A Case of Sjogren’s Syndrome with Sensory Ganglionopathy

Maliha Hakim; Mohammad Nur Uddin; FM Monjur Hasan; Mim Tanzila Mamun; Alif Al Mamun; Mashfiqul Hasan

doi:doi:10.11648/j.cnn.20220604.12

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A Case of Sjogren’s Syndrome with Sensory Ganglionopathy

Maliha Hakim, Mohammad Nur Uddin, FM Monjur Hasan, Mim Tanzila Mamun, Alif Al Mamun, Mashfiqul Hasan

Published in Clinical Neurology and Neuroscience (Volume 6, Issue 4)

Received: 29 October 2022 Accepted: 5 December 2022 Published: 15 December 2022

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Abstract

Sensory ganglionopathy may associate with Sjögren’s syndrome (SS) and its clinical presentation is dominated by symptoms of damage to the type 1a large sensory fibers. Here we describe a case of SS with sensory ganglionopathy. A 27-year-old lady presented with imbalance and tingling-numbness of all limbs for 4 months. Imbalance aggravated during standing and walking, and was more marked in the dark. Tingling and numbness were asymmetrical, more marked in the upper limbs. She also complained of dry mouth and feeling of stickiness during swallowing for 9 months along with significant weight loss (about 25-kg over 6 months). Examination revealed hypotonia and areflexia, but muscle power was normal. There was an athetoid movement of the upper limbs, more marked after closing eyes. All modalities of sensation were impaired in all limbs. Romberg’s sign was positive and she had an unstable gait. Investigation revealed positive ANA, positive RNP, and positive SS-A. Shirmer’s test and saliva flow test was positive. Histopathological examination of labial mucosa revealed a Focus score of >1. Nerve conduction study (NCS) revealed absent sensory nerve action potentials (SNAP) both upper and lower limb nerves. Magnetic resonance imaging (MRI) of the spine revealed mild thickening of dorsal root ganglia and corresponding nerve roots. A diagnosis of primary Sjogren’s syndrome with ganglionopathy was made and the patient was started on corticosteroids and azathioprine. Neurologists should be aware of possible autoimmune origins of neuropathies and neuronopathies, as these are potentially treatable condition.

Published in	Clinical Neurology and Neuroscience (Volume 6, Issue 4)
DOI	10.11648/j.cnn.20220604.12
Page(s)	62-65
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2022. Published by Science Publishing Group

Keywords

Sjögren’s Syndrome, Ganglionopathy, Imbalance

References

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[2]	Brito-Zeron P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, Sivils K, Theander E, Tzioufas A, Ramos-Casals M. Sjögren syndrome. Nature reviews Disease primers. 2016 Jul 7; 2 (1): 1-20.
[3]	Chai J, Logigian EL. Neurological manifestations of primary Sjogren's syndrome. Curr Opin Neurol. 2010; 23 (5): 509-513. doi: 10.1097/WCO.0b013e32833de6ab.
[4]	Pereira PR, Viala K, Maisonobe T, et al. Sjögren Sensory Neuronopathy (Sjögren Ganglionopathy): Long-Term Outcome and Treatment Response in a Series of 13 Cases. Medicine (Baltimore). 2016; 95 (19): e3632. doi: 10.1097/MD.0000000000003632.
[5]	Seeliger T, Prenzler NK, Gingele S, Seeliger B, Körner S, Thiele T, Bönig L, Sühs KW, Witte T, Stangel M, Skripuletz T. Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome. Front Immunol. 2019 Jul 11; 10: 1600. Amato AA, Ropper AH. Sensory Ganglionopathy. N Engl J Med. 2020; 383 (17): 1657-1662. doi: 10.1056/NEJMra2023935.
[6]	Jaskólska M, Chylińska M, Masiak A, Siemiński M, Ziętkiewicz M, Czuszyńska Z, Smoleńska Ż, Zdrojewski Z. Neuro Sjögren: Uncommon or underestimated problem?. Brain and Behavior. 2020 Aug; 10 (8): e01665. Perzyńska-Mazan J, Maślińska M, Gasik R. Neurological manifestations of primary Sjögren's syndrome. Reumatologia. 2018; 56 (2): 99-105. doi: 10.5114/reum.2018.75521.
[7]	Chen YM, Su KY. Acute motor and sensory axonal neuropathy in association with primary Sjögren's syndrome: a case report. BMC Neurol. 2021 Apr 15; 21 (1): 161.
[8]	Amato AA, Ropper AH. Sensory Ganglionopathy. N Engl J Med. 2020; 383 (17): 1657-1662. doi: 10.1056/NEJMra2023935.
[9]	Perzyńska-Mazan J, Maślińska M, Gasik R. Neurological manifestations of primary Sjögren’s syndrome. Reumatologia. 2018; 56 (2): 99-105. Doi: 10.5114/reum.2018.75521. Epub 2018 May 9. PMID: 29853725; PMCID: PMC5974632.
[10]	Birnbaum J, Lalji A, Piccione EA, Izbudak I. Magnetic resonance imaging of the spinal cord in the evaluation of 3 patients with sensory neuronopathies: diagnostic assessment, indications of treatment response, and impact of autoimmunity: a case report. Medicine (Baltimore) 2017; 96 (49): e8483-e8483.
[11]	Provitera V, Gibbons CH, Wendelschafer-Crabb G, et al. The role of skin biopsy in differentiating small-fiber neuropathy from ganglionopathy. Eur J Neurol 2018; 25: 848-853.
[12]	Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 2017; 69 (1): 35-45. Doi: 10.1002/art.39859
[13]	Parisis D, Chivasso C, Perret J, Soyfoo MS, Delporte C. Current State of Knowledge on Primary Sjögren's Syndrome, an Autoimmune Exocrinopathy. J Clin Med. 2020; 9 (7): 2299. doi: 10.3390/jcm9072299. PMID: 32698400; PMCID: PMC7408693.
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APA Style

