Wilson's disease (WD) is an autosomal recessive disease involving a defect of copper transport by the hepatic lysosomes. It leads to excess copper deposition in the liver, the brain, the kidneys and the skeletal system, affecting most commonly children or young adults and running an invariably fatal course if not adequately treated by de-coppering therapy. The last century has witnessed several changes, notable among these are: Increased awareness, improved diagnostic facilities leading to earlier recognition even in the pre-symptomatic phase, clear distinction from its mimics, aggressive therapeutic approaches owing to availability of effective treatment and an overall reduction in the morbidity and mortality. It is widely acknowledged that the disease is not as rare as once believed. Sir SAK Wilson published his landmark article in 1912, but it was only in 1968 that the first patient of WD was reported from our country. Publications from India on WD have focused on phenotypic characterization, documentations of lesser recognized aspects of the disease e.g. seizures, behavior abnormality, speech and cognitive impairment, sub-clinical affection of visual pathway, heart and autonomic function and pre-symptomatic detection. Attempts have been made to understand the clinical heterogeneity of the disease through identification of biochemical and immunological markers, magnetic resonance imaging, neuropathological study and genetic analysis for novel and/or known mutations. Assessment of impairment and severity and effect of various therapeutic interventions namely zinc sulphate on the long-term outcome and quality of life have also been studied. Nevertheless, clinicians often face difficulties in long-term care of these patients. Diagnostic errors leading to delay in diagnosis and initiation of treatment are common, even in patients with positive family history. There is no consensus regarding therapeutic protocols since the use of penicillamine, once a 'gold standard' for treatment, has been debated by experts. Mortality and morbidity of this potentially treatable disease and nonavailability of medications to the poor patients remain a major area of concern.

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Background: Data are conflicting concerning the association between ischemic stroke and methylenetetrahydrofolate reductase (MTHFR) C677T mutation. Studies addressing this matter in developing countries are limited. Aim: This study was undertaken to evaluate MTHFR C677T gene polymorphism as a possible risk factor in patients with ischemic stroke in Iraq. Settings and Design: A case-control study in a major teaching hospital in Northern Iraq. Materials and Methods: Study population included 70 patients with ischemic stroke diagnosed by computed tomography (CT) or magnetic resonance imaging (MRI) and 50 controls matched by age and sex. All the patients and controls had detailed neurologic examination and blood sugar, lipid profile, total homocysteine, as well as, MTHFR gene analysis. The MTHFR C677T mutation status was detected in the amplified products using reverse hybridization to specific mutant and wild oligonucleotide probes by a colorimetric microwell plate method. Statistical Analysis: Mann-Whitney U test and Chi-square tests were used to find the significance. Results: The median age of the patients was 60 years and 54% were males. The MTHFR C677T gene analysis detected TT genotype in 20% of patients and in 6% of controls and CC genotype in 37% of the patients and in 54% of the controls. The calculated risk of ischemic stroke in the subjects with TT genotype was 4.85 times more than the subjects with CC genotype (P=0.03). Serum homocysteine level was significantly higher in the patients than the controls (P=0.02). The serum homocysteine levels were significantly higher in those with TT and CT genotypes when compared to those with CC genotype (P < 0.001 and P=0.04, respectively). Conclusion: In the Iraq population studied MTHFR C677T TT genotype was a significant risk factor for ischemic stroke and it was related to the increased total homocysteine levels and the risk for ischemic stroke was graded with increasing MTHFR 677T allele dose.

