Manganelli Fiore, Dubbioso Raffaele, Nolano Maria, Iodice Rosa, Pisciotta Chiara, Provitera Vincenzo, Ruggiero Lucia, Serlenga Luigi, Barbieri Fabrizio, Santoro Lucio
Department of Neurological Sciences, University Federico II of Naples, Italy.
Arch Neurol. 2011 Apr;68(4):504-7. doi: 10.1001/archneurol.2011.60.
To evaluate postganglionic autonomic and somatic nerve fiber involvement in a patient with chronic autoimmune autonomic ganglionopathy.
Case report.
Department of Neurological Sciences, University Federico II of Naples.
A patient with a 16-year history of severe autonomic failure and a high nicotinic acetylcholine receptor antibody titer underwent an extensive laboratory evaluation.
Evaluation of sympathetic and parasympathetic functions and sural nerve and skin biopsies.
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.
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.
评估慢性自身免疫性自主神经节病患者节后自主神经和躯体神经纤维受累情况。
病例报告。
那不勒斯费德里科二世大学神经科学系。
一名有16年严重自主神经功能衰竭病史且烟碱型乙酰胆碱受体抗体滴度高的患者接受了全面的实验室评估。
评估交感和副交感神经功能以及腓肠神经和皮肤活检。
临床和实验室评估显示心血管、瞳孔、汗腺分泌、胃肠道和膀胱功能受累。汗腺分泌功能研究和皮肤活检结果显示节后自主神经损伤。此外，腓肠神经和皮肤活检标本提供了躯体神经纤维受累的明确证据。
我们证明了节后自主神经损伤可能与长期严重的突触传递受损有关，并且据我们所知，首次报告了自身免疫性自主神经节病中躯体神经纤维受累情况。
