LOWER CRANIAL NERVES (IX ,X ,XI ,XII)

Applied

PRESENTER

Dr. A T M Hasibul Hasan

MD Thesis part student
Department of Neurology
TOPICS FOR DISCUSSION

• Clinical Scenarios
• Radiological Anatomy of Lower Cranial
Nerves
• Individual Cranial Nerve Pathology
• Lower Cranial Nerve syndromes
• Bulbar and Pseudobulbar palsy
• A 43 years old man, while taking dinner,
suddenly developed lancinating pain in the
right side of his throat.
• He rushed to the emergency assuming fish
bone impaction in throat, but the
otolaryngologist found nothing.
• The symptoms recurred a month later, and
suddenly collapsed unconscious for a brief
period, when he stood up from dinner table.

Case-1
• A 46 years old housewife, noticed a whooshing sound in
her left ear when she lays on her left side, over the last few
years.

• But for the last few months it has become persistent, in

addition to her newly appeared symptoms of difficulty in
swallowing and hoarseness of voice.

Case-2
• A 55 years old businessman, with recurrent TIAs, was
found to have 90% narrowing of his LICA and underwent
left CEA.
• After 2 weeks, He was unable to pull a sweater off over his
head along with a constant aching on the left side of his
neck and left ear and a dull pain in left shoulder.
• He had weakness with shoulder elevation on his left side
and was unable to abduct his left arm above the level of his
shoulder.

Case-3
• A 34 years old day labour, experienced a sudden
onset of pain in the left side of his neck radiating to
his head.
• On the vary next morning pain almost disappeared,
but over breakfast he had difficulty moving food
around in his mouth, his tongue felt heavy, and his
speech was slurred.
• At the hospital, when he was asked to protrude his
tongue, it deviated to left. But the taste sensation
was intact and a CT head found nothing.

Case-4
RADIOLOGIC ANATOMY

CRANIAL NERVES:- IX-XII

 • Axial CT (Bone window)
through skull base.

Glossopharyngeal
 • Axial FIESTA sequence.

Glossopharyngeal
 • Axial CT (Bone window)
through skull base.

VAGUS
 Coronal CT Axial T1WI (C) with fat saturation

VAGUS
 • Axial FIESTA sequence.

CN: IX, X
CN- IX, X Axial and Coronal Oblique FIESTA
 • SSFP MRI

Cranial Accessory
 • SSFP MRI (Coronal
Oblique View)

Spinal Accessory
 • Axial CT

Hypoglossal
 • Axial FIESTA

Hypoglossal
 • Axial TIWI (C) with Fat
Saturation

Hypoglossal
Arteries in Relation to Lower CNs
INDIVIDUAL CRANIAL
NERVE
PATHOLOGY
 Motor- Mild dysfunction of Stylopharyngeus Dysphagia
 Sensory- Loss of taste sensation from post 1/3rd of tongue.
 Reflex- Loss of Gag and Palatal reflex.
 Autonomic-
 Altered parotid salivation Dry mouth
 Carotid sinus dysfunction-
 Tachycardia
 Bradycardia
 Hypotension

Evaluation of CN-IX
Supranuclear Lesion: Rare, pseudobulbar palsy.
Nuclear Lesion:
 Neoplasm- BS Glioma
 Inflammatory- ADEM
 Vascular- PICA stroke
 Syringobulbia
Lesion in Subarachnoid space:
 Large CP angel tumor (mass effect)
 Lower CN Schwannoma
 Glossopharyngeal neuralgia (vascular compression)

Levels of Glossopharyngeal Lesion

Lesion in jugular foramen:
 Neoplasm ( Glomus jugulare, schwannoma, meningioma, skull
base metastasis)
 Trauma (Skull base fracture)
Extracranial lesion:
 Neoplasm (Nasopharyngeal carcinoma, carotid body tumor)
 Infection (Abscess)
 Adenopathy
 Aneurysms
 Carotid endarterectomy

Levels of Glossopharyngeal Lesion

 Nuclei & Root Jugular foramen

Axial FLAIR Sequence Axial Post contrast Fat Saturated T1WI

A 43 years old man presented with lancinating pain in throat
 The age group involved is generally older than 40 years of age.

