Whole-body muscle MRI to detect myopathies in non-extrapyramidal bent spine syndrome

Ohana, Mickaël; Durand, Marie-Christine; Marty, C.; Lazareth, J. P.; Maisonobe, Thierry; Mompoint, D.; Carlier, Robert‐Yves

doi:10.1007/s00256-014-1909-3

Cited by 10 publications

(9 citation statements)

References 40 publications

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“…En pacientes con enfermedad de Parkinson, los hallazgos pueden variar desde cambios agudos con edema y tumefacción de la musculatura paravertebral, el cuadrado lumbar y el psoas, en los primeros 31 meses, 36,44 hasta los más habituales hallazgos tardíos crónicos de degeneración y sustitución grasa. 18,45,46 Una desventaja de la RM muscular es su falta de especificidad. Ante el hallazgo de hiperintensidades en el STIR muscular, se debería indicar una biopsia muscular con el objetivo de diferenciar la miositis de un trauma, desnervación u otras causas de enfermedades musculares.…”

Section: Diagnóstico Por Imágenesunclassified

“…Ante el hallazgo de hiperintensidades en el STIR muscular, se debería indicar una biopsia muscular con el objetivo de diferenciar la miositis de un trauma, desnervación u otras causas de enfermedades musculares. 45 tratamiento El tratamiento del SEI dependerá principalmente de su etiología.…”

Section: Diagnóstico Por Imágenesunclassified

“…Para los pacientes con miositis focal, el tratamiento con corticoides por vía endovenosa será suficiente. 45,53 En casos secundarios a polimiositis o dermatomiositis, la combinación de corticoides e inmunosupresores (metotrexato, azatioprina, ciclosporina) deberá estar sujeta a la actividad de la propia enfermedad. 10 Una excepción es la miositis por cuerpos de inclusión, para la cual el tratamiento inmunosupresor y las inmunoglobulinas son ineficaces, por lo que el enfoque terapéutico se deberá centrar en la fisioterapia.…”

Section: Tratamiento Del Sei De Origen Distónicounclassified

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Síndrome de la espalda inclinada. Presentación de cuatro casos y revisión de la bibliografía. [Bent spine syndrome. Presentation of four cases and literature review]

Covaro

Vilà-Canet²,

Ciccolo

et al. 2019

Rev. Asoc. Arg. Ort. y Traumatol

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El síndrome de la espalda inclinada o camptocormia es una causa de desequilibrio sagital del tronco no estructurada de difícil manejo médico y quirúrgico. Puede ser secundario a enfermedades del sistema nervioso central o periférico, o de origen primario muscular, como la atrofia aislada de la musculatura paravertebral espinal. El diagnóstico se basa en la evaluación clínica, los estudios por imágenes, el electromiograma y la biopsia muscular. El síndrome de la espalda inclinada, cualquiera fuera su causa, tiene un pronóstico pobre, los síntomas suelen progresar hasta afectar la bipedestación de manera irreversible. Presentamos un análisis retrospectivo de cuatro casos de síndrome de la espalda inclinada tratados en nuestro centro y una revisión de la bibliografía. AbstractIntroduction: The bent spine syndrome (BSS) or camptocornia is a cause of unstructured sagittal imbalance of difficult medical and surgical management.Objective: To describe the causes of BSS and how to approach its treatment.Materials and Methods: Retrospective analysis of 4 cases of BSS treated at our center and review of the literature.Results: The 4 cases were women between 60 and 82 years of age, 3 due to an isolated atrophy of the paravertebral spinal erectorae musculature and 1 case due to an inflammatory myopathy.Conclusions: The prognosis of any BSS is poor, so the symptoms usually progress to irreversible difficulty to maintain upright posture.