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Background : Pressure ulcers are one of the most common complications in health care settings. Still there are no optimal protocols to manage the pressure ulcers. Aim : To assess the effectiveness of pulsed electromagnetic field therapy (PEMF) in healing of pressure ulcers in patients with neurological disorders. Design : Randomized double blind control trial. Setting : Neurological rehabilitation department in a university research hospital. Participants : Twelve patients (M:F, 9:3) having neurological disorders, with age between 12-50 years (mean 30.16611.32 yrs) and 24 pressure ulcers. Intervention : Six patients with 13 ulcers received PEMF therapy and the remaining 6 patients with 11 ulcers received sham treatment, for 30 sessions (45 minutes each) using the equipment 'Pulsatron'. The frequency of PEMF was set at 1 Hz with sine waves and current intensity of 30 mili ampere. Whole body exposure was given in both the groups. Outcome Measures : Bates-Jensen wound assessment tool (BJWAT) score was used as main outcome measure and scores at the end of session were compared with initial scores and analyzed. Similarly National Pressure Ulcer Advisory Panel (NPUAP) scores were compared and analyzed as secondary outcome measure. Results : Thirteen ulcers were in stage IV and 11 were in stage III at the start of the study. Significant healing of ulcers was noted, BJWAT scores, in both the treatment and sham groups (P < 0.001 and 0.003 respectively) at the completion of the study. However, when comparing between the groups, healing was not significant (P = 0.361). Similarly trend was noted with NPUAP scores with no significant difference between the treatment and sham groups (P = 0.649) at the completion of study. Conclusions : No significant difference in pressure ulcer healing was observed between PEMF treatment and sham group in this study.

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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.

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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 structureal 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 abnormalties 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.

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Background : Tuberculosis is endemic in developing countries. However, calvarial tuberculosis is rare, even in areas where tuberculosis is endemic. In the literature, only few case series of calvarial tuberculosis have been reported. Aim : To report a case series of 11 patients with calvarial tuberculosis, and discuss their presentation and management. Materials and Methods : This study is a retrospective analysis of case records of 11 patients with calvarial tuberculosis treated between 2001 and 2005 in a tertiary hospital. Clinical features, radiological findings, surgical and medical management, and outcomes were reviewed. Results : Of the 11 patients, seven were male and the age ranged between 1 and 25 years (mean 15.09 years). The mean duration of symptoms was 2.9 months (range 1-5 months). The most common presenting features were scalp swelling, discharging sinus, and pain. Computed tomography (CT) scan of brain showed punched out bony defect, with a peripherally enhancing extradural collection in most of the cases. Ten patients underwent surgical excision of necrotic bone and granulation tissue with primary closure of the scalp flap and antituberculous therapy. One patient is being managed with antituberculous therapy only. Of the 10 patients treated surgically and with antituberculous therapy, nine recovered well and one died of tuberculous meningitis and hydrocephalus. The patient being treated with antituberculous therapy is under follow-up. Conclusion : A high index of suspicion and knowledge is required for early diagnosis of calvarial tuberculosis. Surgery and antituberculous therapy remains the mainstay of treatment.

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Background : Ischemic stroke is a frequent heterogeneous multifactorial disease. A number of genetic mutations and environmental factors have been implicated. A polymorphism in the gene for methylenetetrahydrofolate reductase (MTHFR) has been reported to be associated with hyperhomocysteinemia a risk for atherosclerotic vascular diseases. Aim : A cross-sectional study was performed to determine the relationship between the gene polymorphism for MTHFR and ischemic stroke in type 2 diabetes mellitus. Materials and Methods : Of the 215 unrelated patients with type 2 diabetes mellitus recruited, 119 patients had ischemic stroke, Control group included 142 healthy subjects. The genotype of the subjects for the C677T polymorphism of MTHFR was analyzed by using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) followed by HinfI digestion. Plasma total homocysteine (Hcy) levels were measured using high-performance liquid chromatography (HPLC) with fluorescence detection. Results : The genotype distribution did not differ between the control subjects and type 2 diabetic patients (P > 0.05). Plasma homocysteine levels were markedly higher in diabetic patients with TT genotype than those with CC or CT genotype (P > 0.05). Ischemic stroke was more frequently observed in type 2 diabetic patients with the TT genotype than in those with the CT and CC genotype (odds ratio=4.04, 95% CI=1.95-8.34, P=0.0036). Logistic regression analysis revealed that the C677T mutation of MTHFR gene was independently associated with ischemic stroke in type 2 diabetes. Conclusion : MTHFR C677T gene polymorphism associated with a predisposition to hyperhomocysteinemia could constitute a useful predictive marker for ischemic stroke in type 2 diabetic Chinese patients.

