others Flashcards

Question

כל הממצאים הבאים מלווים תסמונת Shy drager syndrome פרט ל : א. Orthostatic hyppotension ב. Erectile dysfunction ג. Laryngeal dysphonia ד. Gastric dilatation ה. Urinary incontinence

Answer 1

ד. gastric dilatation In addition to **orthostatic hypotension**, other features of **autonomic failure include erectile dysfunction loss of sweating, dry mouth, miosis, and urinary retention or incontinence, fecal incontinence**. ***Vocal cord palsy is an important and sometimes initial manifestation of the disorder***; it may cause dysphonia or stridor and airway obstruction requiring tracheostomy. A **dusky discoloration of the hands** as a sign of this disorder was ascribed to poor control of cutaneous blood flow

Answer 2

More than half of the patients with striatonigral degeneration have orthostatic hypotension, which proves at autopsy to be associated with **loss of intermediolateral hom cells and pigmented nuclei of the brainstem**

Answer 3

myotonic dystrophy- CTG

Answer 4

2. פרינאו- לא גורם לניסטגמוס downbeat

Answer 5

APLA לא גורמת להיפרוויסקוסיטי In states of **anemia, polycythemia, thrombocythemia,extreme hyperlipidemia, hyperviscosity from macroglobulinemia, sickle cell anemia, and extreme hyper- or Hypoglycemia**, there may be transient neurologic deficits related to rheologic or other changes in blood, as already mentioned. In some of these cases, the metabolic or rhealogic change appears to have brought out symptoms of **stenosis in a large or small vessel, but just as often the vasculature is normal**. ***Patients with antiphospholipid antibodies may have TIAs, the mechanism of which is undefined.***

Answer 6

תשובה 5- CSF חלבון גבוה ב- * In young persons, the most common cause of **hypercalcemia** is hyperparathyroidism (either primary or secondary); in older persons, osteolytic bone tumors, particularly meta-static carcinoma and multiple myeloma, are often causative. Less-common causes are vitamin D intoxication, prolonged immobilization, hyperthyroidism, sarcoidosis, and decreased calcium excretion (renal failure). **Anorexia, nausea and vomiting, fatigue, and headache are usually the initial symptoms, followed by confusion (rarely a delirium) and drowsiness, progressing to stupor or coma in untreated patients. A history of recent constipation is common. Diffuse myoclonus and rigidity occur occasionally, as do *elevations of spinal fluid protein (up to 175 mg/100 mL). Convulsions are uncommon.*** * **Hypocalcemia** The usual manifestations **are paresthesias, tetany, and seizures. With severe and persistent hypocalcemia, altered mental status in the form of depression, confusion, dementia, or personality change** can occur. Anxiety to the point of panic attack is also known. Even coma may result, in which case there may be **papilledema as a result of increased intracranial pressure**. Aside from the raised pressure, the **CSF shows no consistent abnormality**. This increase in intracranial pressure may be manifest by headache and papilledema without altered mentation or with visual obscurations.

Answer 7

אוליגודנדרוגליומה אנפלסטית Of considerable importance is that many oligodendrogliomas, especially anaplastic ones, respond impressively to chemotherapeutic agents.

Answer 8

1. מקושר עם גידולי ריאה- זו התשובה הלא נכונה. IBM- אין גידול ואין טיפול

Answer 9

2. קרונס. **bilateral facial paralysis** (facial diplegia) is most often a manifestation of the **Guillain-Barre syndrome** (GBS) and may also occur in **Lyme disease** and rarely, with **HIV infection**. There are numerous other causes of bilateral facial palsy, all of them infrequent. Keane (1994) listed the idiopathic (now presumably mainly viral) variety, GBS, and **meningealinfiltration by tumor** as the most common causes, but also found two cases of **syphilis** among 43 patients. The bilateral syndrome has been reported in approximately 7 of every 1,000 patients with **sarcoidosis**, although our impression is that it is more frequent. When acute in onset and associated with parotid gland swelling from sarcoidosis, it has been referred to as uveoparotid fever, or Heerfordt syndrome. In typical cases of sarcoidosis, the paralysis on each side tends to be temporally separated by weeks or more. **Mononucleosis** may affect both sides of the face almost simultaneously; this is probably a form of GBS. Bifacial palsy is also a feature of the developmental disorder, **Mobius syndrome** (see Chap. 38). Less common is the **Melkersson-Rosenthal syndrome**, consisting of the triad of recurrent facial paralysis, facial (particularly labial) edema, and less constantly, plication of the tongue. The syndrome begins in childhood or adolescence and may be familial. Biopsy of the lip or skin may reveal a granulomatous inflammation. The cause is not known and, despite the cardinal feature of angioneurotic edema, complement levels are normal. A series of biopsied cases has been reported by Elias and colleagues. **Kennedy syndrome,** causes bifacial weakness in addition to bulbar palsy as the disease progresses; preceding facial fasciculations are characteristic. **Facioscapulohumeral muscular dystrophy**, as the name implies, incorporates facial weakness but would not be mistaken for Bell's palsy (see Chap. 48). The same is true for the rare form of **amyloidosis** associated with crystal lattice deposits in the cornea that typically involves both facial nerves. Causes of recurrent Bell's palsy have been listed earlier and are surrunarized by Pitts and colleagues ** leprae**- unilateral facial

