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[Botulinum toxin for treatment of gustatory sweating. A prospective randomized study].

BACKGROUND: Botulinum toxin A has meanwhile become a proven method for treatment of gustatory sweating (focal hyperhidrosis, Frey's syndrome). Clear-cut recommendations regarding dosage of botulinum toxin A in Frey's syndrome are currently not available. The aim of this prospective randomized study therefore was to investigate botulinum toxin A with respect to its efficacy in Frey's syndrome, the ideal dose yielding maximal duration of the effect, and patient contentedness as well as unwanted side effects in patients of the Clinic of OMF Surgery at the Ruhr-University of Bochum. PATIENTS AND METHOD: Twenty patients suffering from severe Frey's syndrome as a result of operations of the parotid gland were examined with the starch iodine test according to Minor. The gustatory skin areas were re-examined after intracutaneous injection of botulinum toxin A for up to 1 year. The patients ( n=20) were randomly assigned to two different treatment groups (group I: 2 MU/cm(2), n=10; group II: 3 MU/cm(2), n=10). RESULTS: Mean sweating skin areas in the two treatment groups ranged between 39+/-9 and 32+/-12 cm(2), respectively. A single injection of 3 MU botulinum toxin A resulted in a nearly complete blockade of gustatory sweating for the observation period of 1 year. In the group treated with 2 MU botulinum toxin A, 44% of the total gustatory skin areas were still sweating, thus necessitating a second injection of botulinum toxin A in these patients. CONCLUSION: Intracutaneous injection of botulinum toxin A represents a highly effective and minimally invasive procedure for the treatment of Frey's syndrome. This study shows for the first time that a dosage of 3 MU/cm(2) of botulinum toxin A achieves a complete and reliable blockade of gustatory sweating lasting for at least 12 months. This dose may therefore be recommended for treatment of this syndrome.

Adult↗

Is the internet the best resource for blushers?

Blushing can be a disabling condition that causes sufferers great distress. It causes patients to become socially isolated, frequently being labelled as "shy". Any referral to their condition is embarrassing; even a discussion with a health care professional requires a great deal of courage. Even then, the information from non-specialists is often inaccurate and inadequate and fails to inform patients of the possible treatment options available. In today's era the internet has become an important information medium. This information on almost any subject is widely available to the general public and can be accessed at any time. It is available to blushers and health care professionals alike. It offers a non-embarrassing method of seeking help for an embarrassing condition. It is a medium for specialists to inform sufferers incognito about such a condition. We report our experience from our website: a generalised hyperhidrosis, blushing and sympathectomy information web site (www.sympathectomy.co.uk) by analysing the number of hits and requests for information. We discuss the statistics of internet access to our website including the "hit rate" and enquiry rate. Paradoxically 75% of all enquiries have been from blushers with only 25% coming from patients with hyperhidrosis.A properly designed, accurate and dynamic web site tailored towards users' needs can be used to effectively educate an information hungry but shy group.

Adolescent↗

Symptoms of autonomic failure in Parkinson's disease: prevalence and impact on daily life.

Frequency and clinical importance of autonomic failure in idiopathic Parkinson's disease (PD) are discussed controversially. 141 patients with PD and 50 healthy age-matched control subjects were interviewed for symptoms of autonomic failure and their influence on daily life using a questionnaire. In PD patients, the prevalence of orthostatic dizziness, bladder dysfunction, erectile dysfunction and hyperhidrosis was significantly higher compared with controls. About 50% of PD patients rated the impact of the symptoms of autonomic failure on their daily lives as "a lot" or "very much" due to orthostatic dizziness, bladder dysfunction and constipation, which were more statistically significant than in age-matched controls. Prevalence and number of autonomic symptoms were not correlated with duration and severity of PD. In 32% of patients, impaired cardiovascular regulation was found by standardized cardiovascular function tests. If testing showed abnormal findings, orthostatic dizziness, bladder dysfunction, constipation and erectile dysfunction were significantly more frequent than in patients with normal regulation, but the impact on daily life due to these symptoms differed significantly only for bladder dysfunction between groups. It is concluded that autonomic failure is a clinically relevant, pervasive problem in PD and compromise patients' daily life activities in all stages of the disease. This underlines the necessity to adequately search for and treat these non-dopaminergic symptoms during the whole course of the disease.

