Cases reported "Nervous System Neoplasms"

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1/129. Antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors.

    BACKGROUND: Antiamphiphysin antibodies react with a 128-kd protein found in synaptic vesicles.They were first described in patients with paraneoplastic stiff-man syndrome and breast cancer, but studies suggest that they can also occur in patients with other tumors and neurological disorders. OBJECTIVE: To determine if antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors. patients AND methods: Of 2800 serum samples tested by routine immunohistochemical procedures on sections of paraformaldehyde-fixed rat brain for the detection of autoantibodies associated with paraneoplastic neurological syndromes, 5 were selected because of labeling suggestive of antiamphiphysin antibodies and subsequently confirmed by the results of Western blot analysis using recombinant amphiphysin protein. Controls consisted of 40 patients with various nonparaneoplastic neurological diseases; 101 patients with cancer but without paraneoplastic neurological syndrome; 9 patients with small cell lung cancer, anti-Hu antibodies, and paraneoplastic neurological syndrome; 3 patients with M2-type antimitochondrial antibodies but no neurological disorder; and 30 normal subjects. RESULTS: Of the 5 patients with antiamphiphysin antibodies, patient 1 had sensory neuronopathy, encephalomyelitis, and breast cancer; patient 2 had limbic encephalitis, and small cell lung cancer was detected in the mediastinum after 24 months of follow-up; patient 3 had encephalomyelitis and ovarian carcinoma; and patients 4 and 5 had lambert-eaton myasthenic syndrome and small cell lung cancer (patient 4 subsequently developed cerebellar degeneration). None of the 5 had stiffness. Two patients (Nos. 2 and 4) had antimitochondrial antibodies. The two patients (Nos. 4 and 5) with lambert-eaton myasthenic syndrome had antibodies directed against the voltage-gated calcium channel, and patient 2 subsequently developed anti-Hu antibodies. In the controls, antiamphiphysin antibodies were detected by Western blot analysis in 3 of 8 patients with anti-Hu antibodies, but in none of the other groups. CONCLUSIONS: These data indicate that antiamphiphysin antibodies are not specific for one type of tumor or one neurological syndrome and can be associated with other neural and nonneural antibodies. The simultaneous association of several antibodies in some patients suggests multimodal autoantibody production. ( info)

2/129. skin conductance responses in paediatric Harlequin syndrome.

    We report a novel and simple application of skin conductance response (SCR) testing for diagnosis of a new-onset iatrogenic Harlequin syndrome in an infant. Isolated ipsilateral facial pallor, complicated by thermally induced systemic sympathetic vasodilatation, and preferential lateral decubitus positioning, mimics harlequin colour change. Correct diagnosis as Harlequin syndrome with facial sympathetic interruption was demonstrated by diminution of SCR. ( info)

3/129. Autonomic nerve tumour with skeinoid fibres: ultrastructure of skeinoid fibres examined by quick-freezing and deep-etching method.

    A case of gastrointestinal autonomic nerve tumour with skeinoid fibres (SFs) of the jejunum in a 79-year-old Japanese man, was examined by the quick-freezing and deep-etching (QF-DE) method. The tumour consisted of spindle cells with immunohistochemical reactions for vimentin, NSE and CD34. Electron microscopically, features of the neural cells of the myenteric plexus were observed. The QF-DE method demonstrated intercellular meshwork structures, consisting of thin filaments (7-15 nm), with granular deposits. Fully developed parts of the deposits formed nodular aggregates composed of irregularly surfaced thick fibrils (30-48 nm) with a tendency to linear arrangement (SFs). We detected many interconnecting thin filaments (ICTFs) between the SFs, which were pre-existing components in the meshwork, avoiding the granular deposits. The focal thickening formed by the connection between SFs and ICTFs revealed a periodicity typical of SFs (33-45 nm). We conclude that SFs are formed by decoration of the granular deposits along pre-existing intercellular meshwork structures. ( info)

4/129. brachial plexus catheter reservoir for the treatment of upper-extremity cancer pain: technical case report.

