Rare Case of Neurosarcoidosis in Patient with Lung Sarcoidosis Treated with Corticosteroids

Joana Fernandes, PhD avatar

by Joana Fernandes, PhD |

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case study of rare condition

Symptoms of  neurological impairment linked to lung sarcoidosis, an unusual condition known as neurosarcoidosis,  were treated in a woman using an immunosuppressive therapy based on corticosteroids, researchers reported in a case study.

The study, “Dementia, Gait Disturbance, And Urinary Incontinence In A Patient With Pulmonary Sarcoidosis,” published in the journal Respirology Case Reports, noted that the patient had developed hydrocephalus (an abnormal accumulation of cerebrospinal fluid within the brain). This condition is a rare manifestation of neurosarcoidosis (sarcoidosis that affects the central nervous system), and was linked to her lung sarcoidosis.

The 67-year-old Hispanic woman had hypothyroidism (low thyroid gland activity), elevated blood pressure, and lung sarcoidosis. The patient also complained of progressive loss of consciousness for the past month, resulting in prostration and loss of sphincter control.

She was admitted to the hospital with fever and altered speech, but showed no immediate signs of neurological damage. Laboratory analyses revealed she had leukocytosis (a high number of white blood cells) and mild anemia (a low red blood cell count), but other analyses were considered unremarkable.

Brain imaging analyses, however, revealed certain alterations in the patient’s brain. A CT scan showed she had hydrocephalus without structural lesions, and an electroencephalogram (EEG) showed diffuse encephalopathy due to metabolic abnormalities.

A chest X-ray revealed the patient had stage 2 lung sarcoidosis.

After a multidisciplinary evaluation, the patient received a ventriculoperitoneal (VP) shunt without complications, and started steroid therapy with prednisone (0.5 mg/kg), which improved her symptoms.

After two years of follow-up, the patient remained stable and had a successful recovery. Her current medication includes prednisone (5 mg/day) and azathioprine (100 mg/day).

“Neurological involvement in association with lung sarcoidosis should be suspected in patients with known history of sarcoidosis,” researchers wrote.

According to the authors, immunosuppressive therapy with corticosteroids are the first-line treatment for neurosarcoidosis and should be started immediately if this condition is diagnosed.

“This case shows an uncommon clinical presentation of neurosarcoidosis with rapid progressive loss of consciousness secondary to hydrocephalus and emphasizes the importance of a timely diagnosis to ensure a good prognosis for the patient,” the researchers concluded.

Sarcoidosis is characterized by the accumulation of immune system cells in a variety of organs, affecting their ability to work well. The clinical symptoms of sarcoidosis are usually non-specific and generalized, and include fever, fatigue, anorexia, weight loss, and malaise, although symptoms vary depending on the organs affected.