Der „Ultrasound pattern sum score“ – ein möglicher Algorithmus in der Anwendung des Nervenultraschalls bei Polyneuropathien

Zusammenfassung

Die Wertigkeit des Nervenultraschalls bei Polyneuropathie wird aktuell intensiv diskutiert und untersucht. Ziel der UPSS-Studie war daher die Erarbeitung eines standardisierten und operationalisierten Untersuchungsablaufs für die systematische Erfassung sonografischer Nervenbefunde.

Methodik 61 Patienten mit akuter oder subakuter Polyneuropathie wurden systematisch sonografisch untersucht. Auf Basis der unterschiedlichen Beteiligung verschiedener Nerven bei individuellen PNP-Syndromen konnten Kompartimente eingeteilt (UPSA=sensomotorische Nerven, UPSB=Wurzeln und N. vagus, UPSC=sensible Nerven) und anhand des Ausmaßes einer Vergrößerung der einzelnen Nerven an definierten anatomischen Landmarken Punkte vergeben werden. Hieraus konnte ein Algorithmus abgeleitet werden, der in Folge dann an 24 Patienten validiert wurde.

Ergebnisse Der UPSA und der Summenscore UPSS waren signifikant erhöht bei CIDP-Patienten (p<0,001) gegenüber allen anderen Gruppen, wohingegen bei GBS-Patienten nur der UPSB erhöht war. Bei Vaskulitiden war der UPSS mäßig, aber signifikant im Vergleich zu anderen axonalen Neuropathien erhöht. Mittels ROC Analyse konnten Grenzwerte für diese Scores entwickelt werden, die einen positiv prädiktiven Wert von >85% hatten. In beiden Validierungsgruppen betrug die Trefferquote über 90%.

Diskussion Der UPSS hilft, sonografische Veränderungen der Nerven bei Polyneuropathie zu quantifizieren und zu klassifizieren. Ein erhöhter UPSS oder UPSA sind sensitiv für eine CIDP, verdickte Wurzeln oder ein verdickter N. vagus alleine (UPSB) sind typisch für Patienten mit einem GBS, wohingegen leichte Nervenschwellungen (Score zwischen 3 und 9 Punkten) häufig bei vaskulitischen Neuropathien zu finden sind. Kontrollen und andere Polyneuropathien (insbesondere axonale) haben keine erhöhten Scores.

Schlussfolgerung Punktwerttabellen bieten eine Möglichkeit, Ergebnisse des Nervenultraschalls zu quantifizieren. Eine Kategorisierung von Nervenultraschallbefunden und somit Diagnosestellung anhand typischer Muster scheint möglich.

Abstract

The usefulness of nerve ultrasound in polyneuropathies has been widely discussed in the recent past. Here we suggest a standardized examination protocol and scoring system to operationalize differentiation between distinct neuropathies.

Methods Initially, 61 patients with proven polyneuropathy (symptom duration <9 months) were analyzed retrospectively with regard to nerve ultrasound results at predefined anatomical landmarks. Based on the distinct affection of several compartments depending on the type of polyneuropathy, we built 3 subscores (UPS-A for sensorimotor nerves, UPS-B for cervical roots and the vagal nerve and UPS-C for the sural nerve), the sum of all scores (UPS-S) reaching a maximum of 20 points. Each nerve enlargement was scored according to predefined boundary normal values. Based on the subscore results of different patterns, an algorithm was worked out for diagnosing polyneuropathies. In a second step, the proposed algorithm was validated in 24 consecutive patients presenting with symptoms of polyneuropathy.

Results In chronic inflammatory demyelinating polyneuropathy (CIDP), the UPS-A and UPS-S were significantly increased compared to all other neuropathies (p<0,001), whereas Guillain-Barré syndrome (GBS) patients showed an increase only in UPS-B. Overall, the UPS-S was low in axonal neuropathies except in vasculitis patients, in whom an intermediate UPS-S was found. By ROC curve analysis, boundary values were defined concerning the distinct neuropathies with a positive predictive value (PPV) >85%. In the prospective application, the pattern score could be used with good accuracy >90% in several types of neuropathies.

Discussion The UPSS is a reliable tool to categorize acute and subacute polyneuropathy ultrasound patterns in several neuropathies with high sensitivity for an increased UPS-S and UPS-A for CIDP, while enlarged roots and vagus nerves only point to the diagnosis of GBS. Controls and axonal neuropathies show UPS-S values <3 points.

