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Clinical studies in black women with isolated gonadotrophin-releasing hormone deficiency.

Twelve black patients with primary amenorrhoea as a result of hypogonadotrophic hypogonadism were studied to establish the diagnosis of isolated gonadotrophin-releasing hormone (GnRH) deficiency. All were eunuchoid with poor development of breasts and pubic hair. Chromosomal complement was female and none had midline facial defects or anosmia. Follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels were low or undetectable, while the levels of other pituitary hormones were normal. Patients did not respond to clomiphene citrate administration, but did bleed in response to an oestrogen/progestagen combination and responded to human menopausal gonadotrophin. This study clearly establishes that isolated GnRH deficiency occurs in black women and suggests that the male:female ratio is different from that in white populations.

Adult↗

[Spatial distribution of electrical responses in the olfactory epithelium of mice].

In order to investigate the sensitivity to isovaleric acid (IVA) and isoamylacetate the electroolfactograms (EOGs) were recorded from various parts of the olfactory epithelium in two strains (AKR and C57BL/6) of mice. Odorant-specific regional differences in the responsiveness were observed. This heterogeneity is apparently determined genetically. The regions of maximum responses to two odorants are spatially separated, though their partial overlapping can be observed. When the concentration of the stimuli was changed the maximum responses were recorded from the same regions. The differences in the IVA-response distribution in the males of C57BL/6 strain are probably a result of their specific anosmia to this substance.

Animals↗

[Classification and definition of smell and taste disorders].

The smell and taste disorders are defined in terms due to two principles - the type and site of lesion. The quantitative dysosmias (anosmia, hyposmia, hyperosmia) correspond to the quantitative dysgeusias (ageusia, hypogeusia, hypergeusia) and the qualitative dysosmias (parosmia, pseudo-osmia, phantosmia, agnosmia) correspond to the qualitative dysgeusias (parageusia, pseudogeusia, phantogeusia, agnogeusia). Due to the site of lesion we can differentiate between respiratory, epithelial, combined, neural, and central dysosmias as well as epithelial, neural, and central dysgeusias. Typical examples are demonstrated, denying other classifications and terms as not precisely enough or negligible ones.

Ageusia↗

[A case of adenomatoid odontogenic tumor with intracranial extension].

A 49-year-old female of adenomatoid odontogenic tumor with intracranial extension was reported. She had 3 months history of left nasal obstruction and visual disturbance. Endocrinological examinations revealed a low basal level of serum cortisol and low level of urinary 17-OHCS. Neurological examinations revealed left anosmia and left visual disturbance (hand movement, marked afferent pupillary defect, and lower hemianopsia). Plain X-ray films and frontal tomograms of the skull showed a mass of soft tissue density in the left upper maxillary, left posterior ethmoid, and sphenoid sinuses with extensive destruction of the sella turcica, dorsum sellae, and anterior clinoid process. Plain axial CT scans demonstrated a low density mass filling up the left maxillary, left posterior ethmoid, and sphenoid sinuses and extending into the left middle fossa with bony destructions. Contrast-enhanced coronal CT scans demonstrated an extradural extension of the tumor into the left middle fossa and upward displacement of the pituitary complex. Left carotid angiograms showed a faint narrowing of the left internal carotid artery between the cisternal portion and the origin of the ophthalmic artery. Subtotal removal of the tumor and decompression of the optic canal and superior orbital fissure were performed via a left subtemporal extradural approach. Adenomatoid odontogenic tumor is a comparatively rare benign lesion which exhibits a slowly expansive growth without recurrence or metastasis after the surgical removal. No other case that recurred several times and extended into the intracranial space has been reported in the literature.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain Neoplasms↗

[A case of calcified intracranial tuberculoma presenting unique MRI findings].

