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PubMed1999

beta-amyloid deposition and neurofibrillary tangle formation in the olfactory bulb in ageing and Alzheimer's disease.

Impaired olfaction, hyposmia or anosmia are part of the clinical phenotype in neurodegenerative disorders including Alzheimer's disease (AD). It has been proposed that the most severely affected areas are interconnected with the central olfactory system in contrast to the relative sparing of other sensory areas which lack olfactory connections. The pathology of the first synaptic relay in the olfactory pathway, the olfactory bulb (OB), has been studied in AD, but the results have been inconsistent. In order to define more fully the pathology of the OB, we analysed 15 AD and 15 control cases, using amyloid and tau immunohistochemistry on serial sections. This study demonstrates for the first time that all layers of the OB are severely affected in AD and in normal ageing. The principal effector cells of the OB, the mitral cells, developed neurofibrillary tangles (NFTs) both in AD and in controls. All the cases, with the exception of two of the controls, contained NFTs. Amyloid immunoreactivity was detected in diffuse, primitive, classical and compact deposits in AD, while five control cases contained mainly diffuse deposits. We did not find a correlation between amyloid deposition and NFT formation. Among the control cases, two contained neither amyloid nor NFTs, eight had NFTs but no amyloid and only five had both NFTs and amyloid. All the AD cases had NFT and amyloid deposition. Our data suggest that the earlier pathology in the OB is NFT formation and more than ten NFTs/section is compatible with 93.3% diagnostic accuracy for AD.

Aged↗
PubMed2001

The subcranial approach for the treatment of cerebrospinal fluid rhinorrhea: a report of 10 cases.

PURPOSE: Because of the likelihood of meningitis and other intracranial complications, optimal treatment for a cerebrospinal fluid (CSF) fistula is to close the leak. The neurosurgical approach to the management of CSF rhinorrhea has been by intracranial access. Extracranial approaches are now gaining acceptance as the preferred method for initial treatment of CSF leakage, because the success rates are reasonable, and the morbidity is lower. This report describes the results of using such an approach. PATIENTS AND METHODS: The extended subcranial approach was used in 10 patients with CSF rhinorrhea. Selection criteria included defects of the anterior skull base greater than 15 mm in diameter, defects not accessible by endoscopes, fistula sites that could not be localized preoperatively, and multiple and transverse fractures of the cribriform region. Follow-up ranged from 8 to 23 months, with a mean of 17 months. RESULTS: Resolution of rhinorrhea was achieved in 9 (90%) of the patients. Anosmia was the only postoperative complication, occurring in 8 patients. CONCLUSION: The authors conclude that the extended subcranial approach to the anterior skull base is a safe, versatile, and effective procedure for the surgical treatment of CSF rhinorrhea involving the anterior skull base.

Adolescent↗
PubMed2000

Electroencephalographic olfactometry (EEGO) analysis of odour responses in dogs.

Scientists in many fields have studied olfaction in dogs, but no simple method exists to study this function. The olfactory ability of dogs is used in many fields. However, the owners of the dogs have a frustrating experience because there is not an easy method to measure olfactory function. The purpose of this study was to examine the characteristics of olfactory function of the dog with electroencephalographic olfactometry (EEGO) recording. It was found that slow waves decreased and rapid waves increased in response to odours. From these results, it is suggested that the rapid waves of EEGO activity are important in determining a dog's olfactory ability. The EEGO recording is effective in diagnosing anosmia in the dog and is easier than a behavioural experiment. Additionally, this method may not cause distress to an animal.

Animals↗
PubMed2000

Sinusitis as the first indication of sarcoidosis an incidental finding in a patient with presumed 'odontogenic' sinusitis: case report.

Involvement of the paranasal sinuses and nose by sarcoidosis is uncommon, and has been reported in only 1-4% of patients with sarcoidosis. Clinical symptoms are nasal obstruction, epistaxis, nasal pain, discharge, anosmia or hyposmia, epiphora, and dyspnoea. We present a case of sarcoidosis in which sinusitis was the first clinical sign of the disease.

