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Chuah SL, Jobli AT, Wan SA, Teh CL. Cerebellar degeneration in primary Sjögren syndrome: a case report. J Med Case Rep 2021; 15:526. [PMID: 34663471 PMCID: PMC8524931 DOI: 10.1186/s13256-021-03103-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2019] [Accepted: 09/09/2021] [Indexed: 11/23/2022] Open
Abstract
Background Cerebellar degeneration is a rare and severe presentation of primary Sjögren syndrome. There are few case reports of cerebellar degeneration associated with different autoimmune diseases, especially with systemic lupus erythematosus and neuro-Behcet’s disease. There are only six patients reported worldwide to be affected by cerebellar atrophy associated with primary Sjögren syndrome. In this report, we describe a patient with primary Sjögren syndrome who presented with ataxia due to cerebellar degeneration. Case presentation We report the case of a 37-year-old Chinese woman with primary Sjögren syndrome who presented with ataxia over 3 months associated with tremor of the limbs. Magnetic resonance imaging of the brain revealed bilateral cerebellar atrophy. Based on the presence of cerebellar signs with magnetic resonance imaging brain findings, she was diagnosed as cerebellar degeneration secondary to primary Sjögren syndrome. She was treated with methylprednisolone, hydroxychloroquine, and two cycles of monthly intravenous cyclophosphamide. Subsequently, she refused further treatment, and her neurological symptoms remained the same upon the last clinic review. Primary cerebellar degeneration is rarely associated with primary Sjögren syndrome. The pathogenesis of the neurological manifestations in primary Sjögren syndrome is unclear. Treatment involves corticosteroids and immunosuppressive agents with no consensus of a specific therapy for the management of primary Sjögren syndrome with central nervous system involvement. Conclusions Cerebellar degeneration is a rare presentation of primary Sjögren syndrome. Early diagnosis and treatment of this condition is needed to ensure a good outcome.
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Affiliation(s)
- Seow Lin Chuah
- Rheumatology Unit, Medical Department, Sarawak General Hospital, Kuching, Malaysia
| | - Ahmad Tirmizi Jobli
- Radiology Department, Faculty of Medicine and Health Sciences, University Malaysia Sarawak, Kota Samarahan, Malaysia.
| | - Sharifah Aishah Wan
- Rheumatology Unit, Medical Department, Sarawak General Hospital, Kuching, Malaysia
| | - Cheng Lay Teh
- Rheumatology Unit, Medical Department, Sarawak General Hospital, Kuching, Malaysia
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Immunosuppressive treatment for peripheral neuropathies in Sjogren's syndrome - a systematic review. ACTA ACUST UNITED AC 2020; 58:5-12. [PMID: 31527298 DOI: 10.2478/rjim-2019-0022] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2019] [Indexed: 12/26/2022]
Abstract
BACKGROUND Sjogren's syndrome (SS) is among the most frequent autoimmune diseases and one of its most severe extraglandular manifestations is peripheral neuropathy. There is no consensus about peripheral neuropathy treatment in SS. Our aim is to identify studies proving the efficiency of immunosuppressive treatment on peripheral neuropathies in SS. METHODS The search was conducted on the PubMed (MEDLINE) database. Studies with patients diagnosed with SS and peripheral neuropathy were included. Treatment with one of the following was among inclusion criteria: glucocorticoids (GC), rituximab (RTX), azathioprine (AZA), mycophenolic acid (MMF), cyclophosphamide (CP), methotrexate (MTX), plasmapheresis or iv immunoglobulins (IV IG). RESULTS A total of 116 results were found and abstracts were examined. 103 papers were excluded, and the remaining 13 papers were analyzed. They were 3 case series and 10 case reports, retrospective, totalizing 62 patients of which 22 (35.5%) received IV IG, 8 (13%) received RTX, 7 (11%) CP, and 5 (8%) received only GC. Drug associations containing corticosteroids were frequent. Of those 22 treated with IV IG, 18 patients improved (82%), and 4 stabilized (18%). IV IG was useful in sensory, motor and sensorimotor neuropathies. CP had good results in mononeuritis multiplex, while autonomic neuropathies responded well to GC or RTX. AZA, RTX, MTX, MMF or plasmapheresis were not used alone. Follow-up periods were heterogenous and the evaluation of the neuropathy was not systematic. CONCLUSION There is only low level evidence (retrospective case reports and case series). In most cases, IV IG treatment in patients with peripheral neuropathies and SS resulted in clinical improvement, while other therapies, such as RTX, corticosteroids and CP proved to be useful in a handful of cases.
