Neuromyositis: A Rare Extramuscular Manifestation of Dermatomyositis

Chan Keol Park; Su-Jin Yoo; In Seol Yoo; Jinhyun Kim; Seung Cheol Shim; Seong Wook Kang

doi:10.4078/jrd.2019.26.3.211

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Park, Yoo, Yoo, Kim, Shim, and Kang: Neuromyositis: A Rare Extramuscular Manifestation of Dermatomyositis

Case Report

J Rheum Dis 2019;26(3):211-218.

Published online: 31 October 2019

DOI: https://doi.org/10.4078/jrd.2019.26.3.211

Neuromyositis: A Rare Extramuscular Manifestation of Dermatomyositis

Chan Keol Park, Su-Jin Yoo, In Seol Yoo, Jinhyun Kim, Seung Cheol Shim, Seong Wook Kang

Division of Rheumatology, Department of Internal Medicine, Chungnam National University Hospital, Daejeon, Korea

Corresponding to: Seong Wook Kang http://orcid.org/0000-0002-0076-0822 Division of Rheumatology, Department of Internal Medicine, Chungnam National University Hospital, 282 Munhwa-ro, Jung-gu, Daejeon 35015, Korea. E-mail: kangsw@cnuh.co.kr

Received 26 December 201818 February 2019 Accepted 7 March 2019

This is a Open Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Dermatomyositis (DM) and polymyositis (PM) are representative idiopathic inflammatory myopathies characterized by symmetric and progressive proximal muscle weakness. Especially, DM is identified by characteristic skin lesions and has many extramuscular manifestations including various cardiac abnormalities, interstitial lung disease, and malignancy. However, involvement of peripheral nervous system in DM/PM is very rare and less known. The term “Neuromyositis” was introduced by Senator in 1893 to describe the concomitant involvement of the peripheral nervous system in DM/PM. Since then, a very few cases of neuromyositis have been reported mainly in the United States and Europe. Therefore, the pathogenetic mechanism and disease progression are unclear. In recent years, a few more cases were reported in Asia, specifically, China and Japan; however, none in Korea. Here, we describe a case of DM-associated neuromyositis in a 42-year-old man in Korea and review previous publications through literature research.

Keywords: Dermatomyositis, Peripheral nervous system diseases, Nerve conduction, Electromyography

REFERENCES

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Figure 1.

T2-weighted magnetic resonance images (A, B) and enhanced T1-weighted magnetic resonance image (C) showed high signal intensity in right su-praspinatus and anterior deltoid muscles (arrows). (D) Electron microscopy of right deltoid muscle biopsy demonstrated focal infiltration of fat globules, focal areas of myofibrillar loss, and several tubuloreticular structures (arrow) in the cytoplasm of endothelial cells, which were consistent with dermatomyositis (×30,000).

Figure 2.

Initial chest computed tomography (CT) images showed multifocal patchy ground glass opacities (GGOs) in subpleural portion of both lower lungs (A, B). Follow-up chest CT images obtained 1 month after the initiation of therapy showed aggravation to reticulation with GGOs (C, D). Follow-up chest CT images obtained at the time of neuromyositis diagnosis showed improving state of previous noted reticulation and GGOs (E, F).

Table 1.

Summary of clinical and laboratory findings of the present case

Variable	At the time of dermatomyositis diagnosis	At the time of neuromyositis diagnosis	After recovery of neuromyositis	Reference value
Clinical findings
MMT grade (right/left)
Shoulder abduction	4-/4-	5/5	5/5
Shoulder vertical flexion	4-/4-	5/5	5/5
Hip flexion	4-/4-	5/5	5/5
Ankle dorsiflexion	5/5	5/5	3-/5
Ankle plantarflexion	5/5	5/5	4-/5
Dyspnea (mMRC grade)	0^*	0	0
Laboratory findings
ESR (mm/hr)	34	15	2	0∼10
CRP (mg/dL)	0.1	0.1	0.1	0∼0.5
CK (U/L)	344	136	129	56∼244
AST (U/L)	50	22	23	0∼37
ALT (U/L)	28	21	18	0∼41
LDH (U/L)	598	360	-	200∼400
Pulmonary function test
FVC, L (% predicted)	4.76 (98)	4.67 (96)	5.03 (104)
FEV1, L (% predicted)	3.55 (95)	3.56 (96)	3.59 (97)
FEV1/FVC, %	75	76	71
DLco, mL/mmHg/min (% pred	dicted) 32.5 (129)	37.1 (150)	30.8 (122)

MMT: manual muscle test, mMRC: modified Medical Research Council dyspnea scale, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, CK: creatine kinase, AST: aspartate aminotransferase, ALT: alanine aminotransferase, LDH: lactate dehydrogenase, FVC: forced vital capacity, FEV1: forced expiratory volume in 1 second, DLco: diffuse lung capacity for carbon monoxide.

^* Not troubled by breathlessness except on strenuous exercise.

Table 2.