Diagnosis, Differential↗
PubMed2002

Pneumosinus dilatans of the frontal and ethmoidal sinuses: case report.

Enlargement of paranasal sinuses with air is rare with less than 50 cases reported in the medical literature. This craniofacial malformation was first described by Meyes in 1898 and its aetiology still remains unknown. Local pain, ocular alterations, anosmia, headache and cosmetic disturbances are the most common symptoms. We present a case of pneumosinus dilatans diagnosed in a male, 8 years of age. He presented with left fronto-orbital bossing that enlarged slowly until he was 12 years old, at which point its growth appeared to increase dramatically. Computerized tomography revealed an enlargement of the frontal and ethmoidal sinuses, with marked deformation of the anterior wall and of the roof of the left frontal sinus, as well as the roof of the ethmoid and upper sinus medial orbital wall. The sinus walls were of normal thickness. Access was via a bicoronal incision and osteotomy of the deformed fronto-orbital bossing. Reconstruction was undertaken with a periosteal flap and hydroxyapatite bone cement (Norian) following sinus mucosal stripping and obliteration of the sinus with fat. Six months postoperatively, the patient was without recurrence and had a good cosmetic result.

Air↗
PubMed2002

Symptomatic cranial fibrous dysplasias: clinico-radiological analysis in a series of eight operative cases with follow-up results.

Cranial fibrous dysplasias are rare and comprise less than one percent of all primary bone lesions. These painless progressively expanding destructive bone swellings produce cosmetic deformities (commonest), sino-orbital and auditive complications (less common), peripheral compressive cranial neuropathies (uncommon) and compressive central neurological manifestations (rarest). Until recently some of the primary dysplastic skull base lesions were either treated expectantly or excised incompletely. In a significant percentage of cases, these lesions were considered inaccessible. However, with the development of modern neuro-imaging (CT and MRI) and the applications of current skull base approaches, the more aggressive and radical surgical procedures are being undertaken, with a significant improvement in overall outcome. We present a series of 8 cases of fibrous dysplasia in the age group of 10-33 years with a mean age of 19.75 years. Four cases each had monostotic type and polyostotic type. All patients presented with mild to moderate headaches, and seven cases with some form of cosmetic deformity. In 4 patients there was a unilateral proptosis, while only two had visual deterioration. One patient had anosmia and one hearing impairment. In one case fibrous dysplasia was hemicranial, and in another case the disease was pancranial. Neuro-imaging studies have well delineated the extent of the lesions. In two patients with unilateral fronto-orbital fibrous dysplasia, the removal of dysplastic bone, autoclaving and remodeling and replacement were performed. In two patients of sphenoidal wing fibrous dysplasia a total excision was possible. In the remaining four patients, a partial excision was performed. Overall outcome was good. There was no mortality. One patient had recurrent post-operative extradural haematomas. The authors present the results of an analysis of eight surgically treated cases of symptomatic cranial fibrous dysplasias along with their long-term outcome and the lessons learnt from this series.

Adolescent↗
PubMed1977

[Refsum's syndrome (author's transl)].

Heredopathia atactica polyneuritiformis (Refsum's syndrome) is an autosomal recessively inherited lipidosis characterized by the following signs: peripheral hypertrophic polyneuropathy, cerebellar ataxia, atypical retinitis pigmentosa with night blindness and concentric limitation of the visual fields, anosmia, inner ear hearing disturbances, skeletal anomalies, ichthyotic skin changes, raised protein in the CSF without a cellular increase, and non-specific ECG changes. Biochemically it is a lipidosis with atypical increase of phytanic acid in blood, CSF, and tissues. The metabolic defect results from a degradation disturbance of exogenous phytanic acid. The report of a patient whose clinical picture plus chemical, neurophysiological and histological results led to the diagnosis of Refsum's syndrome is presented. Using a low phytol- and phytanic-acid diet a marked decrease of phytanic acid in the serum and an improvement of the clinico-neurological signs were observed.