Aetiology:
• Mostly idiopathic.
• Secondary causes includes-
1. Neurovascular compression of the nerve root
2. Chiari Malformation
3. Pathology in Brain stem eg, tumor, demyelination
4. Cerebello-pontine angel tumor
5. Infection eg lyme disease

Glossopharyngeal Neuralgia:
C/F:
• Pain- Unilateral lancinating pain in
tonsillar fossa or ear.
• Often precipitated by swallowing,
coughing, chewing, talking.
• May be associated with-
Bradycardia or asystole
Hypotension and
Fainting.

• There is no demonstrable motor

or sensory deficit

Glossopharyngeal Neuralgia
Glossoparyngeal Neuralgia
 Treatment:
Medical-
o Carbamazepine
o Gabapentin
o Pregabalin
o Phenytoin.
Surgical-
o Microvascular decompression
o Intracranial section of the glossopharyngeal & upper
rootlets of the vagus nerve near the medulla.

Glossophar yngeal Neuralgia:

Motor-
 Unilateral Lesion:
o Failure of palatal elevation
o Uvular deviation to opposite side
o Dysphagia, dysarthria, dysphonia
 Bilateral lesion:
o Bilateral palatal palsy
o Profound dysphagia, dysarthria, dysphonia

Evaluation of CN-X
Lesion above SLN:
 Unilateral Lesion:
oMild dysphagia, hoarseness, reduced vocal cord strength
 Bilateral Lesion:
oWeak cough, marked dysphagia, nasal regurgitation
oNo stridor/ Breathlessness
Lesion of RLN:
 Unilateral Lesion:
oHoarseness, Breathless speech, Stridor
 Bilateral Lesion:
oStridor, Breathlessness

Evaluation of CN-X
Sensory- Loss of taste sensation from epiglottis.
Reflex- Loss of Gag, Cough and Vomiting reflex.
Autonomic-
 Carotid sinus dysfuction-
 Tachycardia
 Hypotension

Evaluation of CN-X
Supranuclear Lesion: Rare, pseudobulbar palsy.
Nuclear or Fascicular Lesion:
 Neoplasm- BS Glioma
 Inflammatory- ADEM
 Vascular- PICA stroke
 Syringobulbia
Lesion in jugular foramen:
 Neoplasm ( Glomus jugulare/Vagale, schwannoma,
meningioma, skull base metastasis)
 Trauma (Skull base fracture)

Levels of Vagal Lesion

Extracranial lesion:
 Lesion of Vagus N. Proper:
o Iatrogenic- Thyroid surgery
o Vascular: ICA Dissection
o Infl/Infectous: Carotid space abscess
o Neoplasm: Schwannoma, Thyroid malignancy, NHL, NPC,
Glomus
 Lesion in Mediastinum:
o Vascular- Aortic arch aneurysm
o Infl/Infectous- Mediastenitis, Lymphoma, Sarcoidosis
o Neoplastic- Ca Bronchus, NHL

Levels of Vagal Lesion

 Lesion of Superior Laryngeal nerve: Usually traumatic
 Lesion of Recurrent Laryngeal nerve:
oIatrogenic/Traumatic- Intubation, Surgery
oNeoplastic- Mediastinal tumor
oVascular- AA/SbCA aneurysm
oIdiopathic

Level of Vagal Lesion

 Nuclear Lesion Jugular Foramen

Level of Vagal Lesion

 Vagus Proper Mediastinum

Levels of Vagal Lesion

A 46 years old lady presented with pulsatile tinnitus and dysphagia
It represents 0.6% of all head neck tumors.
Clinical Presentation: Depends on Location
 Glomus Jugulare:
 Pulsatile tinnitus
 Conductive hearing loss
 Jugular foramen syndrome
 Glomus Vagale: Slow growing mass in carotid space
 Glomus Tympanicum:
 Pulsatile tinnitus
 Conductive hearing loss
 Vertigo, Facial weakness
 Carotid body tumor:
 Neck mass with hoarseness and dysphagia
 Catecholamine- HTN, headache, tachycardia, palpitation