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Heidary M, Alesaeidi S, Afshari K. Cerebellar degeneration in primary Sjӧgren syndrome. BMJ Case Rep 2018; 2018:bcr-2017-223952. [PMID: 29880622 DOI: 10.1136/bcr-2017-223952] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
Neurological manifestations are reported as a consequence of primary Sjӧgren syndrome (PSS). Any part of the brain and peripheral nervous system can be involved in PSS. However, cerebellar degeneration and atrophy associated with PSS have been rarely reported. Our report describes a 22-year-old woman who presented with cerebellar ataxia, arthritis and arthralgia. Evaluation of her symptoms, autoantibodies and salivary gland pathology was in favour of the diagnosis of Sjögren syndrome. Also, her brain MRI revealed cerebellar degeneration. There are only four patients reported to be affected by cerebellar atrophy associated with PSS. Administration of high doses of methylprednisolone and cyclophosphamide leads to substantial improvement in the cerebellar symptoms of this case. In addition, after 2 months of follow-up, the patient's ataxia recovered significantly. It could be concluded that in addition to neurological degenerative disorders, in some cases cerebellar atrophy could also be associated with autoimmune conditions such as PSS.
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Affiliation(s)
- Mohammad Heidary
- Department of Internal Medicine, Tehran University of Medical Sciences, Tehran, The Islamic Republic of Iran
| | - Samira Alesaeidi
- Amir Alam Research Center, Tehran University of Medical Sciences, Tehran, Tehran, Iran.,Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Tehran, Iran
| | - Khashayar Afshari
- School of Medicine, Tehran University of Medical Sciences, Tehran, The Islamic Republic of Iran
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Farhat E, Zouari M, Abdelaziz IB, Drissi C, Beyrouti R, Hammouda MB, Hentati F. Progressive cerebellar degeneration revealing Primary Sjögren Syndrome: a case report. CEREBELLUM & ATAXIAS 2016; 3:18. [PMID: 27777786 PMCID: PMC5070353 DOI: 10.1186/s40673-016-0056-0] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/19/2016] [Accepted: 10/04/2016] [Indexed: 12/30/2022]
Abstract
Background Cerebellar ataxia represents a rare and severe complication of Sjӧgren syndrome (SS), especially with a progressive onset and cerebellar atrophy on imaging. Case presentation We report the case of a 30-year-old woman, with a past history of dry eyes and mouth, who presented a severe cerebellar ataxia worsening over 4 years associated with tremor of the limbs and the head. Brain MRI showed bilateral hyperintensities on T2 and FLAIR sequences, affecting periventricular white matter, with marked cerebellar atrophy. Complementary investigations confirmed the diagnosis of primary SS (pSS). The patient was treated by methylprednisolone, Cyclophosphamid and Azathioprine. Her clinical and radiological states are stabilized after 2 years of following. Primary cerebellar degeneration is extremely rarely associated with pSS. Few cases of isolated cerebellar ataxia or belonging to a multifocal disease were reported in the literature, most of them characterized by an acute or rapidly progressive onset. Cerebellar atrophy was described in only three patients. There have been few clarifications of the pathogenesis of the neurological manifestations in pSS. Treatment is based on corticosteroids and immunosuppressive agents with no consensus of a specific therapy. Conclusions Cerebellar ataxia due to pSS may exceptionally mimic a degenerative cerebellar ataxia, especially when the onset is progressive, which represents the particularity of our observation. The role of brain MRI and antibodies remains important for the differential diagnosis.
