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Acharya JN, Satishchandra P, Asha T. Lafora’s disease in South India: A clinical, electrophysiologic, and pathologic study. Epilepsia 1993; 34: 476-87.

has been cited by the following article:

Article

Lafora Body Disease: A Rare Type of Progressive Myoclonic Epilepsy

1National Institute of Neurosciences (NINS), Agargaon, Dhaka, Bangladesh


American Journal of Medical Case Reports. 2016, Vol. 4 No. 3, 80-82
DOI: 10.12691/ajmcr-4-3-2
Copyright © 2016 Science and Education Publishing

Cite this paper:
Md Enayet Hussain, Afjal Momin, Mahmudul Islam, S R Haque, Mohammad Akter Hossain, Rajib Nayan Chowdhury, Md Azharul Hoque. Lafora Body Disease: A Rare Type of Progressive Myoclonic Epilepsy. American Journal of Medical Case Reports. 2016; 4(3):80-82. doi: 10.12691/ajmcr-4-3-2.

Correspondence to: Md  Enayet Hussain, National Institute of Neurosciences (NINS), Agargaon, Dhaka, Bangladesh. Email: enayetdmc@yahoo.com

Abstract

Lafora body disease is one of the inherited progressive myoclonic epilepsy (PME) syndromes. It is an autosomal-recessive disorder with onset in late childhood or early adolescence. The disease is characterized by fragmentary, symmetric, or generalized myoclonic and/or generalized tonic-clonic seizures, visual hallucinations (occipital seizures), and progressive neurologic degeneration including cognitive and/or behavioral deterioration, dysarthria, and ataxia. The frequency and intractability of seizures increase over time. Status epilepticus is common. Emotional disturbance and confusion are common at or soon after onset of seizures and are followed by dementia. Dysarthria and ataxia appear early, spasticity late. Pathologically polyglucosan inclusion bodies (Lafora body) are seen which are pathognomonic of the disease and are not seen in any other types of PMEs. Electroencephalogram (EEG) reveals slowing of background and generalized spike/polyspike-and-waves and photosensitivity. Most affected individuals die within ten years of onset, usually from status epilepticus or from complications related to nervous system degeneration.

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