Cranial Neuropathies Lecture Content
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Neurology Maintenance of Certification: Section 9 Cranial Nerve Palsies Lecture Content: Neuropathies Olfactory Facial Optic Vestibulocochlear Cranial Neuropathies Oculomotor Glossopharyngeal Maria Baldwin, MD Trochlear Spinal accessory Assistant Professor Trigeminal Hypoglossal Epilepsy, Department of Neurology Abducens Loyola University Medical Center, Maywood, IL [email protected] 2 Question 1: A 27 year old female presents with Question 2: A 37 year old male presents six months of right shoulder weakness. She lost with left facial weakness involving the consciousness 6 months ago and landed on a forehead and lower face and reduced taste radiator pipe, sustaining a burn injury to her right sensation. Loud sounds are bothersome. lateral neck. She can raise her arm to 90 °but no higher. There is unilateral scapular winging with What treatment should be offered? arms abducted. What is the affected muscle or A.A. Aspirin 81 mg daily muscles? B.B. Acyclovir only A.A. Serratus anterior C.C. Acyclovir and prednisone B.B. Trapezius D.D. Prednisone only C.C. Deltoid 3 D.D. Rhomboid 4 Maria Baldwin © 2011 BeatTheBoards.com 877-225-8384 1 Neurology Maintenance of Certification: Section 9 Cranial Nerve Palsies Question 3: A 43 year old male presents Question 4: A 73 year old male presents with left-sided weakness and neglect. His with right shoulder droop and weakness of examination is notable for mild right ptosis. head turning, difficulty swallowing, and Eyes are midposition. The right pupil is softening of speech. He reports decreased reduced in diameter compared to the left. taste sensation. There is unilateral palatal What muscle is most likely involved? droop noted on examination. Where is the A.A. Levator palpebrae most likely site of injury? A.A. Median midbrain B.B. Tarsal (Müller’s muscle) B.B. Rostral pons C.C. Orbicularis oris C.C. Lateral medulla D.D. Superior rectus 5 6 D.D. Jugular foramen Question 5: A 41 year old Caucasian male presents for acute onset hearing Olfactory Nerve Pathway loss. Examination is notable for bilateral Olfactory bulb keratitis and an ataxic gait. What is the Olfactory receptors most likely diagnosis? located in wall of the nasal cavity A.A. RamsayRamsay--HuntHunt syndrome Penetrate cribiform plate of ethmoid bone -->> B.B. TolosaTolosa--HuntHunt syndrome olfactory bulb C.C. Multiple sclerosis 22ndnd --orderorder neurons course D.D. Cogan’s syndrome posteriorly as the olfactory tract Receptors 7 8 Crossed and uncrossed Maria Baldwin © 2011 BeatTheBoards.com 877-225-8384 2 Neurology Maintenance of Certification: Section 9 Cranial Nerve Palsies Olfactory Nerve (cont.) Olfactory Neuropathies Pathway AnosmiaAnosmia--lacklack of smell Fibers go to Common cold Olfactory tract Most common cause of bilateral Frontal lobe Olfactory bulb transient anosmia Temporal lobe Head trauma •• Terminate in the amygdala Damage to fibers over cribriform nucleus, hypothalamus, plate septal nuclei Back/side impact more damaging than frontal impact Only sensory nerve that Closed head injury can produce avoids the thalamus impairment of recognition despite preserved detection Cortical representation is Neurodegenerative disease bilateral Sensitive as an initial deficit •• Alzheimer’s, Parkinson’s, Unilateral lesions distal to Receptors Huntington’s disease the decussation do not Other 9 10 produce anosmia Cystic fibrosis and adrenal insufficiency Olfactory Neuropathies Optic Nerve Foster Kennedy syndrome Course Noted with olfactory groove or sphenoid ridge 50 mm long with 4 parts masses Intraocular (nerve head) Commonly seen with meningiomas Intraorbital Ipsilateral anosmia due to direct pressure on the olfactory bulb Intracanalicular Intracranial Ipsilateral optic atrophy due to injury of the ipsilateral optic nerve Optic neuropathies Contralateral papilledema due to raised ICPICP Anterior ischemic optic Do not confuse with….Pseudo Foster Kennedy neuropathy (AION) syndrome Posterior ischemic optic May be noted when increased ICP of any cause occurs in a patient with previous unilateral optic atrophy neuropathy (PION) No anosmia! Optic neuritis Most often due to sequential anterior ischemic optic Leber’s optic neuropathy 11 neuropathy 12 Maria Baldwin © 2011 BeatTheBoards.com 877-225-8384 3 Neurology Maintenance of Certification: Section 9 Cranial Nerve Palsies Optic Neuropathy Anterior ischemic optic neuropathy (AION) Optic Neuropathy More likely in those >50>50,, acute onset, minimal pain, limited recovery, altitudinal defect Optic neuritis Unilateral optic disc swelling Demyelinating inflammatory condition 2 types More likely in those <40, subacute onset, NonNon--arteriticarteritic ––MostMost