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Affiliation(s)
- Emna Farhat
- Department of Neurology, National Institute Mongi Ben Hamida of Neurology, Rue Jébal Lakhdhar La Rabta Bab Saâdoun 1007, Tunis, Tunisia
| | - Mourad Zouari
- Department of Neurology, National Institute Mongi Ben Hamida of Neurology, Rue Jébal Lakhdhar La Rabta Bab Saâdoun 1007, Tunis, Tunisia
| | - Ines Ben Abdelaziz
- Department of Neurology, National Institute Mongi Ben Hamida of Neurology, Rue Jébal Lakhdhar La Rabta Bab Saâdoun 1007, Tunis, Tunisia
| | - Cyrine Drissi
- Department of Radiology, National Institute Mongi Ben Hamida of Neurology, Tunis, Tunisia
| | - Rahma Beyrouti
- Department of Neurology, National Institute Mongi Ben Hamida of Neurology, Rue Jébal Lakhdhar La Rabta Bab Saâdoun 1007, Tunis, Tunisia
| | - Mohamed Ben Hammouda
- Department of Radiology, National Institute Mongi Ben Hamida of Neurology, Tunis, Tunisia
| | - Fayçal Hentati
- Department of Neurology, National Institute Mongi Ben Hamida of Neurology, Rue Jébal Lakhdhar La Rabta Bab Saâdoun 1007, Tunis, Tunisia
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Colaci M, Cassone G, Manfredi A, Sebastiani M, Giuggioli D, Ferri C. Neurologic Complications Associated with Sjögren's Disease: Case Reports and Modern Pathogenic Dilemma. Case Rep Neurol Med 2014; 2014:590292. [PMID: 25161786 PMCID: PMC4139080 DOI: 10.1155/2014/590292] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/26/2014] [Revised: 07/14/2014] [Accepted: 07/18/2014] [Indexed: 12/14/2022] Open
Abstract
Objectives. Sjögren's syndrome (SS) may be complicated by some neurological manifestations, generally sensory polyneuropathy. Furthermore, involvement of cranial nerves was described as rare complications of SS. Methods. We reported 2 cases: the first one was a 40-year-old woman who developed neuritis of the left optic nerve as presenting symptom few years before the diagnosis of SS; the second was a 54-year-old woman who presented a paralysis of the right phrenic nerve 7 years after the SS onset. An exhaustive review of the literature on patients with cranial or phrenic nerve involvements was also carried out. Results. To the best of our knowledge, our second case represents the first observation of SS-associated phrenic nerve mononeuritis, while optic neuritis represents the most frequent cranial nerve involvement detectable in this connective tissue disease. Trigeminal neuropathy is also frequently reported, whereas neuritis involving the other cranial nerves is quite rare. Conclusions. Cranial nerve injury is a harmful complication of SS, even if less commonly recorded compared to peripheral neuropathy. Neurological manifestations may precede the clinical onset of SS; therefore, in patients with apparently isolated cranial nerve involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable.
