UNUSUAL NEUROLOGICAL MANIFESTATIONS FROM A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE REPORT

UNUSUAL NEUROLOGICAL MANIFESTATIONS FROM A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE REPORT

PARENTE, JOSE SAVIO MENEZES; MONTE, MATHEUS BARREIRA; IANNINI, NATALIA GOMES

Pôster:

It is known that Systemic Lupus Erythematosus (SLE) is defined by an autoimmune-mediated disease, one of the most known components in the world, including the central and peripheral nervous system. In fact, studies usually describe the neurological manifestations with a higher prevalence in those of central origin, however, it is known that the signs of peripheral involvement are of paramount importance in this disease, since they may arise before, during or even after the diagnosis of SLE. Cohorts indicate that the prevalence of manifestations of the peripheral nervous system varies between 2 and 10% of the cases, especially polyneuropathies and mononeuropathies, usually occurring between 25-45 years of age and affecting mainly women. This report aims to describe the unusual manifestations of SLE in an adolescent patient evidencing their neurological symptoms.

Case report

S.M.S, female, 16 years old, admitted to a hospital unit with a history of facial mimic loss, neuropathic pattern pain, and decreased strength and peripheral sensitivity. At the physical examination, it was emphasized arreflexia and strength grade 2 in the limbs, causing impairment in daily life activities. Complementary exams revealed a cytoprotein dissociation to the Lumbar Puncture, a discrete widening of the spinal canal at the T6-T11 level to the Neuro-Axis Magnetic Resonance and Electroneuromyography with primarily demyelinating sensory-motor polyneuropathy with discrete asymmetric secondary axonal degeneration , with active denervation and, at the time of examination, no signs of reinnervation. Chronic polyradiculopathy, cranial neuropathy and multiple mononeuropathy were confirmed and, during the investigation, positive for FAN, Anti-SM, Anticardiolipine, Anti-B2-GP1, Anti-P-Ribosomal and Anti-RNP autoantibodies, as well as levels of complement (C3, C4 and CH50), having a confirmed diagnosis of SLE using the SLICC-2012 criteria. Anti-DNA, Anti-RO, Anti-LA, Anti-Histone and ANCA were negative. Due to the patient's severity, initial therapy with immunoglobulin pulse therapy followed with pulse therapy with methylprednisolone and cyclophosphamide, treatment with Azathioprine, Hydroxychloroquine and Prednisone was also used, as well as Gabapentin for pain control and ASA as a prophylactic of thrombotic events. Patient presented gradual and slow improvement of the picture, was discharged to outpatient segment with force grade 3-4, walking with support.

Conclusion

We emphasize that neurological symptoms as the only clinical manifestation in adolescent patients with SLE. The rich autoantibody positivity was also essential for confirmation of the diagnosis, and it is important to consider the diagnosis of SLE in the presence of multiple neurological manifestations, even in the absence of other characteristic clinical symptoms.

It is known that Systemic Lupus Erythematosus (SLE) is defined by an autoimmune-mediated disease, one of the most known components in the world, including the central and peripheral nervous system. In fact, studies usually describe the neurological manifestations with a higher prevalence in those of central origin, however, it is known that the signs of peripheral involvement are of paramount importance in this disease, since they may arise before, during or even after the diagnosis of SLE. Cohorts indicate that the prevalence of manifestations of the peripheral nervous system varies between 2 and 10% of the cases, especially polyneuropathies and mononeuropathies, usually occurring between 25-45 years of age and affecting mainly women. This report aims to describe the unusual manifestations of SLE in an adolescent patient evidencing their neurological symptoms.

Case report

S.M.S, female, 16 years old, admitted to a hospital unit with a history of facial mimic loss, neuropathic pattern pain, and decreased strength and peripheral sensitivity. At the physical examination, it was emphasized arreflexia and strength grade 2 in the limbs, causing impairment in daily life activities. Complementary exams revealed a cytoprotein dissociation to the Lumbar Puncture, a discrete widening of the spinal canal at the T6-T11 level to the Neuro-Axis Magnetic Resonance and Electroneuromyography with primarily demyelinating sensory-motor polyneuropathy with discrete asymmetric secondary axonal degeneration , with active denervation and, at the time of examination, no signs of reinnervation. Chronic polyradiculopathy, cranial neuropathy and multiple mononeuropathy were confirmed and, during the investigation, positive for FAN, Anti-SM, Anticardiolipine, Anti-B2-GP1, Anti-P-Ribosomal and Anti-RNP autoantibodies, as well as levels of complement (C3, C4 and CH50), having a confirmed diagnosis of SLE using the SLICC-2012 criteria. Anti-DNA, Anti-RO, Anti-LA, Anti-Histone and ANCA were negative. Due to the patient's severity, initial therapy with immunoglobulin pulse therapy followed with pulse therapy with methylprednisolone and cyclophosphamide, treatment with Azathioprine, Hydroxychloroquine and Prednisone was also used, as well as Gabapentin for pain control and ASA as a prophylactic of thrombotic events. Patient presented gradual and slow improvement of the picture, was discharged to outpatient segment with force grade 3-4, walking with support.

Conclusion

We emphasize that neurological symptoms as the only clinical manifestation in adolescent patients with SLE. The rich autoantibody positivity was also essential for confirmation of the diagnosis, and it is important to consider the diagnosis of SLE in the presence of multiple neurological manifestations, even in the absence of other characteristic clinical symptoms.

Palavras-chave:

DOI: 10.5151/sbr2019-302

Referências bibliográficas

• [1]

Como citar:

PARENTE, JOSE SAVIO MENEZES; MONTE, MATHEUS BARREIRA; IANNINI, NATALIA GOMES; "UNUSUAL NEUROLOGICAL MANIFESTATIONS FROM A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE REPORT", p-302-302. In: Anais do 36º Congresso Brasileiro de Reumatologia. [ISBN 978-85-212-1892-0]. São Paulo: Blucher, 2019.
ISSN 23577282, DOI 10.5151/sbr2019-302