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Chapter 14 – A Synopsis of the Cranial Nerves of the Brainstem Michelle-Lee Jones February 18, 2009 OUTLINE Introductory Points  Cell Columns & Nuclei – Motor & Sensory  Cranial Nerves of the Medulla Oblongata  Cranial Nerves of the Pons-Medulla Junction  Cranial Nerves of the Pons  Cranial Nerves of the Midbrain  Introductory Points  Regarding the brainstem:  Transit point for all ascending & descending tracts connecting the spinal cord to the forebrain  Associated with the exit/entry & nuclei of 10/12 cranial nerves  Lesions often involve cranial nerves & have long tract signs  great localizing signs Motor Cell Columns and Nuclei    Recap: basal plate derivatives  CN motor nuclei  oriented in discontinuous rostrocaudal cell columns Nuclei from the same column possess common developmental, structural and functional features 3 motor cell columns:  General Somatic Efferent (GSE), General Visceral Efferent (GVE) & Special Visceral Efferent (SVE) Motor Cell Columns and Nuclei GSE column features: 1. • • • Most medial & anterior to the ventricular space Nuclei include hypoglossal (XII), abducens (VI), trochlear (IV) & oculomotor (III) Motor neurons innervate skeletal muscle from head mesoderm – tongue (occipital) & extraocular muscles (orbit) [ mesoderm NOT located in pharyngeal arches] Motor Cell Columns and Nuclei 2. GVE – preganglionic parasympathetic column features: • Lateral to GSE • Forms cranial portion of craniosacral division of visceromotor system (parasympathetic) & preganglionic fibres travel on the CN • Nuclei include dorsal motor vagal nucleus (X), inferior salivatory nucleus (IX), superior salivatory nucleus (VII–intermediate), Edinger-Westphal nucleus (III) • Preganglionic axons  peripheral ganglion  postganglionic fibres  visceral structure Motor Cell Columns and Nuclei 3. SVE: • • • Most lateral motor column in medulla & pontine tegmentum Nuclei include nucleus ambiguus (efferents on IX & X), facial motor nucleus & trigeminal motor nucleus Muscles innervated originate from mesenchyme located within the pharyngeal arches Motor Cell Columns and Nuclei Pharyngeal Arch I II III IV Associated muscle Mastication (V) Facial expression (VII) Stylopharyngeus (IX) Pharynx constrictors, intrinsic laryngeal muscles (including vocalis), palatine muscles (except TVP), skeletal muscle upper half of esophagus (X) GSE GVE SVE Sensory Cell Columns & Nuclei Recap: alar plate derivatives  CN sensory nuclei  oriented in continuous cell column  Lateral location for sensory columns  3 sensory cell columns:   Solitary tract and nucleus  Vestibular/cochlear nuclei  Trigeminal sensory nuclei Sensory Cell Columns & Nuclei Solitary tract & nucleus (CN VII, IX & X) 1. • • • • Visceral afferent centre of brainstem (solitary tract receives all the 1°visceral afferent central processes) Taste or Special Visceral Afferent (SVA) fibres  Gustatory nucleus (rostral area of solitary nucleus) General Visceral Afferent (GVA) fibres  Cardiorespiratory nucleus (caudal area of the solitary nucleus) Solitary tract & nucleus (medulla) do not extend rostrally beyond the pons-medulla junction (most rostral CN = VII) Sensory Cell Columns & Nuclei Vestibular/cochlear nuclei 2. • • • • Just posterior to solitary tract & nucleus Includes medial & spinal vestibular nuclei, anterior and posterior cochlear nuclei at pons-medulla junction, superior and lateral vestibular nuclei (caudal pons) Sensory input from VIII only Hearing (SSA, exteroceptive) & balance and equilibrium (SSA, proprioceptive) http://instruct.uwo.ca/anatomy/530/8nucl2.gif Sensory Cell Columns & Nuclei Trigeminal sensory nuclei 3. • • From spinal cord-medulla junction to rostral midbrain 3 subdivisions    Spinal trigeminal nucleus (pars caudalis, pars interpolaris & pars oralis) – lateral medulla to caudal pons Principal sensory nucleus (mid-pontine level) Mesencephalic nucleus (lateral to periaqueductal