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CENTRAL NERVOUS SYSTEM INVOLVEMENT IN SJOGREN'S SYNDROME: A DIAGNOSTIC CHALLENGE

RESUMO

CASE REPORT: A 26-YEAR-OLD WOMAN, PREVIOUSLY HEALTHY, STARTED IN APRIL 2021 WITH INTERMITTENT PULSATILE HEADACHE, WHICH MONTHS LATER WAS ASSOCIATED WITH PROGRESSIVE SENSORINEURAL HEARING LOSS ON THE RIGHT. AFTER 4 MONTHS, SHE PRESENTED EPISODES OF ROTATIONAL VERTIGO, WITH SUBACUTE ONSET, ATAXIA AND PARESTHESIA IN THE LEFT FOOT AND RIGHT HAND, WHEN SHE WAS ADMITTED FOR INVESTIGATION. MAGNETIC RESONANCE IMAGING (MRI) OF THE BRAIN WAS PERFORMED, WHICH REVEALED MULTIFOCAL LESIONS WITH POST-CONTRAST ENHANCEMENT, AFFECTING THE BASAL NUCLEUS, SUBCORTICAL WHITE MATTER, CEREBELLUM AND PERICEREBELLAR LEPTOMENINGES. SHE HAD SPONTANEOUS IMPROVEMENT OF SYMPTOMS, BEING DISCHARGED FOR FOLLOW-UP INVESTIGATION AS AN OUTPATIENT. HOWEVER, AFTER 02 MONTHS, SHE EVOLVED WITH PAIN IN THE TOPOGRAPHY OF THE RIGHT PAROTID GLAND, PROGRESSIVE LOW VISUAL ACUITY ON THE RIGHT (ACUITY: 20/200), WITH PAIN AND NO TEARS ON THE SAME EYE. SHE WAS HOSPITALIZED AGAIN, PERFORMING A NEW BRAIN MRI, WHICH SHOWED REGRESSION OF THE PREVIOUS LESIONS WITHOUT CONTRAST ENHANCEMENT. A TEAR GLAND BIOPSY REVEALED A TYPICAL PATTERN OF SJÖGREN'S SYNDROME. SHE STARTED PULSE THERAPY WITH METHYLPREDNISOLONE 1 G/DAY FOR 5 DAYS AND THE PATIENT REGRESSED FROM THE VISUAL DEFICIT.

DISCUSSION: SJÖGREN'S SYNDROME (SS) IS AN AUTOIMMUNE DISEASE THAT AFFECTS THE EXOCRINE GLANDS AND, WHEN PRIMARY, IS ASSOCIATED WITH EXTRAGLANDULAR MANIFESTATIONS. NEUROLOGICAL IMPAIRMENT OCCURS IN ABOUT 25% OF CASES, WITH PERIPHERAL MANIFESTATIONS BEING WELL DOCUMENTED IN UP TO 20% OF PATIENTS WITH SS, UNLIKE CENTRAL ALTERATIONS, WHICH ARE RARELY DESCRIBED. CENTRAL INVOLVEMENT USUALLY PRECEDES THE CLASSIC SYMPTOMS OF SICCA SYNDROME, IN ADDITION TO THE FACT THAT ANTIBODIES (ANTI-RO/ANTI-LA) ARE NOT ALTERED IN MOST CASES AT THE BEGINNING OF THE DISEASE AND THE CENTRAL SYMPTOMS CAN MANIFEST IN A RELAPSING-REMITTING MANNER WITH ASYMPTOMATIC PERIODS, DELAYING DIAGNOSIS AND TREATMENT.

CONCLUSION: THE CASE DEMONSTRATES THAT DESPITE THE PREDOMINANTLY CENTRAL PATTERN OF INVOLVEMENT, WITHOUT SYMPTOMS AND WITHOUT SEROLOGICAL MARKERS OF SS, THIS PATIENT SHOULD BE PERIODICALLY REASSESSED FOR THE POSSIBILITY OF BEING SS, DUE TO ITS DIVERSITY OF PRESENTATIONS AND SPECIAL EVOLUTIONARY CHARACTERISTICS, REQUIRING TIME AND HIGH LEVEL OF SUSPICION TO CLOSE THE DIAGNOSIS AND DIRECT THE TREATMENT IN TIME TO REDUCE MORBIDITIES AND CONTROL THE DISEASE.

Palavras Chave

OPTIC NEUROPATHY; SJOGREN'S SYNDROME; CENTRAL NERVOUS SYSTEM