Eye Manifestations↗
PubMed1999

[Gustatory olfactory function test with the Güttich technique: an evaluation of the clinical value].

BACKGROUND: Testing of retronasal olfactory sensitivity is routinely performed in many ENT-departments in German speaking countries. One of the most popular tests relates to an anecdotal report by Güttich [1]. In this test, liquids are placed on the patient's tongue; the patient is then asked to describe the aroma. Assuming that anosmic patients should not use other descriptors than "sweet," "sour," "salty," and "bitter", this test design is frequently applied in disability compensation settings. Surprisingly though, so far there are no studies regarding the sensitivity or specificity of this test in terms of the differentiation between hyposmic and anosmic patients. METHODS: The present investigation was performed in 50 anosmics, 42 hyposmics, and 13 normosmics. The diagnosis "hyposmia" or "anosmia" was based on 1. the patients' history, 2. psychophysical tests using an established test of olfactory function, the "Sniffin' Sticks," and 3. the recording of olfactory evoked potentials. RESULTS: We obtained the following major results: 1. In comparison to hyposmis and normosmics, anosmic patients had greater difficulty identifying the aromas. However, correct identification was not always possible for normosmics. 2. Regarding the differentiation between anosmic and hyposmic patients, the test's sensitivity was 86% with a specificity of 62%. This clearly limits routine clinical application of this test. CONCLUSIONS: Retronasal testing appears to be an elegant and simple means in order to screen olfactory function. However, the presently investigated approach is limited with regard to the discrimination between anosmic and hyposmic patients.

Adult↗
PubMed2000

Presentation and patterns of late recurrence of olfactory groove meningiomas.

The objective of this article is to present the recurrence pattern of olfactory groove meningiomas after surgical resection. Four patients, one female and three males, with surgically resected olfactory groove meningiomas presented with tumor recurrence. All patients underwent resection of an olfactory groove meningioma and later presented with recurrent tumors. The mean age at initial diagnosis was 47 years. All presented initially with vision changes, anosmia, memory dysfunction, and personality changes. Three patients had a preoperative MRI scan. All patients had a craniotomy, with gross total resection achieved in three, and 90% tumor removal achieved in the fourth. Involved dura was coagulated, but not resected, in all cases. Three patients were followed with routine head CT scans postoperatively, and none was followed with MRI scan. The mean time to recurrence was 6 years. Three patients presented with recurrent visual deterioration, and one presented with symptoms of nasal obstruction. Postoperative CT scans failed to document early tumor recurrence, whereas MRI documented tumor recurrence in all patients. Tumor resection and optic nerve decompression improved vision in two patients and stabilized vision in two. Complete resection was not possible because of extensive bony involvement around the anterior clinoid and inferior to the anterior cranial fossa in all cases. Evaluation of four patients with recurrent growth of olfactory groove meningiomas showed the epicenter of recurrence to be inferior to the anterior cranial fossa, with posterior extension involving the optic canals, leading to visual deterioration. This location led to a delay in diagnosis in patients who were followed only with routine CT scans. Initial surgical procedures should include removal of involved dura and bone, and follow-up evaluation should include formal ophthalmologic evaluations and routine head MRI scans.

Journal Article↗
PubMed2004

A case of Kallmann syndrome associated with Dandy-Walker malformation.

