Totally thrombosed giant anterior communicating artery aneurysm.

Giant anterior communicating artery aneurysms are rare. Apatient presented with visual dysfunction, gait ataxia and urinary incontinence. MRI showed a giant suprasellar mass. At surgery, the lesion was identified as being an aneurysm arising from the anterior communicating artery. The difficulty in preoperative diagnosis and relevant literature are reviewed.

Atlantoaxial dislocation in a patient with nonsyndromic symmetrical dwarfism: Report of a rare case.

Congenital anomalies of the craniovertebral junction (CVJ) are complex developmental defects. We describe a patient with atlantoaxial dislocation (AAD) and short stature whose morphopathologydid not fit into any of the previously described syndromic constellations. The patient underwent a reduction of the AAD followed by fixation with C1-C2 transarticular screws. Although numerous syndromes have been linked to both dwarfism and craniovertebral junction anomalies, this patient did not fit into any of these patterns. It is possible that this may be one of the many as yet unrecognized patterns of congenital anomalies.

Actinomycotic osteomyelitis of the cranial vault presenting with headache: an unusual presentation.

A case of left parietal calvarial actinomycotic osteomyelitis in a young woman is described. She had no predisposing illnesses. She had delivered a live child at term and presented in the puerperal period. No extracranial focus of infection was identified. She responded well to a combination of surgery and medical therapy and had an excellent outcome. The authors emphasise the importance of establishing a histopathological diagnosis since radiological signs are non-specific and unreliable.

Trigeminal schwannoma of the sphenoid sinus--report of a rare entity.

Schwannomas are common slow growing benign tumors. About 25-45% of all schwannomas arise in the head and neck region. Less than 4% of these tumors are found in sinonasal regions and usually affect nasal ethmoid. There are very few case reports of a schwannoma arising from the sphenoid sinus. We report a case of sphenoid sinus schwannoma treated by microscopic decompression and review the relevant literature.

Multifocal spinal malignant peripheral nerve sheath tumor in an immunocompromised individual: case report and review of literature.

PURPOSE: Primary intraosseous spinal malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. MPNST with multifocal origin has been described to occur in the extremities. Such a lesion has not been described to occur in the spine. We describe a case of multifocal spinal MPNST and to review the literature relevant to this rare entity and its management. METHODS: A 40-year-old immunodeficient patient presented with rapidly progressive paraparesis and mid back ache. RESULTS: Despite aggressive surgical decompression, he developed multiple metastases 3 months after surgery. However, he remained stable for 1 year without any adjuvant therapy. Presently, he has received palliative radiotherapy for spinal recurrence and cerebral metastasis. CONCLUSION: Multifocal spinal MPNST is a rare lesion. In this instance, the multifocality of the disease and its odd location could be attributed to the immunodeficiency state. The prolonged survival could be due to an improvement in his immune status due to HAART.

Cerebral revascularization with superficial temporal - middle cerebral artery anastomosis for complete carotid occlusion: An emerging modality for preventing recurrent stroke.

Complete long segment carotid occlusion presents a treatment challenge. These patients cannot be managed adequately by endarterectomy or stenting. Despite best medical management, many continue to develop recurrent strokes. In this select group of patients, there may be role for flow augmentation techniques like superficial temporal-middle cerebral artery bypass. We report a patient who was thus successfully treated and remains asymptomatic. The relevant literature is reviewed.

Opportunistic Fonsecaea pedrosoi brain abscess in a patient with non-cirrhotic portal fibrosis-induced hypersplenism--a novel association.

Non-cirrhotic portal fibrosis, a common cause of splenomegaly in tropical countries, can lead to hypersplenism and pancytopenia. Hypersplenism in this setting has not been associated with opportunistic infections. We describe a patient with hypersplenism secondary to non-cirrhotic portal fibrosis who developed a Fonsecaea pedrosoi brain abscess and succumbed to the illness despite aggressive management.

Acute pyogenic necrotizing encephalomyelitis: a fulminant and fatal infection.

In this report the authors describe a rare case of a fulminant, pyogenic, necrotizing infection of the spinal cord and brain. Necrotizing lesions of the brain and spinal cord are usually infectious in origin and are associated with high rates of morbidity and death. Although the pathogens responsible have been identified in a few instances, the causal factors remain unknown in many cases. An 11-year-old girl developed acute, rapidly progressive paraplegia with bladder involvement and sensory loss below T-10. She had been treated recently for a Staphylococcus aureus infection of the knee joint precipitated by a penetrating injury with organic matter in the aftermath of a cyclone. Although appropriate antibiotic therapy was instituted, the spinal cord infection progressed to involve the entire spinal cord, brainstem, and brain. This fulminant course was marked by a rapid deterioration in the patient's clinical condition, ultimately leading to her death. Magnetic resonance imaging demonstrated a previously undescribed pattern of longitudinal enhancement along the spinal cord, as well as the white matter tracts in the brainstem and brain. The possible route of spread of infection along the neuraxis is postulated to be the potential space along the white matter tracts. Treatment is not standardized due to the rarity of the condition.