Answer 10

3. מפחית את כמות הרצפטורים

Answer 11

יל"ד לא מעלה CBF באופן נורמאלי. PCO2- וזודילאטור בכמות גבוהה PO2- ירידה בו יוצרת וזודילאטציה אדנוזין וזודילאטור

Answer 12

1. Marchiafava bignami disease

Answer 13

D3 the junction between th vertebral spinal and aortic circulation typically lies at the T2-T3 segment but mist ischemic lesions lie well below this level

Answer 14

קונאוצרבלר

Answer 15

לא נראה חדרים מוגדלים ב MRI

Answer 16

המוח לא כולו מכוסה במחסום דם מוח- Areas which contain fenestrated capillaries, and thus lack the blood brain barrier, are: circumventricular organs (CVO) area postrema median eminence subforniceal region posterior pituitary gland pineal gland organum vasoculosum lamina terminalis choroid plexus dura mater

Answer 17

אינו חוצה BBB METOCLOPRAMIDE & DOMPERIDONE Metoclopramide and domperidone are **dopamine D2-receptor antagonists**. Within the gastrointestinal tract activation of dop- amine receptors inhibits cholinergic smooth muscle stimulation; blockade of this effect is believed to be the primary prokinetic mechanism of action of these agents. These agents increase esoph- ageal peristaltic amplitude, increase lower esophageal sphincter pressure, and enhance gastric emptying but have no effect on small intestine or colonic motility. **Metoclopramide and domperi- done also block dopamine D2 receptors** in the chemoreceptor trigger zone of the medulla **(area postrema)**, resulting in potent antinausea and antiemetic action.

Answer 18

mycoplasma does not cause recurrent meningitis The causes of recurrent aseptic meningitis are numerous. Many are given in table 1, but this list can be bewildering when applied to the management of individual patients. It is better to group the causes as follows, to avoid missing pertinent investigations: * **Infection**—Chronic infective meningitis may present with recurrent clinical episodes separated by asymptomatic periods, even though the CSF is abnormal throughout. This pattern may be seen with syphilis, brucellosis, Lyme disease, fungal meningitis, and human immunodeficiency virus (HIV). With other organisms, a different mechanism of *genuine reactivation* of latent infection applies. The prime examples here are herpes simplex virus (HSV), Epstein-Barr virus (EBV), and Toxoplasma. * **Structural lesions**—Recurrent chemical meningitis occurs when structural lesions along the neuraxis periodically discharge their contents into the CSF. This phenomenon is well known for craniopharyngiomas, epidermoid cysts, and gliomas, but other causes have been described. * **Drugs**—Many drugs are associated with a risk of recurrent aseptic meningitis (table 3). This list is far from exhaustive—isolated case reports have implicated numerous other agents. * **Chronic inflammatory diseases** (and other non-infective causes)—Recurrent aseptic meningitis is a recognised feature of sarcoidosis, lupus, and other connective tissue disorders, vasculitis, Behçet’s disease, and recurrent hereditary polyserositis. Vogt-Koyanagi-Harada syndrome is a very rare condition in which aseptic meningitis is associated with sensorineural deafness, uveitis, retinal haemorrhages, and skin and hair depigmentation. Migraine may rarely present with fever, symptoms suggestive of meningo-encephalitis, and CSF pleocytosis, but this must be a diagnosis of exclusion.

