Protocol - Neuropathy - Clinical Assessment

Description

The Charcot-Marie-Tooth Neuropathy Score is a nine-item clinical examination that measures sensory symptoms, motor symptoms, arm and leg strength, and nerve conduction velocities.

Specific Instructions

This protocol must be administered by a neurologist or physician with relevant training.

Availability

Available

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Protocol

Score
Parameter	0	1	2	3	4
Sensory Symptoms	None	Limited to toes	Extend up to and may include ankle	Extend up to and may include knee	Extends above knees
Motor Symptoms
Legs	None	Trips, catches toes, slaps feet	AFO on at least 1 leg or ankle support	Cane, walker, ankle surgery	Wheelchair most of the time
Arms	None	Difficulty with buttons/zippers	Unable to do buttons or zippers but can write	Can not write or use keyboard	Proximal arms
Pin sensibility	Normal	Reduced in fingers/toes	Reduced up to and may include wrist/ankle	Reduced up to and may include elbow/knee	Reduced above elbow/knee
Vibration	Normal	Reduced at fingers/toes	Reduced at wrist/ankle	Reduced at elbow/knee	Reduced above elbow/knee
Strength
Legs	Normal	4+, 4, or 4- on foot dorsiflexion	≤3 Foot dorsiflexion	≤3 Dorsiflexion and plantar flexion	Proximal weakness
Arms	Normal	4+, 4, or 4- on intrinsics or finger extensors	≤3 Intrinsics or finger extensors	<5 Wrist extensors	Weak above elbow
Ulnar CMAP	> 6 mV	4.0-5.9 mV	2.0-3.9 mV	0.1-1.9 mV	Absent
(Median)	(>4 mV)	(2.8-3.9)	(1.2-2.7)	(0.1-1.1)	(Absent)
Ulnar SNAP	> 9 uV	6.0 - 8.9 uV	3.0-5.9 uV	0.1-2.9 uV	Absent
(Median)	(>22 uV)	(14.0-21.9)	(7.0-13.9)	(0.1-6.9)	(Absent)

AFO = ankle-foot orthosis
CMAP = compound muscle action potential
SNAP = sensory nerve action potential

Scoring Instructions
Sum scores on all items. Maximum score is 36.
1-10 mildly affected
11-20 moderately affected
21-36 severely affected

Personnel and Training Required

This Charcot-Marie-Tooth Neuropathy Score protocol must be completed by a trained neurologist or by an experienced peripheral nerve clinician.

Equipment Needs

Specialized equipment is necessary to assess the peroneal compound muscle action potential (CMAP) and sural sensory nerve action potential (SNAP).

Requirements

Requirement Category	Required
Major equipment	Yes
Specialized training	Yes
Specialized requirements for biospecimen collection	No
Average time of greater than 15 minutes in an unaffected individual	No

Mode of Administration

Physical Examination

Lifestage

Adult, Senior

Participants

Adult, aged 18 years and older.

Selection Rationale

The Charcot-Marie-Tooth Neuropathy Score was vetted against similar protocols and chosen because it is a relatively short, validated protocol that focuses more on motor function and weakness over the sensory symptoms that characterize toxic and diabetic neuropathies.

Language

English

Standards

Standard	Name	ID	Source
Logical Observation Identifiers Names and Codes (LOINC)	Clinical neuropathy proto	62759-6	LOINC
Human Phenotype Ontology	Peripheral Neuropathy	HP:0009830	HPO
caDSR Form	PhenX PX130201 - Clinical Neuropathy Assessment	6166762	caDSR Form

Derived Variables

None

Process and Review

Expert Review Panel 4 (ERP 4) reviewed the measures in the Neurology, Psychiatric, and Psychosocial domains.

Guidance from ERP 4 included the following:

· Changed name of measure

Protocol Name from Source

Charcot-Marie-Tooth Neuropathy Score

Source

Shy, M., Blake, J., Krajewski, K., Fuerst, D., Laura, M., Hahn, A., Li, J., Lewis, R., & Reilly, M. (2005). Reliability and validity of the CMT Neuropathy Score as a measure of disability. Neurology, 64(7), 1209-1214.

General References

Murphy, S. M., Herrmann, D. N., McDermott M. P., Scherer, S. S., Shy, M. E., Reilly, M. M., & Pareyson, D. (2011). Reliability of the CMT Neuropathy Score (second version) in Charcot-Marie-Tooth disease. Journal of the Peripheral Nervous System, 16(3), 191-198.

Protocol ID

130201

Variables

Export Variables

Variable Name	Variable ID	Variable Description	dbGaP Mapping
PX130201_CMAP
	PX130201080000	Compound Muscle Action Potential (CMAP)	N/A
PX130201_Motor_Symptoms_Arms
	PX130201030000	Motor Symptoms: Arms	N/A
PX130201_Motor_Symptoms_Legs
	PX130201020000	Motor Symptoms: Legs	N/A
PX130201_Pin_Sensibility
	PX130201040000	Pin sensibility	N/A
PX130201_Sensory_Symptoms
	PX130201010000	Sensory Symptoms	N/A
PX130201_Strength_Arms
	PX130201070000	Strength: Arms	N/A
PX130201_Strength_Legs
	PX130201060000	Strength: Legs	N/A
PX130201_Ulnar_CMAP_Median
	PX130201090000	Ulnar Compound Muscle Action Potential (Median)	N/A
PX130201_Ulnar_SNAP
	PX130201100000	Ulnar Sensory Nerve Action Potential	N/A
PX130201_Ulnar_SNAP_Median
	PX130201110000	Ulnar Sensory Nerve Action Potential (Median)	N/A
PX130201_Vibration
	PX130201050000	Vibration	N/A

Neurology

Measure Name

Neuropathy

Release Date

May 12, 2010

Definition

A measure to assess neuropathy.

Purpose

This measure can be used to assess the severity of symptoms and disability associated with peripheral neuropathy caused by a number of disorders including tumors, infections, autoimmune diseases, and inherited disorders such as Charcot-Marie-Tooth disease.

Keywords

Neurology, small fiber neuropathy, diabetic neuropathy, clinical neuropathy assessment, autonomic neuropathy, gerontology, aging, geriatrics

Measure Protocols

Protocol ID	Protocol Name
130201	Neuropathy - Clinical Assessment
130202	Neuropathy - Autonomic Assessment - Adult

Publications

There are no publications listed for this protocol.