Lumbo-costo-vertebral syndrome with posterior spinal dysraphism.

Lumbo-costo-vertebral syndrome, which includes abnormalities of the vertebral bodies, ribs and trunk musculature, is very rare and only few cases have been reported. We report a case of 18-month-old female child with absent ribs, hemivertebrae, superior lumbar hernia (features of lumbo-costo-vertebral syndrome) and posterior spinal dysraphism, which to our knowledge is the first case in the English literature with such a combination of defects. Embryology and management of the case is discussed.

Long-term outcome in patients with central neurocytoma following stereotactic biopsy and radiation therapy.

Total excision is usually the recommended treatment for central neurocytomas. The role of radiation therapy in their management is controversial, and is usually reserved for partially resected or recurrent tumours. Long-term outcome was studied in patients with central neurocytoma who were treated with radiation therapy. By retrospective chart review, eight patients with intraventricular tumours, who had undergone radiation therapy following stereotactic biopsy, were identified. The diagnosis of neurocytoma was confirmed based on positive immunostaining with synaptophysin. Clinical and radiological follow-up was obtained. Of eight patients, one died 5 years after treatment from shunt dysfunction and 1 had disseminated intracranial disease 15 months after treatment. Six patients were symptom-free at a mean follow-up period of 78 months and had good local control as demonstrated by CT. Since this tumour can be confused with an oligodendroglioma or an ependymoma on routine histopathology (as happened in seven of our cases); immunohistochemical studies with synaptophysin should be routinely performed for intraventricular glial tumours. Our study supports the beneficial effect of radiation therapy in the management of these tumours.

Effect of controlled hypercapnia on cerebrospinal fluid pressure and operating conditions during transsphenoidal operations for pituitary macroadenoma.

Controlled hypercapnia was assessed for its effectiveness in raising cerebrospinal fluid pressure to enable descent of the suprasellar portion of pituitary macroadenomas during transsphenoidal surgery. The result was compared in a randomized, single blind trial with intermittent boluses of saline injected intrathecally. Intrathecal pressures were monitored in both groups. Twenty-seven of 29 patients in the study group and 25 of 28 patients in the control group reached the target pressure of 20 mm Hg. The mean ETCO2 at the time of maximum pressure was 42.34 +/- 4.75 mm Hg in the study group and 29.81 +/- 2.61 mm Hg in the control group, (P< .001). Mean arterial carbon dioxide was 46.90 +/- 6.55 and 31.42 +/- 4.87 mm Hg, respectively. Surgeons blinded to the technique assessed the descent of the tumor. The operating conditions were judged by the surgeons to be satisfactory for 20 patients in the study group and 17 patients in the control group. Both techniques were equally effective in raising intracranial pressure and in providing descent of the suprasellar component of the tumor. No untoward side effects occurred while using either technique. The authors conclude that controlled hypercapnia is effective in producing descent of the suprasellar portion of a pituitary adenoma.

Extradural endodermal cyst of posterior fossa: case report, review of the literature, and embryogenesis.

OBJECTIVE AND IMPORTANCE: Posterior fossa endodermal cysts are rare. They are located in the midline, in ventral or ventrolateral locations, or intrinsic to the neural axis. Accordingly, various theories of embryogenesis have been proposed. We report the first case of an extradural, dorsolaterally situated endodermal cyst. CLINICAL PRESENTATION: An adult male patient presented with a short history of headache and cerebellar ataxia. Neuroimaging revealed an extra-axial cystic posterior fossa mass. INTERVENTION: An entirely extradural cyst was found and was totally excised. Immunohistochemistry confirmed the diagnosis of endodermal cyst. CONCLUSION: The extradural, dorsal location of the endodermal cyst suggests gaps at the cranial end of the notochord causing ectodermal-endodermal adhesions during early gastrulation and the persistence of endodermal remnants in the dorsal mesenchyme of the blastemal cranium. The literature is reviewed, and proposed theories of embryogenesis are discussed.

Spontaneous fluctuation in the size of a midline posterior fossa arachnoid cyst.

Arachnoid cysts of the posterior fossa are rather uncommon compared with their supratentorial counterparts. Spontaneous disappearance of middle cranial fossa arachnoid cysts has been reported but there are none in the English literature on the spontaneous fluctuation in size of a posterior fossa arachnoid cyst. We present a 41-year-old male, with a midline posterior fossa arachnoid cyst, which appeared to lessen in size spontaneously with complete disappearance of symptoms and then enlarged with a worsening in neurological status.

