Marios Hadjivassiliou1, Graeme Wild2, Priya Shanmugarajah3, Richard A Grünewald3, Mohammed Akil4. 1. Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Trust and University of Sheffield, Royal Hallamshire Hospital, Glossop Road, Sheffield, S10 2JF, UK. m.hadjivassiliou@sheffield.ac.uk. 2. Clinical Immunology and Allergy Unit, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK. 3. Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Trust and University of Sheffield, Royal Hallamshire Hospital, Glossop Road, Sheffield, S10 2JF, UK. 4. Rheumatology Department, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK.
Abstract
BACKGROUND AND PURPOSE: Immune mediated cerebellar ataxias account for a substantial proportion of all progressive ataxias. A diagnostic serological test is not always available. This is particularly problematic in Primary Autoimmune Cerebellar Ataxia, hence the necessity for diagnostic criteria recently devised and published by an International Task Force. We present our experience in the use of a commercially available indirect immunofluorescence assay, intended to be used for the detection of antibodies associated with paraneoplastic neurological syndromes. METHODS: Retrospective review of patients with ataxia who underwent serological testing using this assay as part of their diagnostic evaluation. We were interested in 3 groups: suspected immune mediated ataxias, genetically confirmed ataxias and patients with cerebellar variant of multi-system atrophy (MSA-C). The indirect immunofluorescence assay was performed using commercially available monkey cerebellum slides and anti-human IgG FITC conjugated antiserum. RESULTS: A total of 300 patients that had this test and fitted into one of these 3 groups (immune ataxias 190, genetic ataxias 60, MSA-C 50) were identified. The prevalence of positive immunofluorescence but negative immunoblot was 172/190 (91%) in the suspected immune ataxia group, 3/60 (5%) in the genetic group and 2/50 (4%) in the MSA-C group. The difference between the first and the other groups was significant χ2 (1, N = 291) = 64.2, p < 00001. CONCLUSIONS: This report demonstrates that a commercially available immunofluorescence assay can be used to provide additional diagnostic aid for suspected immune mediated ataxias and in particular Primary Autoimmune Cerebellar Ataxia where no diagnostic marker exists.
BACKGROUND AND PURPOSE: Immune mediated cerebellar ataxias account for a substantial proportion of all progressive ataxias. A diagnostic serological test is not always available. This is particularly problematic in Primary Autoimmune Cerebellar Ataxia, hence the necessity for diagnostic criteria recently devised and published by an International Task Force. We present our experience in the use of a commercially available indirect immunofluorescence assay, intended to be used for the detection of antibodies associated with paraneoplastic neurological syndromes. METHODS: Retrospective review of patients with ataxia who underwent serological testing using this assay as part of their diagnostic evaluation. We were interested in 3 groups: suspected immune mediated ataxias, genetically confirmed ataxias and patients with cerebellar variant of multi-system atrophy (MSA-C). The indirect immunofluorescence assay was performed using commercially available monkey cerebellum slides and anti-human IgG FITC conjugated antiserum. RESULTS: A total of 300 patients that had this test and fitted into one of these 3 groups (immune ataxias 190, genetic ataxias 60, MSA-C 50) were identified. The prevalence of positive immunofluorescence but negative immunoblot was 172/190 (91%) in the suspected immune ataxia group, 3/60 (5%) in the genetic group and 2/50 (4%) in the MSA-C group. The difference between the first and the other groups was significant χ2 (1, N = 291) = 64.2, p < 00001. CONCLUSIONS: This report demonstrates that a commercially available immunofluorescence assay can be used to provide additional diagnostic aid for suspected immune mediated ataxias and in particular Primary Autoimmune Cerebellar Ataxia where no diagnostic marker exists.
Authors: M Hadjivassiliou; J Martindale; P Shanmugarajah; R A Grünewald; P G Sarrigiannis; N Beauchamp; K Garrard; R Warburton; D S Sanders; D Friend; S Duty; J Taylor; N Hoggard Journal: J Neurol Neurosurg Psychiatry Date: 2016-12-13 Impact factor: 10.154
Authors: M Hadjivassiliou; R A Grunewald; P D Shanmugarajah; P G Sarrigiannis; P Zis; V Skarlatou; N Hoggard Journal: Cerebellum Date: 2020-10 Impact factor: 3.847