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Affiliation(s)
- Michele Colaci
- Chair and Rheumatology Unit, Medical School, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Policlinico di Modena, Via del Pozzo 71, 41100 Modena, Italy
| | - Giulia Cassone
- Chair and Rheumatology Unit, Medical School, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Policlinico di Modena, Via del Pozzo 71, 41100 Modena, Italy
| | - Andreina Manfredi
- Chair and Rheumatology Unit, Medical School, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Policlinico di Modena, Via del Pozzo 71, 41100 Modena, Italy
| | - Marco Sebastiani
- Chair and Rheumatology Unit, Medical School, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Policlinico di Modena, Via del Pozzo 71, 41100 Modena, Italy
| | - Dilia Giuggioli
- Chair and Rheumatology Unit, Medical School, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Policlinico di Modena, Via del Pozzo 71, 41100 Modena, Italy
| | - Clodoveo Ferri
- Chair and Rheumatology Unit, Medical School, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Policlinico di Modena, Via del Pozzo 71, 41100 Modena, Italy
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Carrozzo M, Scally K. Oral manifestations of hepatitis C virus infection. World J Gastroenterol 2014; 20:7534-7543. [PMID: 24976694 PMCID: PMC4069285 DOI: 10.3748/wjg.v20.i24.7534] [Citation(s) in RCA: 47] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/13/2013] [Revised: 01/21/2014] [Accepted: 03/19/2014] [Indexed: 02/06/2023] Open
Abstract
Extrahepatic manifestations (EHMs) of hepatitis C virus (HCV) infection can affect a variety of organ systems with significant morbidity and mortality. Some of the most frequently reported EHM of HCV infection, involve the oral region predominantly or exclusively. Oral lichen planus (OLP) is a chronic inflammatory condition that is potentially malignant and represents cell-mediated reaction to a variety of extrinsic antigens, altered self-antigens, or super antigens. Robust epidemiological evidence support the link between OLP and HCV. As the virus may replicate in the oral mucosa and attract HCV-specific T lymphocytes, HCV may be implicated in OLP pathogenesis. Sjögren syndrome (SjS) is an autoimmune exocrinopathy, characterized by dryness of the mouth and eyes and a multitude of other systemic signs and symptoms. SjS patients have also an increased risk of non-Hodgkin lymphoma. Patients with chronic hepatitis C do frequently have histological signs of Sjögren-like sialadenitis with mild or even absent clinical symptoms. However, it is still unclear if HCV may cause a disease mimicking SjS or it is directly responsible for the development of SjS in a specific subset of patients. Oral squamous cell carcinoma is the most common oral malignant tumour and at least in some part of the world could be linked to HCV.
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Miwa Y, Inokuma S, Yokoe Y, Okazaki Y, Sato T, Maezawa R. Peripheral nervous system involvement complicated due to vasculitis in cases of primary Sjögren's syndrome: case studies in a single hospital and a literature review. Mod Rheumatol 2014; 13:160-7. [DOI: 10.3109/s10165-002-0216-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
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Berkowitz AL, Samuels MA. The neurology of Sjögren's syndrome and the rheumatology of peripheral neuropathy and myelitis. Pract Neurol 2013; 14:14-22. [DOI: 10.1136/practneurol-2013-000651] [Citation(s) in RCA: 52] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/14/2023]
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Differential, size-dependent sensory neuron involvement in the painful and ataxic forms of primary Sjögren's syndrome-associated neuropathy. J Neurol Sci 2012; 319:139-46. [DOI: 10.1016/j.jns.2012.05.022] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2012] [Revised: 05/08/2012] [Accepted: 05/09/2012] [Indexed: 11/22/2022]
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Demarquay G, Honnorat J. Clinical presentation of immune-mediated cerebellar ataxia. Rev Neurol (Paris) 2010; 167:408-17. [PMID: 21055784 DOI: 10.1016/j.neurol.2010.07.032] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2010] [Revised: 06/13/2010] [Accepted: 07/20/2010] [Indexed: 11/25/2022]
Abstract
INTRODUCTION Accumulation of recent clinical evidence indicates that the immune system plays an important role in some central nervous system diseases usually regarded as degenerative. The most striking example is paraneoplastic cerebellar ataxia (PCA), which is characterized by autoimmune cross-reaction between tumoral and nervous system antigens. STATE OF THE ART In the past 20 years, several antibodies directed against neuronal and tumoral antigens have been described in association with PCA, leading to the description of different subtypes of PCA based on the associated antibodies, the clinical course and the type of tumor. In some subtypes, cerebellar ataxia occurs in isolation, whereas in others, cerebellar ataxia is a syndrome that occurs in conjunction with extensive nervous system disease. Circulating antibodies have also been described in patients with non-paraneoplastic cerebellar ataxia (N-PCA), suggesting that the immune system may be involved in certain cases of sporadic cerebellar ataxia. PERSPECTIVE Immune-mediated cerebellar ataxia does not seem to be limited to paraneoplastic neurological syndromes. Further studies are however necessary to understand the exact pathophysiology of these disorders and offer effective treatments. CONCLUSION In this review, the clinical presentation of the different subtypes of potentially immune-mediated PCA and N-PCA will be described, and the associated tumors will be discussed.
