Case: 55-year-old Man Presents with Progressive Weakness of the Legs

00:02 Now let's turn to a case.

00:03 This is a 55-year-old who presents with a three month course of progressive weakness in the legs.

00:10 He first started to notice this at work three months ago and has become progressively weaker to the point that he is not able to work and has been using a cane for support.

00:18 He reports sensory loss in his feet with numbness and tingling in his toes.

00:23 Recently, he's also noticed symptoms in both hands as well.

00:26 He denies bulbar symptoms, and examination confirms four out of five weakness that is symmetric in both legs, except three out of five strength with dorsiflexion bilaterally.

00:37 There is subjective numbness in his feet to the toes, and deep tendon reflexes show diffuse areflexia.

00:45 So this case is very similar to our Guillain-Barre description in case.

00:49 However, this patient's symptoms began over three months.

00:52 He has a slowly progressive, gradually progressive process that has all of the features of a neuropathy.

00:59 There's symmetric weakness, sensory changes and diffuse areflexia.

01:05 This patient underwent workup that included a lumbar puncture showing no white blood cells but elevated CSF protein of 75 and normal CSF glucose.

01:14 So that's an inflammatory LP that's our albuminocytological dissociation.

01:19 Nerve conduction study showed neuropathic changes with significantly reduced conduction velocity, temporal dispersion, which is the demyelinating feature.

01:28 Absent if F-waves also at the myelinating feature, and reduced recruitment, which we see with neuropathies.

01:35 And all of this is consistent with a peripheral nerve demyelinating disorder.

01:40 So what's your diagnosis? Is this chronic inflammatory demyelinating polyneuropathy? Multifocal motor neuropathy? Acute intermittent demyelinating polyneuropathy? Or acute motor and sensory axonal polyneuropathy.

01:54 Well, this presentation isn't consistent with a multifocal pattern.

01:58 This is symmetric, so it's not multifocal motor neuropathy.

02:01 This is not the description of AIDP.

02:04 We see a lot of those features, however, this patient's symptoms continue to progress after eight weeks.

02:10 And that's inconsistent with AIDP and suggestive of CIDP.

02:15 This is not an axonal predominant process.

02:17 We saw a lot of features of dmyelination on the nerve conduction study.

02:21 And so that's inconsistent with acute motor and sensory axonal polyneuropathy.

02:26 And so this is the typical presentation of CIDP.

02:29 A chronic progressive onset, a condition over eight weeks localizing to the peripheral nerve with inflammatory changes on LP and demyelination on the nerve conduction study.

02:41 Let's talk a little bit more about CIDP.

02:44 What's the description? What a patient's describe? Well, it's a rare inflammatory disorder of the peripheral nerves? It is chronic and onset, with symptoms continuing to worsen after eight weeks and that's critically important in differentiating it from AIDP.

02:57 It's slowly progressive.

02:58 There's symmetric weakness, more than numbness.

03:01 So there is a sensory changes but the motor changes are often out of proportion to the sensory findings.

03:07 And areflexia is prominent.

03:08 This is an inflammatory polyradicular neuropathy, where we see diffuse areflexia.