I am seeing double (again)

Dr Heng Li Wei
2 Apr 2009
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 History
36yo / Bangladeshi / Male
No past medical history
Presented in 2008 with
- L upper eye pain x 4 days, a/w L parietal
headache
- Diplopia x 3 days

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 History
Diplopia
- binocular, horizontal, constant throughout
the day, worse on L gaze.
No head injury, trauma, nausea/vomiting.
No limb weakness/numbness.
No change in voice quality.
Had similar episode of diplopia 5 yrs ago.

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 Examination
VR 6/6 ; VL 6/6
Anterior segment normal.
IOP normal.
EOM

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 Examination

I I I 0 0
R L
I I I 0 0 0 -3
0 0
I I I

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 Examination
C.N. IV, V, VII, VII intact.
No ptosis, fatigue.
No Cogan lid twitch, lid peek sign.
Pupils Normal.
Posterior segment NAD. No disc
swelling.
Forced duction test negative.
114
Hertels:
17 17

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 Orthoptics
AHP: L face turn
Stereo sc : Negative (Frisby)
CT sc : (N) LET 20
(D) LET 30 to 35

LET 20 LET 30 to 35 LET 45 to 50

flick L/R

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Hess / BSV

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Hess / BSV

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 Impression
Left isolated VI cranial nerve palsy
- acute onset (3 days)
- young patient

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 Differential diagnosis
True VIn. palsy Pseudo VIn. palsy
Raised ICP Myasthenia gravis
( Space-occupying lesion or Thyroid eye disease
benign intracranial hypt)
Neoplasm (NPC) Medial wall fracture
Ischaemic Duanes syndrome
Infective Convergence spasm
Inflammatory Esotropia (long-
standing)
Infiltrative
Vasculitic (e.g.
aneurysm) TTSH GRAND ROUNDS
 Investigations
Urgent CT Brain - No intracranial
haemorrhage, infarct, mass effect,
hydrocephalus.
Referred to ENT No PNS lesion.
BP 104/78 mmHg ; H/C 4.8mmol.
Old photo Eyes straight.

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 Investigations
FBC TW 12.7, Hb 14.3, plt 325
ESR 1
RPR non-reactive.
MRI Brain

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MRI Brain

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MRI Brain

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 MRI Brain
Focal enhancing bulge in supero-lateral
aspect of left cavernous sinus, measuring
11.1mm x 6.5mm, extending to left orbital
apex.

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 Differential Diagnosis
Infective/inflammatory e.g. TB, sarcoidosis
Infiltrative lymphoma, meningioma.
Tolosa Hunt syndrome.

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 Progress
Lumbar puncture
- Opening pressure 8mmH2O.
- Clear & colorless
- Hypocount 4.7mmol/L, protein 0.5 g/L.
- RBC 564, Nucleated cells 5 (Neut 2%, Lymph
96%)
- Gram stain & c/s negative.
- AFB smear & TB c/s negative.
- Mycobacterium PCR negative.
- VDRL negative, LIA-Syphilis IgM negative.

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 Progress
Autoimmune screen:
ANA-IFA <80 titre, Anti-dsDNA negative.
Angiotensin-converting enzyme
51 U/L (7 46)
HRCT Thorax
no interstitial lung disease or mediastinal
lymphadenopathy.

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 Progress
Old notes traced from previous episode in
2002 from another hospital
Presented in 2002 with binocular diplopia,
L ptosis, bifrontal headache & eye pain.
L partial III (pupil-sparing), IV, VIn. palsies.
Tolosa Hunt syndrome.
For MRI Brain but pt defaulted.

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 Progress
Imp: Tolosa Hunt syndrome
Started on T. prednisolone 50mg om.
3 weeks after T. PNL diplopia
improved, mild L abduction deficit.
Continued on tapering dose of T. PNL
over 9 wks.
Last seen at Neurology clinic in 2009
Diplopia resolved, EOM full, on T. PNL
10mg EOD and TCU 10 wks.
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 Clinical approach to VIn palsy

VIn. Palsy (abduction deficit)

Isolated VIn palsy Combined with other CN

True VIn. palsy Pseudo VIn. palsy

1. R/O raised ICP papilloedema?

2. Cardiovascular risk factors BP, H/C 1. Hx of trauma / thyroid / MG?
3. Infective / Autoimmune screen 2. Signs of TED, ocular MG
4. Refer ENT R/O NPC 3. Narrowing of palpebral aperture
5. Consider Neuroimaging 4. Forced duction test
6. Preceding viral illness

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 Localization of VIn. palsy

Orbital apex
Petrous apex Brainstem
Cavernous sinus

Subarachnoid space
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 Raymonds syndrome Nuclear VI syndrome
- VI + contralateral hemiparesis - VI, VII + horiz gaze palsy
Millard Gubler syndrome
- VI, VII + contralateral hemiparesis
Fovilles syndrome
- V, VI, VII, VIII + horiz gaze palsy + Horners synd.
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Localization of VIn. palsy

Subarachnoid space
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 @ Subarachnoid space
Raised ICP
Downward displacement of brainstem
Stretching of VIn. which is tethered at its
exit from pons and in Dorello canal
False-localising VIn. palsy.

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Localization of VIn. palsy

Petrous apex

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 @ Petrous apex
Gradenigos syndrome
- VI, VII, VIII
- Localised inflammation or extradural
abscess following complicated otitis media
Petrous bone fracture
Pseudo-Gradenigos syndrome
- NPC, Cerebellopontine angle tumour.

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Cavernous Sinus Syndrome -
Anatomy

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 Cavernous Sinus Syndrome
Typically present with:
Periorbital or hemi-cranial pain
Ipsilateral oculomotor CN palsies (III, IV,
VI), sensory loss in V1 & occasionally V2
distribution.
Oculosympathetic paralysis.

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 Cavernous Sinus Syndrome -
Causes
Vascular
- Aneurysm
(Intracavernous carotid artery or posterior cerebral artery)
- Cavernous sinus thrombosis
- Carotid-cavernous fistula.
Inflammatory / Infective
- Bacterial, viral, fungal, spirochete
- Tolosa Hunt, sarcoidosis, Wegeners granulomatosis, orbital
pseudotumor
Neoplasm
- Primary pituitary adenoma, meningioma, craniopharyngioma.
- Secondary NPC, lymphoma, distant mets.

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 Tolosa Hunt syndrome
Characterised by:
- Painful ophthalmoplegia
- Idiopathic granulomatous inflammation of cavernous sinus.
- * Diagnosis of exclusion.

Clinical features:
- No age or gender predilection.
- Usually unilateral.
- Retro-orbital pain several days (up to 2 weeks) prior to
ophthalmoplegia.
- If left untreated, may resolve spontaneously at ~8 wks.
- Relapses are often.
- Involvement of CN III most frequently (85%), VI (70%), V (30%),
IV (29%). Horners syndrome in 20% of pts.

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 Tolosa Hunt Syndrome
MRI findings:
- Enlargement of cavernous sinus, enhances with
contrast.
- Abnormal convexity of wall of cavernous sinus.
- Focal narrowing of intracavernous ICA.

Treatment:
- Systemic steriods with rapid resolution of pain
w/n 72 hrs, ophthalmoplegia over 2-8 wks.
- No evidence tt steriods alter the prognosis.

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Causes of Painful Ophthalmoplegia
Similar to causes of cavernous sinus
syndrome.
Misc causes
- Diabetic ophthalmoplegia
- Giant cell arteritis
- Opthalmoplegic migraine.

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 The End

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