Ultrasound pattern sum score, homogeneity score and regional nerve enlargement index for differentiation of demyelinating inflammatory and hereditary neuropathies.
OBJECTIVE
To investigate the use of nerve ultrasound in the differentiation between Charcot-Marie Tooth hereditary neuropathy (CMT1) and chronic inflammatory demyelinating polyradiculoneuropathies (CIDP), multifocal motor neuropathy (MMN) and multifocal acquired demyelinating sensory and motor neuropathies (MADSAM).


METHODS
Ultrasound/electrophysiology of predefined nerves was performed in CMT1a/b, immunoneuropathies, and healthy controls. Ultrasound pattern sum score (UPSS, sum of the amount of 12 predefined measurement points), homogeneity score (HS) and regional nerve enlargement index (RNEI) in ulnar, median, and tibial nerve were used for evaluation of morphology.


RESULTS
13 CMT1, 27 CIDP, 10 MADSAM, 12 MMN, and 23 controls were included. Significant enlargement was shown in all neuropathies compared to the controls, (p<0.001), however the amount of enlargement as evaluated by the UPSS was most prominent in CMT compared to the others (median UPSS 18 vs. 11/8.5/5 in CIDP/MADSAM/MMN, p<0.001). Homogeneous enlargement was significantly more often seen in CMT (67%, HS 6 vs. 2-3 in immune-mediated PNP, p<0.001), while in CIDP the enlargement was regional, homogeneous or inhomogeneous with equal contribution. In MMN and MADSAM regional enlargement (48%/40%) next to normal segments (∼20%) predominated (RNEI in MMN=2, in MADSAM=1 vs. 0 in the others). CSAs were inversely correlated with motor conduction velocity.


CONCLUSIONS
Ultrasound, quantified by UPSS, HS, and RNEI facilitates a reliable and reproducible differentiation of immunoneuropathies and hereditary neuropathies by the use of boundary values.


CONCLUSIONS
By the use of quantitative scores, ultrasound differentiation of demyelinating neuropathies is operationalized and ameliorated compared to CSA measurements only.
Department of Neurology, Tübingen University Hospital, Tübingen, Germany. Electronic address: alexander.grimm@usb.ch.
Alexander
A
Grimm
Department of Neurology, Tübingen University Hospital, Tübingen, Germany.
Debora
D
Vittore
Department of Neurology, Tübingen University Hospital, Tübingen, Germany.
Victoria
V
Schubert
Department of Neurology, Tübingen University Hospital, Tübingen, Germany.
Christina
C
Lipski
Hans Berger Department of Neurology, Jena University Hospital, Jena, Germany.
Bianka
B
Heiling
Department of Neurology, Basel University Hospital, Basel, Switzerland.
Bernhard F
BF
Décard
Hans Berger Department of Neurology, Jena University Hospital, Jena, Germany.
Hubertus
H
Axer
D000328:Adult; D000368:Aged; D002607:Charcot-Marie-Tooth Disease / Q000000981:diagnostic imaging* / Q000503:physiopathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000000981:diagnostic imaging / Q000503:physiopathology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000000981:diagnostic imaging* / Q000503:physiopathology; D014463:Ultrasonography
CIDP; Charcot–Marie–Tooth; Demyelinating; Inflammatory neuropathy; Nerve ultrasound; Polyneuropathy; UPSS