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Ovid: Fifty Neurologic Cases from Mayo Clinic

Editors: Noseworthy, John H. Title: Fifty Neurologic Cases from Mayo Clinic, 1st Edition Copyright ©2004 Oxford Unversity Press (Copyright 2004 by Mayo Foundation for Medical Education and Research) > Table of Contents > Case 3: Occipital pain with tongue deviation Case 3: Occipital pain with tongue deviation CASE 3 History A 48-year-old man with a known history of pancreatic cancer presented with a 1-week history of severe, continuous pain in the right suboccipital region. The pain radiated to the ipsilateral frontotemporal region and was exacerbated by neck flexion and rotation of the head to the left. One week after the onset of head pain, slurred speech developed. The patient said he had no other neurologic complaints. Examination On examination, he held his neck stiffly and had marked tenderness to palpation over the right occipital region. The tongue deviated to the right when protruded. Neurologic examination findings were otherwise normal. Investigations Unenhanced T1-weighted magnetic resonance images of the base of the skull demonstrated a region of abnormal signal intensity involving the right occipital condyle (Figure). After contrast administration, pathologic enhancement of the lesion and adjacent soft tissues was observed. Pain control was achieved with radiotherapy to the skull base. P.10

FIGURE. A, Top, Sagittal unenhanced T1-weighted magnetic resonance image (MRI) demonstrating the normal appearance of the left occipital condyle (arrow). Bottom, Sagittal unenhanced T1-weighted MRI demonstrating replacement of the normal marrow by hypointense tissue (arrow). B, Top, Axial T1-weighted MRI demonstrating the right condyle lesion (*), with abnormal signal extending into the right hypoglossal canal (arrows). The left hypoglossal canal (arrowheads) is normal. Bottom, Axial T1-weighted postcontrast fat saturation MRI demonstrating replacement of the normal marrow within the right occipital condyle (arrow). (From Capobianco DJ, Brazis PW, Rubino FA, et al. Headache 2002;42:142-6. By permission of the American Headache Society.)

DIAGNOSIS CASE 3 Occipital condyle syndrome due to metastatic cancer
Commentary by Dr. Paul W. Brazis This patient’s presentation is an example of the occipital condyle syndrome. This syndrome consists of unilateral pain in the occipital region associated with ipsilateral paresis of cranial nerve XII (the hypoglossal nerve) and is typically due to metastasis to the skull base. Patients with this syndrome complain of continuous, severe, localized, unilateral occipital pain made worse by neck flexion and often associated with neck stiffness. Rotating the head toward the side of the pain often relieves the discomfort, whereas rotating it to the nonpainful side or palpating the suboccipital area is unbearable. The pain occasionally radiates anteriorly toward the ipsilateral temporal area or eye. About half of the patients complain of dysarthria or dysphagia (or both), specifically related to difficulty moving the tongue. On examination, patients hold their neck stiffly and the occipital area on the involved side is often tender to palpation. The ipsilateral tongue is weak and atrophic and deviates toward the weak side. Inflammation or fracture of the occipital condyle may also cause a unilateral or bilateral palsy of cranial nerve XII associated with occipital pain. Skull metastases to the clivus may also cause a bilateral palsy of cranial nerve XII. We have reported on 11 patients with the occipital condyle syndrome, and all complained of severe pain in the occipital region. Two patients complained of ipsilateral ear or mastoid pain, two noted associated vertex pain, and two had pain in the frontal region. In all patients, the occipital pain was ipsilateral to the hypoglossal nerve paresis. All patients were mildly dysarthric, and three had dysphagia. In seven patients, the pain in the occipital region preceded hypoglossal nerve paresis by several days to 10 weeks. On examination, tenderness to palpation of the occipital region was noted in all patients. All patients had unilateral hypoglossal nerve paresis. Skull films were abnormal in two of the five patients for whom they were obtained, and tomograms were abnormal in one of two patients. Computed tomography, bone scanning, and magnetic resonance imaging were abnormal in all patients in whom they were performed. Nine patients had a known primary malignancy. The most common malignancies were breast cancer in women (two of three women) and prostate cancer in men (four of eight men). In two patients, occipital condyle syndrome P.12
was the initial manifestation of a metastatic lesion. Radiotherapy was the treatment of choice for the occipital region pain. Occipital condyle syndrome is a rare but stereotypic syndrome. Early detection has important therapeutic implications. Evaluation of the craniovertebral junction with attention to the occipital condyles should be a routine part of all brain and cervical spine radiologic examinations, and the possibility of occipital condyle syndrome should be considered, particularly when patients have persistent occipital pain and a history of cancer. Cranial nerve XII has a close spatial relation with cranial nerves IX, X, and XI in the posterior cranial fossa and as it leaves the skull in the hypoglossal canal. Although a basilar skull lesion (e.g., tumor or trauma) may involve cranial nerve XII alone, it frequently involves the other lower cranial nerves. Damage to all four of these cranial nerves results in Collet-Sicard syndrome, which consists of paralysis of the ipsilateral trapezius and sternocleidomastoid muscles, paralysis of the ipsilateral vocal cord and pharynx, hemiparalysis of the tongue, loss of taste on the posterior third of the ipsilateral tongue, and hemianesthesia of the palate, pharynx, and larynx. Other palsy syndromes involving multiple lower cranial nerves may occur with lesions in the posterior cranial fossa, skull, retropharyngeal or retrostyloid space, or neck. Another rare syndrome of clinical concern is the combination of cranial nerve VI (abducens nerve) and cranial nerve XII palsies. This ominous combination may be seen with nasopharyngeal carcinoma (Godtfredsen’s syndrome) and clival lesions, especially tumors, three-fourths of which are malignant. Although the combination of a cranial nerve VI palsy with a cranial nerve XII palsy usually localizes the pathologic process to the clivus, lower brainstem lesions and subarachnoid processes (e.g., meningitis) may also cause this unusual combination of cranial nerve palsies. REFERENCE Capobianco DJ, Brazis PW, Rubino FA, et al. Occipital condyle syndrome. Headache 2002;42:142-6.

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