Significance Using the UPSS and its sub-scores gives a new diagnostic power to the method of the peripheral nerve ultrasound.

Schlüsselwörter

Ultraschall bei Polyneuropathie - ultrasound pattern - Ultraschall score - Homogenitätsscore - ultrasound pattern sum score

Key words

ultrasound polyneuropathy - ultrasound pattern - ultrasound score - homogeneity score - ultrasound pattern sum score

• Literatur

• 1 Schminke U. Sonografie peripherer Nerven: Relevante Indikationen für die klinische Praxis. (Sonography of peripheral nerves: relevant indications for clinical practice). Klin Neurophysiol 2016; 47: 173-179 doi:10.1055/s-0042-118965
• 2 Padua L, Martinoli C, Pazzaglia C. et al. Intra- and internerve cross-sectional area variability: new ultrasound measures. Muscle Nerve 2012; 45: 730-733
• 3 Padua L, Granata G, Sabatelli M. et al. Heterogeneity of root and nerve ultrasound pattern in CIDP patients. Clin Neurophysiol 2014; 125: 160-165
• 4 Goedee HS, van der Pol WL, van Asseldonk JH. et al. Diagnostic value of sonography in treatment-naive chronic inflammatory neuropathies. Neurology 2017; 88: 143-151
• 5 Noto Y, Shiga K, Tsuji Y. et al. Nerve ultrasound depicts peripheral nerve enlargement in patients with genetically distinct Charcot-Marie-Tooth disease. J Neurol Neurosurg Psychiatry 2015; 86: 378-384
• 6 Schreiber S, Oldag A, Kornblum C. et al. Sonography of the median nerve in CMT1A, CMT2A, CMTX, and HNPP. Muscle Nerve 2013; 47: 385-395
• 7 Beekman R, Visser LH. Sonographic detection of diffuse peripheral nerve enlargement in hereditary neuropathy with liability to pressure palsies. J Clin Ultrasound 2002; 30: 433-436
• 8 Grimm A, Heiling B, Schumacher U. et al. Ultrasound differentiation of axonal and demyelinating neuropathies. Muscle Nerve 2014; 50: 976-983
• 9 Zaidman CM, Al-Lozi M, Pestronk A. Peripheral nerve size in normals and patients with polyneuropathy: an ultrasound study. Muscle Nerve 2009; 40: 960-966
• 10 Zaidman CM, Harms MB, Pestronk A. Ultrasound of inherited vs. acquired demyelinating polyneuropathies. J Neurol 2013; 260: 3115-3121
• 11 Di Pasquale A, Morino S, Loreti S. et al. Peripheral nerve ultrasound changes in CIDP and correlations with nerve conduction velocity. Neurology 2015; 84: 803-809
• 12 Scheidl E, Bohm J, Simo M. et al. Different patterns of nerve enlargement in polyneuropathy subtypes as detected by ultrasonography. Ultrasound in medicine & biology 2014; 40: 1138-1145
• 13 Beekman R, van den Berg LH, Franssen H. et al. Ultrasonography shows extensive nerve enlargements in multifocal motor neuropathy. Neurology 2005; 65: 305-307
• 14 Neubauer C, Gruber H, Bauerle J. et al. Ultrasonography of Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (MADSAM). Clinical neuroradiology 2015; 25: 423-425
• 15 Grimm A, Thomaser AL, Peters N. et al. Neurological picture. Vagal hypertrophy in immune-mediated neuropathy visualised with high-resolution ultrasound (HR-US). J Neurol Neurosurg Psychiatry 2015; 86: 1277-1278
• 16 Kerasnoudis A, Pitarokoili K, Behrendt V. et al. Multifocal motor neuropathy: correlation of nerve ultrasound, electrophysiological, and clinical findings. J Peripher Nerv Syst 2014; 19: 165-174
• 17 Grimm A, Decard BF, Athanasopoulou I. et al. Nerve ultrasound for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy. J Neurol 2015; 262: 870-880
• 18 Grimm A, Vittore D, Schubert V. et al. Ultrasound aspects in therapy-naive CIDP compared to long-term treated CIDP. J Neurol 2016; 263: 1074-1082
• 19 Zaidman CM, Pestronk A. Nerve size in chronic inflammatory demyelinating neuropathy varies with disease activity and therapy response over time: a retrospective ultrasound study. Muscle Nerve 2014; 50: 733-738