A 41-year-old male patient was admitted in our Ryukyu University Hospital complaining of parosmia. He had a history of miliary tuberculosis 21 years ago. Neurologically he showed left anosmia and hyperreflexia of the right upper extremity. Plain skull X-P and CT scan revealed a calcified mass, 25mm in diameter, at the left frontal base. In MRI, the mass showed isointensity using the T1 weighted inversion recovery sequence and heterogenously low intensity using the T2 weighted spin echo sequence. Surgery was performed by bifrontal craniotomy. Then the tumor was removed totally including two coexisting small tumors. Histologically, they consisted of calcified caseous tissue and thick collagen capsule, suggesting old calcified tuberculomas. Postoperative course was uneventful and did not result in meningitis. Antituberculous therapy of streptomycin, isoniazid and rifampin was given for 2 weeks, started on the operative day. MRI findings were presented in detail and the guideline of antituberculous therapy to the tuberculoma was discussed.

Adult↗

[Transcervical and transoral approach to clival and upper cervical chordoma: a case report].

The patient, a 48-year-old man, was first admitted in 1981 for the chief complaints of retropharyngeal tumor and anosmia. Computerized tomography scan disclosed destruction of the lower half of the clivus and retropharyngeal tumor predominantly located in the third prevertebral region. The tumor was removed partially via the transcervical approach and was histologically a typical chordoma. Two years later the tumor recurred and was operated on by the same approach. However, only partial removal of the tumor was realized due to difficulties in dissecting the fibrous adhesion. In 1986 (7 years after onset of the symptoms), the patient was readmitted due to severe dysphagia. The sagittal MRI scan demonstrated a huge oval retropharyngeal tumor with precise delineation between the tumor and clivus, clivus upper cervical spine and brain stem. The tumor was extirpated subtotally on the midline via the transoral approach. Dysphagia completely improved postoperatively and the patient regained normal smell sensation. The advantages and disadvantages of the transcervical and transoral approach to clival lesion are discussed and the advantage of the MRI imaging is stressed for preoperative information for the transoral approach.

Chordoma↗

Urticaria associated with antigonadotropin-releasing hormone antibody in a female Kallman's syndrome patient being treated with long-term pulsatile gonadotropin-releasing hormone.

The administration of pulsatile gonadotropin-releasing hormone (GnRH) has received increasing attention as a method of inducing ovulation or initiating puberty. Few side effects have been reported, although urticarial allergic reactions have been reported in the male. An 18-year-old female with hypogonadotropic hypogonadism and anosmia due to lack of endogenous GnRH was treated for 230 days using subcutaneous GnRH in an attempt to induce physiologic puberty. Just before anticipated menarche, therapy was discontinued because of the appearance of an urticarial reaction at the injection site as well as at previous injection sites. The presence of immunoglobulin G (IgG) antibodies against GnRH were subsequently identified in the patient's serum. These results further confirm the potential for antibody production to this small natural peptide in the female not previously exposed to GnRH. Some practical considerations for this form of therapy are highlighted.

Adolescent↗

[Parosmias--definition and clinical picture].

The paper proposes the definition and technical terms with greater precision for the description of qualitative smelling disturbances. Thereafter, parosmias are relevant only for the ENT specialist. Their further classification seems to be unnecessary or even false. These parosmias are always described to be troublesome for the patient since he feels the abnormality of the olfactory sensation. Thus, a clear distinction against phantosmias (olfactory hallucinations) and pseudoosmias (olfactory illusions) is given. The cause of parosmias is frequently speculative. Most frequently there occur traumas and influenza. Their treatment is equally the same as in postinfluenza hyposmias. Additionally, the dripping of 4% novocaine or 10% cocaine on the olfactory mucosa is performed. The parosmia is defined to be an independent entity of disorder, not only accompanying hyposmia. The parosmia may occur separately, too, and it does not necessarily recede together with hyposmia and anosmia.

Hallucinations↗

Autoradiographic localization of cadmium in the rat brain.

Adult rats were injected intravenously with 109CdCl2 and the distribution of the isotope within the brain and neighboring nervous structures was subsequently studied by autoradiography. Cadmium accumulated in regions outside the blood-brain barrier such as the choroid plexus, pineal gland and area postrema, but did not appear in the brain parenchyma. Uptake of cadmium was observed in the trigeminal ganglia close to the nerve cells and in the olfactory bulbs. In addition, cadmium accumulated in the iris, ciliary body and choroid of the eye, but not in the optic nerves. The deposition of cadmium in the olfactory bulbs may be related to the anosmia reported in workers exposed to this metal. The possible harmful effects of accumulation of cadmium in restricted regions of the brain and adjacent nervous structures are discussed.

