Interesting article by Dr. Lewis

I’m having some issues with insomnia lately & decided tonight that I would Google CIDP & IVIG to see if any new info has popped up.

I found an article by Dr. Lewis about CIDP & I found it interesting. He is stating some important info about CIDP that I haven’t read in any other literature up until this point, specifically his info about the human leukocyte antigens Dw3, DRw3, A1, and B8 being found in people with CIDP.

Maybe those who are struggling to get a diagnosis can print this article to give to their physicians.

Here is the link:

http://emedicine.medscape.com/article/1172965-overview

And the text:
[B]Introduction[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL] [B]Background[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]The term chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been used to identify patients with a chronically progressive or relapsing symmetric sensorimotor disorder with cytoalbuminologic dissociation and interstitial and perivascular endoneurial infiltration by lymphocytes and macrophages. In many ways, CIDP can be considered the chronic equivalent of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), the most common form of Guillain-Barré syndrome (GBS).

A number of variants of CIDP have been described that have immune or inflammatory aspects and electrophysiologic and/or pathologic evidence of demyelination in common. No consensus exists on the best approach to the nomenclature of these disorders. CIDP is a major subset of chronic acquired demyelinating polyneuropathies (CADP). In this context, CIDP is considered when patients have a symmetric proximal and distal motor predominant disorder.

CIDP variants include patients with predominantly sensory symptoms, those with a distal symmetric disorder (DADS), those with multifocal sensorimotor neuropathy or sensorimotor mononeuropathy multiplex with prominent conduction block (also known as Lewis-Sumner neuropathy), and those with CIDP with associated CNS demyelination or with other systemic disorders.

The following disorders are considered distinct from CIDP because they have specific pathophysiologic features and respond to treatments differently than do patients with CIDP: Demyelinating neuropathies associated with immunoglobulin M (IgM) paraproteins, including those with anti–myelin-associated glycoprotein (MAG) antibodies; polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome; and multifocal motor neuropathy.
[B]Pathophysiology[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]Chronic inflammatory demyelinating polyradiculoneuropathy is presumed to occur because of immunologic antibody-mediated reaction along with interstitial and perivascular infiltration of the endoneurium with inflammatory T cells and macrophages. The consequence is a segmental demyelination of peripheral nerves.

Human leukocyte antigens Dw3, DRw3, A1, and B8 occur more frequently in patients with CIDP than in the healthy population.

Cytoalbuminologic dissociation is a characteristic finding in cerebrospinal fluid (CSF) pointing to nerve root involvement. Occasionally, CSF studies reveal mild lymphocytic pleocytosis and elevation of gamma globulin level, but this is observed most frequently in HIV-positive patients.
[B]Frequency[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL][B]International[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]CIDP is uncommon. The estimated prevalence of CIDP in populations from the UK, Australia, Italy, Norway, and Japan is 0.8-7.7 per 100,000. A 2009 study showed that the incidence and prevalence is variable depending on diagnostic criteria. In Rutland, UK on May 1, 2008, the prevalence of CIDP was 4.77/100,000 if the EFNS/PNS criteria were used but only 1.97 per 100,000 if the AAN criteria were used. Similarly the annual incidence was 0.7 per 100,000 using the EFNS criteria and 0.35 using the AAN criteria.[URL=”javascript:showcontent(‘active’,’references’);”]1[/URL]
[B]Mortality/Morbidity[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]Chronic inflammatory demyelinating polyradiculoneuropathy most commonly has an insidious onset and either chronic progressive or relapsing course. Occasionally, complete remissions occur. Quadriplegia, respiratory failure, and death have been described but are rare.
[B]Race[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]No racial predilection has been identified.
[B]Sex[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]Both sexes are affected. Of CADP variants, multifocal motor neuropathy has a male predominance of at least 2:1 based on a survey of the largest case series.
[B]Age[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]Chronic inflammatory demyelinating polyradiculoneuropathy may occur at any age, but it is more common in the fifth and sixth decades. Relapsing course is associated with younger age of patients (third and fourth decades). CIDP has been described in childhood.
[B]Clinical[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL] [B]History[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]Chronic inflammatory demyelinating polyradiculoneuropathy most frequently starts insidiously and evolves slowly, either in a slowly progressive (more than 60% of patients) or relapsing manner (approximately one third of patients), with partial or complete recovery between recurrences.

[LIST]
[*]Periods of worsening and improvement usually last weeks or months. Patients with a younger age of onset are said to have a higher frequency of relapsing course.
[*]Because of the insidious onset of CIDP, documenting precipitating illnesses or events is very difficult. However, preceding infection has been reported infrequently. Both respiratory and gastrointestinal infections have been cited, but no causative organism has been identified.
[*]Initial symptoms include weakness of the limbs, both proximal and distal, with proximal muscles affected at least as severely as distal.
[*]Sensory symptoms are common, such as tingling and numbness of hands and feet, but usually motor symptoms predominate.
[*]Only a small proportion of patients (approximately 16%) have a relatively acute or subacute onset of symptoms, with subsequent steadily progressive or fluctuating course.
[*]Children usually have a more precipitous onset of symptoms.
[*]Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made.
[*]Autonomic system dysfunction can occur; in such a case, the patient would complain of orthostatic dizziness, problems with bowel and bladder functions, and cardiac problems.[/LIST][B]Physical[/B]

[URL=”http://www.gbs-cidp.org/forums/”%5D [/URL]Pertinent findings are limited to the nervous system, except for cases of CADP associated with other diseases, as already mentioned. Depending on the associated systemic disorder, abnormalities on physical examination may be found in multiple organ systems. Patients should be examined in detail for signs of autoimmune, inflammatory, and neoplastic conditions.

[LIST]
[*]Cranial nerves[LIST]
[*]Cranial nerves may be involved, particularly CN VII, with paralysis of both upper and lower facial muscles. Diplopia can occur with the involvement of CN III, IV, or VI. Rarely, bulbar muscles (eg, palate, tongue) can be affected.
[*]Papilledema with pseudotumor cerebri syndrome (eg, headaches, transient visual obscurations, pulsatile tinnitus, visual field defects) are observed rarely in patients with CIDP and are due to a very high CSF protein level (usually >1000 mg/mL).[/LIST]
[*]Gait frequently is abnormal.
[*]Type of gait depends on location of weakness and degree of proprioceptive loss. With significant weakness in the lower extremities, patients may walk with steppage (ie, high elevation of both feet to compensate for weakness of foot dorsiflexors) or a slapping gait (ie, due to deficit of proprioception in the feet).
[*]Children are said to have more profound gait abnormalities than adults.
[*]Motor system[LIST]
[*]Usually relatively symmetric weakness of both proximal and distal muscles is present in upper and lower extremities.
[*]Muscle tone can be normal or decreased. Hypotonia, atrophy, and fasciculations may be present.[/LIST]
[*]Deep tendon reflexes: Reflexes characteristically are diminished or absent even in regions with only mild weakness.
[*]Sensory system[LIST]
[*]Large-diameter, heavily myelinated fibers are affected most severely, leading to proprioceptive and vibratory deficits.
[*]Loss or decrease of pain (ie, pinprick) and temperature sensations is less common.
[*]Stocking-glove distribution of sensory deficits is typical.
[*]Neuropathic pain in affected extremities may occur.[/LIST]
[*]Coordination: Patients may have sensory ataxia with positive Romberg sign due to damage to the large nerve fibers that convey proprioception.
[*]Pathological reflexes: Pathological reflexes (eg, Babinski, Chaddock, Oppenheim) usually are not observed in patients with CIDP.[/LIST][B]Continued….
[/B]