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[Superficial siderosis of the central nervous system: an uncommon cause of spastic paraparesia].

INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is an uncommon neurological condition, characterized clinically by cerebellar ataxia, neurosensorial deafness, anosmia, myelopathy and cognitive deterioration. It is associated with the deposition of haemosiderin in the subpial layers of the brain, cerebellum and spinal cord, following chronic bleeding (often clinically silent) in the subarachnoid space. Histopathologically there is gliosis, neurone loss and demyelination of the CNS. CLINICAL CASE: We present the case of a 60 year old woman with a history of progressive worsening of a disorder of myelopathic type with signs of pyramidal liberation and sphincter incontinence associated with cerebellar ataxia, anosmia and bilateral hypoacusia. Initially she had unsteadiness, frequent falls and weakness of the legs. Her deafness was more obvious during the previous year. On lumbar puncture there was hemorrhagic CSF with increased red blood cells, iron, ferritin and protein. High field encephalic MR showed a hypointense image in T2 which surrounded the fissure of Sylvius, the brain stem, medulla oblongata, cerebellar hemispheres and sulci of the cerebellar vermis, suggestive of hemosiderosis, atrophy of the cerebellar vermis and slight cortical atrophy. Angioresonance of the intracranial vessels showed that there were no signs of aneurysms or vascular malformations. Transcranial Doppler studies were normal. CONCLUSIONS: Superficial siderosis of the CNS should be included in the differential diagnosis of the parethospastic and ataxic syndromes. The extensive study done ruled out any secondary cause such as chronic bleeding secondary to an arteriovenous malformation or bleeding aneurysm.

Ataxia↗

[Multifactorial analysis of preoperative functional symptoms in nasal polyposis (report of 403 patients)].

PURPOSE: Nasal polyposis is an invading disease whose evolution is dominated by functional symptoms. The aim of this study was to evaluate in cortico-resistant patients with nasal polyps the pre-operative functional symptoms in order to define factors that might increase them. METHODS: 403 patients were included in this study. All of them met the inclusion criteria of follow-up and initial examination. Anosmia, pain, nasal obstruction, rhinorrhea and sneezing were evaluated by patients themselves. Radiological data, respiratory function test results and histological data were matched. RESULTS: In our series, functional symptoms were dominated by anosmia, nasal obstruction and rhinorrhea. Pain and sneezing appeared to be less frequent. Analysis of co-variables allowed us to define factors which increased pre-operative functional symptoms. Some of them are well known (such as Fernand-Widal triad or stage 3 nasal polyposis), while others appear new or surprising. The same is true for frontal sinus involvement on CT-scan and glandular hyperplasia on histological study. CONCLUSION: Analysis of the complete clinical, radiological and histological picture has allowed us to emphasise the importance of certain factors on the functional symptoms of 403 patients with polyps of the nose and sinuses, resistant to steroid treatment. The same is true for Widal's disease, for a higher endoscopic grading, for frontal sinus involvement, and for histological evidence of glandular hyperplasia.

Adolescent↗

Ineffectiveness of zinc gluconate nasal spray and zinc orotate lozenges in common-cold treatment: a double-blind, placebo-controlled clinical trial.

BACKGROUND: Zinc gluconate and zinc acetate lozenges have been reported to shorten the duration of common colds in a dose-response manner when the amount of the active ingredient, positively charged zinc ions, is sufficient. OBJECTIVE: To improve results using a zinc gluconate nasal spray with zinc orotate lozenges. DESIGN: Double-blind, placebo-controlled clinical trial. SETTING: Private physician's clinic in Austin, Texas. PARTICIPANTS: Seventy-seven volunteers, all of whom had 2 or more signs and symptoms of common colds (with at least 1 nasal symptom) on enrollment in the study. METHODS: Zinc gluconate nasal spray (10 mmol) or placebo was used every 15 to 30 minutes, and lozenges were used each several hours to reduce duration and severity of common colds. An intention of treatment was to keep the nasal tissues wet with zinc gluconate solution during wakeful hours. RESULTS: After 7 days of treatment, 10 of 16 (63%) zinc-treated patients were asymptomatic compared to 9 of 17 (53%) placebo-treated patients (P = .57). This treatment caused olfactory region pain in some patients and did not reduce the duration or severity of common colds. Treatment did not produce anosmia, which has been reported in other studies following olfactory region administration of ionic zinc in many species and in humans from zinc nasal sprays and gels. CONCLUSION: We contend that it is unethical to introduce any potentially permanent anosmia-inducing agent such as zinc or other heavy metals into the interior of the nose in a manner that could result in contact with the olfactory region to treat a temporary discomfort such as a common cold or allergy. We found no reason to recommend intranasal zinc gluconate or zinc orotate lozenges in treating common colds.

