[Case of Fisher syndrome following sphenoiditis].

Fisher syndrome is a subtype of Guillain-Barré syndrome that is characterized by the three chief symptoms of acute-onset external ophthalmoplegia, ataxia, and loss of tendon reflexes. Herein, we report a case of Fisher syndrome encountered by us,with sphenoiditis as the antecedent infection. The patient was a 39-year-old man who visited a local doctor after developing the symptom of rhinorrhea, followed three days later by diplopia. Cranial MRI suggested sphenoid sinusitis, and the patient was referred to our hospital with suspected rhinogenous intraorbital complication. Neurological findings included bilateral abduction deficit, which was considered unlikely to be an ocular manifestation of an intraorbital complication of right unilateral sphenoiditis. In addition, the reduction of the tendon reflexes in the limbs and ataxia suggested the diagnosis of Fisher syndrome, which was then confirmed based on the albuminocytologic dissociation observed on cerebrospinal fluid examination and a positive blood test result for anti-GQ1b antibody. The symptoms were attributed to the production of antibodies against antigens expressed on the oculomotor, trochlear, abducens and other nerves (anti-GQ1b antibody) due to the antecedent infection. The present patient was thought to have developed Fisher syndrome following sphenoiditis as an antecedent infection. Care must be taken to differentiate this condition from rhinogenous intraorbital complication.

Induction of immunological antitumor effects by adenovirus-mediated gene transfer of B7-1 in a murine squamous cell carcinoma cell line.

OBJECTIVE: To evaluate the antitumor immune effects of B7-1 gene expression mediated by adenoviral vectors against squamous cell carcinoma. Transfection of the costimulatory molecule B7-1 gene into certain murine tumors increases antitumor immunity and suppresses tumor growth. DESIGN: In vitro and in vivo study. INTERVENTIONS: A murine squamous cell carcinoma cell line, KLN205, was infected with adenoviral vectors carrying either B7-1 (AdB7) or LacZ (AdCL) genes. Infected cells were injected subcutaneously into the flanks of DBA/2 mice. MAIN OUTCOME MEASURES: The growth of tumors infected with adenviral vectors was measured. RESULTS: AdB7-infected cells grew significantly slower than AdCL-infected cells in vivo, while there was no significant difference in the growth rates between the 2 groups in vitro. Moreover, significant growth suppression of rechallenged noninfected parental cells was observed in the mice immunized with AdB7-infected cells but not in those immunized with AdCL-infected cells. CONCLUSION: These results suggest that the B7-1 gene has therapeutic potential for immunotherapy against head and neck squamous cell carcinoma.

[A case of orbital apex syndrome caused by localized invasive aspergillosis successfully treated with voriconazole].

A 71-year-old man with left periorbital pain and diplopia was hospitalized for evaluation and treatment. He had a past history of untreated diabetes mellitus. Shortly after admission, the patient experienced rapid onset of visual loss in the left eye. MRI and CT showed a lesion expanding from the left orbital apex to the left pterygopalatine fossa. Invasive aspergillosis was diagnosed by open biopsy of intrasinus mucosa via the left maxillary sinus. The patient was treated with voriconazole, an antifungal agent, and marked improvements in left periorbital pain and eye movement were subsequently obtained, although visual acuity was not recovered. This is the first report documenting the clinical utility of voriconazole for sino-orbital invasive aspergillosis.

A paranasal tumor associated with tumor-induced osteomalacia.

BACKGROUND: Tumor-induced osteomalacia (TIO) is an extremely rare paraneoplastic syndrome associated with mesenchymal tumors in the craniofacial region. METHODS: We treated a 24-year-old Japanese woman with paranasal sinus tumor and a low serum phosphate level who presented with nasal obstruction and systemic bone fractures. Serum fibroblast growth factor 23 (FGF-23) was measured before and after surgery. RESULTS: After surgery, her symptoms were resolved and the serum phosphate and FGF-23 levels became normalized. CONCLUSION: FGF-23 is thought to be a useful diagnostic marker in patients with craniofacial tumors associated with multiple systemic fractures.

Quality of life after neck dissection.