A 19-year-old man was admitted to our hospital for delayed puberty. At birth, he had macrocephalia and showed delayed physical and mental development. At 9 years of age, right cryptorchism was diagnosed. His parents had noticed that he could not recognize any smells since his infancy. Physical examination on admission revealed ocular hypertelorism, high myopia, high arched palate, and intermittent external strabismus. Sense of smell was scaled out by olfactometry. External genitalia were infantile. Neurological examination showed on IQ of 83, and mild truncal ataxia. Magnetic resonance imaging (MRI) showed a cystic distension of the IV ventricle, partial aplasia of the cerebellar vermis, elevation of the tentorium cerebelli, enlargement of the III ventricle, and agenesis of the corpus callosum. These findings revealed that the patient had Dandy-Walker malformation. The basal FSH, LH, and testosterone levels were all low compared with normal adult reference values. The serial LH-RH provocation tests showed stepwise LH and FSH elevation. After the fifth day of LH-RH administration, both LH and FSH responses clearly improved. Olfactory tracts were defective in MRI findings. These findings were consistent with hypogonadotropic hypogonadism of hypothalamic origin with anosmia, and the patient was therefore diagnosed with Kallmann syndrome. Sequence analysis of the KAL1 gene showed no mutation in the coding region. To our knowledge, this is the first case report of the coexistence of Kallmann syndrome and Dandy-Walker malformation in the same patient.

Adult↗
PubMed2004

Subcranial transnasal repair of cerebrospinal fluid rhinorrhea with free autologous grafts by the combined overlay and underlay techniques.

OBJECTIVE: To determine the efficacy of subcranial transnasal repair of cerebrospinal fluid rhinorrhea with free autologous grafts by the combined overlay and underlay techniques using the surgical microscope and/or endoscope. PATIENTS AND METHODS: Twenty patients with CSF rhinorrhea were included in this retrospective study. They were 13 males and 7 females. Their age ranged from 7 to 62 years (mean: 39.35). The etiologies of the leak were iatrogenic in 10 cases, spontaneous in 5 cases, traumatic in 4 cases and one case was associated with meningeo-encephalocele. Preoperative nasal endoscopic examination, computed tomography (CT) with intrathecal non-ionic contrast and magnetic resonance imaging (MRI) were done when indicated. Endoscopic and/or microscopic repair of the CSF fistula was done by a combination of both underlay and overlay repair with free autologous grafts as follows: Gelfoam with fibrin glue, strips of fat, facia lata, Gelfoam with fibrin glue (underlay), septal cartilage, Gelfoam with fibrin glue and strips of fat (overlay). RESULTS: Complete closure of the leak was achieved in all patients. In one case of spontaneous CSF leak which was operated endoscopically, the leak recurred 6 months postoperatively and ceased spontaneously after a month with conservative medical treatment. No major complications were seen and no patients developed meningitis or postoperative anosmia. CONCLUSION: Subcranial transnasal repair with free autologous grafts by the combined overlay and underlay techniques using the endoscope or surgical microscope is a safe and successful method of treating CSF leaks, provided that the CSF leak is precisely located and the site can be reached with the endoscope or surgical microscope.

Adolescent↗
PubMed2005

[Preservation of olfaction in bifrontal craniotomies for lesions of the anterior and middle cranial fossa].

OBJECTIVE: The preservation of the olfactory tract during bifrontal approach for lesions located in the frontal skull base and the supra- and parasellar region has not previously been investigated. METHODS: In our study 12 patients underwent surgery for various lesions via the basal subfrontal route. All patients were observed prospectively, olfaction testing was performed pre- and postoperatively by an independent otorhinolaryngologist. RESULTS: We obtained complete preservation of the olfactory tracts and normal postoperative olfaction in all 12 cases. CONCLUSION: The bifrontal approach allowed a bilateral, wide operative field with excellent orientation and views of important structures. The shortcoming of this method, damage of the olfactory tracts and postoperative anosmia can be overcome.

Adolescent↗
PubMed2005

Analysis of different surgical approaches for the treatment of cerebrospinal fluid rhinorrhea.

