Sudheer Kumar Gundamaneni

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.

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.

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.

Giant cavernous carotid artery aneurysm mimicking a fungal granuloma and presenting with massive epistaxis

A 42-year-old man presented with frequent minor nasal bleeds since 1 month. He was undergoing chemotherapy for pulmonary tuberculosis. MRI brain revealed a space occupying lesion in the right cavernous sinus extending to sphenoid sinus, with T2 inversion. An initial diagnosis of fungal granuloma was made and endoscopic trans-nasal biopsy was attempted. During surgery, a pink pulsating mass was seen in the sphenoid sinus and the procedure was abandoned. A cerebral CT-angiography done subsequently revealed a giant right cavernous segment internal carotid artery (ICA) aneurysm. He was then referred to our centre and upon admission he collapsed secondary to a major bout of epistaxis. An emergency cervical carotid artery ligation resulted in transient control of epistaxis. Owing to recurrence of bleed, trapping of the aneurysm was done resulting in cure. The present case shows that a giant cavernous ICA aneurysm can occasionally be erroneously diagnosed as fungal granuloma.

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

Abstract 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.

Tremor and dystonia associated with thalamic cyst: a rare manifestation.

A 26-year-old man presented with a 1-year history of progressive weakness and coarse postural tremor involving the right upper extremity (see Video 1). The tremor was coarse, predominantly involving the elbow, wrist, and hand. The tremor was accentuated on movement. At rest, the tremors were inconspicuous but there was a dystonic posturing of the right hand. Similar tremors of lower amplitude were noted at the right ankle and foot, especially on walking. He had been treated with various medications with no significant relief of the tremor. He had attempted to commit suicide due to the medically refractory nature of the tremor.

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.

Intraventricular hemorrhage: A catastrophic complication after removal of old ventriculoperitoneal shunt

A 12-year-old female who was diagnosed as having Chiari I malformation with hydrocephalus had undergone a ventriculo-peritoneal (VP) shunt in another hospital five years back. She presented to us with complaints of intermittent fever, severe holocranial headache, vomiting and progressive deterioration of sensorium of one week duration. On examination, she was drowsy, opening eyes to call, confused but was obeying commands. Right pupil was dilated and fixed, and the left pupil was 2 mm in size and was reacting to light. Fundi showed bilateral papilledema. She was moving all four limbs spontaneously. Computed tomography (CT) brain showed dilated ventricular system with periventricular lucency [Figure 1]. The shunt was seen in situ with its tip in the right lateral ventricle. In view of high fever and rapid deterioration of sensorium, shunt malfunction secondary to an infection was suspected and her shunt was exteriorized and converted into an external ventricular drain (EVD). Cerebrospinal fluid (CSF) was sent for analysis and she was started on intravenous antibiotics. After three CSF culture reports excluded bacterial growth, she was planned for a VP shunt on the opposite side. Under general anesthesia, she underwent left VP shunt and removal of the right ventricular catheter, which was functioning as an EVD. No resistance was felt during removal of the old ventricular catheter. In the postoperative period, she complained of severe headache and nausea. An urgent postoperative CT scan revealed massive intraventricular hemorrhage and the new shunt catheter was seen in the left ventricle outside the blood cast [Figure 2]. However, the ventricular size had reduced and the periventricular lucency had disappeared indicating a functioning shunt. Since she improved in sensorium and also because the scan showed reduced ventricular size, she was managed conservatively with continuous monitoring. She gradually improved further, and her symptoms resolved completely.

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.