Intracranial inflammatory pseudotumour related to IgG4: A very rare case.

Background: Intracranial inflammatory pseudotumours (IPT) are rare entities that frequently lead to misdiagnosis with malignant lesions. The identification of these lesions is difficult, but important to avoid inadvertent iatrogenicity and to adjust therapeutic protocols. Case Presentation: We report the case of a 30-year-old man who presented a single tonic-clonic seizure. Brain imaging showed a right frontal lesion with intra and extra axial components. Facing the radiologic presentation, a brain tumor was suspected, thus the patient underwent surgery. Pathological exam concluded to a plasma cell granuloma. A whole-body CT-scan showed only a thoracic aortitis. Complete blood work studies came back negative. The patient was also tested for an array of antibodies among which antinuclear antibodies were positive (blood level superior to 1/100). CSF evaluation revealed clear fluid with normal glucose concentration, normal protein levels and lymphocytic pleocytosis. Finally, IgG-4 plasma levels were elevated which led to the diagnosis of an IgG4-RD. The patient was put under prednisolone with a favorable outcome. Conclusion: IPT have several etiologies, among which IgG4 related disease may be one of the less known as only 2 cases have previously been reported. Herein, we report a new case of a young man who presented for seizures related to an intracranial lesion of an IgG4 related disease. The challenge is to suspect such conditions to avoid unnecessary surgeries.

Paediatric Rhabdoid Meningioma: Clinical and Therapeutic Features Findings - Case Series of 3 Patients.

INTRODUCTION: Paediatric rhabdoid meningioma (RM) is the rarest but most aggressive subtype of meningioma, related to a severe prognosis. They account for 1-3% of all intracranial meningiomas. CASE PRESENTATIONS: We report an institutional experience of 3 cases through which we discuss clinical, histological, and therapeutic features of this tumour. Two of our patients were female-gendered (3 years old and 1 year and 6 months old), and one was male-gendered (16 years old). Revealing symptoms were related to intracranial hypertension, cerebellar syndrome, cranial nerve palsy, and skull tumefaction. Imaging showed extra-axial tumour located in the right ponto-cerebellar angle in the first case, in the left occipital region in the second case, left parietal tumour in the third case. All patients underwent a surgical intervention with a gross total resection. Histological evaluation supported by immunohistochemistry confirmed the diagnosis of RM. Tumour recurrence was observed at 45 days in the first case with a fatal outcome. Despite adjuvant radiotherapy, both second and third cases had local recurrence after a mean follow-up of 1 month following the radiotherapy. CONCLUSIONS: RM is very aggressive tumours. Standardized therapeutic guidelines are still under debate as actual approaches are still inefficient to prevent quick recurrence and fatal outcome.

Pediatric head injuries: particularities and neurosurgical experience in a lower-middle income country.

PURPOSE: To the best of our knowledge, this is the first study conducted in Tunisia on the neurosurgical management of child cranial trauma. The objectives of the present work were to identify the causes of pediatric head injuries, explore epidemiological and clinical specificities, and analyze the short- and long-term postoperative evolution. METHODS: A retrospective review was conducted on one hundred children with head injuries over a five-year period at one of the largest neurosurgery departments in Tunisia. The collected data encompassed demographic information, clinical presentation features, neuroimaging characteristics, surgical management, complications, and outcomes. RESULTS: Over a five-year period, we have found 118 children who have undergone surgery, representing an annual incidence of twenty-four children per year. The average age was 10 years. Falls emerged as the primary cause of childhood head injuries in our series, followed by road traffic accidents. The most frequently encountered initial sign was the loss of consciousness (52%), followed by headaches (28%), vomiting (25%), and seizures (8%). The average time between the accident and admission to the operating unit was 10 h. Various neurosurgeries were performed, with the evacuation of an epidural hematoma being the most common procedure. At a median follow-up of 24 months, the outcomes were favorable in 88% of cases. CONCLUSION: The main prognostic factors for head trauma in children included age, circumstances of the accident, association with polytrauma, the initial Glasgow Coma Scale, the nature of the initial cerebral lesions, and the timeliness and quality of initial management.

Lumbar spine epidural meningioma: report of a rare case.

