Heerfordt syndrome

Heerfordt syndrome, also known as Heerfordt-Waldenström syndrome or uveoparotid fever, is a variant of sarcoidosis, comprising of:

• fever
• parotid enlargement
• facial palsy
• ocular involvement (anterior uveitis)

Epidemiology

The exact prevalence is unknown, as only isolated case reports exist. Neurologic involvement may occur in ~5% of patients with sarcoidosis, with facial nerve palsy dominating ^2-4. Interestingly, rarity sections hold a case with the subsequent development of progressive multifocal leukoencephalopathy PML ³.

Clinical presentation

It may present as complete or incomplete (two out of the last three) ³. This holds also true for the single symptoms facial nerve palsy and anterior uveitis ².

Radiographic features

Although no specific imaging characteristics exist, imaging is nonetheless important to show the systemic nature of the disease and/or possible biopsy sites.

Enlargement of the parotid gland(s) and enlargement of cervical lymphadenopathy seem to be typical ² and may be observed clinically and by various imaging modalities.

Treatment and prognosis

The constellation of symptoms along with blood samples and biopsy usually allows the diagnosis. Prognosis equals the underlying systemic disease, as such marked improvement of symptoms often occurs after a few days of immunomodulating therapy, e.g. steroids ^2-4. Possible associated diseases need independent treatment ³.

History and etymology

The first to describe the constellation of symptoms was the Danish ophthalmologist Christian Frederik Heerfordt (1871-1953) in 1909 ⁵. Association with sarcoidosis was observed by the Swedish internist Jan Gösta Waldenström (1906-1996) in 1937 ⁶.

Practical points

• misdiagnosing facial paralysis as simple Bell palsy prevents possible treatment of the underlying systemic disorder ^2-4