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We report two brothers of Cockayne syndrome (CS) with progressive growth retardation, microcephaly, bird headed facies with sunken eyes, cutaneous photosensitivity, retinits pigmentosa, sensorineural deafness, spasticity ataxia, neuropathy and intracranial calcifiactions. These clinical with radiological features of cortical and cerebellar atrophy with basal ganglionic calcification and presence of consanguinity in parents and chromosome studies showing sister chromatid exchange in less than 6% strongly supported the diagnosis of Cockyne syndrome and differentiated it from Bloom′s syndrome and xeroderma pigmentosa. Without genetic analysis or tests for defective DNA repair, the diagnosis is mostly clinical.

Background: While many studies have reported reduced volume of hippocampus in late onset depression (LOD), the status of hippocampus sub-regions (anterior/posterior) is yet to be explored. Evaluating hippocampal sub-regions might facilitate better elucidation of the neurobiological basis of LOD., Methods: Twenty five elderly subjects with LOD (mean age=65.28yr, SD=5.73, 15 females) and 20 healthy controls (mean age=65.35yr, SD=5.67, 7 females) were examined using 3-tesla magnetic resonance imaging (MRI). They were also evaluated with Montgomery Asberg Depression Rating Scale (MADRS) and Hindi Mental State Examination (HMSE). We examined the difference in volume of Hippocampal sub-regions between the LOD group and control group controlling for the age, sex and intracranial volume., Results: Left posterior hippocampus volume was significantly smaller in LOD group than the control group (1.01±0.19ml vs 1.16±0.25ml, F=7.50, p=0.009). There was a similar trend for the right posterior hippocampus (1.08±0.19ml vs 1.18±0.27ml, F=3.18, p=0.082). Depression severity (mean MADRS score=20.64±8.99) had a significant negative correlation with volumes of right posterior hippocampus (r=-0.37, p=0.012) and left posterior hippocampus (r=-0.46, p=0.001) in the LOD group., Conclusions: Specific reduction of posterior hippocampus volume and its relationship with depression severity indicates sub region specific hippocampal volumetric abnormalities in LOD. Future studies need to evaluate sub region specific hippocampal volume in LOD longitudinally for better understanding of the pathogenesis of LOD in view of the functional differences between anterior and posterior hippocampus., (Copyright © 2014 Elsevier B.V. All rights reserved.)

The study objective was to comprehensively evaluate drug-naïve, adult patients with Obsessive Compulsive Disorder (OCD) for cortical structure abnormalities in comparison with healthy controls. In this cross-sectional study of case-control design, Magnetic Resonance Imaging (1-mm) was performed in drug-naïve OCD patients (N = 50) & age- sex-, education- and handedness-matched healthy controls (N = 40). We examined cortical volume, thickness, surface area & local Gyrification Index (LGI) through a completely automated surface-based morphometric analysis using FreeSurfer software. OCD symptoms and insight were assessed using Yale-Brown Obsessive Compulsive Symptom (Y-BOCS) check-list and severity scale. Illness severity was assessed using Clinical Global Impression Severity (CGI-S) Scale. OCD patients had significantly deficient volume, thickness and surface area of right anterior cingulate gyrus (ACG). Right lingual gyrus surface area was found to be significantly decreased in patients. Y-BOCS obsession score had significant negative correlation with left frontal pole volume. Y-BOCS compulsion score had significant negative correlations with right ACG volume and surface area and right lateral orbitofrontal cortex LGI. CGI-Severity score had significant negative correlations with right lingual gyrus volume, thickness and surface area as well as right lateral orbitofrontal area. Y-BOCS insight score showed a significant negative correlation with LGI of left medial OFC and left rostral ACG. Identification of novel deficits involving occipital brain regions and first-time observations of relevant correlations between various illness characteristics and cortical measures in OCD patients supports a network involving anterior cingulate, orbitofrontal and occipital brain regions in the pathogenesis of OCD., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Purpose: Epilepsy following solitary cerebral cysticercosis (SCC) is possibly caused by perilesional gliosis, best visualized on magnetization transfer imaging (MTI).This study aims to describe development of gliosis around SCC by prospective serial MTI and to correlate this gliosis with long-term seizure outcome., Methods: We randomized 123 patients with SCC and new-onset seizures to treatment with albendazole plus antiepileptics (treatment), or antiepileptics only (control), and performed magnetic resonance imaging (MRI) scans at 0, 3, 6, 12, and 24 months. Prospective follow-up data regarding seizure outcome up to 5 years later were collected. MRI studies were analyzed for lesion characteristics and perilesional magnetization transfer (MT) hyperintensity., Key Findings: Clinical and radiologic data of 77 patients were analyzed. Demographic and seizure characteristics were similar in treatment and control groups. Clinical data were available up to 64 months after enrollment. At 12 months, 89.5% patients were seizure-free. MTI is more sensitive than routine imaging for detection of perilesional gliosis. Albendazole treatment did not affect imaging or clinical outcome, including development of gliosis. Independent of duration of follow-up, gliosis was associated with more seizures, and with seizure recurrence at 12 months; duration of seizures and antiepileptic therapy was longer. Gliosis was not dependent on seizure type or stage of degeneration at enrollment or persistence/calcification of the lesion., Significance: Perilesional gliosis around SCC helps prognosticate seizure outcome. Poorer outcome in patients with persistent lesions is likely to be related to mechanisms other than gliosis. The lack of effect of albendazole on seizure outcome may be due to its inability to decrease formation of gliosis., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

