Godtfredsen syndrome, also known as clival (clivus) syndrome, is a rare syndrome of abducens and hypoglossal nerve palsies that localises to a clival mass.
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Clinical presentation
The classic clinical presentation includes 1-3:
abducens nerve palsy: diplopia worse when horizontal gaze is directed towards the affected side
hypoglossal nerve palsy: tongue deviation towards the affected side
Additionally, patients may have other cranial neuropathies depending on the size of the underlying clival mass 1-3.
Pathology
The abducens and hypoglossal nerves run in close proximity along the clivus, thus pathology affecting the clivus, such as a clival mass, may cause the unique palsy of these cranial nerves that defines Godtfredsen syndrome 1-3. The most common clival masses implicated are 1:
nasopharyngeal carcinoma (growing into the clivus)
meningioma (growing into the clivus)
Radiographic features
Radiographic features vary considerably depending on the underlying cause.
Treatment and prognosis
Management options vary considerably depending on the underlying cause.
History and etymology
The syndrome was first described by Erik Godtfredsen (1913-1993), a Danish ophthalmologist, in a case series of patients with nasopharyngeal carcinoma 4,5.
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