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Affiliation(s)
- G Demarquay
- Centre de référence, de diagnostic et de traitement des syndromes neurologiques paranéoplasiques, hôpital neurologique Pierre-Wertheimer, 69677 Bron cedex, France
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Collison K, Rees J. Asymmetric cerebellar ataxia and limbic encephalitis as a presenting feature of primary Sjögren's syndrome. J Neurol 2007; 254:1609-11. [PMID: 17805471 DOI: 10.1007/s00415-007-0596-6] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2006] [Revised: 01/28/2007] [Accepted: 03/13/2007] [Indexed: 10/22/2022]
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Abstract
Examination of the pupil provides an opportunity to detect disturbances in the autonomic innervation of the eye. The pupil is frequently affected in patients with generalized autonomic neuropathies. This literature review confirms a high prevalence of sympathetic deficits and parasympathetic deficits in acute or subacute dysautonomia, diabetes, amyloidosis, pure autonomic failure, paraneoplastic syndromes, Sjögren syndrome, familial dysautonomia, and dopamine beta-hydroxylase deficiency. It confirms the relative scarcity of a pupil abnormality in patients with multiple system atrophy. There are difficulties in clinical diagnosis of pupil abnormalities and interpretation of pupil pharmacologic tests, particularly when combined sympathetic and parasympathetic deficits are present.
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Affiliation(s)
- Fion D Bremner
- Department of Neuro-ophthalmology, National Hospital for Neurology and Neurosurgery, London, United Kingdom
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Mori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H, Katsuno M, Fujita A, Aiba I, Ogata A, Saito T, Asakura K, Yoshida M, Hirayama M, Sobue G. The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy. ACTA ACUST UNITED AC 2005; 128:2518-34. [PMID: 16049042 DOI: 10.1093/brain/awh605] [Citation(s) in RCA: 344] [Impact Index Per Article: 17.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
Abstract
We assessed the clinicopathological features of 92 patients with primary Sjögren's syndrome-associated neuropathy (76 women, 16 men, 54.7 years, age at onset). The majority of patients (93%) were diagnosed with Sjögren's syndrome after neuropathic symptoms appeared. We classified these patients into seven forms of neuropathy: sensory ataxic neuropathy (n = 36), painful sensory neuropathy without sensory ataxia (n = 18), multiple mononeuropathy (n = 11), multiple cranial neuropathy (n = 5), trigeminal neuropathy (n = 15), autonomic neuropathy (n = 3) and radiculoneuropathy (n = 4), based on the predominant neuropathic symptoms. Acute or subacute onset was seen more frequently in multiple mononeuropathy and multiple cranial neuropathy, whereas chronic progression was predominant in other forms of neuropathy. Sensory symptoms without substantial motor involvement were seen predominantly in sensory ataxic, painful sensory, trigeminal and autonomic neuropathy, although the affected sensory modalities and distribution pattern varied. In contrast, motor weakness and muscle atrophy were observed in multiple mononeuropathy, multiple cranial neuropathy and radiculoneuropathy. Autonomic symptoms were often seen in all forms of neuropathy. Abnormal pupils and orthostatic hypotension were particularly frequent in sensory ataxic, painful, trigeminal and autonomic neuropathy. Unelicited somatosensory evoked potentials and spinal cord posterior column abnormalities in MRI were observed in sensory ataxic, painful and autonomic neuropathy. Sural nerve biopsy specimens (n = 55) revealed variable degrees of axon loss. Predominantly large fibre loss was observed in sensory ataxic neuropathy, whereas predominantly small fibre loss occurred in painful sensory neuropathy. Angiitis and perivascular cell invasion were seen most frequently in multiple mononeuropathy, followed by sensory ataxic neuropathy. The autopsy findings of one patient with sensory ataxic neuropathy showed severe large sensory neuron loss paralleling to dorsal root and posterior column involvement of the spinal cord, and severe sympathetic neuron loss. Degrees of neuron loss in the dorsal and sympathetic ganglion corresponded to segmental distribution of sensory and sweating impairment. Multifocal T-cell invasion was seen in the dorsal root and sympathetic ganglion, perineurial space and vessel walls in the nerve trunks. Differential therapeutic responses for corticosteroids and IVIg were seen among the neuropathic forms. These clinicopathological observations suggest that sensory ataxic, painful and perhaps trigeminal neuropathy are related to ganglioneuronopathic process, whereas multiple mononeuropathy and multiple cranial neuropathy would be more closely associated with vasculitic process.