    OBJECTIVE AND IMPORTANCE: Infiltration of the brachial plexus with anesthetics can provide relief of upper-extremity pain from invasive cancer. Because the analgesia is short-lived, however, repeated invasive treatments are necessary. We describe the implantation of a catheter reservoir system, in which anesthetic injections through a subcutaneous port resulted in anesthetic infiltration of the brachial plexus. CLINICAL PRESENTATION: A 47-year-old Hispanic man with squamous cell carcinoma of the larynx had undergone surgical resection, radiation treatment, and chemotherapy. Two years later, he had locally recurrent disease involving the brachial plexus, neck, and chest wall. The patient's pain was minimally responsive to narcotics, which also caused severe nausea and anorexia. TECHNIQUE: The brachial plexus was localized percutaneously with a needle electrode stimulator. Contrast injection under fluoroscopy confirmed entry into the plexus sheath. With use of the Seldinger technique, two Silastic catheters were placed within the brachial plexus and attached with a "Y" connector to a reservoir. The patient experienced complete relief of upper-extremity pain after a test injection with xylocaine. Thereafter, serial injections of bupivacaine with triamcinolone at 1-week intervals provided complete pain relief. After the treatments were initiated, the patient reported improved sleep and an improvement in his quality of life. CONCLUSION: A catheter reservoir system for brachial plexus analgesia can provide safe and effective analgesia for upper-extremity pain. This technique negates the need for repeated invasive procedures and avoids the complications of neurolysis. ( info)

5/129. Cervical sympathetic chain schwannomas masquerading as carotid body tumors.

    Cervical sympathetic chain (CSC) schwannoma is a rare neurogenic tumor that can mimic a carotid body tumor (CBT). Two male patients aged 33 and 49 years old were referred for a cervical mass thought to be a CBT. Both patients were found to have an asymptomatic, pulsatile, nontender mass located at the level of the angle of the mandible. Both patients were neurologically asymptomatic and cranial nerves were normal. Both patients underwent neck exploration and resection of the mass involving the CSC. Postoperatively, a Horner's syndrome was present. Histopathology confirmed both tumors to be schwannomas of the CSC. At follow-up, both patients are asymptomatic with no recurrence. The main imaging criterion to differentiate a CBT from a CSC schwannoma is the lack of hypervascularity of the latter. Malignant transformation is extremely rare. Tumor excision gives excellent results with no local recurrence. Horner's syndrome is an expected postoperative complication. ( info)

6/129. costello syndrome: a cancer predisposing syndrome?

    costello syndrome is a specific MCA/MR syndrome mainly characterized by dysmorphic facial features, peculiar biphasic growth pattern, motor and mental retardation, ectodermal anomalies involving skin and nails, and age dependent development of nasal and perianal papillomata. heart malformations and/or hypertrophic cardiomyopathy are frequently observed. We report a 4-year-old girl with costello syndrome who developed an intrathoracic ganglioneuroblastoma. In previous reports two patients with ectodermal tumours have been described, a ganglioneuroblastoma of the adrenal gland and an epithelioma. This third report suggests that neural crest neoplasia may be a significant risk factor for children with costello syndrome. ( info)

7/129. Unusual eustachian tube mass: glomus tympanicum.

    SUMMARY: A case of recurrent glomus tympanicum presenting with epistaxis is described. CT and MR imaging revealed a homogeneously enhancing mass extending along the entire course of the eustachian tube, with a portion protruding into the nasopharynx. Glomus tumors tend to spread along the path of least resistance and may extend into the eustachian tube. The unique imaging appearance should place a glomus tumor high on the list of differential diagnoses. ( info)

8/129. carpal tunnel syndrome due to an intraneural perineurioma in a 2-year-old child.

    We report a 2-year-old girl with carpal tunnel syndrome due to a large intraneural perineurioma that required resection and nerve reconstruction. ( info)

9/129. Ancient schwannoma masquerading as a thyroid mass.

    Schwannomas are benign, encapsulated nerve sheath cell neoplasms. Cervical sympathetic chain (CSC) schwannomas are rare, with less than 50 cited cases in the literature. CSC schwannomas may mimic a number of parapharyngeal masses. We report a rare variant, "ancient" schwannoma, which presented cytologically and radiologically as a thyroid mass. This is the first report of a CSC schwannoma mimicking a thyroid mass and the first report of an ancient schwannoma of the CSC. ( info)

10/129. Sacrococcygeal ganglioneuroma.

    Ganglioneuromas are benign slow-growing masses that can be treated with complete surgical extirpation without any adjuvant therapy. Such lesions involving the sacrococcygeal region are exceedingly rare. The authors present the case of a 70-year-old woman with a sacrococcygeal ganglioneuroma treated by total en bloc resection. This patient also had a previous coccygeal fracture. To the authors' knowledge, there are no other reports of ganglioneuroma in association with a history of trauma. ( info)
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