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PubMedDate not supplied

Sinusitis in an allergist's office: analysis of 200 consecutive cases.

Sinusitis affects up to 14% of Americans. Traditionally, most patients with sinusitis are evaluated and treated by either primary care physicians or otolaryngologists. In order to gain information regarding the characteristics at presentation and the outcome of treatment of sinusitis by an allergist, the records of 200 consecutive patients seen at the Institute for Asthma and Allergy at the Washington Hospital Center for chronic sinusitis were reviewed. The most common presenting symptoms were nasal congestion, postnasal drip, purulent rhinorrhea, headache, cough, facial pressure, anosmia or hyposmia, hypogeusia, and throat clearing. Initial abnormal physical exam findings included abnormal transillumination, purulent secretions, nasal mucosal swelling, nasal polyps, and nasal crusting. Treatment included 4 weeks of oral antibiotics, nasal corticosteroids, nasal lavage, and topical decongestants. All of the presenting symptoms (23-75% of the patients) and signs (50-84% of patients) improved with medical management. Patients have been followed for 1 to 27 months, with a mean of 6 months, and 6% have required surgery, with one complication of cerebrospinal fluid leak. These findings indicate that medical management of chronic sinusitis in an allergist's office is effective.

Adolescent↗
PubMedDate not supplied

Severe chemotherapy-induced parosmia.

BACKGROUND: Smell and taste disorders are among the side effects of chemo- and radiotherapy. Although direct radionecrosis of the salivary glands and the taste buds might explain the chemosensory problems after radiotherapy, the olfactory and gustatory complaints seen after chemotherapy remain unexplained. The patients reporting olfactory symptoms rarely complain about qualitative olfactory disorders such as parosmia or phantosmia. Quantitative olfactory loss such as anosmia and hyposmia seem to be more frequent. METHODS: We present the case of a 63-year-old woman with chemotherapy-induced parosmia leading to severe nutrition and appetite problems resulting in a life-threatening weight loss. RESULTS: With the aid of a simple nose clip the parosmia could be abolished and oral food intake became possible again. Parosmia resolved gradually over an observation period of 9 months, in parallel to an increase of olfactory sensitivity. The patient progressively gained appetite and weight. CONCLUSION: Parosmia can occur as a severe and potentially life-threatening complication of chemotherapy. This rare presentation of parosmia illustrates the importance of olfactory testing with an adequate recognition of the underlying problem and a consecutive treatment.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Immunohistochemical method for the diagnosis of olfactory disturbance.

To confirm the diagnosis and determine the cause of the olfactory disturbance, we used an immunohistochemical method to examine biopsy specimens of the olfactory mucosa from a patient who complained of anosmia after head injury. Neuron-specific enolase immunoreactivity was found in the olfactory vesicles and dendrites of the receptor cells in the olfactory epithelium. S-100 protein immunoreactivity was found in the ductal cells of Bowman's gland in the olfactory epithelium and in the acinar cells of Bowman's gland in the lamina propria. This suggests that the olfactory receptor cells and Bowman's gland were normal. The olfactory disturbance in this patient was not caused by nerve transection due to the head injury, but by already existing chronic sinusitis. Immunohistochemical methods are useful for diagnosing olfactory disturbance when used in combination with biopsy of olfactory mucosa.

Adolescent↗
PubMed1994

Radiologic assessment of diseased mucosa of the maxillary sinus after functional endoscopic sinus surgery.

