Progressive Cranial Nerve Palsies Research Articles

Management of rare atlantoaxial synovial cyst case with extension to the cerebellopontine angle: illustrative case.

Synovial cysts are a common finding in degenerative spine disease, most frequently involving the facet joints of the lumbar spine. Synovial cysts are less common in the cervical spine and rarely involve the atlantoaxial junction. In this case report, the authors detail a unique presentation of a left atlantoaxial synovial cyst with large intracranial extension into the cerebellopontine angle causing progressive cranial nerve palsies resulting in tinnitus, vertigo, diminished hearing, gait imbalance, left trigeminal hypesthesia, left facial weakness, and dysarthria. The patient underwent a retromastoid craniectomy for resection of the synovial cyst, resulting in improvement and resolution of symptoms. Follow-up occurred at 6 weeks, 3 months, and 5 months postoperatively without recurrence on imaging. The authors describe acute and long-term management of a unique presentation of an atlantoaxial synovial cyst including retromastoid craniectomy, intervals for follow-up for recurrence, and possible treatment options in cases of recurrence. A systematic literature review was also performed to explore all reported cases of craniocervical junction synovial cysts and subsequent surgical management.

Posterior Fossa Calcifying Pseudoneoplasm of the Neuraxis (CAPNON): Presentation of Three Surgical Cases.

Calcifying pseudoneoplasm of the neuraxis (CAPNON) is an extremely rare entity with fewer than 150 cases reported in the literature and mostly with a supratentorial or spinal location. Posterior fossa CAPNON has been reported scarcely, and association with perilesional edema is a topic not yet approached which might play a significant role in treatment decision and clinical progression. Our objective is to report, to our knowledge, the first series of 3 posterior fossa CAPNON surgically treated in a single institution and assess features that help provide a systematic approach to diagnosis and timely treatment. This was a monocentric, retrospective study of surgical patients diagnosed with a posterior fossa CAPNON in the last 5years. A thorough bibliographic research was conducted. Three patients were included. Locations involved IV ventricle, right cerebellopontine angle with extension to foramen magnum, and cerebellar vermis. Two of them presented with symptoms linked to acute hydrocephalus, and the other one presented with progressive cranial nerve palsy and brainstem compression signs. The 3 of them showed radiological signs of perilesional edema on their preoperative magnetic resonance imaging. Gross total resection was accomplished in one case, with near and subtotal resections in the others. There were no complications. The outcome was favorable in all cases. It is essential to contemplate this infrequent diagnosis in cases of calcified lesions involving the posterior fossa. When symptoms manifest, surgery should be considered. Perilesional edema could be associated with symptomatic progression and hence a sign suggesting the need for surgical treatment.

Case report: The unusual case of a sellar/suprasellar Extrarenal Rhabdoid tumour in an adult

Abstract We present the case of a 33 year old lady presenting with progressive cranial nerve palsies caused by an Extrarenal Rhabdoid tumour manifesting as a sellar/suprasellar mass. We share our experience of managing the tumour and review the existing literature.

Severe localised granulomatosis with polyangiitis (Wegener\u2019s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review

BackgroundGranulomatosis 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 presentationWe 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.ConclusionsThis 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.

Cavernous sinus involvement in human papillomavirus associated oropharyngeal squamous cell carcinoma: case report of an atypical site of distant metastasis

BackgroundHPV-associated OSCC (HPV-OSCC) has been determined to be a distinct disease entity from non-HPV associated OSCC. Patients affected by HPV-OSCC generally have a more favourable prognosis, with improved rates of locoregional control and survival compared with their non-HPV counterparts. Despite this, HPV-OSCC has a similar rate of distant metastases. Interestingly, recent evidence has emerged that demonstrates more frequent atypical metastasis patterns when compared to non-HPV associated disease. To the best of our knowledge, this report describes the first case of a confirmed HPV-OSCC with distant metastasis to the cavernous sinus.Case PresentationA 62-year-old non-smoking male presented to the head and neck oncology clinic with a five-month history of enlarging right neck mass causing neck pain, dysphagia, and dysphonia. HPV-associated base of tongue squamous cell carcinoma (cT4aN2c) was diagnosed, and he was treated with primary chemoradiation. Shortly after treatment, he presented with progressive bilateral cranial nerve palsies including left cranial nerve III and right cranial nerve VI involvement. Imaging identified masses in the left cavernous sinus with extension of tumor into the sella and in the right cavernous sinus at the level of Dorello’s canal. Endoscopic Image Guided Transsphenoidal biopsy of the left sellar mass confirmed distant metastases from the previously treated HPV-OSCC primary to the cavernous sinus. The patient was palliated with carboplatin and paclitaxel.ConclusionThe presented report is the first documented case of confirmed HPV-associated oropharyngeal squamous cell carcinoma metastasizing to the cavernous sinus, and the only HPV confirmed head and neck cancer case to present with metastasis to the cavernous sinus and limited extracranial disease. This case demonstrates the importance of recognizing presentations of atypical metastasis that are possible in HPV-associated oropharyngeal cancer. Given the rarity of metastasis to this region, vigilance in follow up is instrumental in early identification and treatment for these patients.

