Chronic inflammatory demyelinating polyneuropathy - Revision history

Danbot: Add verified PubMed references (PMIDs 34327760, 34085743)

2026-03-22T10:59:08Z

Add verified PubMed references (PMIDs 34327760, 34085743)

← Older revision		Revision as of 10:59, 22 March 2026
Line 1:		Line 1:
	==Background==		==Background==
	*Prevalence of 1 to 2 per 100,000 adults		*Prevalence of 1 to 2 per 100,000 adults<ref>Van den Bergh PYK, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. Eur J Neurol. 2021 Nov;28(11):3556-3583. PMID 34327760</ref>
	*Autoimmune condition leading to cellular and humoral immune response to proteins on myelin sheath likely due to molecular mimicry from infectious process		*Autoimmune condition leading to cellular and humoral immune response to proteins on myelin sheath likely due to molecular mimicry from infectious process
	**Has been associated with [[melanoma]]		**Has been associated with [[melanoma]]<ref>Van den Bergh PYK, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. J Peripher Nerv Syst. 2021 Sep;26(3):242-268. PMID 34085743</ref>
	*Acute variant is [[Guillain-Barre syndrome]]		*Acute variant is [[Guillain-Barre syndrome]]
	** GBS is self limited whereas CIPD is progressive for more than two months		** GBS is self limited whereas CIPD is progressive for more than two months

ClaireLewis at 01:15, 22 October 2020

2020-10-22T01:15:45Z

← Older revision		Revision as of 01:15, 22 October 2020
Line 7:		Line 7:

	==Clinical Features==		==Clinical Features==
	*Symmetrical proximal and distal muscle weakness that is progressive for GREATER than two months		*Symmetrical proximal and distal muscle [[weakness]] that is progressive for GREATER than two months
	*Muscle weakness more predominant feature than sensory symptoms		**Muscle weakness more predominant feature than sensory symptoms
	*Reduced or absent deep tendon reflexes in upper and lower extremities		*Reduced or absent deep tendon [[reflexes]] in upper and lower extremities
	*Elevated CSF protein without pleocytosis		*Elevated CSF protein without pleocytosis
	*Electrodiagnostic evidence of a demyelinating neuropathy		*Electrodiagnostic evidence of a demyelinating neuropathy
Line 34:		Line 34:

	===Workup===		===Workup===
	*Lumbar puncture can be performed inpatient		*[[Lumbar puncture]] can be performed inpatient
	**Will show elevated protein without pleocytosis		**Will show elevated protein without pleocytosis
	*Nerve biopsy will show demyelination and demyelination often with signs of inflammation		*Nerve biopsy will show demyelination and demyelination often with signs of inflammation
	**Often unnecessary and of low yield due to proximal nerve involvement, but still performed if diagnosis uncertain		**Often unnecessary and of low yield due to proximal nerve involvement, but still performed if diagnosis uncertain
	*MRI may show gadolinium enhancement and enlargement of proximal nerves or roots		*[[MRI]] may show gadolinium enhancement and enlargement of proximal nerves or roots
	*Electrophysiological diagnostic procedures with show evidence of demyelination		*Electrophysiological diagnostic procedures with show evidence of demyelination
	{\| {{table}}		{\| {{table}}
Line 60:		Line 60:

	==Management==		==Management==
	*Requires ~~Neurology~~ consult		*Requires neurology consult
	*Therapy is tailored to each patient, no difference in efficacy between the three treatment options		*Therapy is tailored to each patient, no difference in efficacy between the three treatment options
	**IVIG		**[[IVIG]]
	**Plasma exchange		**[[Plasma exchange]]
	**Corticosteroids		**[[Corticosteroids]]
	*May be placed on azathioprine, cyclophosphamide, or cyclosporine to prevent relapse		**May be placed on [[azathioprine]], [[cyclophosphamide]], or [[cyclosporine]] to prevent relapse

	==Disposition==		==Disposition==
Line 80:		Line 80:

	<references/>		<references/>
			[[Category:Neurology]]

Mtjordan91: /* Evaluation */

2020-09-22T22:01:03Z

Evaluation

← Older revision		Revision as of 22:01, 22 September 2020
Line 24:		Line 24:
	*Closest differential is [[Guillain-Barre syndrome]]		*Closest differential is [[Guillain-Barre syndrome]]
	**The onset of GBS is usually easily identified, while the precise onset of CIDP is typically less clear		**The onset of GBS is usually easily identified, while the precise onset of CIDP is typically less clear
	Antecedent events are more frequent with GBS than in CIDP		**Antecedent events are more frequent with GBS than in CIDP
	**~70% of GBS cases are preceded by an infectious illness, vaccination, or surgery by three to four weeks prior to the onset of clinical symptoms.		**~70% of GBS cases are preceded by an infectious illness, vaccination, or surgery by three to four weeks prior to the onset of clinical symptoms.
	**By contrast, most studies have found an antecedent event prior to CIDP in no more than 30% of patients.		**By contrast, most studies have found an antecedent event prior to CIDP in no more than 30% of patients.