INTRODUCTION AND IMPORTANCE: Spinal meningiomas are typically intradural lesions. Some may infiltrate the dura mater, thus exhibit direct extradural extension. Pure spinal epidural meningiomas are very rare. Here we present a 64 year-old-male with a purely extradural meningioma, and reviewed 15 previously reported cases from the literature. CASE PRESENTATION AND CLINICAL DISCUSSION: A 64-year-old male presented with a progressive cauda equine syndrome. When Lumbar spine MRI showed two extradural lesions regarding the L3-L4 level, one was fully removed (i.e., the posterolateral lesion), while the other anterior lesion was left alone (i.e., to avoid potential neurologic sequelae). Pathologically, the lesion was a benign meningioma. CONCLUSIONS: Spinal epidural meningiomas are rare and should optimally be fully excised at the index surgery.

Epiduritis related to IgG4 disease: A very rare cause for spinal cord compression.

Background: Inflammatory pseudotumors are rare, and those attributed to immunoglobulin G4 (IgG4) diseases are even less frequently encountered. Here, we reviewed 41 cases from the literature of spinal inflammatory pseudotumors due to IgG4 and have added our single new case. Case Presentation: A 25-year-old male presented with progressive back pain, bilateral paraparesis, and sphincter dysfunction. His deficit was attributed to MR-documented posterolateral lesion between the T5 and T10 levels for which he levels underwent a T1-T1010 laminectomy. The pathology revealed an immunoglobulin G4-related inflammatory pseudotumor. Postoperatively, the patient additionally required systemic and epidural administration of glucocorticoids. Conclusion: IgG4-related disease is an emerging clinical condition that rarely involves the central nervous system. Spinal inflammatory pseudotumors, including IgG4 disease, should be more commonly considered among the potential differential diagnoses of lesions compressing the spinal cord.

A Spectacular Penetrating Craniocerebral Trauma From a Rake: A Case Report.

Penetrating intracranial foreign bodies are rare and have a high potential for death or serious morbidity. Their surgical management is complicated and challenging. Herein, we present the case of a 30-years-old man who was a victim of aggression from a rake blow to the head. The rake's teeth were embedded in his cranium, crossing the midline. The surgery was delicate and had to be well studied because two of the rake's teeth plunged into the superior sagittal sinus (SSS). The patient recovered a perfect state of consciousness and was discharged with squealer right upper limb monoparesia. Penetrating intracranial foreign bodies are rare. Few studies have reported on the removal of such foreign bodies, particularly in cases where the foreign body is close to or penetrates the SSS. They cause spectacular cranial trauma and a greater volume of the object. The aim of surgery is to remove the object without worsening the parenchymal and vascular cerebral lesions. This maneuver was delicate. A craniectomy is recommended around the penetrating object before it is carefully extracted with or without opening the dura mater.

Aseptic ascites and chemical meningitis following ventriculoperitoneal shunting for craniopharyngioma: A first case report.

We report the case of a 4-year-old boy who had a ventriculoperitoneal shunt due to hydrocephalus related to a cystic craniopharyngioma. Postoperative, he presented abdominal distension and meningismus. Imaging showed regression of the tumor. The cyst was drained by the shunt. No similar situations were reported in the literature.

Exploring Rare Traumatic Injuries: A Miniseries of 4 Cases Discussing Epidural Hematomas Bridging the Infratentorial and Supratentorial Regions.

Supratentorial-infratentorial epidural hematomas (SIEH) are a rare occurrence following traumatic head injuries, representing only 2% of traumatic epidural hematomas. Given the unique anatomical characteristics of the infratentorial region, mainly its small size, surgical intervention is commonly undertaken to alleviate the pressure on the posterior fossa components. Consequently, there is ongoing debate surrounding the optimal surgical approaches.In this report, we present four cases of SIEH that were treated surgically. Furthermore, we conduct a comprehensive review of existing literature, encompassing clinical, radiological, and therapeutic aspects associated with this condition.SIEH are uncommon post-traumatic lesions that require urgent and individualized management on a case-by-case basis, as guided by multiplanar cerebral computed tomography scan findings. Preoperative planning is essential; however, intraoperative exploration and identification of transverse sinus and torcula lesions are crucial for optimal patient care. The surgical approach may be modified intraoperatively based on the nature and extent of these lesions. In all cases, prompt hematoma evacuation and meticulous hemostasis are the two primary objectives of this surgery.

