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Primary intraventricular haemorrhage (PIVH) is rare. Dural arteriovenous fistula causing PIVH is extremely rare. We report a case of a 17 year old boy who presented with left hemiparesis, left lower motor neuron facial palsy and ataxia. His computed tomography head revealed primary intraventricular hemorrhage. Catheter super selective angiography revealed a dural arterio venous fistula with arterial feeder arising from the middle meningeal artery as well as from the inferior marginal tentorial artery. Glue injection led to successful disappearance of the fistula and eventual clinical recovery.

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AIM: Moyamoya disease (MMD) is a slowly progressive bilateral stenocclusive process of the distal internal carotid and proximal portions of the anterior and middle cerebral arteries and the formation of an abnormal vascular network at the base of the brain. The purpose of this retrospective study was to identify clinical features, salient features, radiological features and yield of diagnostic cerebral angiography in MMD. MATERIALS AND METHODS: We analyzed the records of 26 patients with MMD evaluated and treated at our institute from August 2010 until March 2013. Diagnosis of MMD was made on the basis of features of angiographic findings. Cerebral angiography showed typically fine network of vessels at the base of the brain with puff of smoke appearance suggestive of MMD. CT angiography (CTA) was done in 25 (96.15%) patients where as Digital substraction angiography (DSA) was done in 18 (69.23%) patients. RESULTS: Out of the 26 patients 13 were in the pediatric age group and 13 were adults. At presentation 14 patients had infarcts and 10 patients had hemorrhages. Among the hemorrhagic group 20% had isolated intracerebral hemorrhage (ICH), 50% patients had ICH with intraventricular extension (IVE) and 30% patients had primary intraventricular hemorrhage (PIVH). 50 % of the patients had involvement of the posterior circulation. CONCLUSION: Posterior circulation involvement is frequent in MMD. Though parenchymal bleed with/without intraventricular extension is the usual presentation of hemorrhagic MMD, isolated intraventricular hemorrhage could also be the mode of presentation.

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BACKGROUND: Primary intraventricular hemorrhage (PIVH) is a rare neurological disorder, with bleeding confined to the ventricles only, without recognizable parenchymal or subarachnoid component. AIM: The purpose of this retrospective study was to identify clinical features, predisposing risk factors, etiology, radiological features and yield of diagnostic cerebral angiography in identifying the etiological causes. SETTINGS AND DESIGN: Records of patients admitted in neurology division were analyzed in a tertiary care teaching hospital. MATERIALS AND METHODS: We analyzed the records of 27 patients with PIVH evaluated and treated at our institute from August 2010 to April 2013. PIVH was diagnosed as hemorrhage in the ventricles only, detected by computed tomography scan without evidence of intraparenchymal, subarachnoid hemorrhage or intraventricular hemorrhage associated with trauma. CT angiography (CTA) alone was done in 10 patients (37.03%), digital subtraction angiography (DSA) in 2 patients (7.4%) and both CTA as well as DSA was done in 15 patients (55.5%). STATISTICAL ANALYSIS USED: Categorical and continuous data were analyzed using SPSS version 17. RESULTS: 17 (62.96%) patients were females and 10 (37.03%) were males with ratio of F:M= 1.7:1. Headache was the commonest mode of presentation (85.18%). Hypertension was most common predisposing factor (29.62%) followed by arterio-venous malformations (AVMs) (25.92%), moyamoya disease (MMD) (11.11%), lenticuostriate artery aneurysm (LSA) (11.11%), arterial dissections (7.4%) and dural arteriovenous fistula (dAVF) (3.7%). CONCLUSIONS: PIVH is rare and hypertension is important predisposing factor. Yield of cerebral angiography is high in diagnosing the etiology. AVMs and other rare etiological causes like MMD, LSA aneurysm, arterial dissection, and dAVF should be kept in mind with a high index of suspicion and warrants cerebral angiography in them, as some of the causes are potentially treatable.

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Hereditary amyloid polyneuropathies are rare, heterogeneous group of autosomal dominant disorders and deserve special attention because of its rare presentation, multisystem involvement and significant therapeutic implications if diagnosed early. We report a male patient of hereditary amyloid polyneuropathy from North West India with peripheral nerve, autonomic nervous system, vitreous and cardiac involvement.

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Spinal neurenteric cysts are rare congenital cysts of endodermal origin. A 34-year-old man presented with burning feet syndrome lasting two years. Magnetic resonance imaging (MRI) revealed an intradural extramedullary cystic mass extending from L2 to L5 causing severe compression of the terminal portion of the conus medullaris and the filum terminale with displacement to the right side. A tethered cord and filar lipoma were also present. The cystic mass and filar lipoma were resected and the tethered cord released. Histopathology confirmed a neurenteric cyst. This case is reported in view of its peculiar presentation.

