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Table 2 Clinical, neuroimaging and serological characteristics of all patients

From: Cerebellar ataxia with sensory ganglionopathy; does autoimmunity have a role to play?

Gender Age at 1st presentation First symptoms Age at first symptoms First diagnosis Second symptoms Age at second diagnosis MRI Abnormal
MRS
SNAPs Gluten serology Duodenum biopsy HLA Other evidence of autoimmunity Cancer Age at cancer diagnosis Genetics Truly idiopathic
Female 48 Unsteadiness in the dark 48 SG Worsening of balance 63 Normal for age Vermis and hemisphere Absent Negative N/A DQ2 ENA+ ANA+, Crohn’s    Negative  
Female 59 Patchy numbness 57 SG No new symptoms 60 Normal for age Vermis Asymmetrical Positive CD DQ2 TPO+, dsDNA+, RF+ Ovarian 38   
Male 40 Patchy numbness 38 SG Worsening of balance 62 Normal for age Vermis Absent Positive Normal DQ2 P-ANCA+, ANA+, GM1+, Sarcoidosis     
Male 60 Patchy burning pain 59 SG No new symptoms 65 Normal for age Hemisphere Asymmetrical Positive Normal DQ2      
Female 76 Unsteadiness/Numbness 76 SG Worsening of balance 84 Normal for age Hemisphere Asymmetrical Positive CD DQ2      
Male 49 Patchy numbness 49 SG No new symptoms 72 Normal for age Hemisphere Absent Negative N/A DQ1 ANA+, RF+    Negative  
Male 52 Pain in feet 49 SG Worsening of balance 70 Normal for age Vermis Asymmetrical Negative N/A DQ1 ANA+, P-ANCA+, SACE+     
Female 55 Patchy numbness 55 SG Worsening of balance 69 Normal for age Vermis Asymmetrical Negative N/A Not done ANA+, ENA+ (Sjogren’s)     
Male 67 Unsteadiness 64 SG Worsening of balance 68 Cerebellar atrophy Vermis Absent Negative N/A DQ2 ANA+, P-ANCA+, MGUS     
Female 61 Patchy pain 61 SG Worsening of balance 67 Normal for age Hemisphere Asymmetrical Negative N/A Not done ANA+, ENA+ (Sjogren’s)     
Female 58 Patchy numbness 57 SG Worsening of balance 74 Normal for age Hemisphere Asymmetrical Negative N/A Other      Yes
Female 35 Patchy numbness 35 SG Worsening of balance 49 Normal for age Vermis and hemisphere Asymmetrical Negative N/A Not done ANA+, ENA+ (Sjogren’s)     
Female 42 Unsteadiness 42 Cerebellar ataxia Patchy sensory loss 46 Cerebellar atrophy Vermis and hemisphere Asymmetrical Negative N/A DQ1 Anti-Yo+ Ovarian 41   
Male 14 Unsteadiness 14 Cerebellar ataxia Worsening of balance 58 Cerebellar atrophy Vermis Asymmetrical Positive CD DQ2 GAD+    Mitochondrial  
Female 57 Unsteadiness/Dizziness 55 Cerebellar ataxia Sensory symptoms 69 Cerebellar atrophy Vermis Absent Positive Normal DQ1 GAD+    SCA18  
Male 52 Unsteadiness 51 Cerebellar ataxia Sensory symptoms 57 Cerebellar atrophy Vermis and hemisphere Absent Positive Normal DQ8     Negative  
Female 50 Unsteadiness/
Slurred speech
47 Cerebellar ataxia Sensory symptoms 54 Cerebellar atrophy Vermis and hemisphere Asymmetrical Positive Not done DQ2 RF+    Negative  
Male 44 Unsteadiness 39 Cerebellar ataxia No new symptoms 46 Cerebellar atrophy Vermis Asymmetrical Positive Normal DQ8     Negative  
Female 54 Unsteadiness 53 Cerebellar ataxia Sensory symptoms 60 Cerebellar atrophy Vermis and hemisphere Asymmetrica Positive Normal DQ2   Colon 69 Negative  