Answer 19

הרעלת אלכוהול אינה גורמת לSIADH of the syndrome of inappropriate antidiuretic Hormone secretion (**SIADH**). This state is of special importance because it **complicates neurologic diseases of many Types**: **head trauma, *bacterial meningitis* and encephalitis, cerebral infarction, subarachnoid hemorrhage, cerebral and systemic neoplasm, *Guillain-Barre syndrome* and the effects of certain medications**. SIADH is the result of excretion of urine that is hypertonic relative to the plasma. . . As the hyponatremia develops, there IS a decrease in alertness, which progresses through stages of confusion to coma, often with convulsions. As with many other metabolic derangements, the severity of the clinical effect is related to the rapidity of decline in serum Na . Lack of recognition of this state may allow the serum Na to fall to dangerously low levels, 100 mEq/L or lower. Treatment Most instances of hyponatremia have developed slowly, allowing for maintenance of brain volume by the extrusion from cells of various osmothic substances. Rapid correction of sodium in these circumstances risks a reversal of osmotic gradient and a reduction in brain volume. This, in turn, is associated with a special type of central nervous system demyelination ("osmotic demyelination" and central pontine myelinolysis)

Answer 20

**Wegener Granulomatosis** (C-ANCA) 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*. 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

Answer 21

FIRDA לא קיים בשלב 4 של השינה **FIRDA is a rhythmic 2 to 3 Hz delta frequency activity with an amplitude of 50–100 mv that predominates in the bilateral frontal regions of the waking adult electroencephalogram (EEG)**. The waves are usually regular with a **sinusoidal pattern**. FIRDA usually occurs in **short bursts lasting 2 to 6 seconds** and must be differentiated from slow eye blink artifact Figure 2. The electrooculogram (EOG) electrodes can aid in the differentiation of the two. FIRDA, unlike eye blink artifact, **may have posterior field extension**. It is *attenuated by alerting or eye opening* and **accentuated by eye closure, hyperventilation, drowsiness, and stage N1 sleep**. It *disappears with the onset of Stage N2 sleep* but may **reappear during REM** sleep. If diagnostic uncertainty is present, a full EEG montage should be used and the EEG recording should include 20 minutes during wakefulness, followed by 3 minutes of hyperventilation and standard intermittent light stimulation to adequately document FIRDA.

Answer 22

מדולובלסטומה ללא נטייה להסתיידות Calcifications found in Astrocytomas, Oligodendroglionas, Ependymoomas and Meningiomas, pituitary adenoma, craniopharyngioma, Hand-Schuller-Christian disease, and sarcoidosis.

Answer 23

**Disconnection syndromes**: * **Conduction aphasia**: The Wernicke area in the temporal lobe is separated from the Broca area, presumably by a lesion in the arcuate fasciculus or external capsule or subcortical white matter. However, most often the lesion is in the supramarginal gyrus. * **Sympathetic apraxia**: loss of fibers that connect the left and right motor association cortices, a lesion in the more anterior parts of the corpus callosum or the subcortical white matter underlying Broca area and contiguous frontal cortex causes an apraxia of commanded movements of the left hand. * **Pure Word Deafness**: loss of ability to discriminate speech sounds. Subcortical Lesion of left temporal lobe, spanning wernickes and fibers that cross corpus callosum From opposite side. Failure to activate left auditory language areas. Also in bilateral lesions of auditory cortex. * **Alexia without Agraphia**: splenium of corpus callosum and left occipital lobe with loss of Connection of visual fibres to left hemisphere language areas.

Answer 24

הפרעה במטבוליזם של חומצות שומן (קטונים נמוכים!) Although it has long been known that lipids are an important source of energy in muscle metabolism (along Wlth glucose), it was only in 1970 reported the abnormal storage of lipid in muscle fibers attributable to a defect in the oxidation of long-chain fatty Acids. Despite the many biochemical abnormalities that have been identified in the fatty acid metabohc pathways, there are essentially **3 clinical patterns** by which these defects are expressed: 1. One constellation of symptoms referred to as the **encephalopathic syndrome** has its onset in **infancy or early childhood**. Its very first manifestation may be sudden death (sudden infant death syndrome[**SIDS**]), or there may be **vomiting, lethargy and coma, *hepatomegaly, cardiomegaly*, muscular weakness**, and ***hypoketotic hypoglycemia, with prominent Hyperammonemia***, that is, a **Reye-like syndrome**. Undoubtedly, instances of this syndrome have not been recognized as abnormalities of fatty acid metabolism but have been designated incorrectly as the Reye syndrome or as SIDS. 2. A second (**myopathic**) syndrome appears in **late infancy, childhood, or adult** life and takes the form of a **progressive myopathy, with or without cardiomyopathy**. The myopathy may follow episodes of **hypoketotic Hypoglycemia** or may develop de novo. 3. The third syndrome is one that usually begins in the **second decade of life** and is induced by a **sustained period of physical activity or fasting**. It is characterized by repeated episodes of **rhabdomyolysis with or without myoglobinuria**.

