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At least 1,315 records · Page 73Linked to original sources
PubMed1989

Ullrich's congenital atonic sclerotic muscular dystrophy. A case report.

A 5-year old girl with Ullrich's atonic-sclerotic muscular dystrophy is reported and 16 previously reported cases are reviewed. The clinical features, in particular proximal contractures, distal hyperextensibility, mild dysmorphism and hyperhidrosis, allow recognition of this subtype of congenital muscular dystrophy, which has no specific pathological characteristics. There is evidence in favour of an autosomal recessive mode of inheritance.

Child, Preschool↗
PubMed1997

Laparoscopic and thoracoscopic surgery in children and adolescents: a 3-year experience.

Our initial experience over the last 3 years with laparoscopic and thoracoscopic surgery in children and adolescents is reported. Between September 1992 and August 1995, a total of 215 laparoscopic and thoracoscopic procedures were performed: 32 appendectomies for acute appendicitis, 10 cholecystectomies for symptomatic gallstones, 11 procedures for adnexal pathology, 6 laparoscopies in children with nonpalpable testes, 3 diagnostic laparoscopies, and 153 thoracoscopic sympathectomies in children suffering from primary palmar hyperhidrosis. The post-operative course was uneventful in all cases. In 2 children with acute appendicitis we converted to the open technique due to technical difficulties. We are encouraged by the results of our initial experience. There is no doubt that laparoscopic cholecystectomy, laparoscopic surgery of adnexal pathology, and thoracoscopic sympathectomy, because of their numerous benefits - shorter operative time, hospitalization, and convalescence as well as less postoperative pain and improved cosmetic results - are replacing the open techniques. We are not convinced as yet of the advantages of laparoscopic appendectomy in children; we are presently performing both laparoscopic and conventional techniques and studying the various parameters in order to reach a more definite conclusion. Various other endoscopic surgical procedures will be carefully considered in the near future.

Acute Disease↗
PubMed1992

Transthoracic endoscopy for upper thoracic chemical sympathectomy.

Beginning in April 1989, we have performed eight upper thoracic chemical sympathectomies by transthoracic endoscopy. The indications were occlusive arterial disease in four patients and Raynaud's syndrome and palmar hyperhidrosis in two patients each. Transthoracic endoscopy was performed under general anesthesia, through the third costal interspace on the anterior mid-clavicular line. Five ml of phenol were injected into the parietal pleura covering the three proximal thoracic ganglia. The duration of thoracic drainage was 24 hours. The postoperative course was uneventful except for one case of subcutaneous emphysema and transient Horner's syndrome in three instances. There were no initial failures. Because of its simplicity and the short hospitalization period, chemical sympathectomy by transthoracic endoscopy constitutes a valuable alternative to conventional surgery. This technique is, however, limited in the case of antecedent pleuropulmonary disorders.

Adult↗
PubMedDate not supplied

State of the peripheral nervous system in patients with hypothalamic insufficiency.

An investigation was carried out by non-invasive techniques of the state of the peripheral nervous system in patients with a constitutional-acquired form of hypothalamic insufficiency. Ten healthy individuals were included in the control group. The presence of subclinical parasympathetic vegetative insufficiency was identified by cardiovascular tests. It was more marked in patients with an early onset of the neuroendocrine disturbances and longer course of the disease. In the opinion of the authors, this suggests the constitutional-acquired character of the vegetative disturbances. In addition, a slowing of the speed of conduction through the sudomotor preganglionic sympathetic fibers in the upper and lower extremities and the predominance of these disturbances in the group of patients with pronounced dysraphic status were identified. The latter suggests a defect of the laying down of these peripheral vegetative fibers. This investigation makes it possible to hypothesize that subclinical peripheral insufficiency is one of the factors governing the occurrence of both permanent (tachycardia, elevations of AP, hyperhidrosis) and paroxysmal vegetative disturbances (panic attacks, migraine) in hypothalamic insufficiency.

Adult↗
PubMed2006

Clinical use of non-A botulinum toxins: botulinum toxin type B.