Administration, Intranasal↗

Olfactory neuropathy in severe acute respiratory syndrome: report of A case.

This case was a 27 years old female with severe acute respiratory syndrome (SARS). She suffered from typical symptoms of SARS. Although she got almost complete recovery from most symptoms after treatment, she noted acute onset complete anosmia 3 weeks after the onset of her first symptom. Her brain MRI examination did not show definite lesion except an incidental finding of left temporal epidermoid cyst. Her anosmia persisted for more than 2 years during following up. Peripheral neuropathy and myopathy have been reported as a concomitant problem during the convalescent stage of SARS, while the sequel of permanent ansomia in SARS was not reported before. Olfactory neuropathy, which rarely occurred in typical peripheral neuropathy, could be a special type of neuropathy induced by corona virus infection in SARS. Olfactory function test should be taken into routine check-up for patients with SARS. The pathophysiology and therapeutic strategy of this special type of permanent olfactory dysfunction deserve further investigation.

Adult↗

Olfaction in rhinology--methods of assessing the sense of smell.

Olfactory disorders frequently occur in rhinological disease. Different subjective and objective test methods are available to assess the sense of olfaction. Among the subjective methods, screening tests and threshold measurements are commonly used to quantify hyposmia or anosmia. Qualitative methods are available using discrimination and identification tests. Objective methods are used in research and in some medicolegal situations. Objective tests include olfactory evoked potentials, functional Magnetic Resonance Imaging and functional Positron Emission Tomography. The measurement of the sense of smell helps to assess the whole spectrum of the effects of nasal disease. This is especially important before rhinological surgery, because a non-detected smell disorder in patients with rhinological disease is common. The assessment of a pre-existing hyposmia or anosmia helps to avoid a postoperative claim that this was caused by surgery. A variety of validated screening tests for olfaction is available and they are a useful tool to document whether a patient is able to smell.

Diagnostic Techniques, Respiratory System↗

[Refsum's disease. Apropos of 2 cases disclosed by myocardiopathy].

Refsum's disease is a polyneuropathy due to a hereditary error in the metabolism of a fatty acid, phytanic acid, usually leading to cardiac failure only at an advanced stage of the disease. The authors report the case of two brothers with Refsum's disease revealed by a heart failure before the clinical stage of the peripheral neuropathy. In the younger brother, the affection started at the age of 22 years by an acute pulmonary oedema which revealed a dilated, hypokinetic myocardiopathy, associated with retinitis pigmentosa, ptosis, anosmia and biological myolysis. The normal plasma concentration of phytanic acid measured several times led to the conclusion of Kearns-Sayre syndrome even if certain aspects were atypical (moderate conduction disorders, no characteristic aspect in the muscle biopsy). Five years later, the older brother, aged 28, presents a dyspnea on effort which leads to the discovery of a hypokinetic, hypertrophic myocardiopathy, slightly dilated, associated with cardiac conduction disorders, retinitis pigmentosa, anosmia and biological myolysis. The plasma concentration of phytanic acid being very high. Refsum's disease was diagnosed and the diagnosis of younger brother was corrected. From the study of these two cases, the characteristics of the cardiac disorders can be specified: the cardiopathy can reveal the disease and correspond to a dilated or hypertrophic myocardiopathy. The diagnosis of the disease can be difficult because the plasma phytanic acid may remain at normal level, thus requiring the assay of the activity of phytanate oxydase. The existence of ophthalmologic signs (retinitis pigmentosa or progressive ophthalmoplegia externa) associated with a myocardiopathy must systematically lead to a search for Refsum's disease, this diagnosis having fundamental therapeutic implications (died, even plasmapheresis).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[3-year experiences with surgical treatment of epilepsy at the Hvidovre hospital].