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Intraspinal primitive neuroectodermal tumors (PNETs) are extremely rare and are aggressive with poor outcomes. Till date only 41 cases of intraspinal PNET have been reported. Here we report four new cases. Total excision of the tumor was done in three patients and partial excision in one patient. The unusual clinical features were vertebral metasis in one patient and short history of symptoms for four dasy. All had surgical excision, total excision in three patients and gross partial excision in one patient. Three patients were treated with craniospinal radiotherapy and chemotherapy. All the four patients are alive and asymptomatic at 6 to 25 months of followup.

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Neuroimaging is fundamental to stroke diagnosis and management. Non-contrast computed tomography (NCCT) has been the primary imaging modality utilized for this purpose for almost four decades. Although NCCT does permit identification of intracranial hemorrhage and parenchymal ischemic changes, insights into blood vessel patency and cerebral perfusion are limited. Advances in reperfusion strategies have made identification of potentially salvageable brain tissue a more practical concern. Advances in CT technology now permit identification of acute and chronic arterial lesions, as well as cerebral blood flow deficits. This review outlines principles of advanced CT image acquisition and its utility in acute stroke management.

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Background : Stereotactic radiosurgery is an effective treatment strategy for selected group of patients with cerebral arteriovenous malformations (AVMs). Aim : The aim of this study was to evaluate the obliteration rates, complications, and patient outcomes after Gamma knife radiosurgery for cerebral arteriovenous malformations (AVMs) located in eloquent regions of the brain with an emphasis on neurological morbidity. Materials and Methods : Between 2000 and December 2005, 37 patients with AVMs in eloquent locations (sensory, motor, speech, visual cortex, basal ganglia, and brain stem) underwent stereotactic radiosurgery. We retrospectively reviewed the clinical data of these patients to asses the outcomes. Of the 37 patients, only two patients had prior embolization. Three underwent prospective staged volume radiosurgery. Two patients needed redo-radiosurgery for residual AVM. Mean target volume was 9.1 cc. Three lesions had nidus volume more than 20 cc. Average marginal dose was 18.75 Gy. The median duration of follow-up was 23 months (range, 6-60 months). 15 patients had follow-up of more than 36 months. Results : A total of 15 patients had follow-up of more than 36 months, thus available for evaluation of angiographic obliteration rates. Complete angiographic obliteration was documented in seven patients (46.7%). Four patients experienced hemorrhage during the latency period. One patient who had subsequent hemorrhage on follow-up developed worsening of neurological deficit. One patient developed significant sensory symptoms which resolved after steroids. No additional clinical deterioration related to treatment was noted in rest of the patients. Conclusions : AVMs located in eloquent and in deep locations can be treated safely with stereotactic radiosurgery with acceptable obliteration rates and minimal morbidity.

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Background : Characteristics of intracranial aneurysms display ethnic variations. Data on this disease from the African continent is scarce and often conflicting. Aim : To describe site, age and gender distribution of intracranial aneurysms among Kenyans. Study Design and Setting : Retrospective study at Kenyatta National Hospital, Kenya. Materials and Methods: All records of black African patients with a diagnosis of intracranial aneurysms seen at Kenyatta National Hospital, the largest referral hospital in the Eastern and Central African region, over the period from January 1998 to December 2007 were examined for site, age and gender distribution. The data gathered were coded, analyzed with SPSS 11.50. Results : Fifty-six cases of intracranial aneurysms were analyzed. The posterior communicating artery was the most affected (35.7%), followed by the anterior communicating artery (26.8%), while the posterior cerebral artery was the least affected (2%). Multiple aneurysms were present in 2%. The mean age at presentation was 50.9 years (range 21-80 years) and the gender distribution was equal. Conclusions : Intracranial aneurysms among Kenyans occur most commonly on the posterior communicating artery, in young individuals, and without gender bias. The distribution differs from that described in the literature and this requires search for risk factors.

