1/69. Neurosurgical implications of carney complex.OBJECT: The authors present their neurosurgical experience with carney complex. carney complex, characterized by spotty skin pigmentation, cardiac myxomas, primary pigmented nodular adrenocortical disease, pituitary tumors, and nerve sheath tumors (NSTs), is a recently described, rare, autosomal-dominant familial syndrome that is relatively unknown to neurosurgeons. neurosurgery is required to treat pituitary adenomas and a rare NST, the psammomatous melanotic schwannoma (PMS), in patients with carney complex. Cushing's syndrome, a common component of the complex, is caused by primary pigmented nodular adrenocortical disease and is not secondary to an adrenocorticotropic hormone-secreting pituitary adenoma. methods: The authors reviewed 14 cases of carney complex, five from the literature and nine from their own experience. Of the 14 pituitary adenomas recognized in association with carney complex, 12 developed growth hormone (GH) hypersecretion (producing gigantism in two patients and acromegaly in 10), and results of immunohistochemical studies in one of the other two were positive for GH. The association of PMSs with carney complex was established in 1990. Of the reported tumors, 28% were associated with spinal nerve sheaths. The spinal tumors occurred in adults (mean age 32 years, range 18-49 years) who presented with pain and radiculopathy. These NSTs may be malignant (10%) and, as with the cardiac myxomas, are associated with significant rates of morbidity and mortality. CONCLUSIONS: Because of the surgical comorbidity associated with cardiac myxoma and/or Cushing's syndrome, recognition of carney complex has important implications for perisurgical patient management and family screening. Study of the genetics of carney complex and of the biological abnormalities associated with the tumors may provide insight into the general pathobiological abnormalities associated with the tumors may provide insight into the general pathobiological features of pituitary adenomas and NSTs.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
2/69. Really painful double vision.A 67-year-old woman with a history of chronic headache and recent removal of two squamous cell lesions from her forehead presented with left facial pain and diplopia. A diagnosis of tolosa-hunt syndrome was made based on clinical presentation and imaging studies. When the patient did not respond to steroids, further studies were done, including biopsy, which revealed perineural spread of squamous cell carcinoma.- - - - - - - - - - ranking = 0.33333333333333keywords = syndrome (Clic here for more details about this article) |
3/69. Fibrolipoma of the median nerve: a case report and review of the literature.A 38 year-old patient presented with right median nerve distribution paresthesias. Electrodiagnostic studies confirmed severe carpal tunnel syndrome. A palmar mass prompted a magnetic resonance imaging scan, which suggested a fibrolipoma of the median nerve. Carpal tunnel release resulted in resolution of preoperative pain and paresthesias. We review the literature dealing with this primary nerve tumor.- - - - - - - - - - ranking = 0.33333333333333keywords = syndrome (Clic here for more details about this article) |
4/69. Haemangioblastoma of a cervical sensory nerve root in Von Hippel-Lindau syndrome.Spinal haemangioblastomas are rare, accounting for only about 7% of all central nervous system cases. The case of a 40-year-old woman with a haemangioblastoma arising solely from a cervical sensory nerve root is presented. At operation via a cervical laminectomy, it was possible to resect the tumour en masse with the sensory ramus, by extending the laminectomy through the exit foramen for C6. Haemangioblastomas are commonly intramedullary, and have only been reported in this location on one previous occasion. The patient has Von Hippel-Lindau syndrome and a history of multiple solid tumours. The possible role of the Von Hippel-Lindau tumour suppressor gene in the pathogenesis of these neoplasms is discussed.- - - - - - - - - - ranking = 1.6666666666667keywords = syndrome (Clic here for more details about this article) |
5/69. Squamous cell carcinoma with perineural invasion presenting as a Tolosa-Hunt-like syndrome: a potential pitfall in diagnosis.PURPOSE: To describe a case of perineural invasion resulting from squamous cell carcinoma of forehead. methods: Case report. RESULTS: Perineural invasion resulting from squamous cell carcinoma of the periocular skin can present as a Tolosa-Hunt-like syndrome with lack of radiologic findings on magnetic resonance imaging (MRI) in its early stages. CONCLUSION: A high level of suspicion for perineural invasion is required when assessing multiple cranial nerve palsies in patients with a history of cutaneous malignancy, despite negative sequential MRI. Perineural invasion must be ruled out by a biopsy of the involved nerves, whenever possible, before empiric therapy with systemic steroids is contemplated.- - - - - - - - - - ranking = 1.6666666666667keywords = syndrome (Clic here for more details about this article) |