We attempted to evaluate postoperative mucosal changes and symptomatic improvement in 99 patients who underwent functional endoscopic sinus surgery from September 1991 through August 1992. The patients were divided into 2-, 4-, 6-, and 12-month postoperative groups. Thickness of the maxillary sinus mucosa measured at the midpoint of the lateral sinus wall on a follow-up ostiomeatal unit computed tomogram (OMU CT) was compared with that of preoperative OMU CT. Postoperative endoscopic findings of the maxillary sinus and changes in presenting symptoms such as rhinorrhea, nasal obstruction, facial pain, headache, anosmia, epiphora, and referred otalgia were analyzed. Improvement in the diseased mucosa of the maxillary sinus, as evaluated on OMU CT, was observed in 69.7% of the patients, and such mucosal changes did not differ significantly among 2-, 4-, 6-, and 12-month follow-up groups. However, apparent mucosal changes exceeding marginal improvement was observed in 32.3% of the patients. The overall symptomatic improvement rate was 57.9% and improvement in endoscopic findings was observed in 46.3% of the patients. Although there was some discrepancy between radiologic and symptomatic improvement rates, symptomatic improvement was significantly related with radiologic improvement. It is suggested that removal of obstructive lesion in the ostiomeatal area might be beneficial in a seemingly early symptomatic improvement, but complete healing of the maxillary sinus mucosa as assessed by OMU CT might take longer than 12 months.

Adolescent↗
PubMed1995

Optic nerve compression from a basal encephalocele.

A woman developed headaches, transient visual obscurations, anosmia, and decreased visual acuity. Ocular examination showed bilateral pulsatile proptosis and disc edema with choroidal folds. Standardized ophthalmic echography showed absence of bony orbital roofs, prominent dural pulsations, direct apposition of brain parenchyma and orbital tissues, and echographic signs suggesting bilateral optic nerve compression. CT and MRI showed a large defect in the floor of the anterior cranial fossa. The cribriform plate, both orbital roofs, and sphenoid bones were displaced by a large basal encephalocele. Clinical improvement followed reconstruction of the anterior cranial fossa and decompression of both optic nerves.

Adult↗
PubMed1990

Genetics of olfactory behavior in Drosophila melanogaster.

We have used a behavioral genetic approach to identify six X-linked loci which specify olfaction in Drosophila melanogaster. Mutations in five of these genes lead to partial anosmias affecting responses either to aldehydes (olfA, olfB, olfE and olfF) or to acetate esters (olfC). Only one of the mutants obtained in our screening (olfD) resulted in a insensitivity to several different odorants. olfA, olfE and olfC map close together in a small region of the chromosome between 7C and 7D. The alleles at the olfC locus fall into two phenotypic classes according to their responses to different acetate esters. The two groups of olfC alleles interact in-trans.

Animals↗
PubMed1984

Differential diagnosis of male central hypogonadism by short-term pulsatile LHRH administration.

UNLABELLED: In order to obtain a clinically valuable differentiation of central hypogonadism (CH), 18 male patients, including 13 with permanent gonadotropin deficiency (GD), age 14.3-41 yrs, bone age 8.5-19 yrs, and 5 with constitutional delay (CD) of puberty (age 15.3-20, bone age 12.5-15 yrs) were studied. Among the GD patients, there were 4 with anosmia (Kallmann's syndrome, KS), 6 with idiopathic hypopituitarism (HP) and 3 with isolated hypogonadotropic hypogonadism (HH). The spontaneous nocturnal plasma profile of LH and FSH was compared with that during pulsatile LHRH infusion (5 micrograms iv every 90 min) by a portable micropump (Zyklomat) for 36 hrs. The pituitary-gonadal response was evaluated by calculating the LH and FSH slopes during pulsatile LHRH and by comparing plasma testosterone (T) before and after. While spontaneous nocturnal FSH pulses were absent in all patients, between 1 and 4 significant LH pulses were seen in all CD but in none of the GD patients. In all patients, mean FSH was significantly higher during pulsatile LHRH than during sleep. During pulsatile LHRH administration, significantly increasing FSH responses were seen in all KS and HP patients, but in only 2 of the 5 CD and in none of the 3 HH patients. In all CD boys (mean testis vol, 8.6 ml), T rose markedly during pulsatile LHRH (mean, from 168 to 414 ng/dl), whereas in all GD patients (mean testis vol, 2 ml) it did not (19 vs 27 ng/dl). CONCLUSIONS: Pulsatile LHRH for 36 hrs differentiates CD and GD far more exact than was previously possible.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Preservation of olfaction in anterior craniofacial approaches.

