Intracranial Involvement Research Articles

A Typical Extramedullary Haematopoiesis in a JAK2 Mutated Primary Myelofibrosis Patient after a Minor Head Injury

Intracranial extramedullary haematopoiesis (EMH) is very rare. We report the case of EMH with intracranial involvement which developed after trauma in a 73-year-old lady affected by JAK2 mutated myelofibrosis (MF). She was diagnosed with MF in 2005 and treated with low-dose-steroids (prednisolone and oxymethalone) for about 5 years with anexcellent response on full blood count and splenomegaly. In June 2011, she fell from the stairs and sustained minor trauma to the forehead. Six weeks later, the patient had a gradually enlarging and moderately painful lump in the centre of her forehead. A contrast-enhanced CT scan of the head was performed, showing a small fracture in the frontal bone infiltrated and surrounded by a soft tissue mass extending intracranially and invading the skin extracranially (Figures 1 and 2). A metastatic deposit from an occult tumour was suspected. In August 2011, we performed a CT-guided trucut biopsy of the forehead mass. The histology report confirmed extramedullary haematopoiesis according to the patchy expression of myeloid (CD45, CD43, myeloperoxidase) and megakaryocytic markers (CD68, CD42b). We concluded that the trauma caused a small fracture which triggered the bone marrow tissue reaction resulting in production of extramedullary haematopoiesis in the site of the trauma. The patient underwent local radiotherapy resulting in complete resolution of the lesion.

Hemangiopericytoma of neck extending to craniovertebral junction treated by surgery, pre- and postoperative radiotherapy

Background:Hemangiopericytoma (HPC) is a rare tumor of uncertain malignant potential arising from mesenchymal cells with pericytic differentiation. It accounts for 3-5% of soft tissue sarcomas, and 1% of vascular tumors. The treatment of choice is a primary wide surgical resection with adjuvant radiotherapy (RT) reserved for cases of incomplete removal.Case Description:We report a case of a 24-year-old female with a rapidly growing, highly vascular swelling in nape of the neck extending deep into the craniovertebral (CV) junction accompanied by extradural/intraspinal, and intracranial involvement. An incisional biopsy revealed a cellular, highly vascular tumor with HPC-like features. The patient received preoperative RT, which reduced both the size and vascularity of the lesion, facilitating subsequent near complete resection. Further postoperative RT resulted in a good clinical outcome, with no tumor recurrence observed at 2 postoperative years.Conclusion:HPC of the soft tissues of neck accompanied by deep extension to the CV junction is uncommon. A high index of suspicion is required to diagnose these cases. which may be treated with preoperative RT (to reduce the lesion size/vascularity), aggressive surgical resection, followed by postoperative adjunctive radiation treatment as well.

Imaging of brain metastases

Imaging plays a key role in the diagnosis of central nervous system (CNS) metastasis. Imaging is used to detect metastases in patients with known malignancies and new neurological signs or symptoms, as well as to screen for CNS involvement in patients with known cancer. Computed tomography (CT) and magnetic resonance imaging (MRI) are the key imaging modalities used in the diagnosis of brain metastases. In difficult cases, such as newly diagnosed solitary enhancing brain lesions in patients without known malignancy, advanced imaging techniques including proton magnetic resonance spectroscopy (MRS), contrast enhanced magnetic resonance perfusion (MRP), diffusion weighted imaging (DWI), and diffusion tensor imaging (DTI) may aid in arriving at the correct diagnosis. This image-rich review discusses the imaging evaluation of patients with suspected intracranial involvement and malignancy, describes typical imaging findings of parenchymal brain metastasis on CT and MRI, and provides clues to specific histological diagnoses such as the presence of hemorrhage. Additionally, the role of advanced imaging techniques is reviewed, specifically in the context of differentiating metastasis from high-grade glioma and other solitary enhancing brain lesions. Extra-axial CNS involvement by metastases, including pachymeningeal and leptomeningeal metastases is also briefly reviewed.