Liponeurocytoma is not exclusive to the cerebellar or fourth ventricular location. Since its inclusion in the central nervous system tumor classification in 2000, six cases with similar radiological, histomorphological and immunohistochemical features have also been described in the lateral ventricles. In the present study, we report clinical, radiological and pathological findings of three supratentorial and one cerebellar liponeurocytoma from our records, evaluated with an extensive panel of immunohistochemistry, and review published cases in the literature. The immunohistochemical pattern of supratentorial and infratentorial liponeurocytomas are almost identical, which indicates that these tumors are homologous.

Coenurosis, a rare zoonotic disease caused by the larval form of Taenia multiceps (bladderworm) is common in sheep rearing countries, but human infections are rare. Central nervous system involvement produces large giant sized cysts that radiologically closely mimic hydatid cysts. Most human infections resulting in cerebral coenuri have been reported from Europe and Africa. We report two cases of cerebral coenurosis from India, the first in a 55-year-old male presenting with a large cystic lesion in the right parietooccipital region and the second occurring in a 36-year-old male involving the left temporal trigonal region, that radiologically closely mimicked hydatid cyst. Histopathologic examination revealed characteristic features of coenuri with multiple protoscolices invaginating into a large cyst lined by outer cuticular layer. Awareness of this rare parasitic infestation is important to discriminate from the more common hydatid and giant cysticercal cysts.

This study aimed to detect perilesional gliosis around solitary cerebral cysticerci (SCC) by magnetisation transfer imaging (MTI), to compare its incidence between patients administered and not administered albendazole. We prospectively randomised patients with SCC and new-onset seizures to treatment with albendazole plus antiepileptics (treatment), or antiepileptics only (control), and performed MRI scans at zero, three, six, 12 and 24 months. Data were analysed for lesion characteristics, perilesional MT hyperintensity and MT ratios, calculated from the lesion and perilesional area. Eighty-one patients' data were analysed (M-41, F-40; ages 6-52 years). About 13% scolices appeared hyperintense on MTI at baseline. T1-isointense cyst walls and perilesional area showed MT hyperintensity in 30 - 41.4%; this proportion increased over time. Persistently visible SCC and stage of degeneration at enrolment did not predict development of MT hyperintensity. MT ratios (range - 98.75 to 49.79) increased over time and differed significantly from normal parenchyma. No difference in MT ratios was noted between treatment and control groups. Qualitative perilesional MT hyperintensity was more often seen in control group. Perilesional gliosis is present in >20% of SCC at six months, and continues to appear on later scans. Gliosis is independent of lesion persistence and stage of degeneration. Pre- and post-contrast MT imaging is equally useful in detection of gliosis. MT ratios from the lesion and perilesional parenchyma are significantly lower than from normal brain tissue at all stages of degeneration, but increase as degeneration occurs and healing progresses. Albendazole therapy does not affect the formation of perilesional gliosis.

Cerebral toxoplasmosis remains one of the most common focal brain lesions in patients with acquired immune deficiency syndrome (AIDS). Diagnosis is a challenge because on cranial imaging it closely mimics central nervous system lymphoma, primary and metastatic central nervous system (CNS) tumors, or other intracranial infections like tuberculoma or abscesses. A magnetic resonance imaging (MRI) feature on postcontrast T1-weighted sequences considered pathognomonic of toxoplasmosis is the "eccentric target sign." The pathological correlate of this imaging sign has been speculative. Herein we correlate the underlying histopathology to the MR feature of eccentric target sign in a patient with autopsy-proven HIV/AIDS-related cerebral toxoplasmosis. The central enhancing core of the target seen on MRI was produced by a leash of inflamed vessels extending down the length of the sulcus that was surrounded by concentric zones of necrosis and a wall composed of histiocytes and proliferating blood vessels, with impaired permeability producing the peripheral enhancing rim.