Answer 25

קמפילובקטר עושה גיליאן ברה ולא מיאליטיס. 1. **מיאליטיס ויראלי ** * אנטרווירוסים כולל פוליו וקוקסקי * HSV,VZV * CMV,EBV * ארבווירוסים * כלבת 2. **מייאליטיס חיידקי** * lyme * סיפיליס * TB * לגיונלה 3. מייליטיס פרזיטרי- שכיסטוזומה בעיקר.

Answer 26

suprachiasmatic nucleus The **cyclic nature of the attacks** has been linked to a hypothalamic mechanism that governs the circadian rhythm. At the onset of the headache, the region of the suprachiasmatic nucleus appears to be active on PET (May et al). Hypothalamic activation has also been found in migraine, SUNCT, chronic paroxysmal hemicrania, and hemicrania continua. Moreover, stimulation of the hypothalamus has proved effective, although highly experimental, in stopping chronic cluster headache and SUNCT

Answer 27

התלבטות בין 1 ל-2. נטייה יותר חזקה ל-2 . פירוט המסיחים: 1. **FMR1- fragile X**- the most common inherited forms of *developmental delay*, estimated to occur in 1 of every 1,500 male live births and accounting for *10 percent of severe developmental delay in males*. Females, with two X chromosomes, are affected about half as frequently, and then only to a slight degree. Unusual site of frequent breakage (“**fragility**") on the X chromosome and related it to a syndrome that included **developmental delay, flaring ears, elongated facies, slightly reduced cranial perimeter, normal stature, and enlarged testes**. ***More recently, rare progressive ataxia has been reported in adults who harbor the chromosomal abnormality and had displayed little or no cognitive deficiency***. The chromosomal fragility appears to be due to a heritable, unstable **CGG** repeating sequence in the X chromosome. **Affected individuals have over 230 repeats and carriers have 60 to 230 repeats.** The prolonged sequence **inactivates a gene (FMR1)** that codes for an RNA-binding protein of as yet an unknown connection to brain function. **the fragile X alteration occasionally turns up in mentally normal males; in some instances, the male children of their daughters have the disease.** In some of the cases we have observed, the intellectual deficit has been *mild in degree* and the main abnormalities have taken the form of *troublesome behavior, logorrhea, an autistic type of gaze aversion, and asociality*. a curious form of **progressive adult onset *ataxia and tremor*, previously thought to be of a degenerative type, has been discovered to be caused by a "premutation“** of the fragile X gene (50 to 200 trinucleotide repeats). - an **unusual variant** of the degenerative process with onset in **mid- or late adulthood**, mainly but not exclusively in men, and consisting of **gait or limb ataxia and mild tremor**. The process affects **carriers of a "premutation“ who have 50 to 200 CGG repeat sequences in the FMRl gene**. In contradistinction to the full mutation of over 200 repeats, there is apparently a buildup of messenger ribonucleic acid (mRNA) in the adult form that interferes in some way with cellular function. Aggregating several studies, the frequency of this genetic abnormality among otherwise unassignable adult ataxia cases is less than 10 percent. Some of these patients have **characteristic symmetrical signal MRI changes in the *middle cerebellar Peduncles* in the T2 sequence.** 2. **Frataxin – Friedrich Ataxia**- - progressive **spinocerebellar ataxia** - accounts for about half of all cases of hereditary ataxias. - its incidence among Europeans and North Americans is 1.5 cases per 100,000 per year - The pattern of inheritance is **autosomal recessive**. - Genetic linkage studies led to the assignment of the gene mutation to **chromosome 9q13-2** in virtually all cases the mutation is an expansion of a **GAA** trinucleotide repeat within a gene that codes for the protein frataxin, (within an intron). the consequence is a **reduction in levels of frataxin and loss of its function**. **Ataxia of gait is nearly always the initial running symptom**. Difficulties in standing steadily and in are early symptoms. The **hands usually become clumsy months or years after** the gait disorder, and **dysarthric