Hydrocephalus in Paget's disease.

Neurological manifestations in Paget's disease are rare. Hydrocephalus is infrequent, its etiology in Paget's disease being multi-factorial. Surgical and anesthetic procedures may be challenging, requiring special precautions. We describe one such case and discuss these peculiarities. An elderly lady, diagnosed to have Paget's disease presented with features of recent onset dementia, gait disturbances with urinary incontinence. Hydrocephalus secondary to posterior fossa crowding was present. A ventriculo-peritoneal shunt was inserted. Endotracheal intubation proved to be difficult. Modification of the surgical technique was required to ensure a safe procedure. She improved immediately following CSF diversion. Timely CSF diversion yields good results. Certain precautions during anesthesia and surgery can ensure a safe and uneventful peri-operative period.

Cerebral perfusion pressure management of severe diffuse head injury: effect on brain compliance and intracranial pressure.

BACKGROUND: Cerebral perfusion pressure management (CPPM) is an accepted modality of treatment of severe diffuse head injury (SDHI). However, CPPM has the potential to cause transcapillary exudation in the presence of a disrupted blood brain barrier and can lead to further increase of intracranial pressure (ICP) and worsening of compliance. AIMS: This study attempts to evaluate the effect of both transient and prolonged changes in cerebral perfusion pressure (CPP) on ICP and cerebral compliance as measured by the Pressure Volume Index (PVI), and to correlate changes in PVI with outcome at 12 months using the Glasgow Outcome Score. SETTINGS AND DESIGN: Prospective study in a neurosurgical ICU. MATERIAL AND METHODS: Twenty-seven SDHI patients managed using standard protocol to maintain CPP above 70 mmHg. Mean arterial pressure (MAP), ICP and CPP were monitored every half-hour. Daily monitoring of the PVI and ICP was done before, and after the induced elevation of MAP using IV Dopamine infusion. The relationship between CPP, MAP, ICP, PVI and outcome was evaluated. STATISTICAL ANALYSIS USED: The paired and independent samples T-test, and the Pearson correlation coefficient. RESULTS: CPPM rarely leads to progressive rise in ICP. Maintaining CPP above 70mmHg does not influence ICP or PVI. Transient elevations in CPP above 70mmHg may produce a small rise in ICP. Trend of change in PVI influenced outcome despite similar ICP and CPP. CONCLUSION: Elevating the CPP above 70mmHg does not either reduce the ICP or worsen the compliance. Monitoring changes in compliance should form an integral part of CPPM.

Outcome prediction model for severe diffuse brain injuries: development and evaluation.

BACKGROUND: Intensive care resources for the management of severe diffuse brain injury patients (SDBI) are limited. Their optimal use is possible only if we can predict at admission which patients are unlikely to improve. AIMS: To develop a simple and effective model to predict poor outcome in patients with SDBI in order to help guide initial therapy. MATERIAL AND METHODS: The prognostic factors and outcomes of 289 patients with severe diffuse brain injury (GCS 3-8) were analyzed retrospectively. The prognostic factors analyzed were age, mode of injury, GCS at admission, pupillary reaction, horizontal oculocephalic reflex, and CT scan findings. Outcome at 1 month was classified as unfavorable--death or persistent vegetative state, or favorable--improvement with or without some disability. A stepwise linear logistic regression analysis was used to identify the most important predictors of poor outcome. A prediction model (NIMHANS model-NM) was developed using these factors. NM and several currently available outcome prediction models were prospectively applied in a separate group of 26 patients with severe diffuse brain injury managed with a different protocol. RESULTS: The most important predictors of poor outcome were found to be the horizontal oculocephalic reflex, motor score of GCS, and midline shift on CT scan. NM was found to be more sensitive (75%) and specific (67%) than most other models in predicting unfavorable outcome. NM had high false pessimistic results (33%). CONCLUSION: Prediction models cannot be used to guide initial therapy.

Prognosis in children with head injury: an analysis of 340 patients.