Aim: To describe the evolution of imaging characteristics of solitary cerebral cysticercal lesions (SCCL) on serial MRI, and to study the effect of treatment with albendazole., Design: Randomised controlled prospective trial., Methods and Material: 123 patients with new-onset seizures and SCCL on contrast MRI were randomised to treatment with albendazole and followed with up to five serial MRIs., Results: 81 patients (M - 41, F - 40) with mean age of 19.6+/-11.7years and 4 or 5 serial MRI were included in the analysis. Analysis was performed on 356 MRI's. Scolex was seen in 61.9% of patients in postcontrast T1 sequence in the first MRI study, and there was a significant drop in visibility from the next scan onwards. Cyst contents were initially T1-hypointense and T2-hyperintense with inversion on FLAIR in 30.8% and later scans showed T2-hypointensity. Cyst wall characteristics changed significantly from initially T2-hypointensity to later hyperintense rim. Initial scan revealed perilesional oedema in 98.5%, which is resolved by the second scan. Around 17.5% showed subtle perilesional T2-hyperintensity in follow-up scans. Enhancement pattern changed significantly from ring to disc, and later to non-enhancement. Initially, 69.7% lesions were in colloid-vesicular stage. Lesions moved through subsequent stages of cyst degeneration: time needed for this process is described. Imaging characteristics, both on the first and on subsequent scans, did not differ between albendazole and control groups., Conclusions: Evolution of SCCL follows a predictable sequence corresponding to morphologic stages described earlier, taking over a year to complete. Contrast enhancement decreases as degeneration progresses, but some calcific lesions continue to enhance. Albendazole therapy may hasten resolution of inflammation around the lesion but affects neither the morphology of the cysticercus nor the process of degeneration and subsequent healing.

Background: Outcome following Acute Disseminated Encephalomyelitis (ADEM) is variable and there are only limited studies from India., Aim: The study aims to evaluate the predictors of functional outcome in a cohort of patients with ADEM., Setting: Tertiary-care teaching hospital., Materials and Methods: Patients admitted with the diagnosis of ADEM from 1999 to 2004 have been included. Clinical features and radiological findings were evaluated. Functional outcome at discharge was scored using modified Rankin Scale and patients were followed up regularly., Statistical Analysis: Chi-Square test or Fisher's exact test, and Student's t test for comparison of categorical and continuous variables, respectively, and logistic regression for multivariate analysis., Results: Sixty-one patients were evaluated (mean age 22+/-15.9 years, 1-65). Fifty-two patients had preceding febrile illness or vaccination with mean 9.1+/-12.5 days interval to first neurological symptom. Non-specific febrile illnesses were the commonest trigger. Commonest findings were motor signs (n=41), impaired consciousness (n=33), bladder symptoms (n=21), ataxia (n=15), and seizures (n=14). Between adult (mean age 30.1+/-13.1 years, 13-65, n=38), and pediatric (mean age 6.2+/-2.8 years, 1-12, n=23) patients, language disturbances were more common in the latter (P=0.047). MR imaging (n=35) demonstrated lesions mostly in frontoparietal white matter (n=23) and thalamus (n=15). Nine patients expired. Patients with poor functional outcome at discharge more often had impaired consciousness (P=0.038) and seizures (P=0.06). At follow-up (n=25), deficits included motor signs (n=15) and bladder symptoms (n=5)., Conclusions: ADEM has a wide range of neurological presentations and language disturbances are more common in pediatric patients. The presence of impaired consciousness, and possibly seizures, predict poor functional outcome at hospital discharge.

Over the past decade preventive endovascular treatment is increasingly being considered for intracranial aneurysms irrespective of whether ruptured or unruptured. Few studies have dealt with in-vivo characteristics of intracranial aneurysms. We compare the angiographic morphology of ruptured and unruptured intracranial aneurysms using short interval serial DSA. 37 patients with intracranial aneurysms and who underwent at least two digital subtraction angiograms were included in the study. Based on the clinical presentation there were two subgroups of patients, Group A patients presenting acutely with Sub arachnoid haemorrhage (SAH) and Group B patients who had no clinical or imaging features suggestive of bleed. Clinical and serial angiographic data were correlated. Aneurysms in Group A (1.04 mm(3)) were significantly (p=0.010) smaller than in Group B (4.53 mm(3)). Aneurysms in group A showed increase in size and those in Group B showed a decrease in size (p=0.019). Hypertensive patients in both the groups showed a tendency for a decrease in the size of the aneurysms. Aneurysms having stasis at the time of initial angiogram had significantly reduced in size on follow up (p=0.013) at a faster rate (p=0.017). Presence of spasm in adjacent vessels was associated with increase in size of aneurysm on follow up in both Groups. There are significant differences between a ruptured aneurysm and an unruptured one. Ruptured aneurysms are small and show rapid increase in size. The presence of spasm increased the size of the aneurysm in the post rupture period and anti hypertensive medication and stasis were associated with decrease in size.