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Affiliation(s)
- Keiko Mori
- Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan
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Mori K, Koike H, Misu K, Hattori N, Ichimura M, Sobue G. Spinal cord magnetic resonance imaging demonstrates sensory neuronal involvement and clinical severity in neuronopathy associated with Sjögren's syndrome. J Neurol Neurosurg Psychiatry 2001; 71:488-92. [PMID: 11561032 PMCID: PMC1763541 DOI: 10.1136/jnnp.71.4.488] [Citation(s) in RCA: 61] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/03/2022]
Abstract
OBJECTIVES To determine spinal cord MRI findings in neuronopathy associated with Sjögren's syndrome and their correlation with severity of sensory impairment. METHODS Clinical and electrophysiological features, pathological findings in the sural nerve, and hyperintensity on T2* weighted MRI in the spinal dorsal columns were evaluated in 14 patients with neuronopathy associated with Sjögren's syndrome. RESULTS Of 14 patients, 12 showed high intensity by T2* weighted MRI in the posterior columns of the cervical cord. High intensity areas were seen in both the fasciculus cuneatus and gracilis in nine patients, who showed severe and widespread sensory deficits in the limbs and trunk; these patients also had a high frequency of autonomic symptoms. Somatosensory evoked potentials often could not be elicited. Hyperintensity restricted to the fasciculus gracilis was seen in three patients, who showed sensory deficits restricted to lower limbs without trunk involvement, or with only partial limb involvement; no autonomic symptoms were noted. The two patients who did not show high intensity areas in the dorsal columns showed restricted sensory involvement in the limbs. All patients showed axonal loss predominantly affecting large fibres, without axonal sprouting. CONCLUSIONS High intensity areas on T2* weighted MRI in the spinal dorsal columns reflect the degree of sensory neuronal involvement in neuronopathy associated with Sjögren's syndrome; this finding could also be a helpful marker for estimating severity of this neuronopathy.
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Affiliation(s)
- K Mori
- Department of Neurology, Nagoya University School of Medicine, Nagoya 466-8550 Japan
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Abstract
Sjögren's syndrome (SS) is a systemic lymphoproliferative, autoimmune disease, which is characterized by dryness of the eyes, mouth, and other mucous membranes. The nervous system may be affected in up to 20% of the cases of primary or secondary SS. We present a case of a 54-year-old woman with trochlear nerve palsy complicating Sjögren's syndrome secondary to rheumatoid arthritis. We suggest that all patients with multiple cranial neuropathies, especially when associated with rheumatoid arthritis, should be carefully examined for the possible presence of secondary SS.