Vertex Epidural Hematomas: Discussion of a Rare Traumatic Injury Through a Mini Series of 3 Cases.

Vertex epidural hematomas are very uncommon complications of traumatic head injury. Besides the volume of the epidural bleeding, compression of the superior sagittal sinus may be source for added elevated intracranial pressure. Clinical presentation of such lesions is heterogenous and symptoms can develop in an acute to a chronic frame. Radiological diagnosis can sometimes be challenging. Due to its rarity, such lesions have been only reported on case reports and small series and the management remain controversial. Hereby we report 3 cases of surgically managed post traumatic acute epidural hematomas of the vertex. Wen also went through a literature-based discussion of clinical, radiological and therapeutic features related to this condition.

Asymmetric Bilateral Traumatic Epidural Hematoma: A Report of a Rare Traumatic Lesion.

Bilateral epidural hematoma is a rare presentation in head trauma injuries, accounting for only 1%-2% of all epidural hematomas, but with a higher mortality rate than the unilateral form. Herein, we report the case of a 27-year-old man admitted to our department following a road traffic accident. On admission,his Glasgow Coma Scale (GCS) score was 13/15. After a few minutes, he became comatose (GCS 6/15) with right anisocoria. CT scan revealed a bilateral asymmetric epidural hematoma with a left extralabyrinthic linear fracture. Surgical evacuation was subsequently performed, starting with the voluminous right hematoma. The patient was discharged on the 23rd postoperative day with a right third-nerve palsy. Conclusions: In this case report, we discuss the etiology, mechanism, and management of bilateral epidural hematoma. Early diagnosis and a judicious surgical approach for bilateral epidural hematoma are necessary to minimize mortality and morbidity. Prevention is key to reducing traumatic brain injuries.

Hydrocephalus associated to posterior fossa tumor-like sarcoidosis: A case report and literature review.

Background: Intracranial inflammatory pseudotumors (IIPT) are one of the differential diagnosis for the central nervous system (CNS) tumors. They represent a rare condition that may mimic clinically and radiologically intracranial tumors and induce their complications. Among their etiologies, neurosarcoidosis is one of the less known and less frequent. To the best of our knowledge, only two cases of posterior fossa IIPT have been reported in the literature. We present here the 3rd case related to a neurosarcoidosis. Case presentation: We report the case of a 55-year-old female patient who presented with an altered state of consciousness associated to severe intracranial hypertension syndrome for four months. Glasgow coma scale on admission was 14/15. Brain imaging revealed bilateral cerebellar micronodular meningeal enhancement regarding the mesencephalon and the pons, as well as a nodular lesion of the 4th ventricle causing a triventricular acute hydrocephalus. The patient had a ventriculo-peritoneal shunt with a favorable outcome. Afterwards, she underwent a salivary gland biopsy which confirmed the diagnosis of neurosarcoidosis. Conclusion: Posterior fossa IIPT are very rare, mainly when located in the posterior fossa, leading to confusion with other pathologies. MRI has an important role in the diagnosis of these lesions, and the determination of their etiology. It shows other than the IIPT itself, many other signs such as leptomeningeal enhancement, nodular lesions or pituitary stalk thickening. These signs can orientate towards the diagnosis. Treatment may associate to symptomatic approach, corticosteroids. Surgical resection may be proposed when the diagnosis remains doubtful.

Polyuria and polydipsia revealing a pituitary metastasis: Report of a rare case.

Sellar region is a rare localization for intracranial metastases. The diagnosis is frequently delayed as symptoms are generally non specific. Radiologic diagnosis may be difficult as these tumours present similiraities to other more frequent sellar tumours. There is still no consensus regarding therapeutic approach. Prognosis is related to several features.

Cerbellar medulloblastoma in the elderly: Case report and review of the literature.

