Download BHS 364 Neuro-Ophthalmic Disorders Date: 01/06/17 3rd Hour Note

BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
Date: 01/06/17 3rd Hour
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HORNER’S CONTINUED
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Types of Horner’s
o 1st Order (Central Horner’s): brainstem and spinal cord
 Etiology
 Ischemic vascular (stroke)**
o Most common etiology
 Most commonly in the
medulla
o Brainstem stoke (Wallenberg’s
syndrome):
 Not as common as Locus Ceruleus stroke (described below)
 Lateral medulla
 Vertebral A/PICA infarct
 Ipsilateral Horner’s in 75% of cases
 Loss of pain/temperature (ipsilateral cornea and face,
contralateral trunk and limbs)
 Dysarthria/dysphagia
 Ataxia (ipsilateral limbs)
 Vertigo- “tilted world”
 Nystagmus- tends to be torsional
 Case example: 62 y/o Man
o H/o stroke (3 months)
o Recent onset oscillopsia
 Due to the nystagmus
o Double vision when looking to the right
o Cannot look to the left
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BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
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Date: 01/06/17 3rd Hour
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Medulla: lateral portion
o Grey portion is typically affected by lateral
medullary stroke
Sympathetics are affected in the grey portion (causes
the Horner’s)
The different nuclei that are present in this portion of
the medulla are what are causing the different
symptoms seen in this type of stroke
o
Tumor
o Spinal and neck metastases can cause Horner’s
 Venous drainage from breast to spinal veins
 Metastatic migration of tumor cells to spine/neck
 Horner’s syndrome
 Because damage can occur to the oculosympathetic
fibers during this venous drainage from the breast to
the spinal veins
 Case example: 40 y/o woman
 Hx of breast cancer and s/p mastectomy
 Recent onset lid droop OS
 +anhydrosis and decreased tearing OS
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OS: ptosis and possibly mild reverse ptosis
 Slightly constricted pupil
o Metastasis at spinal cord
Located in the hypothalamus, brainstem, spinal cord
 Horner’s: Brainstem/Ponto-Mesencephalic Junction
o Commonly the location of the stroke
o Area more often called the “Locus Ceruleus”
o
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BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
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Date: 01/06/17 3rd Hour
Page 3
Patient has a Horner’s on the right side
 Slight reverse ptosis
 Difficult to see the miotic pupil in the picture
 Patient also has a head tilt
 This means that the patient has a CN IV palsy on his
left side (Head tilt to the right)
An ipsilateral Horner’s with a contralateral
SO palsy tells you the problem is in the
brainstem before CN IV crosses over. This
area is called the locus ceruleus. It contains
the trochlear nucleus and the sympathetic
fibers run right along next to the trochlear
nucleus. Damage in this area causes the
ipsilateral Horner’s and contralateral SO
palsy.
o
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Close up of the above patient
 Smaller pupil OD
 CN IV palsy OS
nd
2 order (preganglionic Horner’s): apex of the lung, lower neck
 Etiology
 Trauma
o CABG/pacemaker insertion
o Disc herniation,
o Thymectomy
o Whiplash
o Knife wounds
o Epidural anesthesia
 Right picture: shows 1st
order and 2nd order
 Going over the apex
of the lung and
underneath the
subclavian artery
o
o
BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
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Date: 01/06/17 3rd Hour
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o Both places are prone to possible trauma
The thymus sits at the top of the lungs in between them (very near
the oculosympathetics) so that’s why you can have damage to the
sympathetic from a thymectomy
 Case example: 43 y/o woman
o
s/p thymectomy
+ left facial anhydrosis
Difficult to see pupils but OS pupil is
miotic
Case example: 77 y/o woman after CEA surgery
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CEA= Carotid Endarterectomy
Horner’s on right side: miotic pupil and
reverse ptosis
Right side of neck where the surgery
was done
2nd order nerve goes near the common
carotid and was nicked during the
surgery
Case example:
o
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o
Smaller pupil and ptosis OS
BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
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Brachial plexus: runs very close to the sympathetics
Orange: oculosympathetics
Apex of the lung
Phrenic nerve
Vagus nerve: hoarseness and recurrent laryngitis if
this nerve is affected
Tumor (Pulmonary/Thoracic Tumors)
o Case example: 56 yo, droopy lid for one week
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Date: 01/06/17 3rd Hour
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Left brachial plexus injury
Further questioning: pain in back/shoulder, lower arm
numbness
 Patient’s brachial plexus was impacted because she had a
Pancoast’s lung tumor (apical lung tumor)
o Apical lung cancer (Pancoast’s tumor)
 The oculosympathetics travel over the apex of the lung on
their way upward to the cervical ganglion
 PAIN (because of the brachial plexus)
 DYSPNEA (because of the phrenic nerve)
o Neuroblastoma (in kids)
 Pediatric Horner’s
 Urinalysis (creatinine and VMA)
Chest or neck
3rd order (Postganglionic Horner’s)
 Etiology
 Vascular (more common)
 Tumor
BHS 364 Neuro-Ophthalmic Disorders
Date: 01/06/17 3rd Hour
Note taker: Leah Ruhland
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 Painful Horner’s Syndrome: Cavernous Sinus Syndrome, Cluster headaches (Raeder’s
syndrome), Carotid artery dissection)
 Cavernous sinus syndrome
o Mixed cranial neuropathies (III, IV, V-1, VI)
 Lots of nerves go through the cavernous sinus so if
something were to happen there, you’ll get multiple cranial
nerve palsies
 Patients often have poor EOMs and diplopia
o Pain
o Dysesthesia: abnormal sensation
o Miosis (oculosympathetic paresis)
 Or alternating aniscoria
 Pupils don’t look like a typical Horner’s since the
sympathetics and the parasympathetics both run through the
cavernous sinus and therefore both are affected. Basically,
the pupil is fixed (miosis or mydriasis can occur).