grey) Sensory Cell Columns & Nuclei Trigeminal sensory nuclei 3. • • • Principal sensory nucleus & particularly the spinal trigeminal nucleus  GSA reception centre of brainstem Receives all the general somatic afferent (pain & thermal) central processes CN V, VII, IX & X Cranial Nerves of the Medulla Oblongata (CN XII, XI, X & IX) Hypoglossal Nerve (Motor)  Nucleus - internal to the hypoglossal trigone  Course: anterior medulla  lateral aspects of medial lemniscus & pyramids  preolivary fissure (rootlets)  hypoglossal canal  intrinsic tongue muscles + hypo-, stylo- & genioglossus muscles  Hypoglossal Canal: XII nerve, emissary vein, meningeal branch from ascending pharyngeal artery (dura posterior fossa) Hypoglossal nucleus + fibres:  supplied by anterior spinal artery (ASA)  Medial Medullary Syndrome (ASA)     Deviation of tongue to side of lesion (GG) Contralateral hemiparesis (CST) Contralateral loss of position sense, vibration & 2-point discrimination (ML) Root lesion – tongue deviation to side of lesion Hypoglossal nucleus + fibres:  Internal capsule lesion    tongue deviation to the contralateral side (injury to crossed corticobulbar fibres innervating XII nucleus) Contralateral hemiplegia Drooping of facial muscles contralateral lower quadrant Accessory Nerve (Motor):  SCM and trapezius muscles are innervated by motor neurons in the cervical spinal cord (NOT MEDULLA)  Cranial part of XI  misnomer (XI fibres temporarily join vague, then separate to exit skull)  Course: Cervical SC  axons exit SC laterally  merge to form nerve  foramen magnum  briefly join caudal part of X in post. fossa  jugular foramen Accessory Nerve (Motor):    Root lesions: trapezius & SCM paralysis (ipsilateral shoulder droop & difficulty turning head to contralateral side) C-spine lesion – above deficits are eclipsed by hemiplegia (CST) Internal capsule lesion – similar deficits as above (uncrossed corticobulbar fibres to XI nucleus injured) Vagus Nerve (Motor & Sensory):   Intermediate location (b/w midline & lateral medulla); exits post-olivary sulcus (exits cranial cavity via jugular foramen) 2 ganglia immediately external to the foramen:   Superior Ganglion (GSA) Inferior Ganglion (GVA, SVA) Vagus Nerve - Motor cells of the medulla: 1. Dorsal motor nucleus of the vagus (GVE-PNS preganglionic)  terminal (intramural) ganglia  viscera (trachea, bronchi, heart, GI tract – just proximal to splenic flexure  Effects: bronchiole constriction,  HR,  blood flow, peristalsis, gut secretions 2. Nucleus Ambiguus (SVE)  4th pharyngeal arch muscles (refer to previous table) Vagus Nerve – Sensory (GSA, GVA & SVA) 1. GSA (pain & thermal): – 2. Small area of ear, part of external auditory meatus & dura posterior fossa  superior ganglion (central processes to spinal trigeminal tract, thence to spinal trigeminal nucleus) GVA & SVA: – Heart, aortic arch, pharynx & larynx, lungs, gut to level of splenic flexure (GVA) + taste buds on epiglottis & tongue base (SVA)  inferior ganglion (central processes to solitary tract, thence to solitary nucleus - cardiorespiratory & gustatory portions GSA SVA SVE GVE GVA Vagus Nerve – Sensory (GSA, GVA & SVA)   Root lesion (vagus): dyphagia & dysarthria, no apparent lasting visceromotor dysfunction, taste NA & external auditory meatus GSA loss not key Unilateral medulla injury  nucleus ambiguus (Tumours, vascular lesions, syringobulbia)    Deficits as noted above Bilateral medulla lesions  aphonia, aphagia, dyspnea, or inspiratory stridor  Critical, especially if dorsal motor nucleus Thyroid surgery  recurrent laryngeal n. injury  dysarthria Glossopharyngeal Nerve (Motor & Sensory)  Leaves medulla @ postolivary sulcus, just rostral to vagus, leaves skull via jugular foramen  As with vagus, 2 ganglia: inferior ganglion (GVA, SVA) & superior ganglion (GSA) Glossopharyngeal Nerve - Motor   Inferior salivatory nucleus (GVE PNS): axons join with tympanic nerve, then lesser petrosal