The current treatment method for cerebrospinal fluid (CSF) rhinorrhea is surgical repair of the fistula. The aim of this study was to analyse different surgical approaches used for the treatment of CSF rhinorrhea regarding several preoperative and postoperative variables to determine the optimal method in these patients. Patients' charts were retrospectively reviewed to get the required data. Twenty-six patients who underwent different types of surgical approach for the treatment of CSF rhinorrhea were included in the study. Patients who had extensive comminuted fractures of the anterior cranial base and additional brain injury besides CSF rhinorrhea, mostly as a result of gunshot injuries, underwent craniotomy (n = 14). Osteoplastic frontal sinusotomy was used in two patients with a dural defect located at the posterior wall of the frontal sinus. Uncomplicated CSF fistulas in ten patients, located at the anterior and posterior ethmoid roof and in the sphenoid sinus, were closed with an endonasal endoscopic approach. Postoperative success rate was higher (97 % for intracranial approach, 100 % for extracranial external and endonasal endoscopic approach) for all techniques. Anosmia was the most frequent permanent complication (n = 5), seen after craniotomy. In conclusion, endonasal endoscopic approach can be preferred for the closure of uncomplicated CSF fistula, located at the anterior or posterior ethmoid roof and in the sphenoid sinus, due to its minimal postoperative morbidity. Uncomplicated CSF fistula, located at the posterior wall of frontal sinuses can be repaired extradurally with osteoplastic frontal sinusotomy. Intracranial approaches should be reserved for more complicated CSF rhinorrhea which results from extensive comminuted fractures of the anterior cranial base and is accompanied with intracranial complications.

Adolescent↗
PubMed2006

[Experiences with the subfrontal approach to manage extensive fractures of the frontal skull base].

BACKGROUND: Surgical management of multiple traumatized patients with head and neck trauma is highly individualized and depends on a number of factors including etiology, intracranial pressure, concomitant injuries, patient age and the possibility of an interdisciplinary procedure. Severe head and neck trauma are often connected with fractures of the frontal skull base or nasoethmoido-orbital complex and CSF leakage. If there is suspicion of a CSF leakage surgical management to cover the dura-defect is essential. An intradural approach is necessary in case of concomitant intradural injuries while primary extradural access provides excellent exposure of the rhinobasis with low morbidity and good results. METHODS AND MATERIAL: We report about our surgical experiences of 55 patients with severe frontobasal trauma, who were operated between 1/1999 and 11/2003. The subfrontal approach according to Raveh we had chosen in 20 patients which were operated by an interdisciplinary team together with the neurosurgeons. With an average follow up of 36 month we report about early and late complications. RESULTS: 19/20 patients showed sufficient coverage of the CSF leakage, once a revision surgery was necessary. Finally this patient had also an unobjectionable coverage of the CSF leakage. We saw no major complication like meningitis or brain abscess, intracerebral haematoma or surgical injury of the orbital wall. The most important complication was an anosmia, which depending on the extension of the approach results in any patients. CONCLUSIONS: Our results show that the subfrontal approach is a reliable method to explore extensive frontal dural defects and to reconstruct fractures of the frontal skull base without additional trauma to the frontal lobe.

Adolescent↗
PubMed2006

[Subcranial resection of malignant tumors infiltrating the anterior skull base].

BACKGROUND: Survival and quality of life after subcranial resection of malignant tumors infiltrating the anterior skull base should be evaluated. METHODS: Data were acquired retrospectively from patient charts and by telephone interview. Quality of life was assessed with the EORTC QLQ30 and H&N35 modules. RESULTS: From 1996 to 2004, 19 patients (mean age 52 years, 4 woman, 15 men) were surgically treated via a subcranial approach. Fifteen patients suffered from advanced carcinoma, 3 from advanced esthesioneuroblastoma, and 1 patient had a fibrosarcoma. Fifteen patients received adjuvant radiotherapy. During the mean follow-up period of 44 months (12-109 months), 6 patients died, 1 unrelated to the tumor. The probability to survive 5 years was 50 %, the mean survival time was 72 months. Anosmia was reported by 18 of 19 patients. A tension pneumocephalus was observed in 2 patients, one with lethal outcome, decreased vision in 1 patient, loss of vision in 1, persisting diplopia in 1, deep wound infections in 2, and CSF leak in 2 patients. Quality of life was assessed on the average 36 months following end of therapy and did not differ substantially from other patients with head-neck malignancies. CONCLUSION: Most, malignant tumors infiltrating the anterior skull base can be treated curatively. The treatment outcome is well comparable to other head and neck tumors of corresponding stage.

