Abstract General Information

Title

Typical demyelinating syndromes in atypical patients: case report of two intriguing cases

Case Report

Cases presentation:

Case 1: A 17-year-old healthy male presented with refractory vomiting. Laboratory tests were unremarkable. The neurological examination was unremarkable. Brain magnetic resonance (MRI): hypersignal on T2 sequences without gadolinium enhancement in area postrema. Serologies and antibodies against aquaporin-4 (antiAQP4) were collected. It was decided to start pulse therapy with methylprednisolone before the results, with significant recovery already on the second day of treatment. The HIV test was positive and confirmed. It was decided to continue steroid therapy for 5 days. The antiAQP4 - cell based assay (CBA) method - was negative.

Case 2: A 40-year-old woman started paresthesia in the cervical region. A few months later, started numbness and weakness in four limbs. On physical examination, it was observed: tetraparesis, hyperreflexia and tetrahypoesthesia. Serologies were negative and the MRI revealed: extensive spinal cord lesion in T2 sequences, holomedullary, extending from C2-C7 and D7-D9, with areas of contrast enhancement. She received 7 days of pulse therapy and underwent 5 sessions of plasma exchange, with significant improvement. During pulse therapy, there was a transient increase in liver enzymes. AntiAQP4 was positive. One month later, during screening to start continuous treatment, HBsAg became positive.

Discussion

These two emblematic cases demonstrate the nuances in the acute management of patients with typical demyelinating symptoms, which must be quickly and aggressively treated in order to avoid permanent sequelae, but which are sometimes triggered by infectious conditions and end up being challenges.

Final Comments

The first patient is being followed up by a neurologist and infectologist. Antiretroviral therapy was introduced afterwards, without adverse effects and soon will be retested for anti-AQP4

Patient two is being monitored by a neurologist and hepatologist and, in order not to worsen liver function through the use of anti-CD20, Ravulizumab is being considered.

Area

NMO, ADEM and CIS