The results of a retrospective survey of 48 patients submitted to neurosurgery for medically intractable epilepsy are presented. Twenty-eight patients were treated with selective amygdalohippocampectomy, one with temporal lobe resection, 12 with anterior callosotomy and seven with a total callosotomy. Of the amygdalohippocampectomized patients and the one with temporal lobe resection (n = 29), 52% were seizure free, 17% experienced rare seizures, 7% had a worthwhile improvement while 24% observed no worthwhile improvement (follow-up time 6 to 36 months). Of the callosotomized patients, 11% were free from generalized seizures, 69% had a significant seizure reduction and 18% experienced no worthwhile improvement. The observed neurological complications were: one patient had hemianopia, one had superior quadrant anopia, four developed unilateral anosmia and one complete anosmia. The callosotomized patients, with two exceptions, were all mentally and physically handicapped. In the callosotomy group, two patients died, one from a intracerebral hematoma three months after the operation and another patient seven months postoperatively from unknown causes.

Adult↗

[Bilateral isolated trigeminal neuropathy (author's transl)].

Spillane and Wells (1959) were the first to describe the syndrome known as isolated trigeminal neuropathy in which deficit in the trigeminal nerve occurs either unilaterally or bilaterally without evidence of any local lesion. It may be a truly isolated disorder but can also be the predominant element of a more diffuse neuropathy provoking, more particularly, ageusia and anosmia. The bilateral forms are fairly frequently related to connective tissue diseases but many are of unknown aetiology. A case is reported of the bilateral form with ageusia and anosmia, cubital deficit, and slowing of the conduction rate in the limbs. No aetiology was found.

Aged↗

Surgical management of chemosensory disorders.

As additional understanding of the histopathologic basis of chemosensory dysfunction has been achieved, several actual and potential surgical approaches for correction have been devised. These currently include correction of steroid-dependent anosmia and hyposmia due to septal deviation. Posttraumatic anosmia might be correctable in the future. Surgical approaches to dysgeusia are sparse, probably due to a paucity of information on the causes of this disorder.

Humans↗

[Occupations and the sense of smell].

Olfactometric fitness examinations in relation to the place of work are required since the olfactory organ has support and protective functions. Smelling disorders result at work from trauma, hypersensitivity, acute and chronic intoxications, but most frequently from chemical and physical substances taken up by inhalation. They are different due to their expression (slight hyposmia till to anosmia, parosmia), latency, localization (respiratory, epithelial, nerval, central), and a combination with other disorders. In order to examine rhinologically the patient, the nasal mucosa has to be assessed. Its lesion does not necessarily correlate to the smell disorder. 475 patients were exposed to a total of 10 different substances or classes of substances. It was demonstrated that disorders of the whole nasal physiology can be caused by toxic and nontoxic substances, e.g. calcium carbide when taking effect for a longer period, even below their legally required "maximum absorption concentration". The treatment is started after ending the exposition. The expert's opinion is based on the clinical findings, a standardized investigation of smelling, the patient's history incl. the history of occupation, and an analysis of the place-of-work conditions. The degree of bodily defect is related to the single types of disorder. In case of a complete anosmia it is 20%. It is assumed that a growing number of occupation-related smell disorders become known and, hence, have to be assessed.

Environmental Exposure↗

A female case of Kallmann's syndrome.

A case of 20-year-old woman with hypogonadotropic hypogonadism and anosmia is reported, since very few female cases of Kallmann's syndrome have been reported so far in Japan. Three uncles on the father's side had no children. Height was 168 cm, and arm span 165 cm. The olfactory test revealed complete anosmia. Bone age was 13 year. Chromosome was 46 XX and normal karyotype. Basal levels of serum FSH, LH and estrogens (E1, E2 and E3) were low. Serum FSH and LH levels rose slightly only after LH-RH administration, and did not increase in clomiphene test. Plasma estrogens did not increase after daily injection of 150 IU of HMG for 3 successive days. The response of serum GH to arginine infusion was normal, while that to insulin-induced hypoglycemia was poor.

Adult↗

[Food search by mice solving an extrapolation problem after suppression of smell by zinc sulfate].

Anosmia induced by intranasal zinc administration to mice capable of solving an extrapolation problem (search for food which disappears from animal sight in definite direction), led to disturbance of normal food searching behaviour. In anosmic mice the percentage of task solutions (correct as well as incorrect) in which the trajectory was the shortest ("goal-directed"), was significantly lower than in controls. At the same time the percentages of correct "goal-directed" choices were equal in both groups. The main differences in the number of correct task solutions were among those trials in which non-goal-directed behaviour was delivered. Thus zinc induced anosmia provokes rather severe deteriorations of food searching behaviour. The investigated group of mice possessing Robertsonian translocation Rb(8, 17) 1 IEM, reveals no disturbance of extrapolation capacity during first 20 s after task presentation. This signifies that this capacity which in this group is higher than in other mice, is not determined by peculiarities of their olfaction.

