Collet-Sicard syndrome caused by a paraganglioma in the region of the jugular foramen on one side: A case report and review.

Collet-Sicard syndrome is a rare neurological disorder caused by injury to the cranial nerve pairs IX, X, X, and XII. The author reports on a previously fit 27-year-old man who presented with dysphagia, choking on drinking water, hoarseness, weakness when turning the neck and shrugging the shoulders, and unexplained weight loss. Enhanced magnetic resonance imaging indicated a space-occupying lesion at the right jugular foramen. After surgical resection, the pathologic findings suggested a paraganglioma of the right jugular foramen and confirmed the diagnosis of Collet-Sicard syndrome. After postoperative treatment with a combination of acupuncture and modern medicine, the patient's symptoms significantly improved. This article analyzes previous literature regarding Collet-Sicard syndrome etiology and reports the case of a patient with a rare etiology, whose prognosis improved significantly after treatment with a combination of acupuncture and modern medicine.

Collet-Sicard syndrome: a scoping review.

Collet-Sicard syndrome (CSS) is the unilateral palsy of the cranial nerves (CN) IX, X, XI, and XII. To our knowledge, no review describes the characteristics of patients diagnosed with CSS. Therefore, this review aims to collect and describe all cases in the literature labeled as CSS. We performed a scoping review of the literature and conducted a database search in Embase and PubMed. We included articles and abstracts with case reports or case series of patients with CSS diagnosis. We classified the cases into two groups: "CSS", referring to patients presenting exclusively with IX-XII nerve involvement, and "CSS-plus", which corresponds to cases with CSS and other neurological impairments. We included 135 patients from 126 articles, of which 84 (67.7%) were male. The most common clinical manifestations reported were dysphagia and dysphonia. The most common etiology was tumoral in 53 cases (39.6%) and vascular in 37 cases (27.6%). The majority of patients showed partial or total improvement, with just over half receiving conservative treatment. The most frequent anatomic space was the jugular foramen (44.4%) and the parapharyngeal retrostyloid space (28.9%). Approximately 21% of the patients had other CN impairments, with the seventh and eighth CN most frequently compromised. We conclude that although there is a need for greater rigor in CSS reporting, the syndrome has a clear utility in identifying the localization of jugular foramen and parapharyngeal retrostyloid space pathology.

Course of Rehabilitation Treatment of Neuralgic Amyotrophy Presenting with Collet-Sicard Syndrome / The Japanese Journal of Rehabilitation Medicine

Typical cases of neuralgic amyotrophy present with sudden onset of excruciating pain in the shoulders and upper extremities, followed by marked muscle weakness and atrophy over a period of hours to days. Neuralgic amyotrophy is not confined to the brachial plexus, and difficulties in its diagnosis may delay the start of rehabilitation therapy. Here, we report a case of rehabilitation of a patient with neuralgic amyotrophy presenting with Collet-Sicard syndrome (9th, 10th, 11th, and 12th cranial nerve disorder)．A 44-year-old man developed severe pain from the left posterior neck to the occipital region, followed by sporadic onset of dysarthria, dysphagia, and difficulty in raising the left upper limb over several weeks. Nerve conduction studies showed marked bilateral differences in the amplitude of the compound muscle action potential recorded from the trapezius during accessory nerve stimulation. Needle electromyography showed abnormal resting potentials in the left trapezius and left side of the tongue and a decrease in the interference pattern during voluntary contraction. Based on the clinical course, neurological and laboratory findings, a diagnosis of neuralgic amyotrophy was made. Speech language hearing therapy was performed for dysarthria and dysphagia, and physical therapy was performed for difficulty in raising the left upper limb due to accessory nerve palsy. Rehabilitation along with recovery from inflammation-induced neuropathy allowed the patient to become independent in activities of daily living.