Results of nerve conduction study

Nerve	1st study			2nd study			3rd study
Nerve	Lat	Amp	CV	Lat	Amp	CV	Lat	Amp	CV
Motor nerve
Left peroneal	3.0 ms	5.3 mV	47.7 m/s	3.92 ms	3.2 mV	52.4 m/s	4.04 ms	7.2 mV	53.4 m/s
Right peroneal	3.42 ms	2.8 mV	47.2 m/s	4.13 ms	1.14 mV	48.8 m/s	4.90 ms	3.3 mV	50.0 m/s
Sensory nerve
Left peroneal superficial	2.4 ms	5.7 μ V	58.3 m/s	2.98 ms	5.7 μ V	47.0 m/s	2.69 ms	13.2 μ V	52.0 m/s
Right peroneal superficial	0.0 ms	0.0 μ V	0.0 m/s	3.09 ms	1.46 μ V	45.3 m/s	2.51 ms	7.3 μ V	55.8 m/s

The 1st study means a study performed at the time of dermatomyositis diagnosis. The 2nd study means a study performed at the time of neuromyositis diagnosis. The 3rd study means a study performed after recovery of neuromyositis. Lat: latency, Amp: amplitude, CV: conduction velocity.

Table 3.

Results of electromyography study

Muscle	2nd study
	Spontaneous activity				Voluntary activity
	Fib	PSW	Fasc	Amp	Dur	Polyph	IP
Right EHL Right PL Right TA	1+ 2+ 2+	1+ 2+ 2+	None None None	Normal Normal Normal	Normal Normal Normal	+ Normal +	PIP-CIP PIP-CIP PIP-CIP

The 2nd study means a study performed at the time of neuromyositis diagnosis. EHL: extensor hallucis longus, PL: peroneus longus, TA: tibialis anterior, Fib: fibrillation potentials, PSW: positive sharp waves, Fasc: fasciculation, Amp: amplitude, Dur: duration, Polyph: polyphasic, IP: interference pattern, PIP: partial interference pattern, CIP: complete interference pattern.

Table 4.

Previously reported cases of neuromyositis, including the present case

Case number	Author (country/year)	Age/sex	Type of peripheral neuropathy	Nerve conduction study	Treatment	Response
1	Present case (Korea/2019)	42/M	Axonal neuropathy	Decreased in CMAP and SNAP	Oral PD (40 mg/d) + azathioprine (2∼2.5 mg/kg/d)	Almost completely recovered muscle weakness after 6 months
2	Irie et al. [14] (Japan/2016)	69/F	Axonal neuropathy	Decreased in CMAP and SNAP	First admission: IVMP→oral PD (15 mg/d) Second admission: IVMP→oral PD (40 mg/d)	Improved muscle weakness and hypoesthesia
3	Onder et al. [5] (Turkey/2015)	80/F	Axonal neuropathy	Decreased in CMAP and SNAP	Oral PD (dose is not described)	At least moderate response
4	Nguyen et al. [13] (USA/2014)	67/F	Axonal neuropathy	Absent SNAP	Not described	Not described
5	Cojocaru et al. [15] (Romania/2011)	59/F	Axonal neuropathy	Decreased nerve conduction study and compatible with acute polyradiculoneuritis	IVMP, IVIG, cyclophosphamide pulse therapy	Slowly improved muscle weakness
6	Nomura et al. [12] (Japan/2010)	52/F	Axonal neuropathy	Absent CMAP and SNAP	IVMP→oral PD (50 mg/d)^ IVIG 30 gx5 d	No improvement
7	França et al. [16] (Brazil/2007) Case 1	71/M	Undetermined	Absent SNAP	Azathioprine (3 mg/kg/d) and followed on a regular basis	Although sensory complaints improved, residual proprioceptive, vibratory, and tactile dysfunction are still present
8	França et al. [16] (Brazil/2007) Case 2	41/F	Undetermined	Decreased in CMAP and absent SNAP	Oral PD (1 mg/kg/d)	Persistent proprioceptive hypesthesia with sensory ataxia after 1 year
9	Matsui et al. [8] (Japan/2003) Case 1	68/F	Axonal neuropathy	Decreased in CMAP and SNAP	IVMP^oral PD (30 mg/d)	Normalized muscle weakness, sensory disturbance unknown
10	Matsui et al. [8] (Japan/2003) Case 2	48/F	Axonal neuropathy	Decreased in CMAP	IVMP, twice^oral PD (50 mg/d)	Gradually improved muscle weakness
11	Vogelgesang et al. [11] (USA/ 1995) Case 1	9/M	Axonal neuropathy	Decreased in CMAP and absent SNAP	IVMP, oral PD, IVIG, MTX	Improved muscle weakness, slowly resolved sensory disturbances by MTX
12	Vogelgesang et al. [11] (USA/1995) Case 2	7/F	Axonal neuropathy	Decreased CMAP bilaterally	Oral PD (2 mg/kg), MTX	Resolution of paresthesia by MTX

M: male, F: female, CMAP: compound muscle action potential, SNAP: sensory nerve action potential, PD: prednisolone, IVMP: intravenous methylprednisolone pulse, IVIG: intravenous immunoglobulin, MTX: methotrexate.

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