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Kallmann's syndrome. A case report.

A patient with hypogonadotrophic hypogonadism and anosmia (Kallmann's syndrome) presenting as delayed puberty is described. The clinical, hormonal and testicular histological features are noted. The basic principles of treatment are discussed.

Adolescent↗

[The ENT specialist's responsibility in the diagnosis of the olfactory meningeoma (author's transl)].

The principal symptoms associate with olfactory groove meningeomas are anosmia and headache which lead the patient to an ENT specialist. Frontal sinusitis, migraine and neuralgia are the most frequent incorrect diagnosis. Positive radiological changes are found frequently on the plain films of patients with these olfactory tumors, even up to 70% according to the literature. It is important to bear this in mind regarding olfactory groove meningeomas to avoid mistakes in diagnosis.

Brain Neoplasms↗

[One-stage reconstruction of intraorbital fractures by means of plate osteosynthesis (author's transl)].

Considering the multiplicity of sequelae of central midface fractures (rhinoliquorrhea, anosmia, impediment to the drainage of the superior sinuses and to the lacrimal fluid, traumatic telecanthus, dish-face etc.) the importance of rhinosurgical treatment as the foremost procedure is shown. The small-fragment sets according to Champy as stabilizing elements in operations of the interorbital space (Converse) and its osseous supports are mentioned. Both the technical and the functional advantage of establishing a stable osseous trajectory in the rhinobasis after fractures is emphasized. Stable osteosynthesis as a primary therapeutic procedure enables the rhinosurgeon to find a remarkable synthesis of both functional and aesthetic tasks in this problematic area. The stable fixation makes the rhinosurgical treatment a preliminary one for other surgical procedure to follow.

Bone Plates↗

[Ocular complications in chronic pansinusitis--report of two cases].

Case 1. A 38-year-old male was admitted with an 8-month history of progressive visual disturbance in his right eye. He had a past history of operations for paranasal sinusitis 24 years and 5 years prior to admission. Neurological examination revealed right anosmia, left hyposmia and vision in his right eye reducing to light perception. Case 2. A 61-year-old male was admitted with complaint of headache and a 2-month history of deteriorating vision in his right eye. On admission, vision in his right eye was found to be reduced to hand motions and associated with a concentric contraction of the visual field. Bilateral hyposmia and the right exophthalmos were also noticed. Plain x-ray films, tomography and especially CT scan were useful to demonstrate the definite inflammatory changes of the paranasal sinuses and the extension of the lesion beyond the confines of the sinuses. In both cases, external ethmoidectomy and sphenoidectomy with removal of the infected granulation tissue from the sinuses were performed, but neither mucocele nor pyocele was found. Vision improved postoperatively. Previous reports about ocular complications of infection in the paranasal sinuses without mucocele or pyocele are few. Early diagnosis of ocular complications of sinusitis is important. Once the diagnosis established, a timely surgical approach is essential to prevent visual loss.

Adult↗

[Role of species-specific acoustic signals in the "homing" orientation of intact and olfactory deprived kittens].

Vocal responses of lactating cats were recorded and used to investigate home orientation in 1-30 days old kittens. Two types of species-specific acoustic signals were chosen and used in acoustically-guided homing. Type 1 comprised fricative signals below 1.5 kHz of low intensity and long duration. Type 2 comprised intense tonal calls with 2-4 resonant frequency components below 4 kHz. Presentation of tape-recorded signals 1 and 2 through the speaker placed into the home site increased the ability of the kittens to return to the nest. Zn-induced anosmia eliminated home orientation. Presenting of the signals 1 and 2 to anosmic kittens resulted in the recovery of homing. Acoustically-guided behaviour first appears at 6-9 days of age and fully matures by 25-30 days.

Age Factors↗

[Electrophysiological study of sex pheromone reception in the boar, Sus scrofa].

Electroolfactograms were recorded in isolated olfactory epithelium of pigs, both females and castrated males, of various age. The boar pheromone, 5 alpha-androst-16-en-3-one, elicited negative electrical responses up to 2 mV in many preparations. The threshold response corresponded to as low as 10(-9) g of the pheromone on a filter paper. The specific anosmia to the boar pheromone was discovered in few preparations, where responses to the pheromone were absent and the sensitivity to common odour substances (butyl acetate, camphor, butyric acid, etc.) was unchanged.

