Other Chronic Conditions
Paraproteinaemic Demyelinating Neuropathy (PDN)
Sometimes described as:
- chronic demyelinating neuropathy associated with a benign paraprotein
- CIDP associated with paraprotein
- CIDP with paraproteinaemia
Antibody-producing bone marrow cells go out of control and produce large numbers of the same antibody. The antibody (or immunoglobulin) sometimes damages nerve fibres causing a peripheral neuropathy. Some doctors regard the clinical, electrophysiological and pathological features of the demyelinating paraproteinaemic neuropathies and of CIDP as closely similar and almost indistinguishable.
These neuropathies are usually late-onset in terms of age and are mixed motor and sensory, although the severity of sensory loss tends to be greater compared with CIDP. There is usually more pain but less severe weakness and impairment. Most patients respond to corticosteroids, cytotoxic drugs, or plasma exchange.
Multifocal Motor Neuropathy (MMN) or MMN with Conduction Block (MMNCB)
Sometimes thought of as a rare variant of CIDP. However, there are differences that are more prominent than the similarities. MMN patients commonly have asymmetric weakness of the distal (far) muscles, while in CIDP, proximal (near) symmetric weakness is more common. The remitting and relapsing course that may occur in CIDP is uncommon in MMN. Patients with MMN rarely have significant sensory symptoms, unlike CIDP. Increased protein level in the cerebrospinal fluid of MMN patients is rare. Treatment with IVIg is usually effective.
MADSAM also known as Lewis-Sumner syndrome
Multifocal acquired demyelinating sensory and motor neuropathy is a chronic condition with similarities to multifocal motor neuropathy but with enough differences, especially in treatment, to have acquired its own definition. Some report it to be an asymmetrical variant of CIDP. MMN and MADSAM respond to IVIg.
Chronic axonal neuropathies are common, particularly as a result of diabetes or alcoholism. However, the medical literature does report cases of immune-mediated chronic axonal neuropathy though there are suggestions that this is a secondary result of myelin damage that ultimately appears to be the primary cause of the condition.
Sub-acute Inflammatory Demyelinating Poly(radiculo)neuropathy (SIDP) GBS is defined when the nadir (worst point) occurs within four weeks of first symptoms, and CIDP is defined when the nadir comes after at least eight weeks. An illness peaking after four weeks but before eight weeks may be called subacute and will be treated as CIDP or GBS depending on which it best resembles.
Chronic Idiopathic Axonal Neuropathy
If no cause for the peripheral neuropathy can be discovered, doctors call it ‘idiopathic’ that means ‘of its own cause’. This label probably covers a number of different causes which future research may uncover. With rare exceptions, chronic idiopathic axonal neuropathy occurs in older people, only worsens very slowly (and sometimes remains stationary), and does not become disabling. It is most commonly a sensory neuropathy causing numbness, tingling and discomfort in the feet that may gradually spread up the shins.
People may become slightly unsteady, and weakness of the ankles may develop. The amount of pain is variable. Some people have very little pain but more weakness. Others have little weakness but more pain.
Sensory Neuronopathy
Also known as sensory ganglionopathy, dorsal root ganglionopathy or sensory ganglionopathy, this is a rare subgroup of peripheral nervous system diseases with specific characteristics, such as the primary and selective destruction of the dorsal root ganglia (DRG) neuron in the spinal cord and the trigeminal ganglia neuron in the skull.
It has a typical clinical presentation, with sensory deficits that are not dependent on length and patients often report a lack of coordination of muscle movements. There are several proposed mechanisms for the pathophysiology of the condition. The diagnosis is guided by the presenting symptoms and confirmed with diagnostic tests to differentiate from other related conditions.
Treatment of sensory ganglionopathy is often difficult and patients often stabilize but fail to improve significantly. There are reports of improvement in immune-mediated and paraneoplastic sensory ganglionopathy following treatment with intravenous immunoglobulin. Quickly treating the underlying cause, such as the underlying neoplasia, may also be helpful.
Related Conditions
People that suffer from sensory neuronopathy are more likely to be affected by other autoimmune diseases. A differential diagnosis is important to distinguish sensory neuronopathy from other related conditions, such as sensory and ataxic neuropathy. These conditions may include Sjögren’s Syndrome, autoimmune hepatitis and celiac disease.
POEMS Syndrome
POEMS is a rare condition caused by the body producing abnormal plasma cells (a type of blood cell which produces antibodies to fight off infections). This blood disorder affects multiple organs in the body. It is named after the five common features of the syndrome described below.
Polyneuropathy – nerve damage leading to weakness, numbness and pain in the arms and legs
Organomegaly – an enlarged spleen and/or liver
Endocrinopathy – hormonal problems
M-protein or Monoclonal plasma cell disorder – an overproduction of abnormal plasma cells which lead to other multi system effects
Skin changes – darkening to skin, red spots on the body, hair growth
For more information, please see our POEMS fact sheet click here
Welcome to our CIDP Information Hub.
Here we breakdown what is happening to you or a loved in simple but proper terms. Our information is sourced from our Medical Advisory Board, medical texts, and recognised support providers.
If you have any questions after reading this that you feel haven’t been answered. Please get in touch with us, we will strive to point you in the right direction.
What is CIDP?
We discuss the basics of GBS – what it is, the symptoms, and information on Acute onset CIDP (A-CIDP)
How is CIDP Treated?
We cover Immunoglobulin, Plasma Exchange, Corticosteroids, and is there a “cure”?
What Family & Friends Can Do To Help if Someone is in Hospital?
We cover communication, Mental Stimulation, Financial Worries, Comfort, Coming off a Ventilator, and Staying Positive
What is the advice surrounding vaccinations and CIDP?
Containing information on vaccinations via our Medical Advisory Board and sourced journals.
Mental Health & Wellbeing, Relationships, and Carer Support
Contains information on how to care for your mental health. We also discuss relationships, before a section on looking after yourself as a carer
What Are The Causes of CIDP?
How will I get diagnosed by my Doctor, Nerve Tests, and Lumbar Punctures
Returning to Work
When someone is ready to return to work after an absence, the employer should have a procedure they follow.
You can check your workplace’s absence policy for this. Your workplace might have a policy for meeting with employees after absences.