Heerfordt's Syndrome Associated with Trigeminal Nerve Palsy and Reversed Halo Sign.

Heerfordt's syndrome is a rare subtype of sarcoidosis and features a combination of facial palsy, parotid swelling, and uveitis, associated with a low-grade fever. Cases with two of three symptoms are called "incomplete Heerfordt's syndrome." Heerfordt's syndrome involving other cranial nerve symptoms is relatively rare. We herein report a case of incomplete Heerfordt's syndrome presenting with trigeminal nerve palsy and a reversed halo sign, a rare manifestation of pulmonary sarcoidosis. The histological diagnosis following a biopsy of the parotid gland and endobronchial ultrasound-guided trans-bronchial needle aspiration of the mediastinal lymph nodes was sarcoidosis. The symptoms and lung lesions improved after corticosteroid therapy.

Parotid gland involvement in Heerfordt syndrome: a case report.

Sarcoidosis is a multisystemic granulomatous disease characterized by the presence of noncaseating granulomas, the exact etiology of which is yet to be determined. Most of patients show granulomas located in the lungs or in the related lymph nodes. However, lesions can affect any organ. Noncaseating granulomas are not a pathognomonic sign of sarcoidosis, being observed also in other diseases, therefore the diagnosis is often of exclusion. We report a case of sarcoidosis with parotid gland involvement in the context of a Heerfordt syndrome, discussing about its clinical presentation, pathogenesis, pathology and differential diagnosis with other granulomatous diseases.

Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir.

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by the JC virus; the mortality rate is high and it is usually refractory to treatment. In non-HIV patients, PML occurs as a late consequence of hematologic malignancies or during prolonged immunosuppression for transplantation or autoimmune disease. We describe a 34-year-old PML patient with incomplete Heerfordt syndrome, a rare type of sarcoidosis, who had not received any immunosuppressants, including steroids, at the onset and who was clinically and radiologically responsive to the antiviral drug cidofovir.

Diagnosis of Heerfordt's syndrome by state-of-the-art ultrasound in combination with parotid biopsy: a case report.

The parotid gland is rarely affected by sarcoidosis (6% of all cases). If there is initial parotitis, and other major symptoms include fever, uveitis, or facial paralysis, the condition is referred to as Heerfordt's syndrome. The case presented here describes the diagnostic work-up and differentiation of swelling of the parotid gland by employing state-of-the-art ultrasound techniques in a 33-year-old patient with Heerfordt's syndrome. Color-coded duplex ultrasound demonstrated hypervascularization. Tissue harmonic and photopic imaging ultrasound additionally depicted hypoechoic, septated structural lesions of the glandular parenchyma with an optimized contrast. Since such structural changes are not a specific sonographic criterion for sarcoidosis, ultrasound-guided biopsy was performed for further clarification of the etiology of parotitis and for excluding lymphoma. In conclusion, state-of-the-art ultrasound with the option of obtaining a biopsy at low risk is a useful procedure for diagnosing unclear pathology of the parotid gland with rapid histological confirmation.

Diagnosis of uveo-parotid fever by 67Ga-citrate imaging.

67Ga-citrate scan from skull to mid-abdomen showed increased uptake in both parotid glands, mediastinum, lungs, uvei and/or enlarged lacrimal glands. The differential diagnosis of the positive radiogallium scan is discussed with emphasis on the value of the extrathoracic findings. Tissue diagnosis confirmed the imaging diagnosis of sarcoidosis. The case illustrates the usefulness of double radionuclide study--67Ga and 99mTc-MAA--and of the tomographic scanner in defining the spread of the active granulomatous disease.