Stovner L J, Sjaastad O
Department of Neurology, Trondheim University Hospital, Norway.
Eur Neurol. 1995;35(3):149-55. doi: 10.1159/000117111.
A marked, left-sided segmental hyperhidrosis, verified by quantitative evaporimetry, corresponded to an area of dissociated sensory loss in 2 siblings (male 49 years old, female 47 years old). MRI scans of the head and whole cord demonstrated a Chiari type I malformation in both. In the male, there was a syrinx of the neck. The level of this did not correspond to the hyperhidrosis and dissociated sensory loss areas of the trunk and thigh. The female, who had no syrinx, had hyperhidrosis and dissociated sensory loss of the head, neck, upper trunk, and arm, and anisocoria with the larger pupil ipsilateral to the sweating. This rare disorder may be due to a disinhibition of the preganglionic sympathetic neurons caused by cord lesion(s) interrupting inhibitory descending and spinal pathways which are important for the normal thermoregulatory sweating.
通过定量蒸发测定法证实，2名兄弟姐妹（男性，49岁；女性，47岁）存在明显的左侧节段性多汗症，对应于感觉分离性丧失区域。头部和整个脊髓的MRI扫描显示两人均有Chiari I型畸形。男性患者颈部有一个空洞。其位置与躯干和大腿的多汗症及感觉分离性丧失区域不对应。女性患者没有空洞，出现了头部、颈部、上躯干和手臂的多汗症及感觉分离性丧失，并且存在瞳孔不等大，出汗同侧的瞳孔较大。这种罕见的疾病可能是由于脊髓病变中断了对正常体温调节性出汗很重要的抑制性下行和脊髓通路，导致节前交感神经元去抑制所致。
