Rare occurrence of Hirayama disease in Brazil

Bogoni, Mateos; Teixeira, Bernardo Corrêa de Almeida; Cioni, Marianna

doi:10.1590/0004-282x20190036

Cited by 3 publications

(1 citation statement)

References 5 publications

(1 reference statement)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…1 It occurs predominantly in male adolescents as insidious onset, slowly progressive predominantly unilateral hand and forearm wasting and weakness (sparing the brachioradialis muscle), followed by a spontaneous arrest within several years. 2,3 Several cases or case series have been published, reviewing the demographics, epidemiology, clinical presentation, diagnoses, and treatment of Hirayama disease 1,[3][4][5][6][7] ; however, early recognition and diagnosis remain a clinical challenge among neurologists and non-neurologists.…”

Section: Discussionmentioning

confidence: 99%

Hirayama Disease: An Important Cause of Focal Hand Weakness in Young Adults

Jiang

Ubogu

2021

Journal of Investigative Medicine High Impact Case Reports

View full text Add to dashboard Cite

Patients with progressive hand weakness may be seen in ambulatory medical clinics or in emergency rooms due to direct effects on activities of daily living or inadvertent injury associated with overuse or attempts to maintain normal function. It is important to recognize potential cause(s) and perform appropriate diagnostic tests and referrals that aid guide appropriate treatment that may lead to good outcomes. Hirayama disease is an underrecognized disorder in young adults due to an asymmetric growth-associated cervical spinal cord compression injury. Awareness of this disorder by internists, emergency room physicians, and radiologists would prevent unnecessary tests and interventions that may contribute to disease progression by delaying appropriate treatments or treating inappropriately, with consequential effects on outcomes. In this article, we describe 3 Hirayama disease cases from a single tertiary care institution and demonstrate how delayed diagnosis affected outcomes in 2 patients and early recognition facilitated improved outcomes in a patient.

show abstract

Section: Discussionmentioning

confidence: 99%

Hirayama Disease: An Important Cause of Focal Hand Weakness in Young Adults

Jiang

Ubogu

2021

Journal of Investigative Medicine High Impact Case Reports

View full text Add to dashboard Cite

show abstract

Differential Diagnoses of the Neurological Manifestations of Hansen’s Disease

Deps,

Cunha,

Garbino

2023

Hansen’s Disease

View full text Add to dashboard Cite

Why the patients with Hirayama disease have abnormal cervical sagittal alignment? A radiological measurement analysis of posterior cervical extensors

et al. 2022

View full text Add to dashboard Cite

Purpose To explore the relationship between the strength of posterior cervical extensors (PCEs) and cervical sagittal alignment in Hirayama disease (HD) patients. Methods We analyzed the (magnetic resonance imaging) MRI T2WI and X-rays of 60 HD patients who visited Huashan Hospital from June 2017 to February 2020. Symptoms of these patients include adolescent onset, manifestation of unilateral upper limb muscle weakness and muscle atrophy of the forearm and hand. MRI images were used to measure (the cross-sectional area) CSA of cervical PCEs. The ratio of muscle CSA to vertebral body areas at the same level is defined as R-CSA. Cervical sagittal alignment includes the C2–7 Cobb angle, T1 slope and C2–7 sagittal vertical axis (SVA). The geometric center of the C3–6 vertebral body was determined using the line connecting the C2 inferior endplate and the C7 upper endplate. When located behind the line, it is defined as a “local kyphotic deformity.” The number of vertebral bodies involved in kyphotic deformity was determined by measuring the local kyphosis angle (LKA). Spearman correlation analysis (α = 0.05) was used to determine the relationship between R-CSA and sagittal parameters. ROC curves were used to analyze the sensitivity and specificity of relevant variables. Results Spearman correlation test revealed that R-CSA negatively correlated with T1S (S = 0.34, r = 0.34, p = 0.01) and LKA (S = 0.44, r = 0.5, p = 0.01), but did not correlate with the C2-C7 Cobb angle (S = 0.20, p = 0.12) or C2-C7 SVA (S = − 0.17, p = 0.46). (p < 0.05). ROC curve analysis showed that the areas under the curve (AUCs) of the T1 slope and LKA was 0.6696 and 0.7646, respectively. T1 slope, cutoff value: 17.2°; sensitivity: 0.5806; specificity: 0.7241; p < 0.05. LKA: cutoff value: − 14°; sensitivity: 1; specificity: 0.5333; p < 0.05. Conclusions In patients with Hirayama disease, the strength of posterior cervical extensors and cervical sagittal alignment are closely related. The local kyphosis angle can be used as a reference for the strength of posterior cervical extensors. These results indicate the weakness of PCEs, which may predispose the cervical spine of HD patients to a less stable situation. Therefore, patients with Hirayama disease should strengthen the exercise of the PCEs.

show abstract

Rare occurrence of Hirayama disease in Brazil

Cited by 3 publications

References 5 publications

Hirayama Disease: An Important Cause of Focal Hand Weakness in Young Adults

Hirayama Disease: An Important Cause of Focal Hand Weakness in Young Adults

Differential Diagnoses of the Neurological Manifestations of Hansen’s Disease

Why the patients with Hirayama disease have abnormal cervical sagittal alignment? A radiological measurement analysis of posterior cervical extensors

Contact Info

Product

Resources

About