Dropped-head syndrome due to steroid responsive focal myositis: A case report and review of the literature

Kastrup, Andreas; Gdynia, Hans-Jürgen; Nägele, Thomas; Riecker, Axel

doi:10.1016/j.jns.2007.09.042

Cited by 52 publications

(24 citation statements)

References 19 publications

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“…Three patients demonstrated an inflammatory myopathic change and other patients demonstrated histological characteristics of a necrotizing myopathy or a myopathic change with mitochondrial abnormalities. Most of the cases that had shown inflammatory infiltrates in affected muscles on biopsy responded well to steroids (22,24,(26)(27)(28) and good disease control was achieved with immunosuppressive therapy such as azathioprine (24,27) and intravenous immunoglolin (25). The etiology of the present cases was unclear, but the clinical features seemed to be an immune-mediate mechanism such as an inflammatory type of myopathy because of dramatic response to steroid therapy.…”

Section: Discussionmentioning

confidence: 64%

“…Goh et al (22) suggested that DHS caused by a myopathic origin is a syndrome of mixed etiology and that there is a spectrum of pathological processes ranging from non-inflammatory to pronounced inflammatory myopathy. So far, only 7 reports (9 cases) of DHS due to a restricted myositis have appeared in the literature (22)(23)(24)(25)(26)(27)(28). Gaeta et al (26) used the term "inflammatory INEM" for this condition.…”

Section: Discussionmentioning

confidence: 99%

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Dramatic Response of Dropped Head Sign to Treatment with Steroid in Parkinson's Disease: Report of Three Cases

et al. 2011

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Neck dystonia is the most common cause of dropped head sign in parkinsonism. Isolated neck extensor myopathy, which is a rare condition, can also cause dropped head sign in parkinsonism, but no improvement has been achieved with immunosuppressive therapy. We report three cases of treatable neck extensor myopathy causing dropped head sign in patients with Parkinson's disease. Needle electromyography and magnetic resonance imaging suggested a restrictive active myopathy affecting neck extensor muscles. All cases responded dramatically to steroid therapy. Routine needle electromyography should be performed to explore treatable myopathy in Parkinson's disease.

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 99%

Dramatic Response of Dropped Head Sign to Treatment with Steroid in Parkinson's Disease: Report of Three Cases

et al. 2011

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“…In the context of muscle spasm, MRI of drop head syndrome demonstrated a diffuse muscular edema of the neck extensor muscles on fat-suppressed T2-weighted images (STIR), as well as the enhancement of these muscles after administration of gadolinium on T1-weighted images. 3,7,16,17 Electromyography of the neck extensor muscles (C5-T1) and the trapezius muscles re- vealed a myopathic pattern. 3,7,16,17 The diagnosis of a focal myositis was confirmed by a biopsy of the trapezius muscle, splenius capitis, or semispinalis capitis muscles, and the muscle biopsy revealed necrosis of muscle fibers, myophagia, and T-cell infiltrates.…”

Section: Discussionmentioning

confidence: 96%

“…3,7,16,17 Electromyography of the neck extensor muscles (C5-T1) and the trapezius muscles re- vealed a myopathic pattern. 3,7,16,17 The diagnosis of a focal myositis was confirmed by a biopsy of the trapezius muscle, splenius capitis, or semispinalis capitis muscles, and the muscle biopsy revealed necrosis of muscle fibers, myophagia, and T-cell infiltrates. 3,7,16,17 Therefore, the flexed or dropped head posture that may afflict patients with PD may be attributed at least in part to involuntary cervical muscle contraction with net neck flexion due to neck extensor muscle myopathy.…”

Section: Discussionmentioning

confidence: 96%

Prevalence and type of cervical deformities among adults with Parkinson's disease: a cross-sectional study

Moon

Smith

Ames

et al. 2016

SPI

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OBJECT To identify the characteristics of cervical deformities in Parkinson's disease (PD) and the role of severity of PD in the development of cervical spine deformities, the authors investigated the prevalence of the cervical deformities, cervical kyphosis (CK), and cervical positive sagittal malalignment (CPSM) in patients with PD. They also analyzed the association of severity of cervical deformities with the stage of PD in the context of global sagittal spinopelvic alignment. METHODS This study was a prospective assessment of consecutively treated patients (n = 89) with PD. A control group of the age- and sex-matched patients was selected from patients with degenerative cervical spine disease but without PD. Clinical and demographic parameters including age, sex, duration of PD, and Hoehn and Yahr (H&Y) stage were collected. Full-length standing radiographs were used to assess spinopelvic parameters. CK was defined as a C2–7 Cobb angle < 0°. CPSM was defined as C2–7 sagittal vertical axis (SVA) > 4 cm. RESULTS A significantly higher prevalence of CPSM (28% vs 1.1%, p < 0.001), but not CK (12% vs 10.1%, p = 0.635), was found in PD patients compared with control patients. Among patients with PD, those with CK were younger (62.1 vs 69.0 years, p = 0.013) and had longer duration of PD (56.4 vs 36.2 months, p = 0.034), but the severity of PD was not significantly different. Logistic regression analysis revealed that the presence of CK was associated with younger age, higher mismatch between pelvic incidence and lumbar lordosis, and lower C7–S1 SVA. The patients with CPSM had significantly greater thoracic kyphosis (TK) (p < 0.001) and a trend toward more advanced H&Y stage (p = 0.05). Logistic regression analysis revealed that CPSM was associated with male sex, greater TK, and more advanced H&Y stage. CONCLUSIONS Patients with PD have a significantly higher prevalence of CPSM compared with age- and sex-matched control patients with cervical degenerative disease but without PD. Among patients with PD, CK is not associated with the severity of PD but is associated with overall global sagittal malalignment. In contrast, the presence of CPSM is associated more with the severity of PD than it is with the presence of global sagittal malalignment. Collectively, these data suggest that the neuromuscular pathogenesis of PD may affect the development of CPSM more than of CK.

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“…While camptocormia is an age- and disorder-related symptom, the primary degeneration of the erector spinae muscles is regarded as a muscle disorder, and denominated as bent spine syndrome. Different etiology could result in paraspinal muscle weakness, such as inflammatory myopathies [5,6], metabolic disorders [7,8] and, rarely, genetically determined degenerative muscle disorders such as ryanodine receptor gene (RYR1) mutation [9], D4Z4 gene mutations (which characterize facioscapulohumeral dystrophy [4,10]) and, as described in one case, a DYSF gene mutation [3]. We present the history of a 67-year-old woman with bent spine syndrome, in whom the mutation of the DYSF gene also was the probable background of bent spine syndrome.…”

mentioning

confidence: 99%

Bent Spine Syndrome: A Phenotype of Dysferlinopathy or a Symptomatic DYSF Gene Mutation Carrier

et al. 2012

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Dropped-head syndrome due to steroid responsive focal myositis: A case report and review of the literature

Cited by 52 publications

References 19 publications

Dramatic Response of Dropped Head Sign to Treatment with Steroid in Parkinson's Disease: Report of Three Cases

Dramatic Response of Dropped Head Sign to Treatment with Steroid in Parkinson's Disease: Report of Three Cases

Prevalence and type of cervical deformities among adults with Parkinson's disease: a cross-sectional study

Bent Spine Syndrome: A Phenotype of Dysferlinopathy or a Symptomatic DYSF Gene Mutation Carrier

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