Richard Lewis, MD, Department of Neurology at Cedars-Sinai Medical Center, discusses diagnosing CIDP as well as the revised guidelines used to manage this rare disease.
Transcription:
There have been guidelines created over the years. Because it’s a syndrome, it’s tricky. But the guidelines that we use for this study and pretty much all the studies before that, what was called the EFNS/PNS guidelines, which were developed in 2010.
EFNS Is a European Federation of Neurologic Societies and the PNS is the Peripheral Nerve Society. That was a group of about 20 people looked at all the literature up to date and came up with guidelines, which have to do with clinical, electrophysiological and supportive evidence.
The most common form of CIDP is called Typical CIDP. That is where patients have proximal and distal symmetric weakness. Their shoulders and hips, which are the proximal areas, are weak. The ankles and the hands are weak. They lose all their reflexes. There’s no genetic cause. When you see that pattern of proximal and distal symmetric weakness, there’s almost no other disease that does that if the progressions for over eight weeks.
There are atypical forms of what we call variants which must do distal variants, sensory variants, motor variants, multifocal variants. But the typical form is the most common and accounts for over half the cases. The support of evidence is that the spinal fluid protein is elevated without having many cells, which is very similar to what you see in Guillain-Barré syndrome. Then the other supportive evidence now is imaging studies showing inflammation of the nerve roots and whether the patient responds to treatment.
There are now new guidelines from 2021 that I was part of. This is now called the EAN/PNS guidelines, European Association of Neurology and the Peripheral Nerve Society guidelines, which was an update from the 2010 guidelines.
It goes into more detail of the variants. The whole point to some extent is to tighten up the diagnosis and not get half the patients being misdiagnosed, but hopefully less than 20%. The new guidelines have many more references, a whole bunch of ways to look at it. True guide to clinicians if they would use it.
Trying to get a diagnosis of syndromes can be very, very difficult. This is a relatively rare disease. Probably no more than eight patients per 100,000 population. It’s not easy to do this. We recommend patients to go to Centers of Excellence. The GBS/CIDP Foundation International Patient Advocacy group, has designated about 40 centers around the world, the Centers of Excellence. It certainly makes sense if the diagnosis isn’t clear or you don’t respond to the first treatment, to go ahead and get a second opinion in one of these centers, or a center that you know is highly respected for its understanding of this disease and other neuropathies.
To learn more about CIPD and other neurological conditions: https://checkrare.com/diseases/neurology-nervous-system-diseases/