Case ReportBilateral facial nerve palsies secondary to chronic inflammatory demyelinating polyneuropathy following adalimumab treatment
Introduction
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare peripheral neuropathy of autoimmune origin, with a predicted prevalence between 1–9 in 100 000 worldwide [1]. By definition, CIDP follows a relapsing or progressive course over the course of at least 8 weeks. In its classic form, it presents as progressive, symmetrical loss of power affecting the proximal and distal limbs, with diffuse loss of reflexes as well as large fibre sensory loss distally [2]. Several variants based on patterns of demyelination have been described, such as pure motor, pure sensory and Lewis-Sumner syndrome; the heterogeneous presentation and highly variable clinical course bring controversies as to whether some of these variants are separate entities which share similar pathophysiology [1]. Evidence supports synergistic activation of cell-mediated and humoral immunity towards Schwann cell or myelin antigens that are not yet identified [1]. T-cell activation and migration across the blood nerve barrier, coupled with macrophage and complement complex mediated destruction of node of Ranvier and its surrounding region have been postulated [1].
Accurately diagnosing CIDP is important as the condition is readily treatable. Evidence based treatments for CIDP include corticosteroids, immunoglobulin or plasma exchange, and there is emerging data of other immune-modulatory therapies.
We present the case of a patient who developed CIDP in the setting of adalimumab treatment for Crohn’s disease, which manifested as progressive and relapsing bilateral facial nerve palsy with lagophthalmos and exposure keratopathy.
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Case report
A 37 year-old man with a 20 year history of Crohn’s disease was treated primarily with azathioprine and infliximab, until an infusion reaction prompted a change of treatment to adalimumab. He had a standard induction (160 mg followed by 80 mg at week two) followed by a fortnightly maintenance schedule (40 mg after week four).
8 months after the commencement of adalimumab, he developed acute neurological symptoms with impaired sensation in his hands and feet, which progressed to involve the face. Over
Discussion
Cranial nerve involvement occurs uncommonly in CIDP. There have been reports of facial nerve palsy, diplopia and ptosis due to ophthalmoplegia in 3–10% of case series [1], [3], but the course and prognosis of recovery is not well documented. Other neuro-ophthalmological complications described in CIDP included papilloedema secondary to raised intra-cranial pressure, demyelinating optic neuropathy and proptosis secondary to trigeminal and oculo-motor nerves hypertrophy from repeated cycles of
Conclusion
In conclusion, this case report highlighted bilateral facial nerve involvement as a feature of CIDP in its classic form. The prognosis is good for recovery of facial nerve function with discontinuation of anti-TNF-α therapy and concurrent use of steroid and immunoglobulin in this case.
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