Larsen syndrome with C3-C4 spondyloptosis and atlantoaxial dislocation in an adult.

STUDY DESIGN: This is a clinical case report with a review of relevant literature. OBJECTIVE: To describe a case of Larsen syndrome with C3-C4 spondyloptosis and atlantoaxial dislocation in a middle-aged female patient and to discuss management strategies. SUMMARY OF BACKGROUND DATA: Spondyloptosis of the cervical spine is relatively rare and is caused by trauma, destruction of the vertebral bodies by tumors, or tuberculosis. Such gross vertebral displacement is usually associated with significant neurological deficits. Larsen syndrome is characterized by multiple joint displacements and can, very rarely, be associated with nontraumatic spondyloptosis of the cervical vertebra. A single case report of C1-C2 joint laxity causing atlantoaxial dislocation in a patient with Larsen syndrome is available in literature. No reports of any patient (with Larsen syndrome or nonsyndromic) who had both cervical spondyloptosis and atlantoaxial dislocation are available in literature. METHODS: A 36-year-old female presented with chronic neck pain, bilateral hand deformity, and mild spasticity involving all 4 limbs. Cervical radiograph, computed tomographic scan, and magnetic resonance image revealed C3-C4 spondyloptosis and atlantoaxial dislocation. RESULTS: A combined ventral decompression of subaxial spine and instrumentation from C2 to C5, followed by posterior C1-C2 distraction arthroplasty and lateral mass stabilization of the subaxial spine up to C6, was done. The cervical deformity was corrected, and the patient remains symptom free. CONCLUSION: Patients with spondyloptosis of the cervical spine can rarely present with chronic neck pain and minimal neurological deficits. An additional pathology, such as atlantoaxial dislocation, can add to the complexity. Circumferential stabilization and fusion would be required in such cases to achieve a good outcome. Larsen syndrome is a rare cause of nontraumatic cervical displacements.

Non-infantile desmoplastic cerebellar ganglioglioma in a patient with multiple congenital anomalies: a rare association.

Numerous syndromes with craniofacial, cardiac, cutaneous and vascular anomalies have been described in the literature. Here we report on a 9-year-old girl who was diagnosed as having a clavicular arteriovenous malformation with limb enlargement, an axillary port wine stain and macrocephaly with frontal bossing. She was investigated for enlarged head size; although she did not have hydrocephalus as initially suspected, she was found to have a right cerebellar lesion. This was excised and the pathology report was diagnostic of desmoplastic ganglioglioma. The posterior fossa is a very rare location for gangliogliomas. This was a desmoplastic variant that is only encountered in infants. Thus, the location and the pathology made the tumour rare. In addition, the cranio-vasculo-cutaneous lesions made this previously unreported association noteworthy.

Heparin-induced tumour bleed.

Patients with gliomas are at risk for deep vein thrombosis and thromboembolic events, and pharmacological prophylaxis may be indicated. This recommendation needs to be weighed against the risk of spontaneous haemorrhage from certain brain tumours. A patient, who was hemiparetic owing to a high-grade glioma and was started on heparin prophylaxis, developed a tumour bleed. The relevant issues are briefly discussed.

Tuberculous cerebellar abscess in immunocompetent individuals.

Tubercular abscess of the brain is a rare form of central nervous system tuberculosis. These lesions usually occur in the supratentorial compartment. They are associated with a state of immune deficiency. We report two immunocompetent individuals with tubercular abscess of the cerebellum and discuss the management of these lesions.

Idiopathic intraventricular aneurysm presenting with intraventricular hemorrhage: case report and review of the literature.

Intraventricular hemorrhage (IVH) is a relatively commonly encountered problem in neurosurgical practice. The underlying causes could include hypertension, arteriovenous malformations (AVM), angiomas, trauma, tumors, aneurysms and moyamoya disease. Truly idiopathic intraventricular aneurysms (IVA) are rare. A high index of suspicion needs to be maintained since, with the appropriate treatment, the outcome is generally good. We report the case of a 14-year-old boy who presented with sudden onset headache and vomiting. CT angiogram pointed to the possibility of an IVA. This was confirmed by MRI with three-dimensional constructive interference in steady state (CISS-3D) sequences and digital subtraction angiography. The patient underwent microsurgical excision of the aneurysm and is doing well on follow-up. Idiopathic IVA can present with IVH at any age. MRI with CISS-3D and MR angiography would be the imaging modality of choice since it can also rule out other causes of intraventricular bleeding such as tumors and AVM. These lesions could be managed effectively by microsurgical excision/clipping. The necessity of investigating every patient who presents with IVH is debatable.