פרק 44 Chapter 44 Diseases of the Cranial Nerves Flashcards
Table 44-1
EXTRAMEDULLARY CRANIAL NERVE SYNDROMES
Figure 44-1. Scheme of the trigeminal nuclei and some
of the trigeminal reflex arcs. I, ophthalmic division; II,
maxillary division; III, mandibular division. (Originally
from Ramon y Cajal S: La Textura del Sistema Nervista
del Hombre y los Vertebrados, Madrid, Moya, as adapted
from Carpenter MB, Sutin J: Human Neuroanatomy,
8th ed. Baltimore, Williams & Wilkins, 1982, by
permission.)
Figure 44-2. Left-sided facial sensory loss due to demyelination of
the trigeminal root entry zone in a patient with multiple sclerosis.
Abnormal enhancement of the nerve root is seen on T1 postgadolinium
MRI.
Figure 44-3. Scheme of the seventh cranial (facial) nerve. The motor fibers are represented by the solid purple line originating in the motor nucleus of VII. Parasympathetic fibers are represented by regular dashes; special visceral afferent (taste) fibers are represented by long dashes and dots. A, B, and C denote lesions of the facial nerve at the stylomastoid foramen, distal to the geniculate ganglion, and proximal to the geniculate ganglion. Disturbances resulting from lesions at each of these sites are described in the text. (From Carpenter MB, Sutin J: Human Neuroanatomy, 8th ed. Baltimore, Williams & Wilkins, 1982, by permission.)
Figure 44-4. Anatomic features of the vagus
nerve. Note the relationship to the spinalaccessory
and glossopharyngeal nerves at the
jugular foramen and the long course of the left
recurrent laryngeal nerve, which is longer than
the right and hooks around the aortic arch (not
shown).
Figure 44-5. Nasopharyngeal carcinoma invading the anterior left side of the base of the skull and nasopharynx and causing third and fifth nerve palsies. Axial CT of the anterior skull base.
Table 44-2
CAUSES OF EXTRAMEDULLARY MULTIPLE CRANIAL NERVE
PALSIES
מטופלת שפיתחה פציאליס פריפרי עם דיסאסתזיה בלחי באותו צד לאחר שבוע ירידה בטעם ולאחר עוד שבוע
היפראקזיס, סה”כ 3 שבועות.
בהדמיה מגנטית= האדרה של עצב 7 בתעלה. מה מהבאים לא מתאים ל
Bells Palsy
בסיפור הקליני?
1. האדרת העצב
2. הפרעה תחושתית
3. הפרעה בטעם
4. מהלך של 3 שבועות
מהלך של 3 שבועות לא מתאים לבל’ס פאלסי.
numb chin sign גרורה שלוחצת על העצב
Root entry zone of CN 5 to the brainstem
Demyelination at the trigeminal root entry point into the pons is another well-characterized cause in cases of multiple sclerosis
בת ,57 פנתה למיון עקב כאבים בפנים מימין שהחלו 4 ימים טרם פנייתה. מציינת כאב בעוצמה גבוהה,
בעל אופי של זרם חשמלי, המוחמר בעת אכילה ולעיסה ובעת מגע בפנים. ללא חום, חולשת שרירי פנים
או הפרעה בראייה. שוללת דמעת, נזלת או תלונות אחרות. איזה טיפול מתאים ?
א. חמצן 100%
ב. חוסמי תעלות סידן
ג. תרופות מקבוצת הטריפטנים
ד. טיפולים אנטי אפילפטיים
טיפולים אנטי אפילפטים לטריג’מינל נוירלגיה
(טגרטול)
Principle nucleus of CN 5
CN5- Trigeminal nerve
Mixed sensory and motor nerve
* Sensory – face and head, mucus membranes and cornea and dura
Sensory ganglion – Gasserian in Meckel’s cave (mid cranial fossa)
On entering lateral mid pons they divide:
1.a.Short ascending:
– tactile and light pressure and synapse with 2nd order neurons in PRINCIPAL SENSORY NUCLEUS
- proprioception – MESENCEPHALIC NUCLEUS
1.b. Long Descending: extending from pons to C2 C3 (continuation of medial lemniscus)
- SPINAL TRIGEMINAL NUCLEUS
Pain and temperature
2. 2nd order neurons from principal sensory nucleus and spinal trigeminal nuclei then decussate and ascend to thalamus – lie in medial part of spinothalamic and lateral part of medial lemniscus.
