Giant chordoid meningioma symptomatic immediately after pregnancy: report of a rare case.

BACKGROUND: Chordoid meningioma is a very rare subtype of meningioma with less than 90 cases reported in the literature. Meningiomas related with a pregnancy are also very rare. Herein, we present a pregnancy-associated case of a chordoid meningioma and briefly discuss possible mechanisms. CASE HISTORY: Approximately 10 hours after a Caesarean section, a 37-year-old woman became stuporous with a Glasgow coma scale score of 9 - 10 and the right pupil became dilated without reaction to light. An emergency computed tomography scan showed a right frontotemporal lesion measuring 8 x 7 x 6 cm which enhanced moderately and homogenously after intravenous contrast injection. The patient's hemoglobin level was slightly lower than normal level but there was no dysgammaglobulinemia or lymphoid hyperplasia. The patient was immediately taken to the operating theater and the tumor was gross totally removed. The postoperative period was uneventful; and histopathological diagnosis was chordoid meningioma. CONCLUSION: The exact mechanism causing a pregnancy-associated increase in the size of meningiomas and the amount of peritumoral edema has not been clearly elucidated. As for this case of chordoid meningioma associated with pregnancy, we think mucin accumulation in tumor could be involved in an increase in the tumor size while delivery procedures with the common anaesthetic and sedative drugs may also have enhanced the peritumoral edema by causing a relative decrease in the cerebral blood flow.

An extreme lateral lumbar disc herniation mimicking L4 schwannoma.

OBJECTIVE: We herein describe a case with an extraspinal mass distorting the right L4 dorsal root ganglion. Initially presumed to be a nerve root schwannoma, the lesion turned out to be a free disc fragment. METHODS AND RESULTS: A 46-year-old woman presented with a history of mild low back and intense right leg pain. The leg pain was like electrical discharges. Right knee extension was weak together with hyperalgesia and loss of heat sensation in the right LA dermatome. The right patellar reflex was absent. Electromyography showed acute and chronic denervation of muscles innervated by the right L4 nerve root. MR scan showed a right L4-5 extraforaminal mass distorting the L4 dorsal root ganglion. The mass enhanced homogeneously after gadolinium injection and was thought to be a tumor. It was surgically removed using a midline incision and intraspinal, followed by extraspinal dissection. Under the operating microscope, the mass extended between an intact lateral longitudinal ligament and a swollen dorsal root ganglion. Histopathologic examination ruled out a tumor and showed that the mass consisted of degenerated disc material surrounded by a large peripheral zone of neovascularization. CONCLUSION: Extraforaminal periganglionic free, encapsulated disc fragments may mimic tumoral masses, from which they may not be distinguished on MRI.

Bilateral trigeminal neuralgia and charcot-marie-tooth disease: diagnosis and successful microsurgical treatment of bilateral neurovascular compression.

BACKGROUND: The association of Charcot-Marie-Tooth (CMT) disease and trigeminal neuralgia (TN) is rare. CMT and bilateral TN is even rarer. Our literature review has revealed only 4 previous cases with CMT and bilateral TN. We report the case of a 23-year-old man with a prior diagnosis of CMT and unilateral deafness who initially presented with excruciating and lancinating right-sided facial pain. PATIENT AND METHOD: Initially treated with percutanous ablation elsewhere with only short-lasting relief, the patient was on toxic doses of medication when he presented to us. Neuro-imaging using magnetic resonance imaging showed vascular compression as the possible cause of pain. At initial surgery, two artery loops were found compressing the right nerve superomedially and an artery and a vein was found compressing the nerve inferomedially. Relief was instantaneous and for 1 year there was no recurrence of right facial pain. Left-sided pain which had been minimal before microvascular decompression (MVD) became intolerable immediately after right-sided surgery, yet responded to mild doses of carbamazepine for almost a year. At one year, the patient had to undergo MVD for the left trigeminal nerve with complete relief over both sides of his face without any medication. RESULTS: The patient remains pain-free on both sides at 1-year follow-up after the second MVD. CONCLUSIONS: The underlying neuropathy in CMT makes the trigeminal nerve more vulnerable to vascular compression than usual. The bilateral compression of the trigeminal nerve-pons junction in our patient suggests that the external pressure probably adds to the internal defects in central myelin formation, structure or maintenance. Nevertheless, the mid-term follow-up after MVD clearly shows that the causal treatment for TN can be successfully applied to patients with TN plus CMT.

Retro-tympanic pulsatile mass originating from dumb-bell jugular foramen schwannoma.