Activities of Daily Living↗

Chronic paroxysmal hemicrania: from the index patient to the disease.

The first patient with chronic paroxysmal hemicrania (CPH), a 41-year-old woman, first seen in 1961, was followed until an adequate treatment was found, 12 years later. Clinically, attack frequency and duration differed widely from the general pattern of cluster headache. Ocular variables, such as intraocular pressure and corneal indentation pulse amplitudes, also differed in our case (clear symptomatic side increment during attacks) and cluster headache. Pupil reactions to directly and indirectly acting sympathicomimetic drugs were also vastly different in our case and cluster headache: no signs of Horner s syndrome in our patient, while cluster headache exhibits a "Horner-like pattern." In cluster headache, there is a relative hypohidrosis in the forehead on the symptomatic side if body temperature is increased, and a clear hyperhidrosis on direct parasympathomimetic stimulation. This was not so in our case. Indomethacin was highly effective in our case, while "cluster headache drugs," such as ergotamine/sumatriptan, were ineffective. Indomethacin was inactive in cluster headache. Accordingly, our case seemed to differ decisively from cluster headache: CPH had been discovered.

Humans↗

Thoracoscopic sympathectomy: endobronchial anesthesia vs endotracheal anesthesia with intrathoracic CO2 insufflation.

PURPOSE: To compare clinical advantages and hemodynamic and respiratory changes during one lung-collapsed ventilation (OLCV) using a double-lumen tube (DLT) or a single-lumen tube (SLT) with intrathoracic CO(2) insufflation, in patients undergoing thoracic sympathectomy (TS) under general anesthesia. METHODS: One hundred and twenty-five patients (94 men and 31 women) undergoing TS for the treatment of palmar hyperhidrosis (PH) were randomly allocated to two groups: group A (68 patients; age, 29 +/- 6 years) in whom DLT was used, and group B (57 patients; age, 32 +/- 3 years) in whom SLT with intrathoracic CO(2) insufflation at a rate of 0.5-1 l.min(-1) and sustained intrathoracic pressure at 6 mmHg insufflation were used. Anesthesia was maintained with 1 minimum alveolar concentration (MAC) isoflurane in 50% nitrous oxide in oxygen with incremental doses of sufentanil and atracurium when required. Arterial blood gases were measured in 10 patients in group B. Hemodynamic and respiratory parameters were obtained perioperatively. RESULTS: There were no significant differences in hemodynamic and respiratory parameters between the two groups during the study phases, except for the arterial oxygen saturation (SpO(2)). The times required for anesthesia and surgery were significantly shorter in the SLT group than in the DLT group. SpO(2) during OLCV was 95 +/- 1% with DLT and 98 +/- 1% with SLT, with a significant difference. Three patients had an SpO(2) of less than 90% in the recovery room, where the chest tube position was readjusted, with no further sequelae. CONCLUSION: General anesthesia with SLT and intrathoracic CO(2) insufflation provides optimal operating conditions, adequate oxygenation, and perfect hemodynamic stability during TS.

Clinical Trial↗

Normal sympathetic nerve activity in a reflex sympathetic dystrophy with marked skin vasoconstriction.

Intraneural recording in skin fascicles of the left ulnar nerve was performed in a 51 year old patient with reflex sympathetic dystrophy (allodynia, marked vasoconstriction in the ulnar part of the left hand) following Vth finger amputation. Skin sympathetic activity showed normal characteristics with a weak discharge at rest whereas bursts of impulses could be evoked by sympatho-excitatory stimuli. Sympathetic bursts induced by painful stimuli were few, also during long-lasting allodynic pain. Sympathetic bursts induced by inspiratory gasps were not associated with excacerbation of pain. The discrepancy between the marked skin vasoconstriction and the low resting sympathetic discharge underlines the fact that sympathetic effector organ hyperactivity (i.e. vasoconstriction and/or hyperhidrosis) cannot be taken as evidence for increased sympathetic discharge. In the present case, the neuropathic pain syndrome cannot be considered as maintained by an increased sympathetic neural discharge to the symptomatic limb.

