|CASE BASED REVIEW
|Year : 2021 | Volume
| Issue : 3 | Page : 349-352
Quadriparesis in lupus: A clinical challenge
Rashmi Roongta, Sumantro Mondal, Alakendu Ghosh
Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India
|Date of Submission||16-Feb-2021|
|Date of Acceptance||04-Apr-2021|
|Date of Web Publication||21-Sep-2021|
Dr. Rashmi Roongta
Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, 244, AJC Bose Road, Kolkata - 700 020, West Bengal
Source of Support: None, Conflict of Interest: None
Neuropsychiatric manifestations are commonly seen in lupus and limb paresis is commonly due to noncompressive spinal etiology. Here, we report a case of a 50-year-old female with Jaccoud's arthropathy who had presented with quadriparesis for 3 months. She was diagnosed with lupus during her hospital stay. Her magnetic resonance imaging of the spine showed the presence of a soft-tissue mass in the cervical spine with atlantoaxial dislocation causing severe cord compression. This case highlights the importance of suspecting compressive myelopathy secondary either to infection or inflammation due to disease process in the cervical spine of patients with lupus, especially with Jaccoud's arthritis, presenting with weakness of limbs.
Keywords: Atlantoaxial dislocation, lupus, pannus, quadriparesis
|How to cite this article:|
Roongta R, Mondal S, Ghosh A. Quadriparesis in lupus: A clinical challenge. Indian J Rheumatol 2021;16:349-52
| Introduction|| |
The spectrum of neuropsychiatric (NP) manifestations in systemic lupus erythematosus (SLE) is varied and can range from headaches and mood disorders to seizures and coma. Due to this, the reported the prevalence of NP manifestations vary greatly, but around 56% of SLE patients have NP manifestations. Neurological manifestations can also be the presenting feature of lupus. Case definitions for 19 central and peripheral nervous system syndromes have been established by the American College of Rheumatology. The two spinal pathologies which can cause quadriplegia that has been mentioned in it are transverse myelopathy and demyelinating syndromes-both being noncompressive in nature. Compressive pathologies of the spinal cord causing quadriplegia are mostly limited to trauma, infections, or atlantoaxial dislocation (AAD) causing cord compression. In this case, we describe a rare compressive cause of quadriparesis in a patient of SLE.
| Case Report|| |
A 50-year-old female presented with arthritis involving small and large joints, oral ulcers, and alopecia for the past 3 years. She presented with insidious onset, progressive weakness of all four limbs for the past 3 months, and had been prescribed medications without relief. For the past 1 month, she had become bedridden and had developed bladder and bowel involvement. There was no preceding history of trauma, fall, neck pain, or fever. She had been treated with Sulfasalazine and steroids for possible seronegative rheumatoid arthritis intermittently for the past 3 years. There was no family history of inflammatory polyarthritis or any connective tissue disorder. On examination, she had pallor, oral ulcers, and Jaccoud's arthropathy. There was no evidence of ligamentous laxity. Central nervous system examination revealed generalized muscle atrophy, quadriparesis with a power of 1/5 in all four limbs, exaggerated deep-tendon reflexes, and extensor plantar response. Cranial nerve examination was normal and all sensory modalities were impaired. There was no spinal tenderness. The rest of the systemic examination was unremarkable.
Investigations revealed anemia (hemoglobin: 10.2 g/dl), with normal leukocyte and platelet count. Serum electrolytes, renal, and liver function tests were normal. Her antinuclear antibody (ANA) was positive (4+, homogeneous pattern). ANA profile showed anti-Ro and anti-Ribonucleoprotein P positivity. Anti-double stranded-DNA antibody level was high (450 IU/ml, normal: 100 IU/ml) with hypocomplementemia. Rheumatoid factor and anti-cyclic citrullinated peptide (Anti-CCP) antibody were negative. She was diagnosed with having SLE based on the 2012 SLICC criteria. Magnetic resonance imaging (MRI) of the cervical spine revealed significant AAD with myelomalacia [Figure 1]a and [Figure 1]b. The anteroposterior diameter of the upper cervical spine was 5.5 mm and there was heterogeneous contrast enhancement in the pre- and paravertebral soft tissue in the post contrast T1-weighted images indicating vascularity and low-intensity signal areas on T2-weighted images suggestive of chronicity. The findings of a chronic but vascular soft-tissue mass was suggestive of a pannus. Cerebrospinal fluid (CSF) study showed high protein levels with normal cell counts.