Endovascular Treatment of Children With Cerebral Venous Sinus Thrombosis: A Case Series

Cerebral venous sinus thrombosis is a potentially serious condition affecting 0.56 to 0.67 per 100,000 children annually; adverse outcomes are common. The standard of care is anticoagulation with heparin. A proportion of patients, however, remain in a severe clinical condition and in these, endovascular therapy is an alternative treatment. There is little published literature on the use of endovascular treatments in children with cerebral venous sinus thrombosis. We retrospectively reviewed case notes and imaging in a consecutive series of nine children treated using endovascular therapy after diagnosis of cerebral venous sinus thrombosis. Clinical presentation, decision to escalate therapy, methods of recanalization, and clinical outcome were assessed. Nine children were treated (age range 18 months to 16 years). Diagnosis was made by computed tomography, computed tomography venography, magnetic resonance imaging, or magnetic resonance venography. Seven children were in a coma; one had signs of raised intracranial pressure with progressive cranial nerve palsies; and one was drowsy with a fluctuating hemiparesis. Eight children had been treated with heparin without improvement. Several endovascular methods were used including local tissue plasminogen activator, microguidewire and catheter disruption, balloon angioplasty, and thromboaspiration using the Penumbra device. Eight children had good functional outcomes. One child died as a result of uncontrolled intracranial hypertension secondary to cerebral venous sinus thrombosis. Endovascular therapy may have a role in the treatment of cerebral venous sinus thrombosis in children when medical therapy has failed and the patient is in a poor clinical condition.

Garcin syndrome as the initial presentation of metastatic adenocarcinoma of the prostate

We describe an unusual case of metastasis of the skull base from prostate cancer. A 64-year-old man was referred for progressive cranial nerve palsies. Brain magnetic resonance imaging showed a metastatic tumor in the right petrous bone. He was presenting Garcin syndrome indicating involvement of the right III, IV, V, VI, VII, VIII, IX, X, and XII cranial nerves. The serum PSA level was 5118 ng/mL (standard threshold 4 ng/mL). A transrectal needle biopsy was performed, and poorly differentiated adenocarcinoma was detected in the prostate. Therefore, he was finally diagnosed with skull base metastasis of the prostate cancer. Androgen deprivation therapy and external beam radiation therapy to the skull base resulted in complete resolution of the cranial nerve palsies for more than 30 months. This is the first reported case of metastatic prostate cancer presenting as Garcin syndrome.

Invasive aspergillosis of the temporal bone

Invasive aspergillosis of temporal bone, an extremely rare yet potentially life-threatening disease entity, occurs mostly in immuno-compromised individuals. We report a case of a 65-year-old diabetic male who presented with bilateral ear discharge, bilateral subepithelial ear masses, and progressive left facial palsy of recent onset. The biopsy specimen taken from either side after surgical debridement revealed only nonspecific inflammatory granulation tissue, but the culture of the tissue was positive for Aspergillus flavus. Invasive aspergillosis was confirmed by demonstration of the fungi in the tissues with Gomori-Methanamine Silver staining. The patient was hence put on long-term Itraconazole therapy and is presently doing well. The possibility of invasive fungal otitis always needs to be considered as a differential diagnosis in immuno-compromised patients presenting with atypical otomastoiditis so as to diagnose this rare condition at an early stage to reduce the delay in the treatment and prevent its possible complications such as skull base osteomyelitis and progressive cranial nerve palsies.

Progressive Multiple Bilateral Cranial Nerve Palsies Due to Malignant Peripheral Nerve Sheath Tumour

Malignant peripheral nerve sheath tumours (MPNST) are rare tumours that usually do not involve cranial nerves. The case we report is to the best of our knowledge the first description of bilateral progressive cranial nerve palsies due to a MPNST in a patient without evidence of Neurofibromatosis.