Maliha Hakim, Mohammad Nur Uddin, FM Monjur Hasan, Mim Tanzila Mamun, Alif Al Mamun, et al. (2022). A Case of Sjogren’s Syndrome with Sensory Ganglionopathy. Clinical Neurology and Neuroscience, 6(4), 62-65. https://doi.org/10.11648/j.cnn.20220604.12

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ACS Style

Maliha Hakim; Mohammad Nur Uddin; FM Monjur Hasan; Mim Tanzila Mamun; Alif Al Mamun, et al. A Case of Sjogren’s Syndrome with Sensory Ganglionopathy. Clin. Neurol. Neurosci. 2022, 6(4), 62-65. doi: 10.11648/j.cnn.20220604.12

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AMA Style

Maliha Hakim, Mohammad Nur Uddin, FM Monjur Hasan, Mim Tanzila Mamun, Alif Al Mamun, et al. A Case of Sjogren’s Syndrome with Sensory Ganglionopathy. Clin Neurol Neurosci. 2022;6(4):62-65. doi: 10.11648/j.cnn.20220604.12

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@article{10.11648/j.cnn.20220604.12,
  author = {Maliha Hakim and Mohammad Nur Uddin and FM Monjur Hasan and Mim Tanzila Mamun and Alif Al Mamun and Mashfiqul Hasan},
  title = {A Case of Sjogren’s Syndrome with Sensory Ganglionopathy},
  journal = {Clinical Neurology and Neuroscience},
  volume = {6},
  number = {4},
  pages = {62-65},
  doi = {10.11648/j.cnn.20220604.12},
  url = {https://doi.org/10.11648/j.cnn.20220604.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.cnn.20220604.12},
  abstract = {Sensory ganglionopathy may associate with Sjögren’s syndrome (SS) and its clinical presentation is dominated by symptoms of damage to the type 1a large sensory fibers. Here we describe a case of SS with sensory ganglionopathy. A 27-year-old lady presented with imbalance and tingling-numbness of all limbs for 4 months. Imbalance aggravated during standing and walking, and was more marked in the dark. Tingling and numbness were asymmetrical, more marked in the upper limbs. She also complained of dry mouth and feeling of stickiness during swallowing for 9 months along with significant weight loss (about 25-kg over 6 months). Examination revealed hypotonia and areflexia, but muscle power was normal. There was an athetoid movement of the upper limbs, more marked after closing eyes. All modalities of sensation were impaired in all limbs. Romberg’s sign was positive and she had an unstable gait. Investigation revealed positive ANA, positive RNP, and positive SS-A. Shirmer’s test and saliva flow test was positive. Histopathological examination of labial mucosa revealed a Focus score of >1. Nerve conduction study (NCS) revealed absent sensory nerve action potentials (SNAP) both upper and lower limb nerves. Magnetic resonance imaging (MRI) of the spine revealed mild thickening of dorsal root ganglia and corresponding nerve roots. A diagnosis of primary Sjogren’s syndrome with ganglionopathy was made and the patient was started on corticosteroids and azathioprine. Neurologists should be aware of possible autoimmune origins of neuropathies and neuronopathies, as these are potentially treatable condition.},
 year = {2022}
}