|Figure 1: AAD in lupus. Contrast-enhanced MRI showing (a) AAD and enhancing soft tissue - pannus (black arrow) around the dens and cervical cord compression (white arrowhead) in T1 weighted image. (b) The pannus appears as a low to mixed signal intensity lesion (white arrow) in T2 weighted image|
Click here to view
She was started on intravenous dexamethasone for cord compression. A rigid cervical collar was provided for spinal stability. There was no discernible improvement and she had a sudden cardiac arrest 3 days later. Resuscitation was unsuccessful and she succumbed to her illness.
| Discussion|| |
AAD refers to the instability between the Atlas More Details and the axis and can be due to traumatic, congenital, idiopathic, or inflammatory causes. Among the inflammatory causes, chronic RA is most common. Chronic systemic inflammation leads to chronic synovitis, ligamentous laxity and bone erosions. Laxity of the ligamentous restraints leads to anterior AAD. Symptoms of AAD can range from neck pain to limb weakness, lower cranial nerve involvement, sphincter disturbances, vertebral artery dissection, respiratory failure, and death.
We searched PubMed, Embase, Medline from inception till March 4, 2021, using the keywords "lupus, SLE, atlantoaxial, subluxation and dislocation" for relevant publications. After deduplication, 9 publications were identified. References of the above publications were also studied. Four publications of AAD in SLE were selected [Table 1].,,, Three articles were excluded as they were not relevant and another two were excluded as full text articles were not available., AAD in lupus can occur due to the weakening of the transverse ligament which is vital for the stability of the joint, or less commonly, due to a soft-tissue pannus which is more commonly seen in RA. In a prospective study of lupus patients, five of 59 patients (8.5%) were identified to have an anterior atlantoaxial subluxation in full flexion/extension films. The patients with subluxation had a significantly longer disease duration (12 years) as compared to those without (6.6 years, P < 0.01). Furthermore, patients with subluxation had a significantly increased incidence of Jaccoud's syndrome and articular hypermobility. Our patient had a shorter disease duration of approximately 3 years but had evidence of Jaccoud's arthritis on the examination which is a chronic, deforming, nonerosive arthropathy occurring in SLE causing severe functional limitation.
|Table 1: Published case reports of atlantoaxial dislocation in systemic lupus erythematosus|
Click here to view
Nakamura et al. reported a case of atlantoaxial subluxation in a 44-year-old Japanese woman with lupus diagnosed at the age of 26 years. She had ligamentous laxity and Jaccoud's arthritis on examination and presented with neck discomfort but no neurological deficit like in our patient. Further, imaging revealed an absence of inflammatory hallmarks of synovial proliferation or bony erosions. The atlantoaxial subluxation was attributed to the weakening of supporting tissues like the transverse atlantal ligament in the context of generalized ligamentous laxity. Her anti-CCP titers were weakly positive, but there was no other evidence of rheumatoid arthritis. She was managed conservatively with a cervical collar.
Another case reported by Isenberg was of a 28-year-old female with a 12-year history of RA, who developed a malar rash, positive ANA, and was subsequently diagnosed with having lupus also. She then developed neck pain and was found to have AAD on X-ray. However, it was a case of AAD in a patient with co-existing SLE and long-standing, erosive RA. She was also managed conservatively with a cervical collar.