Bacterial abscess of the medulla oblongata

Isolated brainstem abscess, specifically, medullary abscess, is rare and often fatal. Diagnosis requires appropriate imaging and a high degree of clinical suspicion. Good outcomes are possible. A 69-year-old woman presented with an isolated medulla oblongata abscess that manifested as a syndrome of rapidly progressive multiple cranial nerve palsies and decreased level of consciousness. Microneurosurgical incision and drainage of the medullary abscess were performed. Maximal therapy of antibiotics, intensive care management, and extended rehabilitation was delivered. A prompt diagnosis of medullary abscess, with immediate microneurosurgical intervention and maximal medical therapy, may result in a good outcome.

Development of cranial nerve palsy shortly after endosaccular embolization for asymptomatic cerebral aneurysm: report of two cases and literature review

We report two cases of asymptomatic cerebral aneurysm in which cranial nerve palsy (CNP) developed shortly after symbolization. The CNP occurred immediately in case 1, but case 2 showed the CNP 30 h after symbolization. Although both aneurysms had increased in size on follow-up angiography, case 2 who showed dome re canalization resulted in progressive CNP deterioration. These findings suggest that the CNP may result not only from mechanical compression by coils but also from inflammation induced by perpendicular thrombosis, and that the prognosis of the CNP may be influenced by dome re canalization. This complication should be kept in mind in treatment for asymptomatic aneurysms adjacent to the cranial nerves.

Bilateral intracavernous carotid artery aneurysms presenting as progressive cranial nerve palsies

Bilateral intracavernous carotid artery aneurysms are rare and can present with different clinical findings. We report such a case presenting with progressive cranial nerve palsies due to gradual increase in the size of aneurysms. This case demonstrates the clinician’s difficulty in diagnosis when isolated cranial nerve palsy is the sole presenting sign.

Difficulties in diagnosing progressive bulbar paralysis (Fazio-Londe disease)

Background: Progressive bulbar paralysis of childhood is characterized by progressive cranial nerve palsies especially bilateral facial weakness, swallowing difficulties and bilateral ptosis. Fazio-Londe disease (OMIM 211500) is usually classified with spinal muscular atrophies of autosomal recessive or dominant inheritance. Resembling diseases are Vialetta-van Laere syndrome (OMIM 211530) and Madras-type motor neuron disease. Diagnosis consists of the clinical features, electromyograhic and histologic findings and the exclusion of other causes of progressive bulbar paresis.

Slowly progressive cranial nerve palsies

Perineural spread (PNS) from these lesionsinvolves either small nerves, identified at pathological examina-tion (incidental), or large nerves, presenting clinically as cranialnerve palsies. Basal cell carcinoma is the more common skincancer, but incidental PNS is most frequently associated withsquamous cell carcinoma.

Progressive cranial nerve palsy following shunt placement in an isolated fourth ventricle

Cranial nerve palsy is rarely seen after shunt placement in an isolated fourth ventricle. In the few reports of this complication, neuropathies are thought to be caused by catheter injury to the brainstem nuclei either during the initial cannulations or after shrinkage of the fourth ventricle. The authors treated a child who suffered from delayed, progressive palsies of the sixth, seventh, 10th, and 12th cranial nerves several weeks after undergoing ventriculoperitoneal shunt placement in the fourth ventricle. Magnetic resonance imaging revealed the catheter tip to be placed well away from the ventricular floor but the brainstem had severely shifted backward, suggesting that the pathogenesis of the neuropathies was traction on the affected cranial nerves. The authors postulated that the siphoning effect of the shunt caused rapid collapse of the fourth ventricle and while the cerebellar hemispheres were tented back by adhesions to the dura, the brainstem became the only mobile component in response to the suction forces. Neurological recovery occurred after surgical opening of the closed fourth ventricle and lysis of the basal cistern adhesions, which restored moderate ventricular volume and released the brainstem to its normal position.

