By Marcello Cherchi, MD PhD
For patients
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a nerve disease that causes weakness and problems in touch sensation, which in turn can make patients feel unsteady. Rarely, CIDP can also affect the balance-related nerve between the ear and the brain, and if this is suspected, then your doctor may suggest checking some tests of inner ear balance function. If an inner ear problem is found then it is reasonable to try vestibular rehabilitation therapy (VRT).
For clinicians
Overview
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an idiopathic acquired immune-mediated process involving peripheral nerves and nerve roots. It usually manifests with symmetric muscle weakness of proximal and distal muscles, in a relapsing-remitting course. It is most common in the 6th – 8th decades. Though rare, CIDP can involve the vestibular nerves, resulting in vestibular weakness (unilateral or bilateral), which can be detected on tests of peripheral vestibular function. Treatment of CIDP is with immunotherapy. In CIDP patients found to have vestibular weakness, a trial of vestibular rehabilitation therapy (VRT) is reasonable.
Introduction
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an idiopathic acquired immune-mediated process involving peripheral nerves and nerve roots. It usually manifests with symmetric muscle weakness of proximal and distal muscles, in a relapsing-remitting course.
The most common type of CIDP manifests with symmetric sensorimotor polyneuropathy, whose motor symptoms (proximal and distal muscle weakness) are more pronounced than sensory symptoms.
Less common sub-types of CIDP include specific anatomical affectation (focal versus multifocal; diffuse versus distal involvement) and sensorimotor distribution (purely or predominantly motor, versus purely or predominantly sensory).
Epidemiology
A meta-analytical epidemiological study reported that CIDP has a pooled incidence of 0.33 per 100,000 person-years, and a pooled prevalence of 2.81 per 100,000 persons (Broers et al. 2019). CIDP can occur at any age, though is most common in the 6th – 8th decades (Aotsuka et al. 2024; Hafsteinsdottir and Olafsson 2016; Mahdi-Rogers and Hughes 2014).
Genetics
No clear genetic risk factors for CIDP have been identified.
Pathophysiological mechanism of disease
The etiology of CIDP is unclear, though studies show that both cellular and humoral immunologic components are involved.
Clinical presentation
The most common type of CIDP manifests with symmetric sensorimotor polyneuropathy, whose motor symptoms (proximal and distal muscle weakness) are more pronounced than sensory symptoms.
Involvement of cranial nerves, while possible, is somewhat unusual for CIDP, and should prompt consideration of other etiologies (such as Miller Fisher syndrome).
In the case of CIDP with bilateral vestibular dysfunction described by Frohman and colleagues (Frohman et al. 1996) the patient complained of oscillopsia.
Physical examination
In the case of CIDP with bilateral vestibular dysfunction described by Frohman and colleagues (Frohman et al. 1996) the patient was unable to perform tandem gait.
Ocular motor examination
Frohman and colleagues reported a case of CIDP with bilateral vestibular nerve involvement (Frohman et al. 1996). They documented low retinal gain, poor performance on dynamic visual acuity testing, and positive bedside head impulse tests.
Testing: audiologic
Magliulo and colleagues (Magliulo et al. 2018) studied a series of 20 CIDP patients. They reported:
- 4/20 (20%) had moderately severe hearing loss
- 6/20 (30%) had mild hearing loss
- 4/20 (20%) had slight hearing loss
- 6/20 (30%) had normal hearing.
Testing: vestibular
Magliulo and colleagues (Magliulo et al. 2018) studied a series of 20 CIDP patients. They reported:
- 11/20 (55%) showed absent or abnormal results for both ocular vestibular evoked myogenic potentials (oVEMP) and cervical vestibular evoked myogenic potentials (cVEMP).
- 7/20 (35%) showed abnormalities on bithermal caloric stimulation, while the remaining 13/20 (65%) had normal caloric testing results.
- 11/20 (55%) showed absent or abnormal results on both ocular vestibular evoked myogenic potentials (oVEMP) and cervical vestibular evoked myogenic potentials (cVEMP). Of these eleven patients:
- 5/11 cases were unilateral and 6/11 cases were bilateral.
- 4/11 (36%) exhibited absent ocular vestibular evoked myogenic potentials (oVEMP), of which 2 were unilateral and 2 were bilateral.
- 7/11 (64%) exhibited some ocular vestibular evoked myogenic potentials (oVEMP) response, but with delayed n10 latency.
- All 11 patients with abnormal cervical vestibular evoked myogenic potentials (cVEMP) exhibited delayed p13-n23 latencies and reduced p1-n1 amplitudes.
Akdal and colleagues (Akdal et al. 2023) studied video head impulse testing (vHIT) in a case series of 11 patients with GBS and reported that 8/11 (73%) had “some VOR [vestibulo-ocular reflex] impairment.” Of those 8 affected patients, 4 semicircular canals were affected in 1 patient (13%), 3 semicircular canals were affected in 2 patients (25%), 2 semicircular canals were affected in 4 patients (50%) and 1 semicircular canal were affected in 1 patient (13%).
