Final-Brainstem Syndromes and multiple Cranial nerve palsies.pptx

Clinical neuroanatomy of brain stem lesions and syndromes of multiple cranial nerve palsies.

1. Brainstem Syndromes and multiple Cranial nerve palsies Dr. Chirayu Regmi UDM-NINAS

2. Brainstem

3. 4 Brain stem arteries - anterior view   1. Posterior cerebral artery 2. Superior cerebellar artery 3. Pontine branches of the basilar artery 4. Anterior inferior cerebellar artery 5. Internal auditory artery 6. Vertebral artery 7. Posterior inferior cerebellar a. 8. Anterior spinal artery 9. Basilar artery

4. MID-BRAIN STOKE SYNDROMES

5. Midbrain

6. Internal structure Transverse section of midbrain Common to both at inferior and superior colliculus: Crus cerebri (or basis pedunculi) : - Consists of fibres descending from cerebral cortex. - Its medial one-sixth is occupied by coticopontine fibres from frontal lobe,lateral one-sixth fibres from temporal,occipital and parietal lobes,the intermediate two third by corticospinal and cortico- nuclear fibres. Substantia nigra : - Present immediately behind and medial to basis pedunculi. - It appears dark as neuron within it contain pigment. ( neuromelanin )

7. Medial midbrain syndrome ( paramedian branches of upper basilar and proximal posterior cerebral arteries) ON SIDE OF LESION Eye "down and out" secondary to unopposed action of fourth and sixth cranial nerves, with dilated and unresponsive pupil: Third nerve fibers ON OPPOSITE SIDE Paralysis of face, arm, and leg: Corticobulbar and corticospinal tract descending in crus cerebri

8. Lateral midbrain syndrome (syndrome of small penetrating arteries arising from posterior cerebral artery) On side of lesion Eye "down and out" secondary to unopposed action of fourth and sixth cranial nerves, with dilated and unresponsive pupil: Third nerve fibers and/or third nerve nucleus On side opposite lesion Hemiataxia , hyperkinesias, tremor: Red nucleus, dentatorubrothalamic pathway

9. Mid brain syndromes

10. Medial midbrain syndrome (paramedian branches of upper basilar and proximal posterior cerebral arteries) ON SIDE OF LESION Eye "down and out" secondary to unopposed action of fourth and sixth cranial nerves, with dilated and unresponsive pupil: Third nerve fibers ON OPPOSITE SIDE Paralysis of face, arm, and leg: Corticobulbar and corticospinal tract descending in crus cerebri

11. Weber syndrome  involves the  cerebral peduncle  and the ipsilateral fascicles of the  oculomotor nerve   1-3,5 . Occasionally the  substantia nigra  can also be involved  5 .  ipsilateral  CN III palsy diplopia ptosis  afferent pupillary defect contralateral  hemiplegia  or  hemiparesis involvement of the  corticospinal  and/or  corticobulbar tracts contralateral parkinsonian rigidity (only if  substantia nigra  involved)  5 involving branches of the  posterior cerebral artery   1-3 .

12. Benedikt syndrome  ( paramedian midbrain syndrome) involves the fascicles of the  oculomotor nerve  and the  red nucleus . ipsilateral  CN III palsy   1-4 crossed hemiataxia   1-4 crossed  choreoathetosis   1-4 ischemic stroke, typically involving branches of the  posterior cerebral artery   1-4 . 

13. Claude syndrome  ipsilateral  oculomotor nerve palsy  and contralateral upper and lower limb ataxia  infarction of the dorsomedial aspect of the  midbrain .  occlusion of the small perforating branches of the  posterior cerebral artery  supplying the dorsomedial aspect of the  midbrain medial aspect of the  red nucleus  with the rubrodentate fibers,  CN III  nucleus and  superior cerebellar peduncle

14. Nothnagel syndrome  involves the tectum of the midbrain ( quadrigeminal plate ) and superior cerebellar peduncles oculomotor nerve  fascicles and  superior cerebellar peduncle , leading to ipsilateral  CN III palsy  and limb ataxia respectively   caused by  stroke , especially hemorrhages rather than infarctions, it is more commonly seen due to neoplasm extending from the tectal plate into the superior cerebellar peduncles

15. Pontine syndromes

16. Pons

17. Subdivided into ventral and dorsal part Ventral part of the pons contains Pontine nuclei: Recieves corticopontine fibres from frontal, temporal,parietal and occipital lobes of cerebrum The efferent fibres form the transverse fibres of pons . Vertically running corticospinal and corticopontine fibres . Transversely running fibres arising in pontine nuclei Pontine nuclei