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TY - JOUR
T1 - A Case of Sjogren’s Syndrome with Sensory Ganglionopathy
AU - Maliha Hakim
AU - Mohammad Nur Uddin
AU - FM Monjur Hasan
AU - Mim Tanzila Mamun
AU - Alif Al Mamun
AU - Mashfiqul Hasan
Y1 - 2022/12/15
PY - 2022
N1 - https://doi.org/10.11648/j.cnn.20220604.12
DO - 10.11648/j.cnn.20220604.12
T2 - Clinical Neurology and Neuroscience
JF - Clinical Neurology and Neuroscience
JO - Clinical Neurology and Neuroscience
SP - 62
EP - 65
PB - Science Publishing Group
SN - 2578-8930
UR - https://doi.org/10.11648/j.cnn.20220604.12
AB - Sensory ganglionopathy may associate with Sjögren’s syndrome (SS) and its clinical presentation is dominated by symptoms of damage to the type 1a large sensory fibers. Here we describe a case of SS with sensory ganglionopathy. A 27-year-old lady presented with imbalance and tingling-numbness of all limbs for 4 months. Imbalance aggravated during standing and walking, and was more marked in the dark. Tingling and numbness were asymmetrical, more marked in the upper limbs. She also complained of dry mouth and feeling of stickiness during swallowing for 9 months along with significant weight loss (about 25-kg over 6 months). Examination revealed hypotonia and areflexia, but muscle power was normal. There was an athetoid movement of the upper limbs, more marked after closing eyes. All modalities of sensation were impaired in all limbs. Romberg’s sign was positive and she had an unstable gait. Investigation revealed positive ANA, positive RNP, and positive SS-A. Shirmer’s test and saliva flow test was positive. Histopathological examination of labial mucosa revealed a Focus score of >1. Nerve conduction study (NCS) revealed absent sensory nerve action potentials (SNAP) both upper and lower limb nerves. Magnetic resonance imaging (MRI) of the spine revealed mild thickening of dorsal root ganglia and corresponding nerve roots. A diagnosis of primary Sjogren’s syndrome with ganglionopathy was made and the patient was started on corticosteroids and azathioprine. Neurologists should be aware of possible autoimmune origins of neuropathies and neuronopathies, as these are potentially treatable condition.
VL - 6
IS - 4
ER -

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Author Information

Maliha Hakim

Department of Neurology, National Institute of Neurosciences & Hospital, Dhaka, Bangladesh
Mohammad Nur Uddin

Department of Neurology, National Institute of Neurosciences & Hospital, Dhaka, Bangladesh
FM Monjur Hasan

Department of Neurology, National Institute of Neurosciences & Hospital, Dhaka, Bangladesh
Mim Tanzila Mamun

Army Medical College, University of Chittagong, Chattogram, Bangladesh
Alif Al Mamun

Faculty of Science and Engineering, Macquarie University, Sydney, Australia
Mashfiqul Hasan

Neuroendocrine and Diabetes Team, National Institute of Neurosciences & Hospital, Dhaka, Bangladesh

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APA Style

Maliha Hakim, Mohammad Nur Uddin, FM Monjur Hasan, Mim Tanzila Mamun, Alif Al Mamun, et al. (2022). A Case of Sjogren’s Syndrome with Sensory Ganglionopathy. Clinical Neurology and Neuroscience, 6(4), 62-65. https://doi.org/10.11648/j.cnn.20220604.12

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ACS Style

Maliha Hakim; Mohammad Nur Uddin; FM Monjur Hasan; Mim Tanzila Mamun; Alif Al Mamun, et al. A Case of Sjogren’s Syndrome with Sensory Ganglionopathy. Clin. Neurol. Neurosci. 2022, 6(4), 62-65. doi: 10.11648/j.cnn.20220604.12

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AMA Style

Maliha Hakim, Mohammad Nur Uddin, FM Monjur Hasan, Mim Tanzila Mamun, Alif Al Mamun, et al. A Case of Sjogren’s Syndrome with Sensory Ganglionopathy. Clin Neurol Neurosci. 2022;6(4):62-65. doi: 10.11648/j.cnn.20220604.12

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@article{10.11648/j.cnn.20220604.12,
  author = {Maliha Hakim and Mohammad Nur Uddin and FM Monjur Hasan and Mim Tanzila Mamun and Alif Al Mamun and Mashfiqul Hasan},
  title = {A Case of Sjogren’s Syndrome with Sensory Ganglionopathy},
  journal = {Clinical Neurology and Neuroscience},
  volume = {6},
  number = {4},
  pages = {62-65},
  doi = {10.11648/j.cnn.20220604.12},
  url = {https://doi.org/10.11648/j.cnn.20220604.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.cnn.20220604.12},
  abstract = {Sensory ganglionopathy may associate with Sjögren’s syndrome (SS) and its clinical presentation is dominated by symptoms of damage to the type 1a large sensory fibers. Here we describe a case of SS with sensory ganglionopathy. A 27-year-old lady presented with imbalance and tingling-numbness of all limbs for 4 months. Imbalance aggravated during standing and walking, and was more marked in the dark. Tingling and numbness were asymmetrical, more marked in the upper limbs. She also complained of dry mouth and feeling of stickiness during swallowing for 9 months along with significant weight loss (about 25-kg over 6 months). Examination revealed hypotonia and areflexia, but muscle power was normal. There was an athetoid movement of the upper limbs, more marked after closing eyes. All modalities of sensation were impaired in all limbs. Romberg’s sign was positive and she had an unstable gait. Investigation revealed positive ANA, positive RNP, and positive SS-A. Shirmer’s test and saliva flow test was positive. Histopathological examination of labial mucosa revealed a Focus score of >1. Nerve conduction study (NCS) revealed absent sensory nerve action potentials (SNAP) both upper and lower limb nerves. Magnetic resonance imaging (MRI) of the spine revealed mild thickening of dorsal root ganglia and corresponding nerve roots. A diagnosis of primary Sjogren’s syndrome with ganglionopathy was made and the patient was started on corticosteroids and azathioprine. Neurologists should be aware of possible autoimmune origins of neuropathies and neuronopathies, as these are potentially treatable condition.},
 year = {2022}
}

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