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[The role of magnetic resonance imaging in the diagnosis of Hallervorden-Spatz disease].

A mentally-retarded 21-year-old female with narrowing of the visual field was admitted to our hospital because of dystonia in the body and extremities which appeared 6 months before and was gradually exacerbating. On admission, torsion dystonia, pyramidal tract sign, and retinitis pigmentosa with optic atrophy, urinary incontinence, hyperhidrosis and insomnia were noted. On the Wechsler Adult Intelligence Scale, she achieved less than 60 for verbal-IQ. Laboratory tests including serum copper and ceruloplasmin, were all normal. A computed tomography of the brain showed small hyperdense spot in the globus pallidus on the both sides. There was neither cortical atropy nor ventricular dilatation. A Magnetic Resonance Imaging was obtained using both moderate-field (0.5 Tesla) and high-field (1.5 Tesla) superconducting MR systems. T2-weighted spin echo images demonstrated markedly decreased signal intensity area restricted in the globus pallidus and substantia nigra on both system. Moreover, in the center of the globus pallidus, a small high signal spot was seen ("eye-of the-tiger" sign coined by Sethi et al). There was no abnormal signal in the putamen, caudate nucleus, red nucleus, and dentate nucleus. On the other hand, T1-weighted inversion recovery images were not remarkable. These striking low attenuation on T2-weighted findings suggests an increased iron deposition limited to the globus pallidus and substantia nigra which is characteristic of Hallervorden-Spatz disease/syndrome (H-S). Moreover, "eye-of-the-tiger" sign on T2-weighted MR images in the globus pallidus is previously described in H-S.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Causalgia in neurologic expert assessment].

Rendering an expert opinion on causalgia, after having clear determined it as the disease, poses many problems to the examiner. One ought to make this diagnosis with its serious consequences only under the following conditions: --when it is a case of a mostly partial trauma of a nerve with considerable intermingling of sympathetic fibres; --when the pains have a burning and primarily superficial character; --when the pain appear soon (hours, mostly days or weeks, more rarely months afterward) after the trauma; --when the pains are accompanied by considerable vasovegetative symptoms (cold sweats, cyanosis, hyperhidrosis and others). Secondary algodystrophic changes (muscle, joints, bones, and skin) are to be considered. In quantitatively judging the effects, an over-evaluation, as well as an underevaluation, is to be avoided by all means. Therapeutic effects and spontaneous remission after six to twelve months are to be considered. An exemplary case, in which a deterioriation of the capacity to work from 20 to 100% (!) was assumed, showed the need for taking the personality structure of patient into consideration.

Adult↗

[Unique forms of the postthrombotic syndrome].

A prospective study was made with a view to analyse and to identify two particular forms of the post-thrombotic syndrome. The post-thrombotic syndrome of the shank can be recognized on the basis of the clinical and of the phlebographic examination. It has a serious prognosis and can be treated either conservatively or by surgery according to case. Its frequency is probably higher than it was presumed on the basis of the first observations. The post-thrombotic venous obstruction may be a cause of arterial ischemia during effort but this occurs only in very few cases. This particular syndrome is manifested as intermittent claudication which occurs in the clinical picture of chronic orthostatic venous failure. The probable mechanism is the direct effect of increased pressure in the arterial and venous circulation in vessels with normal walls, as a result of an important obstruction in the return circulation. Lumbal sympathectomy appears to improve durable claudication and hyperhidrosis.

Adult↗

[Eccrine angiomatous nevus].

A case of eccrine angiomatous nevus of the leg in a 21 year-old woman is reported. The lesion present since birth was painful on pressure and exhibited hyperhidrosis. Histological examination of the lesion showed an increased number of eccrine structures without structural abnormalities with an angiomatous vascular component. In addition, there were some neural sheets. Problems of differential diagnosis and histogenesis of the eccrine angiomatous hamartoma are discussed.

Adult↗

[A case of male acromegaly with galactorrhea].