o Cavernous sinus anatomy
o
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Case example: 68 y/o man
 c/o progressive ptosis and diplopia
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Not your typical Horner’s ptosis
CN III is involved, hence the dramatic ptosis
Fields of gaze: patient is limited because right eye is
down and out
Aniscoria= looks like a tonic pupil
o Both bright and dim light
o Oculosympathetic and parasympathetics are
both affected
BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
Date: 01/06/17 3rd Hour
Page 7
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o
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Meningioma filling the right side of his cavernous
sinus
Case example: 57 y/o man
 c/o WHOL (Worst Headache of Life)
 h/o recent auto accident
 double vision (distance and near)
 bi-temporal hemianopia (x confrontation)
 indicates an issue in or around the chiasm
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o
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Motilities: right eye is good but left eye cannot do
much
OS: smaller pupil
T1WI image: pituitary apoplexy
o =Blood filling up the pituitary
True Neuro-Ophthalmic Emergencies (the 5 “A” club)
 Pituitary Apoplexy*
 Acute infarction/hemorrhage of pituitary adenoma
 ~10% of all pituitary adenomas
o Most people that have pituitary adenomas are
benign, but ~10% of these patients can have
a bleed and cause an apoplexy.
BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
Date: 01/06/17 3rd Hour
Page 8
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M>F (2:1)
Prior head trauma, radiation, or postpartum
(Sheehan’s syndrome)
 More prevalent with excess pituitary secretions (ex.
Cushing’s syndrome, acromegaly)
 Sign and Symptoms
o Headache (95%)
o Vomiting (69%)
o Ocular motility dysfunction- cavernous sinus
expansion (78%)
o Visual dysfunction:
 Chiasm
 Optic nerve
 Optic tract
o Stupor, coma, and death (SA hemorrhage)
 Can be deadly= hence it is an
emergency!
 Treatment:
o Immediate corticosteroids (if
hypopituitarism)
o Surgical removal of infarcted pituitary
o Life-long hormonal replacement
 Carotid Artery Dissection*
 Aneurysmal CN III Palsy
 Giant Cell Arteritis*
 PapilledemA
Cluster headaches (Raeder’s syndrome): can cause painful Horner’s
o Hemicranial headaches that have a circadian tempo (can wake you up
from sleep)
o Background:
 M>F (6:1)
 “Man’s version of a migraine” (since migraines are
more common in women)
 Smoking, Alcohol, Gastric Disorders
 Can be exacerbated by these things
 Onset by 3rd decade of life
 Paroxysmal, Severe, Hemicranial HA
 Circadian Tempo (wake from sleep)
 Horner’s syndrome- episodic or chronic
 Rule out carotid artery dissection
o Pathophysiology: no one really knows what is happening and why it
happens, but seems to have a similar cause to migraines
 Trigemino-vascular etiology
 Extracranial vasculature (carotid artery)
 Fluctuating serotonin levels
BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
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Date: 01/06/17 3rd Hour
Page 9
 Light/Dark processing (hypothalamus)
o Management:
 Avoid “triggers”
 Traditional Vasoconstrictors
 DHE
 100% O2
 Serotonin (5-HT1) receptor agonists
 Sumatriptan
 Zolmitriptan
 Botox (NANOS-06)
Carotid artery dissection: can cause painful Horner’s
o Background:
 Pain (ipsilateral face/neck)
 Pulsatile noise
 Horner’s syndrome (40-50%)
 Delayed cerebral/retinal ischemia
 50% progress to stroke
 TRUE EMERGENCY!!! (one of the 5 A’s)
o Caused by abrupt head-turning (sports injury, auto accidents,
chiropractic manipulation.)- anything that
can cause a whiplash effect
 Because of this trauma, you get a
hemorrhage developing between
the intima and media of the ICA.