Course of Rehabilitation Treatment of Neuralgic Amyotrophy Presenting with Collet-Sicard Syndrome / The Japanese Journal of Rehabilitation Medicine

Typical cases of neuralgic amyotrophy present with sudden onset of excruciating pain in the shoulders and upper extremities, followed by marked muscle weakness and atrophy over a period of hours to days. Neuralgic amyotrophy is not confined to the brachial plexus, and difficulties in its diagnosis may delay the start of rehabilitation therapy. Here, we report a case of rehabilitation of a patient with neuralgic amyotrophy presenting with Collet-Sicard syndrome (9th, 10th, 11th, and 12th cranial nerve disorder)．A 44-year-old man developed severe pain from the left posterior neck to the occipital region, followed by sporadic onset of dysarthria, dysphagia, and difficulty in raising the left upper limb over several weeks. Nerve conduction studies showed marked bilateral differences in the amplitude of the compound muscle action potential recorded from the trapezius during accessory nerve stimulation. Needle electromyography showed abnormal resting potentials in the left trapezius and left side of the tongue and a decrease in the interference pattern during voluntary contraction. Based on the clinical course, neurological and laboratory findings, a diagnosis of neuralgic amyotrophy was made. Speech language hearing therapy was performed for dysarthria and dysphagia, and physical therapy was performed for difficulty in raising the left upper limb due to accessory nerve palsy. Rehabilitation along with recovery from inflammation-induced neuropathy allowed the patient to become independent in activities of daily living.

Varicella zoster-associated progressive lower cranial and upper cervical polyneuropathy: a case report.

BACKGROUND: Multiple cranial neuropathies carry a wide range of differential diagnoses, and when combined with cerebrospinal fluid monocytosis they often suggest an infective etiology. Reactivation of varicella zoster virus has been associated with a wide range of neurological complications. Among the cranial nerves, the upper cranial nerves (trigeminal and facial nerves) are more commonly affected; there have been some reports of lower cranial polyneuropathies resulting from varicella zoster virus reactivation. However, polyneuropathy involving both the cranial and cervical nerves is rarely reported. CASE PRESENTATION: This report highlights the case of a 64-year-old Chinese man presenting with an acute, severe dysphagia and dysphonia secondary to herpes zoster-associated progressive polyneuropathy involving the lower cranial and upper cervical nerves, without any mucocutaneous manifestations. CONCLUSIONS: To our knowledge, this is the first case of varicella zoster virus-associated cranial and cervical polyneuropathy in the literature. The report also highlights the poor sensitivity of varicella zoster virus DNA detection by polymerase chain reaction in the cerebrospinal fluid, and proposes that serology be routinely performed in such polymerase chain reaction-negative cases without a clear diagnosis.

Delayed Collet-Sicard syndrome after internal carotid dissection and Jefferson fracture. Case report and Review of Literature.

BACKGROUND: Lower cranial nerve palsies, or Collet-Sicard syndrome, can be caused by many different etiologies including head trauma, basilar occipital fractures, tumors, and interventions. Few reports describe different presentations of this condition, and we present here a case study to increase awareness of and add to the variable spectrum. CASE DESCRIPTION: A 56-year-old who had been hit while diving was admitted to our department. On examination, he was conscious without any signs of lateralization but presented with severe neck pain. CT brain and cervical spine revealed a C1 fracture with bilateral symmetrical fracture of the anterior and posterior arches (Jefferson's fracture) and slight bilateral joint dislocation C1-C2 discreetly predominant on the left. One week later, he presented with dysarthria, dysphonia, swallowing disorder, anisocoria, tongue deviation, and palate deviation (XII, IX, and X). CT Angiography showed dissection of the internal carotid artery immediately after the carotid bulb. He has been treated conservatively with curative anticoagulants with stable symptoms. No surgical intervention had been proposed. CONCLUSION: Adding to the literature, delayed Collet-Sicard syndrome and lower cranial affection can be caused by missed carotid wall hematoma following severe craniocervical trauma associated with Jefferson's fracture.

Glossopharyngeal schwannoma: Clinical case report. / Schwannoma del glosofaríngeo: reporte de caso clínico.

Schwannomas of the glossopharyngeal nerve are extremely rare tumors of the posterior fossa. In a 100-year review, a total of 42 cases were found between 1908-2008. The most common clinical data are associated with its location, the most common being cochlear vestibule symptoms and symptoms of glossopharyngeal nerve function. its diagnosis has now been facilitated by the use of magnetic resonance, however, it is very complicated to define preoperatively if the tumor originates from the ix, x or xi NC. We present the case of a 42-year-old patient with a syndrome of angulopentocerebellar syndrome, posterior torn (jugular) hole syndrome + anterior condyle (Collet-Sicard). The treatment used was surgical with transcondylar lateral extreme approach, with monitoring of cranial nerves and trans-operative evoked potentials.

