Selective Dysarthria Due to Clival Chordoma

PRACTICAL IMPLICATIONS

Selective dysarthria is unusual and requires evaluation of other etiologies in addition to vascular ones (mainly pathology of the oral cavity or brainstem).

Chordoma is a rare, slow-growing malignant bone tumor that originates from the remains of the notochord and predominantly affects the axial skeleton (especially the sacrum and occipital region). Clival chordomas represent 1%–4% of intracranial neoplasms and only 0.2% of CNS tumors; thus, their prevalence is very low (1 case per 2 million inhabitants). The mean age at onset is in the fourth decade of life. The most common symptoms are diplopia due to involvement of the ocular pairs, dysphagia, and headaches.^1,2 More rarely, it affects phonetics in isolation due to the involvement of the lower cranial nerves (hypoglossal or glossopharyngeal).^3-5

Although it has a slow growth, it behaves aggressively; therefore, it is at risk of metastasis to the lymph nodes, heart, lungs, or liver.⁶ Diagnosis is made with imaging tests (radiography, cranial CT with bone window, and cranial MRI).⁷ The gold standard treatment should be surgical and may be completed with radiotherapy in cases with incomplete resections.

We present a case of clivus chordoma whose initial symptom was a selective dysarthria to pronounce the letter “R” (Video 1 and 2).

Video 1

Incorrect pronunciation of the “R” phoneme (in Spanish): “El perro de San Roque no tiene rabo, porque Ramón Rodríguez se lo ha cortado” (in English: Saint Roque's dog doesn't have a tail because Ramón Rodríguez has cut it off).Download Supplementary Video 1 ^{(4MB, mov)} via http://dx.doi.org/10.1212/001104_Video_1

Video 2

Repetition of words with and without the R phoneme (in Spanish): “carro, burro, casa, coche, pero, vaca, niño, en, país, coche, carro” (in English: cart, donkey, house, car, but, cow, child, in, country, car, and cart, respectively).Download Supplementary Video 2 ^{(16.3MB, mov)} via http://dx.doi.org/10.1212/001104_Video_2

Case

A 41-year-old man with diabetes and dyslipidemia treated with insulin and atorvastatin presented with difficulty in pronouncing the letter “R” in Spanish since 1 month, without dysphagia, diplopia, eyelid ptosis, or other symptoms. The rest of the phonemes were preserved, and the patient did not have aphasia or paraphasia and had correct repetition and comprehension (Video 2). The examination revealed left hemilingual atrophy and the absence of the pharyngeal reflex (Figure 1A); the rest of the neurologic examination was normal. No vascular lesions were observed on MRI, but a lytic lesion was described in the lower half of the clivus, which affected the jugular tubercles and occipital condyles, as well as the hypoglossal canal bilaterally. It was accompanied by a soft tissue mass component, and together, they occupy a global area of 37 × 38 × 22 mm (Figure 2, A and B), and also contacted the intracranial segment of both vertebral arteries.

Figure 1. Left Hemilingual Atrophy, Before (A) and After (B) Surgery.

Figure 2. Brain MRI.

(A) Sagittal flair sequence: lytic lesion in the lower half of the clivus (arrow), affecting affecting the jugular tubercles and occipital condyles, affecting the hypoglossal canal. It is accompanied by a component mass of 37 × 38 × 22 mm. (B) Axial flair sequence: the arrow points to the lytic lesion of the clivus, which contacts the contacts the intracranial segment of both vertebral arteries.

Body CT and tumor markers were normal. Laboratory tests revealed the following: total calcium 10.3 mg/dL, vitamin D (25-OH) 18 ng/mL, blood glucose 282 mg/dL, total cholesterol 147 mg/dL, and triglycerides 215 mg/dL.

Endonasal endoscopy with complete resection was performed, improving lingual atrophy and postoperative dysarthria (Figure 1B). The pathologic anatomy confirmed the diagnosis of chordoma (fibrous stroma and immunohistochemical staining with Brachyury and CKAE1/3).

Discussion

The R phoneme in Spanish is the most difficult to learn. The integrity of all the structures of the oral cavity is essential to pronounce this letter. In our case, given the difficulty in pronouncing only this letter, local causes must first be ruled out (alterations in the palate or in the motility of the tongue or weakness in the phonatory muscles dependent on the hypoglossal and glossopharyngeal nerves such as tongue, lips, or masseters muscles). In our patient, the reason for consultation was the difficulty of pronunciation (selective dysarthria) of a single letter, without dysphagia—despite the atrophy of the tongue—or other symptoms and with no other difficulty in production or understanding of the language. Isolated dysarthria in a patient with diabetes with poor control is usually secondary to vascular pathology. However, in our case, no vascular lesions were evidenced in neuroimaging tests, and this highly selective dysarthria was related to atrophy of the tongue (the hypoglossal nerve canal was invaded by chordoma). After surgery, the symptom has improved significantly.

Selective letter pronunciation dysarthria is rarely the initial symptom of a clivus chordoma. Surgical complete resection improves the symptoms and prognosis of the patient.

Appendix. Authors

Study Funding

No targeted funding reported.

Disclosure

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