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Título

Combined Central and Peripheral Demyelination: A Case Report

RESUMO

Case Report
Our patient is a forty-six year old woman, with a previous 9 year diagnosis of Multiple Sclerosis (MS) and using intramuscular interferon beta-1a, a disease-modifying therapy. She has been asymptomatic for 3 years, until she started to report numbness and weakness beginning at her feet and knees ascending to upper limbs symmetrically over the course of 48 hours. She denied a previous history of gastrointestinal or respiratory symptoms. At examination, she presented with grade 2 and 3 muscular strength on lower and upper limbs, respectively, associated with hypotonia and areflexia. We also observed a sensory level at T4 and Lhermitte sign.

Cerebrospinal fluid showed albumin-cytologic dissociation and nerve conduction studies (NCS) presented the findings of acute sensory-motor demyelinating polyradiculoneuropathy, characteristics of Guillain-Barrè Syndrome (GBS). Spinal cord Magnetic Resonance Imaging (MRI) presented a T2 high-intensity lesion from C1 to C5.

We opted to treat the patient with intravenous Methylprednisolone for five days. She started to improve significantly and was walking again 5 weeks after the first symptoms initiated.

Discussion
The co-occurence of MS and GBS is rare, with less than 20 cases described around the world until now.

Demyelinating diseases involving both central nervous system (CNS) and peripheral nervous system (PNS) are usually etiologically heterogeneous. Recently, autoantibodies against nodal and paranodal proteins were discovered (NF186, NF155, CNTN1 and CASPR1) and associated chronic and acute demyelinating diseases.

Combined Central and Peripheral Demyelination (CCPD) is defined fulfilling of the following criteria: a presentation of T2 high-signal intensity lesions in the brain, optic nerves, or spinal cord upon MRI, or abnormalities on visual-evoked potentials (VEPs), and conduction delay, conduction block, temporal dispersion or F-wave abnormalities according to NCS. This condition is highly sensitive to intravenous corticosteroids, with an outcome of symptom recovery of 83,3%.

Our patient presented both criteria to CNS and PNS involvement simultaneously and a significant response to treatment with intravenous Methylprednisolone.

Final Comments
We hope to shed light on the rare association between CNS and PNS demyelinating diseases and encourage a better investigation of ascending acute sensitive symptoms in patients with a previous diagnosis of MS.

Palavras Chave

Multiple Sclerosis, Guillain-Barré Syndrome, Demyelinating