Animals↗

Olfaction in leprosy.

Olfactory test was carried out in 225 cases of Hansen's disease with 75 cases each of lepromatous leprosy (L.L.), borderline leprosy (B.L.) and tuberculoid leprosy (T). Impairment of olfaction was found in 94(41.7%) cases. It was seen in 69.33% cases of L.L., 33.33% cases of B.L. and 22.6% cases of T. Total anosmia was present in 5(6.61%) cases of L.L. only. Males were more affected than the females. Impairment of olfaction was related to the duration of the disease, severity of the nasal condition, and E.N.L. reaction. It was not related to the type of antileprosy drug used.

Adolescent↗

Effects of cadmium on cadmium smelter workers.

The effects of cadmium on 65 workers in a cadmium-refining plant in the People's Republic of China were investigated. The average airborne cadmium oxide concentrations ranged from 0.004 to 0.187 mg/m3 at most worksites. Except for some vague symptoms and reports of anosmia, no significant abnormalities were found in the clinical examinations. The level of cadmium in the blood and urine was increased in the majority of the cadmium-exposed subjects. Nine cases (13.8%) with suspected or mild renal tubular damage were found in this study.

Adolescent↗

[A case of olfactory neuroblastoma presenting symptoms of a brain metastasis--a case report].

A rare case of olfactory neuroblastoma presenting symptoms as a brain metastasis is reported. A 47-year-old-man was admitted to Yamagata City Hospital complaining of head heaviness and headache attack. Neurologically, bilateral slight choked disc and right anosmia were found. The otolaryngologist in this hospital pointed out a reddish-purple polypoid tumor in the right nasal cavity. But there were no episodes of nasal bleeding or obstruction before admission. CT scan on admission showed the tumor density in the right upper nasal cavity and ethmoid sinus, which partially infiltrated into the orbital cavity, and enhanced heterogenous high density mass in the inner basal portion of the right frontal lobe. Carotid angiogram revealed a small tumor blush in the ethmoid region and avascular mass lesion in the right frontal region. On the 11th hospital day, surgical biopsy of the nasal tumor was performed by the otolaryngologist and the evident tumor cells in the fibrous tissue were observed. So the endonasal removal of the tumor and the total removal of the intracranial tumor were achieved. On operation, the continuity of intracranial tumor and nasal tumor was not observed. The histological pictures of both tumors were similar and the diagnosis was olfactory neuroblastoma. From histological and operative findings, we concluded the intracranial tumor was metastatic lesion from nasal origin. CT scan taken after the operation showed the tumor rested only in the right ethmoid sinus.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

The regional distribution of cadmium in the brains of orally exposed adult rats.

Cadmium (Cd) levels were measured in 13 dissected brain regions of adult male rats from 3 treatment groups. Rats (approximately 200 g each) were each fed 10 g/day of diets containing either 20 or 100 micrograms/g (ppm) Cd or control diet to which no Cd was added but contained approximately 0.35 ppm Cd. After 67 days of treatment, the brain of each rat was removed and each was dissected into 13 anatomical regions including olfactory bulbs, frontal cortex, rest of cortex, corpus callosum, hippocampus, amygdalae, corpus striatum, colliculum, tegmentum, thalamus, hypothalamus, pons-medulla, and cerebellum. Cd residues (dry weight) in each sample were determined by flameless atomic absorption spectrophotometry. With exception of the thalamus and olfactory bulbs, each of the brain regions of the 100 ppm Cd rats had more Cd than did those from either the 20 ppm Cd rats or controls which did not differ. There was evidence of selective accumulation of Cd within the olfactory bulbs of control and treated animals. This selective accumulation may be related to anosmia reported in workers with industrial exposure to Cd.

Animals↗

Growth pattern of boys with isolated gonadotropin deficiency.

