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Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Neurology. 2003 Apr 1;60(8 Suppl 3):S8-15.                                                 
Research criteria for defining patients with CIDP.       Sander HW, Latov N.

Peripheral Neuropathy Center, Department of Neurology, Weill Medical College of Cornell University, New York, NY 10022, USA.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is one of several chronic acquired demyelinating neuropathies that are considered to be autoimmune diseases. The prevalence of these illnesses may be underestimated because of limitations in clinical, serologic, and electrophysiologic diagnostic criteria. An Ad Hoc Subcommittee of the American Academy of Neurology (AAN) proposed a set of diagnostic criteria for CIDP to be used for research purposes, and several other criteria followed. Of these, the AAN electrophysiologic criteria are the most restrictive and fit only a subset of patients with CIDP. Other criteria, including the Inflammatory Neuropathy Cause and Treatment (INCAT) clinical and electrophysiologic criteria and the Nicolas or Thaisetthawatkul electrophysiologic criteria, are more sensitive and can therefore identify a broader range of patients with CIDP for clinical trials. Regardless of which criteria are chosen for use in clinical trials, patients who fall outside of these criteria may also have CIDP and may benefit from treatment. Unfortunately, because of the lack of clarity with regard to diagnostic criteria for CIDP, many patients remain untreated. In addition, certain CIDP variants may also respond to treatment. These include sensory CIDP, multifocal motor neuropathy (MMN) with or without conduction block, multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, distal acquired demyelinating sensory (DADS) neuropathy, and multifocal acquired sensory and motor (MASAM) neuropathy. Therefore, although patients may not meet the diagnostic criteria for inclusion in clinical trials of CIDP, they may still benefit from current and future treatments used in CIDP.
: Rinsho Shinkeigaku. 2001 Dec;41(12):1210-3.  

[New trends in neuropathy practice: clinical approach to CIDP]

Baba M Department of Neurological Sciences, Hirosaki University School of Medicine.

Our recent study showed that the overall prevalence of CIDP was estimated as 2.2 per 100,000 population in Aomori Prefecture, in Northan Honshu of Japan. In our series of more than 80 cases with CIDP, a chronic acquired inflammatory demyelinating polyneuropathy, nearly 30% showed clear laterality of weakness, and electrophysiologic laterality or multifocality was apparent in almost all cases. Nearly 90% of patients were able to walk without walking aids or other assistance. Sixty% showed distal dominant muscular weakness. In 12 patients with age of onset under 15, pes cavus deformity was seen in 5. Two thirds complained numbness in the extremities during progressive phase. Four cases initially showed severe sensory ataxia associated with motor conduction block. It should be, thus, reminded that clinical spectrum of CIDP is enormously wide: chronic acquired demyelinating multiple mononeuropathy showing asymmetric involvement (Lewis-Summer syndrome) should be put on one side of the clinical presentation of CIDP. Multifocal motor neuropathy (MMN) is, on the other hand, an unique syndrome mimicking amyotrophic lateral sclerosis (ALS). There may be, however, true association syndrome of CIDP and ALS presenting both peripheral nerve demyelination and pyramidal sign with progressive bulbar involvement. Recently, several atypical varieties of CIDP showing only one-limb involvement, upper limb weakness rather than lower limb power loss, or proximal weakness, etc ... have been reported in the literature. To realize such clinical variations of chronic acquired demyelinating neuropathy is important for early diagnosis and commencement of treatment of CIDP. Clinical guideline for suspicion of CIDP could be useful for general physicians and neurologists unfamiliar to peripheral neuropathies

{From the above article it is clear that some patients who have ALS can be a variant of CIDP. Since CIDP is treatable with IVIG, thus all patients with ALS should consider getting a trial of IVIG as a diagnostic tool.}

Isolated unilateral adduction deficit and ptosis as the presenting features of chronic inflammatory demyelinating polyradiculoneuropathy.

Pieh C, Rossillion B, Heritier-Barras AC, Chofflon M, Landis T, Safran AB.

