Heck A W, Phillips L H
Department of Neurology, University of Virginia Health Sciences Center, Charlottesville.
Neurol Clin. 1989 Aug;7(3):641-54.
Neurologic involvement occurs in 5 per cent of patients with sarcoidosis and is a presenting symptom in about half of those affected. The neurologic symptoms are due to a granulomatous process that mirrors that seen in the systemic form. Its diagnosis is usually established by the demonstration of sarcoidosis in other systems and supported by clinical, radiographic, and laboratory evidence. Any and all portions of the nervous system can be involved in either an acute or a chronic fashion. Early acute presentations tend to have excellent outcomes and the overall prognosis for the disease is good. The cranial nerves, especially the facial nerve, are most frequently involved and have a good prognosis. CNS involvement, usually in the form of a meningoencephalitis involving the hypothalamic region, is usually chronic and associated with poor prognosis. Peripheral nerve and muscle involvement is frequently asymptomatic. Although neuropathy or myopathy may present acutely, the usual presentation is subacute or chronic. There have been no controlled studies of therapy in neurosarcoidosis. Early implementation of corticosteroids remains the mainstay of treatment. Evidence for improvement with treatment is anecdotally reported in many cases, but progression of the disease also occurs despite therapy.
结节病患者中5%会出现神经系统受累,且在约半数受累患者中是首发症状。神经症状是由一种肉芽肿性病变引起的,与系统性结节病所见的病变相似。其诊断通常通过在其他系统中证实结节病来确立,并得到临床、影像学和实验室证据的支持。神经系统的任何部位都可能以急性或慢性方式受累。早期急性表现往往预后良好,该病的总体预后也较好。颅神经,尤其是面神经,最常受累且预后良好。中枢神经系统受累通常表现为累及下丘脑区域的脑膜脑炎,通常为慢性且预后较差。周围神经和肌肉受累通常无症状。虽然神经病变或肌病可能急性发作,但通常表现为亚急性或慢性。目前尚无关于神经结节病治疗的对照研究。早期使用皮质类固醇仍然是主要治疗方法。许多病例中有轶事报道称治疗后病情有所改善,但尽管进行了治疗,疾病仍会进展。