Collet-Sicard syndrome
Collet-Sicard syndrome is an unusual syndrome of lower cranial nerve palsies.
Frédéric Justin Collet, in 1915 termed a “glossolaryngoscapulopharyngeal hemiplegia”, which was later to be named “Collet's syndrome”.
This condition referred to as “Collet-Sicard syndrome”, named in conjunction with Jean-Athanase Sicard, who provided a description of the disorder independent of Collet.
It is a combination of Vernet's syndrome with palsy of the XII nerve without sympathetic involvement.
Etiology
Often signifying disease at the skull base, including malignancy, trauma or vascular causes.
It is the most frequently reported neurologic complication associated with Jefferson fractures.
Developmental abnormalities of the craniocervical junction should be considered in the differential diagnosis.
Reviews
Domenicucci et al., analyzed 14 cases collected from the literature between 1925 and 2013, reported a further personal case and performed an anatomical study of the paracondylar, atlas and styloid process region. The anatomical dissection was performed to assess the anatomic relationships in the site of transit in which the nerves IX, X, XI and XII are injured.
A total of 14 cases of p-CSS were collected: 9 caused by a condyle fracture and 5 by an atlas fracture. The patients were 13 males and only 1 female, 10 of them had a blunt trauma due to the result of axial loading (force directed through the top of the head and through the spine) falling on the head. The nine cases with a condyle fracture were associated to the dislocation of part of it, while those with atlas fractures showed the fracture and/or disjunction of the articular mass. The anatomical evaluations reveal that the lower four cranial nerves, at their emergency, pass through a close osteo-ligamentous space in relationship to the condyle. Below they run through a little wider channel between the articular mass of C1 and the styloid process. Two cases underwent surgical procedure. All the other cases were treated conservatively with immobilization of the cervical spine. During follow-up three cases with condylar fractures were found to be clinically unchanged and six showed modest improvements while one case with atlas fracture had a complete recovery and four improved significantly.
The p-CSS is caused by force directed through the top of the head. We suppose that the nerve injuries are due to their laceration caused by a displacement of a condyle fragment or to their compression and stretching when they pass between the lateral mass of the atlas and the styloid process. These modalities of trauma explain the better clinical results in patients affected by C1 fractures. Conservative treatment is the option of choice. Surgical option, when choosed, is not considered to fix nerve damages 1).
Case reports
A rare neurological presentation of internal jugular vein thrombosis induced by central venous catheter placement in a patient with cancer. A 71-year-old man gave a 3-week history of dysphagia and dysarthria with left-sided neck pain and headache. He was receiving chemotherapy for appendiceal adenocarcinoma. On examination, he had left 9th-12th cranial neuropathies, manifesting as voice hoarseness, decreased palatal movement, absent gag reflex, weakness of scapular elevation and left-sided tongue wasting. CT scan of neck showed the left subclavian central venous catheter tip was in the left internal jugular vein. Skull base MRI showed thrombus within the left jugular foramen extending intracranially.
His headache and neck pain resolved 2 days after removing the catheter and starting anticoagulation 2).
A 21-year-old man presented with slurred speech, hoarseness voice and difficulty swallowing his saliva following closed head trauma. The cranial nerve examination revealed left sided severe dysfunction of cranial nerves VII, IX, X, XI, and XII. A CT-Scan of the neck was performed demonstrating a fracture of the left styloid process at the base of the skull. The Magnetic Resonance Imaging showed unusually well seen lower cranial nerves due to nerve edema. The patient was managed conservatively with steroids and regular sessions of neuromuscular and orthophonic rehabilitation. The nutrition had to be administered by gastrostomy since he was unable to swallow. Six months after the injury a total neurological recovery was noted. It is a exceptional case of Collet-Sicard Syndrome caused by styloid process fracture 3).
A 70-year-old man who developed hoarseness, dysarthria, and dysphagia from developmental abnormalities of the craniocervical junction including a congenital occiput-C1-C3 fusion and hypoplastic dens. On computed tomography, the distance between the left transverse process of the atlas and the left styloid process of the skull was 3 mm 4).
Giant internal carotid artery aneurysm 5).
2015
A 63-year-old man diagnosed with Collet-Sicard syndrome due to a fracture of the right occipital condyle is presented. The cause of injury was falling off a bicycle. Dysphonia and dysphagia were present from the moment of injury, with the gradual development of light atrophy of the tongue muscles and right trapezius muscle. The diagnosis was based on examination by CT and MRI methods, the act of swallowing and physical examination by an otorhinolaryngology specialist and a neurologist who confirmed the diagnosis of injury to cranial nerves IX, X and XI on the right side. The patient was treated conservatively with application of a Philadelphia collar. Dysphagia required PEG tube insertion. Skull fracture healing was evident on a CT scan at 3-month follow-up. However, dysphonia with dysphagia and muscle atrophy remained persistent 6).