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Background : With improvement in neuroimaging, instrumentation and operative microscope optics, and with better understanding of microneuroanatomy, it is now possible to approach intracranial aneurysms of anterior circulation through a small eyebrow incision. Aim : The objective of the study is to highlight the advantages and limitations of transciliary supraorbital keyhole craniotomy for clipping of these aneurysms. Materials and Methods : We present our experience with 55 intracranial aneurysms in the anterior circulation in 52 consecutive patients (23 females and 29 males, age range 22-70 years) operated between 2003 and 2009. All these aneurysms were clipped by a supraorbital transciliary incision and a craniotomy measuring 2.5x1.5 cm. One patient required bilateral keyhole craniotomies for bilateral aneurysms. Results : Of the 52 patients, 37 patients were in Grade I/II, and the rest were in Grade III-IV. Clipping could be done in all the patients, and in twelve patients there was intraoperative rupture of the aneurysm. While there was a learning curve, no limitations were apparent, and none of the patients required revision of the procedure or wrapping. None of the patients had suboptimal clip application. Postoperative check angiogram showed obliteration of the aneurysm in all the patients. Patients with preoperative Grade I/II could be discharged from the hospital within seven days, and cosmetic result was excellent in all the patients. Four patients with preoperative Grade IV died in the postoperative period due to vasospasm. Conclusion : The transciliary supraorbital approach offers clipping of intracranial aneurysm in anterior circulation with low approach related morbidity as compared to standard approach.

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This study was designed to determine the frequency and clinical characteristics of myasthenia gravis (MG) in a large cohort of Persian patients with multiple sclerosis (MS) living in the province of Isfahan. We reviewed the case records of patients with definite MS (McDonald's criteria) registered in the Isfahan MS Society (IMSS) for associated MG. Of the 1,718 patients with MS in the registry, six patients were found to have both MS and MG. The prevalence after excluding one patient with insufficient data, was 291 per 100,000 (0.29%), a higher prevalence than the earlier reports. These results may support the hypothesis that MS and MG share some common immunopathogenic mechanisms.

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Marchiafava-Bignami disease (MBD), a rare disorder most commonly seen in patients with a history of alcohol consumption, involves progressive demyelination and subsequent necrosis of the corpus callosum. Because clinical signs are nonspecific, the role of computed tomography and magnetic resonance imaging is essential to confirm the diagnosis. Early diagnosis with imaging and prompt treatment may improve the prognosis of MBD.

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Background : Apolipoprotein B (ApoB) levels have been shown to be associate with risk of ischemic stroke. The apolipoprotein B gene (APOB) polymorphisms may influence levels of ApoB and risk of ischemic stroke, but whether they are associated with risk of ischemic cerebral infarction (CI) with family history (CIFH) or not is unknown. Aims : To investigate the possible association of the polymorphism of APOB C7673T with CIFH in Han Chinese from Changsha. Settings and Design : The study population included 47 patients with CIFH and 83 patients with cerebral infarction with no family histroy (CINFH). Control population included 100 healthy subjects with no history of ischemic stroke matched for age, sex, and ethnic background. Materials and Methods : The APOB C7673T polymorphism was analyzed by the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Plasma lipids levels were also examined by standard enzymatic methods and enzyme-linked immunosorbent assay (ELISA). Statistics : The statistical analysis was done using SPSS 14.0 software package. A P value < 0.05 was taken as significant. Results : This study showed an association between the APOB C7673T polymorphism and CI, especially CIFH in Han Chinese from Changsha. The T alleles frequency of the C7673T polymorphism was significantly higher in both the CIFH and CINFH groups when compared with the control group (P < 0.01; P < 0.05; respectively). And the T allele frequency in CIFH group (0.17) was higher than CINFH (0.09) group (P=0.056). In both the CIFH and CINFH groups the serum levels of TC and LDL cholesterol were significantly high in the TT and TC genotypes than that in the CC genotype (P < 0.01), while the serum level of HDL cholesterol in the TT and TC genotypes was significantly lower than that in the CC genotype (P < 0.05). Conclusions : The APOB C7673T polymorphism is related to CI, especially CIFH through changing serum lipid levels in a Chinese population.