nerve  otic ganglion  parotid gland Nucleus ambiguus (SVE): innervation of stylopharyngeus that muscle that helps with swallowing & efferent part of gag reflex Glossopharyngeal Nerve Sensory  GSA: Pinna, external auditory canal  superior ganglion  GVA: Parotid gland, oropharynx & carotid body  inferior ganglion  SVA: Taste from posterior 1/3  inferior ganglion Glossopharyngeal Nerve Lesions    Rare, usually with X & XI roots @ jugular foramen Nerve lesion:  taste posterior 1/3, loss of ipsi. gag reflex (s-m X) Glossopharyngeal neuralgia:   attacks of intense idiopathic pain in pharynx, caudal tongue, tonsil,? middle ear Precipitated by spontaneous or artificial stimulation posterior oral cavity, swallowing or talking SVA GSA GVE GVA SVE Jugular Foramen & associated syndromes Right Jugular Foramen – medial, middle & caudal parts Jugular Foramen & associated syndromes  Vernet syndrome    @ or just internal to the foramen Loss of sensation post 1/3 tongue (IX); loss of sensation in larynx & pharynx, dysarthria & dysphagia (X); weakness of ipsil. SCM & trapezius (XI) Collet-Sicard syndrome    Immediately external to the jugular foramen Damage to IX, X, & XI + ipsil tongue weakness (hypoglossal canal is near foramen) Villaret syndrome includes above + sympathetic fibres (SCG)  ipsil Horner’s Cranial Nerves of the Pons-Medulla Junction (CN VIII, VII & VI) Vestibulocochlear Nerve (Almost exclusively sensory)    Most lateral, centrally related to cochlear & vestibular nuclei; 2 parts originate from specialized receptors within petrous temporal bone  combined root in brainstem Internal acoustic meatus (IAM) contains VIII, VII & labyrinthine artery Cochlear part:  Cochlear  Spiral ganglion (bipolar cells)  IAM  Cochlear nuclei (ant. & post.)  brainstem relay nuclei  MGN  auditory cortex Vestibulocochlear Nerve (Almost exclusively sensory)   Note cholinergic cells near the olivary nuclei  olivocochlear tract (efferent cochlear bundle)  inner & outer hair cells (dampen responses) Vestibular part:  Ampullae of semicircular canals, utricle & saccule  vestibular ganglion (bipolar cells)  IAM  PMJ  vestibular nuclei (sup, inf, lat, med) in medulla & caudal pons  cerebellum + oculomotor nuclei, etc. Vestibulocochlear Nerve      VIII nerve lesions: hearing loss, tinnitus, vertigo, dizziness, ataxia Cochlea, spiral ganglion or cochlear fibres lesions: ipsilateral sensorineural hearing loss Lesions to brainstem or higher:  ability to localise/interpret sound in space, no hearing loss per se Conductive hearing loss:  conduction through middle ear (typically ossicles) Tinnitus pertains to auditory portion of VIII (peripheral or central damage) Vestibulocochlear Nerve      Injury to vestibular fibres: vertigo (subjective – pt moves or objective – environment moves), nystagmus ± n/v Nystagmus – vestibular influence over brainstem oculomotor control disconnected Lesions of vestibular nuclei & central connections – vertigo, ataxia, nystagmus, ± n/v Causes of vestibular dysfunction are myriad:  Meds, trauma, DM, cerebellar lesions, vestibular schwannoma etc. Meniere’s syndrome:  hearing loss, sound distortion, vertigo, unsteadiness standing or walking   endolymphatic pressure   size of utricle, saccule & cochlear Cerebellopontine Angle Lesions TUMOR TYPE Vestibular schwannomas Meningiomas Epidermoid tumours 85% 5-10% 5% Prevalence @ CPA Origin/ Description  Schwann cells of vestibular root  Margins of internal acoustic meatus (anterior, superior)   Clinical manifestations   Tinnitus, unsteady gait, progressive hearing loss, later ipsil. facial weakness, if > 3 cm, impinge on V  sensory  ± pain Significant erosion of internal acoustic meatus  Early facial weakness then hearing loss & trigeminal root associated pain   Entrapped clusters of epidermis anywhere in CNS Lined with epithelium & contain cellular debris, proteins & cholesterol Spillage of cyst contents  recurrent aseptic meningitis In CPA, deficits