speech appears after the arms** are involved (this is rarely an early symptom). Exceptionally the ataxia begins rather abruptly after a febrile illness, and one leg may become clumsy before the other. In some patients, **pes cavus and kyphoscoliosis** (scoliosis) are evident well before the neurologic symptoms; in others, they follow by several years. The characteristic foot deformity takes the form of a **high plantar arch** with retraction of the toes at the metatarsophalangeal joints and flexion at the interphalangeal joints **(hammertoes)**. A notable feature in more than half of patients is a **Cardiomyopathy**. Many of the patients die as a result of **cardiac arrhythmia or congestive heart failure**. For this reason, it is essential that individuals have a cardiologic assessment. **Kyphoscoliosis and restricted respiratory function** are additional important contributory causes of death. 10 percent of these patients have diabetes mellitus and a higher proportion have impaired glucose tolerance; there is both **insulin deficiency and peripheral insulin resistance**. In the **fully developed syndrome**, the abnormality of gait is of **mixed sensory and cerebellar type**. The patient stands with feet wide apart, constantly shifting position to maintain balance. Positive Romberg sign. Attempts to correct the imbalance may result in **abrupt, wild movements**. Often there is a **rhythmic tremor of the head.** Eventually, the **arms are grossly ataxic, and both action and intention tremors are manifest**. **Speech is slow, slurred, explosive, and, finally, almost incomprehensible** Breathing, speaking, swallowing, and laughing may be so incoordinated that the **patient nearly chokes while speaking** Although mentation is generally preserved, **emotional lability** has been sufficiently prominent **The tendon reflexes are abolished** in nearly every case. 3. **Ataxin 3 – SCA3 – Machado-Joseph** * A special form of **hereditary ataxia with brainstem and extrapyramidal signs** has been described in patients mainly, but not exclusively, of Portuguese-Azorean origin. * **Autosomal dominant** pattern of inheritance * Slowly progressive **ataxia beginning in adolescence or early adult life in association with *hyperreflexia, extrapyramidal features, dystonia, bulbar signs, distal motor weakness, and ophthalmoplegia**. * There is usually **no impairment of intellect** and in the examples the authors have seen, the extrapyramidal symptoms were mainly rigidity and slowness of movement. * **Early Machado-Joseph disease characteristically** demonstrates the finding of **dysmetric horizontal and vertical saccades**, even before the ataxia is obvious. * Postmortem examination discloses a **degeneration of the dentate nuclei** and **spinocerebellar tracts** and a loss of **anterior horn cells** and neurons of the **pons, substantia nigra, and oculomotor nuclei**. * Unstable number of **CAG repeating sequences in a gene, ataxin-3, spinocerebellar ataxia type 3 (SCA3)**. * the **MRl** findings are of **reduced width of the *superior and middle cerebellar peduncles*, atrophy of the frontal and temporal lobes, and smallness of the pons and globus pallidus**. ## Footnote מקרא לציורים: * ירוק –כוראה * צהוב –אטקסיה * כחול :סימנים פירמידליים * שוט –שמחזיקים-AD שנחבטים –AR * משקפיים –עיוורון * אוזניות-פגיעה בשמיעה * גפיים בכתום-פולינירופתיה * כובע-ורוד-שינוי התנהגות * צירים –EPS * ברקים כחולים –מיוקולוניות * שן אחת –דמנציה * שפם –גנטיקה XLINKED * אדום –מיופתיה? * דגנר\קטיבי – כחול טאופאטיה ,צהוב סינקלופתיה * פסים ורודים –דרמטולוגיה * אדום ורוד -פוליניורופתיה

Answer 28

פתיחת תעלות נתרן

Answer 29

יציאת אשלגן מהתא

Answer 30

eclampsia= magnesium

Answer 31

1.** flexor carpi radiallis**- Median nerve 2. extensor pollicis longus- posterior interosseous nerve 3. abductor pollicis longus- posterior interosseous nerve 4. supinator- posterior interosseous nerve

Answer 32

ventral tegmentum of midbrain= substantia nigra= dopaminergic locus ceroleus- pons= noradrenergic tuberomammillary nucleus (hypothalamus)= histaminergic raphe nucleus (median part of brainstem)= serotonergic

