1 / 23

Anti-ganglioside antibodies

Anti-ganglioside antibodies. The cause of otherwise unexplained ophthalmoplegias? Michelle Gajus and Lionel Kowal. Antiganglioside antibodies are responsible for some autoimmune diseases. Antiganglioside antibody. ANTI Gq1b. Acute conditions Miller-Fisher Syndrome Acute ophthalmoparesis

alyssa
Download Presentation

Anti-ganglioside antibodies

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Anti-ganglioside antibodies The cause of otherwise unexplained ophthalmoplegias? Michelle Gajus and Lionel Kowal

  2. Antiganglioside antibodies are responsible for some autoimmune diseases Antiganglioside antibody

  3. ANTI Gq1b • Acute conditions • Miller-Fisher Syndrome • Acute ophthalmoparesis (ophthalmoplegia without ataxia) • Chronic conditions • Otherwise unexplained ophthalmoplegia Chronic ophthalmoplegia with anti-GQ1b antibody. Reddel et alii Neurology. 54(4):1000-2, Feb 2000 & some Japanese papers

  4. ANTI GM-1 • Guillain-Barre syndrome • Multifocal motor neuropathy • Distal lower motor neuron syndrome • McCombe: Asymmetric patchy muscle weakness

  5. Five cases of anti GM-1 [normal anti-GQ1b] in ophthalmoplegia of obscure cause

  6. EG - 13 y female • ‘Acute’ ET • Intermittent at first • No strabismogenic factors present • 50^  70^ ET in one month • BMR + botox : good result

  7. SN - 31 y female • Ptosis surgery as a child • 3 years of intermittent LXT • Other examination normal

  8. SN - 31 y female • LMR Rs 10 mm to slightly restrict abduction, • LLR Rc to LIO insertion • Cosmesis OK; no diplopia

  9. JL - 50 y male • Previously well; No HT, DM • Sudden onset R vertical diplopia • Impaired depression RE; excyclo RE • ‘ R SOP of indeterminate cause ‘ • No treatment • Symptoms completely resolved over 5 mo

  10. LK - 58 male • No HT, DM • ‘Shimmering’ visual disturbance followed by diplopia • H&V diplopia esp. down left gaze • Exotropia on L gaze • Provisional diagnosis R CN IV • All resolved in 2 months

  11. JP - 67 female • Past history of well-controlled hypertension • 4 months of intermittent diplopia on L gaze • With glasses 6^ ET, with CL 1^ • Orthophoric in primary position • 1^ fusional divergence, 26^ of fusional convergence • Normal ocular rotation,V1, pupillary reaction, MRI • No abduction deficit • Diagnosis: subtle L VI with no localising signs • No treatment

  12. Investigation Results

  13. Investigation Results

  14. Nomenclature • ‘Ganglioside’: lipids from ganglion cells of brain (Klenk ‘42) • Oligoglycosylceramides with sialic acid residues joined to the monosaccharide units. • Short-hand nomenclature system: M, D, T, Q = mono-, di- ,tri- & quatro- sialog-sides hence GQ, GM • 1, 2, 3, etc refer to the order of migration of the g-sides on thin-layer chromatography. Order of migration of monos/g-sides is GM3 > GM2 > GM1 • To indicate variations within the basic structures, further subscripts are added, e.g. GM1a, GD1b.

  15. Distribution of anti-GQ1b • GQ1b g-sides are clustered in paranodal region of human ocular motor nerves • Paranodal region: • - important for nerve conduction • - not protected by blood brain barrier or by connective tissue sheaths of the peripheral nerves. • GQ1b Ab’s can easily target these vulnerable sites.

  16. Distribution of GQ1b Other cranial and peripheral nerves

  17. Anti-GM1 • III, IV,VI : more GQ1b g-sides cf other cranial nn •  propensity for ophthalmoplegia with GQ1b Ab’s. • Concentration of GM1 g-sides in ocular motor nerves no higher than other cranial nn. •  mechanismof action of Ab’s against GM1 less clear than for GQ1b

  18. Anti-GM1 • In about 20 consecutive cases of acquired ophthalmoplegia of obscure cause: 5 positive cases for anti-GM1. • Our finding suggests that they may play a part in some otherwise unexplained ophthalmoplegia. • Pathogenesis yet to be elucidated.

More Related