Glomus Tumor: Paraganglioma

Pathology:
• Arise from extra adrenal neuro-endorine tissue (paraganglia),
which contain round polygonal cells arranged in nests with in a
dense capillary network and neuro-secretory granules.
Imaging:
• CT Scan- Irregular destruction of bone at jugular foramen.
• MRI- Mixed intensity mass in T1WI and mild hyper intense in
T2WI with contrast enhancement.
• DSA- May reveal hypervascular mass supplied by ECA branches.
Treatment:
• Surgery/embolization
• Radiotherapy

Glomus Tumor: Paraganglioma

Motor- Involvement results in paresis and/or
atrophy of Sternocleidomastoid and Trapezius.
 Sternocleidomastoid paresis:
o Weakness in turning head to opposite side
o Bilateral involvement causes weakness in neck flexion
 Trapezius paresis:
o Drooping of shoulder
o Difficulty in raising abducted arm above shoulder
o Bilateral weakness results in weak neck extension

Evaluation of CN-XI
Supranuclear Lesion:
 Hemispheric lesion=> (Irritative- Seizure)
o Head turning away from the side of lesion
 Hemispheric lesion=> (Non irritative- Infarct)
o Hemiplegia + Weakness in shoulder elevation (Contralateral)
o Head turning (Ipsilateral- towards the site of lesion)
Nuclear: Rare, High cervical or low medullary
 Brain stem infarct
 Brain stem tumor
 Syringobulbia/myelia
Lesion in jugular foramen: Also involve CN IX, X, XII
 Neoplasm ( Glomus jugulare, schwannoma, meningioma, metastasis)
 Trauma (Skull base fracture)

Levels of Accessory Nerve Lesion

Extracranial lesion:
 Iatrogenic: Following surgery in neck
o Lymph node biopsy
o CEA
o IJV Cannulation
o Neck dissection in posterior cervical triangle
 Trauma
 Post radiation

Levels of Accessory nerve Lesion

A 55yrs old man presented with left shoulder weakness following CEA
A 45 years old man presented with left vocal cord palsy, weakness of head turning
to right and weakness of left shoulder elevation

Jugular Foramen Schwannoma

Motor- Action of genioglossus is the key in
understanding hypoglossal lesion
 UMN Lesion:
o Weakness of contralateral geniogossus =>
Deviation of tongue away from the side of
lesion.
 Nuclear/ LMN Lesion:
o Ipsilateral tongue palsy, atrophy, fasciculation.
o Deviation of tongue towards the side of lesion.

Evaluation of CN-XII
  Dysarthria:
o Abnormality in articulation, prosody.
o Difficulty in lingual consonant (D, T, L).
Cleival Lesion:
 Hypoglossal and abducent palsy

Evaluation of CN-XII
Supranuclear Lesion:
 Unilateral lesion:
o Hemiplegia + Deviation of tongue (Contralateral)
 Bilateral lesion:
o Spastic dysarthria
Nuclear: Rare, Unilateral lesion cause unilateral LMN
syndrome
 Vascular: Medial medullary syndrome of Dejerine.
 Infection/Inflammation: Polio, IM
 Neoplasm: Brain stem tumor
 Demyelination: MS
 Degenrative: Progressive bulbar palsy
 Syringobulbia

Levels of Hypoglossal Nerve

Premedullary subarachnoid space and Hypoglossal canal lesion
 Neoplasm: Schwannoma, meningioma, metastasis
 Trauma
 Infection: Osteomyelitis
 Vascular: Vertebral dissection

Levels of Hypoglossal nerve Lesion

 Extracranial lesion:
 Carotid space:
o Vascular: ICA dissection
o Infection/Inflammation: TB, RA
o Iatrogenic: IJV Cannulation, CEA
o Neoplasm: SCC, NHL, Paraganglioma
o Trauma
 Lesion in sublingual space and tongue
o Neoplasm
o Infection
o Iatrogenic

Levels of Hypoglossal nerve Lesion

A 34 yrs old man presented with H/O left sided neck pain followed by
difficulty in speech and deviation of tongue towards left on protrusion
LOWER CRANIAL NERVE

