Neurosarcoidosis With Panhypopituitarism: Two Cases and Literature Review

Author:

Niedzialkowska Ewelina1ORCID,Blazin Tatjana1,Shelden Daniel2,Buras Eric D3

Affiliation:

1. Department of Internal Medicine, Corewell Health William Beaumont University Hospital , Royal Oak, MI 48073 , USA

2. Division of Endocrinology, Diabetes and Metabolism, Department of Internal Medicine, Corewell Health William Beaumont University Hospital , Royal Oak, MI 48073 , USA

3. Division of Metabolism, Endocrinology and Diabetes (MEND), Department of Internal Medicine, University of Michigan , Ann Arbor, MI 48105 , USA

Abstract

Abstract Neurosarcoidosis (NS) with hypothalamic-pituitary (HP) involvement (HP-NS) is a rare clinical condition, conferring variable hormonal deficits that are typically irreversible. Here, we present 2 cases of NS with panhypopituitarism. The first patient presented with cauda equina syndrome and arginine vasopressin deficiency, while the second developed recurrent optic neuritis and vision loss in the setting of a sellar mass. In the first case, neurological symptoms resolved after therapy with high-dose glucocorticoids, infliximab, and methotrexate; while in the second, visual restoration followed resection of the granulomatous tissue and immunosuppressive therapy. In both cases, pituitary dysfunction persisted despite neurological improvement. We contextualized the presentations and outcomes through a literature review of HP-NS case reports and case series. This revealed high rates of extraneurologic sarcoidosis in HP-NS patients with panhypopituitarism, while underscoring the need for hormonal replacement—as endocrinopathies rarely respond to sarcoidosis-directed immunosuppression.

Publisher

The Endocrine Society

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