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Unverricht-Lundborg Syndrome (Disease, Unverricht)

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An autosomal recessive condition characterized by recurrent myoclonic and generalized seizures, ATAXIA, slowly progressive intellectual deterioration, dysarthria, and intention tremor. Myoclonic seizures are severe and continuous, and tend to be triggered by movement, stress, and sensory stimuli. The age of onset is between 8 and 13 years, and the condition is relatively frequent in the Baltic region, especially Finland. (From Menkes, Textbook of Child Neurology, 5th ed, pp109-110)

Also Known As:

Disease, Unverricht; Baltic Myoclonus Epilepsy; Unverricht Disease; Baltic Myoclonus Epilepsies; Diseases, Unverricht; Epilepsies, Baltic Myoclonus; Epilepsy, Baltic Myoclonus; Epilepsy, Mediterranean Myoclonic; Lundborg Unverricht Syndrome; Myoclonic Epilepsy, Mediterranean; Myoclonus Epilepsies, Baltic; Myoclonus Epilepsy, Baltic; Myoclonus, Baltic; Syndrome, Lundborg-Unverricht; Syndrome, Unverricht-Lundborg; Unverricht Diseases; Unverricht Lundborg Syndrome; Baltic Myoclonus; Lundborg-Unverricht Syndrome; Mediterranean Myoclonic Epilepsy

Networked: 7 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Multiple Acyl Coenzyme A Dehydrogenase Deficiency
2.	Cerebral Amyloid Angiopathy (Congophilic Angiopathy)
3.	Myoclonus (Nocturnal Myoclonus)
4.	Metachromatic Leukodystrophy (Sulfatide Lipidosis)
5.	Leigh Disease (Leigh's Disease)

Experts

1.	Holmes, David: 1 article (07/2015)
2.	Staniforth, Rosemary A: 1 article (07/2015)
3.	Davis, Peter J: 1 article (07/2015)
4.	Waltho, Jonathan P: 1 article (07/2015)
5.	McMahon, J T: 1 article (06/2001)
6.	Abramovich, C M: 1 article (06/2001)
7.	Cohen, B H: 1 article (06/2001)
8.	Prayson, R A: 1 article (06/2001)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Unverricht-Lundborg Syndrome:

1.	Cystatin BIBA 01/01/1999 - "Cystatin B mutations are now known to account for both Mediterranean myoclonus and for "Baltic" myoclonus, described mainly from Finland, thus solving a long-term controversy and proving that these two disorders are one single disease entity. " 07/31/2015 - "Cystatin C amyloids cause a hereditary form of cerebral amyloid angiopathy whilst cystatin B aggregates are found in cases of Unverricht-Lundborg Syndrome, a progressive form of myoclonic epilepsy. "
2.	Phenytoin (Dilantin)FDA LinkGeneric 08/01/1987 - "Effect of phenytoin on the mental and physical function of patients with Baltic myoclonus epilepsy." 10/08/1983 - ""Baltic" myoclonus epilepsy: hereditary disorder of childhood made worse by phenytoin."
3.	Cystatin CIBA 07/31/2015 - "Cystatin C amyloids cause a hereditary form of cerebral amyloid angiopathy whilst cystatin B aggregates are found in cases of Unverricht-Lundborg Syndrome, a progressive form of myoclonic epilepsy. "
4.	HomocystineIBA 06/01/2001 - "A clinical or biochemical diagnosis was reached in 11 patients: metachromatic leukodystrophy (n = 2), electron transport chain abnormalities (n = 2), glutaric aciduria type II (n = 1), Unverricht disease (n = 1), Lennox-Gastaut syndrome (n = 1), ketotic hypoglycemia of childhood (n = 1), probable Leigh disease (n = 1), 5-methyl tetrahydrofolate homocystine methyltransferase deficiency (n = 1), and pyruvate dehydrogenase deficiency (n = 1). "
5.	Adrenocorticotropic Hormone (ACTH)FDA Link 09/01/1961 - "[ACTH in the Unverricht-Lundborg syndrome]."
6.	Pyruvate dehydrogenase deficiencyIBA 06/01/2001 - "A clinical or biochemical diagnosis was reached in 11 patients: metachromatic leukodystrophy (n = 2), electron transport chain abnormalities (n = 2), glutaric aciduria type II (n = 1), Unverricht disease (n = 1), Lennox-Gastaut syndrome (n = 1), ketotic hypoglycemia of childhood (n = 1), probable Leigh disease (n = 1), 5-methyl tetrahydrofolate homocystine methyltransferase deficiency (n = 1), and pyruvate dehydrogenase deficiency (n = 1). "
7.	Lennox-Gastaut type Epileptic encephalopathyIBA 06/01/2001 - "A clinical or biochemical diagnosis was reached in 11 patients: metachromatic leukodystrophy (n = 2), electron transport chain abnormalities (n = 2), glutaric aciduria type II (n = 1), Unverricht disease (n = 1), Lennox-Gastaut syndrome (n = 1), ketotic hypoglycemia of childhood (n = 1), probable Leigh disease (n = 1), 5-methyl tetrahydrofolate homocystine methyltransferase deficiency (n = 1), and pyruvate dehydrogenase deficiency (n = 1). "
8.	pentigide (HEPP)IBA 03/18/1967 - "[Wagner-Hepp-Unverricht disease (dermatomyositis). "
9.	5,6,7,8-tetrahydrofolic acid (tetrahydrofolate)IBA 06/01/2001 - "A clinical or biochemical diagnosis was reached in 11 patients: metachromatic leukodystrophy (n = 2), electron transport chain abnormalities (n = 2), glutaric aciduria type II (n = 1), Unverricht disease (n = 1), Lennox-Gastaut syndrome (n = 1), ketotic hypoglycemia of childhood (n = 1), probable Leigh disease (n = 1), 5-methyl tetrahydrofolate homocystine methyltransferase deficiency (n = 1), and pyruvate dehydrogenase deficiency (n = 1). "