OBJECTIVE: To assess the impact of modifications to radical neck dissection on postoperative quality of life. DESIGN: Cross-sectional study using a self-administered neck dissection questionnaire and an arm abduction test. SETTING: Department of Otolaryngology-Head and Neck Surgery, Kobe University Hospital. PATIENTS: Seventy-four patients who had undergone neck dissection for the treatment of head and neck cancer. MAIN OUTCOME MEASURES: Arm abduction test results and responses to questions on quality of life related to neck dissection. RESULTS: Forty-one patients underwent bilateral neck dissections, and 33 patients underwent unilateral neck dissection. Level V nodes were dissected in 74 necks. Among them, the spinal accessory nerve (SAN) was resected in 29 necks. Patients who had neck dissections that spared the SAN had better shoulder function. When the SAN was preserved, patients without dissection of level IV and V nodes had better scores on measures of pain and constriction of the neck. Sacrifice of the sternocleidomastoid muscle and/or the SAN had a significant effect on daily activities, work, and leisure. The arm abduction test scores and answers to questions regarding shoulder function were significantly correlated. CONCLUSIONS: Modifications to radical neck dissection contribute to improvements in the postoperative quality of life after neck dissection. A multicenter study using the arm abduction test and questionnaire used in this study is currently in progress to further evaluate the impact of modifications to radical neck dissection on quality of life after surgery.

Distribution and impact of lymph node metastases in oropharyngeal cancer.

CONCLUSIONS: In the treatment of oropharyngeal cancers, possible metastases to retropharyngeal lymph nodes (RPLNs) should be taken into account, especially in tumors arising in the lateral wall and/or posterior wall. Patients with multiple positive neck nodes must have intensified adjuvant therapy, especially when they have extracapsular spread (ECS). OBJECTIVE: To develop optimal treatment strategies for oropharyngeal cancers, we retrospectively analyzed the lymph node metastases of oropharyngeal squamous cell carcinoma. PATIENTS AND METHODS: Between 1988 and 2003, 77 patients with previously untreated oropharyngeal squamous cell carcinoma underwent neck dissections. RESULTS: Among the patients with tumor arising in the lateral wall or posterior wall, retropharyngeal nodes were involved in 29% (11/38), while RPLN metastasis was not observed in patients with tumors arising in the superior wall or anterior wall. The survival rate of patients with two or fewer positive lymph nodes was significantly better than that of patients with three or more positive lymph nodes (p < 0.05). The survival rate of the patients who had ECS was significantly worse than that of the patients who had lymph node metastases but not ECS (p < 0.05). There was no significant difference between the survival rates of the patients with and without RPLN metastases.

Immunohistochemical study on distribution of mast cell phenotypes in human laryngeal mucosa: evidence for laryngeal type I allergy.

It is controversial whether or not type I allergic reactions can occur in the human laryngeal mucosa. To clarify this issue, we studied the distribution of mast cell phenotypes in the human laryngeal mucosa using the immunohistochemical staining method with antihuman tryptase and chymase antibodies. A large number of mast cells are present in the superficial layer of subepithelial connective tissue (SECT) of the epiglottis, arytenoid, and subglottis. Although mast cells containing both tryptase and chymase are predominant in the deep layer of the SECT, the majority of mast cells containing tryptase alone are located in both the epithelial layer and the superficial layer of the SECT. We conclude that the human laryngeal mucosa has the potential to induce type I allergic reaction.

Adenovirus-mediated gene transfer in olfactory epithelium and olfactory bulb: a long-term study.

OBJECTIVES: We sought to study the spatiotemporal gene expression mediated by adenoviral vector in the olfactory pathways. METHODS: The replication-defective adenoviral vector AxCALacZ, which encodes the enzyme Escherichia coli beta-galactosidase, was applied to mouse olfactory epithelium by intranasal instillation. RESULTS: The LacZ gene product, beta-galactosidase, was expressed not only in the olfactory receptor neurons and their axons, but also in the olfactory bulbs. The first evidence of anterograde labeling was observed at postinfection day (PID) 2. At PID 3, beta-galactosidase was strongly expressed in olfactory nerve axons, as well as their terminal glomeruli, in the olfactory bulbs. beta-Galactosidase expression persisted up to PID 90, and there was a significant decrease in the number of labeled neurons at PID 30. CONCLUSIONS: These results suggest possible long-term effects of adenovirus-mediated gene transfer on the olfactory neurons, as well as the olfactory bulbs.

Notch expression in developing olfactory neuroepithelium.