A petrous bone artefact on MRI: technical note.

We report an artefact seen on MRI in a patient with a cerebellopontine angle syndrome due to an epidermoid cyst. Even minute nonferromagnetic metallic particles such as gold can cause large artefacts.

Symptomatic pneumocephalus after transsphenoidal surgery.

BACKGROUND: Symptomatic pneumocephalus after transsphenoidal surgery, though reported, is a rare phenomenon. We report three cases of pneumocephalus in a series of 300 transsphenoidal operations for sellar/suprasellar mass lesions done over the past 12 years. METHODS AND RESULTS: Three cases of symptomatic pneumocephalus occurring after transsphenoidal surgery are presented to illustrate the causative factors, methods of prevention, and management. In case 1, an intraoperative cerebrospinal fluid (CSF) leak occurred and drainage of CSF through a lumbar subarachnoid drain resulted in pneumocephalus, in spite of repair of the sellar floor. In case 2, partial excision of tumor and subsequent reduction of intracranial pressure due to a ventriculoperitoneal (VP) shunt led to pneumocephalus. In case 3, radiotherapy-induced shrinkage of a partially excised tumor resulted in pneumocephalus. The sellar floor had not been repaired in cases 2 and 3 as there was no intraoperative CSF leak and only a partial excision had been done. Conservative management failed in the two patients in whom it was tried. Repair of the sella and sphenoid sinus had to be done in all three cases. CONCLUSIONS: Repair of the sellar floor should be done after a transphenoidal approach in all cases, even when no intraoperative leak has been identified and only a partial excision of tumor has been done. Once pneumocephalus has occurred, the sellar floor and sphenoid sinus should be repaired early before reducing the intracranial pressure (ICP) by tapping ventricular air and draining or diverting CSF.

Clinicoradiological and pathological correlations in patients with solitary cysticercus granuloma and epilepsy: focus on presence of the parasite and oedema formation.

A study of the clinical, radiological, and pathological correlations in 43 patients with solitary cysticercus granuloma and epilepsy focused on factors that might help in predicting the presence of the parasite in the granuloma and those that might influence the formation of oedema around the granuloma. The duration of symptoms (< six months and > or = six months) and CT morphology of the granuloma (ring and disc, type A; nodular lesion, type B) were studied as factors that could possibly predict the presence of the parasite in the granuloma. The influence of sex of the patient and the presence of a neutrophilic response in the granuloma on the intensity of oedema around the lesion as seen on CT was also studied. The pathological features were studied in the excised granulomas. The intact or degenerated form of the cysticercus was evident in 22 of 43 specimens. Neither the duration of seizures (P = 0.17) nor the type of lesion on CT (P = 0.16) was predictive of the presence of the parasite in the granuloma. The sex of the patient (P = 0.51) and the neutrophilic response in the specimen (P = 0.73) did not correlate with the degree of oedema on CT indicating that neither of these host factors was a major determinant of oedema production. The findings point to the varied and unpredictable natural history of solitary cysticercus granulomas and the complex nature of host-parasite interactions in individual patients. The inability to predict the presence of the parasite in the granuloma on the basis of the clinical or radiological features precludes a selection of patients with such lesions for cysticidal drug treatment.

Differentiating solitary small cysticercus granulomas and tuberculomas in patients with epilepsy. Clinical and computerized tomographic criteria.

It is commonly believed that differentiating solitary small cysticercus granulomas and small tuberculomas in patients with seizures is difficult without resorting to an excision biopsy. The aim of this study was to formulate clinical and computerized tomography (CT) criteria to distinguish these two entities in patients with epilepsy. Toward this end, clinical and CT data from six consecutive patients with histologically proven small solitary tuberculomas and 25 consecutive patients with histologically proven solitary cysticercus granulomas were compared. Evidence of raised intracranial tension and a progressive focal neurological deficit was seen only in patients with tuberculomas (two of six cases). All tuberculomas were greater than 20 mm in size and five of the six were irregular in outline. Only tuberculomas were associated with a midline shift on CT (four of six cases). All cysticercus granulomas were less than 20 mm in size and 24 (96%) of the 25 were regular in outline, conforming to one of two characteristic patterns. No cysticercus granuloma was associated with a midline shift. Based on the above clinical findings (evidence of raised intracranial tension and a progressive neurological deficit) and CT criteria (size, shape, and association with a midline shift), it is possible to separate these two entities in a majority of patients with seizures and with a single small lesion on CT.

