Supranuclear Internuclear

1

CN3Nucleus

CN4Nucleus

CN6NucleusNuclear

Supranuclear

Infranuclear

Internuclear

Fascicular

Subarachnoid

Cavernous sinus

Orbital^^ MLF

Neuromuscular junction

Extraocular muscle

This slide captures one way to think about the motility disorders. If it is unfamiliar, I strongly suggest you review the slide-set entitled ‘Motility disorders: Overview’ before proceeding.

Motility Disorders: Fascicular Syndromes

2

CN3Nucleus

CN4Nucleus

CN6NucleusNuclear

Supranuclear

Infranuclear

Internuclear

Fascicular

Subarachnoid

Cavernous sinus

Orbital^^ MLF


Extraocular muscle

In this slide-set, we’ll take a look at fascicular syndromes


Subarachnoid

Cavernous sinus

OrbitalNeuromuscular junction

Extraocular muscle

3

Supranuclear

Infranuclear

Internuclear

?

?

?

Fascicular

CN3Nucleus

CN4Nucleus

CN6NucleusNuclear ^^ MLF

As mentioned in the Overview slide-set, the fascicles (along with the cranial-nerve nuclei) are located within the brainstem. Given this, it shouldn’t come as a surprise that, generally speaking, lesions of the nuclei and/or fascicles do not present as isolated EOM abnormalities; ie, the ophthalmoparesis is almost always accompanied by nonocular signs and symptoms of CNS damage.


Subarachnoid

Cavernous sinus

OrbitalNeuromuscular junction

Extraocular muscle

4

Supranuclear

Infranuclear

Internuclear

?

?

?

Fascicular

CN3Nucleus

CN4Nucleus

CN6NucleusNuclear ^^ MLF

As mentioned in the Overview slide-set, the fascicles (along with the cranial-nerve nuclei) are located within the brainstem. Given this, it shouldn’t come as a surprise that, generally speaking, lesions of the nuclei and/or fascicles do not present as isolated EOM abnormalities; ie, the ophthalmoparesis is almost always accompanied by nonocular signs and symptoms of CNS damage.

As we will see, the fascicular syndromes are defined by the nonocularCNS signs/symptoms that accompany the ophthalmoparesis


CN3 Fascicular Syndromes

Q5

Syndrome

? Contralateral hemiplegia Cerebral peduncle

? Contralateral involuntary movements (often a hand flap)

Red nucleus &substantia nigra

? Contralateral ataxia Cerebellar peduncle

? Ipsilateral ataxia Cerebellar peduncle


The BCSC Neuro book discusses four CN3 fascicular syndromes.What are their names?


A6

Syndrome

Weber Contralateral hemiplegia Cerebral peduncle

Benedikt Contralateral involuntary movements (often a hand flap)


Claude Contralateral ataxia Cerebellar peduncle

Nothnagel Ipsilateral ataxia Cerebellar peduncle


The BCSC Neuro book discusses four CN3 fascicular syndromes.What are their names?


Q7

Syndrome Ipsilateral 3rd plus…







Each is composed in part by a third nerve palsy. Is the palsy ipsilateral, or contralateral to the side of the lesion?

Ipsi- vscontralateral


A8








Each is composed in part by a third nerve palsy. Is the palsy ipsilateral, or contralateral to the side of the lesion?


Q9


Weber Cerebral peduncle






Each has an associated nonocular finding. What is that finding for…Weber syndrome?

?


A10








Each has an associated nonocular finding. What is that finding for…Weber syndrome?


Q11








Each has an associated nonocular finding. What is that finding for…Benedikt syndrome?

?


A12








Each has an associated nonocular finding. What is that finding for…Benedikt syndrome?


Q13








Each has an associated nonocular finding. What is that finding for…Claude syndrome?

?


A14








Each has an associated nonocular finding. What is that finding for…Claude syndrome?


Q15








Each has an associated nonocular finding. What is that finding for…Nothnagel syndrome?

?


A16








Each has an associated nonocular finding. What is that finding for…Nothnagel syndrome?


17








I remember that Claude and Nothnagel involve ataxia by noting that, of the four CN3 syndromes, they are the only to have a letter in their nameA is for ataxia!


18








Note that Nothnagel’s is the only fascicular syndrome for which the nonocular findings are ipsilateral

Nonocular CNS findings are contralateral


19










Nothnagel syndromeis…


20










Nothnagel syndrome

contralateral!is…


Q21

Syndrome Ipsilateral 3rd plus… Lesion location

Weber Contralateral hemiplegia


Claude Contralateral ataxia

Nothnagel Ipsilateral ataxia


What is the location of the lesion in…Weber syndrome?

?


A22







What is the location of the lesion in…Weber syndrome?


Q23







What is the location of the lesion in…Benedikt syndrome?

?


A24








What is the location of the lesion in…Benedikt syndrome?


25








What is the location of the lesion in…Benedikt syndrome? I remember this by thinking of Benedict Arnold,

the infamous Revolutionary War traitor—ie, he was a ‘Red’


Q26








What is the location of the lesion in…Claude syndrome?

?


A27










28









Note: Similar, but not the same!


Q29








What is the location of the lesion in…Nothnagel syndrome?

?


A30








What is the location of the lesion in…Nothnagel syndrome?


31








Note: Not just similar--the same!


32








I remember this because it just makes sense to me that ataxia will be due to a cerebellar lesion

Q33



The BCSC Neuro book discusses two CN6 fascicular syndromes.What are their names?