TRIGEMINOTHALAMIC TRACT
But also project bilaterally to facial and hypoglossal nuclei bilaterally
In the periphery:
V1 – opthalmic division – via cavernous sinus and superior orbital fissure
V2 – Maxillary – cavernous sinus – leaves via foramen rotundum
V3 – Mandibular – exits Meckel’s cave inferiorly through foramen ovale.
-
Motor portion – supply masseter and pterygoid (Jaw Jerk):
Origin – trigeminal motor nucleus mid pons – fibers pass underneath gasserian ganglion and are incorporated into mandibular nerve V3.
מה התפקיד של הגרעים המזנצפלי של העצב ה5
mesencephalic nucleus of trigeminal nerve
1. תחושת מצב מהפנים
2. תחושת כאב מהפנים
3. תחושת מגע מהפנים
תחושת מצב מהפנים
Trigeminal mesencephalic neucleus: reflex proprioception of the muscles of mastication of the jaw.
The mesencephalic nucleus is one of four trigeminal nerve nuclei, three sensory and one motor. The other two sensory nuclei are the chief sensory nucleus (pontine principle sensory nucleus) mediating conscious facial touch and the spinal trigeminal nucleus, mediating pain in the head, and is of importance in headache. The trigeminal motor nucleus innervates the muscles of mastication.
מטופלמגיעעםכאביםבגרוןשמקריניםלאוזן,מהיהיהבנוסף.
א.ברדיקרדיה
ב.מיוזיס
ג.אורתוסטטיזם
ד. טכיקרדיה
ברדיקרדיה
לדעתי שתי תשובות לא מאפיינות גלוסופרינגיאל נוירלגיה.
כאב בלחי- לא נכון- יש כאב בבסיס הלשון והגרון.
ולעיתים קרובות לאחר זוסטר- לא נכון- כתוב בספר לעיתים נדירות…
טיפול בפרגבלין
Glossopharyngeal neuralgia, resembles trigeminal neuralgia in many respects except that the unilateral stabbing pain is localized to one side of the root of the tongue and throat. It is far less common than trigeminal neuralgia. Sometimes the pain overlaps the vagal territory beneath the angle of the jaw and external auditory meatus. It may be triggered by coughing, sneezing, swallowing, and pressure on the tragus of the ear. Temporary blocking of the pain by anesthetizing the tonsillar fauces and posterior pharynx with 10 percent lidocaine spray is diagnostic. Rarely; herpes zoster may involve the glossopharyngeal nerve. Fainting as a manifestation of vagoglossopharyngeal neuralgia. Always the sequence is pain, then bradycardia, and, finally, syncope. Presumably the pain gives rise to a massive volley of afferent impulses along the ninth cranial nerve, activating the medullary vasomotor centers via collateral fibers from the nucleus
of the tractus solitarius. An increase in parasympathetic (vagal) activity slows the heart. in addition to bradycardia, there is an element of hypotension caused by inhibition of peripheral sympathetic activity. Here, the effects of the bradycardia
exceed those of the vasodepressor hypotension, sometimes to the point of asystole, reflecting the opposite relationship from that seen in most other types of syncope.
treatment for idiopathic glossopharyngeal neuralgia, a trial of carbamazepine, gabapentin, pregabalin, or baclofen may be useful. If these are unsuccessful, the conventional surgical procedure had been to interrupt the glossopharyngeal nerve and upper rootlets of the vagus nerve near the medulla but recent observations suggest that a vascular decompression procedure similar to the one used for tic and directed to a small vascular loop
under the ninth nerve relieves the pain in a proportion of patients.
בן 50 מתלונן על כאב חזק המתפתח בבסיס הלשון, מלווה בברדיקרדיה ואיבוד הכרה. מה הטיפול המומלץ?
1. הפרדת כלי דם עורקי מעצב הטריג’מינוס
2. הזרקת אלכוהול לגרעין הnucleus salivatory
3. טיפול בקודאין
4. טיפול בפרגבלין
5. לזייה בתלמוס
טיפול בפרגבלין
Glossopharyngeal neuralgia, resembles trigeminal neuralgia in many respects except that the unilateral stabbing pain is localized to one side of the root of the tongue and throat. It is far less common than trigeminal neuralgia. Sometimes the pain overlaps the vagal territory beneath the angle of the jaw and external auditory meatus. It may be triggered by coughing, sneezing, swallowing, and pressure on the tragus of the ear. Temporary blocking of the pain by anesthetizing the tonsillar fauces and posterior pharynx with 10 percent lidocaine spray is diagnostic. Rarely; herpes zoster may involve the glossopharyngeal nerve. Fainting as a manifestation of vagoglossopharyngeal neuralgia. Always the sequence is pain, then bradycardia, and, finally, syncope. Presumably the pain gives rise to a massive volley of afferent impulses along the ninth cranial nerve, activating the medullary vasomotor centers via collateral fibers from the nucleus
of the tractus solitarius. An increase in parasympathetic (vagal) activity slows the heart. in addition to bradycardia, there is an element of hypotension caused by inhibition of peripheral sympathetic activity. Here, the effects of the bradycardia
exceed those of the vasodepressor hypotension, sometimes to the point of asystole, reflecting the opposite relationship from that seen in most other types of syncope.
