We report a severe case of Dentatorubro-pallidoluysian atrophy (DRPLA) presenting with microcephaly, developmental delay, severe epilepsy, and progressive mental deterioration with a very early onset of disease. The case is notable for the early detection of white matter changes by brain MRI. Neuroradiological findings from the case were compared to those of previously reported patients with disease onset before 10 years of age.
Although there is a substantial body of evidence implicating Myodil or Pantopaque as a cause of lumbar arachnoiditis, assessment of the clinically based evidence is complicated by the additional potentially causative factors present in a high proportion of cases. These include pre-existing spinal pathology, traumatic lumbar puncture and surgery. The aim of this retrospective study was to attempt to ascertain whether Myodil introduced via ventricular catheter was associated with subsequent development of symptomatic lumbar arachnoiditis. In 222 patients in whom clinical records were reviewed there was no excess of back pain following ventriculography compared to the general population. Myodil ventriculography does not appear to be a major cause of symptomatic lumbar arachnoiditis. Several unavoidable problems with the methodology of this study are discussed.
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