Through the combined efforts of neurosurgeons, head and neck surgeons, and craniofacial surgeons, the standard transbasal approach to the frontal fossa has been modified to include removal of the orbital roofs, nasion, and ethmoid sinuses. This approach has been combined further with facial disassembly procedures to provide extensive midline exposure to the midface and clival region. Extended frontal approaches, however, necessitate removal of the crista galli and sectioning of the olfactory rootlets with the associated risk of anosmia, cerebrospinal fluid (CSF) leak, and the need for complex reconstruction of the frontal floor. To avoid these problems, the authors have modified the technique of handling the cribriform plate to preserve the olfactory unit. Circumferential osteotomy cuts are made around the cribriform plate to allow an en bloc removal with its attachment to both the dura and underlying mucosa. Opening of the dura is avoided and the cribriform bone is used to reconstruct the floor. Four patients underwent this approach, for treatment of an angiofibroma in three and a fibrosarcoma in one. The mean follow-up period was 7 months. No patients developed a CSF leak, and within 8 weeks olfaction had returned in all patients. There was no other associated morbidity. These data suggest that this modification of the transbasilar approach can alleviate extensive reconstructive procedures and CSF leaks while preserving olfaction.

Adolescent↗
PubMed1994

Superficial siderosis of the central nervous system after brachial plexus injury. Case report.

Superficial siderosis is a rare condition characterized by deposition of hemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. It is associated with cerebrospinal fluid abnormalities consistent with recurrent bleeding into the subarachnoid space. The usual symptoms are hearing loss, ataxia, spastic paraparesis, sensory and sphincter deficits, and mental deterioration. A case is presented of severe superficial siderosis of the central nervous system in a 51-year-old man who had suffered a brachial plexus injury at the age of 20 years. The diagnosis was made by means of magnetic resonance imaging 16 years after the initial symptoms, which comprised bilateral hearing loss and anosmia. Subarachnoid bleeding was due to traumatic pseudomeningocele of the brachial plexus, a very unusual cause of superficial siderosis. This case is interesting insofar as the surgical treatment prevented further bleeding and possibly progression of the disease.

Brachial Plexus↗
PubMed2005

The extended direct endonasal transsphenoidal approach for nonadenomatous suprasellar tumors.

OBJECT: The extended transsphenoidal approach, which requires a bone and dural opening through the tuberculum sellae and posterior planum sphenoidale, is increasingly used for the treatment of nonadenomatous suprasellar tumors. The authors present their experiences in using the direct endonasal approach in patients with nonadenomatous suprasellar tumors. METHODS: Surgery was performed with the aid of an operating microscope and angled endoscopes were used to assess the completeness of resection. Bone and dural defects were repaired using abdominal fat, collagen sponge, titanium mesh, and, in most cases, lumbar drainage of cerebrospinal fluid (CSF). Twenty-six procedures for tumor removal were performed in 24 patients (ages 9-79 years), including two repeated operations for residual tumor. Gross-total removal could be accomplished in only 46% of patients, with near-gross-total removal or better in 74% of 23 patients (five of eight with craniopharyngiomas, six of seven with meningiomas, five of six with Rathke cleft cysts, and one of two with a dermoid or epidermoid cyst); a patient with a lymphoma only underwent biopsy. Of 13 patients with tumor-related visual loss, 85% improved postoperatively. The complications that occurred included five patients (21%) with postoperative CSF leaks, one patient (4%) with bacterial meningitis; five patients (21%) with new endocrinopathy; and two patients (8%) who needed to undergo repeated operations to downsize suprasellar fat grafts. The only permanent neurological deficit was anosmia in one patient; there were no intracranial vascular injuries. CONCLUSIONS: The direct endonasal skull-base approach provides an effective minimally invasive means for resecting or debulking nonadenomatous suprasellar tumors that have traditionally been approached through a sublabial or transcranial route. Procedures in the supraglandular space can be performed effectively with excellent visualization of the optic apparatus while preserving pituitary function in most cases. The major challenge remains developing consistently effective techniques to prevent postoperative CSF leaks.

