Background: Occipital condyle syndrome is characterized by severe, unilateral, occipital headache and ipsilateral twelfth-nerve palsy. It is associated with skull-base metastasis.
Cases: We identified two patients with sub-acute onset of severe, unilateral, occipital headache and ipsilateral tongue paralysis. The first patient was a 58-year-old woman with a history of limited stage small-cell lung cancer in clinical remission. The second patient was an otherwise healthy 36-year-old man. Neither patient had any other findings on general medical or neurological examination. One patient had only equivocal findings on initial magnetic resonance imaging (MRI), and the other patient's MRI was normal. Although initial work-up for metastatic disease was normal, the first patient developed severe bone pain over the next few months, and follow-up investigations demonstrated metastases to her spine, tibia, skull base and brain. The second patient improved initially, but was admitted to hospital three months later with constitutional symptoms and pancytopenia. Bone marrow and lymph node biopsies were consistent with Stage IVB Hodgkin's lymphoma.
Conclusion: Occipital condyle syndrome can be the first presentation of disseminated malignancy. Initial imaging of the brain and skull base may be normal, and recognition of this syndrome warrants thorough investigation and close follow-up.