Acute ET in a 42 yo male with recent diarrhoea
OMCFumtiaka Nonaka
17/10/2011 ED (2wk from onset)• 42yo Male• c/o binocular diplopia (mainly horizontal, with
vertical and torsional component) • difficulty in focusing, pain behind the eyes for 2/52• 3-4/52 ago had acute gastroenteritis, pins and
needles, unbalanced• denies: ataxia, inco-ordination, other motor/sensory
symptoms • POH: red-green color blindness• PMH: nil, no head trauma
On Examination in ED• VA: R 6/5-3, L 6/9+3 • Ishihara 2/15, R+L• no RAPD• CT: Alternating ET (PCT: not performed)• EOM: LLR-, RIO+, LSR-, end point nystagmus, normal
saccades, pain on looking up, no INO• Bloods: FBE, U+E normal, CRP<1, RF –ve, TSH WNL,
ANA detected (titre 1:80, <1:80 = negative)• MRI brain (25/10/11): normal, no evidence for
intracranial demyelination
What’s next?
What’s next?
Anti-ganglioside antibodies
04/11/11 OMC (5wk from onset)• VA: R 6/5 L 6/6• HVF: W.N.L. • EOM: RLR-, LLR- (see Hess)• PCT: Near 35ΔET’ LH’6Δ Distance 50ΔET LH6Δ• Bloods:
– GQ1b IgG Ab +ve– MAG IgM IFA –ve– GM1 IgG Ab –ve, GM1 IgM Ab –ve– AChR Ab –ve
• S: still diplopia to sides, after midday• PCT: Near 6ΔET’ LH’3Δ Distance 6ΔET LH2Δ (see Hess)
18/11/11 OMC (7wk)• Much better, SV in am, gradually develops diplopia
as day progresses• EOM RLR-, LLR- (see Hess)• PCT: Near 14ΔET’ LH’3Δ Distance 25ΔET LH3Δ• Saccades fast and accurate, no fatigue
02/12/11 OMC (9wk)
04/11/11 (5wk) 18/11/11 (7wk)
02/12/11 (9wk)
Distance 50ΔET LH6Δ Distance 25ΔET LH3Δ
Distance 6ΔET LH2Δ
Miller-Fisher syndrome
a variant of Guillain-Barre syndrome a triad of ophthalmoplegia, ataxia, and areflexia full triad of MFS is not always present anti-GQ1b ganglioside antibodies +ve in 90% of MFS Campylobacter jejuni, cytomegalovirus, Epstein-
Barr virus, and Streptococcus pyogenes have been reported as antecedent infectious agents in MFS. (J Neurol Sci 1998;160:64–6)
good recovery with or without treatment
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Charles Miller Fisher MD1913 - 2011
Acute conditions Miller-Fisher Syndrome Acute ophthalmoparesis (ophthalmoplegia without ataxia)
Chronic conditionsOtherwise unexplained ophthalmoplegia
Anti-GQ1b antibody
Anti-Gq1bAnti-Gq1b
Immunolocalization of GQ1b and Related Gangliosides in Human Extraocular Neuromuscular Junctions and Muscle Spindles. F. Pedrosa-Domellof et al, IOVS 2009;50:3226 –3232
Abundant staining anti-GQ1b Abs: NMJs of human EOMs > limb muscles
Absence of a blood-nerve barrier High capillary supply
NMJs of EOMs may be easily targeted by anti-GQ1b Abs
*NMJ = Neuromuscular junction
*
Anti-ganglioside antibodies& diplopia
“ANTI GM1 ANTIBODIES – THE CAUSE OF OTHERWISE UNEXPLAINED OPHTHALMOLPLEGIAS?” L Kowal et al, 2003
– Four patients with otherwise unexplained ophthalmoplegia – No other neurological problems– Elevated levels of IgM GM1 Ab– Normal anti-GQ1b Ab
Anti-GQ1b IgG antibody syndrome: clinical and immunological range. K Hirata, et al, J Neurol Neurosurg Psychiatry 2001;70:50–55
– 194 patients with anti-GQ1b IgG– 94% had antecedent illnesses
84% upper respiratory tract infection10% diarrhoea
– As initial symptoms67% diplopia29% gait disturbance
• The case described might be labelled “ophthalmoparesis due to presumed microvascular causes” or “presumed breakdown of latent squint” as no other explainable causes have been found.
• Measurement of anti-ganglioside antibodies should be considered in cases of otherwise unexplained ophthalmoplegia.