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

2022 ◽  
Vol 20 (6) ◽  
pp. 158-163
Author(s):  
D. A. Gulyaev ◽  
I. Yu. Belov ◽  
D. V. Nizolin ◽  
L. B. Mitrofanova ◽  
N. A. Primak ◽  
...  

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.

2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Nooraldin Merza ◽  
Ahmed Taha ◽  
John Lung ◽  
Anthony W. Benderman ◽  
Stephen E. Wright

Immunoglobulin G4-related disease (IgG4-RD) is known for forming soft tissue mass lesions that may have compressive effects. It is an extremely rare disease that most frequently affects the pancreas causing autoimmune pancreatitis. It can also affect the gallbladder, salivary glands, and lacrimal glands causing respective organ-specific complications. In our report, we describe an IgG4-RD case that affected the spinal cord. A 60-year-old female presented with cervical spinal cord compression caused by IgG4-RD leading to several neurological deficits. Pathological examination of the excisional biopsy of the mass revealed dense lymphoplasmacytic cells infiltration and stromal fibrosis with IgG4 and plasma cells. The patient showed a dramatic response to the administration of systemic steroids with almost resolution of her neurological symptoms. This case highlights the first case in literature for IgG4-RD of the extradural tissue causing spinal compression. Hereby, we also demonstrate the dramatic response of IgG4-RD to the administration of systemic steroids as the patient had no recurrence after 5 years of close follow-up, the longest reported period of follow-up reported in the literature to date.


2021 ◽  
Vol 8 (4) ◽  
pp. 198-204
Author(s):  
Duc Duy Tri Tran ◽  
Quoc Bao Nguyen ◽  
Van Tri Truong ◽  
Thai Duong Truong ◽  
Dinh Thanh Phan ◽  
...  

Spontaneous spinal epidural hematoma (SSEH) is a rare disease but may lead to life-threatening consequences if not timely diagnosed and managed. Emergent hematoma evacuation is indicated before neurological deficits become irreversible. We report two cases. The first case was a 45-year-old man brought to hospital because of an acute onset of quadriparesis and urinary incontinence. His cervical magnetic resonance imaging (MRI) showed an epidural hematoma at the C5-C6 level with severe spinal cord compression. He underwent an emergency C5-C6 right hemi-laminotomy to remove the clot and decompress the cord. Postoperatively, his left-sided deficits immediately resolved. His urinary function returned to normal two weeks after the surgery. He could independently walk two months later. The second case was a 57-year-old man admitted to the hospital because of severe neck pain and paresthesia in both arms. He had been using an antiplatelet for two months. His MRI revealed an epidural hematoma from C2 to C4 with spinal cord compression on the right. This patient was successfully treated with conservative treatment. If SSEH is left undiagnosed and untreated, the neurological deficits may be permanent. Early emergent hematoma evacuation contributes to a favorable outcome. Conservative management is reasonable if neurological deficits are not severe.


Author(s):  
Ebtesam Abdulla ◽  
Harleen Luther ◽  
Tejal Shah ◽  
Nisha Chandran

Background Immunoglobulin G4–related disease (IgG4-RD) is a recently identified multisystemic fibroinflammatory condition of unclear etiology. IgG4-RD of the epidural tissue causing spinal cord compression is extremely rare. Case description Here, we present a 27-year-old male with epidural mass, causing spinal cord compression at the level of D5-D6. The mass proved pathologically to be epidural inflammatory pseudotumor (IPT) related to IgG4. Spinal decompression was done. The patient was started on steroid treatment and reported a complete resolution of his symptoms over a 3 years’ follow-up period. Conclusion To the authors’ knowledge, this is the first case of IgG4-related epidural IPT and spinal cord compression in Bahrain and the Middle East. IgG4-RD should always be considered as a part of the differential diagnosis of spinal tumors.


1996 ◽  
Vol 84 (3) ◽  
pp. 518-521 ◽  
Author(s):  
Emmanuel K. Labram ◽  
J. Mohan

✓ In diaphyseal aclasis, the exostoses usually involve long bones, although occasionally the spine is also affected. Very few cases of osteochondroma causing spinal cord compression have been cited. The authors report their experience with two cases of diaphyseal aclasis. In the first case spinal cord compression caused by an exostosis of the lamina of C-2 occurred in a 9-year-old boy; in the second case a large osteochondroma of C-5 occurred in a 45-year-old man. Also included in this report is a review of the literature highlighting the incidence of diaphyseal aclasis, its clinical features and its excellent prognosis in treated cases.


2007 ◽  
Vol 65 (2A) ◽  
pp. 279-282 ◽  
Author(s):  
Arthur de Azambuja Pereira Filho ◽  
Gustavo de David ◽  
Gustavo de Azambuja Pereira Filho ◽  
Albert Vincent Berthier Brasil

We report the first case of symptomatic thoracic spinal cord compression caused by postsurgical pseudomeningocele. A 49-year-old man sought treatment for progressive loss of strength in the lower extremities ten months after full neurological recovery for a thoracic (T11) intradural-extramedullary schwannoma. Magnetic resonance imaging revealed a postsurgical thoracic (T11-T12) pseudomeningocele. The surgical approach showed an inadequate dural closure with spontaneous cerebrospinal fluid fistula. The defect was sealed with suture, muscle and biological glue covering. The patient had a good recovery. Pseudomeningocele must take part of the differential diagnosis of myelopathy after thoracic spine surgery.


2020 ◽  
Vol 78 (10) ◽  
pp. 663-664
Author(s):  
Renan Ramon Souza LOPES ◽  
Larissa Soares CARDOSO ◽  
Franz ONISHI

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