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Título

GANGLIONOPATHY AND LONGITUDINALLY EXTENSIVE MYELOPATHY ASSOCIATED WITH SJOGREN'S SYNDROME, IN A PATIENT WITH A BACKGROUND OF POST-VACCINATION POLYMYELITIS: A CASE REPORT

RESUMO

CASE PRESENTATION: In March 2020, arthralgias, perioral numbness, difficulty to speech, voice, swallowing, walking and forgetfulness, developed in a 44-years-old woman. November/2020, she had COVID-19, and trunk/limbs weakness, with abnormal hand movements appeared. In childhood, she presented post-vaccination poliomyelitis (atrophy in the right leg). June/2021, Neurological exam: Alertness, tachycardia, tachypnea, and hypotension. Performed simple commands, reduced fluency. Hypomimic face, dysphonia, dysarthria. Equine right foot and fasciculations in the right leg. Global hypotonia. Force graded MRC 2. Reflexes abolished. Indifferent plantar responses. Apalesthesia from C3. Normal superficial sensibility. Choreic and dystonic movements in face and upper limbs. Brain MRI-hypersignal in deep white matter, pons and gliosis in the right temporal pole. Cervical MRI-hypersignal region C3-T1(posterior). Electroneuromyography- lower motoneuron pathology with a sensory ganglionopathy in four limbs. Polysomnography revealed significant oxygen desaturation. FAN 1:320 and anti-Ro >240. Schirmer's test was positive to xerophthalmia. Anti-aquaporin-4 negative. Initially, methylprednisolone (1g/5days) was performed, without improvement, followed by immunoglobulin (0.4mg/Kg/5days), without major motor gains. Subsequently, she underwent cyclophosphamide (750mg/m2/day-3months), with interaction, swallowing and abnormal movements improved, but continued with force graded MRC 2. Then, Rituximab was realized (1000mg-15/15days), with recovery of cervical and trunk support. She is in homecare, with speech-physical therapies, using nighttime beeper, and waiting for Rituximabe (2nd cycle). Ten months hospitalized, there were complications, requiring ICU, due to dysautonomia, acquired tuberculosis, respiratory infections, which were circumvented. DISCUSSION: Sjögren's syndrome (SS) is a chronic autoimmune inflammatory disease with exocrine gland involvement and extraglandular disease features, affecting peripheral and central nervous systems. Autonomic neuropathy cause difficult control of the symptoms. Myelitis leading a diagnosis of NMO spectrum. The importance of distinguishing NMO and SS is important for different treatments and prognosis. FINAL COMMENTS: Neuro-Sjogren is a defiant illness to immunology, pathophysiology and possible complications inherent to this rare disease. Advances in disease-modifying therapies and early interventions can greatly improve the prognosis.

Palavras Chave

LONGITUDINALLY EXTENSIVE MYELOPATHY. GANGLIONOPATHY. SJOGREN'S SYNDROME.