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>