Browsing by Author "Bastola, P"
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Publication Alport’s syndrome(Kathmandu University, 2010) Bastola, P; Joshi, SN; Chaudhary, M; Shah, DNAbstract Alport’s syndrome (Haemorrhagic Familial Nephritis) is a rare syndrome. It encompasses a group of heterogeneously inherited disorders involving the basement membrane of the kidney frequently involving the cochlea and the eye. We describe here the detailed ocular findings and the systemic problems of a case of Alport's syndrome in a 30 years male from Nepal. The current understanding of the clinical features and aetiopathogenesis are also discussed. Key words: Alport’s syndrome, Anterior lenticonus, Oil droplet sign, Anterior capsular cataract, Perimacular flecksPublication Schwartz - Jampel syndrome(Kathmandu University, 2010) Bastola, PABSTRACT This is a report of a very rare case of Schwartz Jampel syndrome, with few unusual findings, in a 13 years girl from Nepal, who concurrently also had superotemporal subluxation of the crystalline lens along with blepharophimosis syndrome. Key Words blepharophimosis, genetic mapping , schwartz and jampel, subluxation of lens,Publication The role of the injection Botulinum Toxin A in cases of Essential Blepharospasm Syndrome, Hemifacial Spasm and Meige’s Syndrome(Kathmandu University, 2010) Bastola, P; Chaudhary, M; Agrawal, JP; Shah, DNABSTRACT Background Benign Essential Blepharospasm Symdrome is a neuropathologic disorder. The cause of blepharospasm is multifactorial. It is unlikely that a single defect in this elusive control centre is the primary cause of this disease. Objectives To evaluate the role of botulinum toxin A in treating cases of Essential Blepharospasm Symdrome, Hemifacial Spasm and Meige’s Syndrome, and to assess orbicularis oculi muscle post treatment, in the patients who attended neuro-ophthalmology clinic and the general outpatient department of BP Koirala Lions Centre for Ophthalmic studies. Methods A prospective, interventional study was carried out on all the patients of Essential Blepharospasm Symdrome, Hemifacial Spasm and Meige’s syndrome who underwent treatment with botulinum toxin A in BP Koirala Lions Centre for Ophthalmic studies during a study period of one and half years. Pre- treatment grading of the spasm was done with Jankovic spasm grading and post treatment response was seen with assessment of orbicularis oculi muscle and improvement in functional impairment scale. Relevant findings were noted. Results A total of 40 cases were enrolled in the study. The mean Jankovic spasm grading in cases of essential blepharospasm, hemifacial spasm and Meige’s syndrome was 3.61 (+ Standard deviation 0.50, range 3-4), 3.21 (+Standard deviation 0.63, range 2-4) and 3.67 (+- Standard deviation 0.57, range 3-4) respectively. The mean value for reappearance of significant spasms (in months) in cases of essential blepharospasm, hemifacial spasm and Meige’s syndrome was 4.3 (+ Standard deviation 1.6, range 2.0-6.5), 5.8 (+Standard deviation 1.4, range 3-8) and 4.5 (+ Std.deviation 2.8, range 2.5-6.5) respectively. Blepharoptosis was the commonest complication accounting for 66.6% of the complications. Conclusions The movement disorders like Essential Blepharospasm, Hemifacial Hpasm and Meige’s syndrome are treated by different modalities. An acceptable and effective treatment modality has been a long felt need in these cases. Our study has shown that injection of botulinum toxin A has been a safe and effective method of treating these cases in Nepal. Key Words essential blepharospasm syndrome, hemifacial spasm, jankovic spasm grading, meige’s syndrome