Paranasal Sinus Osteomas

Osteoma is the most common benign tumor of the paranasal sinuses. They remain asymptomatic until the tumor reaches a certain size. Although the etiology of the osteomas is controversial, embryologic, traumatic, and infective theories have been proposed. Osteomas may be discovered at any age but are usually found during the fourth and fifth decades, and there is a male preponderance. In this retrospective study, we analyzed 25 patients with paranasal sinus osteomas who were treated surgically between 2005 and 2011. Age and sex of the patient, size and location of the tumor, clinical presentation, type of surgical approach, duration of follow-up, outcome, and any intraoperative or postoperative complications were obtained from the medical records of the patients. Diagnosis of osteoma was based on computed tomography findings and confirmed histopathologically in all cases. There were 9 men (36%) and 16 women (64%) with a mean age of 38.7 years. Tumor localization was divided as follows: ethmoid sinus in 14 patients (56%), frontal sinus in 9 patients (36%), and maxillary sinus in 2 patients (8%). Mean tumor size was 2.04 cm, ranging from 0.5 to 6.5 cm. External approach was used in 9 cases and endonasal endoscopic surgery was performed in 19 cases. Combination of endoscopic surgery and external surgery was performed in 3 cases. After at least 6 months of follow-up, all patients were symptom-free except 2 patients who underwent revision surgery. These 2 patients were also asymptomatic following revision surgery. Although there is an increasing trend to endoscopic surgery, external approach is recommended in large frontal osteomas and ethmoid osteomas with lateral extension and in case of intracranial or intraorbital involvement.

Temporal Myositis with Middle Fossa Epidural Abscess as the Complications of Acute Otitis Media

Acute otitis media is an inflammatory process involving the mucoperiosteum of the middle ear cleft. The clinical spectrum can be extended from a self-limiting benign condition to a complicated disease with intracranial involvement. Intracranial and extracranial complications of acute otitis media is still occur in the modern antibiotic era and has potentially serious consequences with high mortality rate. We report a case of acute otitis media with temporal myositis and middle fossa epidural abscess which is rarely seen as the complications from the disease. DOI: http://dx.doi.org/10.3329/bjms.v11i2.8472 Bangladesh Journal of Medical Science Vol. 11 No. 02 April 2012: 126-128

An unusual case of neuro-Behçet’s disease presenting with co-occurence of cerebral venous sinus thrombosis with basilar artery occlusion

Non-parenchymal neuro-Behçet disease generally affects cerebral venous sinuses, whereas intracranial intracerebral arterial involvement has been rarely reported. But co-involvement of both intracranial intracerebral artery and venous vascular systems in a patient at the same time has not been mentioned before. To the best of our knowledge, this case involving a 25-year-old male with a 7-year history of Behçet disease is the first reported of this type of involvement. He developed occlusion of the basilar artery together with thrombosis of the left sigmoid sinus, distal internal jugular vein, and straight sinus. He was successfully treated with a combination of high-dose steroid and cyclophosphamide. Cranial magnetic resonance angiography demonstrated the resolution of these abnormalities.

Monocular Vision Loss and Headache in a 60-Year-Old Liver Transplant Patient

Monocular Vision Loss and Headache in a 60-Year-Old Liver Transplant Patient

Bilateral inverted papilloma of the middle ear with intracranial involvement and malignant transformation: First reported case

Inverted (Schneiderian) papilloma (IP) is a benign but locally aggressive tumor that is typically located in the sinonasal tract. Middle ear involvement and intracranial extension are rare. We present a patient with a history of a completely resected right nasal cavity IP that returned 7 months later with hearing loss, bilateral aural fullness, and right-sided facial weakness. Work-up revealed middle ear IP, and the patient underwent bilateral mastoidectomies. On both sides, the disease caused erosion of the tegmen and was adherent to the underlying dura. There was dehiscence of the carotid canal wall on the left. On the right, the tumor was discovered to have recurred 3 months after initial resection, resulting in complete facial nerve paralysis and trigeminal paresthesias. A right temporal bone resection was undertaken along with neurosurgery. The IP was discovered to have invaded through the dura of the temporal lobe, incase the internal carotid artery, and infiltrate the trigeminal nerve. The facial and vestibulocochlear nerves were sacrificed on the right. Pathology of the right temporal bone revealed malignant transformation to squamous carcinoma. The patient was referred to radiation oncology for postoperative therapy. To our knowledge, this is the first case of bilateral IP of the middle ear with intracranial involvement and malignant transformation. Discussion points include: 1) management of middle ear IP, 2) carotid canal wall dehiscence in erosive middle ear disease, 3) aggressive surgical excision in locally destructive middle ear tumors, and 4) the role of radiation therapy in malignant transformation of IP.