BACKGROUND: The outcome in children with head injury is distinctive because of the different biophysical properties of the child's skull and brain, and their reaction to injury. METHODS: In this retrospective study of three hundred and forty children with head injury, managed from January 1993 to December 1998, at NIMHANS, the factors influencing outcome were analyzed. RESULTS: On admission there were 40 children in GCS 3-5, 55 children in GCS 6-8, 96 in GCS 9-12 and 152 children in GCS 13-15. Eleven patients were under 2 years of age, 53 were between 3-5 years, 140 were between 6-10 years and 156 were between 11-15 years of age. The prognosis in various intracranial pathologies due to head injury was evaluated and outcome assessed at discharge. There were 95 children with EDH and 8.4% had poor outcome (vegetative state or death). There were 85 patients with contusion and poor outcome was noted in 18.8%. One hundred patients had diffuse cerebral oedema on CT scan and outcome was poor in 25% of these patients. The clinical features associated with poor prognosis were, absence of ocular movements (50%), abnormal pupillary size and reaction (49%) and age less than 2 years (27%).

3D steady-state MR cisternography in CSF rhinorrhoea.

PURPOSE: To determine the utility of 3D steady-state MR cisternography in the demonstration and localisation of cerebrospinal fluid (CSF) leak in patients with clinically suspected CSF rhinorrhoea. MATERIAL AND METHODS: Six consecutive patients with clinically suspected CSF rhinorrhoea were examined with routine MR evaluation and MR cisternography (MRC). All MR examinations included fast spin-echo (SE) T1WI in axial and sagittal planes, fast SE T2WI in axial and coronal planes and fluid attenuated inversion recovery (FLAIR) images in the axial plane. 3D evaluation was done using the CISS technique with 0.7-mm thickness in the sagittal and coronal planes. The site and extent of the defect, and any brain herniation detected on MRC were correlated with surgical findings. RESULTS: In the 6 patients who underwent surgical exploration and repair, intraoperative findings correlated with the defect revealed by MRC in all cases. CONCLUSION: In clinically suspected CSF rhinorrhoea, MRC is highly accurate in localising the site and extent of CSF fistula and may be used as the first investigation due to its efficacy and non-invasive nature.

Prognostic factors in children with severe diffuse brain injuries: a study of 74 patients.

Severe diffuse brain injury in children has a devastating influence on their physical and psychological development. This retrospective study was undertaken to analyse the factors that influence outcome in children with such injuries. The short-term outcomes (Glasgow outcome score) of 74 children (age < or =15 years) with severe diffuse brain injury and no focal operable mass lesions on CT scan, admitted between 1992 and 1998 at the National Institute of Mental Health and Neurosciences were analysed. The prognostic factors analysed were age, gender, time after injury to admission, nature of injury, highest post-resuscitation Glasgow coma score (GCS), pupillary reaction, horizontal oculocephalic reflex and CT scan findings. The following factors were found to be independent predictors of poor outcome: GCS 3-5 (p < 0.003), absent verbal response (p < 0.001), motor response score of 1-3 (p < 0.001), absent pupillary reaction (p < 0.001), absent oculocephalic reflex (p < 0.001) and presence of traumatic subarachnoid haemorrhage on CT scan (p < 0.002). These independent variables were then subjected to a stepwise logistic regression analysis, and the most important variables for predicting outcome were oculocephalic reflex and GCS, which together correctly predicted unfavourable outcome with a sensitivity of 79% and a specificity of 65%. An early CT scan or a single CT scan did not have any prognostic significance.

Endoscopic approach to intraventricular cysticercal lesions.

Various surgical approaches to treat intraventricular cysticercosis have been practiced over the years. We now present our experience with the use of the endoscope in the removal of intraventricular cysticercal cysts in patients with intraventricular cysticercal lesions associated with hydrocephalus. From 1995 to 1998, we have excised intraventricular cysticercal cysts from 9 patients. They were located in lateral ventricle in 4, in the third ventricle in two and in the fourth ventricle in three patients. A Gaab's rigid neuroendoscope system was used to enter and excise cysts in the lateral and third ventricle. The flexible fiberoptic scope was used for excising cysts in the fourth ventricle, through a transaqueductal route. In all cases a precoronal frontal burr hole was used for entry. All have been treated with albendazole in the postoperative period. All the cysts were removed successfully using endoscopic neurosurgery alone. One patient had a superficial injury to the rigid side of the aqueduct with a postoperative deficit, which improved. Three septal perforations, three third ventriculostomies, and one aqueductoplasty were done in the same sitting after cyst removal for CSF diversion. None of the nine cases required further surgery up to date. The follow-up period varied from 12 to 45 months with a median of 18 months. Endoscopic neurosurgery is a minimally invasive technique enabling removal of intraventricular cyticercal cysts from all locations, avoiding major craniotomies/posterior fossa explorations and shunts.

