Abrupt foramen magnum syndrome due to shunt malfunction in a previously asymptomatic chiari I malformation: the hidden predictable risk in long-lasting shunted patients.

Cerebrospinal fluid overdrainage in long-lasting shunted hydrocephalus is a well recognized complication. It may lead to different clinical syndromes which may occur over time, namely cranioencephalic disproportion and hindbrain herniation. Among the latter, Chiari malformation type 1 classically refers to one of the mildest form. When symptomatic, the patients generally exhibit a chronic, slowly progressive disease course. Although well documented in Chiari type II malformation and hydrocephalus, ventriculo-peritoneal shunt malfunction causing acute neurological deterioration in acquired Chiari malformation type 1 has been described only in three cases. All these patients underwent urgent shunt revision and suboccipital decompression in two of them, achieving good clinical improvement. We report on a 20-year-old woman harboring a previously asymptomatic Chiari malformation type 1, who experienced an acute foramen magnum syndrome with ventriculo-peritoneal shunt malfunction. We performed an endoscopic third-ventriculo-cisternostomy and definite removal of the shunt, obtaining the complete resolution of symptoms and shunt independence at 2 years follow-up. The phisiopathogenetic mechanisms and surgical management are discussed.

Isolated intracranial myeloid sarcoma: report of a case and review of the literature.

Myeloid sarcoma is a rare malignant tumor of primitive myeloid cell origin often associated with hematologic disorders. The central nervous system is rarely involved and differentiating between myeloid sarcoma and other tumors is not possible on imaging. Here we present the rare case of an isolated intracranial myeloid sarcoma, initially misdiagnosed radiologically as a meningioma, treated with surgical total resection and subsequent chemotherapy, with no signs of any hematological disorder at follow up. Differential diagnosis and management strategies, as well as follow-up implications are discussed along with literature review, which pointed out that only five cases with no further signs of hematological disorders at follow up have been described in the literature so far and this case has the longest follow up of them at 9 years.

Late Intramedullary Spinal Metastases from Esthesioneuroblastoma: Case Report and Prognostic Implications.

BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon neuroectodermal tumor that originates from the olfactory mucosa and often recurs locally. Distant metastases of ENB have been described, but there are few reports of intramedullary metastases to the spinal cord. CASE DESCRIPTION: Here we report a case of a patient presenting with a progressive paraparesis and magnetic resonance imaging findings of multiple drop metastases to thoracic and lumbar regions of the spinal cord, 17 years after diagnosis and treatment for an intracranial ENB with subsequent neck nodal metastases. The dorsal symptomatic lesion was treated with resection, radiotherapy, and adjuvant chemotherapy. The implications of spinal metastases for the clinical prognosis of this disease are discussed, with a review of the few reported cases of spinal ENB metastases in the literature. CONCLUSIONS: Through the presentation of this case we hope to further contribute to a better understanding of this rare disease's prognosis.

Current management of central nervous system metastasis from cholangiocarcinoma: the neurosurgical perspective. Literature review.

Purpose: Central nervous system (CNS) metastasis from cholangiocarcinoma (CCA) are extremely rare and associated prognosis is poor. The involvement of the CNS by metastatic CCA may discourage any further treatment; however, data from the literature are discordant, due to recent reports of exceptionally long follow-up after surgical resection of a brain metastasis.Material and Methods: Electronic databases, such as PubMed/MEDLINE and Google Scholar, were analyzed for studies published up to October 2018 using the search term "cholangiocarcinoma and central nervous system metastasis or brain metastasis".Results: We found a total of 18 studies cited in the literature of the 30 year span analyzed, and we added a new case we treated at our Institution, reaching a series of 32 patients. Among these, 7 patients had leptomeningeal dissemination and 25 presented solid CNS metastasis. We analyzed the treatment options and the outcomes, addressing also histopathological insights on tumoral markers possibly involved in the mechanism of metastases of cholangiocarcinomasConclusions: According to the literature data, the outcome remains poor, particularly for those with leptomeningeal diffusion. Nevertheless, long term follow-up is reported in case of surgical resection of CNS metastasis, when there is a good control of the primary tumor. Actually, the majority of patients are often in advanced state of disease at diagnosis and not suitable for initial resective procedure; in these cases neo adjuvant and adjuvant therapies have provided a slight improvement of the outcome.

Chylous fistula: management of a rare complication following right anterior cervical spine approach.

