Non-invasive Vagus Nerve Stimulation (nVNS) in Pediatric Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic immune-mediated disease
of the peripheral sensory motor nerves characterized by motor weakness, sensory loss, muscle
wasting and loss of motor ability. Treatment of CIDP involves chronic use of steroids,
intravenous immunoglobulin (IVIG) and rarely plasma exchange (PLEX). Outcome measures such as
patient reported motor disability scores, electrophysiology parameters, functional motor
strength testing are available to look for interval change. Despite above mentioned
treatments, the majority of patients have tremendous disease burden. There is a need for
alternative/adjunctive therapies that can decrease chronic inflammation effectively and
safely in pediatric CIDP patients.

Vagus nerve stimulation (VNS) has received significant scientific and clinical attention and
has been shown to effectively reduce systemic inflammation. Results from early clinical
trials for treatment of Rheumatoid Arthritis have demonstrated significant lifestyle benefits
and reduced symptoms in these patients. Similar benefits of VNS have been observed in Crohn's
patients. In these studies, patients are surgically implanted with a stimulator with
electrodes directly on the nerve. Preliminary results have demonstrated safety and efficacy
in patients that previously were unresponsive to traditional pharmacological therapies.
Unfortunately, surgical implantation of a device is difficult and costly.

Recent investigations have significantly increased our understanding of non-invasive vagus
nerve stimulation (nVNS). Compared to traditional implanted vagus nerve stimulation devices,
nVNS uses electrodes placed on the skin surface to stimulate the vagus nerve and has shown
promise in animal and human models to reduce chronic inflammation in multiple disease states.
By delivering electrical pulses at the skin surface above the vagus nerve, neural pathways
involved in regulating systemic inflammation are activated. Using a handheld device, patients
apply a brief duration of stimulation multiple times per day to achieve therapeutic benefit.
nVNS is currently FDA approved for clinical use in the treatment of migraines and cluster
headaches, with on-going clinical studies on epilepsy and systemic inflammation. Preliminary
published results have demonstrated significant therapeutic benefit to the patients with
minimal side-effects.

Ten study participants will be randomly assigned to one of two groups in the study: those
receiving the standard of care treatment plus electrical neuromuscular stimulation and those
receiving the standard of care treatment and devices that do not deliver electrical
stimulation (sham device). Participants will be requested to deliver nVNS two times per day,
at least five days per week in their home, for two years.

Inclusion Criteria:

- Diagnosis of CIDP based upon clinical/electrophysiological criteria

- Patient should be on treatment for CIDP including IVIG and Steroids

Exclusion Criteria:

- Patients will be excluded from the study if they have inherited polyneuropathy, such
as Charcot Tooth Marie disease.