Laminoplasty: an evaluation of 24 cases.

Cervical expansive laminoplasty has been advocated as an alternative procedure to laminectomy for the decompression of the cervical spine. It provides favourable cord decompression and stabilisation of the cervical spine and is a simpler and safer alternative to anterior fusion and laminectomy for myelopathy and myeloradiculopathy, due to multisegmental cervical spondylosis and ossified posterior longitudinal ligament. We report our experience in 24 patients with this procedure, 12 of whom had myelopathy and another 12 had myeloradiculopathy. The earliest symptom to improve was radicular pain or paraesthesia (75%). A reduction in spasticity was seen in 21 of the 24 patients (87.5%). Eleven patients had improvement in their motor power during a follow up period ranging from 1 month to 14 months. One patient deteriorated following the procedure and developed Brown Sequard features due to under riding of the lamina on the hinged side, another had severe post operative paraesthesias, while one patient had a CT scan evidence of 'closing of the door', without being symptomatic for it. The technique of the procedure is discussed and the pertinent literature reviewed.

Solitary olfactory groove schwannoma: case report with review of the literature.

A 45-year-old man presented with headaches of 6 months' duration and generalized seizures in a cluster 2 days prior to admission. Examination revealed bilateral papilloedema and no focal deficits. CT scan showed an isodense enhancing mass lesion in the right frontobasal region with perilesional edema. Bifrontal craniotomy (with total excision of the tumor) revealed a tumor in the region of the right olfactory groove attached to the cribriform plate. Histopathological examination disclosed it to be a hyalinised schwannoma. Solitary schwannomas arising from the olfactory groove are extremely rare. Only six other cases have been reported so far. The presentation. CT scan findings and histogenesis of the tumor are discussed along with a review of the literature.

Neuroendoscopic third ventriculostomy in the management of fourth ventricular outlet obstruction.

Acquired fourth ventricular outlet obstruction, an uncommon entity, has been conventionally managed by ventriculoperitoneal shunt placements or excision of the obstructing membranes. The role of endoscopic third ventriculostomy is highlighted in the present communication. Three patients presenting with symptoms of raised intracranial pressure were diagnosed to have fourth ventricular outlet obstruction by neuroimaging studies and underwent endoscopic third ventriculostomy. All the patients had relief of their symptoms in the postoperative period. Neuroimaging studies performed at follow-up revealed decrease in ventricular size in all. Endoscopic third ventriculostomy is a useful alternative in the management of acquired fourth ventricular outlet obstruction.

Unilateral meningeal thickening: a rare presentation of tuberculous meningitis.

A rare case of tuberculous meningitis (TBM) causing unilateral thickening of meninges, is described here. The 50 year old male is presented with hemicranial headache and unilateral multiple cranial nerve involvement without any long tract signs. Magnetic resonance imaging (MRI) scan including enhanced studies showed a hemicranial thickening of the meninges. Biopsy of the meninges revealed tuberculosis. The patient improved gradually on antitubercular treatment (ATT). Follow-up at 6 months revealed significant improvement in neurological status. Though tuberculous involvement of the central nervous system (CNS) is very common in India, hemicranial meningeal involvement has never been reported.

Magnetic resonance angiographic assessment after extracranial-intracranial bypass surgery.

Extracranial-intracranial (EC-IC) bypass operation may be performed to augment the distal cerebral circulation. The bypass patency is usually assessed postoperatively with conventional cerebral angiography. Six patients are reported in whom the bypass patency was assessed using magnetic resonance angiography (MRA): Two had intracavernous carotid aneurysms, three had base of skull tumours encompassing the internal carotid artery, and one had occlusion of the right internal carotid artery with poor collateral reserve as confirmed by an acetazolamide stimulation test. Postoperative conventional cerebral angiography was also obtained in three patients for comparison. The MRA correlated reasonably well with conventional cerebral angiography in showing bypass patency, although conventional cerebral angiography was superior in demonstrating small vessels; MRA has the added advantage of showing the relative flow contribution and flow direction under physiological conditions by using targeted presaturation pulses.