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Autonomic Nervous System Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
The clinical approach to the patient with clinical features of dysautonomia aims to determine: Whether autonomic function is affected.Which organ systems are affected.The site of the lesion (central, preganglionic, or postganglionic).The cause of the lesion.
Unusual Inherited Pulmonary Diseases Which Provide Clues to Pulmonary Physiology and Function
Published in Stephen D. Litwin, Genetic Determinants of Pulmonary Disease, 2020
Thomas Κ. C. King, Robert A. Norum
The recurrence of this disease in siblings suggested a genetic etiology. Equal numbers of males and females develop familial dysautonomia. The parents of affected children show no sign of the disease; most patients do not survive to reproductive age and no data are available on the occurrence of dysautonomia in the children of people with this disease. These characteristics of the pedigree pattern are consistent with autosomal recessive inheritance (see Chapter 1) and that hypothesis has been further tested by the enumeration of affected and unaffected siblings in the sibships. From Mendel's laws we expect that one-fourth of the children of parents who are both carriers of the gene will be homozygous and thus show the dysautonomia phenotype. But the only way we can identify these carrier couples is via an affected child. Thus, all couples who by chance have had no affected child will be overlooked and their children, who are unaffected, will not be counted. This biased ascertainment of sibships must be compensated for when the predictions of Mendel's laws for an autosomal recessive phenotype are examined. When the appropriate compensation is made in the case of dysautonomia the ratio of affected to unaffected children closely fits Mendel's prediction [5].
Evaluation of Autonomic Failure
Published in David Robertson, Italo Biaggioni, Disorders of the Autonomic Nervous System, 2019
In the evaluation of patients with severe dysautonomia, clonidine is used to assess how much sympathetic noradrenergic function remains in a given subject (Robertson et al., 1983; Kooner et al., 1991). In patients with dysautonomia, a fall in blood pressure and heart rate indicates that there is residual autonomic control of heart rate and vasculature. This, however, may be very attenuated and may be associated with very low baseline blood pressures in the upright posture. On the other hand, an increase in blood pressure or even a failure of blood pressure to fall in response to clonidine in a patient being evaluated for dysautonomia confirms that the sympathetic denervation of the vasculature is essentially complete (Robertson et al., 1983). In patients with efferent parasympathetic lesions, the bradycardic effect of clonidine is attenuated or absent (Kooner et al., 1991).
Systemic manifestations of Ehlers-Danlos syndrome
Published in Baylor University Medical Center Proceedings, 2021
Bo Song, Peter Yeh, John Harrell
Autonomic dysfunction, or dysautonomia, often presents with palpitations, dizziness, and syncope.4 The prevalence in the literature varies from 31% to 94%.4,14–16 One explanation for this variability is the use of separate methods of autonomic testing, ranging from questionnaires to validated measurements.4,14–16 In addition, studies have used different criteria for dysautonomia; some measured subjective presyncopal symptoms while others used specialized testing and exam maneuvers.4,14–16 Even when utilizing formal diagnostic criteria, current literature is also limited by factors such as age, duration/type of challenge, or medication use during testing.17 Initial treatment is conservative, focusing on optimizing fluid and electrolyte intake.1 Physical therapy consisting of graded, low resistance programs and activity modifications to improve muscle and vascular tone is also recommended.1 As cardiac manifestations can present similarly, referral to a cardiologist is recommended to rule out conditions such as mitral valve prolapse.18
Dysautonomia in the pathogenesis of migraine
Published in Expert Review of Neurotherapeutics, 2018
Parisa Gazerani, Brian Edwin Cairns
Autonomic nervous system (ANS) dysfunction is common in migraine.Migraine-related alterations in ANS function have a complex pattern with features suggestive of an imbalance between the sympathetic and parasympathetic nervous systems.Only some of the underlying mechanisms of dysautonomia have been revealed; among which an imbalance of sympathetic co-transmitters, cortical spreading depression, and changes in hypothalamic connectivity are being studied.It remains to be determined whether ANS dysfunction is a cause or a consequence of migraine.Dysautonomia could be modulated by both pharmacological and non-pharmacological interventions to restore normal autonomic function.
Clinical decision making and application of an active rehabilitation program for a person with the neuromuscular symptoms of Allgrove syndrome: a case report
Published in Physiotherapy Theory and Practice, 2020
The first signs of this disorder tend to occur in early childhood and are often endocrine and gastroenterological (Gazarian et al. 1995; Grant et al. 1993). Neurologic features often occur in late childhood and early adulthood and have been reported in multiple case studies to include symptoms of pyramidal syndrome, cerebellar dysfunction, dysautonomia, peripheral neuropathy, neuro-ophthalmologic signs, and bulbar symptoms (Bentes et al. 2001; Dumic et al. 2012; Houlden et al. 2002; Kimber et al. 2003; Vallet et al. 2012). Additionally, dysautonomia is commonly reported in this population (Bentes et al. 2001; Gilio et al. 2007; Houlden et al. 2002; Ismail et al. 2006; Kimber et al. 2003) and is thought to be due to autonomic neuropathy (Houlden et al. 2002). Dumic et al. (2012) reported cerebellar signs such as intention tremor and dysmetria in 5 out of 8 patients, but all patients experienced motor and sensory impairments, increased deep tendon reflexes with absent ankle reflexes, along with continuously deteriorating neurological impairments in one long-term follow-up study of patients with Allgrove syndrome over 4–29 years. Progressive muscle weakness has also been reported including atrophy of both upper and lower extremities, with marked atrophy of the gastrocnemius-soleus complex and hypothenars, pes cavus, and multiple gait abnormalities (Dumic et al. 2012). Muscle biopsies of a patient with genetically confirmed Allgrove syndrome show angulated atrophic fibers (AAFs). A histologic examination of muscle biopsies showing grouped AAFs typically indicates muscle atrophy from denervation with subsequent reinnervation (McKeever et al. 2012).