Botulinum neurotoxin type B (BT, BT-B) has been used as NeuroBloc/MyoBloc since 1999 for treatment of cervical dystonia, hyperhidrosis, spastic conditions, cerebral palsy, hemifacial spasm, bladder dysfunction, spasmodic dysphonia, sialorrhoea, anal fissures, piriformis syndrome, various pain conditions and cosmetic applications. Generally, its therapeutic effects are comparable to BT type A (BT-A). The adverse effect profiles of BT-B and BT-A, however, differ considerably. BT-B has been found to produce more regional as well as systemic anticholinergic adverse effects, such as dryness of mouth, accommodation difficulties, conjunctival irritation, reduced sweating, dysphagia, heartburn, constipation, bladder voiding difficulties and dryness of nasal mucosa. In BT-B the relationship between autonomic and motor effects known from BT-A is substantially shifted towards autonomic effects. BT-B, therefore, should be used carefully in patients with autonomic disorders and in patients with concomitant anticholinergic therapy. If NeuroBloc/MyoBloc is used to treat cervical dystonia patients with antibody-induced failure of BT-A therapy, 86% of those will develop complete secondary therapy failure after five applications. If NeuroBloc/MyoBloc used to treat cervical dystonia patients without prior exposure to BT, 44% of those will develop complete secondary therapy failure after nine applications. NeuroBloc/MyoBloc, therefore, is associated with substantial antigenicity problems originating from a particular low specific biological potency. Systemic anticholinergic adverse effects and high antigenicity limits the clinical use of NeuroBloc/MyoBloc considerably.

Animals↗
PubMedDate not supplied

Adrenal bilateral incidentaloma by reactivated histoplasmosis.

We report a case of bilateral adrenal incidentaloma caused by the capsulatum variety of Histoplasma capsulatum diagnosed in a 74 years old man born in and a life time resident of Treviso, Italy, with the exception of two years spent in Pakistan (1964-1966) as a well-driller. The patient was hospitalized in 1995 for alcoholic chronic hepatitis, chronic Helicobacter pylori gastritis and post-infarction ischemic cardiomyopathy. Abdominal ultrasound incidentally showed bilateral adrenal masses (the right one 6.3 cm in diameter) confirmed by computed tomography, with adrenal function within normal limits. After three months, the patient was again hospitalized due to evening fever, asthenia, anorexia, weight loss and occasional hyperhidrosis. Abdominal ultrasound showed an increase of the right adrenal lesion with normal adrenal function. Ultrasound-guided fine needle aspiration did not prove useful for diagnosis. Accordingly, a laparotomy with bilateral biopsy was performed; histology showed the presence of numerous tissue form cells of H. capsulatum variety capsulatum. Serum anti-H. capsulatum antibodies were negative. Since March, 1996, the patient was given itraconazole and his symptoms quickly regressed but the computed tomography findings, however, have not changed and the patient has adrenal hypofunction that is being treated with cortisone acetate.

Adrenal Gland Diseases↗
PubMed2001

Skin disorders and thyroid diseases.

Thyroid disorders have a high prevalence in medical practice; they are associated with a wide range of diseases with which they may or may not share etiological factors. One of the organs which best show this wide range of clinical signs is the skin. This review is an attempt to approach most of the dermopathies reflecting several degrees of harmfulness, coming directly or indirectly from thyroid abnormalities, as well as to update current knowledge on the relationship between the thyroid and skin. We have proposed a primary classification of skin disorders, regarding thyroid involvement, into two main groups: 1) dermopathies associated with thyroid abnormalities, mainly with autoimmune thyroid diseases, like melasma, vitiligo, Sjogren's syndrome, alopecia, idiopathic hirsutism, pre-menstrual acne, bullous diseases, connective tissue diseases, hamartoma syndrome, atopy, leprosy and DiGeorge anomaly; and 2) dermopathies depending on the nature of the thyroid disorder, in which the evolution and outcome of the skin disorder depend on the thyroidal treatment in most cases, such as trophism and skin blood flow, myxedema, alopecia, onychodystrophy, hypo- and hyperhidrosis, xanthomas, intraepidermal bullae, carotenodermia, pruritus, flushing, pyodermitis, palmoplantar keratoderma, ecchymosis, etc. In some other cases, the skin disease which developed as a consequence of the thyroid abnormality can remain unaltered despite functional treatment of the thyroid problem, such as pretibial myxedema, thyroid acropachy and some cutaneous manifestations of multiple endocrine neoplasia types 2A and 2B.

