Gil-Diaz Aida, Conde-Martel Alicia, Betancor-Leon Pedro
Department of Internal Medicine, Hospital Universitario de Gran Canaria Dr. Negrin, Las Palmas, Spain.
BMJ Case Rep. 2011 May 16;2011:bcr0320113970. doi: 10.1136/bcr.03.2011.3970.
The pure autonomic failure is a rare entity, with only a few cases reported in the literature. The authors describe a case with compensatory excessive sweating of the right hemithorax as an initial manifestation of a pure autonomic failure, and the authors review the clinical characteristics of this disease. A 69-year-old man presented excessive sweating of the right hemithorax. Physical examination revealed orthostatic hypotension. No other neurological features were present. The autonomic study showed a low heart rate response to the Valsalva maneuver and reduced supine plasma norepinephrine levels. A pure autonomic failure was diagnosed. Treatment did not improve patient's symptoms. Anhidrosis with asymmetrical compensatory hyperhidrosis can be the only symptom of a pure autonomic failure. The authors highlight an unusual form of presentation of a rare disease, difficult to diagnose if it is not taken into consideration.
纯自主神经功能衰竭是一种罕见病症，文献中仅报道过少数病例。作者描述了一例以右半胸代偿性多汗为纯自主神经功能衰竭初始表现的病例，并对该疾病的临床特征进行了综述。一名69岁男性出现右半胸多汗。体格检查发现体位性低血压。未发现其他神经学特征。自主神经检查显示对瓦尔萨尔瓦动作的心率反应降低，仰卧位血浆去甲肾上腺素水平降低。诊断为纯自主神经功能衰竭。治疗未能改善患者症状。无汗伴不对称性代偿性多汗可能是纯自主神经功能衰竭的唯一症状。作者强调了一种罕见疾病的不寻常表现形式，如果不加以考虑则难以诊断。
