1/6. Cord compression secondary to cervical disc herniation associated with calcification of the ligamentum flavum: case report.OBJECTIVE AND IMPORTANCE: Calcification of the ligamentum flavum is a rare disease that occurs almost exclusively in elderly Japanese people. We report the case of a young Caucasian woman who presented with a C5-C6 disc herniation associated with a cervical calcified ligamentum flavum. CLINICAL PRESENTATION: The patient presented with a cord compression syndrome of 76 hours' evolution. At exploration, a brown-sequard syndrome at the C6 level was found. magnetic resonance imaging and computed tomography led to a correct diagnosis and planning for decompression. INTERVENTION: We operated on the patient through a combined anterior and posterior approach. After the patient underwent anterior discectomy with intersomatic arthrodesis, we performed posterior decompression. During the operation, we observed that the dura mater could not be separated from the ligamentum, so an en bloc excision of both structures was performed. Microscopic examination indicated that the excised ligamentum had calcification, and total integration of the dura mater into the structure of the ligamentum was demonstrated. To our knowledge, this circumstance has never been described before. A posterior C3-C7 arthrodesis was performed to prevent postoperative kyphosis. Recovery was successful, with total recovery from neurological deficits 4 months later. CONCLUSION: Calcification of the ligamentum flavum is a progressive disease that starts early in life and becomes symptomatic later in life when spinal stenosis occurs. magnetic resonance imaging and computed tomography provide adequate diagnosis and allow proper surgical planning for decompression. The presence of hyperintense areas within the spinal cord parenchyma, in the absence of a traumatic antecedent, does not preclude a complete recovery.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/6. Atlantoaxial instability in Dyggve-Melchior-Clausen syndrome. Case report and review of the literature.Dyggve-Melchior-Clausen (DMC) syndrome is a very rare disease. Only 58 cases have been reported in the literature. The syndrome is probably an autosomal recessive inherited disorder, one that is characterized by mental retardation, the short-spine type of dwarfism, and skeletal abnormalities, especially of the spine, hands, and pelvis. Atlantoaxial instability-induced spinal cord compression is a serious and preventable complication. The purpose of this report is to describe the first case of DMC syndrome in which anterior transarticular atlantoaxial screw fixation was used to treat atlantoaxial instability. The authors report on a 17-year-old man with DMC syndrome and concomitant severe atlantoaxial instability. Computerized tomography scanning and magnetic resonance angiography demonstrated an irregular course of the vertebral artery (VA) at C-2, which made a posterior fixation procedure impossible. Additionally, transoral fusion was impossible because the patient was unable to open his mouth sufficiently. Therefore, the patient underwent anterior transarticular screw fixation. Follow-up examination 36 weeks after surgery showed solid fusion without implant failure. In conclusion, treatment of atlantoaxial instability in DMC syndrome must be considered. Specific care must be taken to determine the course of the VA. If posterior and transoral fusion are impossible, anterior transarticular atlantoaxial screw fixation might be the only alternative.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/6. Spontaneous spinal epidural hematoma: an uncommon presentation of a rare disease.INTRODUCTION: Spontaneous spinal epidural hematoma is rare in children. The presenting symptoms are usually pain, either local or radicular, followed by progressive bilateral weakness, and sensory loss hours and even days later. In the absence of significant precipitating factors such as severe trauma or previously known coagulopathies the diagnosis is usually delayed, and it is not until the full picture of severe cord compression is developed, that MRI is done and the diagnosis is finally made. CASE REPORT: We describe a case of 10-year-old girl who presented with pain and pure brachial plexus radiculopathy as the only clinical manifestations of spinal epidural hematoma. CONCLUSION: A high index of suspicion can lead to the correct diagnosis even before the development of full cord compression and thus improve the overall prognosis.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |
4/6. Cervical angina caused by atlantoaxial instability.Cervical angina is defined as a paroxysmal precordialgia that resembles true cardiac angina caused by cervical spondylosis. Cervical angina most commonly results from compression of the C7 ventral root. We present here a case of cervical angina caused by atlantoaxial instability. This case had marked atlantoaxial instability but no flexibility of the middle to lower levels of the cervical spine. Although there was mild C7 root compression on the radiologic findings, the chest pain was induced by neck motion, and the precordialgia disappeared after posterior atlantoaxial fusion without C7 root decompression. Therefore, we diagnosed this case as cervical angina caused by spinal cord compression at the C1-C2 level. It was speculated that a perturbation of the sympathetic nervous system or a hypofunction of the pain suppression pathway in the posterior horn of the spinal cord caused the pectoralgia. Although cervical angina is a rare disease, physicians should be aware of it; if there are no abnormal findings on cardiac examinations for angina pectoris, they should examine the cervical spine. Cervical angina due to atlantoaxial instability is one of the differential diagnoses of precordialgia.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/6. Dumbbell-shaped cervical spinal neurilemmoma presenting as neck mass.Cervical neurilemmoma may originate from any nerve sheath tissue in the neck including the vagus nerve, glossopharyngeal nerve, brachial plexus, sympathetic trunk and cervical spine. We report an unusual case of a dumbbell-shaped neurilemmoma arising from the cervical spinal roots in a patient who complained of having had a neck mass for several months. Computed tomographic scan and magnetic resonance imaging revealed a dumbbell-shaped tumour extending from the C4 spinal level through the intervertebral foramen into the right parapharyngeal space. decompression surgery was performed first via the cervical approach. Five months later, the patient received laminectomy and a complete tumour excision. The symptoms and signs were significantly relieved without neurological sequelae. No evidence of recurrence was noted after one-year follow up. This two-staged operation could offer an alternative surgical approach yielding ideal therapeutic results in such a rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/6. Cervical dystonia induced by cervical spine surgery: a case report.STUDY DESIGN: A case report. OBJECTIVES: To describe an interesting patient who underwent a three-level corpectomy of the cervical spine complicated by cervical dystonia and eventually treated successfully with botulinum toxin injections. SUMMARY OF BACKGROUND DATA: Cervical dystonia is a relatively rare disease and unfamiliar to many clinicians. Various types of peripheral trauma or peripheral lesion have been reported to induce cervical dystonia. However, to the best of our knowledge, there have been no reports about cervical dystonia following cervical spine surgery. methods: We present a case of a 45-year-old man who developed severe axial neck pain after cervical anterior corpectomy and fibula strut grafting due to cervical myelopathy. His neck pain gradually worsened, and involuntary spasmodic neck movement developed 6 weeks after operation when his halo-vest was removed. Initially, we considered his complaint to be transient or psychogenic, and diagnosis of cervical dystonia was delayed until 14 weeks after operation. RESULTS: Pharmacologic treatment was unsuccessful, but he was successfully treated with local intramuscular injections of botulinum toxin. CONCLUSIONS: It must be kept in mind that cervical spine surgery is not an exceptional precipitator of cervical dystonia, despite the fact that it is extremely rare.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |