Neuroimaging markers of clinical progression in chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Background: One of the main goals of novel, noninvasive imaging techniques like high-resolution nerve ultrasound (HRUS) and corneal confocal microscopy (CCM) is the prediction of treatment response for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: A total of 17 patients with CIDP were examined prospectively at baseline and every 9 months over a period of 18 months using CCM to quantify corneal nerve degeneration markers and immune cell infiltration as well as HRUS to detect changes of the cross-sectional area (CSA) of the peripheral nerves. Additionally, skin biopsy of the distal and proximal leg as well as quantitative sensory testing were performed at the first follow-up visit. Results: A value of more than 30 total corneal cells/mm2 in CCM at baseline identified patients with clinical progression with a sensitivity/specificity of 100% in our cohort. Corneal nerve fiber density and length remained low and stable over the study period and intra-epidermal fiber density was markedly reduced in the majority of the patients. Furthermore, an increase in Bochum ultrasound score (BUS), which summarizes the CSA of the ulnar nerve in Guyons’ canal, the ulnar nerve in the upper arm, the radial nerve in the spiral groove and the sural nerve between the gastrocnemius muscle, and a maximum BUS of 4 at study initiation identified patients with disease progression (sensitivity 80%, specificity 88%). Conclusions: BUS and corneal total cell infiltration seem to represent early markers for clinical progression in CIDP, thus having the potential to identify at-risk patients and impact treatment decisions.

Correlates of polyneuropathy in Parkinson’s disease

“…HRUS is a recently developed imaging method which very precisely detects morphological alterations of peripheral nerves 21 . We applied HRUS technology for the in vivo examination in peripheral nerves in PD.…”

“…HRUS is a recently developed imaging method which very precisely detects morphological alterations of peripheral nerves. 21 We applied HRUS technology for the in vivo examination in peripheral nerves in PD. First, we examined entrapment sites where peripheral nerve pathology can be pronounced because of constantly higher mechanical burden.…”

Comprehensive approaches for diagnosis, monitoring and treatment of chronic inflammatory demyelinating polyneuropathy

“…The benefit of nerve ultrasound in the diagnosis of CIDP has been proven several times over the past 7 years, but it is still not established as standard diagnostic criterion. The measurement of cross-sectional area (CSA) in ultrasound correlates well to CSA detected by MRI and with the nerve T2-weighted signal intensity [26][27][28]. Morphological changes like swollen, hypoechogenic nerve and fascicles detected in ultrasound represent acute inflammation [29,30], while hyperechogenic nerves rather are supposed to occur in case of fibroid remodeling and axonal damage [29,31].…”

“…It is currently still unclear whether these parameters change dynamically in the course of the disease. Moreover, corneal nerve fibers are surrounded by immune cell populations, which can easily be quantified and change dynamically during disease course and might correlate with disease activity [28].…”

Corneal confocal microscopy differentiates inflammatory from diabetic neuropathy

“…More recently, we have shown an increase in corneal dendritic cells in individuals with CIDP and suggested that this may help to stratify CIDP subtypes, clinical course, and disease activity [ 23 ]. In a prospective study of 17 individuals with CIDP who were followed over 18 months, the presence of > 30 cells/mm 2 at baseline identified clinical progression with a sensitivity and specificity of 100% [ 24 ]. Moreover, in one person with anti-neurofascin-155 neuropathy, treatment with rituximab was associated with a reduction in the serum antibody titer with a clinical and electrophysiological improvement and reduction of corneal inflammatory cell infiltrates [ 25 ].…”