SYNDROME
 1. Intramedullary (Brainstem) syndrome:
 Avellis syndrome
 Jackson syndrome
 Wallenberg syndrome
 Degerine syndrome

2. Extramedullary Cranial Nerve syndrome:

 Eagle syndrome
 Vernet syndrome
 Collet-Sicard syndrome
 Villaret syndrome
 Tapia syndrome

Lower Cranial Nerve

syndromes
Syndrome Involved Additional Feature Location Cause
CN
Avellis X Contralateral Brain stem or Infarct / Tumor
Hemiparesis Peripheral pyramidal
tract

Jackson X, XII Contralateral Brain stem or Infarct / Tumor

Hemiparesis Peripheral pyramidal
tract

Wallenburg V, IX, X, XI Ipsilateral Horner, Lateral Medulla- Occlusion of PICA

cerebellar ataxia, Nucleus Ambiguus, Vertebra artery
Contralateral loss of Nucleus and spinal
pain and temperature tract of trigeminal,
Vestibular nuclei,
Inferior cerebellar
peduncle,
Contralateral spinal
lemniscus-
spinothalaamic tract

Dejerine XII Contralateral Medial medulla- Vertebral artery

hemiparesis, Pyramidal tract,
hemisensory loss Medial lemniscus

CN Syndromes: Summery
Babinsky Combination
Nageotte
Syndrome Involved CN Additional Location Cause
Feature
Eagle IX Styloid process Compression by elongated
process or
Oscified stylohyoid ligament
Vernet IX, X, XI Jugular foramen Tumor,
Venous sinus thrombosis,
Aneurysm
Collet-Sicard IX, X, XI, XII Posterior Tumor of parotid gland,
laterocondylar carotid body, lymph node
space Tubercular adenitis,
Carotid dissection
Villaret IX, X, XI, XII Horner Posterior retro Tumor of parotid gland,
parotid space carotid body, lymph node
Tubercular adenitis,
Granuloma (Sarcoid, fungal)
Carotid dissection
Tapia X, XII With/ Posterior retro Parotid tumor
wthout XI parotid space High neck injury

CN Syndrome: Summery
 Bulbar Palsy
Bilateral involvement of 9th,10th,11th,12th, nerve nuclei in
medulla.
Usual Cause:
Cause Example
Genetic Kennedy’s disease
Vascular Medullary infarction

Degenerative MND, Syringobulbia

Inflamatory/ infective MG, GBS, Poliomyelitis, Diphtheria, Lyme disease,

Vasculitis

Neoplastic Brainstem glioma, Malignant meningioma

C/F:
 LMN type paralysis causing:
o Dysphagia
o Dysarthria
o Nasal regurgitation and nasal intonation
o Dribbling of saliva
 Wasted and fasiculated tongue with absent palatal
movement and absent Gag reflex.

Bulbar Palsy
Pseudobulbar Palsy:
Bilateral Supra-nuclear Lesions affecting Cortex or Corticonuclear
fibers will give UMN type features of 9th to 12th nerve involvement.

Usual Cause:
Cause Example

Vascular Bilateral hemisphere infarction

Degenerative MND

Inflamatory/ infective MS, Cerebral vasculitis

Neoplastic High brain-stem tumor

C/F:
 Dysphagia, Dysarthria, Dysphonia
 Indistinct, Slurred, High-pitched speech.
 Tongue is spastic, unable to protrude, No wasting & no
fasciculation.
 Absent palatal movement.
 Jaw jerk- exaggerated.
 Patient is emotionally labile.