related to V, VII & VIII Facial Nerve (Motor & Sensory) (Petrous temporal bone) Sortie Intermediate nerve: GVE + SVA + GSA + few GVA Facial Nerve  At geniculate ganglion (internal genu), greater petrosal nerve formed by GVE-preganglionic PNS nerves from VII  joins deep petrosal nerve to form nerve of the pterygoid canal  pterygopalatine ganglion  Post-ganglionic parasympathetic fibres join V2  orbit  lacrimal gland  Small SVE branch  stapedius muscle  Larger SVE branch (chorda tympani)  middle ear  joins V3 lingual branch  preglanglionic PNS fibres to submandibular ganglion, collects SVA taste afferent fibres (ant 2/3 tongue)  SVE  muscles of facial expression, post. belly digastric & stylohyoid Facial Nerve  Sensory:  SVA: anterior 2/3 tongue  lingual V3  changeover to chorda tympani to join VII nerve  geniculate ganglion  GSA: fewer in number; external ear & external auditory canal  central course on VII  geniculate ganglion  GVA: few; mucous membrane of palate & nasopharynx  geniculate ganglion  enter brainstem in intermediate nerve Facial Nerve  Ipsilateral face motor cortex provides bilateral innervation to facial motor neurons of the upper face  But, the face motor cortex projects only contralaterally to facial motor neurons of the lower face  Supranuclear lesions (face motor cortex or internal capsule)  drooping of corner of mouth contralateral to the lesion (CENTRAL SEVEN LESION) Facial Nerve  Peripheral lesions of VII (infranuclear):  Bell’s palsy:        Injury proximal to geniculate ganglion & origin of greater petrosal nerve with ipsilateral findings paralysis of upper & lower portions of face  mucosal secretion in nasal & oral cavities  tear fluid production & salivary gland output  cutaneous sensation external ear & external auditory canal  taste sensation on anterior 2/3 tongue hyperacusis Facial Nerve  Peripheral lesions of VII (infranuclear):  Distal to the geniculate ganglion but proximal to the origin of the chorda tympani & stapedial nerve    Ipsilateral  salivation & taste, hyperacusis,  facial expression Intact tear fluid production & mucosal surfaces (nasal & oral cavities) are unaffected b/c greater petrosal nerve is intact Caveat: Lesions distal to or @ stylomastoid foramen  ipsil.  function of all facial muscles in the absence of parasympathetic or taste dysfunction Facial Nerve  Corneal reflex:   Afferent limb travels via V1 (ophthalmic)  trigeminal ganglion  spinal trigeminal tract  spinal trigeminal nucleus  trigeminothalamic fibres  facial motor nucleus  thalamus Facial diplegia    Myotonic muscular dystrophy Mobius syndrome (upper > lower facial weakness typically, extraocular palsies, skeletal & extremity defects) Lyme disease, GBS, Botulism poisoning & Corynebacterium diphtheriae Mobius syndrome Verzijl, Harriette T.F.M., van der Zwaag, Bert, Cruysberg, Johannes R.M., Padberg, George W. Mobius syndrome redefined: A syndrome of rhombencephalic maldevelopment Neurology 2003 61: 327-333 Facial Nerve  Hemifacial spasms:    Irregular painful facial muscle contractions May be precipitated by voluntary facial movements or follows Bell’s palsy Can be 2° to compression of facial nerve root (e.g abnormal AICA branches) Abducens Nerve - Motor  Abducens nucleus:    Internal to the facial colliculus, in rhomboid fossa floor just lateral to median sulcus, above stria medullaris (IV ventricle) Contains motor neurons (GSE) & interneurons Abducens nerve exits @ pons-medulla junction (pre-olivary sulcus)  cavernous sinus in close association with ICA  superior orbital fissure  ipsil. lateral rectus (LR) Abducens Nerve - Motor  Interneurons in VI nucleus  contralateral MLF  oculomotor nucleus (ipsi to MLF)  medial rectus (MR)  Abducens fibres injury e.g. medial pontine syndrome  flaccid paralysis of ipsilateral lateral rectus muscle (introverted eye, impaired ipsil. abduction) Abducens Nerve - Motor   Abducens nucleus injury (motor- & interneurons) e.g. IV ventricle