CIDP: Variants

by Roy Strowd, MD

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

    About the Lecture

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

    Included Quiz Questions

    1. Multifocal acquired demyelinating sensory and motor polyneuropathy
    2. Acute motor axonal neuropathy
    3. Miller Fisher Syndrome
    4. Guillain-Barré syndrome
    5. Diabetic polyneuropathy
    1. Distal acquired demyelinating symmetric - only distal involvement
    2. Multifocal acquired demyelinating sensory and motor neuropathy - symmetrical involvement
    3. Multifocal motor neuropathy - inflammatory markers typically seen in CSF
    4. CIDP - multifocal presentation
    5. Multifocal acquired demyelinating sensory and motor neuropathy - worsening with prednisone
    1. Multifocal motor neuropathy (MMN)
    2. Multifocal acquired demyelinating sensory and motor polyneuropathy (MADSAM)
    3. Chronic inflammatory demyelinating polyneuropathy (CIDP)
    4. Distal acquired demyelinating sensory neuropathy (DADS)

    Author of lecture CIDP: Variants

     Roy Strowd, MD

    Roy Strowd, MD

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