Ascorbic acid and focal cerebral ischaemia in a primate model.

Neuronal cell damage following ischaemia is postulated to be due to free radical induced lipid peroxidation, and ascorbic acid is supposedly an important non-enzymatic scavenger of such free radicals. This study was undertaken to evaluate the protective effect of ascorbic acid on the brain in a primate model after focal cerebral ischemia. Consumption of ascorbic acid in the monkey brain following ischaemia and its effect on macroscopic infarct size as demonstrated by 2, 3, 5, Triphenyl tetrazolium chloride (TTC) staining were used as parameters. The monkeys in the treated group were given 1 gram ascorbic acid parenterally every day for six days. The mean level of total ascorbic acid in right basal ganglia was 35.1 +/- 4.2 micrograms/mg of protein in the treated group as opposed to 22.9 +/- 2.1 micrograms/mg of protein in the nontreated group both before ischaemia. After right middle cerebral artery occlusion to produce focal cerebral ischaemia, the total ascorbic acid in the right basal ganglia 2 hours post ischaemia was 13.3 +/- 3.1 micrograms/mg of protein in the treated group as opposed to 9 +/- 1.6 micrograms/mg of protein in the untreated group. The average consumption of total ascorbic acid was 21.8 micrograms/mg of protein in the treated group and 13.9 micrograms/mg of protein in the nontreated group. Macroscopic infarct size as determined by TTC staining in the right cerebral hemisphere was 11.7 +/- 6.9 in treated group whereas it was 24.4 +/- 4.4 (expressed as percentage of right hemisphere) in the non-treated group. There was significant reduction in the size of the infarct in the treated group.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary Ewing's sarcoma of the skull. A report of two cases.

We report two cases of primary Ewing's sarcoma of the skull. The literature contains previous reports of only four such cases.

Intracranial aspergillus granuloma.

Intracranial aspergillus granulomas over a 12-year period were studied retrospectively. Three distinctive types of presentations were seen, namely nine patients had the rhinocerebral form with primary involvement of the air sinuses and secondary involvement of the skull base, cranial nerves and brain, three patients with the apparently purely intracranial form presented like any other space occupying lesion and one patient who had a 'stroke-like' presentation had a parasellar lesion. None of these patients were immunocompromised. The clinical features, and the medical and surgical options are discussed together with outcome.

Anterior communicating artery aneurysms : the direction of aneurysm and its bearing on surgical outcome.

A series of 44 consecutive patients with aneurysms of anterior communicating (A. Com.) artery during an 8 year period, during which 80 patients with 91 aneurysms were operated, is being reported. It comprised 55.0 percent of all aneurysms operated during this period. Eight patients (18.2 percent) had multiple aneurysms. Twenty seven A.Com. aneurysms (61.4 percent) were directed anteroinferiorly, 8 aneurysms (18.2 percent) posterosuperiorly, 6 aneurysms (13.6 percent) superiorly and 3 aneurysms to the side. Twenty nine. A.Com. Aneurysms (65.9 percent) arose at dominant A1-A.Com. junction. All cases were treated by direct surgery. The results are encouraging, though mortality overall was 15.9 percent. Good grade cases made satisfactory recovery. Direction of aneurysm at AC-A.com. and its bearing on surgical outcome is stressed. Most of the posterosuperior pointing aneurysms presented in poorer grades and outcome was unsatisfactory. In comparison most of anteroinferiorly pointing aneurysms presented better grades and results were satisfactory.

Multicentric gliomas: two case reports and a review of the literature.

Multicentric gliomas are among the rarest of multiple intracranial neoplasms. Two biopsy proven cases are described with a review of the literature. Both cases had supratentorial and posterior fossa lesions. Computed tomography was performed in both cases, open and stereotaxic biopsy was done in one case and open biopsy of both lesions in the other case. The major problem in multicentric gliomas is the management. The two cases were managed differently based on the clinical condition and the location of the lesions.