We present the case of a 71-year-old man who presented to us with unilateral lower motor neuron hypoglossal palsy along with the characteristic occipital headache. He himself forwarded a paper on occipital condyle syndrome to the clinician who initially reviewed him. Later the patient underwent a series of investigations that confirmed the diagnosis of underlying prostatic carcinoma with widespread metastasis to bones including the base of the skull. Seven months after the diagnosis he is doing well, his headache is much better and the tongue deviation is stable. He was initially treated with tapering doses of dexamethasone and is currently receiving the depo gonadorelin analogue leuprorelin.
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Prostate cancer is the most commonly diagnosed cancer in British men.1 Bone is the most common site of spread from prostate cancer, with vertebral bodies, pelvic bones, femur and ribs frequently involved.2 The base of the skull is a less frequent site of metastasis but is nevertheless very important, both clinically and prognostically, as it can cause a number of cranial nerve dysfunction syndromes.3
Unfortunately, such cases have been poorly documented in the medical literature. We present a patient with occipital headache and hypoglossal palsy as the initial manifestation of prostate carcinoma.
A 71-year-old retired man presented to hospital with a history of a sudden-onset deviation of the tongue to the right. On further questioning he reported having had localised pain in the right occipital region 2 weeks before the development of the tongue deviation. He also noticed dysarthria and dry mouth without any history of limb weakness, dysphagia, nasal regurgitation or visual symptoms.
The headache was localised to the occipital region and radiated up to the top of the head, was moderately severe and was worst at night. Significant past medical history included hypertension, rate-controlled atrial fibrillation and resection of a villous adenoma of the rectum several years earlier.
On neurological examination, the patient had signs consistent with right-sided lower motor neuron hypoglossal palsy (fig 1).The remainder of the neurological examination, including a fundus examination, was unremarkable.
Initial investigations included a CT scan of the head and x ray of the cervical spine, both of which were reported normal. Routine laboratory parameters including full blood count, urea and electrolytes, and liver function tests were within normal limits. The patient was sent home with an request for an outpatient MRI of the head and referral to a neurologist. Two days later the patient wrote to one of us (RP) enclosing a copy of a report, entitled “occipital condyle syndrome”,4 printed from a web search and asked for a whole-body scan to be performed on him to exclude underlying metastatic disease. Further investigations carried out revealed an elevated PSA at 137 mcg/l, a chest x ray showing metastasis to the right 3rd and 5th ribs, and a bone scan revealing extensive skeletal metastasis throughout the pelvis, spine, ribs, proximal long bones and base of the skull (fig 2). A contrast MRI scan of the head and base of the skull showed abnormal signals in the right occipital condyle, with widening compatible with local metastasis (fig 3). A transperineal needle biopsy of the prostate confirmed the diagnosis of poorly differentiated adenocarcinoma of the prostate, with a combined Gleason score of 5+5.
The patient was initially treated with the oral antiandrogen bicalutamide and tapering doses of dexamethasone. Currently he is being treated with the depo injectable gonadorelin analogue leuprorelin every 3 months.
OUTCOME AND FOLLOW-UP
Seven months after the diagnosis, the patient feels much better and the headaches have resolved, although he continues to have tongue deviation and slight speech difficulty. He does experience hot flushes as a side effect of leuprorelin.
Occipital condyle syndrome (OCS) consists of unilateral pain in the occipital region along with an ipsilateral paresis of the 12th cranial nerve (hypoglossal nerve) and is typically due to skull base metastasis. The hypoglossal nerve arises from the motor nucleus located beneath the floor of the fourth ventricle, passing in front of the vertebral and posterior inferior cerebellar arteries. It exits the base of the skull through the hypoglossal canal in the occipital bone; it then traverses the neck and curves back, divides and innervates the tongue muscles.
Metastasis to the skull base is a frequent complication of various systemic cancers.5 In the largest published series of 12th nerve paresis, tumours, predominantly malignant, produced half of the palsies. Trauma, infection, stroke and Guillain-Barré syndrome account for the remainder of cases.6
In the largest reported series on OCS,4 of 11 patients reported, 9 had a known primary cancer, breast cancer being the most common malignancy in women (2/3) and prostate cancer, the most common in men (4/8).In two of these patients, OCS was the initial manifestation of metastatic cancer. There has been a single case report previously published on OCS as the initial manifestation of metastatic prostate cancer.9 For patients with suspected skull base metastasis, MRI is the imaging modality of choice.10,11 A high-quality CT or a radionuclide bone scan may complement MRI.11,12
Prostate cancer with metastasis to the skull base carries a poor prognosis; in two different reviews published, the median survival from the time of cranial nerve involvement was 3–4 months.7,8 Interestingly, patients who had a cranial nerve palsy as the initial manifestation of an underlying prostatic malignancy were reported to be alive at 18 months9 and 42 and 84 months8 after the diagnosis in two reviews on skull base metastasis in prostate cancer.
Cranial nerve palsy due to base-of-skull metastasis from prostatic carcinoma is an uncommon condition.
Metastatic prostate carcinoma should be considered in the differential diagnosis of any middle-aged or elderly man presenting with lower cranial nerve palsies, even if there is no past history of prostate cancer.
An isotope bone scan and contrast MRI with focus on the base of the skull is the investigation of choice in such situations.
Occipital condyle syndrome is a well recognised entity but is poorly known to most clinicians.
With the emergence of the internet, patients can now objectively contribute towards making their own diagnosis.
Competing interests: none.
Patient consent: Patient/guardian consent was obtained for publication.