Metastatic Adenocarcinoma of Temporal Bone with Collet-Sicard Syndrome.

INTRODUCTION: Metastatic tumors of the temporal bone are extremely rare. Collet-Sicard syndrome is an uncommon condition characterized by unilateral palsy of the lower four cranial nerves. The clinical features of temporal bone metastasis are nonspecific and mimic infections such as chronic otitis media and mastoiditis. CASE REPORT: This report describes a rare case of metastatic adenocarcinoma of the temporal bone causing Collet-Sicard syndrome, presenting with hearing loss, headache and ipsilateral cranial nerve palsies. The patient was a 68-year old woman initially diagnosed with extensive mastoiditis and later confirmed as having metastatic adenocarcinoma of the temporal bone, based on histopathologic findings. CONCLUSION: Clinical presentation of metastatic carcinoma of the temporal bone can be overshadowed by infective or inflammatory conditions. This case report is to emphasize the point that a high index of clinical suspicion is necessary for the early diagnosis of this aggressive disease which carries relatively poor prognosis. This report highlights that it is crucial to suspect malignant neoplasm in patients with hearing loss, headache and cranial nerve palsies.

A Collet-Sicard syndrome due to internal carotid artery dissection associated with cerebral amyloid angiopathy-related inflammation.

BACKGROUND: Cerebral amyloid angiopathy-related inflammation is a rare condition with approximately 100 reported cases. Its clinical manifestations are varied. We report here a novel presentation of this disease. CASE PRESENTATION: A 61-year-old Caucasian man presented with rapidly progressive paralysis of the IX, X, XI and XII right cranial nerves associated with right central facial nerve palsy. Brain computed tomography angiography and cerebral catheter angiography found a focal fusiform enlargement of the distal cervical portion of the right internal carotid artery, related to a pseudo-aneurysm suggesting an evolution of a dissection and intra-cranial vessel dysplasia. Brain magnetic resonance imaging showed multiple asymmetrical subcortical regions of hyperintensity on T2 fluid-attenuated inversion recovery sequences. Punctiform cortical hyposignals on T2-weighted gradient echo magnetic resonance imaging sequences were mostly congruent with the white matter hyperintensities. There was a decreased cerebral perfusion at the frontal hyperintense fluid-attenuated inversion recovery region. Spectrometry identified a lactate-lipid peak. A brain biopsy showed intravascular amyloid deposits. Corticosteroid therapy was initiated, leading to a dramatic improvement of both clinical condition and magnetic resonance imaging brain lesions. CONCLUSION: This case report suggests that extra-cranial vasculitis and dysplasia can exceptionally be found in patients satisfying cerebral amyloid angiopathy-related inflammation criteria.

Severe localised granulomatosis with polyangiitis (Wegener's granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review.

BACKGROUND: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels. Cranial involvement can result in cranial nerve palsies and, rarely, pituitary infiltration. CASE PRESENTATION: We describe the case of a 32 year-old woman with limited but severe GPA manifesting as progressive cranial nerve palsies and pituitary dysfunction. Our patient initially presented with localised ENT involvement, but despite treatment with methotrexate, she deteriorated. Granulomatous inflammatory tissue around the skull base resulted in cavernous sinus syndrome, facial nerve palsy, palsies of cranial nerves IX-XII (Collet-Sicard syndrome), and the rare complication of cranial diabetes insipidus due to pituitary infiltration. The glossopharyngeal, vagus and accessory nerve palsies resulted in severe dysphagia and she required nasogastric tube feeding. Her neurological deficits substantially improved with treatment including high dose corticosteroid, cyclophosphamide and rituximab. CONCLUSIONS: This case emphasises that serious morbidity can arise from localised cranial Wegener's granulomatosis in the absence of systemic disease. In such cases intensive induction immunosuppression is required. Analysis of previously reported cases of pituitary involvement in GPA reveals that this rare complication predominantly affects female patients.

