בולטים והפרעה ניכרת בשנת לילה.
בבדיקה בוהה, מדי פעם קפיצות בידיים, הפרעת קוארודינציה וביציבות, פרסטזיות ותחושת גרד בגפיים. איזה מרצפי ה-MRI
יעזור באבחנה?
1. T1
2. Gradient Echo
3. DWI
4. T1 with gadollinum
5. SWI
DWI
MRI of the brain has now been appreciated to show hyperintensity of the lenticular nuclei on T2-weighted and diffusion-weighted images in the basal ganglia and cortex when the disease is fully established (Fig. 32-5). Long contiguous segments of the cortex, as well as various parts of the basal ganglia, show these alterations in a pattern that is characteristic and mistakable only perhaps for the appearance of diffuse cerebral anoxia. According to Shiga and colleagues, these changes occur in 90 percent of cases (cortex more often than caudate or lenticular nuclei and sometimes both),
בן 64 החל לסבול מעייפות, שינויי התנהגות, רגזנות וירידה קוגניטיבית, אשר מחמירים במהירות על-פני
השבועות האחרונים. בהמשך שינויים בתפיסה חזותית ותנועות מיוקלוניות באצבעות, שהתפשטו גם
לקבוצות שרירים אחרות.
מהו הממצא השכיח ביותר שניתן לצפות
ב- MRI?
א. אות גבוה של גרעין לנטיפורם ושל רצועות קורטקס ארוכות בסדרות T2W ו- DWI
ב. נגעים קטנים מרובים בעלי אות גבוה בחומר הלבן ב- T2W שעוברים האדרה טבעתית
ג. נגעים מתמזגים גדולים בחומר הלבן ובגזע המח שאינם עוברים האדרה
) confluent (
ד. אות גבוה דיפוזי באונות הטמפורליות התחתונות ב- T2W עם בצקת והאדרה קורטיקלית
אות גבוה של גרעין לנטיפורם ושל רצועות קורטקס ארוכות בסדרות
T2W ו- DWI
MRI of the brain has now been appreciated to show hyperintensity of the lenticular nuclei on T2-weighted and diffusion-weighted images in the basal ganglia and cortex when the disease is fully established (Fig. 32-5). Long contiguous segments of the cortex, as well as various parts of the basal ganglia, show these alterations in a pattern that is characteristic and mistakable only perhaps for the appearance of diffuse cerebral anoxia. According to Shiga and colleagues, these changes occur in 90 percent of cases (cortex more often than caudate or lenticular nuclei and sometimes both),
מהו פריון ?
א. חלקיק וירואידי עם חומצות גרעין
ב. חלבון ברקמת שריר
ג. חלקיק פתוגני שמקודד על ידי גן בכרומוזום 20
חלקיק פתוגני שמקודד על ידי גן בכרומוזום 20
the transmissible pathogen is a proteinaceous infectious particle that is devoid of nucleic acid, resists the action of enzymes that destroy RNA and DNA, fails to produce an immune response, and electron microscopically does not have the structure of a virus. To distinguish this pathogen from viruses and viroids, Prusiner introduced the term “prion.” The very same prion protein (PrP) is normally encoded by a gene on the short arm of chromosome 20.
איזה מהבאים אינה מחלת פריונים ?
א. CJD
ב. BSE
ג. Gerstmann-Straussler
ד. Scrapie
ה. Vogt-Kayanagi-Harada
Vogt-Kayanagi-Harada
אינה מחלת פריונים.
Vogt-Koyanagi-Harada disease adds dysacusis, tinnitus, and sensorineural deafness to the usual manifestations of vitiligo of the eyebrows, poliosis (depigmented forelock of hair), iritis, retinal depigmentation, and recurrent meningitis.
Recurrent meningitis is associated with iridocyclitis and depigmentation of the hair and skin (poliosis and vitiligo). The CSF in these recurrent types may contain large numbers of lymphocytes or polymorphonuclear leukocytes but no bacteria
and the glucose content is not reduced.
—–
Scarpie- spongiform of sheep
בן 60 עם ירידה קוגניטיבית מהירה במהלך של חודש. בבדיקתו מיוקלונוס ב 4- גפיים וסימנים
פירמידליים וצרבלריים. אביו נפטר ממחלה דומה בגיל 60 . איזה מהתרשימים הבאים אופייני
במחלתו ?