We report two patients with Marchiafava-Bignami disease (MBD). A 38-year-old male with chronic alcohol abuse developed acute onset cerebellar ataxia and altered sensorium. He was diagnosed to have acute form (Type II) of MBD. Magnetic resonance imaging (MRI) showed extensive lesions involving the corpus callosum in its entire extent and also bilateral corona radiata and centrum semiovale. Corpus callosum had heterogeneous signal changes with ring enhancement. Positron emission tomography scan demonstrated reduced cerebral glucose metabolism diffusely over both the cerebral hemispheres. The second patient was 55-year-old male with chronic alcohol intake developed acute onset vomiting followed by behavioral abnormalities and altered sensorium. MRI showed diffuse lesion involving entire corpus callosum with suggestion of necrosis. Both the patients subsequently recovered, the first patient is back to his previous occupation and the second patient could be rehabilitated with some lighter work in his previous work place. Functional brain imaging may help to understand the pathogenesis of acute MBD and possibly the behavioral manifestations.

Background: Post-concussion syndrome (PCS) associated with mild traumatic brain injury (MTBI) can cause long-lasting disabilities. Magnetic resonance imaging (MRI) evaluation in these patients may demonstrate structural lesions that correlate with functional deficits on neuropsychological testing. However, little is known about the significance of the relationship between structural lesions on MRI, functional deficits on neuropsychological evaluation and outcome in patients with MTBI., Aims: To assess neuropsychological deficits and structural lesions on MRI in patients with PCS following MTBI, and to find correlation between these findings and PCS., Settings and Design: Prospective, observational, cohort study in a tertiary hospital., Materials and Methods: The study cohort included consecutive patients with MTBI (three months or more duration) and PCS. All the patients in the cohort had neuropsychological testing using the National Institute of Mental Health and Neurological Sciences Neuropsychological Battery for head injury and also MRI using T1, T2 and FLAIR sequences. Statistical analysis was done using Fisher's Exact test of significance., Results: All the 20 patients evaluated had neuropsychological deficits. Eleven patients had lesions on MRI. Disturbances of sleep, behavior and memory and abnormalities in tests for mental speed were more frequent in patients with lesions on MRI, but were not statically significant (P = 0.08). Both the test modalities localized lesions predominantly to the frontal and temporal lobes. All the symptoms observed in the patients were associated with prefrontal dysfunction on neuropsychological testing., Conclusions: Prefrontal dysfunction is invariably associated with PCS following MTBI. Structural lesions on MRI may not always be present but when present may influence the degree or severity of the symptoms.

Staphylococcus aureus is the most common bacterial pathogen implicated in pyomyositis. There are increasing reports of community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) infections. The present case report brings out the diverse clinical manifestations of MRSA infection in the form of paraspinal pyomyositis, myelitis, spinal osteomyelitis, and pneumonia. Molecular typing of the organism confirmed the diagnosis. Patient was successfully treated with vancomycin and surgical drainage. Consideration of the possibility of methicillin-resistance and appropriate antibiotic selection is vital in the treatment of serious community-acquired staphylococcal infections.

Background: Lower urinary tract dysfunction (LUTD) in multiple sclerosis has been well documented. However, its occurrence and outcome in acute disseminated encephalomyelitis (ADEM) has only been variably reported., Objective: To evaluate LUTD in ADEM, correlation with other neurological deficits, and outcome., Methods: Patients with ADEM having significant LUTD were evaluated. LUTD was evaluated by symptom analysis, ultrasonography, and urodynamics. Storage symptoms were managed using antimuscarinics and significant voiding dysfunction by catheterization., Results: Of 61 patients, 20 (33%) had LUTD. Voiding dysfunction was more common and 16 patients were in urinary retention. Cystometry demonstrated detrusor overactivity in four patients and underactivity in four patients. Incontinence was reported more often in patients with frontoparietal white matter changes in MR imaging. LUTD was found to be associated with occurrence of paraparesis or tetraparesis, though did not predict functional outcome at discharge. At 3 months follow up, five patients continued to have LUTD and urgency and hesitancy were commonest symptoms., Conclusion: LUTD is common in ADEM, especially in patients with lower limb pyramidal involvement and its causes multifactorial. Presence of LUTD does not influence the functional outcome of patients with ADEM. Recovery may be incomplete and symptoms may persist even after recovery of other neurological deficits.