INTRODUCTION: Medulloblastoma is a malignant tumor of the posterior fossa which is mainly found in children aged less than 15 years of age. This tumor is uncommon in adults, especially those above 40 years old. We report the very rare case of a 61 years-old woman who was diagnosed with a cerebellar medulloblastoma. CASE PRESENTATION: We present a case of a 61-year-old woman who presented for signs of elevated intracranial and cerebellar syndrome related to an atypical mass located within the left cerebellar hemisphere. The patient underwent an incomplete resection of the mass due to the infiltration of the superior cerebellar peduncle. Histology and immunohistochemistry concluded to a classic medulloblastoma. Thus, she underwent postoperative radiotherapy. DISCUSSION: Medulloblastoma is very rarely diagnosed in patients above 60 years, as only 14 cases have already been published. Therapeutic approach is based on surgery and radiotherapy in both children and adults, whereas addition of chemotherapy is controversial. CONCLUSIONS: Even in adults over 60 years of age, medulloblastoma should be included in the differential diagnosis of a cerebellar mass. This diagnosis should be kept in mind as clinical and radiological present specificities to the features commonly described in infants' medulloblastoma.

Transdural spinal cord herniation: A rare cause of neurologic impairment: Report of two cases and review of the literature.

INTRODUCTION: Herniation of the thoracic spinal cord is a rare pathology related to the genesis of a breach in the anterior dura. Adding to the previously published cases, we report our department's experience with 2 cases of young adults who presented with transdural spinal cord herniation following spinal trauma. PRESENTATION OF THE CASES: We report the cases of 2 men aged 27 and 57, with history of spinal trauma, who presented diverse clinical complaints (motor impairment, genito-sphincteric disorders, etc.). Spinal cord MRI showed an anterior displacement of the spinal cord. Both patients were operated. Peroperative findings and radiological features were concordant with transdural spinal cord herniation. DISCUSSION: Spinal cord herniation is a rare condition which is usually related to a misdiagnosed spinal trauma. Pathogenesis is still being debated. This condition may lead to a myelopathy potentially responsible for severe neurologic impairment, mainly presenting as a Brown-Sequard syndrome. Diagnosis is based on clinical and radiologic features on MRI. Surgical treatment is proposed when neurological signs are found. Long-term clinical and radiological monitoring is necessary for paucisymptomatic, non-operated patients. CONCLUSIONS: Based on the described cases, we tried to review the main clinical, radiological and therapeutic features related to this condition.

Pneumocephalus after posterior fossa surgery in prone position: Is that any clinical effect?

INTRODUCTION: The term pneumocephalus refers to the existence of air in any intracranial compartment. Its presence in the follows of a supratentorial craniotomy is very common, and it usually represents a benign complication as it is very rarely responsible for clinical manifestations. CASE PRESENTATION: We report the case of a 24 years-old man, who underwent posterior fossa surgery in prone position for resection of a vermian tumor. Postoperative, the patient presented a tonic-clonic generalized seizure associated to high levels of arterial pressure and decerebration. Control CT scan showed an important pneumocephalus. On the posterior fossa, the air was responsible for a compression of the brainstem, without any other postoperative complications. Following 12 h of conservative management, a brain MRI showed a total regression of the pneumocephalus. 3 days later, the patient presented a favorable outcome as he was extubated without any major impairments. DISCUSSION: Transformation of pneumocephalus into tension pneumocephalus responsible for clinical inadvertance is rare. This complication is mainly related to surgeries performed in sitting position. The occurrence of compressive pneumocephalus after a posterior fossa craniotomy performed in a prone position is seldom. CONCLUSIONS: Through this case, we discuss pathophysiology and therapeutic approaches for tension pneumocephalus following posterior fossa performed in prone position.

About a rare pediatric case of myxoid meningioma.

Intracranial meningiomas are uncommon in the pediatric group. Myxoid meningioma is a subtype of metaplastic meningioma. It is the rarest WHO grade I meningioma. Only one case of pediatric myxoid meningioma was described in the literature. We report the case of 14-year-old boy who presented with a left parietal dura-inserted tumor. Histological and immunological studies concluded to a myxoid meningioma. We reviewed the literature for this rare pathology and draw attention to the necessity of distinguishing it from other mucinous tumors of the central nervous system, especially chordoid meningioma which has poorer prognosis.

C1 C2 spinal cord compression in hereditary multiple exostoses: case report and review of the literature.