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Section: Diagnóstico Por Imágenesunclassified

Section: Tratamiento Del Sei De Origen Distónicounclassified

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Síndrome de la espalda inclinada. Presentación de cuatro casos y revisión de la bibliografía. [Bent spine syndrome. Presentation of four cases and literature review]

Covaro

Vilà-Canet²,

Ciccolo

et al. 2019

Rev. Asoc. Arg. Ort. y Traumatol

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“…In particular, if muscle MRI shows exclusively acute changes, a muscle biopsy is indicated, because STIR hyperintensities cannot prove myositis. The muscle MRI can be used to determine the most appropriate biopsy site [117]. …”

Section: Diagnosis Of Camptocormiamentioning

confidence: 99%

Pathophysiological Concepts and Treatment of Camptocormia

Margraf

Wrede

Deuschl

et al. 2016

Journal of Parkinson’s Disease

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Camptocormia is a disabling pathological, non-fixed, forward bending of the trunk. The clinical definition using only the bending angle is insufficient; it should include the subjectively perceived inability to stand upright, occurrence of back pain, typical individual complaints, and need for walking aids and compensatory signs (e.g. back-swept wing sign). Due to the heterogeneous etiologies of camptocormia a broad diagnostic approach is necessary. Camptocormia is most frequently encountered in movement disorders (PD and dystonia) and muscles diseases (myositis and myopathy, mainly facio-scapulo-humeral muscular dystrophy (FSHD)). The main diagnostic aim is to discover the etiology by looking for signs of the underlying disease in the neurological examination, EMG, muscle MRI and possibly biopsy. PD and probably myositic camptocormia can be divided into an acute and a chronic stage according to the duration of camptocormia and the findings in the short time inversion recovery (STIR) and T1 sequences of paravertebral muscle MRI. There is no established treatment of camptocormia resulting from any etiology. Case series suggest that deep brain stimulation (DBS) of the subthalamic nucleus (STN-DBS) is effective in the acute but not the chronic stage of PD camptocormia. In chronic stages with degenerated muscles, treatment options are limited to orthoses, walking aids, physiotherapy and pain therapy. In acute myositic camptocormia an escalation strategy with different immunosuppressive drugs is recommended. In dystonic camptocormia, as in dystonia in general, case reports have shown botulinum toxin and DBS of the globus pallidus internus (GPi-DBS) to be effective. Camptocormia in connection with primary myopathies should be treated according to the underlying illness.

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“…Most studies have concentrated on lower limb imaging, where most clinical experience lies. However, whole‐body MRI, or MRI of the truncal and upper limb muscles, is beginning to be explored . The majority of findings described relate to hyperintensity on T1‐weighted (T1w) images, reflecting fatty infiltration of muscle.…”

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confidence: 99%

Muscle magnetic resonance imaging in congenital myasthenic syndromes

et al. 2016

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IntroductionIn this study we investigated muscle magnetic resonance imaging in congenital myasthenic syndromes (CMS).MethodsTwenty‐six patients with 9 CMS subtypes and 10 controls were imaged. T1‐weighted (T1w) and short‐tau inversion recovery (STIR) 3‐Tesla MRI images obtained at thigh and calf levels were scored for severity.ResultsOverall mean the T1w score was increased in GFPT1 and DPAGT1 CMS. T1w scans of the AChR‐deficiency, COLQ, and CHAT subjects were indistinguishable from controls. STIR images from CMS patients did not differ significantly from those of controls. Mean T1w score correlated with age in the CMS cohort.ConclusionsMRI appearances ranged from normal to marked abnormality. T1w images seem to be especially abnormal in some CMS caused by mutations of proteins involved in the glycosylation pathway. A non‐selective pattern of fat infiltration or a normal‐appearing scan in the setting of significant clinical weakness should suggest CMS as a potential diagnosis. Muscle MRI could play a role in differentiating CMS subtypes. Muscle Nerve 54: 211–219, 2016

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Whole-body muscle MRI to detect myopathies in non-extrapyramidal bent spine syndrome

Cited by 10 publications

References 40 publications

Síndrome de la espalda inclinada. Presentación de cuatro casos y revisión de la bibliografía. [Bent spine syndrome. Presentation of four cases and literature review]

Síndrome de la espalda inclinada. Presentación de cuatro casos y revisión de la bibliografía. [Bent spine syndrome. Presentation of four cases and literature review]

Pathophysiological Concepts and Treatment of Camptocormia

Muscle magnetic resonance imaging in congenital myasthenic syndromes

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