Answer 33

אצטזולמיד ודקסמטזון

Answer 34

טאע זקס ואלפא הקסוזאמינידז

Answer 35

אספירין This rare disorder of the microvasculature produces a **burning pain and bright red color change, usually in the toes and forefoot and sometimes in the hands, precipitated by changes in ambient temperature** Most cases are **idiopathic**, some **familial and inherited as a dominant** trait. There are **secondary forms** of the disease, the most important one being that associated with **essential thrombocythemia** (up to 25 percent of patients may have erythromelalgia as the first symptom) but also with other **myeloproliferative disorders** such as polycythemia vera and with collagen vascular diseases, including thrombotic thrombocytopenic purpura (**TTP**), during the use of **calcium channel blockers** and certain **dopaminergic agonists** such as *pergolide and bromocriptine*, and with **occlusive vascular diseases**. Some instances arise as a result of a **painful polyneuropathy that predominantly affects the small sensory fibers**; more often in these latter conditions, the redness and warmth are constant and the result of damage to sympathetic nerve fibers; **The familial form of erythromelalgia has been traced to a mutation in a sodium channel** (NaV 1.7) that is selectively expressed in **dorsal root ganglia nociceptive neurons**. In addition to its inherent value in explaining the manifestations of this disease, the discovery of this channelopathy has evinced interest in novel ways to treat pain by manipulating sodium channels. **Treatment** According to Abbott and to Mitts and others, **aspirin** is useful in the treatment of paroxysms of secondary erythromelalgia and of some primary cases as well

Answer 36

ב-2 יותר מיוזין ATPase * Muscle cells **rich in oxidative enzymes (type 1 fibers**) contain more mitochondria and larger amounts of myoglobin (therefore appearing red), have **slower rates of contraction and relaxation, fire more tonically**, and are **less fatigable** than muscle fibers poor in oxidative enzymes. * The latter (**type 2 fibers**) fire in **bursts** and are used in **quick phasic**, rather than sustained, reactions. * The **amount of myosin ATPase activity, which governs the speed of contraction, is low in oxidative-rich fibers and high in glycolytic-rich fibers.**

Answer 37

miller fisher- opthalmoplegia, areflexia In severe cases, the ocular motor nerves are paralyzed and even the pupils may be unreactive בשביל שבבוטוקס תהיה ארפלקסיה החולשה צריכה להיות כבר severe generalized weakness

Answer 38

strongyloides **Eosinophilic Meningoencephalitis and Meningitis Caused by Parasites** An **eosinophilic meningoencephalitis**, often with **cranial nerve and painful polyradicular findings**, has been reported with **Angiostrongylus cantonensis, Gnathostoma, Paragonimus, and Toxocara canis and cati infections**. In **Angiostrongylus** infections, snails, freshwater prawns, and unwashed lettuce carry the nematode. The resulting illness may last for weeks to months, with **pain, paresthesias, sensorimotor abnormalities, and a confusional state as the main manifestations**. highlighted paresthesias and dysesthesias but noted **eosinophilia in the blood or CSF in only half** of their patients. **Meningeal Hodgkin disease, other lymphomas, and cholesterol emboli also occasionally incite eosinophilic meningitis.** More detailed descriptions of parasitic diseases of the nervous system can be found in the monographs of Bia and of Gutierrez.

Answer 39

Aryl sulfatase- A deficiency

Answer 40

המקלעת הברכיאלית

Answer 41

איבוד ראייה **Cavernous sinus** AKA Foix Jefferson; syndrome of the sphenopetrosal fissure (Bonnet and Bonnet) corresponding in part to the cavernous sinus syndrome of Raeder. **clinical syndromes**: Ophthalmoplegia caused by lesions of the third, fourth, sixth, and often fifth nerves; exophthalmos; vegetative disturbances. Jefferson distinguished three syndromes: (1) the anteriorsuperior, corresponding to the superior orbital fissure syndrome; (2) the middle, causing ophthalmoplegia and lesions of V1 and V2; (3) the caudal, in addition affecting the whole trigeminal nerve. **etiology:** Tumors of the sellar and parasellar area, infraclinoid aneurysms of the internal carotid artery, nasopharyngeal tumors, fistulas of the sinus cavernosus and the carotid artery (traumatic), tumors of the middle cranial fossa, e.g., chondromas, meningiomas, and neurinomas. **Superior orbital fissure**: AKA Rochon Duvigneau; syndrome of the pterygopalatine fossa (Behr) and the base of the orbit (DeJean) commencing with a lesion of the maxillary and pterygoid rami and evolving into the superior orbital fissure syndrome. **clinical symptomes:** Lesions of the third, fourth, sixth, and first divisions of the fifth nerves with ophthalmoplegia, pain, and sensory disturbances in the area of V1; often exophthalmos, some vegetative disturbances. **etiology** Tumors: meningiomas, osteomas, dermoid cysts, giant cell tumors, tumors of the orbit, nasopharyngeal tumors; more rarely, optic nerve gliomas; eosinophilic granulomas, angiomas, local or neighboring infections, trauma.