Male 67 Unsteadiness 54 Cerebellar ataxia No new symptoms 76 Cerebellar atrophy Vermis and hemisphere Absent Positive Normal DQ2 ANA+    Negative  
Male 66 Unsteadiness 66 Cerebellar ataxia No new symptoms 67 Cerebellar atrophy Vermis and hemisphere Absent Negative N/A DQ2 ANA+ Bowel 68   
Female 66 Unsteadiness 63 Cerebellar ataxia Sensory symptoms 68 Normal for age Vermis and hemisphere Absent Negative N/A DQ2     Negative Yes
Female 65 Unsteadiness 59 Cerebellar ataxia Patch sensory loss 68 Cerebellar atrophy Vermis Absent Negative N/A DQ8 C-ANCA+, MGUS Myltiple myeloma - light chain 70 Negative  
Female 46 Unsteadiness 46 Cerebellar ataxia Patchy sensory loss 58 Cerebellar atrophy Vermis Asymmetrical Negative N/A DQ1 TPO+    Negative  
Female 66 Unsteadiness 64 Cerebellar ataxia No new symptoms 69 Normal for age Vermis and hemisphere Asymmetrical Negative N/A DQ2     Negative Yes
Female 68 Unsteadiness 67 Cerebellar ataxia Sensory symptoms 69 Normal for age Vermis and hemisphere Absent Negative N/A DQ2 ANA+, TPO+     
Male 81 Unsteadiness 79 Cerebellar ataxia Worsening of balance 85 Normal for age Vermis and hemisphere Asymmetrical Negative N/A Not done MGUS Melanoma/
Possibly prostate
86   
Female 29 Unsteadiness 8 Cerebellar ataxia Sensory symptoms 33 Cerebellar atrophy Vermis and hemisphere Asymmetrical Positive Normal DQ2 OCB+, TPO+, GAD+ (one occasion only)    Negative  
Male 43 Unsteadiness 34 Both at the same time   43 Cerebellar atrophy Hemisphere Asymmetrical Positive Normal DQ2 RF+     
Female 61 Unsteadiness/Numbness 60 Both at the same time   61 Cerebellar atrophy Vermis Absent Positive Normal DQ1   Malignancy of unknown primary location 64 Negative  
Female 81 Unsteadiness 80 Both at the same time   81 Cerebellar atrophy Vermis Asymmetrical Positive Normal Not done      
Male 51 Unsteadiness/Numbness 51 Both at the same time   51 Cerebellar atrophy Vermis and hemisphere Asymmetrical Positive Normal DQ1      
Female 55 Unsteadiness/Pain 49 Both at the same time   55 Cerebellar atrophy Vermis Absent Negative N/A DQ1 GAD+, P-ANCA+ Uterus 39 Negative  
Male 64 Unsteadiness 54 Both at the same time   64 Cerebellar atrophy Vermis and hemisphere Absent Negative N/A DQ8 MGUS    Negative  
Male 70 Unsteadiness 67 Both at the same time   70 Normal for age Vermis Absent Negative N/A DQ1 C-ANCA+     
Male 54 Unsteadiness 49 Both at the same time   54 Cerebellar atrophy Vermis and hemisphere Asymmetrical Negative N/A DQ2 TPO+     
Female 70 Unsteadiness 69 Both at the same time   70 Normal for age Vermis and hemisphere Asymmetrical Negative N/A Not done ANA+, ENA+ (Sjogren’s), RF+ Bowel 71   
Male 57 Unsteadiness 55 Both at the same time   57 Cerebellar atrophy Vermis Absent Positive Normal DQ2     Negative  
Male 61 Unsteadiness/Numbness 61 Both at the same time   62 Normal for age Vermis and hemisphere Asymmetrical Positive Normal DQ8 ENA+     
Female 69 Unsteadiness 64 Both at the same time   69 Cerebellar atrophy Vermis and hemisphere Asymmetrical Positive Normal DQ8 P-ANCA+