Neurosarcoidosis causing ventricular loculation, hydrocephalus, and death

Area of Science:

• Neurology
• Immunology
• Pathology

Background:

• Sarcoidosis is a multisystem inflammatory disease characterized by non-caseating granulomas.
• Neurosarcoidosis affects the central and peripheral nervous systems, with varied clinical presentations.
• Intracranial mass lesions are a rare but significant manifestation of neurosarcoidosis.

Observation:

• A 33-year-old woman with a history of sarcoidosis developed a severe headache due to a cystic temporal lobe lesion.
• The lesion was identified as a loculated portion of the lateral ventricle, and its drainage provided temporary symptom relief.
• Recurrence of symptoms, including headache and gait disturbance, led to the diagnosis of hydrocephalus requiring a ventriculoperitoneal shunt.

Findings:

• Despite initial improvement after shunt insertion, the patient later experienced seizures, personality changes, incontinence, and gait disturbance.
• Autopsy revealed widespread granulomatous disease, confirming extensive neurosarcoidosis.
• The case highlights the complex neurological sequelae of neurosarcoidosis, including intracranial mass lesions and hydrocephalus.

Implications:

• Surgical management of intracranial mass lesions and hydrocephalus in neurosarcoidosis can provide temporary neurological improvement.
• The progressive nature of neurosarcoidosis underscores the need for comprehensive management strategies beyond surgical intervention.
• This case emphasizes the importance of considering neurosarcoidosis in the differential diagnosis of intracranial mass lesions, particularly in patients with a history of sarcoidosis.