Adenocarcinoma↗
PubMed1997

[Sarcoidosis of the paranasal sinuses as the etiology of therapy refractory dacryocystitis].

BACKGROUND: Sarcoidosis is a chronic systemic disease of unknown etiology characterized by non-caseating granulomatous inflammation of various organs. The clinical findings when nasal mucosa is involved include nasal crusting, epistaxis, pain and/or anosmia, nasal polyps, or submucosal nodularity. CASE REPORT: We describe a case of sarcoidosis of the paranasal sinuses. In a 33-year-old man, the first symptom was therapy resistant putrid dacryocystitis. Further there had been symptoms of chronic sinusitis. A CT scan revealed swelling of the mucosa in the anterior ethmoid, frontal recess, and maxillary sinus. As the disease obviously affected the region of the nasolacrimal duct with consecutive stenosis of this canal, we opted for endoscopic sinus surgery. A few days after this endoscopic procedure, the dacryocystitis had healed. RESULTS: The histopathological examination showed the typical picture of sarcoidosis that had its primary manifestation in the paranasal sinuses. According to these findings further explanation of the patient was necessary. The diagnosis of sarcoidosis was confirmed by lavage and biopsy material of bronchoscopically suspect lung areas. Consequently, the patient underwent a long-term corticoid therapy and today, 10 months after the surgical intervention, the patient is free of symptoms. Follow-up endoscopy of the paranasal sinuses showed no further pathological findings. CONCLUSION: Our case demonstrates that the first manifestation of sarcoidosis can be in the paranasal sinuses.

Adult↗
PubMed1995

[Olfactory evoked potentials and contingent negative variation in expert assessment of disordered sense of smell].

Normally, the sense of smell is assessed by means of traditional subjective tests (sniff test, gustatory smell test, and trigeminus test). When results are inconclusive, an objective smell test is indicated. Simultaneously registering olfactory evoked potentials (OEP) and contingent negative variation (CNV) permits evaluation of both odor perception and odor discrimination. We can even objectively assess the false olfactory sensations in parosmia patients who are unable to discriminate between different odors. In this study, the results of 59 patients with olfactory disorders are presented. Head trauma and upper respiratory infections were the most common causes of the patients' complaints. In twelve cases, lack of cooperation and patient unrest prevented us from evaluating the data. In anosmia, the objective results agreed completely with the patient's subjective assessment. In hyposmia and parosmia, the results agreed with the patient's subjective assessment in most cases; here we were able to arrive at a more specific diagnosis in several cases. The objective smell test can be used to supplement subjective methods and thus provide more reliable assessment of olfactory disorders.

Attention↗
PubMed1983

[Pulsatile GnRH substitution in Kallmann's syndrome in women].

Anosmia and primary amenorrhoea are guiding symptoms of Kallmann's syndrome (olfacto-genital syndrome) in which agenesis of the olfactory lobe is associated with congenital defects in the mediobasal region of the hypothalamus, thus preventing a sufficient GnRH synthesis. In three patients with Kallmann's syndrome, the secretion of gonadotropins on bolus injection of 25 micrograms GnRH was comparable with prepubertal reaction. In one patient, the hypophyseal function was normalized, and ovulatory cycles were induced by pulsatile GnRH substitution via a portable computerized pump (Zyklomat). Pregnancy occurred. The duration of treatment required to induce ovulation was identical during two subsequent treatment cycles, contrary to observations in functional hypothalamic amenorrhoeas. The marked ovarian reaction shows that even if there is no endogenous GnRH secretion, a pulsatile dose of less than 20 micrograms seems to be sufficient.

Amenorrhea↗