1/48. Autonomic dysfunction associated with multiple pelvic ganglioneuromas.We describe the case of a 59-year-old male in which several symptoms of autonomic dysfunction resulted associated with the presence of multiple ganglioneuromas in the adrenal glands and in paravertebral spaces. In a 2-year period, the patient developed sexual dysfunctions, micturitional disturbances and severe orthostatic hypotension and was erroneously diagnosed as having primary autonomic failure. However, the examination of all the autonomic functions showed that papillary function and cardiovascular reflexes were normal. CT scan of the abdomen revealed the presence of several small masses in the adrenal glands and along the lumbar sympathetic chain. Cytologic examination of the adrenal glands showed clusters of ganglionlike cells interspersed with schwann cells, leading to the diagnosis of ganglioneuromas. patients with signs and symptoms of autonomic dysfunction need an extensive clinical and laboratory examination of all the autonomic functions in order to exclude secondary causes of the symptomatology.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/48. Evaluation of autonomic nervous system function with spectral analysis of heart rate variability in a case of tetanus.The autonomic nervous system is affected in a wide variety of neurological disorders. Its dysfunction may play an important role in the clinical course and may result in serious complications, such as cardiac arrest. We report a case of tetanus who presented with severe autonomic nervous system dysfunction which was detected by spectral analysis of heart rate variability monitored over 24 h. This is a semi-quantitative method for evaluation of the status of the autonomic nervous system. In the present case, the analysis revealed profoundly decreased activity of both sympathetic and parasympathetic nervous system modulation of cardiac rhythm. The parasympathetic nervous system activity was more severely impaired than that of the sympathetic nervous system. The relative predominance of the sympathetic nervous system in the present case may have resulted in unopposed sympathetic nervous system hyperactivity manifested in this patient by tachycardia and excessive sweating. We further infer that the documented diminished buffering capacity of the autonomic nervous system may have lead to a sudden cardiac arrest in our case. Thus, spectral analysis of heart rate variability is a non-invasive and sensitive method for evaluating the status of the autonomic nervous system of critically ill patients in the hospital setting.- - - - - - - - - - ranking = 21.685841449987keywords = sweat (Clic here for more details about this article) |
3/48. Auriculotemporal nerve syndrome.INTRODUCTION: Auriculotemporal nerve syndrome is characterized by erythema, perspiration, heat and pain localized in the area supplied by the auriculotemporal nerve in response to gustatory stimuli after the ingestion of different types of food. This syndrome may be confused with food allergy. CASE REPORT: A 21-year-old woman complained of erythema, sweat and heat in the right cheek after intake of several foods such as chocolate, fruits, and nuts for the previous 8 months. She had fractured her jaw two years previously. methods: skin prick tests were performed with a standard battery of common inhalant allergens and with an extensive panel of food allergens. Prick-by-prick tests were also performed with fruits, nuts, and cacao. Total and specific IgE were measured. Open oral food challenge test was performed. RESULTS: skin prick tests were positive for grass and olive pollen. Prick-by-prick tests and specific IgE antibodies to the different foods were all negative. Open oral challenge test with apple reproduced the symptoms. CONCLUSIONS: This benign syndrome is often misdiagnosed as a food allergy.- - - - - - - - - - ranking = 21.685841449987keywords = sweat (Clic here for more details about this article) |
4/48. Triple-A syndrome with prominent ophthalmic features and a novel mutation in the AAAS gene: a case report.BACKGROUND: Triple-A syndrome (Allgrove syndrome) is an autosomal recessive disorder characterized by adrenal insufficiency, alacrima, achalasia, and - occasionally - autonomic instability. Mutations have been found in the AAAS gene on 12q13. CASE PRESENTATION: We present the case of a 12 year-old boy with classic systemic features of triple-A syndrome and several prominent ophthalmic features, including: accommodative spasm, dry eye, superficial punctate keratopathy, and pupillary hypersensitivity to dilute pilocarpine. MRI showed small lacrimal glands bilaterally. dna sequencing of PCR-amplified fragments from the 16 exons of the AAAS gene revealed compound heterozygosity for a new, out-of-frame 5-bp deletion in exon 15, c1368-1372delGCTCA, and a previously-described nonsense mutation in exon 9, c938C>T, R286X. CONCLUSIONS: In addition to known ophthalmic manifestations, triple-A syndrome can present with accommodative dysregulation and ocular signs of autonomic dysfunction.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
