RESUMO
Objective: Hirayama disease is a rare cause of cervical myelopathy predominantly affecting young individuals. The disease is classically characterized by muscle atrophy in the distal upper limbs. While various etiopathogenesis such as dural sac dysplasia, nerve root dysplasia, structural abnormalities of the spinal ligament, and venous dysplasia have been proposed, this study explores the potential role of venous pathology and surgical management on the basis of it. Methodology: This is a prospective descriptive case series of nine cases. The diagnosis was made based on the Huashan diagnostic criteria which includes clinical manifestation, imaging, and electrophysiology. In cases where magnetic resonance imaging (MRI) failed to demonstrate engorged veins, a computed tomography (CT) venogram of the cervical spine was used as an imaging tool. All patients underwent cervical laminectomy and coagulation of the posterior epidural venous plexus with or without laminoplasty. All the patients were followed up regularly; clinical improvement and neck disability index were assessed. Results: All nine patients were male and exhibited classical clinical features, electrophysiological abnormalities, and MRI findings except, in one patient where a CT venogram helped in establishing the diagnosis as the MRI was inconclusive. Postoperatively, all patients had neurological improvement and stabilization of the disease. All patients who underwent CT venogram and cervical spine X-ray in neutral and dynamic position demonstrated no recurrence of engorged venous plexus or significant instability except one patient developing kyphosis. One patient experiencing symptoms in the other limb underwent a second surgery. Conclusion: This comprehensive case series strongly supports venous pathology as a potential etiology of Hirayama disease. Surgical management with laminectomy and venous coagulation with or without expansile laminoplasty has delivered consistent improvement in neurological outcomes and long-term disease stabilization without the restriction of movements and lesser complications. However, further research is warranted to elucidate the mechanism underlying cervical venous dilatation.
RESUMO
Objective: Low back pain (LBP) is a major cause of pain and disability. Identification of the pathology accurately or the pain generators is sometimes difficult with the conventional modalities such as magnetic resonance imaging (MRI), computed tomography (CT), or X-ray. Nuclear medicine investigations such as single-photon emission CT (SPECT/CT) or 18-fluorodeoxyglucose positron emission tomography-CT (18-FDG PET-CT) have emerged as an adjuvant tool in these cases. In this study, we evaluated and analyzed the role of 18-FDG PET-CT in identifying active pain generators and the outcomes of interventions based on that compared to MRI. Methodology: This study included all patients who fell under inclusion criteria presented with chronic LBP with or without radiculopathy. History and clinical examination were done as well as Visual Analog Scale (VAS) and Oswestry Disability Index (ODI) scores were calculated. All the patients underwent MRI lumbosacral spine with sacroiliac (SI) joint and 18-FDG PET-CT whole spine. Patients in whom PET-CT was positive and active pain generator was identified were managed for the specific level or pain generator responsible by appropriate modalities, i.e. surgery, interfacetal injections, transforaminal epidural injections, and SI joint injections. Patients in whom PET-CT was negative were managed according to the pain generator identified on the basis of MRI and clinical correlation. Patients were told to follow-up after 1 week and 1 month, and subsequent improvement was evaluated on the basis of VAS after 1 week and 1 month and ODI score after 1 month. Results: A total of 20 patients were included in the study, with a mean age of 41.9 ± 13.53 years. Twelve patients had multiple level pathology without the indication of significant pain generator and eight patients' symptoms did not correlate with the MRI findings. 18-FDG PET-CT was done in all patients. 10% (2/20) patients were identified with active pain generators on PET-CT which were not identified on MRI. Eleven out of twenty patients underwent intervention in the form of surgery or pain injections. The mean VAS and ODI score in the patients intervened on the basis of 18-FDG PET-CT improved by 70.59% and 50%, respectively, whereas in patients who underwent intervention on the basis of MRI had improvement in mean VAS and ODI score by 58.57% and 30.81%, respectively after 1 month. Conclusion: Inflammation and associated degenerative process in the spine is a continuous process and affects multiple levels and might not be easily picked up on MRI or other conventional modalities. Thus, 18-FDG PET-CT is useful in identifying these active inflammatory processes and thereby helping in the localization of active pain generators. Treating these active pain generators has a better outcome in patients after intervention in terms of better pain relief and quality of life and also reduces the levels being treated.
