QSART in idiopathic pure sudomotor failure

Abstract

QSART (quantitative sudomotor axon reflex testing) was performed in a patient with idiopathic pure sudomotor failure. Generalized reduction in thermoregulatory sweating and complete absence of axon reflex sweating were observed, suggesting a deficit of sweat gland cholinergic synaptic transmission or receptors. QSART responded promptly to treatment. Putative pathophysiological mechanisms are discussed.

Generalized anhidrosis in a child following presumptive H1N1 influenza

“…Neurological causes may involve central control mechanisms (as in multiple system atrophy) or peripheral pathways, the latter either as an isolated abnormality of sympathetic nerve fibers or as part of a more generalized autonomic neuropathy that may be primary (autoimmune autonomic neuropathy) or secondary (amyloidosis, diabetic peripheral neuropathy) [2]. Various terms have been used to describe isolated sudomotor failure of unknown etiologies: acquired idiopathic generalized anhidrosis (AIGA) [3], idiopathic pure sudomotor failure (IPSF) [4], and chronic idiopathic anhidrosis (CIA) [5]. Generalized anhidrosis may result in heat stroke and even death in tropical climates [6].…”

Idiopathic pure sudomotor failure

“…1 We speculated that primary lesion sites in these patients were within cholinergic receptors of the sweat glands, and proposed the term idiopathic pure sudomotor failure (IPSF) to represent this distinct sub-group of AIGA without sudomotor neuropathy or sweat gland failure (table 1). 1 We speculated that primary lesion sites in these patients were within cholinergic receptors of the sweat glands, and proposed the term idiopathic pure sudomotor failure (IPSF) to represent this distinct sub-group of AIGA without sudomotor neuropathy or sweat gland failure (table 1).…”

Generalised anhidrosis: different lesion sites demonstrated by microneurography and skin biopsy

“…The lesion sites causing GA are heterogeneous and estimated to be at several levels: (a) hypothalamic thermoregulatory area; 14 (b) autonomic neuropathy involving sudomotor nerves; 3 (c) idiopathic pure sudomotor failure (IPSF) with neurotransmission failure from terminals to effector organs; 15 (d) sweat gland failure (SGF); 16 and (e) congenital ectodermal dysplasia (CED).…”

“…In addition to anhidrosis, ISPF presents characteristic onset symptoms such as skin dysaesthesia, itching, and red eruptions, and the pathogenesis involves a steroid responsive immunological process that inhibits the transduction signal of cholinergic nerves to sweat gland receptors, altering the muscarinic receptor of acetylcholine. 15 SGF is characterised by sweat gland immunological deposits and by a poor response to steroids. 16 Immunologically related anhidrosis characterising AIGA is different from anhidrosis caused by CED or Ross syndrome (RS), which involves a degenerative pathogenesis without immunological implication.…”