Primary malignant lymphoma of the trigeminal region treated with rapid infusion of high-dose MTX and radiation: case report and review of the literature

Background Extra-axial primary CNS lymphoma, considered rare, mainly arise in the white matter of the brain. Though the tumor responds well to radiation and chemotherapy, the prognosis of primary CNS lymphoma remains poor. We report a case of primary lymphoma of Meckel’s cave mimicking a trigeminal schwannoma radiographically, which achieved complete remission through use of rapid high-dose MTX therapy and radiation therapy. Case presentation The patient, a 55-year-old Japanese male, presented left trigeminal neuralgia. Magnetic resonance imaging (MRI) revealed a mass lesion in the left side of Meckel’s cave, with extension into the cerebellopontine angle and the infratemporal fossa through the foramen ovale, suggesting trigeminal schwannoma. However, the patient suffered radiologically inexplicable progressive cranial nerve palsy, which suggested malignant disease. MRI and CSF disclosed malignant tumor dissemination; biopsy revealed malignant lymphoma. The treatment, composed of the rapid infusion of high-dose MTX and whole brain and spine radiation, resulted in complete remission. Conclusion This case, which included atypical presentation of malignant lymphoma, illustrates the importance of including malignant lymphoma in the differential diagnosis of CP-angle and Meckel’s cave tumor. The results also confirmed the usefulness of combined rapid high-dose MTX therapy and radiation.

Infantile progressive bulbar palsy with deafness

A 12-month-old boy with progressive cranial nerve palsies followed by ventilatory failure demanding artificial ventilation, generalized muscle weakness, and rapid progression to death at the age of 21 months is described. The patient had normal early development and also apparently normal hearing at presentation of illness but, after 6 months of the onset of the disease, hearing loss was documented by brainstem auditory evoked potentials (BAEP). Although the initial clinical and laboratory findings of this infant could fit with the diagnosis of progressive childhood bulbar palsy or Fazio-Londe (FL) disease, the subsequent appearance of hearing loss suggests that this patient represents a case of progressive bulbar palsy with perceptive deafness or Brown-Vialetto-Van Laere (BVVL) syndrome. To our knowledge, this case of BVVL syndrome with severe clinical features and rapid deterioration leading to death is the youngest one reported in the literature. Furthermore, this case emphasizes the need for repeated auditory examinations, including the performance of BAEP in all cases, especially infants and young children with progressive bulbar palsy.

Evidence for intraaxonal spread of Listeria monocytogenes from the periphery to the central nervous system.

Rhombencephalitis due to Listeria monocytogenes is characterized by progressive cranial nerve palsies and subacute inflammation in the brain stem. In this paper, we report observations made on mice infected with L. monocytogenes. Unilateral inoculation of bacteria into facial muscle, or peripheral parts of a cranial nerve, induced clinical and histological signs of mainly ipsilateral rhombencephalitis. Similarly, unilateral inoculation of bacteria into lower leg muscle or peripheral parts of sciatic nerve was followed by lumbar myelitis. In these animals, intraaxonal bacteria were seen in the sciatic nerve and its corresponding nerve roots ipsilateral to the bacterial application site. Development of myelitis was prevented by transsection of the sciatic nerve proximally to the hindleg inoculation site. Altogether, our results support the hypothesis that Listeria rhombencephalitis is caused by intraaxonal bacterial spread from peripheral sites to the central nervous system.

頭蓋底横紋筋肉腫例

A 17-year-old male presented with progressive cranial nerve palsy. CT and MRI revealed a large tumor in the right cranial fossa with bone destruction. Radiation therapy was performed but only a temporary partial remission was obtained. After a 3-month hospitalization he died of respiratory failure due to lung metastasis. Autopsy revealed metastase in the lung, pleura, and adrenal gland. The pathological diagnosis was rhabdomyosarcoma.The difficulty of pathological diagnosis and the importance of early diagnosis and treatment are emphasized.

Idiopathic Hypertrophic Cranial Pachymeningitis

Hypertrophic cranial pachymeningitis is a rare, idiopathic form of granulomatous pachymeningitis. This report describes three cases of hypertrophic cranial pachymeningitis and discusses the clinical, radiographic, and pathological findings in these and other reported cases. These lesions typically cause progressive cranial nerve palsies, headaches, and cerebellar dysfunction. They occur in patients of all age groups; the peak incidence is in the sixth decade. Hypertrophic cranial pachymeningitis is best identified by magnetic resonance imaging. The diagnosis is established by excluding all other granulomatous and infectious diseases. A dural biopsy is essential to confirm the diagnosis. Hypertrophic cranial pachymeningitis is initially responsive to steroid therapy, but in most cases it recurs or progresses despite treatment. Surgical excision of granulomas is occasionally necessary to alleviate a mass effect. The long-term outcome remains uncertain for most patients, but progressive disease is usually fatal owing to cranial neuropathies.