18. The dorsal part of the pons may be regarded as continuation of the part of the medulla behind the pyramids. Superiorly continous with the tegmentum of the midbrain. Occupied predominately by reticular formation Posterior surface help to form floor of fourth ventricle The dorsal part is bounded laterally by inferior cerebellar peduncle in the lower part of the pons and superior cerebellar peduncle in upper part. Dorsal part of pons DORSAL PART Midpons Upper pons

19. Medial inferior pontine syndrome (occlusion of paramedian branch of basilar artery) On side of lesion Paralysis of conjugate gaze to side of lesion (preservation of convergence): Center for conjugate lateral gaze(PPRF) Nystagmus : Vestibular nucleus Ataxia of limbs and gait: Likely middle cerebellar peduncle Diplopia on lateral gaze: Abducens nerve On side opposite lesion Paralysis of face, arm, and leg: Corticobulbar and corticospinal tract in lower pons Impaired tactile and proprioceptive sense over one-half of the body: Medial lemniscus

20. Lateral inferior pontine syndrome (occlusion of anterior inferior cerebellar artery) On side of lesion Horizontal and vertical nystagmus , vertigo, nausea, vomiting, oscillopsia : Vestibular nerve or nucleus Facial paralysis: Seventh nerve Paralysis of conjugate gaze to side of lesion: Center for conjugate lateral gaze Deafness, tinnitus: Auditory nerve or cochlear nucleus Ataxia: Middle cerebellar peduncle and cerebellar hemisphere Impaired sensation over face: Descending tract and nucleus fifth nerve On side opposite lesion Impaired pain and thermal sense over one-half the body (may include face): Spinothalamic tract

21. Medial midpontine syndrome ( paramedian branch of midbasilar artery ) On side of lesion Ataxia of limbs and gait (more prominent in bilateral involvement): Pontine nuclei On side opposite lesion Paralysis of face, arm, and leg: Corticobulbar and corticospinal tract Variable impaired touch and proprioception when lesion extends posteriorly : Medial lemniscus

22. Lateral midpontine syndrome (short circumferential artery) On side of lesion Ataxia of limbs: Middle cerebellar peduncle Paralysis of muscles of mastication: Motor fibers or nucleus of fifth nerve Impaired sensation over side of face: Sensory fibers or nucleus of fifth nerve On side opposite lesion Impaired pain and thermal sense on limbs and trunk: Spinothalamic tract

23. Medial superior pontine syndrome ( paramedian branches of upper basilar artery) On side of lesion Cerebellar ataxia (probably): Superior and/or middle cerebellar peduncle Internuclear ophthalmoplegia : Medial longitudinal fasciculus Myoclonic syndrome, of palate, pharynx, vocal cords, respiratory apparatus, face, oculomotor apparatus, etc.: —central tegmental bundle. On side opposite lesion Paralysis of face, arm, and leg: Corticobulbar and corticospinal tract Rarely touch, vibration, and position are affected(arm>leg): Medial lemniscus

24. Rt internuclear ophthalmoplegia

25. One and a half syndrome RT LT

26. Lateral superior pontine syndrome (syndrome of superior cerebellar artery) On side of lesion Ataxia of limbs and gait, falling to side of lesion: Middle and superior cerebellar peduncles, superior surface of cerebellum, dentate nucleus Dizziness, nausea, vomiting; horizontal nystagmus : Vestibular nucleus Paresis of conjugate gaze ( ipsilateral ): Pontine contralateral gaze Miosis , ptosis , decreased sweating over face (Horner's syndrome): Descending sympathetic fibers On side opposite lesion Impaired pain and thermal sense on face, limbs, and trunk: Spinothalamic tract Impaired touch, vibration, and position sense, more in leg than arm : Medial lemniscus (lateral portion)

27. Inferior medial pontine syndrome ( Foville Syndrome) infarction of the medial inferior aspect of the pons due to occlusion of the paramedian branches of the  basilar artery   corticospinal tract : contralateral hemiplegia/hemiparesis medial lemniscus : contralateral loss of proprioception and vibration middle cerebellar peduncle : ipsilateral ataxia facial nerve (CN VII) nucleus : ipsilateral facial weakness abducens nerve (CN VI) nucleus : lateral gaze paralysis and diplopia  gaze palsy to the side of the lesion, ipsilateral abducens , and ipsilateral facial palsy and contralateral hemiplegia may be present  1-3 . Crossed hemihypesthesia and ipsilateral cerebellar signs may be present in the more complete form

