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. 2015 May 4;39(2):59–63. doi: 10.3109/01658107.2014.991832

TABLE 1. Summary of patients with cerebellar dysfunction presenting with binocular diplopia from esotropia.

Patient Sex Genetic test results Family history Age of diplopia onset Age of ataxia onset Age first examined by us Age at last review Lateral rectus underaction Esodeviation worse to Near(N) or Distance(D) Vertical deviation present Cerebellar eye signs Non-ocular cerebellar signs Brain maging* Diagnosis Treatment
1 F NEG Y 36 39 37 46 L, then B N = D, then D > N Y Broken pursuit, GEN Y Normal ADCA Prisms & surgery
2 M NEG Y 63 NA 70 71 B D > N Y Broken pursuit, GEN N Small vessel disease ADCA Prisms
3 M NEG N 73 74 74 76 B D > N N Broken pursuit, GEN, DBN Y Generalised atrophy ICD Prisms
4 F NEG N 75 78 75 93 R, then B D > N Y Broken bursuit, hypometric saccade, DBN Y Normal ICD Prisms
5 F NEG N 45 75 75 78 B D > N Y Broken bursuit, hypometric saccade, GEN Y Cerebellar atrophy ICD Prisms & botox
6 M ND N 66 NA 69 75 B D > N N Broken pursuit, GEN N Small vessel disease ICD Prisms
7 F SCA expansion Y 31 31 38 42 L, then B D > N Y Broken pursuit, GEN, hypometric saccade Y Cerebellar atrophy ADCA Prisms

NA = not applicable; ND = not done; GEN = gaze-evoked nystagmus; DBN = down-beat nystagmus; NEG = negative for SCA 1, 2, 3, 6, 7; L = left; R = right; B = both; ADCA = autosomal dominant cerebellar ataxia; ICD = idiopathic cerebellar degeneration.

Only Patient 4 was examined by us at onset, but developed ataxia 3 years and DBN 7 years later. All other patients first presented elsewhere and were first observed to have cerebellar eye signs when first examined by us.

*Magnetic resonance imaging was undertaken except for Patient 4 who had computed tomography due to claustrophobia.