Ophthalmology Clinic, Department of Clinical Neurosciences, Geneva University Hospitals, Switzerland.

A patient with chronic inflammatory demyelinating polyneuropathy (CIDP) presented with an isolated unilateral adduction deficit and ptosis. Investigations were negative until the onset of limb weakness and fatigue 2 years later. At that time, electroneuromyography, cerebrospinal fluid examination, and magnetic resonance imaging confirmed the diagnosis of CIDP. Thus, ophthalmic signs can precede extremity and bulbar signs with a long latency in CIDP.

Intravenous immunoglobulin as first treatment in diabetics with concomitant distal symmetric axonal polyneuropathy and CIDP.

Cocito D, Ciaramitaro P, Isoardo G, Barbero P, Migliaretti G, Pipieri A, Proto G, Quadri R, Bergamasco B, Durelli L.

U. O. A. D. U. Neurologia I, Dipartimento di Neuroscienze, Universita di Torino, Via Cherasco 15, 10126 Turin, Italy.

The authors investigated the impact of IVIg as first line treatment of diabetic patients suffering from chronic inflammatory demyelinating polyneuropathy (CIDP) concomitant with distal symmetric axonal polyneuropathy. Nine patients with these clinical and electrophysiological features were treated with IVIg (0.4 g/Kg/day for 5 days). Clinical and electrophysiological evaluations were performed before and after treatment. Following IVIg treatment there was no significant improvement in clinical deficit. However, there was a significant and persistent decrease in the Rankin scale score and an improvement in the demyelinating feature on nerve conduction studies. Our findings suggest that IVIg had small but detectable beneficial effects on diabetic patients with CIDP and a high degree of axonal damage.

Arch Neurol. 2002 May;59(5):758-65.  
Demyelinating neuropathy in diabetes mellitus.

Sharma KR, Cross J, Farronay O, Ayyar DR, Shebert RT, Bradley WG.

Department of Neurology, University of Miami School of Medicine (M740), 1150 NW 14th St, Room 603, Miami, FL 33136, USA.

BACKGROUND: Recent studies have reported that patients with diabetes mellitus (DM) have a predisposition to develop chronic inflammatory demyelinating polyneuropathy (CIDP). OBJECTIVES: To determine whether patients with DM have a polyneuropathy fulfilling electrophysiologic criteria for CIDP, and whether CIDP is more frequent in patients with type 1 than in patients with type 2 DM. METHODS: We prospectively studied the frequency of electrophysiologic changes meeting the criteria for CIDP in patients with DM seen in our electrophysiology laboratory during a 51-month period (period 1). To evaluate the relationship between DM and CIDP, we prospectively determined during a 14-month period (period 2) the frequency of DM in patients seen in our electrophysiology laboratory with other neuromuscular diseases, and the frequency of idiopathic CIDP. RESULTS: During period 1, 120 patients with DM met the electrophysiologic criteria for CIDP (DM-CIDP). The most frequent clinical features of DM-CIDP were those of a predominantly large-fiber sensorimotor neuropathy, with recent motor deterioration and a moderately increased cerebrospinal fluid protein concentration. Twenty-six of the 120 patients were given intravenous immunoglobulin (400 mg/kg per day for 5 days), and 21 patients (80.8%) had significant improvement in the neurologic deficit at the end of 4 weeks of therapy. The DM-CIDP occurred equally in type 1 and type 2 DM. During period 2, 1127 patients were seen. Of these, 189 (16.8%) had DM with various neurologic disorders, including 32 patients (16.9%) with DM-CIDP. Among the remaining 938 patients without DM, 17 (1.8%) had idiopathic CIDP. The odds of occurrence of DM-CIDP was 11 times higher among diabetic than nondiabetic patients (P<.001). CONCLUSIONS: Demyelinating neuropathy meeting the electrophysiologic criteria for CIDP occurred in both types of DM, and its occurrence was significantly higher in diabetic than in nondiabetic patients.