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Hereditary and Metabolic Diseases of the Central Nervous System in Adults
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Skin findings: Typically are small, red, flat or slightly raised telangiectasias called angiokeratoma.Usually around the umbilicus and thighs (bathing-trunk distribution).Abnormal sweating, usually anhidrosis or hypohydrosis but sometimes hyperhidrosis.
The Integumentary (Dermatologic) System and Its Disorders
Published in Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss, Understanding Medical Terms, 2020
Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss
Psoriasis is a chronic skin disease characterized by recurring exacerbations and remissions of thick, scaly lesions. The cause is unknown. Desquamation(squama meaning scale) is the term for scaling of the skin or cuticle. Another term for shedding of the skin is keratolysis(kerat = horny, plus lysis = loosening). Xerosis, a condition of "dry skin," is derived from the root xero-meaning dry and the suffix-ss&f denoting state of or disease. Hidrosis, the formation and excretion of perspiration (hidros ~ sweat), more commonly refers to excessive sweating. Conversely, anhidrosis is an abnormal deficiency of perspiration.
Evaluation of Autonomic Failure
Published in David Robertson, Italo Biaggioni, Disorders of the Autonomic Nervous System, 2019
C) Sweating abnormalities are common in autonomic disorders. Large areas of anhidrosis are found in patients with autonomic neuropathy due to central or peripheral causes (List and Peet, 1938). The pattern of anhidrosis may be of value in localizing lesions causing Horner’s syndrome (Morris, Lee and Lim, 1984), and peripheral nerve abnormalities (Guttmann, 1940). Documentation of reduced sweating confirms that the neurological lesion is not confined to noradrenergic neurons.
Correlation of neurological level and sweating level of injury in persons with spinal cord injury
Published in The Journal of Spinal Cord Medicine, 2021
Michelle Trbovich, Ashley Ford, Yubo Wu, Wouter Koek, Jill Wecht, Dean Kellogg
In this study examining SR over a large body surface area in the largest cohort of motor complete persons with SCI to date, the ten persons with motor complete TP demonstrated complete anhidrosis (including forehead) after a rise in core body temperature of 1°C. Our findings confirm that of Normell with the added finding of anhidrosis in the face and forehead of persons with TP.12 These objective findings confirm the accuracy of the subjective TP ISAFSCI data where all persons with TP reported “hypohidrosis below the lesion.” In conclusion, complete anhidrosis seen in response to heat stress in persons with TP (1) challenges the statement that persons with SCI are only anhidrotic in insensate areas and (2) suggests the option of “hypohidrosis above the LOI” should be added to the “autonomic control of sweating” section of the ISAFSCI to capture the pathophysiology seen in persons with TP and (3) informs clinicians of the high risk of HRI in persons with TP so appropriate education and prevention can be implemented.
Autoimmune autonomic ganglionopathy: an update on diagnosis and treatment
Published in Expert Review of Neurotherapeutics, 2018
Shunya Nakane, Akihiro Mukaino, Osamu Higuchi, Mari Watari, Yasuhiro Maeda, Makoto Yamakawa, Keiichi Nakahara, Koutaro Takamatsu, Hidenori Matsuo, Yukio Ando
Chronic anhidrosis, especially acquired idiopathic generalized anhidrosis (AIGA), is characterized by an acquired impairment in total body sweating despite exposure to heat or exercise. Most cases of AIGA have been reported in Asia, particularly in Japan [63]. Asahina and his colleagues attempted to find the Abs against muscarinic M3 receptors (M3Rs) on ecrine glands, but anti-M3R Abs were detected in 1/12 patients (8%) with AIGA [64]. Chronic anhidrosis may be heterogeneous. We experienced several patients with seropositive AAG associated with severe anhidrosis (Figure 5) [65,66]. Severe anhidrosis came to the front in the clinical manifestations of these cases. We should be alert to severe anhidrosis in the cases of AAG because AAG itself is rare. We should pay attention to autonomic function except for the sudomotor activity.
A Child Presenting with Recurrent Corneal Ulcers: Hereditary Sensory and Autonomic Neuropathy IV (HSAN IV)
Published in Neuro-Ophthalmology, 2019
Beena Suresh, Vaishnavi Reddy, Ingo Kurth, Sujatha Jagadeesh
Absence of pain perception leads to self-mutilating behaviour especially involving the finger tips, tongue and corneal ulcers. Sural nerve biopsies from HSAN4 patients reveal that unmyelinated fibres are absent. These fibres characteristically transmit pain. Skin biopsy from these patients reveals deficient C and Aδ fibres in the epidermis and hypoplastic dermal sweat glands without innervation.9 This accounts for the severe anhidrosis. Hypotonia and delayed development have been reported in literature but our patient had normal tone and appropriate development for age. The patient reported by Prasanth et al. also had normal development. 10