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Occipital condyle syndrome (OCS) consists of bone, then traverses the neck, ultimately curving
unilateral occipital region pain associated with an ip- back to innervate the tongue. In the largest published
silateral paresis of the 12th cranial nerve (hypoglossal series of 12th nerve paresis, tumors, predominantly
nerve) and is typically due to metastasis to the skull malignant, produced nearly half of the pareses.1
base. The hypoglossal nerve is one of the least-regarded Metastasis to the skull base is a frequent compli-
cranial nerves, receiving only passing mention in most cation of various systemic cancers.2 The diagnosis can
neurology textbooks. The hypoglossal nerve leaves the often prove difficult, particularly if the patient is not
skull via the hypoglossal foramen of the occipital known to have cancer. In a retrospective study of 43
patients treated for base-of-skull metastasis, Green-
From the Departments of Neurology (Drs. Capobianco, berg et al identified five clinical syndromes: the or-
Brazis, and Rubino) and Radiology (Dr. Dalton), Mayo Clinic, bital, parasellar, middle fossa, and jugular foramen
Jacksonville, Fla. syndromes, in addition to OCS.3 In that series, OCS
Address all correspondence to Dr. David J. Capobianco, Mayo was the most stereotyped and the most easily diag-
Clinic, 4500 San Pablo Road, Jacksonville, FL 32224. nosed of the skull-based syndromes. Nonetheless,
Accepted for publication October 28, 2001. metastatic disease to the occipital condyles has been
142
Headache 143
Pain Characteristics
Increase With
Unilateral Occipital Increase With
Patient Age, y, Sex Site, Right or Left Duration Intensity Palpation Head Rotation
reported only rarely.3-5 In this article, we report 11 ad- tion findings, site of primary carcinoma, radiologic
ditional cases of OCS that emphasize the unique clin- findings, and treatment were evaluated.
ical and radiologic features of this disease.
Table 3.—Metastatic History and Treatment of Patients With Occipital Condyle Syndrome
Illustrative Case Report.—A 48-year-old man with seen on unenhanced T1-weighted images (Figure 1)
a known history of pancreatic cancer presented with a involving the right occipital condyle. After contrast
1-week history of severe, continuous, right suboccipital administration, pathologic enhancement of the lesion
region pain. The pain radiated to the ipsilateral fronto- and the adjacent soft tissues was observed (Figure 2).
temporal region and was exacerbated by neck flexion Pain control was achieved with radiation therapy to
and rotation of the head to the left. One week af- the skull base.
ter the onset of the head pain, he developed slurred
speech. On examination, he held his neck stiffly. There COMMENTS
was marked tenderness to palpation over the right oc- As suggested by Greenberg et al, OCS is a rare
cipital region. The tongue deviated to the right when but stereotypic clinical syndrome.3 The case series de-
protruded. The neurologic examination was otherwise scribed here is the largest reported to date. All of our
normal. Magnetic resonance imaging of the skull base patients complained of severe, continuous, unilateral
demonstrated a region of abnormal signal intensity, occipital region pain, typically exacerbated by neck
Radionuclide
Patient Skull Film Tomogram Bone Scan CT MRI CSF
pain preceded neurologic symptoms by several days strongly suggested when hypoglossal paralysis occurs
to 10 weeks. All patients ultimately complained of together with severe unilateral occipital headache
both dysarthria and dysphagia. The dysarthria was worsened by contralateral neck rotation and by local
generally considered mild by both the patient and ex- palpation, even if the patient is not known to have
aminer. On examination, tenderness to palpation cancer.4 As noted by Loevner and Yousem, evalua-
over the occipital or suboccipital region was noted in tion of the craniovertebral junction with special at-
all patients. All 11 patients had a unilateral hypoglos- tention to the occipital condyles should be a routine
sal paresis ipsilateral to the occipital region pain. part of all brain and cervical spine radiologic exami-
For patients with suspected tumors involving the nations, particularly when patients have persistent
skull base, magnetic resonance imaging is the imag- occipital pain and a history of cancer.5
ing method of choice.5,6 The finding of replacement of
normal hyperintense fat by hypointense soft tissue on
enhanced sagittal and axial T1-weighted images rep- REFERENCES
resents the most consistent result.5 Although plain
1. Keane JR. Twelfth-nerve palsy. Analysis of 100
skull films directed at the area in question may dem- cases. Arch Neurol. 1996;53:561-566.
onstrate bony erosion, such films were helpful in only 2. Gordon GS, Roof BS, Halden A. Skeletal effects of
one case in our series. In several cases in which mag- cancers and their management. In: Holland JF, Frei
netic resonance imaging demonstrated a lesion of the E III, eds. Cancer Medicine. Philadelphia, Pa: Lea &
occipital condyle, neither computed tomography of Febiger; 1973:1075-1083.
the head nor radionuclide bone scanning showed any 3. Greenberg HS, Deck MD, Vikram B, Chu FC, Pos-
evidence of a skull-based lesion. Nonetheless, a high- ner JB. Metastasis to the base of the skull: clinical
quality computed tomography scan or a radionuclide findings in 43 patients. Neurology. 1981;31:530-537.
bone scan may complement magnetic resonance im- 4. Moris G, Roig C, Misiego M, Alvarez A, Berciano J,
aging.6,7 Pascual J. The distinctive headache of the occipital
condyle syndrome: a report of four cases. Headache.
The treatment of OCS depends on the nature of
1998;38:308-311.
the underlying lesion. In our series, 9 of 11 patients
5. Loevner LA, Yousem DM. Overlooked metastatic
had a known primary tumor. Breast cancer was the
lesions of the occipital condyle: a missed case trea-
most common malignancy in the women (2 of 3), and sure trove. Radiographics. 1997;17:1111-1121.
prostate cancer was the most common cancer in the 6. Thompson EO, Smoker WR. Hypoglossal nerve
men (4 of 8). In 2 patients, OCS was the initial mani- palsy: a segmental approach. Radiographics. 1994;14:
festation of a metastatic lesion. 939-958.
We concur with the conclusion reached by Moris 7. Posner JB. Neurologic Complications of Cancer.
et al that neoplastic erosion of the occipital condyle is Philadelphia, Pa: FA Davis Company; 1995:184.