6/69. Recurrent (nonfamilial) hemangioblastomas involving spinal nerve roots: case report.OBJECTIVE AND IMPORTANCE: Spinal nerve root hemangioblastomas are rare and are reported mainly in patients with von Hippel-Lindau (VHL) syndrome. The pathogenesis of so-called nonfamilial lesions is virtually unknown. We discuss, mainly from a molecular perspective, a unique patient with sporadic, recurrent hemangioblastomas restricted to spinal nerve roots. CLINICAL PRESENTATION: A 53-year-old man who had had a surgically corrected lumbosacral meningomyelocele presented on at least three occasions during a 17-year period with multifocal capillary hemangioblastomas involving spinal nerve roots. On each occasion, tumors appeared on a different nerve root, with the majority located in the midcervical segments. The patient had no clinical features or family history of VHL syndrome. TECHNIQUE: To obtain a clearer understanding of the pathogenesis of this unusual case and its relationship to VHL syndrome, molecular analysis of the VHL gene was performed by use of complete sequence analysis and loss of heterozygosity studies on deoxyribonucleic acid derived from the patient's blood leukocytes and three separately resected hemangioblastomas. CONCLUSION: Germ-line molecular analysis performed on all three exons in the VHL gene coding region did not indicate that any mutations were present. loss of heterozygosity analysis of deoxyribonucleic acid from the three hemangioblastoma resections showed normal heterozygosity in the 3p25-26 region. Complete VHL gene sequence analysis did not demonstrate a somatic mutation in the coding region of the VHL gene in any of the three tumors, thereby supporting the loss of heterozygosity data that a molecular event directly involving the VHL gene may not be the causative factor in their tumorigenesis.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
7/69. Findings of exploration of a vein-wrapped ulnar nerve: report of a case.A 54-year-old woman underwent a revision procedure after failure of 3 previous procedures for recalcitrant cubital tunnel syndrome. She underwent neurolysis and vein wrapping of the ulnar nerve during the fourth procedure. Two years later she developed a neuroma of the medial antebrachial cutaneous nerve necessitating a fifth procedure. At the time of neuroma relocation we noted that the vein was intact around the ulnar nerve and that there was no scarring between the vein and nerve.- - - - - - - - - - ranking = 0.33333333333333keywords = syndrome (Clic here for more details about this article) |
8/69. A posterior tibial nerve neurilemoma unrecognized for 10 years: case report.OBJECTIVE AND IMPORTANCE: neoplasms of peripheral nerves can be obscured, especially during the early phase. The author reports a patient with a posterior tibial nerve neurilemoma (schwannoma). For a decade, the tumor was misdiagnosed as nonspecific S1 radiculopathy and psychogenic chronic pain syndrome. The patient's presentation and initial management are unique. CLINICAL PRESENTATION: A 40-year-old woman reported severe left foot and calf pain, numbness, and weakness. The symptoms were evident during three pregnancies, and they gradually progressed. The neuropathic pain was protracted, despite implantation of a dorsal column stimulator and administration of a wide variety of medications and therapies. The symptoms were unresponsive to both inpatient and outpatient treatments, which resulted in a misdiagnosis of psychogenic pain for more than a decade. Diagnostic scans obtained by computed tomography, ultrasonography, and nuclear scintigraphy confirmed a popliteal fossa mass. INTERVENTION: A high, large posterior tibial nerve neurilemoma was found intraoperatively, positioned just below the sciatic nerve bifurcation with extensive degenerative features and hemorrhages. Surgical resection provided immediate recovery. CONCLUSION: Peripheral nerve tumors are rarely acknowledged clinical entities. Chronic unexplained foot and calf pain and a positive Tinel's sign should raise suspicion of posterior tibial nerve neurilemoma. Even in patients who have had such tumors for a decade, surgical resection remains the treatment of choice.- - - - - - - - - - ranking = 0.33333333333333keywords = syndrome (Clic here for more details about this article) |
9/69. sciatica due to malignant nerve sheath tumour of sciatic nerve in the thigh.Malignant peripheral nerve sheath tumour (MPNST) is a rare malignant neoplasm arising from the supportive non-neural component of the peripheral nerves. An unusual case of pain and weakness of the foot and calf muscles due to a giant MPNST of the sciatic nerve in the posterior compartment of the thigh is presented. The patient was already investigated as a case of sciatica due to a lumbar disc disease with a negative magnetic resonance imaging and then unsuccessfully operated elsewhere twice, with a misdiagnosis of tarsal tunnel syndrome. Neurosurgical referral prompted a diagnostic magnetic resonance study of the thigh, revealing the lesion, which was completely excised microsurgically with total relief in the pain and partial improvement in the weakness and sensations in the sole of the foot.- - - - - - - - - - ranking = 0.33333333333333keywords = syndrome (Clic here for more details about this article) |
10/69. Negative-antibody paraneoplastic syndrome complicating small cell carcinoma.The neurological paraneoplastic syndromes represent nonmetastatic complications of cancer and may affect several levels of the nervous system. They are thought to be immunologically-mediated. The syndrome predates the diagnosis of cancer by months to years in two thirds of cases. We report the case of a female patient presenting with a cerebellar syndrome and a sensory neuronopathy on a background of severe weight loss. We searched for occult malignancy and later diagnosed her to be suffering from a paraneoplastic syndrome secondary to small cell carcinoma of the lung. Paraneoplastic antibodies were negative. She was subsequently treated with chemotherapy.- - - - - - - - - - ranking = 2.6666666666667keywords = syndrome (Clic here for more details about this article) |
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