Occipital condyle syndrome

Occipital condyle syndrome describes the concurrence of unilateral occipital pain and ipsilateral hypoglossal nerve palsy, which localizes to pathology affecting an occipital condyle. While occipital condyle syndrome can have many potential causes, it nearly always represents a manifestation of advanced metastatic malignancy ^1,2.

Clinical presentation

Occipital condyle syndrome presents with unilateral ^1-5:

• occipital headache, often exacerbated by neck movement (especially flexion and contralateral rotation)

  • often the first symptom, preceding hypoglossal nerve palsy by a few days

  • sometimes the pain can radiate temporally

• hypoglossal nerve palsy (tongue weakness, ipsilateral tongue deviation at rest)

  • sometimes associated with dysphagia and dysarthria

Pathology

Occipital condyle syndrome is caused by a lesion to the skull base, specifically localizing to an occipital condyle ^1-5. The occipital condyle is in close proximity to the hypoglossal canal, which lies between it and the jugular tubercle ^1,2. Thus, pathology of the occipital condyle can cause mass-effect on this region, compressing the hypoglossal nerve at the hypoglossal canal, resulting in the typical clinical presentation ^1,2.

Many potential pathologies can cause occipital condyle syndrome, but the most common cause is bony metastatic disease (~90% of cases) ¹. A range of primary malignancies have been described in this context, with common primary malignancies being prostate cancer, lung cancer, and breast cancer ^1,2. Other causes reported include craniovertebral tuberculosis, granulomatosis with polyangiitis, and inflammatory pseudotumor ^1,4,5.

Radiographic features

Radiographic features vary depending on the underlying cause. A thorough work-up may include the following imaging modalities, with the intent to exclude a malignant etiology:

• CT brain and skull base

• MRI, contrast-enhanced with dedicated views of the brain, brainstem, and skull base

Treatment and prognosis

Management is highly variable and depends upon the underlying cause. In available case series of patients with a malignant etiology, radiotherapy is often utilized as a treatment strategy ¹.

History and etymology

Occipital condyle syndrome was first described by Harry S Greenberg, Jerome B Posner and colleagues in their seminal 1981 paper ³.

Differential diagnosis

• occipital condyle fracture: can also present with occipital headache and lower cranial nerve palsies (the neurological deficit is often more profound, e.g. encompassing a Collet-Sicard syndrome) ⁶, however, in the literature 'occipital condyle syndrome' is not used to describe these traumatic cases

See also

• numb chin syndrome: another neurological syndrome often heralding advanced malignancy