Cranial magnetic resonance imaging was performed in three cases of acute hyperammonemic encephalopathy with three diverse etiologies: infantile citrullinemia, acute hepatic encephalopathy, and proximal urea cycle disorder. All three patients exhibited diffuse extensive cortical signal changes and swelling. Neurologic outcome was poor in all three cases. Knowledge of the magnetic resonance imaging findings of hyperammonemic encephalopathy may help in early diagnosis and treatment and could influence the neurologic outcome.

Biotin-responsive basal ganglia disease is a rare childhood neurological disorder of uncertain etiology that is treatable if suspected and diagnosed. Only few cases have been reported earlier in literature. We report a case of biotin-responsive basal ganglia disease suspected clinically, corroborated by neuroimaging and a dramatic response to biotin therapy.

No trials to date have focused on long-term seizure outcome in solitary cerebral cysticercal lesion (SCCL), which is believed to produce a relatively benign form of epilepsy. This is a prospective randomized controlled study to evaluate the effect of Albendazole on long-term seizure outcome in patients with MRI-confirmed solitary cerebral cysticercal lesion (SCCL). One hundred and twenty-three patients with new-onset seizures and SCCL on contrast MRI were randomized to treatment with albendazole and followed for up to five years with serial MRI and clinical evaluation. At final analysis 103 patients (M-54, F-49) with a mean age of 18.6+/-10.7 years and follow-up period more than 12 months were included. The mean follow-up duration was 31.4+/-14.8 months (12-64). At one month follow-up more patients receiving albendazole were seizure-free (62% versus 49% for controls). Subsequently there was no significant difference in overall seizure outcome between the two groups. There was no correlation between seizure semiology, albendazole therapy and long-term seizure outcome. Baseline MRI showed active lesions in all; 23% remained active at 12 months with no difference between the albendazole and control groups. Patients whose lesions resolved at 12 months showed better seizure outcome. Reduction in mean cyst area was greater in the albendazole group as compared to the controls and the difference at six months was significant (p<0.05). At three months follow-up perilesional edema also resolved faster in albendazole group (p<0.05). Thus, albendazole did not alter the long-term seizure outcome in patients with SCCL and epilepsy. However, albendazole hastened resolution of SCCL on MRI, but interestingly 23% of lesions were still active 12 months after treatment.

Central nervous system tuberculosis is still one of the leading causes of morbidity in the developing world, and tuberculous abscess is one of its uncommon manifesting forms. It closely mimics a pyogenic abscess clinically, radiologically, and histologically. An accurate diagnosis is imperative due to therapeutic implications. In this study, 21 cases of tuberculous abscesses encountered over a period of 13 years (1995-2007) were reviewed to study the clinical, radiological, and histopathological spectrum of the disease. The presence of palisading epithelioid cells and sheets of foamy histiocytes, enclosing a neutrophillic exudate rich in fibrin with nuclear debris, were clues as to suspicion of a tuberculous abscess. The demonstration of acid fast bacilli in the wall of the abscess or necrotic contents by microscopy or culture is essential to confirm the diagnosis of tuberculous abscess. A high index of clinical suspicion is necessary particularly in countries endemic for tuberculosis to ensure an accurate diagnosis and application of an appropriate therapy.

Subacute sclerosing panencephalitis (SSPE), a post-measles progressive neurological disorder is still common in India because of indifferent vaccination compliance. However, the acute fulminant form of SSPE is extremely rare. An unusual case of fulminant SSPE in an 18-year-old man from south India with an ultra-short course of 19 days presenting with hemiparesis in absence of myoclonus and progressive cognitive decline, is reported. MRI showed frontal and parieto-occipital demyelination extending to nuclear areas. Antimeasles antibodies were demonstrable in CSF and serum with oligoclonal bands in CSF despite normal CSF protein and cell count. At autopsy, unlike classical SSPE, oligodendroglia containing measles viral antigen was sparse despite florid necrotizing leukoencephalitis with acute demyelination. Measles virus was isolated from the brain with hypermutation in M gene confirming the diagnosis. Phylogenetic analysis of the viral genotype indicated that it belonged to D7 genotype which is considered rare in India.

