Jacobs Journal of Neurology and Neuroscience

Collet-Sicard Syndrome: Case Report

*Kim-Ir-Sen Santos Teixeira
Department Of Radiology And Diagnostic By Imaging, Medicine University, Federal University Of Goiás, Goiás, Brazil

*Corresponding Author:
Kim-Ir-Sen Santos Teixeira
Department Of Radiology And Diagnostic By Imaging, Medicine University, Federal University Of Goiás, Goiás, Brazil
Email:kimirsen@terra.com.br

Published on: 2019-01-22

Abstract

Collet-Sicard syndrome is a unique condition involving lesions of the skull base, which affect both the jugular foramen and the hypoglossal canal, affecting the lower cranial nerves IX, X, XI and XII. Symptoms of this pathology include loss of the palate of the posterior third of the tongue (IX nerve), vocal cord paralysis and dysphagia (X nerve), weakness in the sternocleidomastoid and trapezius (XI nerve) muscles and atrophy and paresis of the tongue muscles (XII nerve). The present report aims to describe the case in question, referring to a syndrome rare and little described in the literature. In the case of the patient reported, the conduct adopted was to perform radiotherapy sessions in order to prevent tumor progression since the surgery was contraindicated.

Keywords

Collet-Sicard syndrome; Schwannoma; Radiotherapy

Introduction

The first mention of the Collet-Sicard syndrome (CSS) occurred in 1915 by Frederic Collet, in reference to a soldier wounded by bullets during The First World War. Subsequently, Jean A. Sicard made new descriptions, culminating in the name of the syndrome [1]. It is described as a rare condition involving lesions of the base of the skull affecting both the jugular foramen and the hypoglossal canal. It is also known as condylar jugular syndrome, resulting from a unilateral and combined affection of the lower cranial nerves IX, X, XI and XII [2].

The possibilities of cause are numerous (table1) and among them it is possible to emphasize: Cranial base metastases, primary intracranial tumors (example: schwannoma), extracranial tumors, trauma, vascular lesions, iatrogenic complications, or inflammatory processes [1].

Fig 1:

Source: [1].

In view of this scenario, the present report aims to describe the case in question, referring to a syndrome rare and not described in the literature comparing clinical findings with current literature.

Case Report

Patient I.L.B, male, 61 years of age reported complaints of dysarthria, dysphasia and reduced tongue mobility, after an otorhinolaryngological evaluation a CT scan of the skull was requested (Figures 1A-B and 2A-B) which found an expansive, solid, hypovascular formation located in the jugular foramen and extending inferiorly to the right carotid space of probable neoplastic etiology. The main hypothesis considered is schwannoma. Subsequent referral to the neurologist and evaluation after detailed neurological examination showed a deviation of the tongue to the left side with right atrophy, impairment of the mobility of the right upper limb and paresis of the right lateral aspect of the neck. In addition to the reported symptoms, there were no alterations in other pairs of cranial nerves and other systems. In Magnetic Resonance Imaging (MRI) (Figures 3A-C and 4A-B), the neoplastic etiology for the neural sheath tumor (schwannoma) is confirmed. The next step was to refer the patient to the radiotherapy, after consulting the neurosurgeon who contraindicated surgery because of the presence of risks to damages in important brain areas and irreversible damage to the corresponding cranial nerves, which could lead to a total loss of their functions. Several radiotherapy sessions were performed without satisfactory results.

Figure 1: In A, axial CT of the base of the skull without contrast. The arrow indicates the hypo attenuating lesion in the jugular foramen. In B, axial CT of the skull base, with iodized contrast, the lesion, indicated again by the arrow, does not emphasize.

Figure 2: In A, sagittal CT of the skull with contrast, in which the key (}) indicates the hypodense lesion in its longitudinal aspect. In B, coronal CT, with contrast, demonstrating, at the base of the arrows, the lesion in its frontal aspect eroding the jugular gulf.

