不完全型ヘールフォルト症候群に伴う神経根障害―自験2症例の報告と文献的考察

Radiculopathy in Patients with Heerfordt's Syndrome: Two Case Presentations and Review of the Literature

はじめに

　ヘールフォルト（Heerfordt）症候群はサルコイドーシスの一亜型として知られおり，ぶどう膜炎・耳下腺腫脹・顔面神経麻痺の3症状と微熱を伴うものを完全型，3症状のうち2症状と微熱を伴うものを不完全型と分類する¹⁾。全サルコイドーシス患者の約4.1～5.6％と比較的頻度の低い症候群である¹⁾。神経症状として顔面神経障害がこの疾患の特徴とされるが，他の神経根障害合併の報告も散見される。

　筆者らは，体幹の神経根障害を合併した不完全型ヘールフォルト症候群の2例を経験した。これまでヘールフォルト症候群に伴う神経根障害に注目した論文はないが，既報告を含め，サルコイドーシスに伴う神経根障害はヘールフォルト症候群に合併する頻度が高く，文献的考察を交えて考察する。

Abstract

　As a subtype of the clinical presentations associated with sarcoidosis, the combination of uveitis, parotid gland swelling, and facial nerve palsy is known as Heerfordt's syndrome. This syndrome is an extremely rare disorder that has been estimated to affect only 4.1-5.6% of patients with sarcoidosis. 　We present 2 cases of Heerfordt's syndrome associated with radiculopathy in the trunk. The 2 patients experienced unilateral or bilateral radiculopathy in the trunk and in the trigeminal nerve area associated with Heerfordt's syndrome. 　Radiculopathy is also a rare manifestation in patients with neurosarcoidosis. A literature review revealed that only 51 cases of radiculopathy associated with sarcoidosis have been documented. A diagnosis of Heerfordt's syndrome was observed in 7 out of these 51 cases. Together with our 2 cases, 9 out of 53 patients with radiculopathy associated with sarcoidosis have been diagnosed as having Heerfordt's syndrome (estimated frequency, 16.9%). In conclusion, radiculopathy is a common neurological manifestation in patients with Heerfordt's syndrome. On the basis of our experience, we suggest that physicians consider the possibility of Heerfordt's syndrome in cases of radiculopathy with unknown cause. (Received: February 6, 2013, Accepted: April 18, 2013)