Notch genes encode receptors for signaling pathways that regulate neurogenesis in various tissues. To better understand the roles of Notch genes in olfactory neurogenesis, we studied the expression of Notch family in developing mouse olfactory epithelium. In the earliest stage of olfactory development, Notch1 was observed in the mesenchyme lateral to the olfactory placode. During the developmental stage, Notch1 was mainly observed around the basal membrane, while Notch3 was observed in the lower compartment of the olfactory neuroepithelium. Notch2 was not detected during the entire observation period. As the olfactory neuroepithelium grew mature, both Notch1 and Notch3 gradually disappeared. These results suggest distinct roles of Notch1 and Notch3 in the neurogenesis of the peripheral olfactory system.

A rare spontaneous temporal meningoencephalocele with dehiscence into the pterygoid fossa.

We present an extremely rare case of basal meningoencephalocele that protruded into the left pterygoid fossa from the middle cranial fossa. After a 30-year-old woman experienced a generalized tonic-clonic seizure, computed tomography (CT) scanning showed a large bone defect of the left middle cranial base. A mass lesion with low signal intensity on T1- and high signal intensity on T2-weighted magnetic resonance imaging (MRI) was visualized in the left pterygoid fossa. A biopsy was performed through the transantral approach. Because cerebrospinal fluid (CSF) was observed to flow from the mass lesion, it was diagnosed as a meningoencephalocele. CT showed bone defects in the skull base and a well-circumscribed expansile mass lesion. MRI demonstrated that the brain parenchyma extended into a CSF-filled meningocele. These findings demonstrate that CT and MRI provide adequate information for the diagnosis of meningoencephaloceles.

Submucous electrocautery following submucous resection of turbinate bone--a rationale of surgical treatment for allergic rhinitis.

OBJECTIVE: Since the majority of the key elements such as trigeminal nerves, parasympathetic nerves, nasal glands, and blood vessels targeted by histamine and leukotrienes are found in the lamina propria of the nasal mucosa, its selective electrocautery has a rationale to improve the symptoms of allergic rhinitis with preservation of epithelial layer. To achieve the above goal, we performed the submucous electrocautery of the lamina propria (SECLP) following the submucous resection of the inferior turbinate bone (submucous turbinectomy: SMT). This paper discusses the efficacy of this procedure for the patients with persistent perennial allergic rhinitis. METHODS: An intranasal initial incision was made along the piriform aperture. The mucoperiosteum was elevated from the inferior turbinate bone followed by its complete resection. The SECLP was performed by applying a high-frequency coagulation current with a ball tip electrode, which was inserted into the mucoperiosteal sack after the completion of the SMT and was drawn forward on the medial surface of the mucoperiosteum with drawing a wavy line. We performed this surgery in 43 patients with perennial allergic rhinitis who were refractory to pharmacotherapy or were reluctant to take medicine. Symptoms, macroscopic intranasal findings, the results of allergic tests, nasal resistance, mucociliary function with saccharin, and the number of mast cells were compared pre- and postoperatively. RESULTS: The patients exhibited satisfactory improvement in symptoms only with a few crust formations. The macroscopic intranasal findings and allergic tests improved after surgery. Saccharin transport time remained normal. The number of anti-tryptase positive mast cells significantly decreased in the epithelial layer and in the superficial layer of the lamina propria of the postoperative inferior turbinate mucosa. CONCLUSION: The SECLP following the SMT is evaluated to be a useful surgical modality for allergic rhinitis with preservation of the nasal mucosal function.

Simple reconstruction with titanium mesh and radial forearm flap after globe-sparing total maxillectomy: a 5-year follow-up study.

BACKGROUND: Reconstruction of eye globe-sparing total maxillectomy defects is one of the major challenges to reconstructive surgeons. In 1994, the authors developed an uncomplicated and easy reconstructive method, where a titanium mesh is applied for the support of orbital contents, a radial forearm free flap for covering the mesh and the cheek lining, and an obturator prosthesis for palatal and dental rehabilitation. METHODS: Five patients who underwent primary reconstruction with the authors' method after globe-sparing maxillectomy with loss of the orbital floor from 1994 to 1999 and who were followed up for more than 5 years were retrospectively reviewed for (1) the presence of diplopia, (2) the shape of the reconstructed orbital floor assessed by coronal section magnetic resonance imaging, and (3) the presence of infection/exposure of the titanium mesh. RESULTS: Only one of the five patients developed slight diplopia. Coronal magnetic resonance imaging showed that the orbital floor restored with titanium mesh had in all cases maintained a proper shape and position for more than 5 years. No infection or exposure of the titanium mesh had developed in any of the cases, despite exposure to irradiation of not less than 30 Gy. All the patients had well-retentive obturator prostheses. CONCLUSION: This long-term follow-up study demonstrated that the authors' method attained a long-lasting successful outcome functionally and is the method of choice for reconstruction after globe-sparing total maxillectomy.

