Journal of the neurological sciences · 2026
Neurosarcoidosis of the hypothalamus, infundibulum, and pituitary gland: A retrospective cohort study.
СтатьяНазвание и аннотация: Перевод в очереди
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Herman Max, Russell Katherine, Shi Hang, Greene James G, Hutto Spencer K
Аннотация
BACKGROUND: Hypothalamic-pituitary involvement is common in neurosarcoidosis, but dedicated studies describing its impact are limited.
OBJECTIVE: To determine the clinicoradiographic characteristics, treatment responsiveness, and long-term outcomes of hypothalamic-pituitary neurosarcoidosis.
METHODS: Patients with definite or probable neurosarcoidosis were included if they exhibited radiographic involvement of the hypothalamus, infundibulum, and/or pituitary and were divided into symptomatic and asymptomatic groups. When available, radiographic features were directly reviewed by the authors.
RESULTS: Thirty-one patients (31/247 total cohort, 12.6%) were included, 19 (61.3%) being symptomatic. Chronic presentations were typical (13/19, 68.4%), frequently with polydipsia, polyuria, memory impairment, and gonadal dysfunction. On MRI, lesions were centered in the infundibulum (28/29, 96.6%) and inferior hypothalamus (28/29, 96.6%), typically expansile (23/28, 82.1%) and homogeneously enhancing (25/28, 89.3%). Other inflammatory foci in the neuraxis (27/29, 93.1%; especially cranial nerves and meninges) and body (30/31, 96.8%) were routinely seen. Nearly all responded to corticosteroids acutely (17/18, 94.4%). Most (13/19, 68.4%) required two or more lines of maintenance treatments, with TNF inhibitor regimens most often successful (8/12, 66.7%). Symptomatic patients experienced worse functional outcomes (mRS 3 vs 1, p 0.027) and a higher relapse risk (12/19 vs 3/12, p 0.048) than asymptomatic patients. Despite improved or resolved imaging (18/19, 94.7%), almost all symptomatic patients (18/19, 94.7%) were on corticosteroids and/or hormonal treatments at last follow-up (median 55 months).
CONCLUSION: Symptomatic hypothalamic-pituitary neurosarcoidosis is a severe phenotype with high rates of disability, relapse, treatment refractoriness, and long-term hormonal and steroidal treatments despite improving radiographic abnormalities.
OBJECTIVE: To determine the clinicoradiographic characteristics, treatment responsiveness, and long-term outcomes of hypothalamic-pituitary neurosarcoidosis.
METHODS: Patients with definite or probable neurosarcoidosis were included if they exhibited radiographic involvement of the hypothalamus, infundibulum, and/or pituitary and were divided into symptomatic and asymptomatic groups. When available, radiographic features were directly reviewed by the authors.
RESULTS: Thirty-one patients (31/247 total cohort, 12.6%) were included, 19 (61.3%) being symptomatic. Chronic presentations were typical (13/19, 68.4%), frequently with polydipsia, polyuria, memory impairment, and gonadal dysfunction. On MRI, lesions were centered in the infundibulum (28/29, 96.6%) and inferior hypothalamus (28/29, 96.6%), typically expansile (23/28, 82.1%) and homogeneously enhancing (25/28, 89.3%). Other inflammatory foci in the neuraxis (27/29, 93.1%; especially cranial nerves and meninges) and body (30/31, 96.8%) were routinely seen. Nearly all responded to corticosteroids acutely (17/18, 94.4%). Most (13/19, 68.4%) required two or more lines of maintenance treatments, with TNF inhibitor regimens most often successful (8/12, 66.7%). Symptomatic patients experienced worse functional outcomes (mRS 3 vs 1, p 0.027) and a higher relapse risk (12/19 vs 3/12, p 0.048) than asymptomatic patients. Despite improved or resolved imaging (18/19, 94.7%), almost all symptomatic patients (18/19, 94.7%) were on corticosteroids and/or hormonal treatments at last follow-up (median 55 months).
CONCLUSION: Symptomatic hypothalamic-pituitary neurosarcoidosis is a severe phenotype with high rates of disability, relapse, treatment refractoriness, and long-term hormonal and steroidal treatments despite improving radiographic abnormalities.