Syndrome Ipsilateral 6th plus…

? Ipsilateral CN7 and contralateral hemiplegia

?Ipsilateral CN7 & facial hypoesthesia, and

loss of taste to anterior tongue, andcontralateral hemiplegia

A34




Millard-Gubler Ipsilateral CN7 and contralateral hemiplegia

FovilleIpsilateral CN7 & facial hypoesthesia, and


The BCSC Neuro book discusses two CN6 fascicular syndromes.What are their names?





Q35


CN6 Fascicular SyndromesIpsi- vscontralateral

Each is composed in part by a sixth nerve palsy. Is the palsy ipsilateral, or contralateral to the side of the lesion?

A36







Each is composed in part by a sixth nerve palsy. Is the palsy ipsilateral, or contralateral to the side of the lesion?

Q37







Each has an associated nonocular finding(s). What is that finding for…Millard-Gubler syndrome?

?

A38







Each has an associated nonocular finding(s). What is that finding for…Millard-Gubler syndrome?

Q39







Each has an associated nonocular finding(s). What is that finding for…Foville syndrome?

?

A40




FovilleIpsilateral CN7 and contralateral hemiplegia

and facial hypoesthesia andloss of taste to anterior tongue



41








Note that both include an ipsilateral 7th along with contralateral hemiplegia…

42








…but Foville includes a number of other findings

Q43


Syndrome Ipsilateral 6th plus… Lesion location





What is the location of the lesion in…Millard-Gubler syndrome?

?

A44



Millard-Gubler Ipsilateral CN7 and contralateral hemiplegia Ventral pons




What is the location of the lesion in…Millard-Gubler syndrome?

Q45







What is the location of the lesion in…Foville syndrome?

?

A46






Tractus solitarius


What is the location of the lesion in…Foville syndrome?

47






Tractus solitarius









(No question--summary/review slide)

48

CN3Nucleus

CN4Nucleus

CN6NucleusNuclear

Supranuclear

Infranuclear

Internuclear

Fascicular

Subarachnoid

Cavernous sinus

Orbital^^ MLF


Extraocular muscle

Finally, we will discuss Gradenigo syndrome. Gradenigo’s is not, repeat not, a fascicular syndrome (because the nerve fascicle is not involved).

Motility Disorders: Fascicular SyndromesGradenigo^

49

CN3Nucleus

CN4Nucleus

CN6NucleusNuclear

Supranuclear

Infranuclear

Internuclear

Fascicular

Subarachnoid

Cavernous sinus

Orbital^^ MLF


Extraocular muscle

Finally, we will discuss Gradenigo syndrome. Gradenigo’s is not, repeat not, a fascicular syndrome (because the nerve fascicle is not involved). Rather, it involves the subarachnoidcourse of the nerve. (We’re discussing Gradenigo’s in this slide-set only because it doesn’t fit well in any other.)


Q50

Syndrome ?

Gradenigo

Gradenigo Syndrome


Which oculo-motor nerve is involved in Gradenigo syndrome?

A51


Gradenigo

Gradenigo Syndrome


Which oculo-motor nerve is involved in Gradenigo syndrome?

Q52


Gradenigo

Gradenigo Syndrome


Gradenigo syndrome involves a sixth nerve palsy. Is the palsy ipsilateral, or contralateral to the side of the lesion?

Ipsi- vscontralateral

A53


Gradenigo

Gradenigo Syndrome


Gradenigo syndrome involves a sixth nerve palsy. Is the palsy ipsilateral, or contralateral to the side of the lesion?

Q54


Gradenigo ?

Gradenigo Syndrome


Gradenigo syndrome has an associated nonocular finding. What is it?

A55


Gradenigo Ipsilateral facial pain

Gradenigo Syndrome


Gradenigo syndrome has an associated nonocular finding. What is it?

Q56


Gradenigo Ipsilateral facial pain ?

Gradenigo Syndrome


Where is the lesion located?

A57


Gradenigo Ipsilateral facial pain Petrous bone

Gradenigo Syndrome


Where is the lesion located?

Q58



Gradenigo Syndrome


What is the fundamental pathogenesis in Gradenigo’s?

A59



Gradenigo Syndrome


What is the fundamental pathogenesis in Gradenigo’s?Chronic inflammation in the mastoid air cells of the petrous portion of the temporal bone knocks out CN6 and the first two divisions of CN5

Q60



Gradenigo Syndrome



By what noneponymous name is Gradenigo’s syndrome also known?Petrous apex syndrome

A61



Gradenigo Syndrome



By what noneponymous name is Gradenigo’s syndrome also known?Petrous apex syndrome

Q62



Gradenigo Syndrome



Which population is especially at risk?

A63



Gradenigo Syndrome



Which population is especially at risk?Children

Q64



Gradenigo Syndrome




Which group of children is especially at risk?

Q/A65



Gradenigo Syndrome




Which group of children is especially at risk?Those who suffer chronic and recurrent bouts of otitis mediatwo words

A66



Gradenigo Syndrome




Which group of children is especially at risk?Those who suffer chronic and recurrent bouts of otitis media

Q67



Gradenigo Syndrome





In addition to pain, what other ipsilateral ‘face finding’ is often found in Gradenigo’s syndrome?Hemifacial palsy

A68



Gradenigo Syndrome





In addition to pain, what other ipsilateral ‘face finding’ is often found in Gradenigo’s syndrome?Hemifacial palsy

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Supranuclear Internuclear