Answer 42

**merkel= pressure** Golgi= golgi tendon organ= muscle stretch and speed of contraction free nerve endings= force and pain meissner= touch ruffini= stretch pacini= vibration

Answer 43

**object agnosia**= temporal lobe (the what) Balint syndrome- bilateral parietooccipital= * impaired visually directed reaching with hand- optic ataxia * difficulty directing gaze- visual apraxia * simultagnosia

Answer 44

קולחיצין טבלה 45-6

Answer 45

3. פרונאלי פגוע F גל With lesions of the first sacral root (S1), the pain is felt in the midgluteal region, midposterior part of the thigh, posterior region of the calf to the heel, outer plantar surface of the foot, and fourth and fifth toes. Tenderness is most pronounced over the midgluteal region. Paresthesia and sensory loss are mainly in the lower part of the leg and outer toes, and weakness, if present, involves the plantar flexor muscles of the foot and toes, abductors of the toes, and hamstring muscles. The Achilles reflex is diminished or absent in the majority of cases. In fact, loss of the Achilles reflex may be the only objective sign. Walking on the toes is more difficult and uncomfortable than walking on the heels because of weakness of the plantar flexors. The needle EMG study is abnormal, showing fibrillation potentials in denervated muscles after 1 or 2 weeks of symptoms in more than 90 percent of cases according to Leyshon and colleagues but less frequently in other studies. Loss or marked asymmetry of the H reflex is another useful indication of S1 radiculopathy, but this finding simply corroborates the loss of an Achilles reflex. The finding of denervation potentials in the paraspinal muscles (indicating root rather than peripheral nerve lesions) and in muscles that conform to a root distribution is also helpful, but at least 2 or 3 weeks must have elapsed from the onset of root pain for these findings to be present. We emphasize that, while useful information is gained from EMG, it is not routinely required and often provides mainly corroborating data. פלסטר= פלנטר פלקסציה ל= לטרל סנסיישן ס= S1 ט= גלו-ט-אוס ר= רפלקס

Answer 46

ממילרי בודי לזיות בו לא עושות פגיעה משמעותית בזכרון (תחשבו על וורניקה לפני אנצפליטיס)

Answer 47

5. רמות אלפא- פטו פרוטאין ואצטילכולין אסטרז בנסיוב האם מוגברות

Answer 48

4. עיכוב האנזים acethylcholinesterase Considerable interest was created in the late 1970s by the finding of a marked reduction in choline acetyltransferase (ChAT) and acetylcholine in the hippocampus and neocortex of patients with Alzheimer disease. This loss of cholinergic synthetic capacity was attributed to a reduction in the number of cells in the basal forebrain nuclei (mainly the nucleus basalis of Meynert), from which the major portion of neocortical cholinergic terminals originate (Whitehouse et al, 1981). However, a 50 percent reduction in ChAT activity has been found in regions such as the caudate nucleus, which shows neither plaques nor tangles. The specificity of the nucleus basalis cholinergic Alzheimer brain also shows a loss of monoaminergic neurons and a diminution of noradrenergic, gabanergic, and serotonergic functions in the affected neocortex. The concentration of amino acid transmitters, particularly of glutamate, is also reduced in cortical and subcortical areas (Sasaki et al) and the concentration of several neuropeptide transmitters—notably substance P, somatostatin, and cholecystokinin are likewise low—but it has not been determined whether any of these biochemical abnormalities, including the cholinergic ones, are primary or secondary to heterogeneous neuronal loss. Nevertheless, the administration of cholinomimetics—either acetylcholine precursors (e.g., choline or lecithin), degradation inhibitors (e.g., physostigmine), or muscarinic agonists that act directly on postsynaptic receptors—have had a mild and unsustained therapeutic effect (see further under “Treatment”). changes has been questioned for other reasons as well. For one,