Animals↗

Different olfactory system deficits affect the antigonadal action of light deprivation differently in male rats.

Twenty-eight day-old male rats were subjected to: Blinding-olfactory bulbectomy, Blinding-peripheral anosmia, Blinding-accessory olfactory bulbectomy and Blinding-sham olfactory operation. A set of rats remained intact. Six weeks later, their pituitary-gonads-accessory sex organs were studied. Bulbectomy as well as peripheral anosmia exaggerated the antigonadal effects of blindness, while the accessory olfactory system impairment did not. It is suggested that olfactory bulbectomy potentiation of the antigonadal effects of light deprivation is due to a lack of sensory function rather than to bulbectomy itself and that the accessory olfactory system which is involved in the priming pheromonal effects does not play any role in the inhibition of the antigonadal effects of blindness.

Animals↗

[Hypogonadism with spermatogenesis (fertile eunuch syndrome) (author's transl)].

Hypogonadism with spermatogenesis (fertile eunuch syndrome) occupies a special nosologic situation because of it's clinical and paraclinical features. These include: eunuchism of testosterone's target tissues, contrasting with clinically normal testes; normal embryonic male differentiation; low serum testosterone concentrations; moderately decreased or normal LH serum concentrations which are coincident with low testosterone levels therefore reflecting at least relative LH insufficiency; normal FSH levels; normal LHRH response; variable clomifen response; poor spermatogenesis improved by HCG and/or testosterone, and in some cases by HMG; atrophic leydig cells. Although in very rare instances it may include anosmia, the fertile eunuch syndrome should be clearly distinguished from complete FSH and LH deficiency with or without anosmia. It must also be distinguished from the very uncommon isolated FSH deficiencies. The fertile eunuch syndrome probably originates in insufficient hypothalamic production of LHRH. The mechanism by which spermatogenesis is at least partly spared remains unknown.

Androgens↗

[Data concerning a case of long-term, asymptomatic, calcified cerebral tumor with late evolution of olfactory meningioma].

In a lot of 3 patients with olfactory meningioma, one patient drew particular attention by the particularities of its evolution, which was asymptomatic for a long time and atypical. The disease started at the age of 38 with asthenia, headaches, dizziness and X-ray images of left parasellar calcified brain tumour, without neurological signs of a focus at any of the repeated clinical or paraclinical examinations. After eight years focal signs appears: left pyramidal irritation, absences, left hyposmia, without altered visual acuity or conclusive scintigraphic and EEG alterations. Left carotid arteriography indicated a space-occupying process. The disease progressed slowly with left hemiparesis and corresponding effects. A diagnosis of "left olfactory meningioma" was established in the Neurosurgical Clinic, Bucharest. The situation deteriorated in 1977: total left anosmia, Jacksonian seizures, sometimes generalized, uncinate fits, paroxysmal headaches, amnesia disturbances and marked right hemiparesis. Recent scintigraphic and EEG alterations likewise lent support to a diagnosis of brain tumour. The existence of calcified, asymptomatic, trailing brain tumours, with an atypical and late evolution of unilateral olfactory meningioma, without Foster-Kennedy syndrome, demands from the beginning close surveillance and complex investigations, since a prolonged absence of anosmia and focal phenomena does not exclude the possibility of a meningioma of the olfactory groove, and may often lead to errors of diagnosis.

Adult↗

Olfactory involvement in learning processes.

The purpose of this study is to investigate the existing relations between olfaction and acquisition of the two-way conditioned avoidance response (CAR). For this investigation we have used white adult male Wistar rats. Taking into consideration that several structures of the limbic system participate in learning and memory processes and also that olfactory pathway is believed to have connections with the limbic system, we thought it would be interesting to investigate, if peripheral anosmia influences the performance of rats in the two-way avoidance response. It was shown that peripheral anosmia in rats impaired conditioned avoidance response.

Animals↗

Olfactory meningiomas. The missed diagnosis.

Olfactory meningiomas are benign, slow-growing intracranial tumors arising from the dura along the cribriform plate. The first clinical symptom is anosmia followed, usually after several years, by dementia and visual deterioration. A series of 36 patients are presented; in all cases but one, their conditions were diagnosed late and not until the tumor had reached a very large size. By the time the proper diagnosis was made and the tumors were surgically removed, mental or visual disability was often irreversible. Conditions of patients initially seen with anosmia should be investigated by presently available noninvasive diagnostic methods including computed tomographic scanning.

Dementia↗