Fingers↗

Pachyonychia congenita with laryngeal involvement.

Pachyonychia congenita (Jadassohn-Lewandowsky Syndrome) is a rare autosomal dominant disorder characterized by nail dystrophy, hyperkeratosis of the palms and soles, leukoplakia of the mucosa of the upper respiratory tract and anus, follicular keratoses especially about the knees and elbows, and palmar and plantar hyperhidrosis. We present a patient with pachyonychia congenita and an exophytic lesion in the larynx at the posterior commissure. He is the youngest of 4 family members with this disorder covering 3 generations. Each of the 4 patients also exhibited both oral leukoplakia compatible with the Jadassohn-Lewandowsky syndrome (Ikonograph Dermatol. Lab., 1 (1906) p. 29), and subcutaneous cysts of the face and scalp as described by Jackson and Lawler (Ann. Eugenics (1951) 142.

Adolescent↗

Dermatopathia pigmentosa reticularis: a report of a family demonstrating autosomal dominant inheritance.

Dermatopathia pigmentosa reticularis is a rare heritable disorder consisting of a triad of cutaneous findings including reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. In addition to this triad, other variable features of dermatopathia pigmentosa reticularis have been described, including adermatoglyphia, hypohidrosis or hyperhidrosis, and palmoplantar hyperkeratosis. Only 10 cases have been described in the world literature to date, and no clear inheritance pattern has been demonstrated. We present a patient with the classic features of the dermatopathia pigmentosa reticularis triad, along with adermatoglyphia, hypohidrosis, and punctate hyperkeratosis of the palms and soles. A family pedigree demonstrates the autosomal dominant inheritance of dermatopathia pigmentosa reticularis.

Adult↗

Reflex sympathetic dystrophy: occurrence of chronic edema and nonimmune bullous skin lesions.

BACKGROUND: Reflex sympathetic dystrophy (RSD) is a poorly understood syndrome of post-traumatic pain, autonomic dysfunction, and progressive tissue atrophy. Classical descriptions of the cutaneous manifestations of RSD are usually limited to skin atrophy, vascular instability, and hyperhidrosis. OBJECTIVE: Our objective was to further delineate the cutaneous changes in RSD. METHODS: We have observed RSD-related inflammatory and bullous lesions in nine patients with active RSD. RESULTS: Eight patients had significant edema of involved skin, two patients had evidence of a pigmented purpura-like inflammatory dermatitis, and two other patients had bullae on involved skin. Ultrastructural studies on a biopsy specimen from one patient with recurrent bullae revealed a disrupted basement membrane and abnormal anchoring fibrils. CONCLUSION: Skin disease in RSD is more diverse than commonly appreciated and includes severe edema, inflammatory lesions, and a nonimmune bullous eruption.

Adult↗

Reflex sympathetic dystrophy with prominent involvement of the nail apparatus.

After closed hand trauma, a 17-year-old boy had acute inflammatory changes that resembled bacterial whitlows of the third and fourth right fingers. Clearing of the inflammatory changes was followed by the development of cyanosis, hyperhidrosis, and roentgenographic evidence of patchy osteoporosis in the involved extremity. Findings of a biopsy specimen revealed that the inflammatory lesions in the proximal nail folds were caused by proliferation of capillary vessels embedded in edematous loose connective tissue. This is the first report of cutaneous histopathologic findings in the first stage of reflex sympathetic dystrophy, although similar features have been described in synovial and bone biopsy specimens of patients with reflex sympathetic dystrophy.

Adolescent↗

Inhibiting noradrenergic overactivity by inhibition of thromboxane and concomitant activation of opiate receptors via dietary means.