Collet-Sicard Syndrome Attributable to Extramedullary Plasmacytoma of the Jugular Foramen.

BACKGROUND: Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma-multiple myeloma (EP-MM) and the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder. CASE DESCRIPTION: A 59-year-old woman presented with 4 months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia. Examination identified left tongue weakness and nonspecific sensory abnormalities of the ear, pharynx, and throat localizing to cranial nerves IX-XII. Imaging revealed a 3.4 × 1.4 × 2.8 cm lytic lesion extending from the left jugular foramen into the posterior fossa, for which she was referred to neurosurgery and otolaryngology for consideration of resection. A second, much smaller (1.1-cm) lytic lesion in the left posterior occipital bone was incidentally discovered by the surgeon during preoperative consultation. A stereotactic biopsy of the occipital lesion was subsequently recommended, which identified plasma cell neoplasm. Serum studies and skeletal survey were consistent with MM, and a definitive pathologic diagnosis of MM with cranial EP was confirmed by bone marrow biopsy. CONCLUSION: Tumors of the jugular foramen present with a diverse array of lower cranial nerve deficits, including Collet-Sicard syndrome, a rare subset of JFS-spectrum diseases. Paragangliomas are the most common jugular foramen neoplasms, followed by schwannomas and meningiomas; however, many other rare entities have been reported as masqueraders, and diligent work-up with consideration for preliminary biopsy is recommended, particularly in the presence of additional lesions, equivocal imaging findings, or cases arousing high suspicion.

Collet-Sicard Syndrome With Hypoglossal Nerve Schwannoma: A Case Report.

Collet-Sicard syndrome is a rare syndrome that involves paralysis of 9th to 12th cranial nerves. We report an uncommon case of schwannoma of the hypoglossal nerve in a 39-year-old woman presented with slurred speech, hoarse voice, and swallowing difficulty. Physical examination revealed decreased gag reflex on the right side, decreased laryngeal elevation, tongue deviation to the right side, and weakness of right trapezius muscle. MRI revealed a mass lesion in the right parapharyngeal space below the jugular foramen. The tumor was surgically removed. It was confirmed as hypoglossal nerve schwannoma via pathologic examination. Videofluoroscopic swallowing study revealed aspiration of liquid food and severe bolus retention in the vallecula and piriform sinus. Laryngoscopy revealed right vocal cord palsy. Electrodiagnostic study revealed paralysis of the right 11th cranial nerve. In summary, we report an uncommon case of schwannoma of the hypoglossal nerve with 9th to 12th cranial nerve palsy presenting as Collet-Sicard syndrome.

Collet-Sicard Syndrome With Hypoglossal Nerve Schwannoma: A Case Report

Collet-Sicard syndrome is a rare syndrome that involves paralysis of 9th to 12th cranial nerves. We report an uncommon case of schwannoma of the hypoglossal nerve in a 39-year-old woman presented with slurred speech, hoarse voice, and swallowing difficulty. Physical examination revealed decreased gag reflex on the right side, decreased laryngeal elevation, tongue deviation to the right side, and weakness of right trapezius muscle. MRI revealed a mass lesion in the right parapharyngeal space below the jugular foramen. The tumor was surgically removed. It was confirmed as hypoglossal nerve schwannoma via pathologic examination. Videofluoroscopic swallowing study revealed aspiration of liquid food and severe bolus retention in the vallecula and piriform sinus. Laryngoscopy revealed right vocal cord palsy. Electrodiagnostic study revealed paralysis of the right 11th cranial nerve. In summary, we report an uncommon case of schwannoma of the hypoglossal nerve with 9th to 12th cranial nerve palsy presenting as Collet-Sicard syndrome.

Developmental abnormalities of the craniocervical junction resulting in Collet-Sicard syndrome.

BACKGROUND CONTEXT: Collet-Sicard syndrome describes the paralysis of cranial nerves IX-XII and is the most frequently reported neurologic complication associated with Jefferson fractures. As the lateral mass of the atlas is displaced laterally toward the styloid process and the stylohyoid ligament, the lateral mass impinges on cranial nerves IX-XII. However, Collet-Sicard syndrome in association with other anomalies of the atlas has rarely been reported. PURPOSE: The aim of this study was to report an unusual case of Collet-Sicard syndrome as a result of developmental abnormalities of the craniocervical junction. STUDY DESIGN/SETTING: This is a case report of a single patient. METHODS: Chart and radiographic data were reviewed and reported. RESULTS: We report 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. CONCLUSION: In suspected Collet-Sicard syndrome, developmental abnormalities of the craniocervical junction should be considered in the differential diagnosis.