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Affiliation(s)
- K Chu
- Department of Neurology, Seoul National University Hospital, Neuroscience Research Institute, SNUMRC, 28 Yongon-dong, Chongno-gu, 110-744, Seoul, South Korea
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Tesar JT, McMillan V, Molina R, Armstrong J. Optic neuropathy and central nervous system disease associated with primary Sjögren's syndrome. Am J Med 1992; 92:686-92. [PMID: 1605151 DOI: 10.1016/0002-9343(92)90788-d] [Citation(s) in RCA: 34] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
Three cases of optic neuropathy associated with primary Sjögren's syndrome are reported. All three patients had clinical manifestations of primary Sjögren's syndrome, although two of the patients did not report sicca symptoms at initial examination. Two patients had focal neurologic signs in addition to optic neuropathy. The differentiation of this syndrome of optic neuropathy, focal neurologic signs, and Sjögren's syndrome from multiple sclerosis and antiphospholipid antibody syndrome is important for reasons of treatment and prognosis. This diagnostic differentiation was facilitated by positive tests for xerophthalmia and findings of positive minor salivary gland biopsy. High titers of antinuclear antibody, anti-SSA(Ro), and anti-SSB(La), and the absence of antiphospholipid antibodies provided additional help in the differential diagnosis. In 5 years of observation, none of the patients developed symptoms of multiple sclerosis or additional connective tissue disorders.
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Affiliation(s)
- J T Tesar
- Department of Medicine, Walter Reed Army Medical Center, Washington, D.C. 20307-5001
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Hietaharju A, Korpela M, Ilonen J, Frey H. Nervous system disease, immunological features, and HLA phenotype in Sjögren's syndrome. Ann Rheum Dis 1992; 51:506-9. [PMID: 1586250 PMCID: PMC1004702 DOI: 10.1136/ard.51.4.506] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
Twenty seven patients with primary or possible Sjögren's syndrome with neurological manifestations were compared immunologically with 21 patients with Sjögren's syndrome with an intact nervous system. Patients with Sjögren's syndrome were divided into seropositive and seronegative subgroups with respect to the occurrence of one or more autoantibodies (antinuclear antibodies, rheumatoid factor, antibodies to SS-B) in their serum samples. This study of 48 patients indicates that the spectrum of nervous system disease in seronegative and seropositive subgroups is almost indistinguishable. No significant differences were found in the occurrence of circulating immune complexes, the levels of serum complement C3 and C4, or serum IgA, IgM, and beta 2 microglobulin with respect to the neurological manifestations. The serum IgG level, however, was significantly higher in the patients with Sjögren's syndrome with intact nervous systems than in those with neurological manifestations. No significant association was found between the HLA phenotype and nervous system disease. The occurrence of HLA-B8 and DR3 antigens was, however, significantly higher in those patients with antibodies to SS-B than in those without. This finding supports the suggestion that HLA-B8/DR3 may modulate autoantibody responses rather than disease expression in Sjögren's syndrome.
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Affiliation(s)
- A Hietaharju
- Institute of Clinical Sciences, University of Tampere, Finland
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Kaplan JG, Rosenberg R, Reinitz E, Buchbinder S, Schaumburg HH. Invited review: peripheral neuropathy in Sjogren's syndrome. Muscle Nerve 1990; 13:570-9. [PMID: 2167450 DOI: 10.1002/mus.880130703] [Citation(s) in RCA: 63] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
Our experience and review of the literature reveal that Sjogren's syndrome (SS) is an important, poorly recognized cause of peripheral neuropathy. Several forms of peripheral nerve dysfunction occur in SS including trigeminal sensory neuropathy, mononeuropathy multiplex, distal sensory neuropathy, distal sensorimotor peripheral neuropathy and a pure sensory neuronopathy syndrome. Rarely, chronic relapsing inflammatory polyneuropathy and multiple cranial neuropathies appear. Clinical evidence of glandular involvement is often minimal or absent when patients with SS develop peripheral neuropathy; and the diagnosis of the underlying condition is elusive. We review clinical and laboratory features of this disorder and suggest appropriate evaluation of patients with neuropathy and suspected SS.