treatment for idiopathic glossopharyngeal neuralgia, a trial of carbamazepine, gabapentin, pregabalin, or baclofen may be useful. If these are unsuccessful, the conventional surgical procedure had been to interrupt the glossopharyngeal nerve and upper rootlets of the vagus nerve near the medulla but recent observations suggest that a vascular decompression procedure similar to the one used for tic and directed to a small vascular loop
under the ninth nerve relieves the pain in a proportion of patients.
קושי לנפח את הלחי באותו הצד
CN 7:
Mainly motor
sensory- taste from anterior 2/3 of tongue and anterior external auditory canal.**
- Taste fibres go with lingual nerve (trigeminal) and then join chorda tympani – conveying taste to nucleus tractus solitarius.
- The autonomic functions – originate superior salivatory nucleus and innervate lacrimal gland through greater superficial petrosal. The sublingual and submaxillary glands through the chorda tympani.
- Motor nucleus of CN7 lies ventral and lateral to CN6 – in the pons the fibres encircle to abducens nucleus before emerging from lower pons.
Facial nerve enters internal auditory meatus with the CN8 bundle and bends sharply (genu) forward and downward in inner ear.
There lies the geniculate ganglion which is the sensory ganglion
The nerve then continues in bony canal – the facial canal and provides the pterygopalatine ganglion – to innervate lacrimal, nasal and palatine glands.
Distally gives motor branch to stapedius and then joined by chorda tympani - projects to submandibular ganglion and to submandibular and sublingual glands.
*Motor component of CN7 exits skull via stylomastoid foramen – and then through the parotid gland divides to supply facial muscles.
- Lesion at stylomastoid foramen - paralyses facial muscles
- If lesions in facial canal above chorda tympani but below geniculate – facial weakness plus loss of taste anterior 2/3 of tongue plus hyperacusis (due to stapedius nerve)
- If geniculate ganglion or motor root proximal - lacrimation and salivation reduced and may involve CN8 – deafness, tinnitus, dizziness.
פציאליספריפרי עם פגיעהבאיזורה-stylomastoid foramen
1. פגיעה רק בשרירים
2. שרירים+טעם
3. שרירים+היפראקוזיס
4. שרירים+בלוטותרוק
רק שרירים
CN 7:
Mainly motor
sensory- taste from anterior 2/3 of tongue and anterior external auditory canal.**
- Taste fibres go with lingual nerve (trigeminal) and then join chorda tympani – conveying taste to nucleus tractus solitarius.
- The autonomic functions – originate superior salivatory nucleus and innervate lacrimal gland through greater superficial petrosal. The sublingual and submaxillary glands through the chorda tympani.
- Motor nucleus of CN7 lies ventral and lateral to CN6 – in the pons the fibres encircle to abducens nucleus before emerging from lower pons.
Facial nerve enters internal auditory meatus with the CN8 bundle and bends sharply (genu) forward and downward in inner ear.
There lies the geniculate ganglion which is the sensory ganglion
The nerve then continues in bony canal – the facial canal and provides the pterygopalatine ganglion – to innervate lacrimal, nasal and palatine glands.
Distally gives motor branch to stapedius and then joined by chorda tympani - projects to submandibular ganglion and to submandibular and sublingual glands.
*Motor component of CN7 exits skull via stylomastoid foramen – and then through the parotid gland divides to supply facial muscles.
- Lesion at stylomastoid foramen - paralyses facial muscles
- If lesions in facial canal above chorda tympani but below geniculate – facial weakness plus loss of taste anterior 2/3 of tongue plus hyperacusis (due to stapedius nerve)
- If geniculate ganglion or motor root proximal - lacrimation and salivation reduced and may involve CN8 – deafness, tinnitus, dizziness.