The clinical manifestations and histopathologic and neuroimaging findings in 4 Indian patients with a variant form of mucolipidosis IV are described. The presenting symptoms were psychomotor delay, spastic paraplegia, and mild mental retardation. One patient also had visual deterioration due to optic atrophy. None had corneal or retinal abnormalities. Magnetic resonance imaging in 3 patients showed a uniformly thin corpus callosum in all patients and white matter changes in 2 patients. Electron microscopic examination of skin biopsy specimens revealed storage bodies characteristic of mucolipidosis IV. These patients differ from previously described patients with this disorder in the absence of corneal abnormalities and in their presentation with spastic paraplegia during the second decade of life. Correct diagnosis is needed for genetic counseling, prognostication. and reduction of additional familial burden of this rare disease.

Patients with vitamin B12 deficiency have protean neurological manifestations that are often insidious. Acute onset of cerebellar dysfunction and extrapyramidal manifestations like dystonia and chorea are rather uncommon in adults. We describe a patient who manifested with acute onset of language dysfunction, chorea and ataxia. There was no history of hypertension, diabetes or ischemic heart disease. He had low serum vitamin B12 and elevated serum homocystine levels. He improved dramatically following B12 replacement therapy. Our patient provides insight into the pathophysiological mechanism of this rare manifestation. Further the importance of considering vitamin B12 deficiency, in country like India, where vegetarian food practice is quite common, is being emphasized.

Choroid plexus neoplasms are rare intracranial neoplasms. Significant differences exist in their presentation and management in paediatric and adult populations. The present study aims to study the differences among the paediatric and adult population, various factors affecting the outcome, and the clinical and histological correlation. This is a retrospective study of 47 patients with choroid plexus neoplasms managed at NIMHANS from 1984 to 2004. The case records and images were retrieved and reviewed. The various histopathological features were outlined and histopathology reviewed accordingly. For follow-up, patients were contacted by letter or telephone and the necessary information obtained. Follow-up was available in 41 out of 47 patients. Sixty per cent patients were in the paediatric age group and 40% were adults. Forty-three per cent of children with tumours were less than 1 year of age. The lateral ventricle was the most common site of involvement in the paediatric group compared with the fourth ventricle in adults. Calcification is seen on CT scan more often in papillomas and in adult tumours. Invasion of surrounding parenchyma may be seen in both papillomas and carcinomas. However, in papillomas it is by nests of tumour cells compared with carcinomas wherein invasion is by individual tumour cells. Hydrocephalus is present irrespective of location and size of the tumour. Gross total excision is more feasible in adults. Large tumour size, excessive blood loss, higher incidence of carcinomas result in partial excision of these tumours in the paediatric group. Subdural collections and tumour bed haematomas are more common complications in the paediatric group after resection of tumour. These tumours have significant differences among paediatric and adult groups. Carcinomas are predominantly seen in younger children. Invasion of brain parenchyma by nests of cells does not carry a poor prognosis. The outcomes are better in adults.

Spinal neurenteric cysts are intradural cystic lesions. These represent a part of the spectrum of developmental anomalies. These are rare lesions. Most of the data in literature is review of case reports. This made us evaluate our results of 23 cases and review the literature. This is a retrospective study of 23 patients managed at our institute over 20 years. The slides were retrieved and histopathological features studied. Twenty patients were less than 30 years old and 21 were males. Follow-up was available for 21 patients with mean duration of 71 months (range 2 months to 23 years). The typical presentation was backache with progressive neurological deficits pertaining to the level. Atypical presentations as acute onset, recurrent episodic events and aseptic meningitis were also noted. The cervicothoracic region was the most common site. 16 patients had cyst in intradural extramedullary plane and seven had intramedullary location. Associated vertebral anomalies as hemivertebrae, Klippel-Feil and spina bifida were noted in seven patients. There were two histological types of cysts with no correlation between the type of cysts and associated vertebral anomalies and extent of outcome. Partial excision though had higher risk of recurrence, was not associated with poorer outcome. Dorsal approach is an acceptable route with reasonably good results for this lesion. Spinal neurenteric cysts present at younger age with varied clinical presentations. These are commonly located intradurally ventral to the cord. Histological types have no effect on the outcome. Total excision is the choice of treatment. However, partial excision is a feasible option in intramedullary lesions and when significant adhesions occur. Although associated with higher risk of recurrence, the outcome is still good in these patients on re-excision.

Many viral infections of the nervous system cause stereotyped pathologic features and overlapping clinical and imaging features. Neuroimaging usually offers neuroanatomical localization of the pathology, degree of involvement of the nervous system, and response to therapy during follow up in a few instances. Neuroimaging is a useful adjunct for diagnosis.