Burkitt's lymphoma: a rare cause of isolated trigeminal neuralgia in a child

A 13-year-old girl presented with the chief complaint of right facial pain of 4 weeks’ duration. The pain was sharp, intermittent on the right cheek, and provoked by eating and speaking without epistaxis or fever. Her medical history was unremarkable without comorbid conditions. On neurologic examination, cranial nerve functions were normal. She had neither cerebellar nor brainstem signs. Her chewing, swallowing, hearing, facial motor and sensory functions were normal. An ophthalmology examination confirmed normal extraocular movements and visual acuity. She underwent a craniofacial computed tomography (CT) scan which demonstrated a right infratemporal process with intracranial involvement through a skull base osteolysis (Fig. 1a). Magnetic resonance imaging (MRI) with gadolinium enhancement revealed enhancement of the temporal dura of the skull base and the convexity (hypertrophic cranial pachymeningitis) with extension to the cavernous sinus on the right side (Fig. 1b–d). Laboratory findings were normal. Transnasal endoscopy showed edematous nasal mucosa and a firm grayish mass occupying the right maxillary sinus and extending into the sphenoethmoidal sinuses. The mass was biopsied. Histology and immunohistochemical staining were consistent with Burkitt’s lymphoma. Staging investigations showed no other localizations. The patient was referred to the oncology department for chemotherapy regimen with an improvement in his maxillary tumor. Despite continued outpatient chemotherapy, she died approximately 3 months after diagnosis. Burkitt lymphoma is a high-grade B cell non-Hodgkin lymphoma. The sporadic form of this malignancy occurs primarily in children aged 10 to 14 years, and often manifests as an abdominal mass; it rarely involves the head and neck [1, 2]. The presence of Burkitt’s lymphoma in the paranasal sinuses is rare. This case is one of the few reported cases of true Burkitt lymphoma originating in the pterygomaxillary area with central skull base destruction and intracranial extension [2]. This serious disease may have rapid progression with minimal neurological symptoms. There are multiple possible explanations for the trigeminal neuralgia in our patient: direct compression and/or infiltration of the nerve with neoplasic cells into the cavernous sinus, in the skull base with bony destruction of the foramen rotundum, or into the pterygopalatine fossa compressing and/or involving the maxillary branch. Prompt diagnosis A. Akhaddar (*) :H. Belfquih :M. Boucetta Department of Neurosurgery, Mohammed V Military Teaching Hospital, University of Mohammed V Souissi, Bloc V2, appt 5, Avenue Kamal Zebdi, Secteur 21, Hay Riyad, 10100 Rabat, Morocco e-mail: akhaddar@hotmail.fr

Posterior circulation ischemic stroke-clinical characteristics, risk factors, and subtypes in a north Indian population: a prospective study.

Posterior circulation stroke accounts for approximately 20% of all strokes with varied clinical presentation, which differ from strokes in anterior circulation, with reference to etiology, clinical features, and prognosis. Short penetrating and circumferential branches in the posterior circulation supply the brain stem, thalamus, cerebellum, occipital, and medial temporal lobes. We prospectively analyzed 80 participants of posterior circulation ischemic stroke from a registry of 944 participants attending a tertiary care referral university hospital. Patients were analyzed for demographics, stroke risk factors, clinical characteristics, neuroimaging, and stroke subtypes. Posterior circulation ischemic stroke accounted for 80 (8.5%) of 944 of all strokes and 80 (10.45%) of 765 of ischemic stroke. Sixty-three were males with mean age 51.7 ± 14.4 years. Twenty-one participants were young (defined as age less than 45 years). Hypertension was found to be the most common risk factor (63.75%). Vertigo was the most common clinical symptom reported in 45 (56.25%) cases. Sixty-eight (85%) patients had large artery disease, 8 (10%) had documented cardioembolic source, 3 (3.75%) small artery disease, and 2 (2.5%) vasculitis. Posterior cerebral artery was most commonly involved. Topographically distal intracranial involvement was most frequent (66.25%) followed by proximal (30%) and middle intracranial territory (3.75%). Our study demonatrated the occurrence of posterior circulation stroke in relatively younger age group compared to the Western world. We also found higher percentage of large artery disease, while cardioembolism as a less frequent cause of posterior circulation ischemic stroke in North Indian population. Distal territory involvement was most common in our study.

Risk Factors Associated With Repair of Orbital and Lateral Skull Defects

To explore the complications and associated risks factors after orbital exenteration and lateral skull base defect repair. Patients who had undergone a reconstruction of their orbital cavity and lateral skull base defects were selected from our departmental database. The outcome of interest was postoperative complications. The risks factors were defined as age, sex, history of radiation therapy, and intracranial involvement (with and without dural involvement). Information was collected on the type of reconstruction used after the orbital cavity repair. The χ2 test and logistic regression were used to analyze associations between postoperative complications and the various risks factors. Of the 32 identified patients, 19 had intracranial involvement (9 with dural involvement). Twenty-four patients underwent reconstruction with free tissue transfer in the same setting. Reconstruction with free tissue transfer was significantly associated with fewer major postoperative complications (P < .053). There was a trend toward more complications with a history of radiation therapy or intracranial involvement. Reconstruction of the orbital cavity and lateral skull base can be challenging, especially if there is a history of radiation therapy and intracranial involvement. Free tissue transfer is a safe and effective method for reconstruction of such defects.

