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Protocol - Neuropathy - Clinical Assessment

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Protocol Name from Source:

Charcot-Marie-Tooth Neuropathy Score

Availability:

Publicly available

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.

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 CategoryRequired
Average time of greater than 15 minutes in an unaffected individualNo
Major equipmentYes
Specialized requirements for biospecimen collectionNo
Specialized trainingYes

Mode of Administration

Health Professional

Life Stage:

Adult, Senior

Specific Instructions:

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

Research Domain Information

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.

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

StandardNameIDSource
Common Data Elements (CDE)Person Clinical Neuropathy Assessment Score3076102CDE Browser
Logical Observation Identifiers Names and Codes (LOINC)Clinical neuropathy proto62759-6LOINC

Process and Review

[link[phenx.org/node/103|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

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 NameVariable IDVariable DescriptionVersiondbGaP Mapping
PX130201_Sensory_SymptomsPX130201010000Sensory Symptoms4N/A
PX130201_Motor_Symptoms_LegsPX130201020000Motor Symptoms: Legs4N/A
PX130201_Motor_Symptoms_ArmsPX130201030000Motor Symptoms: Arms4N/A
PX130201_Pin_SensibilityPX130201040000Pin sensibility4N/A
PX130201_VibrationPX130201050000Vibration4N/A
PX130201_Strength_LegsPX130201060000Strength: Legs4N/A
PX130201_Strength_ArmsPX130201070000Strength: Arms4N/A
PX130201_CMAPPX130201080000Compound Muscle Action Potential (CMAP)4N/A
PX130201_Ulnar_CMAP_MedianPX130201090000Ulnar Compound Muscle Action Potential (Median)4N/A
PX130201_Ulnar_SNAPPX130201100000Ulnar Sensory Nerve Action Potential4N/A
PX130201_Ulnar_SNAP_MedianPX130201110000Ulnar Sensory Nerve Action Potential (Median)4N/A