Akademik Veri Yönetim Sistemi
TURKISH JOURNAL OF NEUROLOGY, cilt.19, sa.2, ss.56-59, 2013 (ESCI)
Objective: Myotonic dystrophy type 1 (DM1) is the most common adult muscular dystrophy involving not only the striated muscle but also multiple systems including central nervous system, and resulting from a gene mutation with autosomal dominant inheritance causing trinucleotid repeats. Neuroimaging findings in patients with DM1 are ventricular enlargement, hyperintensity of white matter at the posterior- superior trigone (HWMPST), hyperostosis frontalis interna and thinning of corpus callosum. Although ventriculomegaly is reportedly the most common finding, DM1 in association with normal pressure hydrocephalus (NPH) has been reported in anecdotal case reports. The aim of this study is to investigate the frequency of ventriculomegaly and also clinical diagnosis of NPH in patients with DM1 in a retrospective case series. Material and Method: Neuroimaging (mostly cranial MRI) findings in 20 patients (9 women; mean age: 38.25 years +/- 10.26[ range: 18- 60]; mean disease duration: 18.9 years +/- 10.5 [5-43]) with DM1 diagnosed with clinical and electromyographic findings are examined retrospectively. Results: The available neuroimaging data came from MRI scans in 19 cases and CT scans in the remaining case. Ventriculomegaly as decided by increased ventriculocerebral index (VCI) was evident in only two patients who were siblings, one of whom also had the classical NPH clinical triad. After a ventriculoperitoneal shunt his urinary incontinence and gait difficulty had improved. Discussion: The increase in the serum levels of MDA, TOS, and TAS in ICH patients may demonstrate that there is an increase in oxidative stress and this supports the fact that that oxidative stress may play a significant role in the pathogenesis of the ICH. However, the increase of these parameters was not found to be associated with hematoma volume and GCS in patients with