Postganglionic cholinergic dysautonomia with incomplete recovery: a clinical, neurophysiological and immunological case study.
A 26-year-old man presented with signs and symptoms of marked postganglionic cholinergic autonomic dysfunction manifested by non-reacting dilated pupils, paresis of accommodation, decreased salivation, dry skin, atony of the bladder, erectile impotence and complete gastrointestinal paresis. Standard neurophysiological tests for myelinated sensory and motor fibre function and quantitative methods to examine unmyelinated parasympathetic, sympathetic and afferent fibres were performed: parasympathetic function was measured by heart rate variation tests. Sympathetic cutaneous vasoconstrictor responses induced by deep inspiration were examined with laser Doppler flowmetry. Cutaneous nociceptive C-fibre function was assessed by measurement of axon reflex vasodilatation and flare size induced by histamine iontophoresis. The findings confirmed that the abnormalities were restricted exclusively to the cholinergic postganglionic autonomic systems. All other functions were completely preserved. Modern neurophysiological methods of testing sympathetic and afferent small fibre function might help in the diagnosis of cholinergic postganglionic dysautonomia in the early stages. The specificity of the dysfunction argues in favour of an immunological pathogenesis. However, antibody screening including acetylcholine receptor antibodies and voltage-gated calcium channel antibodies gave negative results. Whatever autoimmunological mechanism might be involved, the postulated antibodies act highly specifically on unknown structures of the cholinergic postganglionic autonomic neurons.
Baron
R
R
Klinik für Neurologie, Christian-Albrechts-Universität Kiel, Germany.
Engler
F
F
D000328:Adult; D000344:Afferent Pathways / Q000503:physiopathology; D001338:Autonomic Fibers, Postganglionic / Q000503:physiopathology*; D001342:Autonomic Nervous System Diseases / Q000503:physiopathology*; D006801:Humans; D007107:Immune System / Q000503:physiopathology*; D008297:Male; D009043:Motor Activity / Q000502:physiology; D009413:Nerve Fibers, Myelinated / Q000502:physiology; D017777:Parasympathetic Fibers, Postganglionic / Q000503:physiopathology*; D010275:Parasympathetic Nervous System / Q000503:physiopathology*; D012677:Sensation / Q000502:physiology; D013564:Sympathetic Nervous System / Q000503:physiopathology