Disorder of lower cranial nerves

PRESENTER
Dr. A T M Hasibul Hasan
MD Thesis part student
Department of Neurology
LOWER CRANIAL NERVES (IX ,X ,XI ,XII)
Applied

TOPICS FOR DISCUSSION
• Clinical Scenarios
• Radiological Anatomy of Lower Cranial
Nerves
• Individual Cranial Nerve Pathology
• Lower Cranial Nerve syndromes
• Bulbar and Pseudobulbar palsy

Case-1
• 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-2
• 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-3
• 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-4
• 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.

RADIOLOGIC ANATOMY
CRANIAL NERVES:- IX-XII

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

Glossopharyngeal
• Axial FIESTA sequence.

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

VAGUS
Coronal CT Axial T1WI (C) with fat saturation

CN: IX, X
• Axial FIESTA sequence.

CN- IX, X Axial and Coronal Oblique FIESTA

Cranial Accessory
• SSFP MRI

Spinal Accessory
• SSFP MRI (Coronal
Oblique View)

Hypoglossal
• Axial TIWI (C) with Fat
Saturation

Disorder of lower cranial nerves

Arteries in Relation to Lower CNs

INDIVIDUAL CRANIAL
NERVE
PATHOLOGY

Evaluation of CN-IX
 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

Levels of Glossopharyngeal Lesion
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

Nuclei & Root Jugular foramen
Axial FLAIR Sequence Axial Post contrast Fat Saturated T1WI

A 43 years old man presented with lancinating pain in throat

Glossopharyngeal Neuralgia:
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:
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.

Evaluation of CN-X
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:
oMild dysphagia, hoarseness, reduced vocal cord strength
 Bilateral Lesion:
oWeak cough, marked dysphagia, nasal regurgitation
oNo stridor/ Breathlessness
Lesion of RLN:
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

Levels of Vagal Lesion
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)

 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

Level 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

Vagus Proper Mediastinum

A 46 years old lady presented with pulsatile tinnitus and dysphagia

Glomus Tumor: Paraganglioma
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

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

Evaluation of CN-XI
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

Levels of Accessory Nerve Lesion
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)

Levels of Accessory nerve 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

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

Evaluation of CN-XII
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.

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

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

Levels of Hypoglossal nerve 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

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
syndromes
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

CN Syndromes: Summery
Syndrome Involved
CN
Additional Feature Location Cause
Avellis X Contralateral
Hemiparesis
Brain stem or
Peripheral pyramidal
tract
Infarct / Tumor
Jackson X, XII Contralateral
Hemiparesis
Brain stem or
Peripheral pyramidal
tract
Infarct / Tumor
Wallenburg V, IX, X, XI Ipsilateral Horner,
cerebellar ataxia,
Contralateral loss of
pain and temperature
Lateral Medulla-
Nucleus Ambiguus,
Nucleus and spinal
tract of trigeminal,
Vestibular nuclei,
Inferior cerebellar
peduncle,
Contralateral spinal
lemniscus-
spinothalaamic tract
Occlusion of PICA
Vertebra artery
Dejerine XII Contralateral
hemiparesis,
hemisensory loss
Medial medulla-
Pyramidal tract,
Medial lemniscus
Vertebral artery
Babinsky
Nageotte
Combination

CN Syndrome: Summery
Syndrome Involved CN Additional
Feature
Location Cause
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
laterocondylar
space
Tumor of parotid gland,
carotid body, lymph node
Tubercular adenitis,
Carotid dissection
Villaret IX, X, XI, XII Horner Posterior retro
parotid space
Tumor of parotid gland,
carotid body, lymph node
Tubercular adenitis,
Granuloma (Sarcoid, fungal)
Carotid dissection
Tapia X, XII With/
wthout XI
Posterior retro
parotid space
Parotid tumor
High neck injury

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

Bulbar Palsy
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.

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

Pseudobulbar Palsy
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.

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

Reference
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. 6th
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.

• 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

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

Jackson 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.

Wallenburg 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

Signs/symptoms: Ipsilat.V,IX,X,XI palsy,Horner syndrome and
Cerebellar ataxia, contralateral loss of pain and temparature sense
Wallenburg Syndrome

Eagle Syndrome:
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

Vernet 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

Collet-Sicard 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

Villaret 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

Tapia 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

Disorder of lower cranial nerves

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Disorder of lower cranial nerves

Editor's Notes