I thought for my journal club entry this month a would share these articles that were relevant to the case I presented at the symposium in Auckland in December, as not everyone was there and most people like me hadn’t heard of this syndrome before.

Our neuro-ophthalmologist who reviewed the patient (the day before I presented her!) thought this represented her most likely diagnosis although it is still not confirmed.

A brief synopsis of the case

71 female presenting for surgery for routine decompensating exophoria on background of long standing intermittent peripheral sensory neuropathy, AF, hypertension, thrombocytopenia and lymphocytosis (these were considered not particularly relevant at the time)

Had routine surgery of bilateral LR recessions with great initial result.

Sudden onset of recurrence of XT with diplopia and limitation of elevation and adduction approx. 4/52 post-op

??possible partial 3rdN/haematoma due to platelet issue, improved with steroids, MRI brain didn’t show anything

Motility stabilised but very symptomatic with diplopia so proceeded to further surgery addressing XT and R/L

Again good initial result but again regressed about 4/52 PO – connection was made to this timeline coincided with stopping her steroid cover due to her platelet issue.

Unfortunately things haven’t improved and she has been left with significant XT with diplopia and limitation of OM

She did get a temporary improvement while on steroids but this regressed when steroids weaned off.

We sought an opinion from our Neuro-ophthalmologist to help with a diagnosis/ any treatment.

She had tested negative for MG

Her thoughts-

History of peripheral neuropathy with UL weakness with ophthalmoplegia – likely neurological aetiology for ophthalmic presentation

-variability of exo

– atypical EOM limitation

-regression/relapse post EOM surgery = possible chronic peripheral demyelinating condition

– EOM responsiveness to oral steroid is a possible clue

She felt CANOMAD was a high possibility given clinical presentation and some of her lab results -due with anti-glycolipid antibodie

So when surgery regresses or new EOM limitations present in settings of other neurological symptom – you must think of a neurological cause for the strabismus

CANOMAD is a rare syndrome that belongs to the group of peripheral neuropathies associated with IgM monoclonal gammopathy – clinical features include chronic sensory ataxic neuropathy, ophthalmoplegia,  M-protein with cold-agglutinin activity and anti-disialosyl antibodies

Yet another condition to consider for our differential diagnosis list!!!!!!

Happy reading

Jo