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.

Optic, trigeminal, and facial neuropathy related to anti‐neurofascin 155 antibody

“…Pathology of hypertrophic intra-orbital nerves from some CIDP patients demonstrated onion bulb formation reflecting repeated demyelination and remyelination, infiltration of inflammatory cells, and abundant mucopolysaccharide deposition. 4,34 Although sural nerve biopsied from NF155 + CIDP patients showed only perineurial and endoneurial edema without onion bulb formation or inflammatory cell infiltration, 14,35,36 nothing is known about the pathology of the hypertrophic parts of somatic or cranial nerves. Anti-NF155 antibodies are assumed to invade the distal nerve terminals and nerve roots where the blood-nerve barrier is absent or loose, and to induce axo-glial detachment at the node of Ranvier.…”

Hydrocephalus due to marked enlargement of spinal roots in a patient with chronic inflammatory demyelinating polyradiculoneuropathy

“…Nine of the 13 reported cases improved after treatment with these immune therapies. Four patients who were resistant to immune therapies received VP shunt or lumboperitoneal shunt, and all four improved completely or partially following surgeries [7,8,10,11]. Our patient showed marked hypertrophic nerve roots, which could block CSF flow and compress the spinal cord, resulting in hydrocephalus.…”

“…Several reports have suggested that elevated CSF proteins are attributable to an impairment of CSF absorption, thereby causing hydrocephalus or papilledema [3,[6][7][8]; however, other studies have shown no correlation between CSF proteins and the severity of papilledema in Guillain-Barr e syndrome [4,9]. All four patients with papilledema or hydrocephalus who underwent an evaluation of their nerve roots showed hypertrophic nerves (Table 1) [7][8][9][10][11][12][13][14]. In our patient, the low opening pressure on CSF examination, variance between the protein levels in CSF obtained by lumbar puncture and lateral ventricle, and spinal cord compression due to swollen nerve roots on MRI examination strongly suggested that the CSF flow was blocked at the cervical levels, thereby inducing hydrocephalus.…”

“…The duration from onset of CIDP symptoms to the development of hydrocephalus or papilledema varies considerably, from 5 days to 14 years. Several reports have suggested that elevated CSF proteins are attributable to an impairment of CSF absorption, thereby causing hydrocephalus or papilledema [3,6–8]; however, other studies have shown no correlation between CSF proteins and the severity of papilledema in Guillain–Barré syndrome [4,9]. All four patients with papilledema or hydrocephalus who underwent an evaluation of their nerve roots showed hypertrophic nerves (Table 1) [7–14].…”

Papilledema in Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP): A Pediatric Case and Review of the Literature

“…This is supported by the observation that neuroophthalmologic complications (including papilledema) tend to occur more often in CIDP patients who have experienced a more prolonged disease course. 2 However, it is important to note that Guillain-Barre syndrome remains a distinct disorder with its own spectrum of severity that can include catastrophic complications, including death.…”