28. Lateral pontine syndrome Marie- Foix syndrome  lateral aspect of the pons. ipsilateral limb ataxia: cerebellar tracts loss of pain and temperature sensation of the face: spinal trigeminal tract and nucleus facial weakness:  facial nerve (CN VII)   hearing loss, vertigo and nystagmus:  vestibulocochlear nerve (CN VIII) contralateral loss of pain and temperature sensation:  spinothalamic tracts occlusion of perforating branches of the  basilar  and  anterior inferior cerebellar (AICA) arteries   1,2 .

29. Millard- Gubler syndrome ventral pontine syndrome cranial nerves  VI  and  VII  palsies with contralateral body motor or sensory disturbances  1-3 . ipsilateral facial and contralateral body hemiplegia due to pyramidal tract involvement  1-3 isotropism and diplopia that is worsened while the patient looks toward the lesion  1-3 ipsilateral facial paresis and loss of corneal reflex  Lesions in ventral aspect of the caudal pons involving the fibers of the cranial nerves  VI  and  VII  as well as  corticospinal tract   young tumor demyelination (e.g.  MS ) viral infection (e.g.  rhombencephalitis ) older vascular

30. Locked-in syndrome damages the ventral brainstem, pyramidal bundles and corticobulbar tracts  conscious with preserved cognitive function but cannot move or communicate due to the complete paralysis of all voluntary muscles of the body including those of respiration. Mechanical ventilation is therefore required As the stroke spares the midbrain and therefore the  oculomotor nerve , affected individuals can open his/her eyes and vertical eye movements are preserved ventral pontine damage is  basilar artery thrombosis . Other causes include blunt trauma or cardiocirculatory failure

31. Raymond syndrome  posterior circulatory stroke syndrome due to a lesion in the ventral medial mid-pons classic type abducens nerve (CN VI)  - fascicle: ipsilateral lateral gaze paresis corticospinal fibers - undecussated : contralateral hemiparesis corticofacial fibers: facial paresis common type  (more frequently reported) abducens nerve (CN VI)  - fascicle​: ipsilateral lateral gaze paresis corticospinal fibers - undecussated : contralateral hemiparesis NB: corticofacial fibers are spared in the common type

32. Brissaud-Sicard syndrome Brissaud-Sicard syndrome  is a very rare  pontine stroke syndrome  that involves the anterolateral and inferior pons  ipsilateral facial cramps and contralateral hemiparesis  damage to the pons involving the  corticospinal tract  and the  CN VII  nucleus or nerve root  posterior circulation ischemic stroke, as well as other lesions such as neoplasm (e.g.  brainstem glioma

33. Facial colliculus syndrome due to a lesion at the  facial colliculus , involving: abducens nerve (CN VI)  nucleus facial nerve (CN VII)  fibers at the genu medial longitudinal fasciculus Clinical presentation lower motor neuron  facial nerve palsy  proximal to  geniculate ganglion  hence including loss of sensation of the taste in anterior two-thirds of the  tongue  and hyperacusis diplopia horizontal conjugate gaze palsy (including  internuclear ophthalmoplegia ) Causes of facial colliculus syndrome vary by age: younger patients tumor demyelination (e.g.  multiple sclerosis ) viral infection (e.g.  rhombencephalitis ) older patients vascular (e.g. stroke)

34. Gasperini syndrome rare  pontine stroke syndrome  that involves the caudal pontine tegmentum. the syndrome presents with  1-3 : involvement of the  CN V  nucleus ( sensory nucleus of the trigeminal nerve ): ipsilateral facial sensory loss involvement of the  CN VI  nucleus ( abducens nucleus ): ipsilateral impaired eye abduction involvement of the  CN VII  nucleus ( facial nucleus ): ipsilateral facial palsy and hyperacusis involvement of the  CN VIII  nucleus: vertigo and ipsilateral nystagmus involvement of the  spinothalamic tract : contralateral hemi-sensory impairment occlusion of either pontine branches of the  basilar artery  or the long circumferential branch of the  anterior inferior cerebellar artery   1,2 , but can also be seen due to demyelination or hemorrhage  3

