Unverricht-Lundborg Disease, a Condition with Self-limited Progression: Long-term Follow-up of 20 Patients
نویسندگان
چکیده
منابع مشابه
Long-term follow-up of cortical hyperexcitability in Japanese Unverricht–Lundborg disease
PURPOSE To delineate chronological changes of cortical hyperexcitability by long-term follow-up of the amplitudes of somatosensory evoked potentials (SEPs) in patients with Japanese Unverricht-Lundborg disease (ULD). METHOD SEPs to median nerve stimulation were repeatedly examined in 7 genetically diagnosed ULD patients with the mean interval of 11.9 years. The degree of temporal changes in t...
متن کاملA pathogenetic hypothesis of Unverricht-Lundborg disease onset and progression.
Unverricht-Lundborg disease (EPM1), the most common progressive myoclonic epilepsy, is associated with a defect of cystatin B (CSTB), a protease inhibitor. We used CSTB knockout mice to test the hypothesis that EPM1 onset is related to a latent hyperexcitability and that progression depends on higher susceptibility to seizure-induced cell damage. Hippocampal slices prepared from CSTB-deficient ...
متن کاملIncrease in antimullerian hormone in long-term follow-up of patients with endometrioma after laparoscopic surgery
Abstract Background: Endometriosis is a common disease which is characterized by the development of the endometrial tissue outside the uterus. The most common location of endometriosis is the ovary, occurring in 17-44% of affected patients . It is a chronic disease mostly affecting women at reproductive age. Therefore, it is important to predict and protect the patients’ ovarian function. The...
متن کاملA Native Haitian Woman with Unverricht-Lundborg Disease
Unverricht-Lundborg disease (ULD) is an autosomal recessive progressive myoclonic epilepsy. The prevalence is highest in specific European countries and North Africa. Affected individuals have myoclonic and tonic-clonic seizures and a variable degree of ataxia and cognitive impairment. We report a native Haitian woman with ULD who was wheelchair bound due to nearly continuous myoclonic seizures...
متن کاملRate of disease progression during long-term follow-up of patients with late-onset Pompe disease.
To determine the rate of disease progression in patients with late-onset Pompe disease, we collected longitudinal data on pulmonary function and skeletal muscle strength in 16 patients whose symptoms had started in childhood or adulthood. The mean duration of follow-up was 16 years (range 4-29 years). During the follow-up period, eight patients (50%) became wheelchair bound and three (19%) beca...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Epilepsia
سال: 2006
ISSN: 0013-9580,1528-1167
DOI: 10.1111/j.1528-1167.2006.00553.x