Treatment of refractory amblyopia with levodopa associated with full-time occlusion in the dominant eye / Tratamento da ambliopia refratária com o uso de levodopa associada à oclusão total do olho dominante

Abstract Purpose: To evaluate visual outcomes of levodopa treatment associated with full occlusion of the dominant eye in patients with refractory amblyopia. Methods: A prospective study of 19 attended patients who were subject to treatment with Levodopa and Carbidopa on doses of 0.7mg/kg/day, a ratio of 4:1 divided into three daily doses for 5 weeks, combined with full occlusion (24 hours/day) of the dominant eye. The ophthalmologic exam from previous consultations up to treatment and after 8 weeks of therapy were collected from medical record data. Patients who had completed treatment for more than 12 months were included for complete eye examination. Results: The mean age before treatment with levodopa was 11.0 ± 4.2 years old (varying from 7 to 23 years). The best-corrected visual acuity (Snellen chart) of the amblyopic eye before treatment was 0.24 (0.6 in logMAR) ± 0.16, after 8 weeks of treatment it was 0.47(0.3 in logMAR) ± 0.33, while during the final evaluation it was 0.46 (0.3 in logMAR) ± 0.34. There was a statistically significant improvement in vision after 8 weeks of therapy which was maintained until the final evaluation (p = 0.007). Conclusion: Levodopa/Carbidopa therapyat doses of 0.7 mg/kg/day at a ratio of 4:1 divided in three daily doses, associated with full occlusion of the dominant eye during 5 weeks had a significant improvement on the visual acuity of the amblyopic eye, and persisted up to 1 year after the treatment.

Resumo Objetivo: Avaliar os resultados visuais do tratamento com levodopa associada à oclusão total do olho dominante em pacientes amblíopes. Métodos: Estudo prospectivo de 19 pacientes atendidos e submetidos ao tratamento com levodopa e carbidopa na dose de 0,7 mg/kg/dia e proporção de 4:1, divididos em três doses diárias, durante cinco semanas, combinada a oclusão total (24 horas/dia) do olho dominante. Foram coletados dados do prontuário referentes ao exame oftalmológico da consulta anterior ao tratamento e após 8 semanas de terapia. Os pacientes com término do tratamento com mais de 12 meses foram reconvocados para exame oftalmológico completo. Resultados: A média de idade dos pacientes previamente ao tratamento com levodopa foi de 11,0 ± 4,2 anos (variando de 7 a 23 anos). A acuidade visual melhor corrigida (Snellen) do olho amblíope antes do tratamento foi de 0,24 (0,6 em logMAR) ± 0,16, após 8 semanas de tratamento foi de 0,47 (0,3 em logMAR) ± 0,33 e na avaliação final foi de 0,46 (0,3 em logMAR) ± 0,34. Houve melhora estatisticamente significante da visão após 8 semanas de tratamento que se manteve até a avaliação final (p = 0,007) Conclusão: A terapia com levodopa/carbidopa em doses de 0,7mg/kg/dia na proporção de 4:1 dividida em três doses diárias, associada à oclusão total do olho dominante durante 5 semanas, apresentou uma melhora significativa na acuidade visual do olho ambliópico e persistiu até 1 ano após o tratamento.

Actualización en enfermedad con cuerpos de Lewy / Update in Lewy body disease

La enfermedad con cuerpos de Lewy incluye 2 entidades que podrían ser consideradas variantes clínicas de una misma patología: la demencia con cuerpos de Lewy y la demencia en enfermedad de Parkinson. Con la finalidad de describir correctamente lo que sucede en la evolución de la enfermedad se divide el cuadro en etapa prodrómica y de demencia propiamente dicha. La primera está clínicamente representada por aquel período en el cual, si bien el paciente exhibe algunos signos y síntomas propios de la enfermedad, no reúne criterios de demencia. A pesar de ser difícil de definir y por carecerse todavía de contundentes datos clínicos y biomarcadores, se caracteriza principalmente por deterioro leve selectivo en función atencional ­ visuoespacial, trastorno del sueño REM y disautonomía‒. La segunda etapa está claramente caracterizada en los criterios de consenso del año 2005. Recientemente hemos publicado la validación de un instrumento llamado ALBA Screening Instrument, que permite diagnosticar con alta sensibilidad y especificidad la enfermedad aun en etapas tempranas y diferenciarla de otras patologías semejantes. La tomografía por emisión de positrones (PET) para transportador de dopamina es el procedimiento de referencia (gold standard) del diagnóstico. El tratamiento sintomático con anticolinesterásicos y neurolépticos atípicos favorece una buena evolución de la enfermedad y es fundamental tener en cuenta evitar medicamentos que pueden dañar gravemente a los pacientes como los anticolinérgicos y antipsicóticos típicos. Los avances en el diagnóstico y la difusión del impacto de esta enfermedad en la población contribuirán a generar mayores esfuerzos de investigación para hallar un tratamiento eficaz, preventivo o curativo o de ambas características. (AU)