Answer 49

היפותירואידיזם

Answer 50

אנצפלופתיה מטבולית

Answer 51

anterior cerebral artery

Answer 52

מלפורמציה על שם כיארי

Answer 53

טיפות פילוקרפין טיפול בגלאוקומה

Answer 54

akathisia תופעת לוואי של דופמינרגיים

Answer 55

primary systemic amyloidosis

Answer 56

cryptococcal meningitis

Answer 57

VEP | לא מעורב בכלל בגזע המוח

Answer 58

low grade astrocytoma

Answer 59

lamotrigine למוטריג'ין מושפע מאסטרוגן אך לא משפיע על אסטרוגן

Answer 60

4. בדיקה לvery long chain fatty acids

Answer 61

טוברקולומה

Answer 62

doxycycline

Answer 63

בנזטרופין אצטיל כולין אחראי על כמות הגאבא שמשתחררת מהסטריאטום אך אינו קשור בבחירת המסלול. כלומר- אם יש לי מצב של היפרקינזיה (דיסטוניה או מייג' סינדרום) באופן טבעי המסלול שמאוקטב יותר הוא המסלול הישיר ולכן בתפקוד תקין של אצטיל כולין הוא גורם לאקטוב של תנועה- במצב זה מתן אנטיכולינרגים יגרום לעיכוב תנועה במצב של ברדיקינזיה (פרקינסון) באופן טבעי המסלול שמאוקטב יותר הוא המסלול הבלתי ישיר ולכן בתפקוד תקין של אצטיל כולין הוא גורם לאקטוב של חוסר תנועה- במצב זה מתן אנטיכולינרגים יגרום לעידוד תנועה

Answer 64

Deltoid לא מעורב ב IBM

Answer 65

Deltoid לא מעורב ב IBM

Answer 66

היפונתרמיה לא גורמת ל restless leg syndrome. טיפול בתופעה זו- דופמין אגוניסט, פרהגבלין

Answer 67

propranolol

Answer 68

amantadine

Answer 69

epineurium

Answer 70

vincristine

Answer 71

Porphyria עושה או דיסטלית או פרוקסימלית

Answer 72

לציין כי גם א וגם ב נכונה.. אם כי במבחן קיבלו רק את ב פגיעה אוניברסלית בכל סוגי התחושה | עמודים 1314 ו1318

Answer 73

איבוד ראייה מתקדם עד עיוורון עם הזמן- כנראה הכי פחות נכון.. הם לא חייבים להתעוור לחלוטין

Answer 74

a problem with light touch and temperature

Answer 75

בפגיעה נרחבת של ה midbrain מופיעים גלים איטיים מסונכרנים דו"צ

Answer 76

corticosteroids מסתבר שבדיסקציה מתן קורטיקוסטרואידים מפחית כאב.

Answer 77

Glut-1- deficiency syndrome

Answer 78

supportive therapy trichonosis לרוב לא מצריך טיפול. אם יש חולשה קשה וכאבים אפשר לתת תיאבנדזול+/- פרדניזון או אלבנדזול או מבנדזול

Answer 79

פעילות יתר פאראסימפטטית ופעילות יתר סימפטית בטא אדרנרגית

Answer 80

נטילת מינון עודף של לורזפם

Answer 81

צניחה של העפעף וחולשת שרירי הנעת העין הם המופע הראשוני בכ50% מהמטופלים

Answer 82

anosmia and/or aguesia

Answer 83

ב+ג אם הפרכוסים נמשכים לאחר מתן העמסת וולפרואט דרך הוריד, בתנאי שגם העמסת לבטיראצטאם לא מפסיקה את הפרכוסים אם הפרכוסים נמשכים לאחר מתן דיאזפם והעמסת וולפרואט

Answer 84

opsoclonus- myoclonus

Answer 85

Large vessel thrombosis

Answer 86

Heat stroke

Answer 87

תנועת גפיים לאחר ניתוק מהמנשם

Answer 88

Left temporal subcortical

Answer 89

Atherosclerosis

Answer 90

משקעי neurofibrilary tangles בכמות גדולה

Answer 91

גיל מתחת ל40 בעת האבחנה

others Flashcards

(131 cards)