תשובה ג.
מהעושהHHV?6
1. לימביק אנצפליטיס לאחר השתלה
2. cerebellar degeneration
3. retinal degeneration
4. aseptic meningitis
לימביק אנצפליטיס לאחר השתלה
Table 32-2_NEUROLOGIC COMPLICATIONS OF HIV-AIDS
מהם מאפייני המיאלופתיה של HIV
IRIS
Immune reconstitution inflammatory syndrome (IRIS) A special comment should be made regarding the transient but sometimes severe clinical worsening of PML that may occur during the initial treatment of HIV infection with antiretroviral drugs. This syndrome has been attributed to the emergence of acute inflammation surrounding the demyelinating lesions as a result of reconstitution of the immune system (immune reconstitution inflammatory syndrome or IRIS that has been mentioned in the section on the treatment of HIV). In support of this mechanism, there is a parallel blossoming of the lesions with gadolinium enhancement on MRI. Treatment with corticosteroids has been suggested and is said to allow survival and temporary remission of PML, although we have seen at least one dramatic exception. Judicious use of corticosteroids is implemented to mute this reaction
AidsDementia
HIV-Encephalopathy-Dementia
In the later stages of HIV infection, the most common neurologic complication is a subacute or chronic HIV encephalitis presenting as a form of dementia; formerly it was called AIDS encephalopathy or AIDS dementia complex but it is now generally referred to as HIV encephalopathy. It has been estimated that only 3 percent of HIV cases present in this manner, but the frequency is far higher, close to two-thirds, after the constitutional symptoms and opportunistic infections of AIDS have become established. In children with AIDS, dementia is more common than all opportunistic infections, more than 60 percent of children eventually being affected.
:HIV Dementia complex
* Subautely progressive dementia accompanied by abnormalities of motor function.
Ataxia, incoordination of limbs, impaired smooth pursuit and saccadic eye movements.
Paraplegia, Positive babinski, Front reflexes, Bladder and bowel incontinence, Abulia, Mutism.
-
CSF may be normal or slight protein increase less frequently with mild pleocytosis
HIV can be isolated from CSF - MRI – patchy confluent diffuse white matter changes with ill defined margins, enlarged ventricles
** *Pathology**: multifocal rareification, a few foamy macrophages and multinucleated giant cells.
** *Treatment** with antiretroviral drugs improves symptoms
** HIV MYELOPATHY:
** *Vacuolar degeneration resemblance to subacute combined degeneration with vacuolar degeneration
** *Peripheral neuropathy** – distal symmetrical, axonal, predominantly sensory and dysesthetic.
** *Mononeuropathy multiplex** – painful
- Polyradiculitis – cauda equina - caused by CMV
- GBS/CIDP like
- Facial palsy
- Inflammatory polymyositis – primary myopathy
NSAIDS MENINGITIS
SLE:
Wide manifestations:
Seizures
CN invovlemet
Hemiparesis
Paraparesis
Aphasia
Homonymous hemianopia
Chorea
Emboli (Libman Sacks)
Longtitudinally extensive myelopathy
Lupus sclerosis – white matter changes
Peripheral neuropathy
Vascular injury due to attachment of immune complexes to vessel wall
APLA
IX: ANA
Anti Ds DNACSF commonly normal – or increased protein
אסימטרים
PML:
* Widespread demyelinating lesions mainly of cerebral hemispheres, sometimes of brainstem and cerebellum.
* Vary in size – microscopic to massive multifocal zones of destruction of both myelin and axons
* Abnormalities of glia – deformed bizarre shaped nuclei and mitotic figures, nuclei of oligodendorcytes contain inclusions.
* PML develops in patients with AIDS, neoplasm, immunodeficiency states, CLL, Hodgkins, Myeloproliferative diseases, TB, Sarcoid.
Immunosuppresive drugs – renal transplant, MS (Tysabri – Natalizumab)
* S&S:
Personality changes, intellectual impairement. Hemiparesis, quadriparesis, visual field defects, cortical blindness, aphasia, ataxia, dysarthria, dementia, confusional state and coma, cerebellar syndrome. Death in 3-6 months.