Occipital condyle fracture and lower cranial nerve palsy after blunt head trauma - a literature review and case report.

BACKGROUND: Lower cranial nerve (IX-XII) palsy is a rare condition with numerous causes, usually non-traumatic. In the literature it has been described only a few times after trauma, mostly accompanied by a fracture of the occipital condyle. Although these types of fractures have rarely been reported one could suspect they have been under-diagnosed. During the past decade they have been seen more frequently, most probably due to increased use of CT- and MRI-scanning. The purpose of this review is to increase the awareness of complications following injuries in the craniocervical region. METHODS: We based this article on a retrospective review of the medical record of a 24-year old woman admitted to our trauma center after being involved in a car accident and a review of the literature on occipital condyle fractures associated with lower cranial nerve palsy. RESULTS: The multitraumatized patient had suffered a dislocated occipital condyle fracture. Months later she was diagnosed with palsy to cranial nerve IX-XII. Literature review shows that occipital condyle fractures are rare as isolated injuries and are in many cases accompanied by further injuries to the cervical spine and soft tissue structures, in many cases ending with severe disability. The exact mechanism leading to these injuries cannot always be explained. CONCLUSION: Recognition of soft tissue injuries in patients with blunt head trauma is important. CT findings involving the craniocervical junction in these patients advocates further investigations including a thorough neurological examination and liberal use of MRI.

Síndrome de Collet-Sicard como forma de presentación de fracturas combinadas del atlas y cóndilos occipitales / Collet-Sicard syndrome as a presentation of combined fractures of atlas and occipital condyles

El síndrome de Collet - Sicard consiste en una afectación unilateral y combinada de los nervios craneales bajos, originado por lesiones en la base craneal. CASO CLÍNICO: Paciente masculino con antecedentes de sufrir accidente del tránsito. A su llegada al hospital refiere dolor cervical alto, voz apagada y dificultad para tragar. Al examen físico se contacta paresia de pares craneales bajos. Se realizan Rx de columna cervical y tomografía axial de región cráneo espinal. Se diagnostica una fractura de los cóndilos occipitales y una fractura tipo II del atlas. Se coloca un Halo chaleco. Se consulta al paciente al final del primer mes de tratamiento con alivio del dolor cervical y sin empeoramiento neurológico. CONCLUSIONES: La afectación de pares craneales bajos puede ser la forma clínica de presentación de las lesiones traumáticas de la región cráneo espinal. Su reconocimiento temprano favorece el pronóstico de estos pacientes

Collet syndrome - Sicard is a combined unilateral involvement and lower cranial nerves, caused by damage to the cranial base. Case report. Male patient with a history of developing traffic accident. Upon arrival at the hospital referred high cervical pain, muffled voice and difficulty swallowing. Physical examination contact lower cranial nerve paresis. Rx are performed CT cervical spine and skull spinal region. Was diagnosed with a fracture of the occipital condyles and type II fracture of the atlas. Place a Halo vest. They see patients at the end of the first month of treatment with cervical pain relief without neurological deterioration. CONCLUSIONS: The lower cranial nerve involvement may be the clinical presentation of traumatic injuries of the skull spinal region. Its early recognition and the prognosis of these patients.

A síndrome de Villaret e o espaço retroparotídeo / Villaret syndrome and retroparotid space

A partir do relato de um caso sobre a síndrome de Villaret, o autor ressalta a importância da relação anátomo-clínica, aspectos clínicos e etiologias das síndromes que cursam com o comprometimento dos quatro últimos nervos cranianos quando da sua passagem extracranial.

From the case study of a patient with the Villaret syndrome the author emphasizes the importance of the anatomical-clinical relationship, clinical aspects, and etiologies of syndromes that are associated with the impairment of the last four cranial nerves during its out of skull passage.