11/89. Familial association of autonomic and gastrointestinal symptoms.Autonomic dysfunction occurs in the adult population with irritable bowel syndrome, but this association is not recognized in children. A mother and son with functional abdominal pain unresponsive to conventional treatment had complete resolution of symptoms with treatment directed at the autonomic dysfunction identified by testing. The authors recommend autonomic testing in patients with functional abdominal pain and suggest that autonomic dysfunction plays a direct and intrinsic role in the mechanism of these disorders and their symptoms.- - - - - - - - - - ranking = 1keywords = pain, abdominal pain (Clic here for more details about this article) |
12/89. cluster headache sine headache: two new cases in one family.We report two cases of cluster headache sine headache occurring in a family of cluster headache sufferers. A 54-year-old female, suffered for 15 years from short-lasting, right-sided attacks of eye reddening, lacrimation, rhinorrea, palpebral oedema and miosis, seven to eight times per day, in periods of 20-30 days. She did not experience headache, either with or without these phenomena. Her son suffers from periorbital, short-lasting, right-sided painful attacks accompanied by eye reddening, lacrimation, rhinorrea and nasal stiffnesss, occurring in periods of 30-35 days. Soon after the end of the current cluster period he still had daily attacks lasting 10-15 min, with the oculo-nasal autonomic phenomena only. The occurrence of cluster headache sine headache and cluster headache in the same family strongly suggests that the first condition is related to the cluster headache syndrome. cluster headache sine headache should be considered as a subtype of the cluster headache syndrome.- - - - - - - - - - ranking = 73.173377627394keywords = headache, pain (Clic here for more details about this article) |
13/89. A case of chorea-acanthocytosis with dysautonomia; quantitative autonomic deficits using CASS.The purpose of this study was to quantitatively assess cardiovagal, adrenergic and postganglionic sudomotor functions in a patient with chorea-acanthocytosis. We describe a 25-year-old woman with acanthocytosis, lip and tongue biting, chorea like movements and increased levels of serum creatine phosphokinase. The patient presented with orthostatic hypotension. The heart period response to deep breathing was moderately decreased and the quantitative sudomotor axon reflex test (QSART) was mildly abnormal in the lower extremities. The composite autonomic score (CASS) for this patient was 6 points which indicates moderate autonomic failure. Although a few previous reports have described autonomic dysfunction associated with chorea-acanthocytosis and have indicated that impairment is primarily sympathetic, our case study suggests that there is also parasympathetic dysfunction. We conclude that there is widespread autonomic involvement in choreaacanthocytosis and that autonomic studies are useful in monitoring their course.- - - - - - - - - - ranking = 3.2989385570418keywords = breathing (Clic here for more details about this article) |
14/89. The importance of renal localization with MIBG scintigraphy.Although I-123 metaiodobenzylguanidine (MIBG) imaging is a well-validated technique for identification and evaluation of a pheochromocytoma, accurate interpretation can be confounded by tracer retention within the renal pelvis. The authors report two cases of abnormal MIBG accumulation in which renal localization was essential. In the first case, renal localization revealed uptake inferomedial to the left kidney in what was proved to be an extra-adrenal paraganglioma. In the second case, MIBG uptake in a similar location on the right was caused by uptake in the renal pelvis, a physiologic finding. When the location of MIBG uptake in the upper abdomen is uncertain, a renal scan should be obtained to exclude the possibility of radiolabeled MIBG tracer retention within the collecting system, a potential false-positive diagnosis of pheochromocytoma.- - - - - - - - - - ranking = 0.029537876250899keywords = upper (Clic here for more details about this article) |
15/89. Resolution of diabetic autonomic neuropathy.Many consider diabetic autonomic neuropathy to be an irreversible complication of diabetes of long duration. Three patients developed symptoms of autonomic neuropathy which subsequently resolved. Their autonomic neuropathy was not associated with long duration of diabetes, but with weight loss. Each had marked weight loss and resolution occurred on regaining remembered premorbid weight. A woman aged 20 was admitted with anorexia nervosa (weight loss 6 kg). She complained of feeling bloated. Gastroenterological investigations showed delayed gastric emptying. RR ratio (respiration and standing) was abnormal. Resolution occurred after two years. A male aged 18 developed diabetic symptoms, which were overlooked. Twelve months later he presented underweight and ketonuric; insulin treatment was started but within one month he became impotent. Resolution occurred after 18 months. An 80 year old man presented after six months trial of diet and sulphonylurea therapy. He was underweight, had ketonuria, and such muscle loss that he was unable to sit unaided. insulin treatment was started. He developed severe symptomatic postural hypotension. This resolved six months later by which time he had regained his normal weight. These cases illustrated symptomatic autonomic neuropathy occurring in relation to weight loss with resolution on recovery of normal weight, a temporal pattern mimicking that of acute cachectic painful neuropathy. Treatment of autonomic neuropathy should be like that of cachectic neuropathy, that is with an expectation of recovery and should include strategies to regain premorbid weight and achieve glycaemic control.- - - - - - - - - - ranking = 0.27940786892508keywords = pain (Clic here for more details about this article) |
