Acute-onset chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 antibodies and bilateral facial nerve enhancement.

Polineuropatía desmielinizante inflamatoria crónica de inicio agudo con anticuerpos anti-neurofascina-155 y realce bilateral del nervio facial.

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ID: 23010
2019
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Abstract
A 26-year-old female presented with acute onset distal paraparesis, upper limb tremor and bilateral facial palsy. Neurophysiology revealed a sensorimotor demyelinating polyneuropathy and lumbar puncture revealed an albuminocytologic dissociation. Neuroaxis MRI revealed bilateral facial nerve and cauda equina enhancement. Initially diagnosed as Guillain-Barré Syndrome, poor response to intravenous immunoglobulin, persistent deterioration, anti-neurofascin-155 antibodies and clinical response to steroid therapy led to diagnosis of acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP). CIDP patients with anti-neurofascin-155 antibodies are younger, with distal predominant weakness, tremor, and poor response to intravenous immunoglobulin. Up to 16% can present acutely, however bilateral facial weakness is rare.
Una mujer de 26 años presentó paraparesia distal de inicio agudo, temblor en los miembros superiores y parálisis facial bilateral. El estudio neurofisiológico reveló una polineuropatía desmielinizante sensitivomotora y la punción lumbar evidenció disociación albuminocitológica. La resonancia magnética del neuroeje mostró realce bilateral de los nervios faciales y de la cauda equina. Diagnosticada inicialmente como síndrome de Guillain-Barré, la escasa respuesta a la inmunoglobulina intravenosa, el deterioro persistente, la presencia de anticuerpos antineurofascina-155 y la respuesta clínica a la corticoterapia condujeron al diagnóstico de polineuropatía desmielinizante inflamatoria crónica (PDIC) de inicio agudo. Los pacientes con PDIC y anticuerpos antineurofascina-155 suelen ser más jóvenes y presentan debilidad de predominio distal, temblor y una respuesta deficiente a la inmunoglobulina intravenosa. Hasta un 16 % puede debutar de forma aguda; sin embargo, la debilidad facial bilateral es infrecuente.
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Authors André Caetano, Filipa Ladeira, Marco Fernandes, Pedro Pires, Elmira Medeiros
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Journal journal of neuroimmunology
Year 2019
DOI
S0165-5728(19)30298-X
URL
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