Causes of intracranial calcification in children are numerous. This article describes an unusual cause of intracranial calcification and white matter changes in a child, namely, nephrogenic diabetes insipidus. A 4-year-old boy presented with history of polyuria, polydipsia, failure to thrive, and developmental delay. On examination, he had mild dysmorphic features and spastic paraparesis. Evaluation showed findings suggestive of nephrogenic diabetes insipidus. Computed tomography and magnetic resonance imaging revealed calcification and signal changes in the frontal and parietal subcortical white matter and gray white junction in the parietal and occipital lobes. The involvement of the white matter, in addition to the calcification in this disease, is stressed because it may predict the neurologic outcome.

Early and non-invasive evaluation of hydatid infestation of brain and spine is of paramount importance, especially in endemic areas. We present a spectrum of imaging findings in neurohydatidosis with a brief review of literature.

Superior ophthalmic vein thrombosis is uncommon, and bilateral superior ophthalmic vein thrombosis is rarer still. The resolution of bilateral superior ophthalmic vein thrombosis takes a long time. The spontaneous resolution of bilateral superior ophthalmic vein thrombosis is not known and not reported in the literature so far. Here we present MRI of bilateral superior ophthalmic vein thrombosis and its spontaneous resolution.

Introduction: Acute disseminated encephalomyelitis (ADEM) is usually a monophasic illness characterized by multiple lesions involving gray and white matter. Quantitative MR techniques were used to characterize and stage these lesions., Methods: Eight patients (seven males and one female; mean age 19 years, range 5 to 36 years) were studied using conventional MRI (T2- and T1-weighted and FLAIR sequences), diffusion-weighted imaging (DWI) and proton magnetic resonance spectroscopy (MRS). Apparent diffusion coefficient (ADC) values and MRS ratios were calculated for the lesion and for normal-appearing white matter (NAWM). Three patients were imaged in the acute stage (within 7 days of the onset of neurological symptoms) and five in the subacute stage (after 7 days from the onset of symptoms)., Results: ADC values in NAWM were in the range 0.7-1.24 x 10(-3) mm/s2 (mean 0.937 +/- 0.17 mm/s2). ADC values of ADEM lesions in the acute stage were in the range 0.37-0.68 x 10(-3) mm/s2 (mean 0.56 +/- 0.16 mm/s2) and 1.01-1.31 x 10(-3) mm/s2 (mean 1.24 +/- 0.13 mm/s2) in the subacute stage. MRS ratios were obtained for all patients. NAA/Cho ratios were in the range 1.1-3.5 (mean 1.93 +/- 0.86) in the NAWM. NAA/Cho ratios within ADEM lesions in the acute stage were in the range 0.63-1.48 (mean 1.18 +/- 0.48) and 0.29-0.84 (mean 0.49 +/- 0.22) in the subacute stage. The ADC values, NAA/Cho and Cho/Cr ratios were significantly different between lesions in the acute and subacute stages (P < 0.001, P < 0.027, P < 0.047, respectively). ADC values were significantly different between lesions in the acute (P < 0.009) and subacute stages (P < 0.005) with NAWM. In addition, NAA/Cho and Cho/Cr ratios were significantly different between lesions in the subacute stage and NAWM (P < 0.006, P < 0.007, respectively)., Conclusion: ADEM lesions were characterized in the acute stage by restricted diffusion and in the subacute stage by free diffusion and a decrease in NAA/Cho ratios. Restricted diffusion and progressive decrease in NAA/Cho ratios may help in staging the disease.

In developing countries hydatidosis is both a medical and economic problem related to environmental hygiene and healthy veterinary practice. This cestode parasitic infestation, uncommonly involving the nervous system, presents with varied clinical manifestation, at times causing diagnostic dilemmas. Multiple intracranial and spinal hydatidosis is rare. A series of 29 histologically confirmed cases of hydatidosis of neuraxis (21 intracranial and 8 spinal) from South India are presented. Among the 21 cases of intracranial hydatidosis, 12 cases were in pediatric age, while only 1 spinal lesion was noted in a 5-year-old child. The clinical presentation of intracranial lesions was predominantly that of raised intracranial pressure and visual symptoms, while spinal hydatidosis manifested with severe back pain, weakness and sphincter disturbances. The cranial cysts were usually single and uniloculated (12 cases), multiple in 7 and single but multiloculated in 2. In spinal hydatidosis, the cysts are usually multiple and extradural, rare ones being intramedullary and intradural. Based on clinical features and imaging, the differential diagnosis for intracranial lesions were cystic tumors and arachnoid cyst while metastasis and tuberculosis were considered in cases of spinal hydatidosis because of vertebral bony involvement. The majority of the cysts could be surgically resected totally and some were aspirated under control suction and resected. None of the cases had anaphylactic reaction, with no significant post-operative morbidity and no mortality. One intracranial and 2 spinal lesions caused by fertile cysts recurred to undergo repeated surgery.