זיהום חיידקי שפרץ את האפקס של עצם הפטרוס, יוכל לפגוע באילו עצבים:
א. 5+7
ב 5+6
ג. 7+8
ד. 9+10+11
5+6
Gardenigo
א+ ד
זיהום בהרפס של גנגיליון גסריאני אינו סיבה לפציאליס פריפרי וכן גידול במידבריין לא יהיה סיבה לפציאליס פריפרי
- The geniculate ganglion is an L-shaped collection of fibers and sensory neurons of the facial nerve located in the facial canal of the head. It receives fibers from the motor, sensory, and parasympathetic components of the facial nerve and sends fibers that will innervate the lacrimal glands, submandibular glands, sublingual glands, tongue, palate, pharynx, external auditory meatus, stapedius, posterior belly of the digastric muscle, stylohyoid muscle, and muscles of facial expression.
- Gasserian Ganglion- Trigeminal ganglion a sensory ganglion of the trigeminal nerve (CN V) that occupies a cavity (Meckel’s cave) in the dura mater, covering the trigeminal impression near the apex of the petrous part of the temporal bone.
Causes of Bell’s Palsy:
* Idiopathic - ?HSV
* Ramsay Hunt – VZV
* Lyme (often bilateral – pleocytosis in CSF)
* HIV (may be bilateral – pleocytosis in CSF)
* Chickenpox
* TB of mastoid and middle ear or petrous bone
* Mononucleosis
* Poliomyelitis
* Leprosy
* Sarcoidosis
* Tumors of Parotid gland
* Tumors of temporal bone (carotid body, cholesteatoma)
* Pachymeningitis of base of skull
* Fracture of temporal bone
* Otitis media
* acoustic neuroma, neurofibroma, jugular tumor, aneurysm of vertebral or basilar artery
Perineural skin cancer
BILATERAL:
* GBS
* Lyme
* HIV
* Meningeal tumor infiltrate
* Syphilis
* Sarcoid
* Mononucleosis
* Mobius Syndrome
* Melkersson-Rosenthal Syndrome
* Kennedy Syndrome
במהלך מחלת פציאליס פריפרי, איזה ממצא מהווה מדד פרוגנוסטי חיובי?
1. חזרת טעם תוך שבוע מהופעת הפציאליס
2. בבדיקת הדמייה מגנטית רואים האדרה של עצב 7
3. היפראקוזיס וחולשת סטפדיוס
חזרת טעם תוך שבוע מהופעת הפציאליס הינה סימן פרוגנוסטי חיובי
- Recovery of taste precedes recovery of motor function; if taste returns in the first week, it is a good prognostic sign. But early recovery of some motor function in the first 5 to 7 days is the most
favorable sign. - Hyperacusis or distortion of sound may be experienced in the ipsilateral ear and, as mentioned, indicates paralysis of the stapedius muscle.
- The facial nerve in Bell’s palsy often displays abnormal signal on gadolinium-enhanced MRI although this may be difficult to appreciate in axial sections if the change is in the vertical part of the facial canal.
Cases with more pronounced contrast enhancement of the facial nerve apparently have a worse prognosis (Kress). The enhancement presumably reflects inflammation and swelling along the course of the facial nerve. - if there is evidence of denervation after 10 days, one may expect a long delay in the onset of recovery, measured in terms of months.
- Recurrent forms of facial paralysis also occur
with Lyme disease and sarcoidosis, and in a familial variety.
תיאור של אישה עם הורנר חד צדדי, הפרעה בבליעה, החזר גאג ירוד בצד אחד, אטרופיה של הלשון וחולשה של סטרנוקליידו מסטואיד. שואלים איפה הפתולוגיה?
1. jugular foramen
2. posterior retroparotid space
3. cavernous sinus
posterior retroparotid space
פציאליס פריפרי עם שלפוחיות על הלשון באותו הצד
1. בכצ’ט
2. ראמזי האנט
3. Bell’s Palsy
רמזי האנט
במחלת רמזי האנט, איזה גנגליון מעורב?
geniculate ganglion
איזה גנגליון מעורב ב
opthalmic herpes zoster?
gasserion ganglion (trigeminal)
בן 56 , מזה מספר חודשים מתלונן על אירועים חוזרים של התכווצויות לא רצונית של סגירת עין ימין
והרמת שפה מימין. בדיקה נוירולוגית תקינה פרט לאירועים כמתואר. MRI מוח תקין. הטיפול המומלץ
במצב זה הוא:
א. amitriptyline
ב. botulinum toxin
ג. carbamazepine
ד. verapamil
carbamazepine
ורק אם הוא נכשל אז
בוטוקס
בת 30 הסובלת מטרשת נפוצה מתלוננת על קפיצות של שרירים במחצית הפנים מימין. בבדיקתה יש התכווצויות עדינות של השרירים בפנים מימין. היכן נמצא הפלאק בהמדיה
1. subcortical frontal
2. medulla
3. pons
4. periventricular white matter
5. corpus callosum
Pons- hemifacial spasm