Ishchenko et al. reported a case of a 67-year old Asian female with SLE for 26 years who came with subacute onset of quadriparesis. She had severe Jaccoud's arthritis and had recently developed occipital headaches, with weakness of upper and lower limbs along with paresthesias of upper limbs. Examination revealed weakness of all four limbs with sensory abnormalities. Her MRI showed severe narrowing of the cervical spine at the C1 level with an AP diameter of 6 mm, due to a soft-tissue mass around the odontoid process. High signal intensity in the cord on T2-weighted images suggested myelopathy. She received pulse methylprednisolone of 1 g for 3 days and showed rapid improvement to an ambulant state in a week. Surgery was deferred due to a lack of cervical instability. Subsequent MRI scans done showed regression of the pannus and resolution of the signs of myelopathy. Our patient had a similar MRI picture with severe cervical cord compression with odontoid erosions but did not improve despite steroids. It could be because of the delayed presentation and initiation of treatment. Such severe manifestations of AAD are rare and to the best of our knowledge, it is the second case report of AAD in SLE presenting with quadriparesis.
This case highlights the lesser-known entity of AAD in SLE. It should be suspected in the presence of symptoms of neck pain or limb weakness, especially if there is ligamentous laxity or Jaccoud's arthritis. The subacute course of weakness (over 3 months), quadriparesis, absence of positive sensory symptoms, with the absence of CSF pleocytosis were pointers against a noncompressive myelopathy in this patient. It is a potentially devastating condition if allowed to progress. Prompt suspicion and use of a cervical collar can possibly prevent life-threatening complications. Early detection of cord compression and use of high dose steroids may lead to complete recovery and avoidance of surgery.
| Conclusion|| |
This case is of a 50-year-old female who had symptoms suggestive of lupus and came with weakness of all four limbs with sensory and bowel involvement for 3 months. She was subsequently diagnosed to have lupus with AAD secondary to a pannus at the atlantoaxial joint. She also had Jaccoud's arthritis which is associated with AAD and ligamentous laxity in lupus patients. This case highlights the importance of suspecting compressive myelopathy secondary either to infection or inflammation due to disease process in the cervical spine of patients with SLE, especially with Jaccoud's arthritis, presenting with weakness of limbs. Early recognition and treatment in the form of a cervical collar and initiation of high dose steroids may lead to recovery.
Written informed consent was taken from the patient's relatives.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Unterman A, Nolte JE, Boaz M, Abady M, Shoenfeld Y, Zandman-Goddard G. Neuropsychiatric syndromes in systemic lupus erythematosus: A meta-analysis. Semin Arthritis Rheum 2011;41:1-11.
The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999;42:599-608.
Yang SY, Boniello AJ, Poorman CE, Chang AL, Wang S, Passias PG. A review of the diagnosis and treatment of atlantoaxial dislocations. Global Spine J 2014;4:197-210.
Wasserman BR, Moskovich R, Razi AE. Rheumatoid arthritis of the cervical spine - clinical considerations. Bull NYU Hosp Jt Dis 2011;69:136-48.
Babini SM, Cocco JA, Babini JC, de la Sota M, Arturi A, Marcos JC. Atlantoaxial subluxation in systemic lupus erythematosus: Further evidence of tendinous alterations. J Rheumatol 1990;17:173-7.
Nakamura K, Tajima Y, Takai O. Generalized laxity of connective tissue as a possible syndrome in systemic lupus erythematosus. Mod Rheumatol 2010;20:522-7.
Ishchenko A, Malghem J, Banse X, Houssiau FA. Unusual case of tetraparesis in a patient with systemic lupus erythematosus. Lupus 2015;24:760-3.
Isenberg DA, Tookman A, White AG, Hoffbrand BI, Mowbray J. Erosive rheumatoid arthritis co-existing with systemic lupus erythematosus. A report of a case, also showing atlanto-axial subluxation. Clin Rheumatol 1982;1:216-20.
Klemp P, Meyers OL, Keyzer C. Atlanto-axial subluxation in systemic lupus erythematosus: A case report. S Afr Med J 1977;52:331-2.
Okazaki T, Saito T, Ikeda M. Panel discussion. 1. Systemic lupus erythematodes with advanced dislocation of the atlanto-axial articulation. Ryumachi 1973;13:58.
Cruz Whitley J, Aronowitz P. Jaccoud's arthropathy. J Gen Intern Med 2018;33:1583.