Adolescent↗
PubMed2000

Odorant quality perception: a metric individual differences approach.

Perceptual spaces, in which similar stimuli are located close to each other and dissimilar stimuli are located far apart, have aided in the understanding of the physiological and psychological bases for sensory quality coding. Differences in perception between individuals should be reflected by differences in the spatial relationships between stimuli. If the dimensionality of the perceptual space is small (e.g., color space), individual differences that reflect specific pathologies are readily apparent from visual inspection. On the other hand, if the dimensionality of the perceptual space is large (as is proposed for odor space), visual inspection alone may not reveal individual differences in quality perception. The present work presents an information-theory-based method for quantifying individual differences in quality perception from perceptual confusion matrices. The ability of this method to quantify individual differences in quality perception is shown in a hypothetical example of specific anosmia. Finally, the method is applied to the examination of intrasubject consistency of odorant quality perception.

Adult↗
PubMed1999

[Local injection of dexamethasone acetate suspension into the nasal mucosa in cases of olfactory disturbance].

Local injections of suspended steroid solution into the nasal mucosa were performed for 102 patients with olfactory disturbances. Dexamethasone acetate suspension in a concentration of 4 mg/0.5 ml was injected into the mucosa of the nasal septum on each side every 2 weeks for a total of 8 times. Mecobalamin and ATP were also administrated in a daily dose of 750 micrograms and 300 mg, respectively. Of the 102 olfactory disturbance patients, 32 patients had chronic sinusitis, 24 patients had common cold, 14 patients had allergic rhinitis, 9 patients had head trauma and the remaining 23 patients the reason for the disturbance was unknown. Using a visual analogue scale, improvements were found in 63.7%. We assessed the effect of this therapy on the threshold of olfactory sensation and perception using a T & T olfactogram. The overall mean values of olfactory sensation and perception were significantly improved; from 4.75 +/- 1.44 (SD) to 3.01 +/- 2.13 (SD) and from 5.30 +/- 0.88 (SD) to 4.19 +/- 1.60 (SD), respectively. All but the cases of head trauma showed significant improvement. Overall, 44 (43.1%) of the 102 patients were defined as having had a positive olfactory response to this treatment (an improvement in the average perception olfactory score of > = 1.0). In cases of chronic sinusitis, 15 (46.9%) of the 32 patients were positive. 12 (50.0%) of the common cold 24 patients, 7 (50.0%) of the 14 allergic rhinitis patients, 2 (22.2%) of the 9 head trauma patients and 11 (47.8%) of the 23 unknown etiology patients were positive for this treatment. Patients with severe olfactory dysfunction and anosmia showed a lower response than the patients who were mildly to moderately affected. Patients showing no response in the Alinamin intravenous test showed also lower improvement rates than those showing a normal response. Interestingly, 8 (27.6%) of 29 patients with a normal appearance of the olfactory clefts and no response for the Alinamin test responded positively to this treatment. This meant that the olfactory disturbance might be due to the existence of edema surrounding the olfactory nerve, not of the olfactory cleft. The improvement rate of patients treated within one year from the onset of these olfactory disturbance was better than the rate of those over one year. The present new therapeutic modality is very useful as a treatment which showed only be performed by otolaryngologists.

Adenosine Triphosphate↗
PubMed1993

[A case of superficial siderosis of the central nervous system with total deafness].