16/89. Extensive Riga-Fede disease of the lip and tongue.Riga-Fede disease presents in early infancy and is characterized by firm, verrucous plaques arising on the oral mucosal surfaces. These histologically benign lesions occur as a result of repetitive trauma of the oral mucosal surfaces by the teeth. Early recognition of this entity is important, because it may be the presenting sign of an underlying neurologic disorder. We report the case of a 10-month-old boy with extensive Riga-Fede disease involving the lip and tongue that prompted a diagnosis of congenital autonomic dysfunction with universal pain loss.- - - - - - - - - - ranking = 0.27940786892508keywords = pain (Clic here for more details about this article) |
17/89. Upper sympathetic trunk lesion after video-assisted fracture stabilization of the thoracic spine: a case report.This case report draws attention to the upper sympathetic trunk lesion as a complication of video-assisted thoracic spine surgery. A 39-year-old man developed an upper sympathetic trunk lesion after right-sided thoracoscopic fracture stabilization of T5 and T6. dizziness and reduced perspiration persisted at the most recent follow-up 8 months after surgery. This rare complication can be overlooked and remain undiagnosed. diagnosis is based on clinical symptoms and neurologic examination. There are no treatment options. Symptoms can be bothersome for the patient and may persist. In the upper thoracic spine, the course of the sympathetic trunk lies in close proximity of the vertebral bodies; thus, care must be taken to avoid it when resecting the posterior parts of the vertebral body.- - - - - - - - - - ranking = 0.088613628752696keywords = upper (Clic here for more details about this article) |
18/89. Decoupling of autonomic and cognitive emotional reactions after cerebellar stroke.Emotional blunting can be found after cerebellar lesions. However, the mechanism of such a modification is not clear. We present a patient with emotional flattening and increased risk taking after left cerebellar infarct who had an impaired autonomic reactivity to negative as compared with positive reinforcement. This impairment was demonstrated by the patient's undifferentiated skin conductance responses to negative and positive reinforcement, whereas controls produced larger skin conductance responses after negative feedback. The cooccurrence of emotional flattening and undifferentiated autonomic reactions to positively and negatively valenced stimuli strengthens the role of the cerebellum in the modulation of the autonomic responses.- - - - - - - - - - ranking = 0.020463311952512keywords = back (Clic here for more details about this article) |
19/89. The spectrum of autoimmune autonomic neuropathies.We analyzed the clinical characteristics of 18 patients (13 female, 5 male) who had autoimmune autonomic neuropathy (AAN) and ganglionic acetylcholine receptor (AChR) autoantibodies. Mean age was 61.4 years (standard deviation, 12.0 years). Ten patients had subacute symptom onset, six with an antecedent event. Eight patients had chronic AAN, characterized by insidious symptom onset, without antecedent event, and gradual progression. A majority of patients with high antibody values (>1.00 nmol/L) had a combination of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light response, upper gastrointestinal symptoms, and neurogenic bladder. Chronic AAN segregated into two subgroups. One subgroup (N = 4) had low antibody titer (0.09 /- 0.01 nmol/L) and a paucity of cholinergic symptoms. It was indistinguishable from pure autonomic failure. The other subgroup (N = 4) had high antibody titer (11.6 /- 2.08 nmol/L), sicca complex, abnormal pupils, and neurogenic bladder; three had severe upper gastrointestinal dysfunction. Higher antibody titers correlated with greater autonomic dysfunction and more frequent cholinergic dysautonomia. These observations expand the clinical spectrum of AAN to include chronic cases, some being indistinguishable from pure autonomic failure, and support the concept that ganglionic AChR antibodies are important diagnostically and pathophysiologically in acquired dysautonomia.- - - - - - - - - - ranking = 0.059075752501797keywords = upper (Clic here for more details about this article) |
20/89. Orthostatic headaches without CSF leak in postural tachycardia syndrome.Four women age 17 to 28 years presented with orthostatic headaches as the most prominent feature of their symptom complex. None had CSF leak or intracranial hypotension. Autonomic studies showed evidence of orthostatic intolerance with tachycardia in all cases. Treatment of orthostatic intolerance, mainly with volume expansion, was only partially effective. Orthostatic headaches are not always caused by CSF leak or supine intracranial hypotension. Occasionally they may be the major clinical manifestation of postural tachycardia syndrome or orthostatic intolerance.- - - - - - - - - - ranking = 23.01914834478keywords = headache (Clic here for more details about this article) |
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