Figure 3: In A and B, coronal T2-MRI, demonstrating lesion with hypersignal in the jugular foramen. In C, coronal MRI, T1 after contrast (gadolinium). The tumor enhances intensely. The base of the arrows represents the tumor of the neural sheath (schwannoma).

Figure 4: In A, axial MRI CISS, demonstrating the heterogeneous lesion in the jugular foramen (arrow). In B, T1 axial MRI after contrast, intense tumor enhancement (arrow base).

Discussion

The symptoms of Collet-Sicard’s syndrome described in the literature [3] involve loss of the palate of the posterior third of the tongue (IX nerve), vocal cord paralysis and dysphagia (X nerve), weakness in the sternocleidomastoid and trapezius muscles (XI nerve) and atrophy and paresis of the tongue muscles (nerve XII). Symptoms manifested by I.L.B.

Most intracranial primary tumors affect adults, especially from 45 to 50 years, and among the causes, the most frequent are vascular [4]. The cause of CSS in the patient in question was the intracranial tumor in the neural sheath (schwannoma). Schwannomas are benign tumors, which develop mainly in the sensory part of the nerves (VIII and X) and rarely affect the nerve XII [5]. They mainly affect the female sex, and may be related to hereditary syndromes and exposure to ionizing radiation. By affecting important cranial nerves surgery is somewhat controversial [6]. In this sense, the most recommended treatment is the neuroradiological follow-up associated with curative radiotherapy because it is a slowly evolving tumor. This therapy may be associated with microsurgical resection [7].

In the case of the patient reported, the conduct adopted was the accomplishment of radiotherapy sessions. Surgery was contraindicated due to the risk of affecting the sensitivity and tasting of the posterior third of the tongue, laryngeal and palate sensitivity, speech, sternocleidomastoid and hypoglossal muscle movements and tongue on the injured side if there were intercurrences. However, tongue movement, swallowing and elevation of the right shoulder were affected and there was no improvement after treatment.

Final Considerations

The present approach on Collet-Sicard syndrome is of extreme relevance for the medical community because it is a rare occurrence with only three cases reported by the year 2015.

The symptoms initially reported by the patient and the adoption of radiotherapeutic therapy converge with data found in the literature in which the radiotherapeutic alternative is predominant. In addition, there is no vast database of the rare syndrome, therefore, studies on comorbidity are still necessary in order to improve the associated symptomatology. Therefore, it is of fundamental importance to continue research on Collet-Sicard syndrome, including its symptoms, diagnosis, treatment and clinical evolution, since it is a rare condition and still little described in the literature.

Appreciation to Professor Kim-Ir-Sen Santos Teixeira Radiologist and Head of the Department of Radiology and Diagnostic Imaging of the Faculty of Medicine of the Federal University of Goiás (UFG) and the Diagnostic Imaging Clinic for the support in the manuscript and availability of the iconographies used.

References

  1. RÍOS, R G et al. Collet-Sicard syndrome. Neurologia (English Edition). 2015, 30(2): 130-132.
  2. MNARI W. et al. An unusual etiology of posttraumatic Collet–Sicard Syndrome: a case report. Pan African Medical Journal. 2016, 23: 143.
  3. BRAVO F. et al. The collet-sicard syndrome and dentistry: case report. RGO- Revista Gaúcha de Odontologia. 2018, 66(2): 177-180.
  4. Brasileiro Filho, G. Bogliolo patologia geral. In: Bogliolo patologia geral. 2016. 
  5. LEE SH et al. Collet-Sicard SyndromeWith Hypoglossal Nerve Schwannoma: A Case Report. Annals of rehabilitation medicine. 2017, 41(6): 1100-1104. 
  6. MUZEVIC D et al. Stereotactic radiotherapy for vestibular schwannoma. Cochrane Database of Systematic Reviews. 2014:12.
  7. Instituto Nacional De Câncer José Alencar Gomes Da Silva. 2018.