Superficial siderosis of the central nervous system (SSCN) in a disease characterized by chronic deposition of hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves. Previously the diagnosis of SSCN could only be made at autopsy or during a neurosurgical procedure. Now, however, a diagnosis of SSCN can be made non-invasively by magnetic resonance imaging (MRI). We present the case of a 50-year-old male with SSCN accompanied by bilateral sensorineural hearing loss which gradually progressed to total deafness over a seven year period. This patient also had associated bilateral caloric weakness with episodes of severe recurrent headaches over the two preceding years. The deafness and gait disturbance, which were his chief complaints, were followed by other neurological manifestations including pyramidal tract signs, anosmia, and ageusia. High-field MRI on T-2 weighted images of the CNS showed diffuse marginal hypointensity of the cerebrum, brain stem, and cerebellum. Atrophy of the cerebellum and brain stem was also apparent. Low signal intensity along the proximal segment of the acoustic nerve and the facial nerve was noted from the cistern to the internal auditory canal. Neither bilateral transtympanic promontory nor round window electrical stimulation elicited any sound sensation. In this case it was thought that the acoustic nerve alone or both the acoustic nerve and the cochlea were affected by this disease. Consequently, cochlear implantation was not indicated. The source of bleeding into the subarachnoid space could not be detected despite thorough examination. This diagnosis will be made only by physicians who are aware of this rare entity and have knowledge of the characteristic clinical pathology.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗
PubMedDate not supplied

Atypical presentation of a patient with both kallmann syndrome and a craniopharyngioma: case report and literature review.

OBJECTIVE: To describe an unusual presentation of a patient with Kallmann syndrome, without the typical eunuchoid features, who had additional hormonal abnormalities caused by a craniopharyngioma. METHODS: This patient's clinical features, endocrine evaluation, and treatment are described, and the literature regarding Kallmann syndrome is reviewed. The expected phenotype of Kallmann syndrome is contrasted with this case presentation. A literature search was also performed to determine whether the combination of craniopharyngioma and Kallmann syndrome had been described previously. RESULTS: A 23-year-old man had a suprasellar tumor in conjunction with hypogonadotropic hypogonadism and growth hormone deficiency. Subsequently, he was also noted to have anosmia, a cleft palate, and bilateral olfactory bulb hypoplasia. His height was less than his calculated midparental height and exceeded his arm span. Defective neuronal migration in Kallmann syndrome is caused by absence of adhesion proteins needed for cellular, neuronal, and axonal guidance. This results in failure of olfactory and gonadotropin-releasing hormone neurons to complete normal migration. Defective migration can also cause midline craniofacial abnormalities, renal agenesis, and cardiovascular defects. Arachnoid cysts have been reported in two patients with Kallmann syndrome, although whether a migration defect underlies their occurrence is speculative. No prior reports of craniopharyngioma in a patient with Kallmann syndrome could be identified. CONCLUSION: It is postulated that although this patient had Kallmann syndrome, he did not present with a eunuchoid body habitus because of concomitant growth hormone deficiency caused by his craniopharyngioma. Although midline craniofacial abnormalities have been seen in patients with Kallmann syndrome, this patient's craniopharyngioma seems more likely to be coincidental, rather than being one of the developmental anomalies that are part of the spectrum of this syndrome.

Adult↗
PubMed2003

Schwannoma of the sinonasal tract: a clinicopathologic and immunohistochemical study of 5 cases.

CONTEXT: Peripheral nerve sheath tumors are soft tissue neoplasms rarely encountered in the nasal cavity and paranasal sinuses. OBJECTIVE: To describe the clinicopathologic and immunohistochemical features of a series of schwannomas of the sinonasal tract. DESIGN: Surgical pathology files were searched for the diagnosis "sinonasal schwannoma." All histologic documents and clinical data were reviewed. Immunohistochemistry was performed on paraffin-embedded tissue with antibodies to S100 protein, epithelial membrane antigen, CD34, and MIB-1. RESULSTS: Five cases of sinonasal schwannoma were retrieved; patients included 3 women and 2 men, aged 20 to 56 years. Three cases were located in the ethmoid sinus. Clinical symptoms were nonspecific (nasal obstruction, epistaxis, and anosmia). All tumors were treated with conservative surgical resection. Pathologic examination showed a spindle cell proliferation without encapsulation in all cases. No cytologic atypia was seen, and the mitotic activity was low (<3 mitotic figures/10 high-power fields). Immunohistochemistry showed diffuse positivity with S100 protein and negativity with CD34 and epithelial membrane antigen. MIB-1 staining was low (1%-5% of tumor cell nuclei stained). During the follow-up (median, 6 years), no recurrence or metastasis was observed. CONCLUSIONS: Schwannoma is a very unusual tumor of the sinonasal tract and is associated with nonspecific symptoms. Histologically, sinonasal schwannomas differ from schwannomas of other locations by their lack of a peripheral capsule and possible ulceration of the epithelial covering. Sinonasal schwannomas are treated with conservative surgical resection and have an excellent prognosis.