common, painless, acute, altitudinal painful, good recovery Arteritic ––GreaterGreater than 70 years of age •• Usually giant cell arteritis Four subtypes Associated with HTN, DM, OSA, hypercholesterolemia Retrobulbar neuritis: optic nerve Posterior ischemic optic neuropathy (PION) Papillitis: optic disc Similar to AION but ischemia behind the optic disc Do not appreciate optic disc swelling Perineuritis: optic nerve sheath (sparing the nerve) Bilateral PION commonly seen with cardiac and spinal surgeries •• Infection (syphilis) Surgeries greater than 6 hours •• Sarcoid 13 Patients with DM and carotid atherosclerosis 14 Neuroretinitis: swelling of the nerve & macula Optic Neuritis Clinical features Differential diagnosis Optic Neuritis Vision loss Commonly seen with GradualGradual--occursoccurs hours to inflammatory/autoimmu MRI days ne disease GdE fat saturated T1T1-- Nadir within 11--22 weeks, Multiple sclerosis recovery within 22--44 weighted MRI of the Neuromyelitis optica weeks, 66--1212 months for orbits best sequence nerve to fully heal Syphilis 2/3rds have 20/20 vision GdE shows enhancement Cat scratch disease once recovered in 95% of cases Sarcoidosis Eye pain Rarely occurs in AION Lupus 87% report pain, worse with movement Those without Loss of color vision concomitant brain 88% with abnormal color vision (usually red and green) lesions have a 25% risk Relative APD of MS vs. 72% with 15 Persists in >90% of cases 16 lesions Maria Baldwin © 2011 BeatTheBoards.com 877-225-8384 4 Neurology Maintenance of Certification: Section 9 Cranial Nerve Palsies Optic Neuropathies Oculomotor Nerve Leber optic neuropathy Function Extraocular muscles Genetic cause of optic neuropathy Superior rectus Point mutation in mitochondrial DNA Medial rectus Adolescent males Inferior rectus Inferior oblique Painless vision loss over weeks to months Levator palpebrae 1. Annulus tendinous 7. Trochlea of S.O. Cardiac anomalies: atrioventricular conduction Constricts the pupil 2. Superior rectus 8. Inferior oblique pathway defects (Wolf(Wolf--ParkinsonParkinson white) Accommodates 3. Inferior rectus 9. Levator palpebrae Converges 4. Medial rectus 10. Eyelid 5. Lateral rectus 11. Eyeball 6. Superior oblique 12. Optic nerve 17 18 Oculomotor Nerve Pathway Oculomotor Neuropathies Exits medial midbrain between midbrain & pons Posterior cerebral artery Categorized by location Runs between the SCA and Nuclear lesions PCA Classic 3 rdrd nerve Then parallel to the posterior Parinaud’s syndrome palsy communicating artery Fascicular lesions Eye is “down & out” Parasympathetic fibers ride CN III Weber Dilated pupil atop the nerve Claude Through cavernous sinus Paralysis of Benedikt accommodation Exits at superior orbital (cycloplegia) fissure Subarachnoid lesions Ptosis Splits into 2 divisions Superior cerebellar Carvernous sinus lesions artery •• Superior division Tolosa Hunt syndrome 19 20 •• Inferior division Maria Baldwin © 2011 BeatTheBoards.com 877-225-8384 5 Neurology Maintenance of Certification: Section 9 Cranial Nerve Palsies Oculomotor Neuropathies Oculomotor Neuropathies Nuclear lesions Causes Fascicular lesions •• Pinealomas Parinaud’s syndrome •• Multiple sclerosis Weber’s syndrome Lesion location •• Stroke Lesion location •• Dorsal midbrain •• Hydrocephalus •• Base of midbrain •• Periaqueductal grey ––VPVP shunt failure •• CNIII Clinical features •• Cortical spinal tracts •• Supranuclear upgaze Dorsal paralysis Clinical features rdrd •• Setting sun sign Periaqueductal grey •• Ipsilateral 3 nerve palsy ––ConjugateConjugate downgaze •• Contralateral hemiplegia in primary position •• Convergence and Lesion Eyelid retraction CNIII fibers 21 ––Collier’sCollier’s sign 22 Spinal tracts Ventral Oculomotor Neuropathies Oculomotor Neuropathies Red nucleus Fascicular lesions Red nucleus Fascicular lesions Claude syndrome Benedikt syndrome Lesion location Lesion Location •• CNIII •• CNIII •• Red nucleus •• Red nucleus •• Brachium •• Cortical spinal tract conjunctivum Clinical features Clinical symptoms •• Ipsilateral 3rd nerve •• Ipsilateral 3 rdrd nerve palsy palsy •• Contralateral tremor •• Contralateral ataxia •• Contralateral •• Contralateral tremor hemiplegia CNIII fibers Spinal tracts CNIII fibers 23 24 Maria Baldwin © 2011 BeatTheBoards.com 877-225-8384 6 Neurology Maintenance of Certification: Section 9 Cranial Nerve Palsies Oculomotor Neuropathies Oculomotor Neuropathies Subarachnoid lesions Cavernous sinus lesions Compressive lesions CN III, CNIV and CNVI Resulting in oculomotor paresis Tumors, aneurysms Fixed and dilated pupil •• Dilated, unresponsive pupil ––AbsenceAbsence of an affected pupil with complete motor Trigeminal nerve VV--1,1, VV--22 paresis almost always excludes an aneurysm Sensory loss over •• Posterior