Humans↗
PubMed1995

[Pachydermoperiostosis].

Pachydermoperiostosis (Friedreich-Erb-Arnold syndrome, Touraine-Solente-Golé syndrome) is a rare disease with an autosomal dominant mode of inheritance; it occurs almost exclusively in men. A report of typical pachydermoperiostosis in a 31-year-old man is presented. Associated features were clubbed digits of the hands and feet with watch-crystal nails, thickening of the skin and soft tissues, hyperhidrosis of hands and feet, hyperplasia of the sebaceous glands with seborrhoea, gynaecomastia, and ophthalmic abnormalities that had been present since puberty. Radiological examination revealed periosteal hyperostosis of the short and long bones. Endocrine disturbances were not detected. Skin biopsies showed hypertrophy of the skin and skin appendages. Primary (idiopathic, hereditary) pachydermoperiostosis should be distinguished from secondary (symptomatic) forms of the disease, which are often associated with lung tumours.

Adult↗
PubMed1997

[Multiple eccrine hidrocystomas in Parkinson disease].

We describe clinically and histologically typical eccrine hidrocystomas on the eyelids and forehead of a 60-year-old woman which were successfully treated with topical atropine. Concomitantly the patient had long-standing Parkinson's disease with typical facial seborrhea and hyperhidrosis. In this report, we review the literature about eccrine hidrocystomas and discuss the question of a causal relationship with Parkinson's disease in our patient.

Administration, Topical↗
PubMed2000

[Congenital pachyonychia type II (Jackson-Lawler syndrome)].

Pachyonychia congenita (PC) is a rare ectodermal dysplasia with variable expression. The condition is usually inherited as an autosomal dominant trait. Several classifications of PC have been proposed. Feinstein and colleagues suggested four clinical types of PC. Type II, the Jackson-Lawler-Syndrome, is characterized by multiple epidermal cysts, palmoplantar bullae and hyperhidrosis as well as natal teeth in addition to the main findings of pachyonychia, palmoplantar hyperkeratosis and follicular keratosis. We report two patients (father and son) with Jackson-Lawler-Syndrome and describe in detail pathogenesis, diagnostic criteria and treatment approaches as well as the different classifications of pachyonychia congenita.

Adolescent↗
PubMed2000

[Botulinum toxin: from poison to drug. A historical review].

Botulinumtoxin (BTX) is a neurotoxin produced from Clostridium botulinum under anaerobic conditions and is responsible for botulism, a notifiable, bacterial form of food poisoning. The first case of botulism is believed to have occurred in 1735. An epidemic in Southern Germany in 1793 claimed the death of over the half of those patients who had become ill through eating uncooked blood sausages. The term "pharmakon" is Greek and implicates that a drug originates from poison (potion, remedy). Theophrastus Bombast von Hohenheim known as Paracelsus (1493/94-1541) first described this duality with his dictum "alle ding sind gift und nichts on gift; alein die dosis macht das ein ding kein gift ist" (only the dose makes a remedy poisonous). In Baden-Württemberg in 1817, the poet and physician Dr. Justinus Christian Kerner described the symptoms of botulism, so that at this time botulism was also called Kerner disease. Until the turn of the century the reason for poisoning was not known. Van Ermengem succeeded in isolating the anaerobic bacterium causing botulism, but the specific mechanism of BTX was only established after the second World War. In the late seventies the ophthalmologist Dr. Alan Scott used BTX the first time in the treatment of strabismus. The drug was then used in the treatment of several muscle spasticities such as, for example, torticollis or hemifacial spasm. Only recently BTX has been successfully used for focal hyperhidrosis. We review the history of botulinum toxin from its discovery in the nineteenth century and the research into its effect in the middle of the 20th century up to its clinical use at the present time.