Summary: P2 segment aneurysms are located on the posterior cerebral artery (PCA) between the junction of the posterior communicating artery with the PCA and the quadrigeminal cisternal part of the PCA. We reviewed our experience with endovascular coiling in such aneurysms. Clinical and pre-procedural data from four patients, referred for endovascular treatment of P2 segment aneurysms, were retrospectively studied for factors influencing post-interventional neurological deficits caused by ischemia of the PCA distal territory. Balloon occlusion was done in three patients and patient tolerance was assessed using clinical and anatomic criteria. Embryologic and anatomic features of the PCA were reviewed. Balloon occlusion test and endovascular coiling of aneurysms was possible in three patients. Control angiogram after embolization showed elimination of aneurysms from the circulation and the distal PCA filled through leptomeningeal anastomoses. One patient deteriorated due to aneurysmal rupture soon after the balloon occlusion test and coiling could not be done. In the other three patients post-intervention CT and MRI images showed PCA territory infarcts in spite of demonstration of good collateral circulation distal to the occluded PCA. In conclusion, P2 aneurysms can be effectively treated by endovascular coiling without a balloon occlusion test. While the balloon occlusion test does not contribute to clinical decision-making it may be associated with potential morbidity and mortality.

Purpose: To study intracranial cestodal cysts using in vivo proton magnetic resonance spectroscopy ((1)H MRS) in an effort to identify metabolite(s) that may help in recognizing the parasitic etiology and, perhaps, viability of such tapeworm cysts. Cestodal infestations of the human central nervous system (CNS)-cysticercosis and hydatidosis-are not rare. Identification of a scolex is considered diagnostic of cysticercosis on imaging. In its absence, however, the features are non-specific., Materials and Methods: Three patients with intracranial hydatid cysts and 13 patients with intracranial cysticercal cysts (four intraventricular, seven parenchymal, and two subarachnoid racemose cysts) were studied on a 1.5-T MR system. In vivo (1)H MRS was performed by multivoxel two-dimensional hybrid chemical shift imaging technique (TE = 135 msec). In vitro (1)H NMR and mass spectroscopy (matrix assisted laser desorption/ionization [MALDI]) were performed on excised cysticercal and hydatid cyst fluid. MALDI spectra for pyruvate and succinate were also obtained., Results: Alanine, pyruvate, and acetate were seen in all the three hydatid cysts. Lactate was seen in racemose cysticercal cysts. A large resonance at 2.4 ppm, confirmed as pyruvate at mass spectroscopy, was seen in 13 cestodal cysts. Pyruvate was not seen in one each of racemose, intraventricular, and parenchymal cysticercal cysts., Conclusion: Pyruvate is the predominant metabolite in cestodal cysts infesting the human CNS. It may be a marker of parasitic etiology and perhaps that of viability of such intracranial cysts., (Copyright 2003 Wiley-Liss, Inc.)

Persistent mirror movements are unwanted movements restricted to muscles homologous to those moved intentionally on the opposite body half. It is rarely observed and the functional MRI findings in a case of persistent mirror movement are described.

Although known, histologically proven pulmonary metastasis from a benign intracranial meningioma is exceedingly rare. We report a case of meningiothelial meningioma producing a solitary pulmonary deposit.

Cryptococcosis is a relatively common mycotic infection of the CNS caused by a ubiquitous saprophytic fungus. We present an unusual case of CNS cryptococcosis in an immunocompetent patient. Florid choroid plexitis resulted in the formation of intraventricular enhancing mass lesions that filled the ventricles and were hyperintense to associated periventricular edema on T2-weighted MR images. We also noted lesions corresponding to microcystic, dilated Virchow-Robin spaces in the basal ganglia that were characteristic of cryptococcal infection.

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.

Infective intracranial aneurysms are relatively uncommon. They develop due to septic embolisation of the vasa vasorum or lumen of the artery, with resultant focal arteritis and necrosis, leading to aneurysm formation. They are an important cause for intracranial haemorrhage. Six cases of infective aneurysms are described. A focus of infection could be detected in all the patients. Surgery was done in three cases, out of which two patients made significant recovery, while one patient died in the immediate postoperative period. Out of the three cases, treated conservatively with antibiotic therapy, there was total resolution on follow up angiogram in two, while one patient was lost to follow up.