Pseudobulbar Palsy
 Bulbar and Psudobulbar: Differences
Trait Bulbar Palsy Pseudobulbar Palsy

Type of Lesion LMN UMN

Usual Site Brainstem Bilateral internal capsule

Emotion Normal Labile

Speech Nasal Slow, Slurred, Indistinct

Nasal Regurgitation Present Absent

Tongue Wasted, Fasciculation Small, Stiff, Spastic

Jaw Jerk Absent Brisk

Fasciculated Tongue Spastic Tongue
1. Diseases of Cranial Nerves. Allen HR, Martin AS, Joshua PK, editors.
Adams and Victor’s Principal of Neurology. 10th edition. McGraw-Hill
Education; 2014:1391-1407.
2. Janet C Rucker. Cranial Neuropathies. In: Robert BD, Jerald MF, Joseph
J, John CM, editors. Bradley’s Neurology in Clinical Practice. 6 th edition.
Elsevier Limited; 2012: 1757-1760.
3. Devin KB, Sonne DC, nancy JF, editors. Cranial Nerves: Anatomy,
Pathology and Imaging. Thieme Medical Publisher, 2010.
4. Linda WP, Elizabeth JA, editors. Cranial Nerves in Health and Disease.
2nd edition. Linda WP 2002.

Reference
Thank You….
• Site of lesion: Tegmentum of medulla
• Cranial Nerve Involved: X
• Cause: Infarct or tumor
• Tracts Involved: Spinothalamic tract;
sometimes descending pupillary fibres; with
Horner syndrome.

Avellis Syndrome
• Signs/symptoms: Paralysis of soft palate and
vocal cord and contralateral
hemiparesis/hemianesthesia.

Avellis Syndrome:
Site of lesion: Tegmentum of medulla
Cranial Nerve Involved: X, XII
Usual Cause: Infarct or tumor
Tracts Involved: Corticospinal tract

Jackson Syndrome:
Signs/symptoms:Like Avellis syndrome
plus ipsilateral tongue paralysis.

Jackson Syndrome
Site of lesion:Lateral tegmentum of medulla
Cranial Nerve Involved:Spinal V,IX, X, XI
Usual Cause: Occlusion of V.Artery or PICA
Tracts Involved: Lat.spinothalamic tract,
Descending pupillo dilator fibres,
Spinocerebellar and olivocerebellaar tracts

Wallenburg Syndrome
 Wallenburg Syndrome

Signs/symptoms: Ipsilat.V,IX,X,XI palsy,Horner syndrome and

Cerebellar ataxia, contralateral loss of pain and temparature sense
Site of lesion:At the level of styloid process
Cranial Nerve Involved: IX
Usual Cause:Compression of the glossopharyngeal
nerve by an elongated styloid process or ossified
stylohyoid ligament
Signs/symptoms: Mimic glossopharyngeal Neuralgia
but the pain tends to be more persistent and dull in
nature

Eagle Syndrome:
Site of lesion: Jugular foramen
Cranial Nerve Involved: IX,X,XI
Usual Cause: Tumor and aneurysm

Vernet Syndrome:
Signs/symptoms:
 Ipsilateral paresis of sternocleidomastoid and trapezius
 Dysphonia
 Dysphagia
 Ipsilateral vocal cord palsy
 Loss of taste sensation from posterior 1/3rd of tongue
 Loss of sensation from ipsilateral palate, uvula, pharynx
 Loss of Gag reflex

Vernet Syndrome:
Site of lesion: Posterior laterocondylar space
Cranial Nerve Involved: IX,X,XI & XII
Usual Cause: Tumor of parotid gland,carotid body,secondary and
lymph node tumor,tubercular adenitis,carotid artery dissection

Collet-Sicard Syndrome:
Signs/symptoms:
 Headache/ Neck pain (Depending on aeitilogy)
 Dysphonia
 Dysphagia
 Ipsilateral paresis of tongue, palate, uvula and vocal cord
 Loss of taste sensation from posterior 1/3rd of tongue
 Loss of sensation from ipsilateral palate, uvula, pharynx
 Loss of Gag reflex

Collet-Sicard Syndrome:
Site of lesion: Posterior retroparotid space near carotid
artery
Cranial Nerve Involved: IX,X,XI & XII, and Horner
syndrome
Usual Cause: Tumor of parotid gland, carotid body,
secondary and lymph node tumor,
tubercular adenitis, carotid artery dissection

Villaret syndrome:
Site of lesion: Posterior retroparotid space
Cranial Nerve Involved: X, XII with or without XI
Usual Cause: Parotid and other tumor of, or injuries to, the high
neck

Tapia syndrome: