Sydromes (non brainstem)

Question 1

what is cavernous sinus syndrome

Answer 1

Rare condition characterised by ophthalmoplegia, proptosis, ocular and conjunctival congestion, trigeminal sensory loss and Horner’s syndrome.

These signs and symptoms result from the involvement of the cranial nerves passing through the cavernous sinus.

Question 2

what is carvenous sinus syndrome clinically defined as

Answer 2

Clinically defined as one or more (usually ipsilateral) cranial nerve palsies sometimes associated with pain on the affected side (Tullos, 1991)

Can occur acutely in hours or can be chronic (sometimes developing over years)

Question 3

what are the characteristics of cavernous sinus syndrome

Answer 3

IIIrd, IVth, VIth nerve palsies (various combinations)
oculosympathetic paralysis (Horners syndrome)
proptosis
sometimes ophthalmic and maxillary divisions of the Vth nerve may be affected
periorbital or hemicranial pain
trigeminal neuralgia – sudden, severe facial pain

Question 4

what is the ateiology of cavernous sinus syndrome

Answer 4

Trauma

Vascular including
carotid cavernous fistula
intra-cavernous aneurysm
cavernous sinus thrombosis

Tumours including
cavernous sinus meningioma
pituitary apoplexy (hemorrhagic necrosis of pituitary adenoma)
metastatic tumours

Question 5

What is the management of cavernous sinus syndrome

Answer 5

Depends greatly on the cause. Treat main cause first and then consider ocular problems
Ocular motility review – monitor disease progression

Question 6

what is superior orbital fissure syndrome

Answer 6

Rare condition

AKA Rochen-Duvigneaud syndrome in the medical community

The signs and symptoms result from the involvement of the cranial nerves passing through the SOF.

Question 7

what is the differentiating feature between orbital apex syndrome and superior orbital fissure syndrome

Answer 7

Usually the optic nerve is spared and is the differentiating feature of orbital apex syndrome.

Question 8

what nerves pass through the superior orbital fissure

Answer 8

lacrimal nerve

frontal nerve

inferior division of the ouclomotor nerve

trochlear nerve

superior division of oculomotor nerve

nasocillary division of oculomotor nerve

Question 9

what is the ateiology of superior orbital fissure

Answer 9

Usually occurs due to trauma, injury or foreign body

Question 10

what are the two pathogenic mechanisms that have been identified has the ateiology of superior orbital fissure syndrome

Answer 10

Two pathogenetic mechanisms have been identified:
The direct mechanism - The nerves going through the fissure are interrupted or compressed by displaced bone fragments
The indirect mechanism - In which the orbital walls behave like a non-expandable box, so an increase in pressure will cause symptoms
The symptoms will be complete or partial depending on the degree of compression of anatomical structures

Question 11

what are some of the signs and symptoms that might be demonstrated

Answer 11

Ophthalmoplegia
Ptosis
Proptosis
Fixed and dilated pupils
Paresthesia of forehead (pins and needles sensation)
Hypolacrimation (over flow of tears)
diplopia and decreased visual acuity

Question 12

how to manage superior orbtial fissure syndrome

Answer 12

Depends greatly on the cause. Treat main cause first and then consider ocular problems

These cases are often initially observed for 10-14 days before any surgical intervention (Subhi et al 2019)
Ocular motility review – monitor disease progression

Question 13

what is Tolosa hunt syndrome

Answer 13

Rare disorder characterized by severe periorbital headaches, decreased and painful eye movements (ophthalmoplegia)

Non specific granulomatous inflammation in the anterior part of the cavernous sinus (pseudotumour)

Symptoms usually unilateral

The affected eye often abnormally protrudes (proptosis)

May exhibit signs of paralysis of certain cranial nerves such as drooping of the upper eyelid (ptosis), double vision (diplopia), large pupil, and facial numbness.

Symptoms often will subside without intervention (spontaneous remission) and may recur without a distinct pattern (randomly).

Question 14

ateiology of tosola hunt syndrome

Answer 14

The exact cause of Tolosa-Hunt syndrome is not known,

?? Associated with inflammation of specific areas behind the eye (cavernous sinus and superior orbital fissure).

Question 15

what are the clinical characteristics of Tolosa hunt syndrome

Answer 15

Ophthalmoplegia - ?? involvement IIIrd, IVth and VIth CN
severe ocular pain
loss of sensation over the area covered by the ophthalmic division of the Vth cranial nerve
visual loss if optic nerve is involved
ptosis
sluggish pupils

Question 16

what is oculosympathetic paralysis

Answer 16

OCULOSYMPATHETIC paralysis is a paralysis caused by damage to the sympathetic nerve fibers that innervate the smooth muscles of eye and orbit; it manifests itself clinically by a slight droop of the upper eyelid and a narrowing of the homo-lateral pupil. The paralysis differs from a complete Horner syndrome because anhidrosis of face and forehead, sometimes present in the latter, is absent in oculosympathetic paralysis. Although this type of paralysis has received most attention as an accompanying sign of unilateral vascular headaches in adults1 some clinical reports2 3 4 5 have called attention to its presence after surgery of the middle ear or . . .

Question 17

how is tosla hunt syndrome managed

Answer 17

Exclude serious systemic disease, neoplasms, aneurysms and carotid cavernous fistula
Confirm diagnosis with CT scan (where possible)
Prescribe systemic steroids
Full orthoptic investigation including Hess chart, field of BSV (if any) and measurements

(Afistulais an abnormalconnectionbetween two hollow spaces, such asblood vessels,intestines, or other holloworgans)

Question 18

what is a fistula

Answer 18

Afistulais an abnormalconnectionbetween two hollow spaces (technically, twoepithelializedsurfaces), such asblood vessels,intestines, or other holloworgans

Question 19

how does tosloa hunt syndrome naturally progress

Answer 19

Symptoms may last for weeks or months and return months or years after the initial attack
Response to systemic corticosteroids (e.g. prednisolone) is quick and often rules out other more serious causes

Question 20

what are the differential diagnoses for tosola hunt syndrome

Answer 20

Other causes of non-infectious orbital inflammation: leukaemia, syphillis, tuberculosis, sarcoidosis
Indirect carotid cavernous fistulas will not respond to steroids
Blood tests will confirm the diseases mentioned above and CT scans will confirm the presence of any space occupying lesion
Erythrocyte sedimentation rate (ESR) is often raised in inflammatory conditions such as Tolosa-Hunt syndrome, but not always.

Question 21

what is Gillian barre syndrome

Answer 21

Guillain-Barré (pronounced ghee-yan bar-ray) syndrome is a very rare and serious condition that affects the nerves.

Symptoms often start in your feet and hands before spreading to your arms and legs.:
numbness
pins and needles
muscle weakness
pain
problems with balance and co-ordination

Question 22

what are the characteristics of toslola hunt syndrome

Answer 22

Sudden, acute ascending motor paresis peaking within 4 weeks
Diminished or absent muscle stretch reflexes (tendon reflexes)
Sensory symptoms with minimum objective sensory loss
Electrophysiological evidence of demyelinating neuropathy
CSF albuminocytologic dissociation
Increased protein in the cerebrospinal fluid without increase in cell count, characteristic of the Guillain-Barré syndrome
No pain or elevation of temperature
Recovery is generally complete and within 7 weeks of onset if initial phase is survived

Question 23

what is a characteristic feature of csf in Gullian barre syndrome

Answer 23

increased protein in the cerebrospinal fluid without increase in cell count, characteristic of the Guillain-Barré syndrome; it is also associated with spinal block and with intracranial neoplasia, and is seen in the last phases of poliomyelitis.

Question 24

what are the symptoms of gullian barre syndrome (ocular)

Answer 24

?Ocular nerves can be involved to a varying extent
?Complete ophthalmoplegia may occur
?fixed dilated pupils
?optic neuritis
?facial nerve paresis including ectropion

Question 25

what is the treatment of Gillian barre syndrome during the acute phase

Answer 25

intravenous immunoglobulin (IVIG) – a treatment made from donated blood that helps bring your immune system under control

plasma exchange (plasmapheresis) – an alternative to IVIG where a machine is used to filter your blood to remove the harmful substances that are attacking your nerves

treatments to reduce symptoms such as painkillers

treatments to support body functions, such as a machine to help with breathing and/or a feeding tube
 Most people need to stay in hospital for a few weeks to a few months

Question 26

what is the ateiology of Gillian barre syndrome

Answer 26

Not fully understood

Often occurs following viral infection.

Possibly an allergic response of the peripheral nervous system to an antigen of viral origin.

Widespread inflammation occurs and some demyelination of spinal, peripheral and cranial nerves

Question 27

what is miller fisher syndrome

Answer 27

First described by Fisher in 1956
A form of polyneuritis
Rare, acquired nerve disease that is considered to be a variant of Guillain-Barré syndrome.

Question 28

what is miller fisher syndrome characterised by

Answer 28

It is characterized by abnormal muscle coordination, paralysis of the eye muscles, and absence of the tendon reflexes.

External and internal ophthalmoplegia
(External ophthalmoplegia means paralysis of the extraocular (extrinsic) muscles that move the eyes. Internal ophthalmoplegia means paralysis of the intrinsic (internal) eye muscles that control pupil size and accommodation (focusing))
Ataxia ( difficulty with balance, speech, swallowing, talking)
Tendon hypo-reflexia (abnormal or absent reflexes)

Question 29

what is internuclear opthalmopelgia

Answer 29

Internulcear ophthalmoplegia is caused by nerve damage to the nerve fibers that coordinate lateral eye movement. This leads to double vision. This condition is associated with some other disorders.

Question 30

what is cpeo

Answer 30

Chronic progressive external ophthalmoplegia
Chronic progressive external ophthalmoplegia typically appears in adults between the ages of 18 and 40 years. It typically begins with drooping eyelids and difficulty controlling the muscles that coordinate the eyes.

Question 31

what are the charcaterstics of cpeo

Answer 31

Upper respiratory tract infection
Rapid development of external ophthalmoplegia (development and recovery are symmetrical)
Diplopia
Ptosis (50%)
Almost complete internal ophthalmoplegia
Ataxia: difficulty with balance and walking. speaking. swallowing
Hyporeflexia refers to below normal or absent reflexes (areflexia).
Rapid clinical improvement

hyporeflexiarefers to below normal or absent reflexes (areflexia).

Question 32

how is cpeo diagnosed

Answer 32

Lumbar puncture – increased protein in cerebro-spinal fluid

Axonal changes with low evoked response amplitudes – Electrodiagnostic teststesting

Most carry antibodies against GQ1b

Question 33

what is the ateiology of gullian barre syndrome

Answer 33

Variant of Guillain Barré syndrome without limb weakness

Onset with upper respiratory tract infection is suggestive of viral infection

Ultimately controversy exists over aetiology

Question 34

how is Gillian barre syndrome managed

Answer 34

Exclude presence of other neurological or serious disease
Full orthoptic investigation and serial record of ocular aspects of disease

Intravenous immunoglobins

Murdoch et al (1991) report use of plasmapheresis in a patient with Miller-Fisher syndrome

Question 35

what is the prognosis for patients with Gillian barre syndrome

Answer 35

Despite horrendous symptoms the prognosis for recovery is good – sometimes recovery in 3 months (but can take much longer)

Some evidence to show that it can reoccur

Question 36

what do patients who have Tolosa hunt syndrome take to manage there symptons

Answer 36

presdilone

Question 37

what is the diagnosis for this patient

40 year old female presented to eye casualty with 3 day history of constant diplopia, severe eye pain and ptosis
GH was good. No previous eye problems. No trauma preceding diplopia.-

VA 6/6 either eye
Fundi normal
Normal pupil responses
Moderate RCS with diplopia when lid raised
Ptosis covering most of right pupil
Limitation of RE on dextroversion and dextroelevation
Blood tests and X-rays requested
Prednisolone 40mg daily prescribed

Blood tests and X-ray results normal
Diplopia only present on extreme dextroelevation
Episodic orbital pain getting better
Swelling reduced
Prednisolone reduced to 20Mg for one week, then to nil

Answer 37

tosola hunt syndrome due to symptoms of pain , swelling and diplopia

Question 38

what does this patient have

History:
59 year old male presented c/o horizotal diplopia for 3 days. No headaches, no trauma, no diabetes, hypertension or thyroid disease. Good GH.
Orthoptic examination:VA cgls 6/4 R 6/5 LCT : Nr: NAD D:small ACS with diplopia for distanceOM - small u/a of LE on laevoversion and slight u/a RE on dextroversion PCT N: 1 Δ eso D: 8 Δ eso
Diplopia relieved with 6 Δ Fresnel base out

Patient returned to cas. c/o progressive weakness in legs and slight but transient weakness in right arm 2 days before
Patient admitted for neurological investigations

all cranial nerves intact except VIth
full visual fields
normal fundi
no papilloedema
decreased lower limb tone and muscle power
knee jerk, ankle jerk and plantar responses were decreased
proprioception responses normal
cardiovascular, respiratory and gastro-intestinal systems were normal
chest X-rays, blood tests and Tensilon test normal

Lumbar puncture showed elevated CSF protein level
Limb power deteriorated further and he developed bilateral facial pareses.
3 days later the condition began to improve

Answer 38

gullian barre syndrome

Question 39

what is the diagnosis for this patient

63 year old female noticed horizontal diplopia 1 year prior to attending
Left VIth nerve palsy
VA 6/5 either eye (corrected) N5
PCT nr 1Δeso dist 8ΔesoFurther investigations reported as normal
Fresnel prism prescribed

Complete left VIth nerve palsy
Face turn to compensateInvestigations still normalSome months later:
Slight left ptosis developed
VA still 6/5 either eye

6 months later

acute frontal headache, nausea and vomiting
gradual failure of left vision
increasing left ptosis
6 months later:No PL in LE, small left pupil, complete L ptosis, left IIIrd, IVth,VIth nerve palsies, left corneal anaesthesia, left optic atrophyRight VA, fundus, movement and fields normal

Skull X-rays showed splaying of left superior orbital fissure and erosion of left anterior clinoid process and lateral wall of sphenoid sinus
Carotid angiography showed large aneurysm within cavernous sinus
CT scan confirmed presence of aneurysm
Treatment considered too dangerous

Answer 39

cavernous sinus syndrome