CIDP: Presentation, Diagnosis, and Treatments

00:02 When we think about reflexes we want to differentiate distal areflexia from diffuse areflexia.

00:07 Distal areflexia which is reduced ankle jerks with intact or preserved knee jerk reflexes indicates a distal symmetric polyneuropathy like diabetes.

00:17 And this is common in the length-dependent polyneuropathies.

00:20 That's different from diffuse areflexia, which indicates proximal involvement of the nerves.

00:26 It suggests a polyradiculopathy, which we see with AIDP/CIDP, West Nile, Lyme and other associated conditions.

00:35 So let's talk about the typical clinical presentation of CIDP.

00:38 The typical pattern is symmetric. There is sensory involvement.

00:42 There's both a distal and proximal involvement of weakness.

00:46 Patients have early areflexia.

00:48 The disease course is progressing but it can relapse over time.

00:52 Cerebral spinal fluid protein is elevated.

00:54 This is an inflammatory process.

00:56 And we see that increased protein pointing us to inflammation.

01:00 We rarely see anti-ganglioside antibodies like we do see in Guillain Barre.

01:04 It can respond to IVIg.

01:06 But we also see a response to corticosteroids, which we don't tend to see as robustly in Guillain Barre.

01:13 CIDP and the chronic inflammatory polyneuropathies are an important group of neuromuscular disorders that present chronically and progressive for more than eight weeks.

01:23 And there are a few key features that we want to take home about these.

01:27 Their chronic and progressive often with stepwise progression of weakness over time.

01:32 When treated symptoms can improve but we can see relapses as a result of flares or exacerbations of the immune system.

01:39 Weakness is typically symmetric and characterized by proximal and distal muscle group involvement.

01:45 We do see sensory symptoms like numbness, tingling, gait imbalance.

01:49 At time painful paraesthesia, though those are not prominent.

01:52 Preceding viral infections are less common in CIDP compared to Guillain-Barre, and the CSF evaluation is the most sensitive measure showing elevated protein in 94% of cases.

02:04 How do we manage CIDP? Well, there's been a number of studies including randomized controlled trials that have confirmed the efficacy of corticosteroids, plasma exchange and intravenous immuneglobulin.

02:15 And so we use all three of those to manage these patients.

02:18 Prednisone therapy is the mainstay of treatment.

02:21 We typically start with a high dose and taper slowly over time to calm the immune system down.

02:27 The prednisone doses slowly taper typically five milligrams every two to three weeks or so.

02:31 And so you can tell this is a prolonged to taper in these patients.

02:35 IVIg when given is given at a dose of 2gm/kilogram over two to five days, and we can consider maintenance therapy.

02:44 For patients presenting with an acute relapse, we would often consider IVIg or plasmapheresis, followed by reinitiation, or reescalation of steroids to maintain long term immunomodulation.

02:55 We treat the patients typically for six months and then reevaluate to determine if further therapy is needed.

03:01 We'd like to avoid long term corticosteroid treatment and if necessary, corticosteroid sparing agents may be considered.

03:08 And then plasmapheresis is used in patients who are severely weak or who present with a fulminant course, or experience relapses on prednisone or on IVIg and particularly if they're unresponsive to those therapies.