A 43-year-old man was admitted to our clinic with complaints of headache and nasal obstruction. He has noted increasing hand and foot size with decreasing libido and pollakiuria for ten years. On admission, he showed galactorrhea. His visual symptoms were slightly decreased. Endocrinological evaluation revealed high serum levels of GH and prolactin. A plain skull X-ray showed acromegalic features with remarkable destruction of dorsum sellae. A contrast enhanced CT demonstrated an intrasellar high density mass extending to the sphenoid sinus and the right ethmoidal sinus. A cerebral angiogram was nor.al. Surgery was then performed with the transsphenoidal approach. A soft reddish brown mass was found in the sphenoid sinus and the bilateral cavernous sinus extending from the sella turcica. Histologically the tumor was eosinophilic adenoma. There were numerous cells exhibiting immunostaining for both GH and PRL in the immunocytochemical study. Postoperative course was uneventful. His visual impairment improved soon after the operation. Serum GH and PRL levels decreased to 38 and 130 ng/ml. He was treated with conventional irradiation (500 rads), so remained galactorrhea and hyperhidrosis. One year after the operation, there is no regrowth of the residual tumor.

Acromegaly↗

[Clinical aspects, epidemiology and economic impact of foot epidermomycosis in an industrial milieu].

Fungal infections of the feet are very common in some professions. This has been particularly studied in coal mine workers up to 50 p. 100 of whom were found to have dermatophytosis of the feet. The purpose of this study was to determine the clinical, epidemiological and evolutive characteristics of interdigital and plantar intertrigo of the feet among people working in a coking plant, a potash mine and a motorcar factory. We have also evaluated the cost of treatments in relation to their immediate effectiveness and their preventive effects on relapses. Initially, 208 workers entered the study: 84 (40.7 p. 100) had a mycotic infection (M) and 124 (59.3 p. 100) presented with a non-mycotic (NM) inflammation between the toes and on the soles, characterized by maceration of the skin, hyperhidrosis and bromhidrosis. Change in the lesions under treatment were subjected to statistical analysis. In both M and NM groups the workers were allocated by randomization to a double-blind treatment consisting of: group M: either 2 p. 100 miconazole powder or talcum powder with 2 p. 100 salicylic acid and boric acid; group NM: either 2 p. 100 miconazole powder or ordinary talcum powder. Each subject was seen on at least two occasions after 3 and 12 months of daily foot care and topical treatment. Mycological examinations in group M subjects showed that fungal infections of the feet were primarily due to Trichophyton mentagrophytes or to Trichophyton rubrum. A logistic regression analysis (BMDPLR program) of anamnestic and clinical data (table IV) led to a "mathematical model of the mycotic foot", characterized by 6 main parameters of statistically significant occurrence: a history of interdigital-plantar intertrigo, recurrent in 90 p. 100 of subjects in group M (significant association at 0.1 p. 100); a functional symptom, pruritus, present in 71 p. 100 of subjects with mycosis; the stronger, more disturbing the pruritus, the greater the probability of it being of fungal origin (significant association at 0.1 p. 100); 4 physical signs: lack of maceration (0.1 p. 100), lesions limited to the interdigital spaces and respecting the soles (0.1 p. 100), presence of interdigital fissures (0.5 p. 100) and vesiculation (2.5 p. 100). When the respective diagnostic values of these elementary symptoms were considered, it appeared that their various combinations might have predictive values which could be calculated.(ABSTRACT TRUNCATED AT 400 WORDS)

Computer Simulation↗

Reflex sympathetic dystrophy in children: treatment with transcutaneous electric nerve stimulation.

During the past 6 years, ten children with reflex sympathetic dystrophy were treated. Pain in an extremity was the initial complaint in all patients. The pain was unilateral in 90% of the patients; upper and lower extremities were affected with equal frequency. Tenderness to palpation, extreme hyperesthesia, and dysesthesia were other dominant features. All patients had some evidence of autonomic nervous system dysfunction in the affected extremity (swelling, color change, decreased temperature, and/or hyperhidrosis). The median duration of symptoms prior to referral and diagnosis was 5 months. All children were treated as outpatients with a transcutaneous electric nerve stimulator and home-based physical therapy. With this regimen, seven patients had complete remission within 2 months. Two other patients improved with transcutaneous electric nerve stimulation therapy, and one patient had no response to transcutaneous electric nerve stimulation. Reflex sympathetic dystrophy is frequently underdiagnosed in children. Increased awareness of this syndrome is important because accurate diagnosis is crucial and transcutaneous electric nerve stimulation offers a safe, simple, and effective outpatient therapy for reflex sympathetic dystrophy in children.

Adolescent↗

Natal teeth and steatocystoma multiplex: a newly recognized syndrome.

A Chinese family is reported in which five generations have exhibited natal teeth and generalized multiple steatocystomas. This autosomal dominant condition is not similar to the two reported types of pachyonychia congenita, because nail lesions, palmoplantar keratosis and hyperhidrosis, follicular keratosis, and oral leukokeratosis were not observed. Therefore, it is suggested that this family exhibits a newly recognized syndrome.

