A case of AQP4-IgG-negative neuromyelitis optica spectrum disorders with initial clinical manifestation of painful trigeminal neuropathy and literature review / 中国医师杂志

ABSTRACT

Objective:To explore the clinical manifestations and imaging of neuromyelitis optica spectrum disorders (NMOSD) with negative aquaporin-4 immunoglobulin G antibody (AQP4-IgG) with initial clinical manifestation of painful trigeminal neuropathy.Methods:The symptoms, signs, imaging examinations, diagnosis and treatment of a case of AQP4-IgG-negative NMOSD in neurology department of Affiliated Hospital of Southwest Medical University were reported and the relevant literatures were reviewed.Results:In the first episode, the patient started with right-sided facial pain with segmental paresthesia in the left limb. And In the second episode, the patient started with intractable hiccups and vomiting with weakness and paresthesia in the left limb. Magnetic resonance imaging of the skull and spinal cord revealed that the lesion involved the medulla oblongata and cervical medulla. AQP4 antibody, myelin oligodendrocytes glycoprotein (MOG) antibody, myelin basic protein (MBP) antibody and oligoclonal bands(OCB) of serum and cerebrospinal fluid were negative. The clinical diagnosis was AQP4-IgG-negative NMOSD. After treatments with hormonal anti-inflammatory and analgesic therapy, the patient′s facial pain, sensory abnormalities, hiccups, vomiting and limb weakness improved significantly.Conclusions:Painful trigeminal neuropathy may be the initial clinical manifestation of AQP4-IgG-negative NMOSD. NMOSD of presenting solely with painful trigeminal neuropathy is easy to be misdiagnosed and missed. Magnetic resonance examination is helpful for the early diagnosis of NMOSD.