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Background : Due to overlapping histomorphological features, difference in clinical behavior and treatment response, establishing potential molecular markers to facilitate diagnosis of various genetic subtypes of diffuse gliomas is essential. Aim : To analyze 1p/19q status in diffuse gliomas and correlate it with epidermal growth factor receptor (EGFR) and p53 protein expression. Materials and Methods : 1p/19q status in 43 cases was evaluated by fluorescence in situ hybridization assay. Glial fibrillary acidic protein (GFAP), EGFR and p53 were assessed by immunohistochemistry. Results : Glial fibrillary acidic protein immunopositivity was observed in oligodendrogliomas within minigemistocytes and gliofibrillary oligodendrocytes as perinuclear homogenous blobs. It also highlighted the intermingled reactive astrocytes. Astrocytomas and the astrocytic component of oligoastrocytomas showed a diffuse fibrillary type of staining. 1p and/or 19q loss was seen in 65% (13/20) of oligodendrogliomas and 66.6% (5/9) of mixed oligoastrocytomas. There was one case each of pediatric oligodendroglioma and mixed oligoastrocytoma, none of which showed 1p/19q loss. None of the astrocytomas including two pediatric cases showed this alteration (P < 0.05). p53 was expressed in 57.1% of astrocytomas (8/14), 33% of mixed oligoastrocytomas (3/9) and 10% of oligodendrogliomas (2/20). Majority of oligodendrogliomas (85%; 17/20) and oligodendroglial areas in mixed oligoastrocytomas (77.7%; 7/9) showed a membranous lace-like immunopositivity with EGFR. In contrast, all astrocytomas (Grade II and III) were EGFR negative. Conclusion : Loss of 1p/19q is strongly associated with oligodendroglial phenotype, while astrocytic tumors are more likely to show p53 over-expression. p53 expression and 1p/19q status appear to be mutually exclusive.

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Worldwide, non-contrast computed tomography (NCCT) has been the imaging modality of choice in acute stroke, primarily due to its accessibility and short acquisition time. However, magnetic resonance imaging (MRI), specifically diffusion-weighted imaging (DWI), is much more sensitive to hyperacute ischemic changes. Multimodal MRI studies provide significantly more pathophysiological and prognostic information than NCCT. Finally, perfusion-weighted MRI permits visualization of the extent of the ischemic penumbra in individual patients. MRI-based penumbral imaging appears to be the most promising approach to expanding the thrombolysis population base to include patients with prolonged symptoms. This article describes advances in MRI techniques and their application to acute stroke management.

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Stroke is the third leading cause of mortality worldwide. Combined carotid intima-media thickness (CIMT) is a marker of atherosclerosis and is also a predictor for ischemic stroke. We determined the frequency of CIMT in patients with acute ischemic stroke and in matched controls and also the risk factors for CIMT. Sixty patients with ischemic stroke diagnosed by computer tomography (CT) scan and 50 controls matched by age, gender, diabetes, and hypertension were studied. Subjects in both groups underwent carotid duplex scanning (ACUSON 128 x P/10 machine) with a 7.5 MHz linear superficial array probe in B-mode to determine the CIMT and presence of plaques. The mean age in the patient group was 62 years and 63.3% were males. The average CIMT in the patient group was 0.798 mm and it was 0.6 mm in the control group (P < 0.0001). Patients with carotid plaque had significantly increased IMT (0.95±0.22) when compared to patients without plaques (0.71±0.12) (P < 0.001). When the differences in mean IMT were compared among the different age groups in the patient group, there was significance (P < 0.05). In this study the CIMT was independently associated with increasing age and with the presence of carotid plaques.

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Ischemic stroke is a complex multifactorial disease and approximately 30%, especially in the young, are cryptogenic. In some of the patients with cryptogenic ischemic stroke the underlying risk factor may be a prothrombotic state. We studied 101 patients with ischemic stroke under 55 years of age. All the patients underwent an extensive diagnostic evaluation to determine the cause of stroke. Common variations in the genes encoding factor V, prothrombin, 5,10-methylenetetrahydrofolate reductase, plasminogen activator inhibitor-1, and human platelet alloantigens-1 were evaluated. Of the 101 patients with ischemic stroke, 28 patients had cryptogenic ischemic stroke. At least one of the different genetic polymorphisms investigated was present in 44% patients in the total group and in 48% of patients with cryptogenic ischemic stroke. In this study population under 55 years of age there was no significant difference in the prevalence of various genetic polymorphisms, factor V, prothrombin, 5,10-methylenetetrahydrofolate reductase, plasminogen activator inhibitor-1, and human platelet alloantigens) in patients with cryptogenic ischemic stroke and in patients with ischemic stroke of determined cause.

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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.

