Neuro-ophthalmological Complications of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) can lead to prominent nerve hypertrophy, which can mimic other forms of neuropathy radiologically. Neuro-ophthalmological complications can also occur in CIDP, either at presentation or chronically in the disorder. This can also cause diagnostic difficulties. We report three cases of neuro-ophthalmological complications of CIDP: two cases of papilloedema and one case of proptosis. In all three cases cranial nerve hypertrophy was present. CIDP should be considered in neuro-ophthalmological presentations associated with cranial/spinal nerve root hypertrophy.

Keywords: Chronic inflammatory demyelinating polyradiculoneuropathy; papilloedema; proptosis.

FIGURE 1

Case 1. Fundi (a) at presentation; (b) 3 months after immunosuppression was stopped; and (c) 3 months after immunosuppression was restarted. Note: Figures 1, 3, 6 and 7 of this article are available in colour online at www.informahealthcare.com/oph.

FIGURE 2

Case 1. Coronal T2- (a) and post-gadolinum fat-saturated T1- (b) weighted images demonstrating enlarged supra-orbital (black arrows) and infra-orbital (white arrows) nerves that also show gadolinium enhancement. Coronal T2- (c) and post-gadolinum fat-saturated T1- (d) weighted images demonstrating enlargement and gadolinium enhancement of both mandibular divisions of the trigeminal nerve (white arrows).

FIGURE 3

Case 1. Occipital nerve biopsy. (a) High-power haematoxylin and eosin–stained specimen demonstrating predominantly connective tissue surrounding nerve fascicles. The fascicles are permeated by mucopolysaccharide material, which accumulates beneath the perineurium. No inflammatory infiltrates are seen. (b) Alcian blue–stained specimen confirming the mucopolysaccharide deposition.

FIGURE 4.

Case 2. Axial post-gadolinum T1-weighted image demonstrating enhancement of the right oculomotor nerve (white arrow).

FIGURE 5

Case 3. Axial fat-saturated T2- (a) and post-gadolinum T1- (b) weighted images demonstrating ill-defined high T2 signal with gadolinium enhancement within the intra-conal fat, primarily surrounding both optic nerve sheaths (white arrows). Coronal fat-saturated T2- (c) and post-gadolinum T1- (d) weighted images demonstrating round masses with high T2 signal and intermediate T1 signal in both lacrimal areas (white arrows), with enlarged and gadolinium-enhancing infra-orbital (white arrowheads) and supra-orbital (black arrowheads) nerves.

FIGURE 6

Case 3. Intra-operative photographs. (a) A right-sided extended superior-lateral eyelid crease incision was performed, to allow access to the superior lesion and lateral optic nerve without removing the lateral orbital rim. (b) A translucent mass was encounter immediately deep to the superior rim (black arrow). A fine-needle aspiration was performed but intra-operative cytological analysis was unrevealing. Therefore an incisional biopsy was performed. (c) Images c-e are photographed using a standard rigid 30-degree endoscope. After incision, the mass partially “deflated” and is visible in the upper right hand corner of the picture as a deeper red structure. Now the underlying frontal branch of the trigeminal nerve is visible as a massively enlarged nerve, projecting deep towards the orbital apex (black arrow). An acutely branching trunk, just deep to the deflated cyst, projects to the bony orbital roof and probably represents either the supra-orbital or supra-trochlear nerve (black arrowhead). (d) A magnified view of this branch is seen in this figure (black arrowhead), with the deflated deeper red cystic structure in the upper right hand corner of the image. (e) A tangle of nerves embedded in orbital fat, surrounding the optic nerve was encountered (black arrow). A smaller nerve was grasped, and a segment excised for biopsy. The anatomical location of these nerves is consistent with and likely represents enlarged short posterior ciliary nerves.

FIGURE 7

Case 3. Right upper lacrimal nerve biopsy specimen showing severe loss of axons, a complete absence of normal myelinated axons, and clumps of proliferating Schwann cells with processes forming onion bulbs (black arrow). Again, there is a striking absence of inflammatory cells.