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Affiliation(s)
- J G Kaplan
- Department of Neurology, Albert Einstein College of Medicine, Bronx, New York
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Alexander EL, Lijewski JE, Jerdan MS, Alexander GE. Evidence of an immunopathogenic basis for central nervous system disease in primary Sjögren's syndrome. ARTHRITIS AND RHEUMATISM 1986; 29:1223-31. [PMID: 2429673 DOI: 10.1002/art.1780291007] [Citation(s) in RCA: 42] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
The pathogenesis of central nervous system complications in primary Sjögren's syndrome (CNS-SS) is unknown. In order to determine whether patients with active CNS-SS have cerebrospinal fluid (CSF) abnormalities indicative of CNS inflammation, CSF analyses from 30 patients with active CNS-SS (SSA) were contrasted with those from 20 SS patients without CNS involvement (SSI) and 20 patients with systemic lupus erythematosus and active CNS disease (SLEA). Elevations of total protein concentration, IgG concentration, IgG to total protein ratio, and IgG index were observed in patients with SSA, but not in those with SSI. Agarose gel electrophoresis results were abnormal, with 1 or more bands, in 25 of 29 SSA patients (86%), but in only 3 of 18 SSI patients (17%). Similar, but less striking, CSF abnormalities were seen in a minority of SLEA patients. Fifteen SSA patients (50%) had transient, mild-to-moderate CSF pleocytosis, while only 1 SSI patient and 2 SLEA patients had similar findings. Cytologic findings were abnormal in 18 SSA patients (60%); these included atypical mononuclear cells, lymphoblastoid cells, and plasma cells. The presence of immunocompetent cells and evidence for the intrathecal synthesis of IgG within the CSF of SSA, but not SSI, patients provide diagnostic parameters which are indicative of active disease and which can be monitored serially during therapy.
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Abstract
Three patients with the sicca syndrome and chronic sensory neuropathy are described; in two of them neuropathy was the presenting feature of the disease. The sicca syndrome can give rise to a characteristic neurological syndrome comprising areflexia and asymmetrical sensory loss, particularly of proprioception, in the limbs. This is often associated with tonic pupils and trigeminal anaesthesia.
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de la Monte SM, Hutchins GM, Gupta PK. Polymorphous meningitis with atypical mononuclear cells in Sjögren's syndrome. Ann Neurol 1983; 14:455-61. [PMID: 6638957 DOI: 10.1002/ana.410140410] [Citation(s) in RCA: 33] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
Central nervous system complications in Sjögren's syndrome have been reported sporadically for years. We examined the nature and frequency of central nervous system abnormalities in 11 patients with clinically documented Sjögren's syndrome on whom postmortem examination was performed. In 9, characteristic mixed (polymorphous) inflammatory infiltrates containing large atypical mononuclear cells were observed in the leptomeninges, choroid plexus, or both; only 5 of the 9 neurological symptoms, however. Among patients with central nervous system lesions, 3 had definite vasculocentric inflammation and 1 had a necrotizing vasculitis with extensive subarachnoid hemorrhage. Four patients had evidence of chronic subarachnoid microhemorrhage associated with polymorphous meningitis. Atypical mononuclear cells in a polymorphous inflammatory exudate were observed in antemortem cerebrospinal fluid cytological specimens from 2 of the patients. The findings suggest that central nervous system involvement in Sjögren's syndrome is common and that neurological symptoms are related to polymorphous meningitis and vasculitis. Detection of atypical mononuclear cells in cerebrospinal fluid specimens may be of diagnostic value.
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Marbini A, Gemignani F, Manganelli P, Govoni E, Bragaglia MM, Ambanelli U. Hypertrophic neuropathy in Sjögren;s syndrome. Acta Neuropathol 1982; 57:309-12. [PMID: 6291308 DOI: 10.1007/bf00692189] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/19/2023]
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Abstract
The occurrence of carcinoma in situ in the stomach and dysimmunoglobulinaemia in a patient who had Sjögren's syndrome is described. This is believed to be the first report of such a case to be found in the literature. Although carcinoma has occasionally been described, in this syndrome its occurrence is probably coincidental and not part of the disease process. There is a greater incidence of lymphoid tumours in Sjögren's syndrome than in the general population. Corticosteroid therapy favourably influenced the immunoglobulin abnormalities and some of the clinical manifestations of the disease. A brief review of the literature concerning Sjögren's syndrome, auto-immunity and malignant disease is included.
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