BACKGROUND: Jugular foramen (JF) tumours are uncommon with paraganglioma, schwannoma and meningioma occurring most commonly in this location. JF schwannoma with extension to the retro-tympanic area has been described only once. METHODS: 20-year-old man presented with headache, blurred vision, vomiting and diplopia. FINDINGS: A left pulsatile retro-tympanic mass was seen at otoscopy. A jugular foramen tumour was found on CT and MR images. The intracranial portion of the tumour later diagnosed as schwannoma was removed. Control ENT examination confirmed that the residual retro-tympanic mass was no-longer pulsatile. CONCLUSIONS: Jugular foramen schwannomas may also extend into the retro-tympanic area.

Grisel's syndrome: a case of potentially lethal spinal cord injury in the adult.

A 22-year-old woman presented with respiratory difficulty and quadriparesis two weeks after an upper respiratory tract infection. CT showed mild (Type I) rotatory atiantoaxial subluxation, but MRI demonstrated a severely contused and oedematous spinal cord at C2-3. The case was managed conservatively with collar, steroid and antibiotics. The outcome was excellent.

Giant aneurysm of the proximal (A1) anterior cerebral artery.

BACKGROUND: Aneurysms arising from the proximal (A1) segment of the anterior cerebral artery are quite rare. A giant aneurysm in this location is a true exception. METHOD-FINDINGS: A 66-year-old man who presented with drowsiness and confusion was diagnosed to harbor a giant ovoid (30 x 25 mm) aneurysm originating from the right A1. The aneurysm arose from the mid 3/5th of the artery and there was no neck. There was good cross-flow through the anterior communicating artery. Instead of trapping, the flow through the A1 was maintained by connecting the axis of inflowing A1 and outflowing A1 segments using a fenestrated clip. The sac was finally decompressed. The patient is alive and well 3 years postoperatively with complete resolution of the pre-operative hydrocephalus. INTERPRETATION: A review of the literature revealed 14 such cases. The presentation, management and the outcome of these rarities differ from routine A1 aneurysms or other paraclinoid giant aneurysms and are briefly reviewed.

Spontaneous spinal cord herniation: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Spontaneous herniation of the spinal cord substance through a previously uninjured and/or untouched dura is a very exceptional occurrence. Spontaneous spinal cord herniation, which was first reported 25 years ago, is a cause of myelopathy that is treatable but difficult to diagnose. CLINICAL PRESENTATION: A 49-year-old female patient who presented with a 3-year history of a burning sensation and hyperesthesia in her right leg and a 3-month history of left leg stiffness was diagnosed as exhibiting signs of Brown-Séquard syndrome. Magnetic resonance imaging of the thoracic spinal canal demonstrated S-shaped anterior kinking and transdural herniation of the spinal cord at the T3-T4 levels. INTERVENTION: The patient underwent surgery via a three-level laminectomy. The herniated part of the spinal cord was microsurgically reduced, and the dural defect was repaired with Gore-Tex membrane (WL Gore & Associates, Flagstaff, AZ). The outcome of surgery was excellent. CONCLUSION: Review of the world literature revealed 29 reported cases, with 27 of these cases being published since 1990. The clinical features, radiological diagnosis, and treatment options for this unique entity are summarized, with a synopsis of numerous misconceptions that appeared in the literature. With more familiarity with and increased awareness of this entity, more cases will be diagnosed.

Intracranial plasma cell granuloma.

Plasma cell granulomas (PCGs) are benign, inflammatory masses of unknown etiology composed of polyclonal mature plasma and lymphoid cells. The lung is their most common location, and occurrence within the cranial cavity is extremely rare. We report the case of an 11-year-old girl who presented with seizures and was diagnosed as having a dural-based right frontal tumor that extended toward the sagittal sinus and the falx. The lesion was totally excised, together with the abnormal dura. A limited cortical excision was also performed using electrocorticographic guidance. Histopathologic diagnosis of intracranial plasma cell granuloma was reached after extensive immunohistochemical tests and electron microscopy. This is the third case of PCG with description of changes in the neighboring cerebral tissue. Although PCGs are well-circumscribed lesions, lymphoplasmocytic inflammation, neuronal loss, and reactive gliosis occur within the adjacent cortex. Disturbed cortical lamination, as we have observed, appears to be a histological finding that has not been described previously.

Syringostomy using myringostomy tube: technical note.

OBJECTIVE: We describe the concept of a new syringostomy technique. METHODS: The technique includes the insertion of a myringostomy tube through a small myelotomy at the level of maximum enlargement of the syrinx. The inner diameter of the myringostomy tube is 1.14 mm. RESULTS: This technique reduces the potential risks that are associated with other techniques that use bulky silastic catheters that are left inside the spinal cord. CONCLUSION: We think that this technique is the least invasive of all similar techniques and that, in terms of drainage, the myringostomy tube is as efficient as commonly used silastic catheters.

Spontaneous intracranial hemorrhage of structural origin during the first year of life.

