TY - JOUR
T1 - Autoimmune autonomic ganglionopathy
T2 - A possible postganglionic neuropathy
AU - Manganelli, Fiore
AU - Dubbioso, Raffaele
AU - Nolano, Maria
AU - Iodice, Rosa
AU - Pisciotta, Chiara
AU - Provitera, Vincenzo
AU - Ruggiero, Lucia
AU - Serlenga, Luigi
AU - Barbieri, Fabrizio
AU - Santoro, Lucio
PY - 2011/4
Y1 - 2011/4
N2 - Objective: To evaluate postganglionic autonomic and somatic nerve fiber involvement in a patient with chronic autoimmune autonomic ganglionopathy. Design: Case report. Setting: Department of Neurological Sciences, University Federico II of Naples. Patient: A patient with a 16-year history of severe autonomic failure and a high nicotinic acetylcholine receptor antibody titer underwent an extensive laboratory evaluation. Main Outcome Measures: Evaluation of sympathetic and parasympathetic functions and sural nerve and skin biopsies. Results: Clinical and laboratory evaluations showed the involvement of cardiovascular, pupillary, sudomotor, gastrointestinal, and bladder functions. Sudomotor function study and skin biopsy findings revealed postganglionic autonomic damage. Moreover, sural nerve and skin biopsy specimens provided clear evidence of somatic nerve fiber involvement. Conclusions: We demonstrated postganglionic autonomic damage that could be related to a prolonged and severe impaired synaptic transmission and we report, for the first time to our knowledge, a somatic nerve fiber involvement in autoimmune autonomic ganglionopathy.
AB - Objective: To evaluate postganglionic autonomic and somatic nerve fiber involvement in a patient with chronic autoimmune autonomic ganglionopathy. Design: Case report. Setting: Department of Neurological Sciences, University Federico II of Naples. Patient: A patient with a 16-year history of severe autonomic failure and a high nicotinic acetylcholine receptor antibody titer underwent an extensive laboratory evaluation. Main Outcome Measures: Evaluation of sympathetic and parasympathetic functions and sural nerve and skin biopsies. Results: Clinical and laboratory evaluations showed the involvement of cardiovascular, pupillary, sudomotor, gastrointestinal, and bladder functions. Sudomotor function study and skin biopsy findings revealed postganglionic autonomic damage. Moreover, sural nerve and skin biopsy specimens provided clear evidence of somatic nerve fiber involvement. Conclusions: We demonstrated postganglionic autonomic damage that could be related to a prolonged and severe impaired synaptic transmission and we report, for the first time to our knowledge, a somatic nerve fiber involvement in autoimmune autonomic ganglionopathy.
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U2 - 10.1001/archneurol.2011.60
DO - 10.1001/archneurol.2011.60
M3 - Article
C2 - 21482929
AN - SCOPUS:79953847143
VL - 68
SP - 504
EP - 507
JO - Archives of Neurology
JF - Archives of Neurology
SN - 0003-9942
IS - 4
ER -