A yin-yang hypothesis is presented linking noradrenergic activity, thromboxane, melatonin, left hemisphere functioning, and cyclic AMP on the one hand, and dopamine, beta-endorphin, calcium, right hemisphere functioning, and cyclic GMP on the other. It is further suggested that there is a yoking of NA, TXA2, serotonin and melatonin in the left hemisphere, and a similar yoking of DA, BE, calcium and cGMP in the right. Evidence is presented to support the hypothesis that each element (NA, TXA2, etc.) on one side can modulate or balance a corresponding element (DA, BE, etc.) on the other. It is suggested that thromboxane is the key element in noradrenergic overactivity and that not taking this into consideration has confounded much prior research. This theory takes into account information processing models as well as pharmacological data and neurochemical theory on coupling of adenylate cyclase to its hormone receptors. Inhibiting noradrenergic overactivity can be obtained by inhibiting thromboxane and concomitantly activating opiate receptors. This protocol may have clinical utility in treating a wide range of disorders such as: anxiety, depression, schizophrenia, sleeplessness, withdrawal states, enuresis, Gilles de la Tourette syndrome, Parkinsonism, Alzheimers, dementia, anorexia, infant ruminations, essential tremor, spasticity of spinal cord injury, diarrhoea, ulcerative colitis, extrapyramidal symptoms, akathisia, neuroleptic malignant syndrome, attention deficit disorder, hyperhidrosis, and possibly AIDS.

Brain Chemistry↗

Athlete's foot caused by pseudomonas aeruginosa.

An enzymatically active pigment-producing clinical isolate of Pseudomonas aeruginosa was found to produce a diffusible antifungal product that was shown to be inhibitory to the growth of several dermatophytes, specifically, Trichophyton rubrum, Trichophyton mentagrophytes, Microsporum gypseum, and Microsporum audouini. In this study, Trichophyton rubrum was used as the test organism. The antifungal product was partially purified by Sephadex column chromatography and was found to be stable at 5 degrees, 25 degrees, and 37 degrees C. Several investigators have alluded to the fact that as asymptomatic cases of dermatophytosis simplex progress to symptomatic dermatophytosis complex, the bacterial profile changes from a gram-positive bacterial ecosystem to a gram-negative bacterial over-growth. The primary event in the pathogenesis of interdigital athlete's foot is the invasion of the horny layer by dermatophytes. This presents as a mild to moderate scaly lesion and is asymptomatic. As a result of predisposing factors, such as hyperhidrosis, occlusion by tight shoes, minute abrasions due to friction, and fungal-infected skin surfaces, dynamic overgrowth of opportunistic gram-negative bacilli prevails. As the gram-negative population increases, the recovery of dermatophytes dramatically diminishes, until a point is reached when no dermatophytes can be recovered from clinically symptomatic tinea pedis. Pseudomonas aeruginosa is inhibiting its fungal competitor Trichophyton rubrum by producing a diffusible antifungal agent into the infectious environment of the intertriginous foot lesion. Clinically, the patient is diagnosed as having tinea pedis; laboratory culture for fungus and KOH are negative, and what was a paradox just a few years ago can currently be identified and treated appropriately as gram-negative athlete's foot.

Gram-Negative Bacteria↗

Diagnostic and therapeutic thoracoscopy: techniques and indications in pulmonary medicine.

Recently thoracoscopy has been used with increasing frequency for the diagnosis and treatment of pleuropulmonary diseases. METHODS: The main requirements for thoracoscopy are rigid telescopes, forceps, scissors, stapler and a video recorder. The procedure can be performed either under general anaesthesia with or without double lumen intubation or under neuroleptanalgesia after inducing an artificial pneumothorax. At the end of the procedure a chest tube should always be inserted even if it is only for a few minutes until the lung re-expands after diagnostic thoracoscopy. Complications are exceptional and mortality is less than 0.017%. INDICATIONS AND RESULTS: Thoracoscopy is useful for diagnosis of a number of lung diseases. For pleural effusion, the sensitivity of thoracoscopy is 92-97% and its specificity is 99%. This is much better than needle pleural biopsy and/or fluid cytology. In malignant mesothelioma, thoracoscopy allows accurate staging. Similarly in spontaneous pneumothorax, classification based on the endoscopic aspects of the lung according to the classification of Vanderschueren allows a better selection of therapeutic alternatives. For diffuse pulmonary diseases, thoracoscopic lung biopsy has a sensitivity ranging from 60-98% depending on whether the underlying disease is sarcoidosis, idiopathic fibrosis, collagenous diseases or other rare diseases. Interventional thoracoscopy is a rapidly expanding domain. In this review the most widespread techniques are summarized. Thoracoscopic pleurodesis is performed for pleural effusion. It can be achieved by talc poudrage but other methods are available. For spontaneous pneumothorax, pleurodesis must be associated with treatment of the causal lesions. The other therapeutic procedures described here are sympathectomy for palmar hyperhidrosis, pulmonary biopsy using an endo-GIA stapler and pericardial biopsy.