Mtjordan91: /* Evaluation */

2020-09-22T22:00:47Z

Evaluation

@@ Line 22: / Line 22: @@
 *3 separate criteria can be used to diagnose CIPD
 **Only the American Academy of Neurology requires a lumbar puncture and nerve-biopsy specimen
 ===Workup===
 *Lumbar puncture can be performed inpatient

Mtjordan91: /* Workup */

2020-09-22T21:58:14Z

Workup

← Older revision		Revision as of 21:58, 22 September 2020
Line 43:		Line 43:
	\| Electrodiagnostic Criteria\|\|Any 3 of the following 4 criteria: partial conduction block of ≥1 motor nerve, reduced conduction velocity of ≥2 motor nerves, prolonged distal latency of ≥2 motor nerves, or prolonged F-wave latencies of ≥2 motor nerves or the absence of F waves\|\|2 of the 4 AAN electrodiagnostic criteria\|\|Partial conduction block of ≥2 motor nerves and abnormal conduction velocity or distal latency or F-wave latency in 1 other nerve; or, in the absence of partial conduction block, abnormal conduction velocity, distal latency, or F-wave latency in 3 motor nerves; or electrodiagnostic abnormalities indicating demyelination in 2 nerves and histologic evidence of demyelination		\| Electrodiagnostic Criteria\|\|Any 3 of the following 4 criteria: partial conduction block of ≥1 motor nerve, reduced conduction velocity of ≥2 motor nerves, prolonged distal latency of ≥2 motor nerves, or prolonged F-wave latencies of ≥2 motor nerves or the absence of F waves\|\|2 of the 4 AAN electrodiagnostic criteria\|\|Partial conduction block of ≥2 motor nerves and abnormal conduction velocity or distal latency or F-wave latency in 1 other nerve; or, in the absence of partial conduction block, abnormal conduction velocity, distal latency, or F-wave latency in 3 motor nerves; or electrodiagnostic abnormalities indicating demyelination in 2 nerves and histologic evidence of demyelination
	\|-		\|-
	\| CSF\|\|WBC < 10/mm^3, negative VDRL' elevated protein\|\|Protein > 45mg/dL; WBC <10/mm^3\|\|Recommended but not mandatory		\| CSF\|\|WBC < 10/mm^3, negative VDRL, elevated protein\|\|Protein > 45mg/dL; WBC <10/mm^3\|\|Recommended but not mandatory
	\|-		\|-
	\| Biopsy\|\|Demylelination and remyelination\|\|Demyelination\|\|Not mandatory		\| Biopsy\|\|Demylelination and remyelination\|\|Demyelination\|\|Not mandatory

Mtjordan91: /* Clinical Features */

2020-09-22T21:57:20Z

Clinical Features

← Older revision		Revision as of 21:57, 22 September 2020
Line 8:		Line 8:
	==Clinical Features==		==Clinical Features==
	*Symmetrical proximal and distal muscle weakness that is progressive for GREATER than two months		*Symmetrical proximal and distal muscle weakness that is progressive for GREATER than two months
	*~~Impaired sensation~~		*Muscle weakness more predominant feature than sensory symptoms
	*~~Areflexia~~ or ~~hyporeflexia~~		*Reduced or absent deep tendon reflexes in upper and lower extremities
	*Elevated CSF protein		*Elevated CSF protein without pleocytosis
	*~~EMG studies consistent with demyelination~~		*Electrodiagnostic evidence of a demyelinating neuropathy
	*~~Nerve~~ demyelination on nerve biopsies		*Segmental nerve demyelination on nerve biopsies
	*Can be relapsing or chronic and progressive		*Can be relapsing or chronic and progressive

Mtjordan91: /* Clinical Features */

2020-09-12T18:43:07Z

Clinical Features

← Older revision		Revision as of 18:43, 12 September 2020
Line 9:		Line 9:
	*Symmetrical proximal and distal muscle weakness that is progressive for GREATER than two months		*Symmetrical proximal and distal muscle weakness that is progressive for GREATER than two months
	*Impaired sensation		*Impaired sensation
	*Areflexia		*Areflexia or hyporeflexia
	*Elevated CSF protein		*Elevated CSF protein
	*EMG studies consistent with demyelination		*EMG studies consistent with demyelination

Mtjordan91: /* Management */

2020-09-12T18:42:25Z

Management

← Older revision		Revision as of 18:42, 12 September 2020
Line 50:		Line 50:
	==Management==		==Management==
	*Requires Neurology consult		*Requires Neurology consult
	*IVIG
	*Plasma exchange
	*Corticosteroids
	*Therapy is tailored to each patient, no difference in efficacy between the three treatment options		*Therapy is tailored to each patient, no difference in efficacy between the three treatment options
			**IVIG
			**Plasma exchange
			**Corticosteroids
	*May be placed on azathioprine, cyclophosphamide, or cyclosporine to prevent relapse		*May be placed on azathioprine, cyclophosphamide, or cyclosporine to prevent relapse

Mtjordan91: /* References */

2020-09-12T18:41:25Z

References

← Older revision		Revision as of 18:41, 12 September 2020
Line 66:		Line 66:

	==References==		==References==
	*Köller, H., Kieseier, B. C., Jander, S., & Hartung, H.-P. (2005). Chronic inflammatory demyelinating polyneuropathy. The New England Journal of Medicine, 352(13), 1343–1356.		*Köller, H., Kieseier, B. C., Jander, S., & Hartung, H.-P. (2005). Chronic inflammatory demyelinating polyneuropathy. The New England Journal of Medicine, 352(13), 1343–1356.

	<references/>		<references/>

Mtjordan91: /* References */

2020-09-12T18:41:01Z

References

← Older revision		Revision as of 18:41, 12 September 2020
Line 66:		Line 66:

	==References==		==References==
			*Köller, H., Kieseier, B. C., Jander, S., & Hartung, H.-P. (2005). Chronic inflammatory demyelinating polyneuropathy. The New England Journal of Medicine, 352(13), 1343–1356.
	<references/>		<references/>