Orbital apex syndrome due to trigeminal perineural spread of sinonasal leprosy: a case report

Leprosy is a granulomatous disease primarily affecting the skin and peripheral nerves caused by Mycobacterium leprae, but also significantly involving sinonasal cavities and cranial nerves. It continues to be a significant public health problem, and despite multidrug therapy, it can still cause significant morbidity. The awareness of cranial nerve, intracranial and orbital apex involvement as in our case is important for appropriate treatment measures.

Management and Treatment of Orbital Fungal Sinusitis with Intracranial Involvement

Fungal sinusitis is being reported more now in the literature than it was previously. Even with increased data and information, the intracranial involvement is still infrequent, but it can be fatal. As such, it is crucial that there is early diagnosis and treatment. Fungal sinusitis can be divided into two main groups, invasive and noninvasive, and the treatment is not the same for both groups. This case report will discuss in detail the presentation of our patient with a careful look at imaging, diagnosis, treatment, and management. It will be shown that the involvement of neurosurgery is critical in cases where there is intracranial extension. Early detection and diagnosis along with medical therapy and surgical techniques, such as endoscopy, have decreased the morbidity and mortality. Prognosis is dependent on the degree of invasiveness of the fungus.

A Case of Intracranial Involvement in Plasma Cell Myeloma

Plasma cell myelomas generally manifest as bone or soft-tissue tumors with variable mass effects, pain, and infiltrative behavior. Extramedullary involvement occurs most commonly in the spleen, liver, lymph nodes, and kidneys, but intracranial involvement in plasma cell myeloma is a rare extramedullary manifestation. These authors recently encountered a case of intracranial involvement of plasma cell myeloma. A 69-year-old man was hospitalized for headache and mental changes. Brain CT showed subdural hemorrhage caused by plasma cell myeloma. Plasma cell myeloma with intracranial involvement has poor prognosis, and the patient in this case died from acute complications, such as subdural hemorrhage. Based on this case report, it is suggested that more effective treatment regimens of plasma cell myeloma with intracranial involvement be developed. Moreover, a screening method and decision on the appropriate time for intracranial involvement are needed for plasma cell myeloma patients.

Spinal glioblastoma multiforme of the conus medullaris with holocordal and intracranial spread in a child: a case report and review of the literature

Spinal glioblastoma multiforme (GBM) is a rare clinical entity. According to our review of the literature, only 15 cases of spinal GBM originating from the conus medullaris (CM) have been reported. Furthermore, there has been no case of spinal GBM originating from the CM with holocordal and intracranial involvements, which were already present at the time of initial diagnosis. Despite a variety of treatments, the previous studies have uniformly reported poor results of this lethal condition. The present report illustrates a 10-year-old girl with spinal GBM with rare involvement pattern, that is, the tumor originating from the CM with the holocordal and intracranial involvements, undergoing a novel chemotherapy regimen. A case report and review of literature. Magnetic resonance (MR) imaging with gadolinium enhancement clearly revealed holocordal and intracranial lesions, which were otherwise unidentifiable by plane MR imaging. Open biopsy was performed. After histologic diagnosis, novel chemotherapy regimen, that is, simultaneous high-dose chemotherapy (cyclophosphamide, cisplatin, vincristine, and etoposide) combined with autologous peripheral blood stem cell transplantation (auto-PBSCT), intrathecal injections of both methotrexate and dexamethasone, and radiotherapy, which respected the tolerance threshold of the spinal cord, were performed. Novel chemotherapy regimen achieved marked tumor regression until the 12th month of treatment. The patient became ambulatory with T-shaped canes and has returned to the school life. Unfortunately, the patient died because of the relapse of the tumor 14 months after the initial diagnosis; however, this strategy has achieved longer survival than previously reported mean survival (12 months). The authors advocate enhanced MR imaging of the whole central nervous system for the potential spreading of this disease. This is the first report of simultaneous high-dose chemotherapy combined with auto-PBSCT, intrathecal injections of antineoplastic agents, and radiotherapy for the treatment of spinal GBM, which achieved marked tumor regression. We believe that accumulated experiences in the treatment of this lethal condition might contribute well to improve its therapeutic outcome.

