Segmental neurofibromatosis with compression of the spinal cord at the cervical level. Literature review and case report

Background. Neurofibromatosis is a fairly rare disease (1/3000). In 1992, V. Riccardi described seven types of neurofibromatosis. Segmental neurofibromatosis (sh), also known as type V neurofibromatosis, is an extremely rare variant characterized by the development of typical cutaneous manifestations or one body segment neurofibromas. Clinical case. Currently, the literature describes about 100 cases of sh and only one of them with compression of the spinal cord. We present our first case of this nosological form with spinal cord compression in a Russian patient. A 70-year-old patient, due to an increasing paresis in the left extremities, underwent mri of the cervical spine, which revealed solid tumors located extramedullary intra-extradurally at the level of c2-c3 vertebrae with pronounced compression of the spinal cord. At the time of hospitalization, clinical presentation was characterized by deep spastic tetraparesis (1–2 points), impairment of all types of sensitivity from the c4 level by the conductive type, and dysfunction of the pelvic organs by the type of delay. Karnofsky index was 50 %, 2 points on the Fim scale. Standard c2-c3 vertebrae laminectomy was performed. Spinal cord compression was eliminated due to the removal of intradural tumors. Subsequently, extradural tumors were removed step by step. On histological examination, tumors were represented by intertwining bundles of elongated schwann cells with wavy nuclei with pointed ends and ileogenic fibers. Mucin present in the stroma separated cells and fibers. Conclusion. Sn is a rare type of neurofibromatosis. However, from the point of view of genetics, it is most likely incorrect to attribute it to a separate type of neurofibromatosis, since the cause of its development is mosaicism of somatic cells due to mutation of the NF 1 gene. Sn is rarely manifested by the development of spinal nerves multiple neurofibromas, however, it can be accompanied by a gross neurological deficit caused by compression of the spinal cord such neurofibromas. Surgical treatment is based on basic and special surgical principles that determine the anatomical and morphological characteristics of the area of intervention, the compliance of which allows for good treatment results.

Management and outcome of spontaneous cervical epidural hematoma: Case report and review of the literature

Spontaneous spinal epidural hematomas are rare and potentially disabling neurological emergencies. Its lead to devastating neurologic outcomes and most patient does not recover completely. The clinical presentation is diverse and includes a severe acute attack, radiating pain at the back, interscapular, or neurological deficits. We report a case of a young woman, 24-year-old, that was admitted to our department for sudden non-traumatic cervical spinal cord compression syndrome (Type A of the American Spinal Cord Injury Association “ASIA A”) including intense cervical back pain, sensory loss, and tetraplegia. Her past medical history was unremarkable. The MRI confirmed a cervical mass responsible for the spinal cord compression and the emergent surgical intervention allow us to evacuate acute C3-C7 hematoma. The patient never recovers from the neurologic deficit despite the emergent management of her case followed by functional musculoskeletal rehabilitation for two years.

Symptomatic thoracic ossification of the ligamentum flavum in a patient with ankylosing spondylitis: Report of a case and review

Background: Thoracic spinal cord compression due to both ankylosing spondylitis (AS) and ossification of the ligamentum flavum (OLF) is rare. Case Description: A 33-year-old male with AS presented with a paraparesis attributed to MR documented T9-T10 OLF/stenosis. He was successfully managed with a decompressive laminectomy; this resulted in marked improvement of his deficit. Conclusion: Thoracic OLF and AS rarely contribute T9-T10 spinal cord compression that may be readily relieved with a decompressive laminectomy.

Acute spinal cord compression due to spinal subdural hematoma after spinal anaesthesia in-patient under anticoagulant drug

Background Subdural spinal cord hematomas are very rare condition. They most often occur in patients with primary or secondary blood haemostasis disorders and following lumbar punctures. Early diagnosis and management preserve functional prognosis. Case description We report the case of a female 69-year-old patient on oral anticoagulant, Acenocoumarol 4 mg (SINTROM) for previous aortic prosthesis. The patient had undergone surgery for appendicitis under spinal anaesthesia 2 days before her admission in neurosurgery department. She was admitted in emergency for 1/5 central flaccid paraplegia with sensitive umbilical level. A spinal MRI performed showed a collection intradural in intermediate signal in T1 and hyposignal in T2 with echo gradient of 8 mm thickness extended from D8 to L2 compressing the marrow with anomalies of intramedullary signal extended from D8 to the conus medullaris. We retained indication to operate the patient early in emergency because of acute spinal cord compression. We performed T12-L2 laminectomy, durotomy and evacuated hematoma. Postoperative marked by an immediate recovery of sensitivity and an onset of motor recovery from 1/5 to 2/5 and 4/5 follow up at on year with physiotherapy. Conclusion Spinal cord compression due to subdural spinal hematomas not often described especially in patients with haemostasis blood disorders due to anticoagulants drugs. In addition, we should pay attention with lumbar puncture in these patients. Emergency surgery allows a good prognosis about recovery of neurological disorders.

A missed C5 vertebral brown tumor causing spinal cord compression and myelopathy: a case report and literature review

We aim to report a patient with vertebral brown tumor in the context of primary hyperparathyroidism presented with shoulder pain. This is the first report of C5 involvement by Brown tumor in a primary hyperparathyroid patient and emphasizes the consideration of cervical vertebral evaluation in patients with persistent shoulder pain.