PURPOSE: Chylorrhea resulting from injury of the lymphatic system during neck dissection is a well-known complication. It is an uncommon occurrence in spinal surgery, and only one case after right anterior cervical spine surgery has been described so far. Despite its rarity, chylous leakage deserves a particular attention since it may become a serious and occasionally fatal complication if not detected early and managed appropriately. METHODS: We report the case of a 42-year-old man who underwent a standard anterior cervical discectomy and fusion according to Cloward approach for a C6-C7 disk herniation. The patient developed a delayed prevertebral chyle collection on postoperative day 5, presenting with mild breathing and swallowing difficulties. RESULTS: He was managed with conservative care, including bed rest, low-fat diet and drainage pouch positioning, which led to the complete resolution of the fluid collection. CONCLUSIONS: Knowledge of the normal anatomy of the lymphatic system and of its variations is essential when planning an anterior spinal procedure, and represents the first measure to be adopted in order to avoid such complication. The prompt identification of a postoperative chylous fistula and the applicability of an individually based management's protocol may help in the majority of the cases to reduce the potential morbidity, without significant long-term effects.

Supratentorial Embryonal Tumors in the Elderly: Diagnostic Pitfalls and Clinical Prognosis.

Supratentorial embryonal tumors are very rare malignant tumors of neuroectodermal origin, characterized by an aggressive clinical behavior. They occur prevalently in children. They have been sporadically described in adults and represent an even rarer occurrence in elderly patients, raising many issues on the diagnostic pitfalls and their appropriate management. We present an unusual case of embryonal tumor in a 62-year-old man who presented with speech disorder, and partial deficit of the left 3rd and 5th cranial nerves secondary to a left temporo-insular embryonal tumor: the clinico-radiological features, histopathological insights, therapeutic options and results are discussed along with a review of the most relevant literature, addressing the specific issue of differential diagnosis and the expected results in the elderly population.

Repeat Bleeding 18 Years After Hemorrhagic Pilocytic Astrocytoma: Prognostic Implications of Conservative Management-Case Report and Literature Review.

BACKGROUND: Acute hemorrhagic occurrence of pilocytic astrocytomas is extremely rare but has become increasingly recognized and often presents in clinically emergent situations. The precise significance of hemorrhage in these tumors and its pathogenetic mechanisms have not yet been defined. Nothing is known about the natural history of bleeding pilocytic astrocytomas. CASE DESCRIPTION: A 21-year-old man presented with acute biventricular hydrocephalus for repeat bleeding of a midbrain lesion that had been diagnosed 18 years earlier after a similar acute hemorrhagic episode. The patient had been initially treated conservatively, considering the high risk of surgery. He underwent urgent biventricular external shunt surgery and, subsequently, total removal of the lesion. Histopathological analysis revealed pilocytic astrocytoma. Including the present case, 59 cases were analyzed and reviewed, including 5 cases with a pilomyxoid variant. Different pathogenetic mechanisms have been proposed, including histological characteristics, rupture of the bridging veins torn by the neoplastic mass, and vascular instability determined by overexpression of vascular endothelial growth factor. CONCLUSIONS: To the best of our knowledge, the presented case is completely unique owing to the long-term observational follow-up data available. The hemorrhagic potential did not decrease over time, representing a specific feature of this tumor. The occurrence of bleeding might not reflect either malignancy or aggressiveness. However, the worse prognosis is related to the clinical behavior, with life-threatening complications often associated. Surgery remains the standard treatment and will influence the final prognosis.

Hypertrophic Pachymeningitis and Hydrocephalus-The Role of Neuroendoscopy: Case Report and Review of the Literature.

BACKGROUND: Hypertrophic pachymeningitis (HP) is a rare primary or secondary inflammatory disorder that manifests with thickening of dura mater involving predominantly the tentorium and falx in the focal form. Hydrocephalus has not been reported in association with secondary HP. CASE DESCRIPTION: A 61-year-old woman presented with intracranial HP of the posterior fossa secondary to Wegener granulomatosis not responsive to corticosteroid therapy. Owing to the association of noncommunicating hydrocephalus, endoscopic third ventriculostomy was performed. There was immediate improvement of neurologic symptoms and no postoperative complications. The patient had very good clinical and radiologic outcome at 1-year follow-up. CONCLUSIONS: In a case of noncommunicating hydrocephalus related to focal HP of the posterior fossa, endoscopic third ventriculostomy was effective in resolving symptoms related to increased intracranial pressure. The physiopathogenetic mechanisms and therapeutic strategies were discussed along with a review of the most relevant literature.

Brain Granuloma: Rare Complication of a Retained Catheter.

BACKGROUND: Ventriculoperitoneal (VP) shunt is the most frequent treatment for hydrocephalus. VP shunt malfunction is a very common problem in neurosurgical practice, often requiring shunt revision procedures. In some cases, complete removal of a VP shunt may present difficulties, leading the surgeon to leave the ventricular catheter in situ. This decision is often made to avoid possible adverse events, primarily risk of life-threatening hemorrhage. However, a nonfunctioning catheter left in the ventricular system may lead to further complications. CASE DESCRIPTION: We report the case of an adult man who presented with a tumor-like cystic lesion, with mural and nodular postcontrast enhancement, caused by a huge granuloma formed around the tip of a retained ventricular catheter. CONCLUSIONS: This occurrence is extremely rare, and this is the first reported case in the literature. Preoperative differential diagnosis was challenging and included infection, metastasis, dysembryogenetic lesions, and rare reversible porencephalic cysts. According to several physiopathogenetic theories recently reported in the literature, the granuloma may develop as the result of persistent chronic inflammatory reactions between the ventricular catheter left in situ and the brain parenchyma. Application of neuroendoscopic techniques is improving management of VP shunt revisions, allowing safe removal of catheters stuck or lost in the ventricular system. Neuroendoscopy may represent an additional option to avoid possible complications related to retained ventricular catheters.

Prolactin Secreting Pituitary Microadenoma: Results of Transsphenoidal Surgery After Medical Therapy With Dopamine Agonist.

Medical therapy with dopamine agonist is very effective in controlling prolactin serum levels and it usually represents the first therapeutic choice for prolactin secreting pituitary adenomas. However, many patients present increase of prolactinemia after withdrawal of medical therapy which consequently should be taken for long time; other present intolerance to medical therapy; women with pregnancy need to withdraw dopamine agonists with consequent potential related problems: in these patients transsphenoidal surgery can be requested. The presented study concerns the efficacy of transsphenoidal surgery in patients affected by microprolactinoma after treatment with medical therapy for different periods of time. Different postsurgical results were achieved in 2 groups of 24 (group 1) and 25 (group 2) patients affected by microprolactinoma who had taken medical therapy for a period of time respectively longer or shorter than 1 year. In summary, the authors observed in group 1 a rate of hormonal remission of 33.3% and an overall prolactinemia improvement with possibility of medical therapy withdrawal in 49.9% of patients. In group 2, the authors observed a rate of hormonal remission of 84% and an overall rate of improvement with no necessity of medical therapy of 92%. Therefore, the authors' experience showed that the surgical option for the therapy of microprolactinomas should be indicated within 1 year from the beginning of medical therapy with dopamine agonist, to achieve a high rate of hormonal remission and possibility to withdraw medical therapy. The authors' protocol for microprolactinoma treatment is presented.

Isolated Oculomotor Nerve Palsy as Presenting Symptom of Bilateral Chronic Subdural Hematomas: Two Consecutive Case Report and Review of the Literature.

BACKGROUND: Isolated oculomotor nerve palsy (ONP) is caused most commonly by vascular disease, posterior circulation aneurysms, and inflammatory or traumatic injury. ONP usually occurs in chronic subdural hematoma (CSDH) as a common sign of cerebral herniation that typically is associated with a deterioration of consciousness. CASE DESCRIPTION: We report 2 cases of bilateral CSDH who presented with ONP without deterioration of consciousness. An extensive literature review revealed this is an extremely rare finding. We also investigated all the possible pathogenic mechanisms producing nerve impairment and found a strong association with bilateral subdural hematoma. Vascular compression between posterior circulation arteries and tentorial edge abnormalities also could be involved. Vulnerability of the oculomotor nerve seems to be a necessary condition leading to clinical onset and is caused by predisposing factors to nerve damage, including vascular disease, head trauma, or herpes zoster infection. CONCLUSIONS: Although isolated ONP is a very rare presentation of CSDH, a differential diagnosis is absolutely necessary, because surgical treatment allows good recovery of third nerve palsy in most of the cases.

Paraganglioma of the cauda equina region.

BACKGROUND CONTEXT: Cauda equina paragangliomas (CEPs) are rare neuroendocrine tumors. The difficulty in differential diagnosis with other tumors of this region may be misleading for surgical planning and prognostic expectations. PURPOSE: To report on a rare case of CEP and review the most current information regarding the diagnosis, treatment options, and outcomes. STUDY DESIGN: Case report and literature review. PATIENT SAMPLE: One patient affected by CEP. METHODS: We report on a 33-year-old woman with a 2-month history of worsening low back pain, aggravated by sitting, bending, and coughing. Neurological examination revealed normal power and muscular tone, no sensory or sphincter abnormality, and normal reflex. Magnetic resonance imaging of the lumbar spine demonstrated an intradural extramedullary lesion at L3, with homogeneous contrast enhancement and hypointense punctate foci. The patient underwent an L3 laminectomy and tumor removal. Relevant articles covering CEPs from 1970 to the present were reviewed. RESULTS: The histopathological examinations described paraganglioma features. The postoperative course was uneventful, and all the symptoms resolved, with no tumor recurrence after 3 years' follow-up. CONCLUSIONS: Cauda equina paragangliomas are rare, benign, and slow-growing tumors. Except for its secreting tumor characteristics, preoperative CEP diagnosis is very difficult. Magnetic resonance imaging is important and may suggest specific radiological features for these tumors; however, these are only relative, and it is rare that diagnosis is made before surgery. Diagnosis is established by histological examination and electron microscopy, and immunohistochemical techniques must be used to achieve a correct diagnosis. Cauda equina paragangliomas are well-encapsulated tumors that may be cured by surgery alone, whereas radiotherapy is reserved for incompletely resected tumors. Overall, prolonged postoperative observation is mandatory because of the slow tumor evolution and the possibility of tumor relapse even up to 30 years after surgery.

