Supranuclear Internuclear

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

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

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

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

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?

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?

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

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

Weber Cerebral peduncle

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

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

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

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

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!

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

Nonocular CNS findings are contralateral

Nothnagel syndromeis…

Nothnagel syndrome

contralateral!is…

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?

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

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’

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

Note: Similar, but not the same!

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

Note: Not just similar--the same!

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

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

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?

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?

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

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

FovilleIpsilateral CN7 and contralateral hemiplegia

and facial hypoesthesia andloss of taste to anterior tongue

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

…but Foville includes a number of other findings

Syndrome Ipsilateral 6th plus… Lesion location

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

Millard-Gubler Ipsilateral CN7 and contralateral hemiplegia Ventral pons

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

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

Tractus solitarius

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

Tractus solitarius

(No question--summary/review slide)

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^

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.)

Syndrome ?

Gradenigo

Gradenigo Syndrome

Which oculo-motor nerve is involved in Gradenigo syndrome?

Gradenigo

Gradenigo Syndrome

Which oculo-motor nerve is involved in Gradenigo syndrome?

Gradenigo

Gradenigo Syndrome

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

Ipsi- vscontralateral

Gradenigo

Gradenigo Syndrome

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

Gradenigo ?

Gradenigo Syndrome

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

Gradenigo Ipsilateral facial pain

Gradenigo Syndrome

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

Gradenigo Ipsilateral facial pain ?

Gradenigo Syndrome

Where is the lesion located?

Gradenigo Ipsilateral facial pain Petrous bone

Gradenigo Syndrome

Where is the lesion located?

Gradenigo Syndrome

What is the fundamental pathogenesis in Gradenigo’s?

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

Gradenigo Syndrome

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

Gradenigo Syndrome

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

Gradenigo Syndrome

Which population is especially at risk?

Gradenigo Syndrome

Which population is especially at risk?Children

Gradenigo Syndrome

Which group of children is especially at risk?

Gradenigo Syndrome

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

Gradenigo Syndrome

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

Gradenigo Syndrome

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

Gradenigo Syndrome

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

Supranuclear Internuclear

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