35. MEDULLA OBLONGATA

36. Anterior median fissure Pyramid Anterolateral fissure Olive

37. Medial medullary syndrome (occlusion of vertebral artery or of branch of vertebral or lower basilar artery) On side of lesion Paralysis with atrophy of one-half half the tongue: Ipsilateral twelfth nerve On side opposite lesion Paralysis of arm and leg, sparing face; impaired tactile and proprioceptive sense over one-half the body: Contralateral pyramidal tract and medial lemniscus

38. Lateral medullary syndrome (occlusion of any of five vessels may be responsible—vertebral, posterior inferior cerebellar, superior, middle, or inferior lateral medullary arteries) On side of lesion Pain, numbness, impaired sensation over one-half the face: Descending tract and nucleus fifth nerve Ataxia of limbs, falling to side of lesion: Uncertain— restiform body, cerebellar hemisphere, cerebellar fibers , spinocerebellar tract (?) Nystagmus , diplopia , oscillopsia , vertigo, nausea, vomiting: Vestibular nucleus Horner's syndrome ( miosis , ptosis , decreased sweating): Descending sympathetic tract Dysphagia , hoarseness, paralysis of palate, paralysis of vocal cord, diminished gag reflex: Issuing fibers ninth and tenth nerves Loss of taste: Nucleus and tractus solitarius Numbness of ipsilateral arm, trunk, or leg: Cuneate and gracile nuclei Weakness of lower face: Genuflected upper motor neuron fibers to ipsilateral facial nucleus On side opposite lesion Impaired pain and thermal sense over half the body, sometimes face: Spinothalamic tract

39. Affected structures can include: inferior cerebellar peduncle dorsolateral medulla descending spinal tract nucleus of the trigeminal nerve vagus nucleus and nerve glossopharyngeal nucleus and nerve descending sympathetic tract fibers

40. Lateral medullary syndrome Wallenberg syndrome acute ischemia or infarction of the lateral  medulla oblongata  due to occlusion of the intracranial portion of the  vertebral artery ,  PICA  or its branches Lateral medullary syndrome is the most prevalent posterior ischemic stroke syndrome  5 . This syndrome is characterized by: vestibulocerebellar symptoms: ipsilateral hemiataxia , vertigo, falling towards the side of lesion, multidirectional nystagmus ( inferior cerebellar peduncle  and  vestibular nucleus )  1-3 autonomic dysfunction: ipsilateral  Horner syndrome ,  hiccups   1-3 sensory symptoms: loss of pain and temperature over the ipsilateral face (this may be variable  8 ) and contralateral side of body  1-3 ipsilateral bulbar muscle weakness: hoarseness, dysphonia,  dysphagia , and dysarthria, decreased gag reflex ( nucleus ambiguus )  1-3

41. atherothrombotic occlusion of the vertebral artery, the posterior inferior cerebellar artery or the medullary arteries cerebral embolism vertebral artery dissection, the commonest cause in young patients hypoplastic vertebral artery  7

42. Medial medullary syndrome   Déjerine syndrome Secondary to thrombotic or embolic occlusion of small perforating branches from vertebral or proximal basilar artery supplying the medial aspect of  medulla oblongata 1,2 .  contralateral hemiplegia/hemiparesis as well as hemisensory loss with ipsilateral hypoglossal palsy (ipsilateral tongue weakness and atrophy) from involvement of  CN XII  nucleus  1,2 . Other manifestations such as vertigo, nausea, or contralateral limb ataxia are also reported  1,2 .

43. Cestan-Chenais syndrome hemimedullary syndrome  (Reinhold syndrome) and the  lateral medullary syndrome  (Wallenberg syndrome), but distinct to that of  Babinski- Nageotte syndrome  an intermediolateral medullary syndrome, which includes most features of the  lateral medullary syndrome  except ipsilateral hemiataxia , but also having contralateral hemiparesis/hemiplegia  1,2 .  posterior circulation  ischemic stroke   1 .

44. Hemimedullary syndrome  result of the occlusion of the ipsilateral  vertebral artery  proximal to the  posterior inferior cerebellar artery  and its anterior spinal artery branches  1-3 . This situation causes  lateral medullary infarct  and  medial medullary infarct  simultaneously  1-3 .

