11/148. life-threatening orthostatic hypotension in a case with bulbo-myelo-radiculo-neuropathy.A 59-year-old female developed acute autonomic failure accompanied by life-threatening orthostatic hypotension. Reduced plasma noradrenaline levels and enhanced pressure response to noradrenaline infusion were compatible with a diagnosis of acute pan-dysautonomia. However, nerve conduction tests clearly revealed motor and sensory nerve involvement and abnormal F-responses. A sural nerve biopsy and catecholamine fluorescence study of the rectal mucosa revealed relatively preserved postganglionic unmyelinated nerve fibers. Six weeks later, the patient developed another episode of bulbar palsy and right hemiparesis; the MRI showed lesions in the medulla oblongata and right cervical spinal cord. The prognosis of acute pan-dysautonomia is usually unsatisfactory, but the present patient showed good steroid-responsiveness probably because impaired preganglionic sympathetic myelinated fibers and medulla oblongata recovered quickly.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
12/148. Rapid improvement of autonomic and peripheral neuropathy after liver transplantation: a single case report.Peripheral and autonomic neuropathies are known but often unrecognized associations of cirrhosis from any cause. The pathogenesis of these effects are ill understood. liver transplantation has been shown to reverse autonomic manifestations, but little evidence exists for an effect on peripheral neuropathy. This case report documents improvement in peripheral and autonomic neuropathy in a 40-year-old man with hepatitis b virus--related cirrhosis. A return to normal neurophysiological function was seen within 9 months of successful liver transplantation, suggesting a metabolic, rather than a structural, cause of such changes in the peripheral nervous system.- - - - - - - - - - ranking = 1.5keywords = neuropathy (Clic here for more details about this article) |
13/148. 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 = 5.0536658614407E-6keywords = deep (Clic here for more details about this article) |
14/148. 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 = 2.75keywords = neuropathy (Clic here for more details about this article) |
15/148. Isolated autonomic cardiovascular neuropathy in a patient with primary Sjogren syndrome: a case of successful treatment with glucocorticoid.Autonomic failure is rare in patients with Sjogren syndrome (SS). We report the case of a 46-year-old woman with severe autonomic cardiovascular failure, manifested by incapacitating postural hypotension, as the first symptom of primary SS. Treatment with glucocorticoid resulted in a dramatic improvement of her symptoms and objective findings of autonomic cardiovascular dysfunction. We suggest that SS should be considered in patients with idiopathic autonomic cardiovascular neuropathy, especially in those with idiopathic orthostatic hypotension. Furthermore, glucocorticoid therapy seems to be very efficacious in the treatment of SS-associated autonomic cardiovascular neuropathy.- - - - - - - - - - ranking = 1.5keywords = neuropathy (Clic here for more details about this article) |
16/148. Observations on haematological and cardiovascular effects of erythropoietin treatment in multiple system atrophy with sympathetic failure.Symptomatic autonomic neuropathy and in particular sympathetic failure mediated postural hypotension often accompanies multiple system atrophy (MSA). Release of erythropietin is under sympathetic control and we and others have previously reported that severe autonomic failure may be associated with a normocytic normochromic anaemia and erythopietin (EPO) deficiency. In this paper we describe the haematological, cardiovascular and clinical observations on a patient with MSA and severe symptomatic autonomic neuropathy who had haematological and clinical improvement (substantial increase in standing blood pressure) following subcutaneous EPO treatment.- - - - - - - - - - ranking = 0.5keywords = neuropathy (Clic here for more details about this article) |
17/148. Pandysautonomia associated with impaired ganglionic neurotransmission and circulating antibody to the neuronal nicotinic receptor.We report the case of a patient with chronic autonomic failure who had evidence of decreased postganglionic traffic to intact sympathetic nerve terminals. The patient complained mainly of decreased salivation, constipation, dry skin, and orthostatic intolerance. There was no evidence of central neurodegeneration. Autonomic function testing showed orthostatic hypotension without tachycardia and abnormal blood pressure and pulse rate responses to the valsalva maneuver, indicating combined sympathetic and parasympathetic neurocirculatory failure. In contrast to patients with pure autonomic failure, the patient had normal left ventricular myocardial concentrations of 6-[(18)F]fluorodopamine-derived radioactivity, establishing intact postganglionic sympathetic innervation; and in contrast to patients with multiple system atrophy or baroreflex failure, the patient had a low plasma norepinephrine concentration and brisk norepinephrine response to orthostasis. These findings indicated an impediment to ganglionic neurotransmission. Serologic testing demonstrated a circulating antibody to the ganglionic nicotinic acetylcholine receptor. The findings in this case support the concept that circulating antibodies to this receptor can interfere with ganglionic neurotransmission and produce autoimmune autonomic neuropathy.- - - - - - - - - - ranking = 0.25keywords = neuropathy (Clic here for more details about this article) |
18/148. Clinical and morphofunctional features of idiopathic myenteric ganglionitis underlying severe intestinal motor dysfunction: a study of three cases.Ganglionitis, i.e., the inflammatory neuropathy characterized by a marked lymphoplasmacellular infiltrate in the myenteric plexus, may underlie a variety of paraneoplastic, infectious, or neurological disorders, although occasional cases are idiopathic in origin. We report clinical, manometric, morphofunctional, and immunological features of three cases of idiopathic ganglionitis. All patients had megacolon and underwent surgery for repeated episodes of intestinal subocclusion. Esophageal, GI, and colonic manometry performed in one patient showed dysmotility of the whole gut. Histological examination of colonic and ileum specimens identified a prominent lymphoplasmacellular infiltrate within the myenteric plexus along with a marked decrease of a wide array of neuronal peptides/transmitters. In one patient, tissue analysis revealed progressive neuronal changes up to marked myenteric neuron damage. The inflammatory infiltrate in all patients comprised CD4 and CD8 T lymphocytes. Abundance of both subclasses of lymphocytes suggests that immune-mediated mechanisms were responsible for neuronal degeneration. In one patient, systemic steroid therapy brought a significant clinical improvement. The immunosuppressive approach deserves further investigation in patients with severe gut motor abnormalities attributable to idiopathic myenteric ganglionitis.- - - - - - - - - - ranking = 0.25keywords = neuropathy (Clic here for more details about this article) |
19/148. 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 = 0.25keywords = neuropathy (Clic here for more details about this article) |
20/148. Severe autonomic dysfunction in an 11-year-old girl with generalised tetanus.tetanus can occur even after minor injuries, and proper immunisation status must be ascertained and documented in every surgical patient. Failure to do so can have dramatic consequences. An 11-year-old non-immunised girl was admitted to the intensive care unit with severe generalised tetanus 8 days following an open fracture of the right forearm. Although she was under deep sedation and received high doses of opiates while being paralysed and mechanically ventilated, she developed pronounced cardiovascular instability with tachycardia and periods of rapid changes between arterial hyper- and hypotension secondary to severe autonomic dysfunction. Her cardiovascular status only began to stabilise after 14 days under concomitant treatment with clonidine, magnesium sulphate and labetalol.- - - - - - - - - - ranking = 5.0536658614407E-6keywords = deep (Clic here for more details about this article) |
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