Lewy body disease includes 2 entities that could be considered clinical variants of the same pathology: Dementia with Lewy bodies and Parkinson's disease Dementia. Two stages of the disease are described in this review, a prodromal stage and one of explicit dementia. The first one is clinically represented by that period in which, the patient exhibits some typical features of the disease, but not dementia criteria. Despite being difficult to define the prodromal stage and that strong clinical data and biomarkers are still lacking, there is evidence to characterize it mainly by mild selective impairment in attention and visuo-spatial function, REM sleep disorder and dysautonomia. The second stage is clearly characterized in the known consensus criteria of 2005. We have recently published the validation of an instrument called ALBA Screening Instrument which showed a high sensitivity and specificity for diagnosis of the disease even in the early stages. It´s useful to differentiate the disease from other similar pathologies. Positron Emission Tomography for dopamine transporter is the gold standard of diagnosis in life. Symptomatic treatment with anticholinesterases and atypical neuroleptics help patients in their evolution of the disease. Anticholinergics and typical antipsychotics are agents to avoid in the treatmen of the disease because can severely damage patients. Future advances in the diagnosis and dissemination of the knowledge of the disease will contribute to generate greater research efforts to find an effective preventive and / or curative treatment. (AU)

Periodic limb movement disorder : a clinical and polysomnographic study.

Periodic limb movement disorder (PLMD) is one of the commonest neurological disorders and causes significant disability, if left untreated. However, it is rarely diagnosed in clinical practice, probably due to lack of awareness and/or lack of necessary diagnostic facilities. Restless leg syndrome (RLS), aging, pregnancy, uraemia, iron deficiency, polyneuropathy are some of the common causes of secondary PLMD. Clinical presentation, polysomnographic findings and management of six patients of PLMD have been discussed in this report.

Transplant of cultured neuron-like differentiated chromaffin cells in a parkinson's disease patient. A preliminary report

Background. Treatment of Parkinson's Disease (PD) has been attempted by others by transplanting either the patient's own adrenal medullary tissue or fetal substantia nigra into caudate or putamen areas. However, the difficulties inherent in using the patient's own adrenal gland, or the difficulty in obtaining human fetal tissue, has generated the need to find alternative methods. Methods. We report here of an alternative to both procedures by using as transplant metrial cultured human adrenal chromaffin cells differentiated into neuron-like cells by extremely low frequency magnetic fields (ELF MF). Results. The results of this study show that human differentiated chromaffin cells can be grafted into the caudate nucleus of a PD patient, generating substantial clinical improvement, as measured by the unified Rating Scale for PD, which correlated with glucose metabolism and D2 DA receptor increases as seen in a PET scan, while allowing a 70 percent de crease in L-Dopa medication. Discussion. This is the first preliminary report showing that transplants of cultured differentiates neuron-like cells can be successfully used to treat a PD patient

Management of neuroleptic malignant syndrome--a series of eight cases.

Neuroleptic Malignant Syndrome (NMS) is a rare and life threatening complication of treatment with Neuroleptic medication. Phenothiazine and butyrophenones are most frequently implicated in this syndrome even though there are case reports with other drugs. In this paper we describe the classical presentation of this syndrome in eight cases who had been on either a butyrophenone, a phenothiazine or a combination of these drugs. In addition some of our patients were also on either Lithium and/or Amitrytiline for control of depressive symptoms. Fever, muscule rigidity and elevated CPK are the important criteria for diagnosis and they were noted in all our patients. In addition four of our patients fulfilled five of the six minor criteria laid down by Levenson. Both Sinemet and Bromocriptine were found to be effective in the treatment of seven of the eight cases. However treatment with Dantroline sodium was associated with severe muscle wasting in one of our cases and the drug had to be discontinued. Two of our patients developed suspected myoglobinuria with dark coloured urine and progressive renal failure. One patient died after development of renal failure, pulmonary edema and hyperkalemia as a consequence of this disorders.