The linear growth and skeletal maturation of 19 boys with isolated gonadotropin deficiency, including four with anosmia (Kallmann's syndrome), are presented. With the exception of one prepubertal-age boy (less than or equal to 10 years) and five pubertal-age boys (greater than 10 years), all heights were within normal limits, but below the 50th percentile. With one exception, the bone age was retarded in all boys, even in prepuberty.

Body Height↗

The Kallmann syndrome.

The endocrinologic status of three patients with the Kallmann syndrome and anosmia were studied. Other than hypogondotropic hypogonadism, no abnormalities were found. Although the testosterone response to human chorionic gonadotropin was normal in all three patients, none of them responded to the antiestrogen, clomiphene. These patients are compared to others from the literature and it is concluded that the Kallmann syndrome is a heterogeneous category, comprising a number of clinically and genetically distinct disorders.

Adolescent↗

Urinary steroid pattern in male hypogonadotrophic hypogonadism.

During clinical studies on a 34-year-old man with marked hypogonadism secondary to pituitary gonadotrophin insufficiency, an attempt was made to determine whether the testes were capable of androgen production when stimulated by exogenous chorionic gonadotrophin (A.P.L.).Androgen excretion before and after chorionic gonadotrophin administration was studied. Total and fractionated 17-ketosteroid patterns suggested a severe Leydig cell deficiency which was confirmed by testicular biopsy.Panhypopituitarism was considered excluded by a normal response to metyrapone ditartrate, by a normal serum PBI and a normal radioactive iodine uptake. Genetic disorders were likewise excluded to the extent possible by determining that the chromosome pattern was normal and by determining that no similar condition existed in another member of the same family. The association of anosmia with gonadotrophin insufficiency in this case suggests a primary disorder in the hypothalamo-mamillary-tuber cinereum area.

17-Ketosteroids↗

[Refsum syndrome, heredopathia atactica polyneuritiformis in the view of the otolaryngologist (author's transl)].

An own observation of Refsum's disease is described. The female patient of 39 presented all characteristic symptoms of the disease, which had developed progressively starting at the age of 25: atypical retinitis pigmentosa with nightblindness and concentric constriction of the visual fields, cataracts, peripheral polyneuropathy with reduced nerve conduction velocity, ataxia, high protein level in CSF, ichthyosis-like cutaneous manifestations, sceletal anomalies, progressive sensorineural deafness, anosmia. Refsum's disease is a metabolic disorder based on an inborn enzyme defect, inherited by autosomal recessive transmission. It causes storage of phytanic acid in the body. Treatment consists in diet low in phytol and phytanic acid as well as large volume plasma exchanges.

Adult↗

[Sibling case of osteosclerosis with cranial nerve symptoms].

We experienced two cases of "Osteosclerosis" who were 12 and 15 year old sisters. Previous reported cases of this disease are 50 cases and among them only one patient was reported in Japan. Osteosclerosis seems to be inherited as an autosomal recessive trait. Parental consanguinity is also observed. The peculiar facies are evident in infancy, characterized by broad, flat nasal bridge, ocular hypertelorism and prognathic, broadened mandible. Commonly, they have cutaneous syndactylies in bilateral hands and feet, especially between the second and third finger and toe. Roentgenographically, hyperostosis with osteosclerosis can be observed in systemic bones, particularly the calvarium is greatly thickened. Since such a bony change occurs most severely at the base of the skull, important clinical symptoms of this disease are cranial nerve palsies resulting from obliterations of unilateral or bilateral several cranial nerve foramina. In many cases deafness due to progressive encroachment upon the middle ear cavities and auditory nerve canals appears early in infancy. Transient palsy of the facial nerve occurs somewhat later, and bilateral facial paralyses are usually permanent in adulthood. In some cases optic atrophy and visual field defect due to compression of the optic nerves are late complications. Other ocular symptoms are strabismus, nystagmus and exophthalmos. Anosmia and trigeminal nerve palsy are less common. Lower cranial nerve symptoms can not be noted but the reason is unclear. Chronic headache, convulsion and mental retardation are occasionally present. They are considered as a result from increased intracranial pressure due to progressive diminution of the cranial capacity. By same mechanism, several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum in early adulthood. Then, some reporter puts emphasis on prophylactic opening of the foramen magnum in all adult cases.

Adolescent↗