Humans↗

Treatment of facial chromhidrosis with botulinum toxin type A.

BACKGROUND: Hyperhidrosis is an idiopathic condition of exaggerated sweat production by the eccrine glands that affects approximately 1% of the population. There are many viable therapeutic options and the use of botulinum toxin has become an important treatment option and received FDA approval for this disorder in July 2004. The other forms of aberrant sweating; bromhidrosis (malodorous) and chromhidrosis (pigmented) are much rarer and more recalcitrant to treatment. METHOD: This is the first case report of dark-colored facial sweat in a young woman that was adequately controlled with botulinum toxin. CONCLUSION: Chromhidrosis is an unusual clinical entity with an ill-defined glandular etiology. The successful response to botulinum toxin supports the eccrine gland as a source of the sweat production and, furthermore, that the administration of this toxin should be considered as a form of therapy.

Adult↗

New indications for botulinum toxin in rheumatology.

Previously known only as a deadly bacterial poison responsible for severe paralysis, botulinum toxin is now a well-recognized therapeutic agent used to relieve involuntary movements, dystonia-related functional impairments, spasticity, and autonomic disorders such as hyperhidrosis. Musculoskeletal pain in patients with rheumatic disorders is among the emerging indications for botulinum toxin therapy. Preliminary data have been obtained in patients with cervical or thoracolumbar myofascial pain syndrome, chronic low back pain, piriformis muscle syndrome, tennis elbow, and stiff person syndrome. At present, the effects of botulinum toxin and its use for pain relief remain controversial. Carefully designed prospective trials are needed to investigate the efficacy and safety of botulinum toxin in pain disorders.

Arthralgia↗

Left cardiac sympathetic denervation as the first-line therapy for congenital long QT syndrome.

Congenital long QT syndrome (LQTS) is a cardiac electrophysiological disorder due to genetic mutations. Patients with LQTS, if untreated, have a high incidence of ventricular tachycardia and cardiac arrest. Adrenergic activities are believed to play a major role in triggering the onset of cardiac events. The current mainstay of therapy for LQTS is oral beta-blockers, which improves clinical symptoms and reduces the incidence of sudden cardiac death in approximately 70% of the patients. Left cardiac sympathetic denervation (LCSD) is an alternative therapy for patients who are resistant to beta-blockers. Its clinical use, however, has been hindered by the complexity of the procedure and complications after the surgery. Video-assisted thoracoscopic sympathectomy has been used to treat patients with palmar and axillary hyperhidrosis. We suggest that the use of the microinvasice thoracoscopic technique may greatly simplify the LSCD procedure, making it the first-line therapy for LQTS.

Cardiac Surgical Procedures↗

Inherited neuromyotonia: a clinical and genetic study of a family.

Neuromyotonia is a disorder of peripheral nerve hyperexcitability characterized by myokymia, muscle cramps and stiffness, delayed muscle relaxation after contraction (pseudomyotonia), and hyperhidrosis, associated with well described spontaneous electromyographic features. It is usually an acquired disorder associated with autoantibodies against neuronal voltage-gated potassium channels. However, mutations of KCNA1, encoding the K(+) channel subunit hKv1.1, have been reported in rare families with neuromyotonia, and mutations in KCNQ2, encoding voltage-gated potassium M channel subunit, in families with benign neonatal seizures and myokymia. We report a three-generation family with inherited neuromyotonia without evidence of immunological involvement. Genetic study excluded mutations in KCNA1, KCNA2, KCNA6 and KCNQ2 genes. Our study does not completely exclude the involvement of other genes encoding ion channels subunits in the pathogenesis of this disorder. Further studies of familial cases will shed light on the molecular basis of inherited neuromyotonia.

Adult↗