AIDP: Treatment and Variants

00:01 After an attack of Guillain-Barre there's a number of potential complications that can happen.

00:06 We like to see the early treatment in with early initiation of treatment.

00:10 We want to reduce the nadir of the illness and see spontaneous recovery over weeks, months, or sometimes years.

00:16 Occasionally, symptoms come back and there are a couple of considerations we need to have.

00:20 The first is recurrent AIDP.

00:22 This can happen after an initial attack of Guillain-Barre, patients can have recurrence of symptoms, but this is rare.

00:30 It occurs in less than 5% of cases.

00:33 It's characterized by recurrent weakness and numbness, after the initial attack.

00:37 Classically Guillain-Barre is a monophasic illness.

00:41 There's rapid onset early sub-acute onset of symptoms, it nadirs within 14 days, and symptoms spontaneously improve and do not recur.

00:51 Another consideration and a patient who develops worsening of symptoms early after their acute attack is a treatment related fluctuation.

01:00 This occurs as a result of continued immune destruction of nerves, which initially improves with immunomodulatory therapy, but then continues beyond the half-life of the treatment.

01:11 So patients are developing symptoms, we give them treatment.

01:15 That treatment works temporarily, but the disease is continuing to progress.

01:19 And so as the treatment stops and wears off, we see the continued progression of the disease and this is called a treatment related fluctuation.

01:26 Patients in this situation present with recurrent symptoms several weeks after the initial treatment of Guillain-Barre.

01:33 They have a nadir, a plateau, and then ultimately what we like to see recovery.

01:39 And then lastly, we may have the diagnosis wrong.

01:42 Patients that have continued worsening of symptoms.

01:45 More weakness after their initial presentation of Guillain-Barre.

01:48 May actually not have acute inflammatory demyelinating polyneuropathy, but chronic inflammatory demyelinating polyneuropathy.

01:56 A CIDP is a chronic onset condition, but occasionally it will present with a more fulminant acute presentation.

02:04 The disease does not nadir at four weeks like Guillain-Barre, but continues by definition to persist.

02:10 Symptoms persist beyond eight weeks.

02:13 This tends to be responsive to corticosteroids, unlike Guillain-Barre, and we do initiate corticosteroid treatment, but also use IVIg and plasmapheresis to manage these patients.

02:26 Now, let's talk about some of the variants of typical AIDP.

02:30 There are both axonal variants, as well as other variants.

02:34 The four that I would like for you to remember are acute, motor, axonal neuropathy, and that is exactly what it says.

02:41 It's acute and onset just like Guillain-Barre, but it's motor predominant.

02:45 There's no sensory symptoms, and it's an axonal neuropathy.

02:48 We don't see demyelinating features, we see axonal features on our nerve conduction.

02:53 The second is acute motor and sensory axonal neuropathy.

02:57 So this is very similar. It's acute and onset, but there's both motor and sensory deficits, and it's also an axonal neuropathy, and we don't see demyelinating features.

03:07 The important reason to call out these axonal variants of classic Guillain-Barre is that they have a worse prognosis.

03:14 Patients may have incomplete recovery and prolonged weakness and lack of improvement back to baseline.

03:21 There are also some other variants.

03:23 There is an autonomic variant that has prominent autonomic dysfunction, and dysautonomia, and then Miller-Fisher syndrome, which is a unique bulbar form of this disease.

03:33 Patients present with ophthalmoplegia, ataxia, and areflexia.

03:37 And that combination of those three symptoms that triad should point squarely to a diagnosis of Miller-Fisher syndrome.

03:44 This is important to recognize because prognosis is more favorable.

03:49 And some patients with Miller-Fisher syndrome will not even require treatment not IVIg or plasmapheresis, with a mild nadir and gradual or rapid improvement of symptoms over time.