tumour invading facial colliculus  paralysis ipsi LR + contra. MR on gaze toward side of lesion (LMN of LR + INO) Medial longitudinal fasciculus lesions  internuclear ophthalmoplegia e.g. MS    Injury only to internuclear axons Ipsil abduction intact ± nystagmus but adduction of contralateral eye is impaired Preserved adduction with convergence (vs. III ) MLF - INO Abducens Nerve - Motor  One and a Half syndrome:   Seen with pontine lesion affecting abducens nucleus & fibres + adjacent MLF  No movement of ipsilateral eye horizontally, contralateral eye horizontal movements restricted to abduction ± nystagmus Cortical Influence:  Frontal eye fields (FEF)  bilateral projection to PPRF (horizontal gaze centre) + ipsil. superior colliculus (SC)  SC  contralateral PPRF; PPRF  ipsil VI nucleus  Cortical damage (e.g. CVA, trauma) to FEF  involuntary conjugate deviation of eyes to the side of the lesion One-and-a-half syndrome. This results from a pontine lesion (shaded area) involving the paramedian pontine reticular formation (lateral gaze center) and medial longitudinal fasciculus, and sometimes also the abducens (VI) nucleus, and affecting the neuronal pathways indicated by dotted lines. Attempted gaze away from the lesion (A) activates the uninvolved right lateral gaze center and abducens (VI) nucleus; the right lateral rectus muscle contracts and the right eye abducts normally. Involvement of the medial longitudinal fasciculus interrupts the pathway to the left oculomotor (III) nucleus, and the left eye fails to adduct. On attempted gaze toward the lesion (B), the left lateral gaze center cannot be activated, and the eyes do not move. There is a complete (bilateral) gaze palsy in one direction (toward the lesion) and one-half (unilateral) gaze palsy in the other direction (away from the lesion), accounting for the name of the syndrome. Clinical Neurology – 6th ed. Greenberg et. al Cranial Nerves of the Pons (CN V, IV, III) Trigeminal - Motor & Sensory  Introduction:  Largest CN, exits from lateral pons  Large sensory root (portio major) & small motor root (portio minor)  Exits border between basilar pons & middle cerebral peduncle V1 V2 V3 Trigeminal - Motor & Sensory  Brief review:  Sensory nuclei (caudal  rostral):  Spinal trigeminal nucleus (pars caudalis, interpolaris & oralis), principal sensory nucleus & mesencephalic nucleus  GSA exteroception: Pain, thermal & nondiscriminative touch fibres from head  trigeminal ganglion + geniculate ganglion + superior ganglia for CN IX & X (ext. ear)  spinal trigeminal tract/nucleus  GSA exteroception for discriminative touch  as above but go to principal sensory nucleus Trigeminal - Motor & Sensory  GSA proprioceptive input from masticatory muscles, EOM & periodontal ligament receptors  mesencephalic nucleus  Sensory input to spinal trigeminal nucleus & to principal sensory nucleus  anterior & posterior trigeminothalamic tracts  thalamus  somatosensory cortex Trigeminal - Motor & Sensory    Motor nucleus of V  1st pharyngeal arch (medial & lateral pterygoid, tensor tympani, tensor veli palatini, mylohyoid, & anterior belly digastric) The motor root of V exits in the foramen ovale along with V3. Jaw Jerk reflex:  Masticatory muscle receptors  V2  mesencephalic nucleus  bilateral projections from afferent collaterals  trigeminal motor nucleus Trigeminal - Motor & Sensory   Corneal (previously discussed) Lesions of V nerve or central nuclei  Sensory sx in nerve distribution & masticatory muscle paralysis  Sensory deficits include:    Complete loss of pain, temperature, tactile sensation ipsilateral face & scalp Loss of above sensations in oral cavity ipsil. Loss of corneal reflex Trigeminal - Motor & Sensory  Tic douloureux (Trigeminal neuralgia)         Severe paroxysmal attacks of lacinating pain restricted to ≥ 1 subdvisions of V Trigger zones – lip, nose, cheek Precipitants e.g. shaving, make-up application, chewing, talking etc. Possible