Botulinum Toxins↗
PubMed2004

[Isaacs' syndrome. Diagnosis and differential diagnosis of neuromyotonia].

Neuromyotonia is a clinical and electrophysiological syndrome of spontaneous muscle fiber activity due to hyperexcitability of peripheral nerve origin causing generalised, visible myokymia and muscular cramps. Electromyography shows abnormal doublet and triplet discharges of high intraburst frequency as well as myokymic and neuromyotonic discharges. Fasciculations and fibrillation potentials are common. Most commonly, neuromyotonia is an acquired immune-mediated disorder (Isaacs' syndrome) showing elevated antibody levels against presynaptic, voltage-gated, potassium channels. Some of these patients have additional autonomic (hyperhidrosis) and/or CNS symptoms similar to those from limbic encephalitis (referred to then as Morvan's syndrome). We report on a patient with Isaacs' syndrome and discuss the clinical and electrophysiological features, pathophysiology, diagnosis, and differential diagnosis of diseases with peripheral nerve hyperexcitability.

Diagnosis, Differential↗
PubMed1996

[CT-guided neurolysis. Status of the technique and current results].

INTRODUCTION: Both thoracic and lumbar sympathectomy and celiac ganglion block could be established as effective methods; side effects are rarely reported. Only the high accuracy of CT-guided positioning can make this possible. Severe side effects are merely sporadic if the proper technique is applied. MATERIAL AND METHODS: We used 21G or 22G fine needles. All CT-guided percutaneous neurolysis methods require a proper blood coagulation. Most common CT scanners are suitable for neurolysis if there is enough room for maintaining sterile conditions. All neurolysis methods involve sterile puncture of the ganglia under local anesthesia, a test block with anesthetic and contrast agent to assess the clinical effect and the definitive block with a mixture of 96% ethanol and local anesthetic. This allows us to correct the position of the needle if we see improper distribution of the test block or unwanted side effects. Though inflammatory complications of the peritoneum due to puncture are rarely seen, we prefer the dorsal approach whenever possible. RESULTS: Seven of 20 legs showed at least transient clinical improvement after CT-guided lumbar sympathectomies; 13 legs had to be amputated. Results of the methods in the literature differ. For lumbar sympathectomy, improved perfusion is reported in 39-89%, depending on the pre-selection of the patient group. DISCUSSION: It was recently proved that sympathectomy not only improves perfusion of the skin but also of the muscle. The hypothesis of a steal effect after sympathectomy towards skin perfusion was disproved. Modern aggressive surgical and interventional treatment often leaves patients to sympathectomy whose reserves of collateralization are nearly exhausted. We presume this is the reason for the different results we found in our patient group. For thoracic sympathectomy the clinical treatment depends very much on the indications. Whereas palmar hyperhidrosis offers nearly 100% success, only 60-70% of patients with disturbance of perfusion have benefited. Results in celiac ganglia block also differ. Patients with carcinoma of the pancreas and other organs of the upper abdomen benefit in 80-100% of all cases, patients with chronic pancreatitis in 60-80%.

Humans↗
PubMed1999

Partial transient osteoporosis of the hand.

OBJECTIVE: To describe the radiographic and scintigraphic findings of partial transient osteoporosis (PTO) of the hand. To discuss the relationship of PTO and other localized demineralizing diseases. DESIGN AND PATIENTS: Three patients with PTO that affected two or three digits of the hand are reported. Two patients were middle-aged women and the third was a young man. All presented with a history of trauma to the hand. All patients experienced localized burning pain, swelling and vasomotor changes including redness of the skin, hyperhidrosis and signs of vasomotor instability of the involved fingers. Plain radiography and bone scanning were used in the diagnosis and follow-up of these cases. RESULTS: All patients had a radial distribution of the osteoporosis that involved adjacent rays. In all patients two rays were involved. The radiographic changes manifested as minimal patchy osteoporosis involving the cortical, cancellous, subarticular and subperiosteal bone with no articular involvement. The increased uptake on scintigraphy coincided with the radial distribution of the osteoporosis. All patients improved on physical therapy and were symptom-free approximately 6 months after the initial injury. These patients were followed up for more than 2 years. CONCLUSION: PTO of the hand is an uncommon disease with typical clinical and radiographic findings. Bone scintigraphy confirms the partial involvement of the hand.

