Severe acute polyradiculoneuritis revealing systemic lupus erythematosus: a case report

¹Department of Neurology, University Teaching Hospital Mohammed VI, Marrakech, Morocco, ² Moroccan Society of Neurophysiology, Marrakech, Morocco, ³Laboratory of Physiology, Faculty of Medicine and Pharmacy of Marrakech, Marrakech, Morocco, ⁴Laboratory of Clinical and Experimental Neuroscience, Faculty of Medicine, Cadi Ayyad University Marrakech, Marrakech, Morocco

^&Corresponding author
Assma Boudanga, Department of Neurology, University Teaching Hospital Mohammed VI, Marrakech, Morocco

Introduction    

The neurological manifestations of systemic Lupus Erythematosus (SLE) are frequent but extremely heterogeneous in their clinical expression. Depending on the criteria used, peripheral nervous system involvement is not significant and varies from 2% to 30% [1]. Multiple mononeuropathies, including axonal, distal and symmetrical, sensory or sensori-motor polyneuropathies, or cranial nerve involvement are common [2]. However, few cases have been reported in the last 50 years where the initial manifestation of SLE is Guillain-Barré syndrome (GBS). The first case was confirmed in 1964 by Gargour et al. [3]. Our work highlights the importance of evoking SLE as a potential etiology in a patient presenting with acute polyradiculoneuritis.

Patient and observation     

Patient information: a 41-year-old woman with a history of three miscarriages and skin photosensitivity, without other medical, family, psycho-social history or past interventions, who presented with a symptomatology of abrupt onset, admitted in the plateau phase, made up of a synchronous and symmetrical ascending functional impotence of both lower limbs 20 days before admission.

Clinical findings: the clinical examination found a dyspneic patient with a motor deficit evaluated at 4/5 proximal and 3/5 distal to the four limbs, a hypotonia more accentuated in the two lower limbs and an osteotendinous areflexia in the four limbs. Sensibility and cranial pairs were intact with a plantar skin reflex in flexion. Dermatological examination revealed generalized myxedema associated with alopecia (Figure 1).

Diagnostic assessment: electroneuromyography (ENMG) revealed collapse of motor potential and absence of F waves in all four limbs. The study of the sensory nerve conduction is without abnormality suggesting an acute axonal motor polyradiculoneuropathy more marked in the two lower limbs (Figure 2). Biological abnormalities were in favor of SLE (Table 1). Radiological exploration revealed hepatomegaly and large effusions: peritoneal, pericardial, and bilateral pleural, which explain the severity of the clinical picture. Complete brain and spinal cord magnetic resonance imaging (MRI) were normal as part of the extension workup.

Diagnostic/therapeutic intervention: the diagnosis of acute Guillain-Barré type Pro Re Nata (PRN) associated with lupus disease was retained, and a bolus of methyl-prednisolone at a dose of 1g per day was established for five days. The patient benefited from monthly courses of cyclophosphamide 1g, synthetic antimalarials such as hydroxychloroquine (Plaquenil) 400 mg per day, and complementary corticosteroid therapy of 60 mg for six weeks with progressive reduction of 5 mg every 15 days.

Follow-up and outcomes: the evolution is marked by a clear clinical improvement with recovery of walking, a markedly improved muscle testing and no side effects related to the treatment. The prognosis of our case is clearly good explained by the therapeutic precocity.

The patient's perspective: the patient reports satisfaction following the recovery of motor functions and her autonomy with a marked improvement in her quality of life. The psychological interview of the patient also demonstrates a stable post-therapeutic psychic state.

Discussion     

Systemic lupus is retained for the patient according to the 1982 American College of Rheumatology (ACR) criteria, revised in 1997 and the EULAR/ACR 2019 classification criteria [4]. Peripheral neuropathies are only considered in the list of neuropsychological manifestations in the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for systemic lupus, which are central, peripheral, autonomic nervous system, and psychiatric syndromes. Peripheral involvement is exceptionally revealing of the disease. Symmetrical distal polyneuropathies, pure sensory or sensory-motor polyneuropathies and multiple mononeuropathies are the most common peripheral disorders [1]. Subacute or chronic PRN characterized by the presence of an inflammatory syndrome and hyperproteinorachia are rare [5], but GBS in its severe form is much rarer. According to prospective studies based on electrophysiological data rather than symptoms, acute PRN can take several forms, the most common of which is sensorimotor axonal neuropathy [6]. In the light of the literature, Hess et al. reported in 1990 the first case of Miller Fisher syndrome, a variant of Guillain-Barré, complicating systemic lupus erythematosus with improvement by five sessions of plasmapheresis after therapeutic failure of immunoglobulins. This result is confirmed by the study of Bingisser et al. in 1994 [7]. Ait Benhaddou et al. 2003, reported a case of acute sensitive motor axonal PRN with SLE, while Quaid Nadri et al. 2015, presented the case of a young woman with GBS who was later diagnosed with SLE and class V lupus nephritis (LN) [5,8]. In the case of FZ Haounou et al. 2014, it was a demyelinating disease with axonal loss [2]. The other cases described in the literature are mainly demyelinating forms, however in our observation; it is a pure motor axonal involvement.

Like our observation, the cerebrospinal fluid (CSF) study showed no abnormality in most of the other cases (Table 2). The evolution of the patient, in our investigation, is marked by the improvement of neurological signs after the first courses of cyclophosphamide 1g preceded by five boluses of 1g of methylprednisolone. Furthermore, Santiago-Casas Y et al. 2013, report the observation of two cases of acute axonal neuropathies of GBS type, the first with acute motor axonal neuropathy, the second with acute motor and sensory axonal neuropathy in the context of SLE. The first case did not respond to treatment with intravenous immunoglobulin (IVIG) and plasmapheresis, whereas the second case progressed favorably after administration of high-dose glucocorticoids and monthly infusions of low-dose intravenous cyclophosphamide. Both patients remained in clinical remission and without neurological sequelae after 10 and 3 years of follow-up, respectively [9]. Furthermore, Quaid Nadri et al. 2015 noted the disappearance of neurological symptoms after plasmapheresis, IVIG, and corticosteroid treatment. Cyclophosphamide was not administered, although others have reported success when used [8]. The diagnostic and therapeutic management of neurolupus is a challenge for the clinician. It is still poorly codified and largely determined by the experience of the teams [10]. It is difficult to predict the prognosis. Although a favorable outcome is usual, mortality is not zero, especially in severe forms, including acute PRN with respiratory involvement, where immunosuppressant, plasma exchange and IVIG are often required [5].

Conclusion     

Acute PRN remains a rare neurological manifestation in SLE. Bolus methylprednisolone and monthly courses of cyclophosphamide are proposed in this clinical presentation, in which a favorable clinical response was obtained despite the severity of the picture, which explains that early management of patients can improve the prognosis of the disease.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

Assma Boudanga: conception, execution, writing of the first draft; Mohamed Chraa: organization, design, writing of the first draft; Oussama Cherkaoui Rhazouani: conception, review and critic; Najib Kissani: conception, review and critic. All the authors have read and agreed to the final manuscript.

Tables and figures     

Table 1: the results of the biological assessment

Table 2: cases of acute polyradiculoneuritis in the context of systemic lupus erythematosus published in the literature

Figure 1: alopecia

Figure 2: collapse of motor action potential in the lower limbs

References