Short summary

Full title:
Variation I axonal nerve function and clinical neurological function in chronic inflammatory demyelinating polyneuropathy (CIDP) in relation to treatment with immunoglobulin intravenously (IVIG) and subcutaneously (SCIG).

Chronic inflammatory demyelinating polyneuropathy CIDP is a autoimmune disease characterized by demyelination of peripheral nerves resulting in reduction of nerve conduction velocity and conduction block causing paresis and sensory dysfunction in the extremities.

Immunoglobulin infusion is considered as first line treatment. However, the mechanism responsible for the effect is not completely understood.

We intend to study the mechanisms of immunoglobulin in CIDP patient.

Rationale

Background: Chronic inflammatory demyelinating polyneuropathy CIDP is characterized by paresis and sensory dysfunction in the extremities. The disease is caused by demyelination of peripheral nerves resulting in reduced nerve conduction velocity and conduction block, but dysfunction of the axonal membrane itself may also play a significant role. The mechanism responsible for the effect of the main current treatment, immunoglobulin infusion, is not completely understood and clinical observations suggest that it is not limited to modulation of the immune system, but may also involve direct changes of axonal membrane properties. 

Aim: We intend to study the clinical and neurophysiological effects of intravenous and subcutaneous immunoglobulins in CIDP patients with a specific focus on axonal function. 

Method: Nerve excitability studies will be used to study the axonal membrane properties during treatment. Data will be correlated to clinical testing during steady-state treatment and during discontinuation of treatment. 

Perspectives: The results may provide new insights into the pathophysiological mechanisms for nerve dysfunction and the mechanism of action of immunoglobulins in CIDP. 

Description of the cohort

Patients with CIDP and MGUS-associated polyneuropathy that are associated with the neuromuscular clinic at the department of neurology at University Hospital of Odense.

Inclusion criteria:

• Patients diagnosed with CIDP or MGUS-associated polyneuropathy
• Ongoing treatment with IVIG or SCIG
• Age between 18 and 80 years.

Exclusion criteria:

• Known or obvious vascular disease in lower extremities.
• Clinically significant affection of medulla spinalis, spinal roots or spinal stenosis.
• Significant systemic disease
• Patients with other diseases causing affected with of gait. 

Data and biological material

• Nerve excitability studies using Threshold Tracking
• Nerve conduction studies
• Clinical testing

Collaborating researchers and departments

Department of Neurology, Odense University Hospital

• Kanita Zubcevic, MD
• Clinical Lector Jakob Holbech, MD, PhD
• Thomas Krøigård, MD, PhD-stir
• Professor Søren Sindrup, MD, Dr med.