Efficacy and feasibility of cisplatin-based concurrent chemoradiotherapy for nasopharyngeal carcinoma.

OBJECTIVE: To investigate the efficacy and feasibility of a cisplatin-based concurrent chemoradiotherapy (CRT) protocol based on Intergroup Study 0099 for nasopharyngeal carcinoma (NPC). METHODS: Sixteen patients with stage II-IVB NPC were treated with a protocol of cisplatin-based concurrent CRT and adjuvant chemotherapy from 1998 to 2002. Three courses of cisplatin (80 mg/m2) were scheduled during 70 Gy of radiotherapy (RT), and two agents of adjuvant chemotherapy (FP regimen: cisplatin 80 mg/m2 and 5-fluorouracil 800 mg/m2/day by 4-day continuous infusion) were challenged. Overall survival (OS) and relapse-free survival (RFS) rates were calculated by the Kaplan-Meier method. RESULTS: Median follow-up duration was 45 months. Both 3-year OS and RFS rates were 81%. Proportions of patients who tolerated each scheduled treatment were 94% for RT, 63% for concurrent chemotherapy and 38% for adjuvant chemotherapy. CONCLUSIONS: Our protocol of the cisplatin-based concurrent CRT followed by adjuvant chemotherapy consisting of FP regimen was effective for Japanese patients with NPC. However, the doses and numbers of cycle of chemotherapy need to be modified because of the low compliance rate. Larger numbers of data accumulation and/or multi-institutional trials may be warranted to confirm the efficacy of this protocol.

Invasion of the skull base by carcinomas: histopathologically evidenced findings with CT and MRI.

The depth and extent of the invasion of the skull base by a tumor are the most critical information for successful en bloc resection of the tumor. The only means available for the evaluation of these factors are CT or MRI images. In order to clarify the ability of these imaging modes to delineate the invasion of the skull base, preoperative images of ten patients who underwent en bloc resection of skull base tumors at Kobe University Hospital were compared with the histopathological findings of the resected specimens. CT proved to be superior to MRI for evaluating bone destruction of the skull base. On the other hand, MRI provided more useful information about intracranial invasion than CT. As a hypertrophic linear shadow on Gd-enhanced MRI represented dural invasion or thickened dura mater adjacent to the tumor, this technique should be taken into consideration to determine the dural resection. We concluded that preoperative evaluation of the depth of skull base invasion by both CT and Gd-enhanced MRI is essential for planning complete tumor resection.

Quantitative analysis of expression of NeuroD, GAP43 and receptor tyrosine kinase B in developing mouse olfactory neuroepithelium.

OBJECTIVE: The mammalian olfactory neuroepithelium (OE), which harbors olfactory receptor neurons (ORNs), has the unusual characteristic of continuous neurogenesis throughout its lifetime. This unique feature provides an excellent model for neuronal differentiation. Recently, we found dual-phase expression of NeuroD, a member of the basic helix-loop-helix transcription factor family, in the developing mouse OE, suggesting multiple roles of NeuroD during the development of mammalian ORNs. MATERIAL AND METHODS: In order to better understand the molecular mechanism of the development of ORNs, we performed quantitative analysis of expression of NeuroD, GAP43 and receptor tyrosine kinase B (TrkB), as well as 5-bromo-2'-deoxyuridine (BrdU)-labeled cells, in the developing mouse OE from gestational Day 10 to postnatal Day 28. RESULTS: During the embryonic period, NeuroD expression is mostly confined to the basal compartment. During the neonatal period, NeuroD expression is detected in two compartments: the middle compartment and the basal compartment. GAP43-expressing cells were located between these two NeuroD-positive layers. TrkB-expressing cells were located above the NeuroD-positive layer in the middle compartment. As the mice grew, the numbers of NeuroD-expressing cells and BrdU-labeled cells in the basal compartment significantly decreased, while the number of NeuroD-expressing cells in the middle compartment gradually increased. The number of TrkB-expressing cells dramatically increased. The number of GAP43-expressing cells also gradually increased. However, the relative proportion of GAP43 cells decreased as the OE developed. CONCLUSION: NeuroD is a useful molecular marker for studying olfactory neurogenesis.