This decreases the size of the
lumen and can completely block
the artery or cause turbulent flow
which can throw off an embolus.
o Management:
 MRI/MRA
 Extracranial distribution of ICA
 Anticoagulation: to avoid bleeding/stroking out
o Case example: 37 y/o man
 Hx of recent auto accident with whiplash injury
 Transient monocular blindness, OD
 Right side neck pain with intracranial noise
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Right side= Horner’s
o Miotic pupil and slight ptosis
BHS 364 Neuro-Ophthalmic Disorders
Note taker: Leah Ruhland
Date: 01/06/17 3rd Hour
Page 10
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MRA
Right ICA: mostly bright with only a small black dot
(lumen)= limited blood flow
o High risk for a stroke
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Imaging of the blood vessels
right: obvious limited flow
left: narrowed area
Dr. Biousse: does a lot of stroke studies abut also neuroophthalmology
Study looking at carotid dissection and the findings associated with
it
Pharmacologic Testing for Horner’s Syndrome
o Diagnosis
 Cocaine – gold standard for determining the presence (not location) of Horner’s
 4-10% (formulated by pharmacy and short-shelf life)
 Indirect sympathomimetic (blocks re-uptake of norepinephrine at presynaptic terminal) – so it will dilate a normal pupil
 Horner’s pupil DOES NOT dilate!
BHS 364 Neuro-Ophthalmic Disorders
Date: 01/06/17 3rd Hour
Note taker: Leah Ruhland
Page 11
o Damage of any of the three neurons results in decreased
norepinephrine released by the third neuron
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Cocaine blocks NE from re-uptake and causes dilation in a normal pupil
In Horner’s there are very few if any NE red dots and cannot cause dilation
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 Can see left pupil dilated some, but right pupil did not  Horner’s
 Cocaine isn’t used often because it has a very short shelf-life and you aren’t seeing
these patients very often
Localization – once the Horner’s is confirmed
 Hydroxyamphetamine
 1.0%
 Indirect acting sympathomimetic (forces norepinephrine out of the presynaptic terminal)
 Requires “intact” pre-synaptic terminal (adequate quanta of
norepinephrine)
 Dilates Horner’s pupil if CENTRAL OR PRE-GANGLIONIC
o If third order neuron (post-ganglionic) is damaged, it will not dilate
 Have to wait several days after using cocaine diagnosis to perform this test
to allow the cocaine to leave their system
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Both pupils dilated  this means that Horner’s is in central or preganglionic neurons (1st or 2nd order) but NOT the 3rd order neuron (postganglionic)
BHS 364 Neuro-Ophthalmic Disorders
Date: 01/06/17 3rd Hour
Note taker: Leah Ruhland
Page 12
o This narrows down the location of the issue when referring to the
neurologist
o But hydroxyamphetamine is no longer commercially available and cocaine is hard to get!
 So these two ways of testing aren’t really available to use
 The alternative to this is Apraclonidine/iopidine:
 0.5% or 1% Apraclonidine in the diagnosis of Horner’s
syndrome
 Weak alpha-1 agonist (along with alpha-2 agonist
properties so it is a similar drug to brimonidine – also
want to apraclonidine on hand for angle closure because
it is fast-acting!)
o Receptors on dilator muscle
 No dilation of normal pupil
o May even cause a slight constriction in a normal
pupil
 Dilation of Horner’s pupil
o Sympathetic denervation supersensitivity
o Doesn’t matter what order of neurons that are affected
o This allows the pupil to react to the alpha-1 agonist activity
 “Reversal of anisocoria” – Horner’s pupil gets larger than it was, and ptosis is
also reversed (will go back to being ptotic once the drugs wear off)
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Note how the OD is the Horner’s eye – and dilated after the apraclonidine was
instilled and the OS did not dilate
Horner’s work-up:
o MRI
o MRA/MRV
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You can appreciate how OS stayed the same and OD (Horner’s pupil) dilated
after an hour
(typically cautious about using this drop in children)
BHS 364 Neuro-Ophthalmic Disorders
Date: 01/06/17 3rd Hour
Note taker: Leah Ruhland
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o Needs to be evaluated right away, especially if you suspect it is one of the emergencies
 Unless you can prove that it is congenital and they are an adult who is fine and
healthy, you need to work the patient up
o It helps if you have some idea on where the issue may be located
 Use case history and patient’s complaints
 Palsies that are present
 All miosis is not Horner’s syndrome
o Causes of miosis:
 Old tonic pupil: end up constricting eventually
 Pharmacologic – pilocarpine, brimonidine
 Iritis
o
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Overview of the different drop testing discussed above
Key points about pupils:
o Afferent pupillary defects
o Light-near dissociation pupils
o Physiologic anisocoria
o Tonic pupils
o Horner’s syndrome