PRESENTER

Dr. A T M Hasibul HasanMD Thesis part studentDepartment 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.

VAGUSCoronal 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 CT

Hypoglossal

• Axial FIESTA

Hypoglossal

• Axial TIWI (C) with Fat Saturation

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-

TachycardiaBradycardiaHypotension

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 root2. Chiari Malformation3. Pathology in Brain stem eg, tumor, demyelination4. Cerebello-pontine angel tumor5. 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

Glossoparyngeal Neuralgia

Glossopharyngeal Neuralgia:

Treatment:

Medical-o Carbamazepineo Gabapentin o Pregabalin o Phenytoin.

Surgical-o Microvascular decompressiono 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 elevationo Uvular deviation to opposite sideo Dysphagia, dysarthria, dysphonia

Bilateral lesion: o Bilateral palatal palsyo 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 regurgitationoNo 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-TachycardiaHypotension

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) Trauma (Skull base fracture)

Levels of Vagal Lesion

Extracranial lesion: Lesion of Vagus N. Proper:

o Iatrogenic- Thyroid surgeryo Vascular: ICA Dissectiono Infl/Infectous: Carotid space abscesso Neoplasm: Schwannoma, Thyroid malignancy, NHL, NPC,

Glomus

Lesion in Mediastinum: o Vascular- Aortic arch aneurysmo Infl/Infectous- Mediastenitis, Lymphoma, Sarcoidosiso Neoplastic- Ca Bronchus, NHL

Level of Vagal Lesion

Lesion of Superior Laryngeal nerve: Usually traumatic Lesion of Recurrent Laryngeal nerve:

oIatrogenic/Traumatic- Intubation, SurgeryoNeoplastic- Mediastinal tumoroVascular- AA/SbCA aneurysmoIdiopathic

Level of Vagal Lesion

Nuclear Lesion Jugular Foramen

Levels of Vagal Lesion

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 LocationGlomus Jugulare:

Pulsatile tinnitusConductive hearing lossJugular foramen syndrome

Glomus Vagale: Slow growing mass in carotid spaceGlomus Tympanicum:

Pulsatile tinnitusConductive hearing lossVertigo, Facial weakness

Carotid body tumor:Neck mass with hoarseness and dysphagiaCatecholamine- 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

• RadiotherapyGlomus 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 sideo Bilateral involvement causes weakness in neck flexion

Trapezius paresis: o Drooping of shouldero Difficulty in raising abducted arm above shouldero 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) Trauma (Skull base fracture)

Levels of Accessory nerve Lesion

Extracranial lesion: Iatrogenic: Following surgery in neck

o Lymph node biopsyo CEAo IJV Cannulationo 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

Extracranial lesion: Carotid space:

o Vascular: ICA dissectiono Infection/Inflammation: TB, RAo Iatrogenic: IJV Cannulation, CEAo Neoplasm: SCC, NHL, Paragangliomao Trauma

Lesion in sublingual space and tongueo Neoplasmo Infectiono 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

SYNDROME

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 PICAVertebra 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 orOscified 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 nodeTubercular adenitis,Carotid dissection

Villaret IX, X, XI, XII Horner Posterior retro parotid space

Tumor of parotid gland, carotid body, lymph nodeTubercular adenitis,Granuloma (Sarcoid, fungal)Carotid dissection

Tapia X, XII With/ wthout XI

Posterior retro parotid space

Parotid tumorHigh 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 Dysarthriao 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.

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

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