45. Multiple Cranial Nerve Palsies

46. Lateral wall CN3 : Oculomotor nerve CN4: trochlear nerve CN5-i: opthalmic nerve CN5-ii: maxillary nerve Trigeminal ganglion Centre of sinus -ICA with venous and sympathetic plexus around it -CN6 Cavernous Sinus Syndrome

47. CAVERNOUS SINUS SYNDROME Signs and symptoms: ophthalmoplegia , chemosis , proptosis , Horner syndrome, or trigeminal sensory loss CLINICAL FEATURES -Pure cavernous sinus CN 3, 4, 6, 5i, 5ii, Horner syndrome (depend on extend of involvement) -Superior orbital fissure CN 3, 4, 6, 5i -orbital apex CN 3,4,6, with 5i, CN 2

48. ETIOLOGY Vascular -Aneurysm- intracavernous sinus carotid aneurysm, post cerebral aneurysm -cavernous sinus thrombosis (primary infectious process involving paranasal sinuses and/or orbital cellulitis) -Giant cell arteritis Inflammatory - Tolosa -Hunt syndrome -Wegener granulomatosis - Sarcoidosis -Meningitis -Bacterial-syphilis, TB -Viral-Herpes zoster Tumor - priomary : pituitary adenoma, meningoma -secondary : NPC -lymphoma, metastasis (breast, prostate, lung)

49. Clinical Presentation Cavernous sinus tumors - Acute or slowly progressive - Ophthalmoplegia ,with diplopia being the most common symptom. - painful diplopia + -H/o cancer . First manifestation of a systemic neoplasm. - Exophthalmos Carotid-cavernous aneurysms -Frequently are elderly and present with subacute or chronic ophthalmoplegia . Spontaneous rupture of a carotid-cavernous aneurysm leads to an abrupt onset of a direct CCF . Result in acute onset of massive exophthalmos with orbital, ocular, and conjunctival chemosis , binocular diplopia, and visual loss.

50. Carotid cavernous fistula * Development of arteriovanous fistula ( carotid artery & venous cavernous sinus) Direct: -High flow shunt- carotid artery blood pass direct to cavernous sinus ( defect wall intracavernous ICA ) -trauma 75% (surgery), spontaneous rupture of aneurysm (HPT) Indirect: - dural shunt , intracavernous ICA wall intact -arterial flow through meningeal branch if external or internal carotid arteries indirectly into cavernous sinus

51. Signs and symptoms -Direct *presentation: days-weeks after head injury, classic triad ( pulsatile proptosis , conj chemosis , whooshing noise in head ) *visual loss (acute: ON damage) (delayed: CRVO, ischemic optic neurpathy ) * epibulbar vascular dilatation * chemosis *pulsatile proptosis with bruit/thrill (both can be abolish by ipsilateral carotid Compression) *ptosis(CN3) * ophthalmoplegia (most common =CN6) *may show OD swelling, venous dilatation, intraretinal hemorrhage (venous stasis and impair retinal blood flow)

52. Signs and symptoms -Indirect *presentation: gradual onset redness(one or both eyes) *mild epibulbar vascular dilatation *corkscrew epibulbar vessel *raised IOP, proptosis , mild bruit * ophthalmoplegia *fundus: Normal @ moderate venous Tortousity

53. CPA Cerebellopontine angle= shape (subarachnoid) anterior surface cerebellum & lateral surface pons Content -CN 7,8 -foramen Lushka (4 th ventricle) -Anterior inferior cerebellar artery AICA Relation to Superior- CN 5, Inferior- CN 9( glossopharygeal ),10( vagus ) 11accesory Etilogy - Tumor : Vestibular schwannoma (acoustic neuroma) Nasopharygeal Ca Clivus meningoma – pontine glioma -Trauma : basal skull fracture Cerebellopontine angle syndrome

54. Clinical features -vestibular schwannoma : 80% CPA tumor -first :CN 5, 8 : -decrease facial and corneal sensitivity - nystagmus -next: CN 6,7 -facial paralysis -raised Intracranial pressure-papilledema -hearing loss, tinnitus (hallmark symptom of acoustic neuroma) -unsteadiness Cerebellopontine angle syndrome

55. Lower Cranial Nerve Syndromes J ugular foramen syndrome (JFS) characterized by ipsilateral paralysis of CNs IX, X, and XI. JFS is caused by a lesion at the jugular foramenor in the retroparotid space. Collet-Sicard syndrome is the additional involvement of CN XII. Villaret’s syndrome is Collet-Sicard with the addition of Horner’s syndrome. Glomus tumors (paragangliomas, chemodectomas ) arise from the glomera of the chemoreceptor system- a common cause of JFS.

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