Epidermal Cyst↗

Bacteriology of pustules occurring during treatment of psoriasis.

Pustular lesions developed in 16% of our psoriatic inpatients treated with a modified Goeckerman regimen. The role of bacterial infection in the development of these lesions remains controversial. Representative lesions were cultured, and the recovered organisms were quantitated. We were not able to culture a significant number of bacteria from these lesions. Pustules were prone to develop during the summer months in hirsute patients, in patients with hyperhidrosis, and in patients in whom topical treatment of a more occlusive nature was applied. The male-female ratio was 4.8:1. Topical antibiotic treatment of these pustules in psoriatic patients appeared to be unwarranted since the lesions resolved when topical therapy was discontinued. These pustules appeared to be distinct from pustular psoriasis.

Anti-Bacterial Agents↗

[Indications and possibilities of blockade of the sympathetic nerve].

Treatment of chronic pain through permanent or temporary interruption of sympathetic activity is marked by great clinical success, but nevertheless there are rather skeptical reports about long-term results of these blocks as therapeutic measures. There are many symptoms and signs of chronic pain, while diagnosis is expensive, the pathogenesis is complex, and the etiology is generally due to multiple factors. Indications for sympathetic blockade depend upon the possible means of access, as in the cervicothoracic, thoracic, lumbar, or sacral regions. General indications are: symptoms not limited segmentally within peripheral body areas; pain resulting from microtraumata and lesions of peripheral nerve branches; and pain caused by intensified sympathetic tone with consequent circulatory disturbances. Peripheral circulatory disturbances are the most common indication for sympathetic blockade, as the block produces a vasomotor reaction that leads to increased capillary circulation. Pain caused by herpes zoster, sudden hearing loss, hyperhidrosis, and pseudesthesia can also be influenced by sympathetic blockade. There are several possibilities for reducing or interrupting sympathetic activity; for us, however, blocking of the sympathetic trunk is the most important. During the last 16 years we performed 15,726 sympathetic blockades on 2385 patients, which included: 3735 stellate ganglion blocks, 6121 blocks of the lumbar sympathetic trunk, 5037 continuous peridural anesthesias, 29 blocks of the thoracic sympathetic trunk, and 12 celiac blocks. In 792 cases sympathetic blocks were performed using neurolytic drugs, in most cases 96% ethyl alcohol and less often 10% ammonium sulphate. Other possibilities, such as enteral administration or infusion of sympatholytic drugs, were not taken into consideration; regional intravascular injection of guanethidine can be recommended, however.(ABSTRACT TRUNCATED AT 250 WORDS)

Arterial Occlusive Diseases↗

[Therapy of gustatory sweating following parotidectomy. Frey's syndrome].

A new suggestion for treating marked sweating with Frey's syndrome namely, sweat suppression by aluminium chloride, is presented, together with a survey of the different attempts to suppress the hyperhidrosis. The mechanism of sweat suppression, the form of application and the results in 28 patients suffering from marked gustatory sweating, are outlined. After a single application, an interval of several weeks free of symptoms can be expected.

Administration, Topical↗

Lumbar sympathectomy.

Lumbar sympathectomy is used in patients with threatened or early gangrene of the toes where reconstructive arterial surgery is not possible--usually because arteriography has demonstrated absence of a "run off" suitable for endarteriectomy or a bypass graft. It may also be a supplement to reconstructive surgery of the aorta or the common iliac arteries. It is indicated in intractable Raynaud's disease affecting the feet and is particularly helpful in severe hyperhidrosis of the feet. In many cases the procedure can be performed nonoperatively by chemical sympathectomy using an injection of aqueous phenol into the lumbar chain.

Autonomic Nerve Block↗

Mal de Meleda treated with 13-cis retinoic acid.

Identical twins with mal de Meleda, a rare genodermatosis, displayed the characteristic "glove and sock" hyperkeratosis, hyperhidrosis, and malodor. Their parents, who are first cousins, are unaffected and originated from Calabria, Italy, which is not far from Meleda (Mljet). The disorder is probably transmitted as an autosomal recessive trait. Previous therapy with grenz rays, topical preparations of adrenocorticosteroids, lactic acid, retinoic acid, and bland emollients had been unsuccessful. One of the twins was treated with 13-cis retinoic acid taken by mouth for 16 weeks with dramatic improvement. The major adverse effect was cheilitis, which did not force discontinuation of the treatment.