Animals↗

Phenomenology, pathogenesis, diagnosis and treatment of aspirin-sensitive rhinosinusitis.

Aspirin-sensitive rhinosinusitis is a non-allergic, non-infectious perennial eosinophilic rhinitis starting in middle age and rarely seen in children. It may also been seen in atopic patients who have developed a mixed type rhinitis with recurrent airway infections. There is an intolerance to aspirin and most other NSAID. An intolerance to tartrazine, food additives, alcohol, narcotics and local anaesthetics can follow. Most aspirin-sensitive patients develop nasal polyps. Untreated, it can lead to asthma. The frequency of aspirin intolerance is 6.18% in patients with perennial rhinitis and 14.68% in patients with nasal polyps. Immunologic studies of the blood and the nasal polyps show a hyperreactive immune system with an activation of the eosinophil granulocytes due to a TH1-lymphocyte-activation. In atopic subjects with a mixed type rhinitis, we found a TH2- and B-lymphocyte-activation as well. Inhibition of eosinophil apoptosis might be a second remarkable change in the immune system of aspirin-sensitive patients. A key pathogenic event for aspirin sensitivity is the change of the leukotriene pathway for arachidonic acid metabolism releasing high amounts of leukotrienes LTC4, LTD4 and LTE4, effective chemoattractants and activators of inflammatory cells. For the diagnosis of aspirin intolerance, nasal, bronchial and oral challenge are available. The sensitivity of nasal challenge with lysine-aspirin for the diagnosis of aspirin-sensitive rhinitis is 0.93, the specificity 0.97. It is the safest test in aspirin-sensitive asthmatics causing bronchial side effects only in 0.45%. Therapy of aspirin-sensitive rhinosinusitis includes avoidance of aspirin and NSAID. A general down regulation of the immune response with glucocorticosteroids is an effective means. We prefer a maintenance dose of budesonid 400 micrograms a day. Systemic steroids for a reversibility test or in exacerbation due to viral infection are given in a dose of 50 mg a day for one week. If steroids don't work well enough, we combine them with aspirin desensitizations at a maintenance dose of 500 mg a day. Gastrointestinal side effects occur in 20% of the patients with a dose of 500 mg aspirin a day, in 46% with a mean dose of 1300 mg a day. The combined treatment of topical nasal steroids and aspirin-desensitization is effective in 65% of the patients with improvement in the symptoms of hyper-secretion, irritation and blockage, while 73% show improvement of polyps, hyposmia and anosmia. Endonasal endoscopic surgery of the ethmoids, turbinectoms and septoplasty should be done if necessary, especially in cases where conservative treatment fails. After surgery a further antiinflammatory treatment is absolutely necessary otherwise polyps reoccur in 90% of the cases after weeks or months. Unfortunately there is so far no curative treatment. New drugs like cytokine or leukotriene receptor antagonists give hope for better results in treatment of aspirin intolerance in the future.

Adrenal Cortex Hormones↗

[Disorders of the sense of smell and taste].

Disorders of olfaction and taste are infrequent, but a complete loss of smell or taste reduces the quality of life significantly. The sensitivity of human olfaction is remarkable, even for specific stimuli: Just a few molecules are enough to induce the correct identification of sterilised and ultraheated milk. Olfaction and taste are called 'chemical senses' because in both cases the adequate stimulus consists of molecules that bind to receptors of the sensory cells. The perceptions of smell and taste are often combined. Taste differentiates only four qualities: sweet, sour, salty, and bitter. The typical flavor of food or drink is detected by olfaction. Disturbances of olfaction can be due to respiratory disorders such as nasal polyps, a deviation of the nasal septum or chronic sinusitis. Such conditions can reduce airflow through the olfactory cleft at the roof of the nasal cavity. They can be corrected by modern endoscopic surgery of the nose. Epithelial disorders involving the sensory cells are most often caused by viral infections (influenza-anosmia) or toxic destruction of the sensory epithelium (solvents or gases). Epithelial disorders can be cured only rarely by any treatment. Corticosteroids, zinc, and vitamin A are tried frequently. Neural disorders occur after frontobasal trauma and during neurological diseases such as Parkinson's or Alzheimer's disease. Disorders of olfaction can be an early sign of such neurological diseases and sophisticated examination of this sense can contribute to their early diagnosis. However, no specific treatments have yet been identified. Disorders of taste can be due to toxic, chemical or inflammatory damage to the sensory cells of the tongue.(ABSTRACT TRUNCATED AT 250 WORDS)

Central Nervous System Diseases↗

[Disorders of olfactory function in penetrating head injuries sustained during war].

