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Brief Communication

Occipital Condyle Syndrome


David J. Capobianco, MD; Paul W. Brazis, MD; Frank A. Rubino, MD; Jon N. Dalton, MD

Objective.—Review the clinical features of occipital condyle syndrome.


Background.—Occipital condyle syndrome consists of unilateral occipital region pain associated with ipsilat-
eral 12th cranial nerve paresis. It is typically due to metastasis to the skull base and is underdiagnosed.
Design.—We report a retrospective case series of 11 patients (8 men, 3 women), aged 32 to 72 years.
Results.—Eleven cases of occipital condyle syndrome were identified. All patients complained of severe oc-
cipital region pain. In addition, 2 patients complained of ipsilateral ear or mastoid pain, 2 noted associated vertex
pain, and 2 had frontal region pain. Six of the 11 cases involved the right side. In all patients, the occipital pain was
ipsilateral to the 12th nerve paresis. All patients were mildly dysarthric, and 3 had dysphagia. In 7 of the 11 pa-
tients, occipital region pain preceded the hypoglossal paresis by several days to 10 weeks. On examination, tender-
ness to palpation of the occipital region was noted in all patients. All 11 patients had unilateral hypoglossal pare-
sis. Skull films were abnormal in 2 of 5 patients for whom they were obtained, and tomograms were abnormal in 1
of 2 patients. High-quality computed tomography, bone scanning, and magnetic resonance imaging were abnor-
mal in all cases in which they were performed. Nine patients had a known primary malignancy. The most common
malignancies were breast cancer in women (2 of 3) and prostate cancer in men (4 of 8). In 2 patients, occipital
condyle syndrome was the initial manifestation of a metastatic lesion. Radiation therapy was the treatment of
choice for the occipital region pain.
Conclusion.—Occipital condyle syndrome is a rare, but stereotypic syndrome. Early detection has important
therapeutic implications. Evaluation of the craniovertebral junction with special attention to the occipital
condyles should be a routine part of all brain and cervical spine radiologic examinations, and the possibility of oc-
cipital condyle syndrome, particularly when patients have persistent occipital pain and a history of cancer, should
be considered.
Key words: headache, hypoglossal nerve palsy, occipital condyle syndrome, skull base metastases
Abbreviations: OCS occipital condyle syndrome
(Headache. 2002;42:142-146)

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

Table 1.—Patient Demographics and Pain Characteristics of Occipital Condyle Syndrome*

Pain Characteristics

Increase With
Unilateral Occipital Increase With
Patient Age, y, Sex Site, Right or Left Duration Intensity Palpation Head Rotation

1 60, F Occipital, R Constant Severe Yes Yes


2 63, M Occipital, eye, forehead, R Constant Severe Yes Yes
3 64, M Occipital, vertex, R Constant NR Yes NR
4 63, M Occipital, ear, R Constant Severe Yes NR
5 32, M Occipital, R NR NR Yes NR
6 58, M Occipital, L Constant Severe Yes NR
7 63, M Occipital, vertex, L Constant Severe Yes NR
8 72, M Occipital, frontal, L Constant Severe Yes NR
9 52, F Occipital, L NR NR NR NR
10 48, M Occipital, frontotemporal, R Constant Severe Yes Yes
11 56, F Occipital, mastoid, L Constant Severe Yes Yes

*NR indicates not recorded

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.

DESIGN AND METHODS RESULTS


The medical records of all patients seen at Mayo Eleven cases of OCS were identified (8 men, 3
Clinic from 1982 through 1999 with a diagnosis of women; aged 32 to 72 years). The pertinent findings
OCS were studied retrospectively. Patient age, sex, in the 11 patients are summarized in Tables 1, 2, 3,
history of metastatic disease, symptoms and examina- and 4.

Table 2.—Clinical Features of Occipital Condyle Syndrome*

Difficulty Moving Complaint of Dysarthria Noted Duration of Occipital Unilateral 12th


Patient Tongue Dysarthria on Exam Dysphagia Pain, wk† Nerve Paresis

1 Yes Yes Yes Yes 8 Yes


2 Yes No Yes No NR Yes
3 Yes Yes Yes No 1 Yes
4 Yes No Yes No 10 Yes
5 Yes No Yes No 2 days Yes
6 Yes Yes Yes Yes NR Yes
7 Yes Yes Yes No Simultaneous Yes
8 Yes Yes Yes Yes 8 Yes
9 Yes Yes Yes No 7 Yes
10 Yes Yes Yes No 1 Yes
11 Yes Yes Yes No Simultaneous Yes

*NR indicates not recorded.


†Before onset of cranial nerve palsy.
144 February 2002

Table 3.—Metastatic History and Treatment of Patients With Occipital Condyle Syndrome

Cancer Previously Newly


Patient Known Diagnosed Type of Cancer Treatment

1 Yes No Adenocarcinoma (sigmoid) Radiation


2 Yes No Prostate Radiation
3 Yes No Prostate Radiation
4 No Yes Prostate Orchiectomy, diethylstilbestrol
5 No Yes Undifferentiated Radiation
6 Yes No Rectal Radiation
7 Yes No Prostate Radiation
8 Yes No Osteosarcoma Radiation
9 Yes No Breast Radiation, chemotherapy
10 Yes No Pancreatic Radiation
11 Yes No Breast Radiation

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

Table 4.—Radiographic Findings in Patients With Occipital Condyle Syndrome

Radionuclide
Patient Skull Film Tomogram Bone Scan CT MRI CSF

1 Normal Normal Positive Initial normal, Not done Not done


repeat abnormal
2 Not done Not done Positive Positive Not done Not done
3 Not done Not done Positive Positive Positive Not done
4 Normal Abnormal Negative Positive Not done Not done
5 Not done Not done Negative Negative Positive Negative
6 Normal Not done Negative Not done Positive Not done
7 Not done Not done Positive Positive Not done Negative
8 Not done Not done Not done Not done Positive Not done
9 Abnormal Not done Positive Negative Positive Not done
10 Not done Not done Positive Not done Positive Not done
11 Abnormal Not done Positive Not done Positive Not done
Headache 145

Fig 1.—Patient 10. Top, Sagittal unenhanced T1-weighted


magnetic resonance image (MRI) demonstrating the normal
appearance of the left occipital condyle (arrow). Bottom, Sag-
Fig 2.—Patient 10. Top, Axial T1-weighted magnetic reso-
ittal unenhanced T1-weighted MRI demonstrating replace-
nance image (MRI) demonstrating the right condyle lesion (*)
ment of the normal marrow by hypointense tissue (arrow).
with abnormal signal extending into the right hypoglossal ca-
nal (arrows). The left hypoglossal canal (arrowheads) is nor-
mal. Bottom, Axial T1-weighted postcontrast fat saturation
MRI demonstrating replacement of the normal marrow within
flexion and rotation of the head to the side contralat- the right occipital condyle (arrow).
eral to the pain. In several patients, the pain radiated
anteriorly toward the ipsilateral ear, temple, vertex,
or forehead. In 7 of our 11 patients, occipital region
146 February 2002

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
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