Adolescent↗
PubMed2005

Anatomical variations of the second thoracic ganglion.

In recent years the second thoracic ganglion has gained anatomical significance as an important conduit for sympathetic innervation of the upper extremity. Thoracoscopic excision of the second thoracic ganglion is now widely recognized as affording the most effective treatment option for palmar hyperhidrosis. This study recorded the incidence, location and associated additional neural connections of the second thoracic ganglion. Bilateral dissection of 20 adult cadavers was undertaken, and all neural connections of the second thoracic ganglion were recorded. Nineteen cadavers (95%) demonstrated additional neural connections between the first thoracic ventral ramus and second intercostal nerve. These were classified as either type A (47.5%) or type B (45%) using the intrathoracic ramus (nerve of Kuntz) between the second intercostal nerve and the ventral ramus of the first thoracic nerve as a basis on both right and left sides. The second thoracic ganglion was commonly located (92.5%) in the second intercostal space at the level of the intervertebral disc between the second and third thoracic vertebrae. Fused ganglia between the second thoracic and first thoracic (5%) and stellate (5%) ganglia were noted. These findings should assist the operating surgeon with a clear knowledge of the anatomy of the second thoracic ganglion during thoracoscopic sympathectomy with a view to improving the success rate for upper limb sympathectomy.

Adult↗
PubMed2002

A combination of carbamazepine/tiapride in outpatient alcohol detoxification. Results from an open clinical study.

This was an open, prospective study to examine the efficacy, practicability and medical safety of a combination of carbamazepine and tiapride in outpatient detoxification of alcohol-dependent patients. Patients were carefully screened for relevant neuropsychiatric disorders and then seen on a daily outpatient basis. Patients received medication if the initial CIWA-A score exceeded 16 points. Fifty consecutively admitted patients entered the program; 49 (98 %) successfully ended treatment. The mean initial dose for carbamazepine was 628 mg or 332 mg for tiapride. No serious medical complications or adverse events were observed. In general, medication was well tolerated. Withdrawal symptomatology as indicated by CIWA-A scores clearly decreased over time. Most frequently named side effects were sedation (63 %), hyperhidrosis (49 %), lack of drive (38 %), dry mouth (31 %) and orthostatic dysregulation or vertigo (22 %). Results from this study suggest that a combination of the anticonvulsant carbamazepine and tiapride is an effective and safe treatment for outpatient alcohol detoxification in patients with moderate severity of withdrawal syndrome.

Adult↗
PubMed2006

Botulinum toxin in multiple sclerosis.

Local administration of botulinum toxin (BoTx) inhibits presynaptic acetylcholine release. All cholinergically innervated muscles and glands can be paralyzed accordingly. Studies on the application of BoTx in multiple sclerosis have shown good results for focal spasticity, in particular, of the extremities. The first promising data were published on bladder disorders, especially with regard to detrusor sphincter dyssynergia and detrusor hyperreflexia. Hyperhidrosis, hypersalivation and proctologic symptoms might be other areas of application. Approval, however, has been greatly restricted which limits the therapeutic range of use.

Botulinum Toxins, Type A↗
PubMed1991

Contrast radiography and effects of thoracic sympathetic ganglion block--anatomical analysis.

Thoracic sympathetic ganglion block is less effective than lumbar sympathetic ganglion block due to differences in the anatomical structure of these regions. Contrast radiographic findings and an analysis of the effects of lumbar sympathetic ganglion block have been reported, but there are few reports concerning thoracic sympathetic ganglion block. The relationship between contrast radiography findings and the effects of thoracic sympathetic ganglion block were studied in 131 block procedures which mainly had hyperhidrosis.

Journal Article↗