Olfactory function was estimated in the group of 37 patients with the penetrating war head injury. In 4 (10.8%) patients mutual anosmia was found. In comparison to healthy volunteers the injured showed worse performance on the level of the tested olfactory parameters. In 9 (24.3%) patients the offered smells were sensed as very unpleasant. The subgroup of patients from Glasgow Coma Scale (GSC) < or = 8 showed the highest degree of olfactive disfunction. The patients with complicated penetrating head injury were less successful at the level of all olfactive qualities comparing to those without injury complications. The localization of the focal penetrating wound, regardless of the kind of a weapon causing the brain injury, was not significantly correlated with the results of olfactometry in distinction from hemisphere lateralization of the lesion. The recovery of the retested was minimal.

Adult↗

Allergic rhinitis and olfactory loss.

BACKGROUND: Allergic rhinitis is associated with reports of olfactory loss, but there are few formal investigations. Patients with diminished smell function frequently have nasal polyps or sinusitis, making it difficult to separate the impact of allergic rhinitis from the effects of these other problems. OBJECTIVE: The goals of this descriptive study were to establish the prevalence of positive skin tests in patients reporting rhinitis and olfactory deficiency, and to assess olfactory function and the results of skin testing in a patient group with chronic rhinitis but without concomitant sinusitis or nasal polyps. METHODS: Sixty-two patients reporting olfactory loss and chronic rhinitis were examined by history, physical examination, olfactory testing, skin testing with perennial and seasonal allergens, endoscopic rhinoscopy, and CT scan of the paranasal sinuses. RESULTS: Seventy-one percent of all the subjects had at least one positive skin test, 69% to a perennial allergen, and 58% to mite. Eighty-two percent of the 28 subjects with chronic rhinitis but no evidence of polyps or sinusitis had positive tests. The mean olfactory score for this rhinitis group was 4.35, consistent with moderate hyposmia. The mean olfactory score of 34 subjects with polyps and/or chronic sinusitis was 0.61, consistent with anosmia, and significantly lower (P < .001). Sixty-two percent of this group had positive skin tests. CONCLUSION: These subjects who experienced olfactory loss and rhinitis appeared to have a high prevalence of allergic rhinitis as suggested by the number of positive skin tests. Olfactory loss was observed in patients without polyps or sinusitis, which suggests that allergic processes may have affected olfactory function.

Adolescent↗

FSH and LH response to LHRF in Kallman's syndrome.

The olfacto-genital syndrome (Kallman's syndrome) is believed to be primarily an hypothalamic disorder resulting in hypogonadotropic hypogonadism or hypo-ovarianism and anosmia. The pituitary response in 2 patients was measured on dialy subcutaneous injections of 100 mug of LHRF. One of the patients was also studied in a similar fashion by administration of LHRF intravenously for 4 consecutive days. A greater response occurred with the subcutaneous route.

Adult↗

[Male hypogonadotrophic hypogonadism: successful treatment of infertility with HMG + HCG (author's transl)].

Ten typical cases of male eunuchoidism (two with anosmia) are reported. After administration of clomifene citrate to five patients there was no change in blood levels of gonadotrophins in four cases; in the fifth, a small and transitory increase of LH was noted. The intravenous injection of LHRH (100 mcg) to five patients induced an increase of serum LH in all cases and serum FSH in three cases. The initial site of the dysfunction is possibly hypothalamic with secondary gonadotrophic pituitary insufficiency. Among six patients desiring paternity, prolonged treatment (for 36 to 98 weeks), with HCG(1700-7000 I.U. weekly) + HMG (450-825 I.U. FSG weekly) resulted in the appearance of spermatozoa in the seminal fluid in five cases and a pregnancy was obtained in four cases. Methods of treatment are discussed.

Adult↗

[Intermittent sea-saw nystagmus successfully treated with clonazepam].