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A 37-year-old man presented with recurrent secondary generalised seizures from right focal onset. An MRI scan of the brain revealed multiple contrast enhancing lesions mainly involving the frontal, parietal and temporal regions. The left frontotemporal lesion was biopsied and histopathology confirmed it to be a rare case of adult Langerhans cell histiocytosis. He was given a short course of oral corticosteroids. Two years later his clinical condition was much improved on antiepileptic therapy alone, and repeat MRI brain and MR spectroscopy showed significant resolution of the lesions.

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A 37 year old man presented with recurrent secondarily generalized seizures from right partial onset since December 1999, MRI scan of brain (contrast study) revealed multiple enhancing lesions predominantly involving frontal, parietal and temporal regions. Left frontotemporal lesion was biopsied and histopathology confirmed it to be rare case of adult Langerhans cell histiocytosis. He was given short course of oral corticosteroids. Two years postoperative course on antiepileptic therapy alone is uneventful and repeat MRI brain and MR spectroscopy showed significant resolution of lesions.

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Spinal neurenteric cysts are rare congenital cysts of endodermal origin. A 34 years old man presented with burning feet syndrome of two years duration. Magnetic resonance imaging (MRI) revealed an intradural extramedullary cystic mass lesion extending from L2 to L5 vertebrae causing severe compression and displacement of terminal portion of conus medullaris and filum terminale to the right side. Additionally, tethered cord and filar lipoma were also present. Cystic mass and filar lipoma were resected along with release of tethered cord. Histopathology confirmed a neurenteric cyst. This case is reported in view of rare occurrence and peculiar presentation.

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All patients admitted with provisional diagnosis of an encephalitic illness over a period of 30 months, were studied. Special investigations included CSF analysis, EEG, CT scan and MRI. Herpes simplex virus (HSV) antibody estimation in CSF and blood was done simultaneously using ELISA. Patients with diagnosis of cerebral venous thrombosis, cerebral malaria, tubercular meningitis etc, who resembled herpes simplex encephalitis (HSE), were excluded systematically with relevant investigations. 28 patients showed electroencephalographic, serologic and/or neuroradiological evidence of herpes simplex encephalitis. Males were affected more than females. Age ranged from 4 years to 65 years. Main clinical features included altered sensorium (100%) and seizures (89%). Serological test for HSV antibody in CSF and blood was positive in 14 patients. Fronto-temporal localisation was seen in EEG of 18 patients. CT and MRI were fairly characteristic with bilateral asymmetric fronto-temporal lesions. Patients with mild disease and who reported earlier responded well to treatment with acyclovir. Mortality was higher if treatment was delayed or if the disease was severe. Delayed treatment even in less severe cases produced neurological deficit in many survivors. Despite limitations of non-availability of CSF-PCR and serial estimation of HSV antibodies, the study is an attempt to highlight the value of high index of suspicion of HSE on clinical grounds, systematically excluding cases with different aetiologies resembling HSE and planning early antiviral therapy to reduce both mortality and morbidity associated with this fatal disease.

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Creutzfeldt-Jakob disease (CJD) is a degenerative process of the brain, induced by novel infectious agent or prion, and is usually characterized by a rapidly progressive dementia in association with myoclonus. However different patterns of disease presentation have been identified. The authors describe three probable cases of CJD. None of them had positive family history or any known modes of iatrogenic transmission. Interestingly, all the cases presented like a stroke. This is the first series of CJD cases from this part of country.

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Global and regional left ventricular performance was assessed by multigated radionuclide technique in thirty patients sustaining acute myocardial infarction on two occasions during in-hospital phase. Thirteen (76.5%) of the seventeen patients with anterior infarction had depressed initial left ventricular ejection fraction compared with seven (53.8%) of the thirteen patients with inferior infarction. From initial to discharge study, change in ejection fraction was statistically insignificant in either group of infarctions. All except three (10%) patients had regional wall motion abnormality on initial evaluation with little subsequent alteration. Our data demonstrates that ejection fraction changes variably during the course of illness, and location of infarction has profound effect upon degree of left ventricular dysfunction. Assessment by non-invasive radionuclide technique may have prognostic implications.

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Dental care plans grew in prominence from 1980 to 1986; plan cost control measures, as well as plan benefits, kept pace with the rising cost of dental care.

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