Hypophysitis secondary to ruptured Rathke's cyst mimicking neurosarcoidosis

Cassandra Janeczko1, John McHugh, Daniel Rawluk

  • 1Department of Neurology, Beaumont Hospital, P.O. Box 1297, Beaumont Road, Dublin 9, Ireland. cejaneczko@hotmail.com

Insights

Ruptured Rathke's cysts can cause hypophysitis, a rare condition. A pituitary biopsy confirmed lymphocytic hypophysitis secondary to a ruptured Rathke's cyst, emphasizing the need for tissue diagnosis.

Area of Science:

  • Endocrinology
  • Neurology
  • Pathology

Background:

  • Hypophysitis, inflammation of the pituitary gland, is uncommon.
  • Rathke's cysts are congenital remnants in the pituitary region.
  • Ruptured Rathke's cysts are a rare cause of hypophysitis.

Observation:

  • A 53-year-old female presented with headache, aseptic meningitis, and panhypopituitarism.
  • Initial MRI findings suggested neurosarcoidosis, supported by cardiac arrhythmia and vasculitis.
  • Empirical steroid treatment showed partial improvement.

Findings:

  • Pituitary biopsy revealed lymphocytic hypophysitis.
  • The hypophysitis was secondary to a ruptured Rathke's cyst.
  • This diagnosis differed from the initial suspicion of neurosarcoidosis.

Implications:

  • Highlights a rare cause of hypophysitis and panhypopituitarism.
  • Underscores the critical importance of tissue diagnosis for pituitary masses.
  • Informs management decisions, preventing unnecessary long-term immunosuppression.

Related Concept Videos

Cushing Syndrome II: Pathophysiology01:19

Cushing Syndrome II: Pathophysiology

Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial infections,...
Cushing Syndrome I: Introduction01:26

Cushing Syndrome I: Introduction

Cushing syndrome refers to the collection of clinical manifestations that arise when tissues are exposed to excessive amounts of cortisol or cortisol-like medications over an extended period. Cortisol, a glucocorticoid produced by the adrenal cortex, regulates metabolism, immune responses, and the body’s adaptation to stress. When its concentration remains chronically elevated, these physiological pathways become dysregulated, resulting in the characteristic features of the syndrome.Exogenous...
Cryptococcal Meningitis01:27

Cryptococcal Meningitis

Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...