See-saw nystagmus is a pendular nystagmus with two distinct components: a conjugate torsional component and a disjonctive vertical component. The acquired form of nystagmus is usually seen with parasellar mass and less frequently in head injury or brainstem infarction. Almost all patients with parasellar mass or head injury have associated bitemporal hemianopia. We report the case of a 27-year-old man who developed intermittent daily oscillopsia five years after a severe head injury. On neurological examination, the patient showed a congenital left eye divergence with left eye convergence paresis, an anosmia, a right optic atrophy and a bitemporal hemianopia. Visual acuity was 7/10 and 3/10 for the right and left eyes. The rest of the neurological examination was unremarkable. Electroencephalogram during oscillopsia was normal. MRI revealed bilateral orbito-frontal low signal intensity on T1 weighted images. The brainstem, the diencephalon and the ventricular system appeared to be intact. Eye movement recordings exhibited intermittent see-saw nystagmus. Clonazepam treatment resolved the see-saw nystagmus. Slowly withdrawal of clonazepam was done without return of the see-saw nystagmus.

Adult↗

[Primary diffuse leptomeningeal leiomyomatosis].

A female patient aged 28 fell ill with manifestations of sensory aphasia, headache and signs of cerebral compression. Except for a bilateral papilledema there were no further pathological clinical and paraclinical findings. Later on, amaurosis, deafness, anosmia and generalized muscular hypotonia developed. The nuclear magnetic resonance image revealed a major accumulation of contrast medium in the leptomeninx. Biopsy demonstrated a mesenchymal neoplasm in the leptomeninx. After a strong rise in intracranial pressure, the patient died from a bulbar brain syndrome. Microscopy revealed a diffuse neoplasm limited to the leptomeninx of brain and spinal cord as well as the immediate neighbourhood of small cortical vessels which, by morphological criteria, was classified as low-grade malignant. With the aid of electron microscopy, the tumour cells could be identified as descendants of smooth muscle cells.

Adult↗

Superficial hemosiderosis of the central nervous system. A case report.

A case of idiopathic superficial hemosiderosis (SH) of the central nervous system and a review of the literature are presented. The patient suffered from progressive cerebellar ataxia, hearing loss, anosmia, spastic paraparesis, but no mental deterioration. The diagnosis was made with brain and spinal MRI, that showed in T2 weighted images superficial hypointensity of spinal cord, medulla oblungata, pons, mesencephalon, cerebellum and cerebral hemispheres, images that are considered pathognomonic of SH. Repeated spinal fluid examinations were negative, suggesting that evidence of overt subarachnoidal bleeding is not essential in the diagnosis. In patients with SH of unknown etiology no valid therapy is yet available.

Aged↗

[Penetrating craniocerebral injuries during downhill skiing].

Two cases of penetrating craniocerebral injuries which occurred during alpine skiing were presented. The first was a 25-year-old lady whose calvarium was impaled with a ski pole in the anterior part of the orbital roof following an interpersonal collision. She was brought to the Critical Care and Emergency Center, Iwate Medical University. On admission, her consciousness was clear and a stab wound was observed in her upper eyelid. Initial CT showed bone fracture of the anterior part of the orbital roof and a small intracerebral hematoma associated with bone fragment was revealed. Immediate removal of the hematoma and foreign body was carried out and duralplasty was performed. She had a very good postoperative course but residual anosmia. The second was a 9-year-old boy who lost control and collided with a tree during downhill skiing. A twig penetrated through his left maxillary sinus. He was brought to the Critical Care and Emergency Center, Iwate Medical University. On admission, his consciousness was clear. CT showed a round low density area from his anterior lobe to the Nucleus caudatus, indicating the tract of the twig. Immediately, duralplasty and removal of the foreign body was performed. He had a very good postoperative course.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Successful treatment of male infertility due to hypogonadotropic hypogonadism--report of three cases.

Exogenous gonadotropins or pulsatile gonadotropin-releasing hormone is now most commonly used to treat male infertility due to hypogonadotropic hypogonadism. We report three cases of hypogonadotropic hypogonadism with variable etiologies and presentations who were successfully treated with exogenous gonadotropins and/or testosterone for their infertility. The diagnosis and clinical presentations of these three patients are summarized as follows. The first patient was a case of Kallmann's syndrome presented with short stature, infantile genitalia and anosmia. The second patient was a case of idiopathic hypogonadotropic hypogonadism presented with small genitalia and impotence. The third patient was a case of acquired hypogonadotropic hypogonadism due to pituitary adenoma presented with impotence, cold intolerance and visual field defect. After adequate therapy with human chorionic gonadotropin, human menopausal gonadotropin and/or testosterone, the secondary male characteristics of these three patients improved and the fertility were all restored.

Adolescent↗