Results 151 to 160 of about 5,960 (193)
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Photic Reflex Myoclonus: A Neurophysiological Study in Progressive Myoclonus Epilepsies
Epilepsia, 1999Summary:Purpose: To investigate the neurophysiological features of photic reflex myoclonus (PRM) in patients with progressive myoclonus epilepsies (PMEs) of different types (Unverricht‐Lundborg disease, Lafora's disease, cryptogenic).Methods: All patients underwent computerized video‐polygraphic recordings, collecting electromyographic (EMG) activity
G. Rubboli +6 more
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Antimyoclonic effects of alcohol in progressive myoclonus epilepsy
Neurology, 1990We studied the effect of a single oral intake of 30 to 40 grams of alcohol in 4 consecutive patients presenting with Ramsay Hunt syndrome, the most common type of degenerative progressive myoclonic epilepsy (PME) encountered in southern Europe. Clinical and polygraphic monitoring demonstrated abatement of myoclonus in all patients, although the degree ...
GENTON P, GUERRINI, RENZO
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Progressive myoclonus epilepsy
Human Genetics, 1979The syndrome of myoclonus, epilepsy, and mental deficiency is observed in a number of distinct nosologic entities differing with respect to clinical course, (--) pathologic, and biochemical findings. Genetically, the heterogeneity within this group of disorders is shown by the occurrence of autosomal recessive and dominant forms with incomplete ...
T F, Wienker +2 more
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Advances in Lafora progressive myoclonus epilepsy
Current Neurology and Neuroscience Reports, 2007Abstract Lafora progressive myoclonus epilepsy is an autosomal recessive, fatal, generalized polyglucosan storage disorder that occurs in childhood or adolescence with stimulus sensitive epilepsy (resting and action myoclonias, grand mal, and absence), dementia, ataxia and rapid neurologic deterioration.
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Myoclonus and seizures in progressive myoclonus epilepsies: pharmacology and therapeutic trials
Epileptic Disorders, 2016AbstractGeneralized motor seizures, usually tonic‐clonic, tonic‐vibratory, myoclonic or clonic, and stimulus‐sensitive/action myoclonus are typical features of progressive myoclonus epilepsies (PMEs). Despite the introduction of many anticonvulsants, the treatment of these symptoms, particularly myoclonus, remains challenging, due to the incomplete and
Roberto, Michelucci +5 more
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Progressive Myoclonus Epilepsies: Specific Causes and Diagnosis
New England Journal of Medicine, 1986RECENT advances in the diagnosis and classifcxation of epileptic seizures and epileptic syndromes, together with improvemenss in anticonvulsant therapy, have enabled the great majority of patients ...
S F, Berkovic +3 more
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Urinary glycosaminoglycans in patients with progressive myoclonus epilepsy
Journal of Neurology, 1979Urinary GAGs analysis in Progressive Myoclonus Epilepsy (PME) showed an accumulation of uronic acid in the fraction eluted by 1 M NaCl and 3 M NaCl. As analogous changes were found in other myoclonic and epileptic patients receiving large doses of anticonvulsant drugs, these alterations in the GAG urinary pattern were not considered a primary ...
Federico, A., D'Auria, N.
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Progressive Myoclonus with Epilepsy
Southern Medical Journal, 1972D W, Nixon, W E, Mayger
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The Clinical Challenge pf Progressive Myoclonus Epilepsy
The Nurse Practitioner, 1993Health care providers who care for patients with seizure disorders should be able to recognize progressive myoclonus epilepsy. Progressive myoclonus epilepsy is a syndrome confused with myoclonic seizures and other epilepsies. The main symptom is myoclonus, a brief involuntary muscle jerk of varying intensity that can throw a patient against a wall or ...
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Cochleovestibular investigation in progressive myoclonus epilepsy.
Acta neurologica, 1983A cochleovestibular investigation including tone audiometry, speech audiometry, impedance tests, stapedial reflex threshold, tone decay test, spontaneous nystagmus, tracking, caloric stimulation and visual suppression test, was performed in 13 patients affected by different types of progressive familial myoclonus epilepsy.
Nuti, D., Bernabei, L., Federico, A.
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