Browsing by Author "Rana, PVS"
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Publication Acute Intermittent Porphyria with SIADH and Fluctuating Dysautonomia(Kathmandu University, 2012) Nabin, A; Thapa, LJ; Paudel, R; Rana, PVSABSTRACT Three cases of acute intermittent porphyria are reported. While in first case severe pain in abdomen with intermittent exacerbation was the only presentation, the second patient presented as accelerated hypertension and acute abdominal crises in whom the clinical course was characterized by development of deep coma due to inappropriate secretion of antidiuretic hormone before she made complete recovery. The third patient, initially manifested as acute encephalitic syndrome. After initial improvement, she developed features of acute intermittent porphyria i.e. acute abdomen, neuropsychiatric symptoms, and rapidly progressing acute motor neuropathy leading to respiratory and bulbar paralysis. In addition, she developed severe and fluctuating dysautonomia leading to cardiac arrest and fatal termination. The importance of early diagnosis, recognition of autonomic disturbances, prompt treatment and counseling for avoidance of precipitating factors is stressed. KEY WORDS Acute intermittent porphyria (AIP), Autonomic neuropathy, Dysautonomia, d-aminolevulinic acid (d-ALA)’ Inappropriate secretion of antiduretic hormone (SIADH, Hematin, Porphobilinogen (PBG)Publication Association of Seizure, Facial Dysmorphism, Congenital Umbilical Hernia and Undescended Testes(Kathmandu University, 2012) Thapa, LJ; Pokharel, BR; Paudel, R; Rana, PVSABSTRACT With the advances in neurogenetics association of epilepsy and intellectual disability with chromosomal abnormalities are being increasingly recognized. While onset of seizures with mental retardation at an early age indicate chromosomal abnormality, combination of characteristics facial dysmorphism and congenital abnormalities gives a clue of a particular syndrome. In addition MRI findings may help in confirming the diagnosis. A nine years old boy is presented where early onset seizure, mental retardation, delayed development of speech, presence of facial dysmorphism,, umbilical hernia and undescended testes suggested possibility of chromosomal 6q deletion disorder. Important deletion disorders are discussed and importance of clinical examination is stressed. KEY WORDS Chromosome deletion, Cryptorchidism, Facial dysmorphism, Seizures, Umbilical herniaPublication Congenital cytomegalovirus virus infection(Kathmandu University, 2009) Rana, PVSAbstract A 14 months old male child with psychomotor retardation and hypotonia is reported, where computerized axial tomography revealed multiple calcification and ventriculomegaly secondary to cortical atrophy. Investigation suggested the diagnosis of cytomegalovirus infection. Importance of early diagnosis is emphasized as the potential long term sequelae can be prevented or reduced markedly with available therapeutic options. Key words: Congenital, Cytomegalovirus, Psychomotor retardation, Calcification, Ganciclovir, Hyper immune globulinPublication C–reactive Protein and Early Mortality in Acute Ischemic Stroke(Kathmandu University, 2011) Dewan, KR; Rana, PVSABSTRACT Background There is growing evidence that inflammation plays an important role in atherogenesis. Several studies have shown that C-reactive protein (CRP), an inflammatory marker, is associated with stroke severity and outcome. But limited studies are there which show the relationship of CRP with early mortality i.e within seven days. Objective To study the association of CRP within 24 hours after acute ischemic stroke onset with severity during admission, types of ischemic stroke and outcome. Methods This cross sectional study was done including 100 consecutive cases of acute ischemic stroke admitted to Neurology center of College of Medical Sciences, Bharatpur (Chitwan), Nepal. The cases were classified as per TOAST classification and severity at admission assessed using National Institutes of Health Stroke Scale. C-reative protein (CRP) level was estimated by latex particle agglutination test. Result Thirteen percent patients expired by 7th day. In the expired group, CRP was positive in 15.3 percent, 15.3 percent and 61.5 percent in patients with lacunar, cardioembolic and large artery atherosclerotic infarction respectively (p 0.19). CRP was positive in all 7 patients (53.8%) who had expired with severe NIHS scale (p 0.004). Conclusion High CRP level is associated with stroke severity at admission and is an independent predictor of early seven day mortality after ischemic stroke KEY WORDS atherosclerosis, C-reactive protein, cardioembolic. ischemic stroke, lacunarPublication Early management of acute ischemic stroke: review of literature and proposed guidelines(Kathmandu University, 2004) Rana, PVSNAPublication Early onset myasthenia gravis with atypical features(Kathmandu University, 2008) Agarwal, P; Chapagain, U; Deewan, KR; Rana, PVSAbstract A 14 year old boy with atypical myasthenia gravis is reported. The interesting features of the case were the onset in first decade with progressive weakness of limb muscles simulating limb girdle myopathy, presence of bilateral symmetrical non fluctuating external ophthalmoplegia with ptosis and the absence of diplopia. Differential response to choline esterase inhibitors was clinically apparent. In contrast to excellent improvement in limb weakness with choline esterase inhibitors, there was no improvement in external ophthalmoplegia or ptosis. The possibilities are discussed and literature is reviewed. Key words: Myasthenia gravis (MG), External ophthalmoplegia, Acetylcholine receptor (Ach-R), Acetylcholine receptor antibodies, (Ach-R ab), Ragged red fibers, Muscular dystrophy, Congenital myasthenia gravis, Choline esterase inhibitors, Overlap syndrome