* CSF normal
* Diagnosis – Detection of JC Virus
* MRI features of PML:
T1 C+ (Gd): typically there is no enhancement. enhancement can be seen in PML-IRIS, AIDS with HAART, and in patients on natalizumab. when enhancement is present, it may be associated with improved survival
PML
PML:
* Widespread demyelinating lesions mainly of cerebral hemispheres, sometimes of brainstem and cerebellum.
* Vary in size – microscopic to massive multifocal zones of destruction of both myelin and axons
* Abnormalities of glia – deformed bizarre shaped nuclei and mitotic figures, nuclei of oligodendorcytes contain inclusions.
* PML develops in patients with AIDS, neoplasm, immunodeficiency states, CLL, Hodgkins, Myeloproliferative diseases, TB, Sarcoid.
Immunosuppresive drugs – renal transplant, MS (Tysabri – Natalizumab)
* S&S:
Personality changes, intellectual impairement. Hemiparesis, quadriparesis, visual field defects, cortical blindness, aphasia, ataxia, dysarthria, dementia, confusional state and coma, cerebellar syndrome. Death in 3-6 months.
* CSF normal
* Diagnosis – Detection of JC Virus
* MRI features of PML:
T1 C+ (Gd): typically there is no enhancement. enhancement can be seen in PML-IRIS, AIDS with HAART, and in patients on natalizumab. when enhancement is present, it may be associated with improved survival
optic nerve לא אופייני
PML is 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.
Clinical Features
An uncommon disease of late adult life, PML usually develops in a patient with a neoplasm or chronic immunodeficiency state. The large majority of cases are now observed in patients with AIDS in whom the incidence of PML approaches 5 percent. Viewed from another perspective, more than 75 percent of cases of PML in the current era are associated with HIV. Indeed, the incidence is so much higher than in any other form of immunosuppression that an interaction between HIV and the causative virus of PML has been suggested. Other important associations are with chronic neoplastic disease (mainly chronic lymphocytic leukemia, Hodgkin disease, lymphosarcoma, and myeloproliferative disease) and less often, with nonneoplastic granulomatosis, such as tuberculosis or sarcoidosis. A number of cases occur
in patients receiving immunosuppressive drugs for renal transplantation, multiple sclerosis (see Chap. 36), or for other reasons.
Personality changes and intellectual impairment may introduce the neurologic syndrome, which then evolves over a period of several days to weeks. Any one or some combination of hemiparesis progressing to quadriparesis, visual field defects, cortical blindness, aphasia, ataxia, dysarthria, dementia, confusional states, and coma are Manifestations. Some of the cases under our observation had a predominantly cerebellar syndrome. Seizures are infrequent, occurring in only about 10 percent of cases. In most cases, death occurs in 3 to 6 months from the onset of neurologic symptoms and even more rapidly in patients with AIDS unless aggressive antiretroviral treatment is undertaken. The CSF is usually normal. CT and MRI localize the nonenhancing demyelinating lesions with clarity (Fig. 33-4) but the variability in size, location, and multiplicity make the diagnosis more dependent on viral DNA isolation from CSF and on the context of Immunosuppression.
ליסטריה עושה מנינגיטיס חיידקית במדוכאי חיסון
Figure 32-1. Herpes simplex encephalitis. A. T2-FLAIR coronal MRI, taken during the acute stage of the illness. There is increased signal in
the inferior and medial temporal lobe and the insular cortex of the left hemisphere. B. T1-weighted image after gadolinium infusion showing
enhancement of the left insular and medical temporal cortices (arrows).
Figure 32-2. MRI of HIV leukoencephalopathy. There are large areas of white matter change that underlie one form of AIDS dementia;
cortical atrophy and ventricular enlargement are evident.
Figure 32-3. MRI of the cervical cord in a patient with nonpoliovirus poliomyelitis and an asymmetrical, flaccid, bibrachial paralysis. There is T2 signal change in the anterior regions of the gray matter.
Figure 32-4. Progressive multifocal leukoencephalopathy (PML). MRI, T2-FLAIR, demonstrates multiple subcortical white matter lesions in both hemispheres (A) and in the left pons (B) in a 31-year-old man with HIV. The lesions did not enhance.
Figure 32-5. MRI showing T2 signal changes in the striatum in a patient with sporadic CJD (left) of 1 month’s duration. DWI sequence showing
restriction of diffusion in contiguous bands of cortex and in the striatum (right) in the same case.