INTRODUCTION AND IMPORTANCE: Hereditary multiple exostoses (HME) is a benign disease, usually encountered in the metaphysis of long bones. On the opposite, spinal localizations are very rare. We herein describe a case of HME with a spinal cord compression. CASE PRESENTATION: A 31-years-old patient with a history of HME, presented with signs of cervical spinal cord compression that progressively appeared in the last 10 years. Cervical MRI and CT scan showed a compressive osseous tumor arising from the surface of the right side of the dorsal arch of the axis. Our patient underwent C1C2 laminectomy, but no clinical improvement has been recorded postoperatively. CLINICAL DISCUSSION: Spinal cord compression is an extremely serious complication of hereditary multiple exostoses (HME). The cervical and thoracic areas were predominantly affected, with the symptoms usually developing slowly. Early surgery is correlated to good postoperative outcomes. CONCLUSION: Despite its rarity, HME should be considered as a possible cause for spinal cord compression.

Unintentional direct intraventricular injection of gadolinium with fatal outcome: report of a case.

Gadolinium diethylenetriamine penta-acetic acid (Gd-DTPA) is the main contrast agent used in MRI, known for its good tolerance and rare toxicity. Even intrathecal injection of limited doses of Gadolinium can be performed in some indications. To our knowledge, only 3cases of accidental intraventricular injection of Gadolinium have been yet reported in the literature. We report the case of a 40-year-old male patient, who presented with headaches and vomiting. Brain MRI showed a right parietal abscess. The patient underwent emergent surgery for drainage of the septic collection. Postoperative MRI showed the development of a hydrocephalus related to a ventriculitis. Another surgery was performed to set up an external ventricular shunt, which lead to an improvement of the neurological status. A control brain MRI was scheduled for the patient, which revealed extensive abnormal enhancement inside the right lateral ventricle, on the basal cisterns as well as a leptomeningeal enhancement. Shortly after Gadolinium injection, the patient presented a tonic-clonic seizure. This clinico-radiological context leads to discover of the inadvertent intraventricular administration. Afterward, the patient's condition quickly deteriorated. Two days after the MRI he presented a cardiorespiratory arrest followed by death. Direct administration of Gadolinium into a ventriculostomy mistaken for intravenous catheter is a rare but harmful situation. Despite their rarity, such cases prove the importance of tracing all lines to their insertion sites to be confident of their appropriateness for injection.

Ischemic stroke following operated head trauma in children: Discussion of a rare clinical case.

INTRODUCTION: Traumatic brain injury (TBI) is the leading cause of morbidity and mortality in children. TBI in children are responsible for a range of clinical symptoms and signs that are comparable to those in adults, but present several differences in both physiopathology and management. Many postoperative complications may occur, ischemic stroke among others, which is generally related to an injury of an intracranial artery. Out of this case, it may be more difficult to find a suitable explanation to this complication. CASE PRESENTATION: We report the case of a child aged years old, who was collided by a car causing a polytrauma with head and chest injury. On body scan, she had a fracture of the sixth left rib, and a frontal cranio-cerebral wound. The patient was operated for debridement of the wound, and tight closure of the injured dura mater. Initial postoperative course was uneventful, but 5 days after first surgery patient presented an acute onset of a right hemiplegia followed by an alteration of her state of consciousness, and a left anisocoria. Follow up CT scan showed a stroke of the whole left carotid territory. The patient was re-operated through a left decompressive craniectomy. Following the second surgery, she showed an improvement of her level of consciousness and a normalization of the size of her pupils, but aphasia and a right hemiplegia persisted. 2 weeks after surgery, the patient had a progressive necrosis of the surgical scar, followed by an exposure of the underlying cerebral cortex. Despite of intensive local care, a plastic surgery to recover the wound and antibiotics, the patient presented a meningitis, followed by a septic shock and death. CLINICAL DISCUSSION AND CONCLUSIONS: Ischemic stroke is probably the most harsh and unpredictable complication that may occur after TBI, mainly in children. Only rigorous surgical approach followed by stringent post-operative care may prevent such outcomes.

Villonodular synovitis of the lumbar spine: Case report of a rare pathology.

Lumbar pigmented villonodular synovitis is rarely localized on the lumbar spine. Awareness of pigmented villonodular synovitis is important despite its rarity because it may mimic several other lesions of this location.