Lobar hemorrhages.

The lobar localization of a spontaneous intracerebral hemorrhage occurs in one third of all cases; the most frequent cause is represented by amyloid angiopathy. The clinical symptomatology depends on the dimensions of the hematic collection and of the lobe. The diagnosis is made by CT, but also MRI must performed in order to exclude any vascular malformation or neoplasms, and in young patients a cerebral angiography must be done in order to exclude small arteriovenous or dural malformations.

Occipital arteriovenous malformations and migraine.

BACKGROUND: Headache has been reported to be the first clinical presentation in several patients with cerebral arteriovenous malformations (AVMs). Headache associated with AVMs often shows characteristics of migraine with and without aura. Angiographic characteristics of AVMs, such as their location, could determine the 'migraine-like' features of attacks. METHODS: We performed an observational study of the clinical and angiographic characteristics of a cohort of 40 consecutive patients with AVMs who had been admitted to our institute for endovascular embolization over a 4-year period. Headache was characterized according to ICHD-II criteria. The relationship between headaches and the angioarchitectural features of AVMs was also analysed. RESULTS: Migraine-like headache was the first clinical manifestation in 22.5% of patients. The location of the malformation was significantly associated with migraine-like presentation (p=0.03) and the occipital lobe was the predominant site. CONCLUSIONS: An occipital location may be linked with spreading depression, a pathogenic mechanism of migraine. Headache associated with arteriovenous malformations in the occipital lobe, although secondary in nature, could have clinical features similar to migraine.

An unusual growth of an intraventricular meningioma: a case report.

Intraventricular meningiomas are rare often histologically benign tumors arising most always from the trigonal region of the lateral ventricle. We report the first described case of a rapidly growing histologically benign intraventricular meningioma in a 68-year-old woman whose magnetic resonance imaging (MRI) executed 1 year before surgical operation was negative for intracranial mass lesion.

Pseudoglandular schwannoma of the cauda equina. Case report.

The authors present a case of pseudoglandular schwannoma with immunohistochemical findings consistent with epithelial metaplasia. Pseudoglandular schwannoma is a rare morphological variant of benign schwannoma characterized by the presence of glandlike structures lined with Schwann cells. To the best of the authors' knowledge, this is only the fifth case of pseudoglandular schwannoma reported in the literature. Clinical, imaging, and pathological findings are described. The pathological findings were consistent with a pseudoglandular schwannoma composed of typical Schwann cells arranged in an Antoni B pattern, with numerous large pseudocystic spaces. Serial immunohistochemical studies of tissue sections revealed that the cells lining the pseudoglandular spaces were not only diffusely reactive for S100 protein, but also demonstrated focal positivity for epithelial membrane antigen and cytokeratins AE and AE3. The particular immunohistochemical features of incompletely differentiated Schwann cells in the present case give support to the metaplastic theory of the origin of glandlike structures in benign peripheral nerve sheath tumors.

Low-grade primary meningeal lymphoma: case report and review of the literature.

Primary meningeal lymphoma is a rare occurrence. We present a case of an immunocompetent patient operated on for a fronto-parietal lesion similar to a meningioma, which the histological examination diagnosed as a mucosa-associated lymphoid tissue (MALT)-type lymphoma. She received no further post-operative treatment and after 36 months showed no evidence of disease. In a review of the literature, we identified 14 similar cases of MALT lymphoma pre-operatively diagnosed as meningioma. Recognition of this rare meningeal location of a lymphoma involving the central nervous system is useful for a proper diagnosis and adequate treatment.

Spinal epidural abscess complicating chemotherapy in patients with leukemia.

The authors reviewed the basic facts about infections that complicate chemotherapy, and found that a difference exists between those that are medically treated, for which several standard protocols have been adopted, and infections amenable to surgical treatment, such as spinal epidural abscess, which are managed according to the experience at individual institutions. The authors believe that patients with leukemia who manifest a spinal epidural abscess should always be surgically treated when the infection occurs between the induction and remission phases, whereas medical treatment options can be considered for spinal epidural abscesses occurring at the end of the chemotherapy course.