Adolescent↗

Pachyonychia congenita. A clinical, histological and microradiographic study with special reference to oral manifestations.

This paper rresents a clinical, histological and microradiographic study of three patients with pachyonychia congenita with special reference to oral manifestations. The patients, who are relatives, exhibited thickening of finger- and toe-nails, follicular keratosis, palmoplantar keratosis and hyperhidrosis, oral leukokeratosis, and natal teeth. It is stated in the discussion that natal teeth and oral leukokeratosis may constitute the earliest clinical manifestations of pachyonychia congenita and that they appear to accur earlier than nail lesions. When there is a hereditary disposition for pachyonychia congenita, it is important to inspect the oral cavity at an early stage.

Adult↗

[Constitutional painful callosities. Analgesic efficacy of etretinate].

We have recently evaluated eight patients with an unusual genetic dermatosis that was characterized by the development of painful callosities at pressure points of soles, with tender yellowish-brown hyperkeratosis. These lesions are very painful: walking produced pain that was severe. The patients performing manual labor, in addition, had multiple callosities on the palms. Hyperhidrosis of the soles and palms was frequently present. Skin lesions were first noted in adolescence. There was no similar family history. There was no personal nor family history of hair, nail or dental disease. A biopsy was performed in one case and showed a simple hyperkeratosis similar to these observed in callosities. Classification of our cases is difficult. They are not similar to the entity named "hereditary painful callosities" by Roth et al. Review of the literature did not show similar cases. The patients received Etretinate (1 mg/day/kg). In all 8 cases, a dramatic improvement was obtained within seven days; the pain disappeared entirely and the patients were able to walk again without any pain. Then, the per-day dose was diminished without reappearance of the pain.

Adult↗

[Cranial hemihypertrophy in Klippel-Trenaunay syndrome (author's transl)].

Klippel-Trenaunay Syndrome with hemicranial hypertrophy and macrodactyly is reported. The patient was a 3-year-old Japanese woman with an extensive nevus flammeus which extended over the right side of the neck, axilla, upper arm, and leg. Her right extremities were longer and thicker than left, and showed apparent macrodactyly in the right hand. Her skin of right side body was irregular, rough surface and was pigmented prominently. Hyperthermia was present in the hypertrophic limbs, but hyperhidrosis was none. She had moderate mental retardation, delayed speech. Her hemicranial hypertrophy (same side as hypertrophic limbs) was examined by CT scan. Her right lateral ventricle was dilatated and cortical sulci was well demonstrated. Her right hemicranial volume was enlarged but rt-cerebral hemisphere was atrophic, and her infratentorial structures were symmetrical. Presented case, Klippel-Trenaunay Syndrome with hemicranial hypertrophy, suggested the embryological theory affecting on trilaminar disc in embryonal stage as the genesis of this rare syndrome.

Angiomatosis↗

The relationship between certain clinical characteristics and course of disease in milk fever in dairy cows.

Recovery rate after one treatment and mortality rate in cows with clinical milk fever were related to several clinical characteristics recorded immediately prior to first treatment, namely: Appetite, mobility, demeanour, whether or not hyperhidrosis was present, pulse quality, pulse rhythm, pulse rate and rectal temperature. Furthermore, the same symptoms at second treatment and subsequent course of disease in milk fever cows which received at least two treatments were found to be related. Recovery rate after first treatment was lower in cows which had been recumbent for more than one hour before treatment than in those which had been down for a shorter period. As regards cows which received at least two treatments, the recovery rate after second treatment was higher in those which had been on their feet for a shorter or longer period between first and second treatment than in those which has been recumbent during this period.

Animals↗

[A successfully operated case of atrial septal defect with pheochromocytoma].

A 16 year-old male, who had marked hypertension and complained of palpitation, hyperhidrosis, headache and weight-loss, was diagnosed as atrial septal defect with pheochromocytoma. The first operation was performed for tumor on September 3, 1981. The hemodynamic change was recorded through the operation. The total systemic peripheral resistance and the total pulmonary resistance were improved after surgery. The systemic and pulmonary blood pressure were also decreased to normal level after surgery, although pulmonary arterial flow was increased. On one hundred and third day after this operation, radical correction for the cardiac malformation was performed. Operative and postoperative course were uneventful and the patient is free from all untoward complaints at this moment. Blood or urine catecholamine levels have also improved and are within normal limits.

Adolescent↗