• 20 Kerasnoudis A, Pitarokoili K, Gold R. et al. Nerve ultrasound and electrophysiology for therapy monitoring in chronic inflammatory demyelinating polyneuropathy. J Neuroimaging 2015; 25: 931-939
• 21 Grimm A, Décard BF, Bischof A. et al. Ultrasound of the peripheral nerves in systemic vasculitic neuropathies. Journal of the neurological sciences 2014; 347: 44-49
• 22 Üçeyler N, Schäfer KA, Mackenrodt D et al. High-resolution ultrasonography of the superficial peroneal motor and sural sensory nerves may be a non-invasive approach to the diagnosis of vasculitic neuropathy. Frontiers in neurology 2016; 7
• 23 Kerasnoudis A, Woitalla D, Gold R. et al. Sarcoid neuropathy: correlation of nerve ultrasound, electrophysiological and clinical findings. Journal of the neurological sciences 2014; 347: 129-136
• 24 Rasenack M, Proebstel AK, Athanasopoulou IM et al. Nerve hypertrophy in primary amyloidosis. Muscle Nerve 2016
• 25 Grimm A, Schäffer E, Just J. et al. Thickening of the peripheral nerves in metachromatic leukodystrophy. J Neurol Sci. 2016; 368: 399-401
• 26 Kerasnoudis A, Pitarokoili K, Haghikia A. et al. Nerve ultrasound protocol in differentiating chronic immune-mediated neuropathies. Muscle Nerve 2016; 54: 864-871
• 27 Kerasnoudis A, Pitarokoili K, Gold R. et al. Bochum ultrasound score allows distinction of chronic inflammatory from multifocal acquired demyelinating polyneuropathies. Journal of the neurological sciences 2015; 348: 211-215
• 28 Kerasnoudis A, Pitarokoili K, Behrendt V. et al. Nerve ultrasound score in distinguishing chronic from acute inflammatory demyelinating polyneuropathy. Clin Neurophysiol 2014; 125: 635-641
• 29 Grimm A, Decard BF, Axer H. et al. The Ultrasound pattern sum score - UPSS. A new method to differentiate acute and subacute neuropathies using ultrasound of the peripheral nerves. Clin Neurophysiol 2015; 126: 2216-2225
• 30 Joint Task Force of the E, the PNS . European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society–first revision. J Peripher Nerv Syst 2010; 15: 295-301
• 31 Grimm A, Decard BF, Axer H. Ultrasonography of the peripheral nervous system in the early stage of Guillain-Barre syndrome. J Peripher Nerv Syst 2014; 19: 234-241
• 32 Décard BF, Fladt J, Axer H. et al. Nerve ultrasound in miller fisher variant of Guillain–Barré syndrome. Muscle Nerve 2015; 52: 1106-1110
• 33 Grimm A, Decard BF, Schramm A. et al. Ultrasound and electrophysiologic findings in patients with Guillain-Barre syndrome at disease onset and over a period of six months. Clin Neurophysiol 2016; 127: 1657-1663
• 34 Gallardo E, Sedano MJ, Orizaola P. et al. Spinal nerve involvement in early Guillain-Barre syndrome: a clinico-electrophysiological, ultrasonographic and pathological study. Clin Neurophysiol 2015; 126: 810-819
• 35 Grimm A, Rasenack M, Athanasopoulou IM. et al. The modified ultrasound pattern sum score mUPSS as additional diagnostic tool for genetically distinct hereditary neuropathies. J Neurol 2016; 263: 221-230
• 36 Grimm A, Vittore D, Schubert V. et al. Ultrasound pattern sum score, homogeneity score and regional nerve enlargement index for differentiation of demyelinating inflammatory and hereditary neuropathies. Clin Neurophysiol 2016; 127: 2618-2624
• 37 Grimm A, Rattay TW, Winter N. et al. Peripheral nerve ultrasound scoring systems: benchmarking and comparative analysis. J Neurol. 2017; 264: 243-253
• 38 Breiner A, Qrimli M, Ebadi H. et al. Peripheral nerve high-resolution ultrasound in diabetes. Muscle Nerve. 2017; 55: 171-178 doi:10.1002/mus.25223