Symptomatic intracranial hemorrhage (ICH) in term infants is not common, but when it occurs it is usually secondary to trauma, coagulation disorders and/or hypoxia. The possibility of a structural cause for an infantile ICH is unfortunately not seriously considered until very late. In this paper we report the cases of five full-term infants, each of whom developed ICH secondary to a structural lesion during the 1st year of life. Three presented during the newborn period. A congenital saccular aneurysm of the middle cerebral artery in an 8-month old male infant; a posterior fossa arteriovenous malformation in a 2-week old female neonate; a deep parietal cavernous angioma in a 6.5-month-old male infant; a temporoparietal low-grade astrocytoma in a 12-day old male neonate and a temporoparietal desmoplastic ganglioglioma in a 9-day-old male neonate were the structural lesions that were causative for hemorrhage. In all cases but one, the diagnosis was reached by computerized tomography and/or magnetic resonance imaging. All infants underwent surgery for the removal of the hematoma and of the lesion causative for the bleed. All are alive at 19, 3, 11.5, 10, and 5 years, respectively. We discuss the diagnosis of ICH with special emphasis on contemporary imaging modalities and stress the benefits of aggressive and timely surgical treatment. We then consider a concise analysis of the world literature on the occurrence of structural causes of ICH during infancy.

Jugular foramen meningioma. Report of a case and review of the literature.

The occurrence of a meningioma within the jugular foramen is very rare. We present the case of a 33-year-old man who presented with five-year history of hoarseness and was found to have paralysis of lower four cranial nerves on the left. High resolution computed tomography showed a dumbbell shaped tumor of the left jugular foramen extending intracranially over the jugular tubercle and extracranially into the parapharyngeal space. The patient underwent staged neuro-otological surgery and the mass was removed subtotally with no recurrence in six years. Histopathologic diagnosis was transitional meningioma. Our review of the literature revealed 33 previously reported cases. Four of these cases had neurofibromatosis. All but one underwent some form of otolaryngologic surgery. Although twenty had an intracranial component to their tumor, only 11 had a craniotomy. The majority of the tumors were meningothelial meningiomas (60%). Only two cases were malignant meningiomas. We reviewed the clinical and radiological characteristics and summarized the benefits and pitfalls of existing surgical options.

Distal end revision of ventriculoperitoneal shunts sparing minilaparotomy. Technical note.

Distal end malfunction of a ventriculoperitoneal shunt occurs secondary to outgrown, disconnected, fractured, or occluded peritoneal catheters. Replacement of such catheters normally requires a minilaparotomy. The authors describe a simple technique for peritoneal catheter replacement without minilaparotomy.

Posttraumatic gas-containing brain abscess caused by Clostridium perfringens with unique simultaneous fungal suppuration by Myceliophthora thermophila: case report.

OBJECTIVE AND IMPORTANCE: Gas-containing brain abscesses are rare, and the vast majority are caused by Clostridium perfringens. Significant simultaneous fungal infection in a bacterial abscess is even rarer. We present such a case and review the literature. CLINICAL PRESENTATION: A 21-month-old male patient sustained a penetrating head injury in a barnyard, developed a gas-containing left parietal brain abscess, and presented with high fever, galeal swelling, and seizure. INTERVENTION: The patient initially underwent debridement of his wound and then repeated aspirations. The initial cultures revealed pure growth of Clostridium perfringens. Despite appropriate antibiotic therapy, serial neuroimaging did not demonstrate a decrease in the size of the cavity. An excision had to be undertaken 6 weeks after the injury. The culture from the excised specimen revealed an unexpected growth of a saprophytic and opportunistic fungus, Myceliophthora thermophila. Antifungal treatment consisting of the administration of liposomal amphotericin B and itraconazole was then performed. The child was well and neurologically intact 6 months after the excision. CONCLUSION: Our review revealed 38 cases of clostridial brain abscess in the literature. Despite the reputation of the organism, the outcome with clostridial brain abscesses was relatively benign. The main characteristics of clostridial brain abscesses are highlighted, with reference to their optimal treatment. Our review also revealed that fungal infection after a penetrating head injury is extremely rare and often fatal. Our case seems to be the first in the medical literature with growth of M. thermophila as a causative agent for intracranial suppuration.

De novo familial cavernous malformation presenting with hemorrhage 12.5 years after the initial hemorrhagic Ictus: natural history of an infantile form.

Cerebral cavernous malformations are congenital, non-neoplastic lesions, but they are known for their potential for growth. De novo lesion genesis is exceptional and occurs more commonly with the familial form of the disease. We report the case of a 13-year-old Caucasian boy with a positive family history, who underwent surgery for a left parietal hemorrhage during the 1st year of life and who recently presented with a new hemorrhage in the left temporal lobe quite distant from the previous hematoma bed. Both hemorrhages were caused by cavernous malformations. Sequential magnetic resonance scans prior to the recent presentation failed to disclose the growth of the new lesion. We reviewed the literature for mechanisms of new lesion formation.