Adult↗
PubMed1999

Detection of KAL-1 gene deletion with fluorescence in situ hybridization.

We investigated the molecular cytogenetic status of two unrelated boys and their family members because they had features consistent with Kallmann syndrome but normal karyotypes. The first patient was a 6-year-old boy who suffered from ichthyosis, bilateral cryptorchidism, hyposmia, and neurologic disorders including mirror movements of the hands and nystagmus. Mild to moderate mental retardation was also noted in this boy, his mother, and maternal grandmother. Fluorescence in situ hybridization (FISH) study using probes for Kallmann (KAL), steroid sulfatase, and ocular albinism type 1 all showed nullisomy on Xp22.3 in this patient, and hemizygosity in his older sister, mother, and maternal grandmother. The second patient was a 1-year-old boy who had micropenis, cryptorchidism, and hypoplastic scrotum since birth. Family study disclosed a 28-year-old maternal uncle with cryptorchidism, lack of secondary sexual characteristics, and anosmia. FISH showed only the KAL gene deletion. Polymerase chain reaction analysis also showed an absence of the KAL-1 sequence. FISH is a useful tool for the detection of KAL-1 deletion in people with normal karyotypes but features consistent with Kallmann syndrome.

Child↗
PubMed1999

Physiologic and genetic insights into the pathophysiology and management of hypogonadotropic hypogonadism.

Initiation and maintenance of reproductive capacity is contingent upon the pulsatile secretion of gonadotropin-releasing hormone (GnRH) from the hypothalamus. While GnRH deficiency manifests itself clinically as hypogonadism, the time of onset of the defect in GnRH secretion (i.e. before or after the onset of puberty) determines the clinical presentation that is further modified by the severity of the defect. GnRH deficiency can be associated with structural lesions that interfere with the normal pattern of GnRH secretion through compression or destruction of critical areas of the median eminence of the hypothalamus. Idiopathic hypogonadotropic hypogonadism (IHH) has been described and is characterized by isolated GnRH deficiency in the absence of a hypothalamic or pituitary lesion. Typically, patients with hypogonadotropic hypogonadism and anosmia have been given the diagnosis of Kallmann's syndrome and those with normal olfaction, IHH. However, both entities can be present in the same family, demonstrating variability in phenotypic expression and raising questions about the usefulness of this distinction. GnRH deficiency may be inherited via autosomal dominant, autosomal recessive and X-linked modes of inheritance. Specific defects in a number of genes have now been found in association with GnRH deficiency in the human and including the KAL gene, the DAX gene and the GnRH receptor gene, but not the gene encoding GnRH itself. While these mutations account for only a small fraction of cases, genetic studies not only provide promise for future treatment, but also insights into the complexity of the GnRH neuronal system. The treatment of GnRH deficiency is highly successful whether designed for steroid hormone replacement or for fertility. The challenge in the treatment of these abnormalities is in the early and accurate diagnosis and the use of replacement therapies that are as close to physiologic as possible.

Adolescent↗
PubMed1999

[An electrophysiological evaluation of the role of the olfactory analyzer in brain integrative activity].

Summary electrical activity of olfactory bulb and amygdala of unrested rats with reversible anosmia during conditioning with application of light and odor as conditioned stimuli was studied. It was shown, that recovery of capacity for reception of odor wasn't connected with appearance in electrical activity of investigated structures of high-frequency high-amplitude components. Additional investigations of neuronal isolation of olfactory bulb and discovery of correlative changes of power of high-frequency components in dependency on level of common excitation give the possibility to consider this rhythm as one of the mechanisms of nonsensory modulator influences of olfactory analyser.

Amygdala↗