In the case of CIDP with bilateral vestibular dysfunction described by Frohman and colleagues (Frohman et al. 1996), rotatory chair testing showed a “severe bilateral paretic disorder with reduction in VOR [vestibulo-ocular reflex] gain.”
Testing: other
CIDP exhibits a profile of abnormalities on electromyography with nerve conduction velocities that are typical for demyelinating neuropathies, including partial conduction block, slowing of conduction velocities, prolonged distal motor latencies, delayed or absent F waves, temporal dispersion and distance-dependent reduction of compound motor action potentials.
Cerebrospinal fluid (CSF) analysis in CIDP usually shows elevated protein without pleocytosis (similar to AIDP).
Imaging
Imaging is not necessary for the diagnosis, but MRI of the spine usually shows enhancement and/or hypertrophy of spinal nerve roots, hypertrophy of the cauda equina, the brachial plexus and lumbosacral plexus.
In the case of CIDP with bilateral vestibular dysfunction described by Frohman and colleagues (Frohman et al. 1996), MRI of the internal auditory canals without and with contrast demonstrated enhancement of the vestibular nerves.
Histopathology
CIDP typically has histopathologic findings of peripheral nerve segmental demyelination/remyelination, producing “onion bulb” appearance of whorled Schwann cells of affected nerves in cross-section.
Differential diagnosis
The differential diagnosis of CIDP is broad and includes:
- CIDP usually presents insidiously, but when it presents abruptly it can resemble Guillain-Barre syndrome (a type of acute inflammatory demyelinating polyradiculoneuropathy).
- Multifocal motor neuropathy (MMN).
- Distal acquired demyelinating symmetric neuropathy (DADS).
- Charcot-Marie-Tooth disease.
- Hereditary neuropathy with liability to pressure palsies (HNPP).
- Transthyretin familial amyloid polyneuropathy.
Treatment
Treatment with intravenous immunoglobulin, plasma exchange or glucocorticoids (methylprednisolone, prednisone) is reasonable. In refractory cases, immunomodulatory agents may be considered (cyclosporine, methotrexate, rituximab, cyclophosphamide).
When patients with CIDP complain of disequilibrium, this is usually attributable to the sensorimotor deficiencies (Frohman et al. 1996).
Vestibular weakness has rarely been described in CIDP, and there are no treatment trials regarding this specific deficit.
If a CIDP patient is being treated with immune therapies and exhibiting improvement in sensorimotor deficits, yet is still suffering from disequilibrium that appears out of proportion to any residual sensorimotor deficits, then it is medically reasonable to check peripheral vestibular function with cervical vestibular evoked myogenic potentials (cVEMP), ocular vestibular evoked myogenic potentials (oVEMP), video head impulse testing (vHIT), videonystagmography (VNG) and rotatory chair testing (RCT). If vestibular weakness is identified, then it would be medically reasonable to attempt a trial of vestibular rehabilitation therapy (VRT).
References
Akdal G, Koçoğlu K, Ala RT, Tanrıverdizade T, Özçelik P, Şengün İŞ (2023) Vestibular impairment in Guillain-Barré syndrome. Journal of the Peripheral Nervous System 28: 677-678. doi: https://doi.org/10.1111/jns.12593
Aotsuka Y, Misawa S, Suichi T, Shibuya K, Nakamura K, Kano H, Otani R, Morooka M, Ogushi M, Nagashima K, Sato Y, Kuriyama N, Kuwabara S (2024) Prevalence, Clinical Profiles, and Prognosis of CIDP in Japanese Nationwide Survey: Analyses of 1,257 Diagnosis-Confirmed Patients. Neurology 102: e209130. doi: 10.1212/WNL.0000000000209130
Broers MC, Bunschoten C, Nieboer D, Lingsma HF, Jacobs BC (2019) Incidence and Prevalence of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-Analysis. Neuroepidemiology 52: 161-172. doi: 10.1159/000494291
Frohman EM, Tusa R, Mark AS, Cornblath DR (1996) Vestibular dysfunction in chronic inflammatory demyelinating polyneuropathy. Ann Neurol 39: 529-35. doi: 10.1002/ana.410390415
Hafsteinsdottir B, Olafsson E (2016) Incidence and Natural History of Idiopathic Chronic Inflammatory Demyelinating Polyneuropathy: A Population-Based Study in Iceland. Eur Neurol 75: 263-8. doi: 10.1159/000445884
Magliulo G, Iannella G, Manno A, Libonati L, Onesti E, Vestri A, Fegatelli DA, Angeletti D, Pace A, Gulotta G, Gagliardi S, Inghilleri M (2018) Chronic inflammatory demyelinating polyneuropathy: evaluation of the vestibular system with cervical and ocular vestibular evoked myogenic potentials. Eur Arch Otorhinolaryngol 275: 1507-1512. doi: 10.1007/s00405-018-4981-9
Mahdi-Rogers M, Hughes RA (2014) Epidemiology of chronic inflammatory neuropathies in southeast England. Eur J Neurol 21: 28-33. doi: 10.1111/ene.12190