Nasopharyngeal adenoid cystic carcinoma: magnetic resonance imaging features in ten cases

Nasopharyngeal adenoid cystic carcinoma (NACC) is a rare malignancy with high local invasiveness. To date, there is no consensus on the imaging characteristics of NACC. To address this, we retrospectively reviewed 10 cases of NACC and summarized the magnetic resonance imaging (MRI) features. MR images of 10 patients with histologically validated NACC were reviewed by two experienced radiologists. The location, shape, margin, signal intensity, lesion texture, contrast enhancement patterns, local invasion, and cervical lymphadenopathy of all tumors were evaluated. Clinical and pathologic records were also reviewed. No patients were positive for antibodies against Epstein-Barr virus (EBV). The imaging patterns of primary tumors were classified into two types as determined by location, shape, and margin. Of all patients, 7 had tumors with a type 1 imaging pattern and 3 had tumors with a type 2 imaging pattern. The 4 tubular NACCs were all homogeneous tumors, whereas 3 (60%) of 5 cribriform NACCs and the sole solid NACC were heterogeneous tumors with separations or central necrosis on MR images. Five patients had perineural infiltration and intracranial involvement, and only 2 had cervical lymphadenopathy. Based on these results, we conclude that NACC is a local, aggressive neoplasm that is often negative for EBV infection and associated with a low incidence of cervical lymphadenopathy. Furthermore, MRI features of NACC vary in locations and histological subtypes.

Paraganglioma with intracranial metastasis: a case report and review of the literature

Paragangliomas are rare neuroendocrine tumors of neural crest origin. They are mostly benign, however; malignant tumors with aggressive behavior and distant metastasis can also occur. Intracranial involvement is extremely rare and has been sporadically reported in the literature. Here we report a case who presented with progressive neurologic deficits due to multiple intracranial lesions found to be metastasis from an occult retroperitoneal malignant paraganglioma.

Risk Factors Associated With Repair of Orbital and Lateral Skull Defects

To explore the complications and associated risks factors after orbital exenteration and lateral skull base defect repair. Patients who had undergone a reconstruction of their orbital cavity and lateral skull base defects were selected from our departmental database. The outcome of interest was postoperative complications. The risks factors were defined as age, sex, history of radiation therapy, and intracranial involvement (with and without dural involvement). Information was collected on the type of reconstruction used after the orbital cavity repair. The χ(2) test and logistic regression were used to analyze associations between postoperative complications and the various risks factors. Of the 32 identified patients, 19 had intracranial involvement (9 with dural involvement). Twenty-four patients underwent reconstruction with free tissue transfer in the same setting. Reconstruction with free tissue transfer was significantly associated with fewer major postoperative complications (P < .053). There was a trend toward more complications with a history of radiation therapy or intracranial involvement. Reconstruction of the orbital cavity and lateral skull base can be challenging, especially if there is a history of radiation therapy and intracranial involvement. Free tissue transfer is a safe and effective method for reconstruction of such defects.

Determinants of Neurologic Deterioration and Stroke-Free Survival After Spontaneous Cervicocranial Dissections: A Multicenter Study

Patients with spontaneous cervicocranial dissection (SCCD) may experience new or recurrent ischemic events despite antiplatelet or anticoagulant therapy. Treatment with stent placement is an available option; however, the literature on patient selection is limited. Thus, identifying patients at high risk for neurologic deterioration after SCCD is of critical importance. The present study examined the rate of neurologic deterioration in medically treated patients with SCCD and evaluated demographic, clinical, and radiologic factors affecting this deterioration. We retrospectively identified consecutive patients with SCCD over a 7-year period from 3 medical institutions, and evaluated the relationships between demographic data, clinical characteristics, and angiographical findings and subsequent neurologic outcomes. Neurologic deterioration was defined as transient ischemic attack (TIA), ischemic stroke, or death occurring during hospitalization or within 1 year of diagnosis. Kaplan-Meier curves were used to determine neurologic event-free survival up to 12 months. A total of 69 patients (mean age, 47.8 ± 14 years; 45 males) with SCCD were included in the study. Eleven patients (16%) experienced in-hospital neurologic deterioration (TIA in 9, ischemic stroke in 1) or death (1 patient). An additional 8 patients developed neurologic deterioration within 1 year after discharge (TIA in 5, ischemic stroke in 2, and death in 1). The overall 1-year event-free survival rate was 72%. Women (P = .046), patients with involvement of both vertebral arteries (P = .02), and those with intracranial arterial involvement (P = .018) had significantly higher rates of neurologic deterioration. Our findings indicate that neurologic deterioration is relatively common after SCCD despite medical treatment in women, patients with bilateral vertebral artery involvement, and those with intracranial vessel involvement.

Clinical Results of Radiotherapy for Primary Intraocular Non-Hodgkin's Lymphoma

Clinical Results of Radiotherapy for Primary Intraocular Non-Hodgkin's Lymphoma