CIDP: Variants

Name: CIDP: Variants
Uploaded: 2022-05-05
Duration: 6 min 8 s
Description: The lecture CIDP: Variants by Roy Strowd, MD is from the course Chronic Inflammatory Demyelinating Polyneuropathy (CIDP).

by Roy Strowd, MD

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00:01 There are a number of variants of CIDPs. So there's similar processes but affect patients differently that I'd like you to understand. We see motor predominant variants and sensory predominant variants, and they can be symmetric or multifocal. CIDP is a motor predominant condition. There's prominent weakness with minimal sensory changes, and it is symmetric. In contrast, DADS or distal acquired demyelinating sensory neuropathy is a sensory involving neuropathy that is also symmetric. So patients report distal sensory symptoms really with a paucity of weakness. In contrast to the symmetric disorders, the multifocal disorders are multifocal motor neuropathy, which is exactly what the name says. It's a multifocal, motor predominant neuropathy. Patients present with multiple areas of nerve dysfunction in different myotomal patterns, and is an inflammatory disorder. And that contrasts with MADSAM or multifocal acquired demyelinating sensory and motor neuropathy which is multifocal, with sensory and motor involvement. And all of these are inflammatory polyneuropathies. Let's talk through some of the different features of each of these CIDP variants. Multifocal motor neuropathy is asymmetric, there's no sensory symptoms. It is a motor neuropathy. We can see proximal and distal involvement. Deep tendon reflexes are induced but often in a multifocal pattern. The disease course is slowly progressive often over years. It is an inflammatory disorder. So we see elevated CSF protein. And occasionally with antiganglioside antibodies, there is a response to IVIg but we don't see a response to steroids. In fact, this is one of the only inflammatory disorders that can present with worsening of symptoms with steroid initiation, which is very important to remember. Next, motor neuron disease. This is often asymmetrical. We don't see sensory involvement. We can see proximal and distal involvement. Deep tendon reflexes can be increased in motor neuron disease like Lou Gehrig disease or a Amyotrophic lateral sclerosis where there's peripheral and central motor neuron disorders. This is often more rapidly progressive than multifocal motor neuropathy. We can see elevated protein but that's uncommon. 10% of patients capacity will develop antiganglioside antibodies and there's no response to IVIg or steroids. CIDP in contrast is symmetrical. We do see sensory involvement. Patients are diffusely areflexic with a slightly more progressive course that can be relapsing and patients respond to IVIg and steroids. And then finally MADSAM, or multifocal acquired demyelinating sensory and motor neuropathy. It's multifocal. So there's an asymmetric pattern. We do see sensory symptoms, including pain, reflexes are reduced. There's progressive and relapsing course that we can see with that protein is often elevated, and this can respond to IVIg and steroids. So let's talk through some of these CIDP variants in slightly greater detail. DADS is distal acquired demyelinating symmetric neuropathy. It is a distal sensory only variant of CIDP. It tends to be symmetric like CIDP, with absent deep tendon reflexes. Motor symptoms are not present or prominent at all. And there's a predominance of sensory neuropathy. Some of the key features. This is distal as opposed to the proximal involvement in the other conditions. Nerve conduction studies shows prolonged distal latencies often with conduction block because it's inflammatory, and our LP does show increased protein. The treatment here, we see variable response to corticosteroids and can use IVIg and plasmapheresis as well as steroid sparing agents in this inflammatory neuropathy. Now, let's talk about MADSAM, or multifocal acquired demyelinating sensory and motor polyneuropathy. This is a multifocal sensory and motor polyneuropathy. It's chronic and progressive with multiple nerves that are involved in an asymmetric pattern. There's motor involvement and sensory involvement with weakness, reduced deep tendon reflexes and sensory abnormalities. The typical description of this condition it's immune mediated polyneuropathy. It presents often with a subacute onset course and then it can be progressive and relapsing over time. And importantly, we see length-dependent sensory neuropathy and areflexia. In terms of diagnosis, our nerve conduction study shows asymmetry, which is very important and differentiates it from CIDP. The LP shows inflammation with elevated protein, and there's variable response to corticosteroids, IVIg, and plasmapheresis. Then multifocal motor neuropathy. This is a multifocal motor predominant neuropathy that follows a chronic progressive course. It is asymmetric given the multifocal pattern and there is exclusively motor nerve involvement. This is degenerative more than inflammatory. So we don't see all of those inflammatory findings and protein is typically not elevated in the spinal tap. Other features include asymmetric neuropathy WITHOUT sensory changes. We look on our nerve conduction study for conduction block. Motor abnormalities with normal snaps, normal sensory responses. And typically a treatment is focused on IVIg and cyclophosphamide as steroids can worsen this condition. So let's summarize some of the treatments we use for CIDP and its variant. For CIDP, we think about prednisone and we treat acute exacerbations with IVIg and plasmapheresis. For DADS, we use prednisone and also use IVIg and plasmapheresis, particularly for fulminant presentations, or worsening For multifocal motor neuropathy, this worsens with prednisone. And so we really focus our treatment efforts around IVIg and plasmapheresis. And MADSAM is treated with prednisone and IVIg and plasmapheresis in selected cases.

About the Lecture

The lecture CIDP: Variants by Roy Strowd, MD is from the course Chronic Inflammatory Demyelinating Polyneuropathy (CIDP).

Included Quiz Questions

Which of the following is a variant of chronic inflammatory demyelinating polyneuropathy?

Multifocal acquired demyelinating sensory and motor polyneuropathy
Acute motor axonal neuropathy
Miller Fisher Syndrome
Guillain-Barré syndrome
Diabetic polyneuropathy

Which of the following chronic inflammatory demyelinating polyneuropathy (CIDP) variants and its clinical features is paired correctly?

Distal acquired demyelinating symmetric - only distal involvement
Multifocal acquired demyelinating sensory and motor neuropathy - symmetrical involvement
Multifocal motor neuropathy - inflammatory markers typically seen in CSF
CIDP - multifocal presentation
Multifocal acquired demyelinating sensory and motor neuropathy - worsening with prednisone

A course of steroids may worsen which of the following?

Multifocal motor neuropathy (MMN)
Multifocal acquired demyelinating sensory and motor polyneuropathy (MADSAM)
Chronic inflammatory demyelinating polyneuropathy (CIDP)
Distal acquired demyelinating sensory neuropathy (DADS)

Author of lecture CIDP: Variants

Roy Strowd, MD

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