malnourishment!!! Patients usually > 35 years Maxillary > Mandibular > Ophthalmic Associated with MS, degenerative changes in trigeminal ganglion, vascular abn (e.g. SCA) – contradicting autopsy evidence Status trigeminus  tic like contractions masticatory muscles Trigeminal - Motor & Sensory  Motor deficits:  V root lesion  ipsil masticatory muscle weakness, jaw deviation to weak side (unopposed contralateral pterygoid)  Central lesions (tumour, AVM, mets, vascular obstruction)  Lateral Medullary syndrome (Wallenberg syndrome) Cranial Nerves of the Midbrain (CN IV, III) Trochlear – Motor  Only motor CN to cross midline prior to exit & has long intracranial course  Course: Trochlear nucleus (post. to MLF) @ level of IC  arch around PAG  decussates in anterior medullary velum  exits brainstem immediately caudal to the contra. IC  GSE motor axons  superior cistern  ambient cistern  dura  cavernous sinus  superior orbital fissure  superior oblique (inf-lat) Trochlear – Motor   Trochlear motor neurons innervate contralateral superior oblique MLF + trochlear nucleus lesion e.g. MS:    Contralateral S.O. paralysis Ipsil INO Cotical input:     FEF  rostral interstitial nucleus of the MLF & superior colliculus riMLF = vertical gaze centre riMLF  larger projection to ipsil IV nucleus & smaller projection to contra nucleus FEF injury involuntary conjugate gaze deviation to side of lesion Oculomotor – Motor  Situated within ventral PAG, posterior to MLF, in rostral half of midbrain  GSE  all EOM except SO & LR  Ipsilateral innervation except for SR neurons (decussate within the nucleus)  Just posterior to III complex is EdingerWestphal nucleus (GVE – PNS)  ciliary ganglion Oculomotor – Motor  Course:    III nerve fibres travel ventrally through & around the red nucleus  exit @ interpeduncular fossa  cavernous sinus  superior orbital fissure Superior & inferior divisions of III in the orbit  few branches to ciliary ganglion Ciliary ganglion (PNS)   short ciliary nerves  sphincter pupillae & ciliary muscles Oculomotor – Motor  Post ganglionic sympathetic fibres from SCG travel on ICA plexus  ophthalmic artery  optic canal  ciliary ganglion directly, nasociliary branch (V2) or oculomotor on route to ciliary ganglion  In ciliary ganglion: SNS fibres continue directly into short ciliary nerves to dilator pupillae muscle, and others travel further via III nerve  superior tarsal muscle Oculomotor – Motor     Sensory input from the orbit via frontal & nasociliary nerves  V1  trigeminal ganglion The III nucleus does not receive direct cortical input via the corticobulbar system Cortical control of III neurons via riMLF and SC; SC also projects to the riMLF; riMLF send significant input to ipsi III, less on contralateral side Cortical damage to FEF  involuntary conjugate deviation towards the side of lesion Oculomotor – Motor  Lesions of III nucleus, oculomotor nerve in interpeduncular cistern or III in cavernous sinus:  EOM paralysis except for SO & LR ( abducted & depressed)  Diplopia  Pupil dilation, non-reactive to light, defective accommodation & ptosis  Note that because of peripheral location of PNS fibres, subtle initial signs with external compression may be ptosis or mildly diminished pupil reactivity prior to EOM dysfunction  DM: EOM dysfunction sine visceromotor changes  Ischemia affects the larger internal GSE motor axons 1st
 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
									 
                                             
                                             
                                             
                                             
                                             
                                             
                                             
                                             
                                             
                                             
                                            