5/48. Hypothalamic activation in trigeminal autonomic cephalgia: functional imaging of an atypical case.We report headache induced BOLD changes in an atypical case of trigeminal autonomic cephalgia (TAC). A 68-year-old patient was imaged using fMRI during three attacks of a periorbital head-pain with a average duration of 3 min. During the attacks, left sided conjunctival injection, rhinorrhea, lacrimation, facial sweating and hypersalivation were apparent. These attacks were usually partly responsive to oxygen administration but otherwise refractory to any drug. The patient described either attacks with a duration of one minute or less or longer attacks persisting for maximum of 20 min with headaches occurring up to 100 times a day. When considering the symptoms, frequency, duration and therapeutic response of the patient's headache, no clear-cut classification to one of the subtypes of trigeminal autonomic cephalgias (cluster headache, paroxysmal hemicrania, SUNCT) or trigeminal neuralgia was possible. The cerebral activation pattern was similar but not identical to those previously observed in cluster headache and SUNCT with a prominent activation in the hypothalamic grey matter. This case study underlines the conceptual value of the term TAC for the group of headaches focusing around the trigeminal-autonomic reflex. Our results emphasize the importance of the hypothalamus as key region in the pathophysiology of this entity.- - - - - - - - - - ranking = 21.685841449987keywords = sweat (Clic here for more details about this article) |
6/48. Harlequin syndrome in two athletes.Two cases are reported of harlequin syndrome, a disorder of the sympathetic nervous system in which sweating and flushing of the skin in response to exercise is diminished. This condition is most likely to be first noticed in sporting situations.- - - - - - - - - - ranking = 21.685841449987keywords = sweat (Clic here for more details about this article) |
7/48. Segmental facial anhidrosis and tonic pupils with preserved deep tendon reflexes: a novel autonomic neuropathy.A 31-year-old woman had exertional right-sided hemifacial flushing and sweating. Examination demonstrated slightly dilated pupils with absent constriction to light and a tonic near response and redilatation, features consistent with adie syndrome. Neurological examination was otherwise normal, including preservation of deep tendon reflexes. magnetic resonance imaging of brain and spine were normal. The combination of unilateral loss of sudomotor and vasomotor activity without loss of ocular sympathetic innervation fulfills the diagnosis of Harlequin syndrome. The combination of Harlequin and Adie syndromes has been called Ross syndrome, but the preservation of deep tendon reflexes precludes a diagnosis of Ross syndrome in our patient. This previously undescribed variant adds further complexity to the spectrum of autonomic neuropathies.- - - - - - - - - - ranking = 21.685841449987keywords = sweat (Clic here for more details about this article) |
8/48. Harlequin syndrome with crossed sympathetic deficit of the face and arm.Harlequin syndrome is characterized by unilateral hyperhidrosis and flushing, which are predominantly induced by heat or exercise. Usually, the sympathetic deficits confine to the face. Rarely, the autonomic deficits involve the arm or the parasympathetic neurons in the ciliary ganglia. We report a 43-yr-old woman who presented with facial flushing and sweating in the right side, which were mainly induced by exercise. The facial flushing accompanied relative coldness in the right arm. valsalva maneuver, cold pressure and 0.125% pilocarpine test, and computed tomography of the chest were normal. The crossed sympathetic deficit in the left face and right arm suggested that the lesions were multifocal. The sympathetic impairment in our patient may lie on a spectrum of pre- and postganglionic autonomic dysfunction, which was observed in Holmes-Adie, Ross, and guillain-barre syndrome.- - - - - - - - - - ranking = 21.685841449987keywords = sweat (Clic here for more details about this article) |
9/48. Blisters, ulceration and autonomic neuropathy in carpal tunnel syndrome.We describe three patients with trophic ulceration and blistering of the fingertips associated with carpal tunnel syndrome. One of the patients also had non-insulin-dependent diabetes mellitus. Autonomic neuropathy distal to the carpal tunnel was probably present in all subjects at the time of presentation; in the patient with recent symptoms the skin was warm, and sweating was virtually absent, whilst the other two patients described cold skin, consistent with prolonged autonomic neuropathy.- - - - - - - - - - ranking = 21.685841449987keywords = sweat (Clic here for more details about this article) |
10/48. QSART in idiopathic pure sudomotor failure.QSART (quantitative sudomotor axon reflex testing) was performed in a patient with idiopathic pure sudomotor failure. Generalized reduction in thermoregulatory sweating and complete absence of axon reflex sweating were observed, suggesting a deficit of sweat gland cholinergic synaptic transmission or receptors. QSART responded promptly to treatment. Putative pathophysiological mechanisms are discussed.- - - - - - - - - - ranking = 156.14817166044keywords = sweat gland, sweat, gland (Clic here for more details about this article) |
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