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Background : Intraventricular hemorrhage (IVH) is an independent risk factor for both morbidity and mortality in patients with intracerebral hemorrhage and subarchnoid hemorrhage. The pathophysiological mechanisms by which blood within the ventricles causes brain damage are still poorly understood. Settings and Design : We developed a canine (dog) model with long-term survival. Aims : To study the mechanisms of pathological changes associated with IVH. Materials and Methods : The neurological status, cranial computed tomographic findings, and the pathological changes were studied in the dogs with IVH and also in the control dogs, intraventiricular saline injection. Results : In all the dogs in the control group there were no abnormalities in all the three parameters studied. The dogs in the IVH group developed neurological deficits after the blood injection. There was linear relationship between the ventricular volume and blood clot volume in the first week. After the first week, there was progressive enlargement of the ventricular volume, while the clots continued to shrink. There was complete lysis of the clots within 4 weeks. Pathological studies showed distruction of the ependymal lining of the ventricular system, subependymal gliosis and ischemia of the neurons in the subependymal areas, prominently around the aqueduct. Conclusion : Ventricular dilation was the prominent feature following intraventricular injection of the blood. The other pathological features included disruption of ependymal lining, subependymal gliosis, and ischemic necrosis of neurons in the periventricular tissue of the third ventricle, aqueduct, and the fourth ventricle. These pathological may have some role in the ventricular dilatation following IVH.

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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited adult-onset microangiopathy caused by missense mutations in the Notch3gene on chromosome 19. However, common vascular risk factors may additionally modify clinical expression and progression of the disease. The role of various prothrombotic factors has also been implied. We report a case of a middle-aged man with typical clinical, neuroimaging and histological features of CADASIL, but with notably prolonged activated partial thromboplastin time. Hematological investigations revealed severe clotting Factor XII deficiency. This case illustrates that the occurrence of vascular risk factors should not be overlooked in patients with CADASIL.

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Background : Brainstem and cerebellar atrophy are the most important features in magnetic resonance imaging (MRI) in spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD). However, the correlation between brainstem and cerebellar atrophy and the clinical features has not been well studied. Aim : To study the correlation between MRI features of the brainstem and cerebellum and predominant clinical features in SCA3/MJD. Design and Setting : University teaching hospital. Patients and Methods : By using the linear measurement method, we assessed 32 patients with SCA3/MJD to study the correlations between the morphometric data of the brainstem and cerebellum and clinical features: Duration of the disease, age of onset, total international cooperative ataxia rating scale (ICARS) score; total scale for the assessment and rating of ataxia (SARA) score; ICARS subscores, and SARA subscores. Statistical Analysis : Pearson correlation test. Results : There was a significant invese correlation between anteroposterior diameter of the midbrain and pons and total ICARS scores, total SARA scores, ICARS and SARA subscores (r= -0.381~-0.57, P < 0.05 or 0.01) and disease duration (r=- 0.42~-0.51, P < 0.05 or 0.01). Additionally, superoinferior diameter of the cerebellum was inversely correlated with total SARA scores and ICARS and SARA subscores except for ataxia of posture and gait in both scales (r=- 0.37~-0.44, P < 0.05). The superoinferior diameter of the fourth ventricle was inversely correlated with age of onset (r=-0.45, P < 0.05). Conclusion : The effect on the cerebellum and brainstem is related to predominant clinical features in SCA3/MJD patients.

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Multiple peripheral aneurysms of the posterior inferior cerebellar artery (PICA) are extremely rare. We describe a patient with four distinct aneurysms in the peripheral segments of the left PICA succefully treated surgically with an excellent outcome. Pathological examination of the excised aneurysmal sac showed no evidence of infectious etiology.

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The incidence of subarachnoid haemorrhage from intracranial aneurysms in the paediatric age group is extremely rare. Interestingly, occurrence of vasospasm has been reported to be less in comparison to the adults. Both coiling and clipping have been advocated in selected cases. Because of the thinness of the wall of the arteries, utmost care should be taken while handling these arteries during surgery. The overall results of surgery in children have been reported to be better than their adult counterparts. We present four such cases from our own experience. All these children were operated upon, where the solitary aneurysm in each case was clipped and all of them made a good recovery.

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