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Botulinum toxin in the management of focal hyperhidrosis
Published in Anthony V. Benedetto, Botulinum Toxins in Clinical Aesthetic Practice, 2017
David M. Pariser, DeeAnna Glaser
Ross syndrome was first described by the neurologist Alexander Ross in 1958.109 It is characterized by the triad of unilateral tonic pupils, generalized areflexia (Holmes–Adie syndrome), and progressive segmental anhidrosis with a compensatory band of excessive perspiration. Patients suffering from Ross syndrome usually do not perceive the HH; instead, it is the compensatory segmental HH that is bothersome. In addition, many patients suffer from several symptoms of vegetative dysfunction, such as palpitation, stenocardia, orthostatic hypotonia, and irritable colon.110 The pathogenesis of Ross syndrome is unknown. Multiple neuropathies of the autonomic nervous system or a failure in the synthesis or release of neurotransmitters have been suggested as possible causes.109 There is no histologic evidence of nerve fiber destruction. Therefore, Ross postulated a defect in acetylcholine cholinesterase activity, rather than the degeneration of sweat glands. The progression of Ross syndrome is very slow. There is no therapy for the segmental progressive anhidrosis. The bothersome compensatory HH can be improved, however, with systemic antimuscarinic drugs or with injections of BoNTA into the affected areas, usually the face. In 1992, Itin et al.111 presented a case study of a patient suffering from Ross syndrome with a defined area of anhidrosis in the right hand, the right axilla, and the right side of the face. In follow-up, after 11 years, the patient presented with additional anhidrotic areas in the right hemithorax and the underside of the left arm (Figure 9.12). Unfortunately, the patient refused treatment with BoNT, even though the HH was so severe that electrolyte replacement was necessary (unpublished data).
Ross Syndrome
Published in Neuro-Ophthalmology, 2020
Manikanta Damagatla, Pratyusha Ganne, Rakesh Upparakadiyala, Prabhakaran N
The first case of Ross syndrome was described by Ross in 1958.2 Ever since there have been only a handful of cases reported worldwide. Some believe this to be a combination of two separate syndromes namely Harlequin syndrome and Holmes-Adie syndrome.3 Recent evidence suggests a decrease in sudomotor, vasomotor and pilomotor innervation in the skin of patients with Ross syndrome.4–6 Hence, there is a lack of sweating and cutaneous vasoregulation leading to heat intolerance. The iodine-starch test helps to demarcate areas of intact sweating and anhidrotic areas. Areas of intact sweating stain positive.7 Our patient showed no staining on the left side of her back and had patchy staining on the right side of her back (Figure 2). Tonic pupil results from damage to the ciliary ganglion or postganglionic parasympathetic nerve fibres.8 The exact cause of areflexia has not been found. Some postulate damage to the dorsal roots, sensory ganglia or the afferent fibres in the spinal cord.9,10 Tonic pupil may be present unilaterally or bilaterally and areflexia may be generalised. The tonic pupil shows a hypersensitivity response to dilute pilocarpine drops and this may be performed to confirm the diagnosis of postganglionic parasympathetic denervation hypersensitivity.11 The exact pathogenesis of the autonomic ganglion damage is uncertain but it is postulated that there is a congenital lack of factors that promote the survival of these neurons leading to their apoptosis. Some studies have suggested an autoimmune basis for the disease with some patients testing positive for different antibodies like anti-nuclear (ANA), SSA, SSB and anti-thyroid antibodies.12,13 Nolano et al. showed that this condition is progressive but benign.6