Stiff Person Syndrome and Gluten Sensitivity
Stiff person syndrome (SPS) is a rare autoimmune disease characterised by axial stiffness and episodic painful spasms. It is associated with additional autoimmune diseases and cerebellar ataxia. Most patients with SPS have high levels of glutamic acid decarboxylase (GAD) antibodies. The aetiology of...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-04-01
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| Series: | Nutrients |
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| Online Access: | https://www.mdpi.com/2072-6643/13/4/1373 |
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| author | Marios Hadjivassiliou Panagiotis Zis David S. Sanders Nigel Hoggard Ptolemaios G. Sarrigiannis |
| author_facet | Marios Hadjivassiliou Panagiotis Zis David S. Sanders Nigel Hoggard Ptolemaios G. Sarrigiannis |
| author_sort | Marios Hadjivassiliou |
| collection | DOAJ |
| description | Stiff person syndrome (SPS) is a rare autoimmune disease characterised by axial stiffness and episodic painful spasms. It is associated with additional autoimmune diseases and cerebellar ataxia. Most patients with SPS have high levels of glutamic acid decarboxylase (GAD) antibodies. The aetiology of SPS remains unclear but autoimmunity is thought to play a major part. We have previously demonstrated overlap between anti-GAD ataxia and gluten sensitivity. We have also demonstrated the beneficial effect of a gluten-free diet (GFD) in patients with anti-GAD ataxia. Here, we describe our experience in the management of 20 patients with SPS. The mean age at symptom onset was 52 years. Additional autoimmune diseases were seen in 15/20. Nineteen of the 20 patients had serological evidence of gluten sensitivity and 6 had coeliac disease. Fourteen of the 15 patients who had brain imaging had evidence of cerebellar involvement. Twelve patients improved on GFD and in seven GFD alone was the only treatment required long term. Twelve patients had immunosuppression but only three remained on such medication. Gluten sensitivity plays an important part in the pathogenesis of SPS and GFD is an effective therapeutic intervention. |
| first_indexed | 2024-03-10T12:11:03Z |
| format | Article |
| id | doaj.art-e41f52b582244c228960046e68b14fb0 |
| institution | Directory Open Access Journal |
| issn | 2072-6643 |
| language | English |
| last_indexed | 2024-03-10T12:11:03Z |
| publishDate | 2021-04-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Nutrients |
| spelling | doaj.art-e41f52b582244c228960046e68b14fb02023-11-21T16:16:04ZengMDPI AGNutrients2072-66432021-04-01134137310.3390/nu13041373Stiff Person Syndrome and Gluten SensitivityMarios Hadjivassiliou0Panagiotis Zis1David S. Sanders2Nigel Hoggard3Ptolemaios G. Sarrigiannis4Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Trust, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UKAcademic Department of Neurosciences, Sheffield Teaching Hospitals NHS Trust, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UKAcademic Department of Gastroenterology, Sheffield Teaching Hospitals NHS Trust, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UKDepartment of Infection, Immunity & Cardiovascular Disease, University of Sheffield, Sheffield S10 2JF, UKAcademic Department of Neurosciences, Sheffield Teaching Hospitals NHS Trust, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UKStiff person syndrome (SPS) is a rare autoimmune disease characterised by axial stiffness and episodic painful spasms. It is associated with additional autoimmune diseases and cerebellar ataxia. Most patients with SPS have high levels of glutamic acid decarboxylase (GAD) antibodies. The aetiology of SPS remains unclear but autoimmunity is thought to play a major part. We have previously demonstrated overlap between anti-GAD ataxia and gluten sensitivity. We have also demonstrated the beneficial effect of a gluten-free diet (GFD) in patients with anti-GAD ataxia. Here, we describe our experience in the management of 20 patients with SPS. The mean age at symptom onset was 52 years. Additional autoimmune diseases were seen in 15/20. Nineteen of the 20 patients had serological evidence of gluten sensitivity and 6 had coeliac disease. Fourteen of the 15 patients who had brain imaging had evidence of cerebellar involvement. Twelve patients improved on GFD and in seven GFD alone was the only treatment required long term. Twelve patients had immunosuppression but only three remained on such medication. Gluten sensitivity plays an important part in the pathogenesis of SPS and GFD is an effective therapeutic intervention.https://www.mdpi.com/2072-6643/13/4/1373stiff person syndromeanti-GAD antibodiesgluten sensitivitycoeliac diseasecerebellar ataxiagluten free diet |
| spellingShingle | Marios Hadjivassiliou Panagiotis Zis David S. Sanders Nigel Hoggard Ptolemaios G. Sarrigiannis Stiff Person Syndrome and Gluten Sensitivity Nutrients stiff person syndrome anti-GAD antibodies gluten sensitivity coeliac disease cerebellar ataxia gluten free diet |
| title | Stiff Person Syndrome and Gluten Sensitivity |
| title_full | Stiff Person Syndrome and Gluten Sensitivity |
| title_fullStr | Stiff Person Syndrome and Gluten Sensitivity |
| title_full_unstemmed | Stiff Person Syndrome and Gluten Sensitivity |
| title_short | Stiff Person Syndrome and Gluten Sensitivity |
| title_sort | stiff person syndrome and gluten sensitivity |
| topic | stiff person syndrome anti-GAD antibodies gluten sensitivity coeliac disease cerebellar ataxia gluten free diet |
| url | https://www.mdpi.com/2072-6643/13/4/1373 |
| work_keys_str_mv | AT marioshadjivassiliou stiffpersonsyndromeandglutensensitivity AT panagiotiszis stiffpersonsyndromeandglutensensitivity AT davidssanders stiffpersonsyndromeandglutensensitivity AT nigelhoggard stiffpersonsyndromeandglutensensitivity AT ptolemaiosgsarrigiannis stiffpersonsyndromeandglutensensitivity |